Kaposi sarcoma: clinical guidelines

List of Abbreviations

HIV – human immunodeficiency virus

WHO – World Health Organization

IU – International Unit

GI – gastrointestinal tract

ICD – International Classification of Diseases

ICD-10 – International Classification of Diseases 10th Revision

KS – Kaposi sarcoma

AIDS – acquired immunodeficiency syndrome

HHV-8 (human herpesvirus 8) – human herpesvirus type 8

LANA-1 (latency-associated nuclear antigen-1) – latency-associated nuclear antigen-1

ORF (open reading frame) – open reading frame

vFLIP (viral FLICE-inhibitory protein) – viral FLICE-inhibitory protein

** – vital and essential drugs

# – a drug used not in accordance with the approved indications, contraindications, routes of administration, or dosages contained in the product leaflet (off-label)

Terms and Definitions

Kaposi sarcoma is a multicentric neoplastic process developing from the endothelium of blood and lymphatic vessels, predominantly affecting the skin and involving internal organs.

HHV-8 (human herpesvirus 8) is a gammaherpesvirus associated with Kaposi sarcoma.

ECOG (Eastern Cooperative Oncology Group) scale: a 5-point grading system recommended by the Eastern Cooperative Oncology Group for assessing the general condition of a cancer patient before, during, and after treatment.

PD-1 inhibitor – a monoclonal antibody that blocks the interaction between the programmed cell death protein 1 (PD-1) receptor and its ligands.

Prehabilitation – rehabilitation from the moment of diagnosis until the start of treatment (surgery/chemotherapy/radiation therapy).

First stage of rehabilitation – rehabilitation during the specialized treatment of the underlying disease (including surgery/chemotherapy/radiation therapy) in departments of medical organizations specialized in the primary disease profile.

Second stage of rehabilitation – rehabilitation in inpatient settings of medical organizations (rehabilitation centers, rehabilitation departments) during the early recovery period, late rehabilitation period, or the period of residual effects of the disease.

Third stage of rehabilitation – rehabilitation during the early and late rehabilitation periods and the period of residual effects of the disease in departments (rooms) for rehabilitation, physiotherapy, exercise therapy, reflexology, manual therapy, psychotherapy, medical psychology, and speech therapy (defectology) providing outpatient medical care, day care settings, as well as by mobile teams at home (including in sanatorium-resort organizations).

1. Brief information on the disease or condition

1.1 Definition of the disease or condition

According to the WHO definition, Kaposi sarcoma is a locally aggressive endothelial proliferation that typically manifests as cutaneous lesions in the form of multiple macules, plaques, or nodules, but may also involve mucosal sites, lymph nodes, and internal organs. KS is always associated with HHV-8 infection and represents an example of virus-induced vascular proliferation. According to the WHO classification, it belongs to the category of vascular tumors.

The skin is most frequently affected. Mucosal membranes, lymph nodes, and internal organs may also be involved, sometimes without cutaneous lesions. Involvement of the brain and bones is rare, even in disseminated disease [1, 13, 14, 71–77].

1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)

The cause of Kaposi sarcoma (KS) is human herpesvirus type 8 (HHV-8, KSHV – Kaposi sarcoma herpesvirus), which belongs to the subfamily Gammaherpesvirinae, genus Rhadinovirus.

HHV-8 is subdivided into three variants: A, B, and C, based on nucleotide sequence differences in genome subsegments.

Variant A is associated with classical KS and AIDS-related skin and internal organ lesions, while variants B and C are associated with lymphoproliferative disorders (lymphomas, generalized lymphadenopathies, Castleman disease).

Like other herpesviruses, gene expression depends on the stage of infection: latent or lytic. During latent infection, the HHV-8 genome exists as an episome and expresses the products of 3 viral genes: ORF 72 (viral cyclin D, which inhibits apoptosis of infected cells), ORF 73 (LANA-1), and K13 (vFLIP). LANA-1 promotes viral dissemination and transcription in cells by attaching HHV-8 DNA to chromatin H1 histone. LANA-1 inhibits the transcriptional activity of p53, while vFLIP protects latently HHV-8-infected cells from apoptosis by blocking the Fas receptor and destruction by cytotoxic T lymphocytes. The product of the ORF 50 gene (RTA) initiates the lytic phase of the HHV-8 life cycle. Following the initiation of lytic replication, gene products are synthesized in an ordered sequence typical of other human herpesviruses. It has been established that up to 10% of HHV-8 viruses detected in KS lesions are in the lytic phase; however, HHV-8 in peripheral blood lymphocytes is generally detected only in patients with marked immunosuppression (AIDS-associated and immunosuppressive disease types). The oropharynx is the reservoir for the most pronounced viral replication; saliva contains a large number of HHV-8 copies.

HHV-8 (KS-associated herpesvirus) is detected in endothelial cells in all forms of the disease; it is a DNA virus that encodes the latent nuclear antigen, a product of the viral gene ORF73.

Sequencing revealed a correlation between HHV-8 genetic variants and patient populations (Europe, Africa, North America). HHV-8 is transmitted mainly through sexual contact. HHV-8 DNA is detected in all forms of KS (>95% of AIDS-related and non-AIDS-related KS). Antibodies against HHV-8 nuclear antigens appear early, prior to the clinical manifestations of KS, and can be detected in the peripheral blood. Not all HHV-8 infections lead to clinical manifestations of KS, and the majority of primary infections are asymptomatic. Not all seropositive individuals develop KS. The presence of HHV-8 infection is necessary but not sufficient for the development of KS, which results from a complex interaction of genetic, immunological, and environmental factors.

1.3 Epidemiology of the Disease or Condition

The incidence of the classical form of Kaposi sarcoma (KS) varies significantly across different geographical regions, ranging from 0.14 per 1 million inhabitants (both men and women) to 10.5 per 1 million men and 2.7 per 1 million women. Men are affected 3 times more often. The onset of KS is recorded at 35–39 years of age in men and 25–39 years of age in women. Men are affected 3–10 times more often. The onset of KS is recorded at 50–70 years of age [1, 2]. A higher incidence of KS is observed in countries bordering the Mediterranean Sea and in Central Africa (endemic Kaposi sarcoma). In developed countries among human immunodeficiency virus (HIV)–1 infected patients, HHV-8 detectability is highest among gay and bisexual men.

Transplant-associated KS is observed following organ transplantation, predominantly kidney transplantation. KS may also develop in patients receiving immunosuppressive therapy for autoimmune diseases [3, 4]. Kaposi sarcoma is the most common AIDS-defining tumor in men who have sex with men and is observed in 20% of AIDS patients.

One of the main routes of transmission of HHV-8 is sexual, with the risk of infection increasing with the number of sexual partners and practice of non-traditional sexual contacts. The non-sexual mechanism of viral transmission via saliva is widely recognized and plays an important role in infecting children in endemic areas. The vertical transmission route of HHV-8 from mother to foetus during pregnancy and childbirth is not of major importance for viral dissemination. HHV-8 can also be transmitted via blood, blood products, and donor organs following transplantation. The HHV-8 viral genome is detected in all KS lesions across all disease stages regardless of the clinical variant [1]. The virus is widespread in the population: from approximately 1–3% of blood donors in North America to up to 70% in regions of Africa where HHV-8 is endemic; 90% of HIV-infected individuals have antibodies to HHV-8 lytic proteins, and serum antibodies to this virus were detected in 9.6% of healthy donors in Russia. The prevalence of HHV-8 infection roughly reflects the prevalence of KS.

1.4 Features of Coding the Disease or Condition

According to the International Statistical Classification of Diseases and Related Health Problems (hereinafter referred to as ICD-10), such tumors are classified according to the organ of primary origin.

Kaposi sarcoma (C46, B21.0)

C46.0 – KS of skin

C46.1 – KS of soft tissue

C46.2 – KS of palate

C46.3 – KS of lymph nodes

C46.7 – KS of other sites

C46.8 – KS of multiple organs

C46.9 – KS, unspecified

B21.0 – HIV disease resulting in Kaposi sarcoma

1.5 Classification of the disease or condition

International Histological Classification (WHO Classification of Tumours of Soft Tissue and Bone, 5th edition, 2020):

according to the ICD-O morphological code 9140/3 [16, 17]

− classical KS (idiopathic, sporadic, European);

− endemic KS (African);

− epidemic KS associated with AIDS;

− immunosuppression-associated KS (iatrogenic, occurring during immunosuppressive therapy, particularly after organ transplantation).

There is no universally accepted staging classification for classical, endemic, and immunosuppression-associated variants of Kaposi sarcoma.

Comment: The only approved and validated staging system for AIDS-related KS. It was developed by the AIDS Clinical Trials Group Oncology Committee in the pre-combination antiretroviral therapy era and included measuring the extent of disease (T - tumor) as localized or disseminated; the state of the immune system (I - immunodeficiency) based on CD4 cell count (high or low); and the presence or absence of symptoms and systemic diseases (S - systemic diseases/conditions) [1, 2, 3, 16]. In the post-combination antiretroviral therapy era, only T and S are recommended to identify patients with a poor prognosis (Table 1).

Table 1. Staging classification of AIDS-related KS [1, 2, 3]

1.6 Clinical presentation of the disease or condition

Classic (idiopathic) KS

The typical localization of lesions in the idiopathic variant of KS is the skin of the distal lower extremities. It is characterized by the appearance of symmetric or asymmetric bluish-red macules, prone to peripheral and gradual vertical growth. In most cases, the course is subjectively asymptomatic. With KS progression, macular elements sequentially transform into papular and plaque forms, and less frequently, nodular forms. Neoplasms grow slowly, without subjective sensations; some may remain unchanged in size for years, although rapidly progressing forms are also described. Pain may appear after 2-10 years upon ulceration of the nodules. Progression of KS is accompanied by dissemination of skin lesions and rarely by metastasis to the lymph nodes and gastrointestinal mucosa [1].

Endemic (African) KS

It predominantly affects children and young adults, and is characterized by a more "aggressive" and fulminant course. This may be accompanied by the appearance of numerous painful subcutaneous nodules and ulcers, along with internal organ dysfunction.

AIDS-related KS

This form is characterized by either a rapid or indolent course, depending on the level of HIV control. Initially, rashes appear on the scalp (nose, eyelids, auricles) and trunk. The rashes are initially painless, later becoming painful and leading to impaired organ function.

Depending on the localization of the process, both localized and disseminated forms occur.

The clinical picture is characterized by a symptom complex of the affected zones: skin manifestations, or involvement of visceral organs or mucous membranes.

Immunosuppression-associated KS

Skin rashes are limited in character and asymptomatic. Subsequently, the spread of rashes is accompanied by the formation of nodules and their ulceration, leading to intense local pain and resembling the manifestations of AIDS-related KS. Reducing the dose or discontinuing immunosuppressive drugs leads to complete regression of skin and visceral manifestations of KS in 24–80% of patients, although resuming their use leads to disease relapse.

Clinical manifestations of various KS subtypes are summarized in Table 2.

Table 2. Clinical manifestations of KS [6, 14, 17–21, 71–77].

2. Diagnostics, medical indications and contraindications for diagnostic methods

2.1 Complaints and Anamnesis

The course and severity of clinical symptoms depend on the disease variant. Subjective clinical manifestations of Kaposi sarcoma are described in the "Clinical Presentation" section.

2.2 Physical Examination

Classic (idiopathic) KS (no gender differences)

Criteria for establishing the diagnosis/condition: based on pathognomonic physical examination findings:

- single or bilateral bluish-red macules 1–5 cm in diameter with distinct borders, resembling a hematoma, appear on the skin of the distal lower extremities;

- then the elements grow slowly, transforming into firm papules (forming plaques upon coalescence) and nodules;

- large nodules on the lower extremities darken (due to hemosiderin deposition) and ulcerate, causing sloughing of necrotic tissues;

- pronounced edema with lymphedema phenomena is observed at the periphery of the nodules (fibrotic areas form subsequently);

- spread of rashes to other areas of the body, lymph nodes, oral and genital mucosa, and internal organs (often with an asymptomatic course);

- death in classic KS most often results from other causes, as the disease is observed in elderly individuals and typically progresses slowly.

Endemic (African) KS

Criteria for establishing the diagnosis/condition: based on pathognomonic physical examination findings:

- KS manifests in nodular, "florid", infiltrative, and lymphadenopathic variants;

- the "florid", vegetative, and infiltrative variants are characterized by a more aggressive course with deep involvement of the dermis, subcutaneous tissue, muscles, and bones;

- African KS can affect children, young adults, and adults. In adults, there is a male predisposition, whereas in children, there is no sex predilection. The skin and mucous membranes are affected to a lesser extent. In adulthood, internal organ involvement is frequently observed, while in children, lymph nodes and internal organs are primarily affected. It can have a fulminant course with very rapid progression.

Epidemic KS (AIDS-related)

Criteria for establishing the diagnosis/condition: based on pathognomonic physical examination findings:

- A rapid course with multifocal dissemination is characteristic;

- Initial manifestations of KS on the face are more frequently observed on the skin of the nose, eyelids, and auricles; on the trunk, they occur along skin tension lines;

- In the absence of treatment, disseminated lesions may coalesce to form large plaques spreading to the skin of the face, trunk, or extremities, which frequently leads to organ dysfunction;

- The oral mucosa is affected in 10-15% of patients. Eruptions are most typically localized in the hard and soft palate and the base of the tongue, where they rapidly ulcerate;

- Pharyngeal involvement is frequently observed and may lead to difficulty in eating, speaking, and breathing;

- The stomach and duodenum are common sites of KS localization, and the disease is frequently accompanied by bleeding and intestinal obstruction;

- In the pulmonary form of KS, respiratory symptoms may be observed: bronchospasm, cough, and progressive respiratory failure.

Immunosuppression-associated KS

Criteria for establishing the diagnosis/condition: based on pathognomonic physical examination findings: develops after internal organ transplantation and is caused by the iatrogenic effect of immunosuppressants (L04A);

- Skin manifestations initially have a localized character, then spread and resemble the rashes of epidemic KS.

Reduction of the dose or discontinuation of immunosuppressants (L04A) leads to complete regression of skin and visceral manifestations of KS in 24-80% of patients; however, resumption of their use leads to a recurrence of the disease.

2.3 Laboratory Diagnostics

Diagnosis of KS is based on physical examination data—identifying typical manifestations for the respective stages of KS; dermoscopy reveals a bluish-reddish coloration, a "rainbow pattern", a scaly surface, and small brown globules. The diagnosis is confirmed by pathomorphological examination of biopsy (surgical) material. Considering the high frequency of association of the disease with AIDS, serological testing for HIV is necessary. If KS is suspected, screening for specific antibodies to HHV-8 is indicated.

In disseminated forms, additional laboratory and instrumental examinations are indicated to assess visceral manifestations of KS.

Comments: Pathomorphological examination distinguishes angiomatous, fibroblastic, and mixed variants of KS. Macular lesions show an increase in the number of dermal vessels surrounded by endothelial cells, with adjacent areas containing hemosiderin deposits and extravasated erythrocytes with a moderate perivascular infiltrate. In KS plaques, pronounced vascular proliferation is detected in all structures of the dermis, including collagen fibers. The presence of dense bundles and spindle cells between jagged vascular cells is characteristic. In the nodules of KS patients, predominantly spindle cells, areas of angiomatosis, and dense sarcomatous fibers are determined. The inflammatory infiltrate consists of lymphocytes, histiocytes, and plasma cells. Immunohistochemically, KS reveals the expression of vascular markers CD31, CD34, and the lymphatic marker D2-40 (podoplanin) [1].

2.4 Instrumental Diagnostics

Comments: dermoscopy of KS reveals the following dermoscopic features: bluish-red coloration (in 84% of lesions), "rainbow pattern" (36%), scaly surface, and small brown globules (15%). The multicolored rainbow pattern is the most significant dermoscopic pattern detected by a polarized dermatoscope. The rainbow pattern is not observed in other vascular neoplasms.

2.5 Other Procedures

3. Treatment, including drug and non-drug therapies, diet therapy, and pain management

General treatment comments

Treatment methods for Kaposi sarcoma depend on the clinical form of the disease, the size of the lesions, and the organs and/or systems involved in the pathological process, as well as the patient's WHO/ECOG performance status.

Despite the proven role of oncogenic herpesvirus infection caused by HHV-8 in the pathogenesis of Kaposi sarcoma, J05AB nucleosides and nucleotides, other than reverse transcriptase inhibitors (antiherpetic agents), are not used due to their low efficacy [49].

3.1. Surgical treatment

Comments It must be taken into account that due to the high recurrence rate, this procedure should be performed for well-demarcated and superficial tumors. This treatment option should not be considered as primary for extensive lesions. The principles of non-melanoma skin cancer surgery should be applied with adequate margins and plastic surgery stages as needed. Surgical margins should be evaluated. In case of positive margins, re-operation is advisable. When single lesions are located in complex anatomical areas (face, hand), surgical excision with intraoperative margin assessment should be preferred.

3.2. Medical treatment

#doxorubicin** 20 mg/m² (liposomal) every 3 weeks, treatment duration is determined by achieving the maximum effect followed by 2 consolidation courses of chemotherapy [14, 15, 45, 77].

GGR CLOE 5Grade of Recommendation C (Level of Evidence 5)

#Paclitaxel** 80 mg/m² weekly or 100 mg/m² every 2 weeks until response is achieved, under the control of laboratory and functional parameters [14, 15, 45, 77, 84-86].

GGR CLEL 5Grade of Recommendations C (Level of Evidence 5)

#pomalidomide** 5 mg once daily from days 1 to 21, cycle every 28 days [47, 48, 77]

GGR BLEL 3Grade of Recommendations B (Level of Evidence 3)

Comments: In clinical trials of #pomalidomide**, a dose of 5 mg/day was also used as first-line therapy. However, #pomalidomide** is supplied in a 4 mg dose, and the expert co-authors of the clinical guidelines for KS treatment, as well as NCCN experts, consider 4 mg to be a sufficient dose [54, 77].

# Interferon alfa-2b** – 3 or 6 million IU subcutaneously 3 times a week – 6 months [14, 82]

GGR CLEL 5Grade of Recommendations C (Level of Evidence 5)

1) #gemcitabine** 1000 mg/m² IV on day 1 and day 8, cycle every 21 days or 1200 mg IV on day 1 and day 8, cycle every 21 days [2, 14, 26, 77, 80, 81]

GGR CLEL 4Grade of Recommendations C (Level of Evidence 4)

2) Etoposide** 50 mg orally daily for 7 days, cycle of 14 days; after 2 cycles, if tolerability is satisfactory, dose escalation to 100 mg p.o. daily for 7 days is possible, cycle every 14 days [14, 28, 77]

GCR CLOCE 5Grade of Recommendation C (Level of Evidence 5)

Comments: for the treatment of patients with Kaposi's sarcoma, the dose of etoposide** is established individually, depending on the line of therapy, the patient's hematological parameters, and the patient's age. Due to the rare incidence, most drug therapy studies have been conducted in the population of patients with HIV infection, where KS is more common, but chemotherapy efficacy data are also used for patients without HIV. The indicated doses only partially correspond to the package insert for oral etoposide** registered in the Russian Federation.

3) #Imatinib** 400 mg daily p.o. long-term for up to 12 months of therapy with a possible dose increase to 600 mg/day after 3 months if disease stabilization is noted. [24, 29, 77]

GGR CLEL 5Grade of Recommendations C (Level of Evidence 5)

Comment: this regimen was studied in a phase II trial for patients with HIV-associated Kaposi's sarcoma; the expert community believes that these data can be extrapolated to patients with KS without HIV due to the rare occurrence of this nosological entity. This is confirmed by the inclusion of this treatment option in the NCCN guidelines.

GGR CLEL 5Grade of Recommendations C (Level of Evidence 5)

# pembrolizumab** 200 mg IV infusion once every 21 days, long-term [60-62, 77].

GCR CLEL 4Grade of Recommendations C (Level of Evidence 4)

3.3. Other local treatment options

Cryodestruction of KS skin lesions [6, 14, 77, 83]

LeR CLoE 5Level of Recommendation C (Level of Evidence — 5)

Application radiation therapy with the production and application of individual applicators. 3D-4D planning, Intraoperative radiation therapy, Conformal external beam radiation therapy, Conformal external beam radiation therapy with neutron, proton and heavy ion beams [51]

GCR CLEL 5Grade of Recommendations C (Level of Evidence 5)

Comments: the choice of radiation type and fields depends on the localization and number of KS lesions.

Radiation therapy is one of the most effective local treatment methods and is used to treat all variants of locally advanced KS. The response rate to radiation therapy ranges from 47% to 99% [1-8, 31-38]. Recommended radiation therapy doses are 30-30 Gy at 2-3 Gy daily. Doses exceeding 3 Gy per fraction combined with systemic therapy should be avoided to reduce the risk of late complications.

The use of radiation therapy should be discussed at a multidisciplinary tumor board. Radiotherapy for lesions localized on the plantar surface of the foot and palmar surface of the hand is limited by side effects. In all cases, radiotherapy should be considered when systemic therapy is ineffective and surgery is not feasible.

#Imiquimod (5%) on KS lesions, 3 times a week, for at least 16 weeks [9, 14, 45, 53, 77-79]. Grade of Recommendations C (Level of Evidence 5)

3.4 Pain Management

The principles of pain management and the optimal selection of analgesic therapy in patients with Kaposi's sarcoma with chronic pain syndrome comply with the pain management principles outlined in the clinical guidelines "Chronic Pain Syndrome in Adult Patients Requiring Palliative Medical Care".

3.5 Concomitant therapy in patients with Kaposi sarcoma

The principles of prevention and treatment of anemia in patients with Kaposi's sarcoma comply with the principles outlined in the clinical guidelines "Anemia in Malignant Neoplasms", available in the rubricator of clinical guidelines of the Russian Ministry of Health https://cr.minzdrav.gov.ru

The principles of treatment and prevention of nausea and vomiting in patients with Kaposi's sarcoma comply with the principles outlined in the guidelines "Prevention and Treatment of Nausea and Vomiting". Authors: Vladimirova L.Yu., Gladkov O.A., Koroleva I.A., Rumyantsev A.A., Semiglazova T.Yu., Tryakin A.A. et al. DOI: 10.18027/2224-5057-2023-13-3s2-2-29-44, https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-38.pdf

The principles of treatment and prevention of bone complications in patients with Kaposi's sarcoma comply with the principles outlined in the guidelines "Bone Pathology". Authors: Bagrova S.G., Basin E.M., Borzov K.A., Bychkova N.M., Dengina N.V., Kopp M.V., Krylov V.V., Kochetova T.Yu., Semiglazova T.Yu. DOI: 10.18027/2224-5057-2023-13-3s2-2-44-59. https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-39.pdf

The principles of prevention and treatment of febrile neutropenia in patients with Kaposi's sarcoma comply with the principles outlined in the guidelines "Febrile Neutropenia". Authors: Sakaeva D.D., Borisov K.E., Bulavina I.S., Kogonia L.M., Kurmukov I.A., Orlova R.V., Shabaeva M.M. DOI: 10.18027/2224-5057-2023-13-3s2-2-60-68. https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-40.pdf

The principles of prevention and treatment of hepatotoxicity in patients with Kaposi's sarcoma comply with the principles outlined in the guidelines "Hepatotoxicity". Authors: Tkachenko P.E., Ivashkin V.T., Maevskaya M.V. DOI: 10.18027/2224-5057-2023-13-3s2-2-69-82. https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-41.pdf

The principles of prevention and treatment of cardiovascular complications in patients with Kaposi's sarcoma comply with the principles outlined in the guidelines "Cardiovascular Toxicity". Authors: Vitsenya M.V., Ageev F.T., Orlova R.V., Poltavskaya M.G., Potievskaya V.I. DOI: 10.18027/2224-5057-2023-13-3s2-2-83-107. https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-42.pdf

The principles of prevention and treatment of skin complications in patients with Kaposi's sarcoma comply with the principles outlined in the guidelines "Dermatological Reactions". Authors: Koroleva I.A., Bolotina L.V., Gladkov O.A., Gorbunova V.A., Kogonia L.M., Kruglova L.S., Orlova E.V., Orlova R.V. DOI: 10.18027/2224-5057-2023-13-3s2-2-108-131. https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-43.pdf

The principles of nutritional support in patients with Kaposi sarcoma correspond to the principles outlined in the clinical guidelines "Nutritional Support". Authors: Sytov A.V., Zuzov S.A., Kukosh M.Yu., Leiderman I.N., Obukhova O.A., Potapov A.L., Khoteev A.Zh. DOI: 10.18027/2224-5057-2023-13-3s2-2-132-142. https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-44.pdf

The principles of prevention and treatment of nephrotoxicity in patients with Kaposi sarcoma correspond to the principles outlined in the clinical guidelines "Nephrotoxicity". Authors: Gromova E.G., Biryukova L.S., Dzhumabaeva B.T., Kurmukov I.A. DOI: 10.18027/2224-5057-2023-13-3s2-2-153-166. https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-47.pdf

The principles of prevention and treatment of thromboembolic complications in patients with Kaposi sarcoma correspond to the principles outlined in the clinical guidelines "Thromboembolic Complications". Authors: Somonova O.V., Antukh E.A., Vardanyan A.V., Gromova E.G., Dolgushin B.I., Elizarova A.L., Sakaeva D.D., Selchuk V.Yu., Tryakin A.A., Cherkasov V.A. DOI: 10.18027/2224-5057-2023-13-3s2-2-167-178. https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-48.pdf

The principles of prevention and treatment of the consequences of drug extravasation in patients with Kaposi sarcoma correspond to the principles outlined in the clinical guidelines "Extravasation of Antineoplastic Drugs". Authors: Buydenok Yu.V., Obukhova O.A. DOI: 10.18027/2224-5057-2023-13-3s2-2-179-189. https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-49.pdf

The principles of prevention and treatment of immune-mediated adverse events in patients with Kaposi sarcoma correspond to the principles outlined in the clinical guidelines "Immune-Mediated Adverse Events". Authors: Protsenko S.A., Ballyuzek M.F., Vasiliev D.A., Zhukova N.V., Novik A.V., Nosov D.A., Petenko N.N., Semenova A.I., Kharkevich G.Yu., Yudin D.I. DOI: 10.18027/2224-5057-2023-13-3s2-2-210-249. https://rosoncoweb.ru/standarts/RUSSCO/2023/2023-51.pdf

3.6 Nutritional Therapy

Diet therapy is not used for the treatment of Kaposi sarcoma.

4. Medical Rehabilitation and Sanatorium-Resort Treatment

These recommendations are based on numerous studies proving that various types of medical rehabilitation significantly accelerate functional recovery, reduce postoperative hospital stay, and decrease the incidence of complications and mortality in patients with other malignant neoplasms.

4.1 Pre-rehabilitation

Comment: It is advisable to advise the patient to increase physical activity 2 weeks prior to surgery to reduce the length of hospital stay and the risk of postoperative complications, as well as to improve postoperative quality of life [64].

4.2 First stage of rehabilitation

4.3 Second stage of rehabilitation

4.4 Third stage of rehabilitation

5. Prevention and Dispensary Observation

Adults with oncological diseases included in ICD-10 categories C00-D09 are subject to dispensary observation (follow-up).

Dispensary observation is organized at the outpatient oncological care center, or in the primary oncological office of a medical organization, oncological dispensary (oncological hospital), or other medical organizations providing medical care to patients with oncological diseases.

Comments:To date, there is no consensus regarding the frequency and intensity of monitoring for patients with Kaposi sarcoma. The goals of patient monitoring should be early detection of disease relapse, early detection of secondary tumors, and psychosocial support for patients.

The required scope of examination during the monitoring period for patients with Kaposi sarcoma depends on the KS subtype (Table 4).

Table 4. Scope of examination during the monitoring period for patients with KS after completion of therapy

Comment: the goal of monitoring is the earliest possible detection of disease progression for the prompt initiation of chemotherapy, radiation therapy, or surgical treatment, depending on the identified situation.

6. Organization of Medical Care

When Kaposi sarcoma is suspected, the dermatologist performs diagnostic tests (skin examination under magnification (dermatoscopy), and sampling of material for pathological anatomical examination of biopsy (surgical) skin material).

When Kaposi sarcoma is suspected or detected in a patient, dermatologists, as well as general practitioners, district general practitioners, general family physicians, medical specialists, and mid-level medical personnel shall, in the prescribed manner, refer the patient for a consultation to an outpatient oncological care center, or, in its absence, to a primary oncology office or the outpatient department of an oncological dispensary (oncology hospital) to receive primary specialized health care.

Consultation at an outpatient oncological care center, primary oncological office, or outpatient department of an oncological dispensary (oncological hospital) must be performed no later than 3 working days from the date of issuing the referral for consultation. The oncologist of the outpatient oncological care center (in the absence of such a center, the oncologist of the primary oncological office or outpatient department of the oncological dispensary/hospital) arranges for the acquisition of biopsy (surgical) material, as well as the performance of other diagnostic tests necessary to establish the diagnosis, including the extent of the oncological process and the stage of the disease.

If it is impossible to obtain biopsy (surgical) material or perform other diagnostic tests within the medical organization that includes the ambulatory cancer care center (primary oncology office), the patient is referred by the oncologist to an oncological dispensary (cancer hospital) or to a medical organization providing medical care to patients with oncological diseases.

The timeframe for performing pathological examinations necessary for the histological verification of malignant neoplasms shall not exceed 15 working days from the date of receipt of the biopsy (surgical) material by the pathoanatomical bureau (department).

The timeframe for diagnostic instrumental and laboratory examinations in case of suspected oncological disease shall not exceed the timeframes established in the state guarantee program for the free provision of medical care to citizens, approved by the Government of the Russian Federation, which is 7 working days from the date of prescribing the examinations.

The diagnosis of an oncological disease is established by a medical specialist based on the results of diagnostic examinations, including cytological and/or histological verification of the diagnosis, except for cases where obtaining biopsy and/or puncture material is not feasible.

An oncologist from an outpatient oncological care center (primary oncology office) refers the patient to an oncological dispensary (oncology hospital) or another medical organization providing medical care to cancer patients, including one subordinate to a federal executive body (hereinafter referred to as a federal medical organization), to clarify the diagnosis (if establishing the diagnosis is impossible, including the extent of the oncological process and disease stage), determine treatment tactics, and in the presence of medical indications for specialized, including high-tech, medical care.

For oncological diseases included in ICD-10 categories C37, C38, C40–C41, C45–C49, C58, D39, C62, C69–C70, C72, C74, as well as those corresponding to the codes of the International Classification of Diseases for Oncology (ICD-O), 3rd Edition 8936, 906-909, 8247/3, 8013/3, 8240/3, 8244/3, 8246/3, 8249/3, an oncologist at an oncological dispensary (oncological hospital) or other medical organization providing medical care to patients with oncological diseases shall organize a consultation or a medical board (consilium), including via telemedicine technologies, at federal state budgetary institutions subordinate to the Russian Ministry of Health that provide medical care (hereinafter referred to as national medical research centers for the purposes of this Procedure), in order to determine the treatment tactics.

In complex clinical cases, to clarify the diagnosis (in the event of the impossibility of establishing the diagnosis, including the extent of the oncological process and the stage of the disease) for the purpose of evaluation, interpretation, and description of the results, the oncologist shall arrange for the referral of:

digital images obtained from pathomorphological studies to the pathological anatomical bureau (department) of the fourth group (reference center)[1] via information interaction, including the use of telemedicine technologies for remote interaction between medical workers;

of digital images obtained from diagnostic imaging methods to the remote diagnostic imaging consultation center via information interaction, including the use of telemedicine technologies for remote interaction between medical workers;

biopsy (surgical) material for repeated pathomorphological, immunohistochemical, and molecular genetic studies: to the pathoanatomical bureau (department) of the fourth group (reference center), as well as to molecular genetic laboratories for molecular genetic testing.

Treatment tactics are established by a medical board (consilium) comprising oncologists, a radiation therapist, and a neurosurgeon (in cases of nervous system tumors) of the medical organization that includes departments of surgical methods for treating malignant neoplasms, antineoplastic drug therapy, and radiotherapy (hereinafter referred to as the oncological consilium), including an oncological consilium conducted using telemedicine technologies, with the involvement of other specialist physicians when necessary.

Dispensary follow-up by an oncologist for a patient with a diagnosed oncological disease is established and carried out in accordance with the procedure for dispensary observation of adults with oncological diseases.

For accounting purposes, information about a newly diagnosed case of an oncological disease is sent within 3 working days by the oncologist of the medical organization where the corresponding diagnosis was established to the oncological dispensary or the organization of a constituent entity of the Russian Federation that performs the registration of patients with newly diagnosed malignant neoplasms, including through the use of the Unified State Health Information System. If the presence of an oncological disease is confirmed in a patient, information about the refined diagnosis is sent from the oncological dispensary or the organization of the constituent entity of the Russian Federation that performs the registration of patients with newly diagnosed malignant neoplasms to the medical organization providing clinical follow-up of the patient.

Specialized, including high-tech, medical care in medical organizations providing medical care to the adult population for oncological diseases is provided based on medical indications stipulated by the regulations on organizing the provision of specialized, including high-tech, medical care.

Specialized medical care, excluding high-tech medical care, in medical organizations subordinate to federal executive authorities is provided based on medical indications provided for in clause 5 of the procedure for referring patients to medical organizations and other organizations subordinate to federal executive authorities to provide specialized (excluding high-tech) medical care, stipulated in the appendix to the regulations on organizing the provision of specialized, including high-tech, medical care. (Clause 5: to receive specialized medical care on a planned basis, the federal medical organization is chosen upon the referral of the attending physician.) If several federal medical organizations participating in the implementation of the Program provide specialized medical care for diseases and conditions (group of diseases and conditions) corresponding to the patient's diseases and conditions (group of diseases and conditions), the attending physician is obliged to inform the patient (the patient's legal representative) about the possibility of choosing a federal medical organization, including the potential waiting times for specialized medical care, which may exceed the waiting times established by the program of state guarantees of free provision of medical care to citizens.

The waiting time for the provision of specialized (excluding high-tech) medical care must not exceed the timeframe established in the program of state guarantees of free medical care for citizens, approved by the Government of the Russian Federation, which is 7 working days.

If a patient with an oncological disease has medical indications for medical rehabilitation, the oncologist arranges for it to be provided in accordance with the procedure for organizing adult medical rehabilitation.

If a patient with an oncological disease has medical indications for sanatorium-resort treatment, the oncologist arranges for it in accordance with the procedure for organizing sanatorium-resort treatment.

Palliative medical care for a patient with oncological diseases is provided in accordance with the regulations on organizing the provision of palliative medical care, including the procedure for interaction between medical organizations, social service organizations, and public associations and other non-profit organizations operating in the field of health protection.

If an oncological disease is suspected and/or detected in a patient during the provision of emergency medical care, the patient is transferred or referred to medical organizations providing medical care to patients with oncological diseases to determine management tactics and the need for additional other methods of specialized antitumor treatment.

Indications for hospitalization in a medical organization in an emergency or urgent form are:

1) the presence of complications of the oncological disease requiring the provision of specialized medical care in an emergency and urgent form;

2) presence of complications of oncological disease treatment (surgery, radiotherapy, drug therapy, etc.) requiring the provision of specialized medical care in an emergency and urgent form

Indications for hospitalization in a medical organization in a planned form are:

1) the need to perform complex interventional diagnostic medical procedures, as well as endoscopic examinations when it is impossible to perform them on an outpatient basis, requiring subsequent monitoring in a round-the-clock or day hospital setting;

2) the presence of indications for specialized antitumor treatment (surgical intervention, RT, including contact, EBRD and other types of RT, drug therapy, etc.) requiring monitoring in an inpatient or day hospital setting.

Indications for discharging a patient from a medical organization are:

1) completion of a course of treatment or one of the stages of providing specialized, including high-tech, medical care in an inpatient or day hospital setting, provided there are no treatment complications requiring drug correction and/or medical interventions in an inpatient setting;

2) refusal of the patient or their legal representative from specialized, including high-tech, medical care in an inpatient or day-care setting determined by the concilium of the medical organization providing oncological care;

3) in cases of non-compliance by the patient with prescriptions or the internal regulations of the medical and prophylactic institution, provided this does not threaten the patient's life and the health of others;

4) the need to transfer the patient to another medical organization according to the relevant medical specialty profile.

The conclusion on the advisability of transferring a patient to a specialized medical organization is made after a preliminary consultation on the provided medical documents and/or a preliminary examination of the patient by specialist physicians of the medical organization to which the transfer is planned.

7. Additional Information

Currently known prognostic factors used for staging are presented in Table 5.

Table 5. Staging classification of AIDS-related KS [1, 2, 3]

Criteria for Evaluating the Quality of Medical Care

Criteria for assessing the quality of medical care are given in Table 6.

Table 6. Criteria for assessing the quality of medical care

References

Appendix A1. Working Group Composition

1. Kubanov A.A., Corresponding Member of the RAS, Doctor of Medical Sciences, Professor, Director of the State Research Center of Dermatovenereology and Cosmetology of the Russian Ministry of Health, President of the Russian Society of Dermatovenereologists and Cosmetologists.

2. Snarskaya E.S., Doctor of Medical Sciences, Professor at the Department of Skin and Venereal Diseases, I.M. Sechenov First Moscow State Medical University.

3. Dubensky V.V., Doctor of Medical Sciences, Professor, Honored Doctor of the Russian Federation, Head of the Department of Dermatovenereology with a Course in Cosmetology, Tver State Medical University of the Russian Ministry of Health, Tver, member of the Russian Society of Dermatovenereologists and Cosmetologists.

4. Dubensky V.V., Candidate of Medical Sciences, Deputy Chief Physician for Medical Affairs, V.P. Avaev Center for Specialized Medical Care, Professor at the Department of Dermatovenereology with a Course in Cosmetology, Tver State Medical University of the Russian Ministry of Health, member of the Russian Society of Dermatovenereologists and Cosmetologists.

5. Zhuchkov M.V., Candidate of Medical Sciences, Deputy Chief Physician for Organizational and Methodological Work, Ryazan Regional Clinical Dermatovenereological Dispensary.

6. Fedenko A.A., Head of the Department of Drug Treatment of Tumors, P.A. Herzen Moscow Oncology Research Institute, branch of the National Medical Research Radiological Center of the Russian Ministry of Health.

7. Demidov L.V., Doctor of Medical Sciences, Professor, Head of the Department of Skin Tumors at the Department of Drug Treatment, N.N. Blokhin National Medical Research Center of Oncology of the Russian Ministry of Health, Chairman of the Board of the Association of Melanoma Specialists.

8. Orlova K.V., Candidate of Medical Sciences, Senior Researcher at the Department of Skin Tumors, Department of Drug Treatment, N.N. Blokhin National Medical Research Center of Oncology of the Russian Ministry of Health; member of the Board of the Association of Melanoma Specialists

9. Bokhyan B.Yu., Candidate of Medical Sciences, Senior Researcher at the Department of General Oncology, N.N. Blokhin National Medical Research Center of Oncology of the Russian Ministry of Health, member of the Eastern European Sarcoma Group

10. Bukharov A.V., Candidate of Medical Sciences, Senior Researcher, National Medical Research Radiological Center of the Russian Ministry of Health.

11. Kurilchik A.A., Candidate of Medical Sciences, Leading Researcher, A.F. Tsyb Medical Radiological Research Center, branch of the National Medical Research Radiological Center of the Russian Ministry of Health, expert of the Eastern European Sarcoma Group.

Medical Rehabilitation Section:

1. Butenko A.V., Doctor of Medical Sciences, Professor, Chairman of the Scientific Board of the Association of Specialists in Oncological Rehabilitation

2. Obukhova O.A., Candidate of Medical Sciences, Physiotherapist, Head of the Rehabilitation Department, N.N. Blokhin National Medical Research Center of Oncology of the Russian Ministry of Health, member of the European Society for Clinical Nutrition and Metabolism (ESPEN), Russian Society for Parenteral and Enteral Nutrition (RESPEN);

3. Semiglazova T.Yu., Doctor of Medical Sciences, Leading Researcher at the Research Department of Innovative Methods of Therapeutic Oncology and Rehabilitation, N.N. Petrov National Medical Research Center of Oncology of the Russian Ministry of Health, Associate Professor at the Department of Oncology, North-Western State Medical University named after I.I. Mechnikov of the Russian Ministry of Health;

4. Filonenko E.V., Doctor of Medical Sciences, Professor, Head of the Center for Laser and Photodynamic Diagnostics and Therapy of Tumors, P.A. Herzen Moscow Oncology Research Institute, branch of the National Medical Research Radiological Center of the Russian Ministry of Health, oncologist;

Healthcare Organization Section:

1. Ivanov Sergey Anatolyevich, Professor of the RAS, Doctor of Medical Sciences, Director of the A.F. Tsyb Medical Radiological Research Center, branch of the National Medical Research Radiological Center of the Russian Ministry of Health, Moscow Oblast, Obninsk.

2. Nevolskikh A.A., Doctor of Medical Sciences, Professor, Deputy Director for Clinical Work, A.F. Tsyb Medical Radiological Research Center, branch of the National Medical Research Radiological Center of the Russian Ministry of Health;

3. Khaylova Zh.V., Candidate of Medical Sciences, Chief Physician of the Clinic, A.F. Tsyb Medical Radiological Research Center, branch of the National Medical Research Radiological Center of the Russian Ministry of Health.

4. Gevorkyan Tigran Gagikovich, Deputy Director of the Research Institute of Clinical and Experimental Radiology, N.N. Blokhin National Medical Research Center of Oncology, Moscow.

No conflicts of interest.

Appendix A2. Methodology for Developing Clinical Guidelines

Target audience for these clinical guidelines:

• oncologists;

• surgeons;

• radiologists;

• medical geneticists;

• dermatovenerologists;

• pathologists;

• general practitioners (internal medicine);

• district general practitioners;

• general practitioners (family doctors);

• medical students, residents, and graduate students.

Table 1. Level of Evidence (LoE) grading scale for diagnostic methods (diagnostic interventions)

Table 2. Level of evidence (LoE) grading scale for prevention, treatment, and rehabilitation methods (preventive, therapeutic, and rehabilitative interventions)

Table 3. Grade of Recommendation (GoR) scale for prevention, diagnostic, treatment, and rehabilitation methods (preventive, diagnostic, therapeutic, and rehabilitative interventions)

Procedure for updating clinical guidelines.

The mechanism for updating clinical guidelines provides for their systematic revision at least once every three years, as well as upon the emergence of new evidence-based medicine data on the diagnosis, treatment, prevention, and rehabilitation of specific diseases, or in the presence of justified additions/comments to previously approved clinical guidelines, but no more frequently than once every 6 months.

Appendix A3. Reference Materials

These clinical guidelines were developed taking into account the following regulatory legal documents:

1. Order of the Russian Ministry of Health No. 116n dated February 19, 2021, "On Approval of the Procedure for Providing Medical Care to Adult Patients with Oncological Diseases".

2. Order of the Russian Ministry of Health No. 548n dated June 4, 2020, "On Approval of the Procedure for Dispensary Observation of Adults with Oncological Diseases".

3. Order of the Russian Ministry of Health No. 404n dated April 27, 2021, "On Approval of the Procedure for Conducting Preventive Medical Examinations and Clinical Examinations (Dispanserization) of Certain Groups of the Adult Population".

4. Order of the Russian Ministry of Health No. 203n dated May 10, 2017, "On Approval of Criteria for Assessing the Quality of Medical Care".

5. Decree of the Government of the Russian Federation No. 2406-r dated October 12, 2019 (as amended on December 24, 2022) "On Approval of the List of Vital and Essential Drugs, as well as Lists of Drugs for Medical Use and the Minimum Assortment of Drugs Necessary for the Provision of Medical Care".

6. Order of the Russian Ministry of Health No. 103n dated February 28, 2019, "On Approval of the Procedure and Deadlines for the Development of Clinical Guidelines, Their Revision, the Standard Form of Clinical Guidelines and Requirements for Their Structure, Composition, and Scientific Validity of Information Included in Clinical Guidelines" (registered with the Russian Ministry of Justice on May 8, 2019, No. 54588).

7. Methodological recommendations for assessing the scientific validity of information included in clinical guidelines, Center for Expertise and Quality Control of Medical Care of the Ministry of Health of the Russian Federation, 2019.

8. Current instructions for medicinal products mentioned in these clinical guidelines can be found on the website http://grls.rosminzdrav.ru.

Appendix B. Physician Action Algorithms

Flowchart 1. Patient Management Algorithm

Appendix C. Patient Information

Recommendations for chemotherapy complications

In case of chemotherapy complications, it is necessary to contact an oncologist (chemotherapist).

1. If body temperature rises to +38 °C or higher, start taking antibiotics as prescribed by the oncologist.

2. In case of stomatitis:

• diet with mechanical and thermal sparing of the mucosa;

• frequently (every hour) rinse the oral cavity with decoctions of chamomile, oak bark, sage, and lubricate the gums with sea buckthorn (peach) oil;

• treat the oral cavity as recommended by the oncologist.

3. In case of diarrhea:

• diet – exclude fatty, spicy, smoked, sweet, dairy products, and fiber. Lean meat, flour products, fermented milk products, and rice water are allowed. Drink plenty of fluids;

• take medications as prescribed by the oncologist.

4. In case of nausea, take medications as prescribed by your oncologist.

Information for patients receiving therapy with PD-1 blocking monoclonal antibodies (ATC L01FF02, # pembrolizumab** (ATC – L01XC18))

Timely communication with your attending physician and nurse regarding your health status is an important part of helping the medical team protect you throughout your treatment as safely as possible.

It is very important that any symptoms (adverse events) associated with PD-1 blocker treatment (ATC L01FF02, # pembrolizumab** (ATC – L01XC18)) are identified and treated at the very beginning of their manifestation, which will help prevent them from progressing to more severe grades.

PD-1 blocking mAbs (ATC L01FF02, # pembrolizumab** (ATC – L01XC18)) are designed to help your body's immune system fight the tumor process. The most common side effects you may encounter during treatment are the result of high immune system activity. Such side effects are called immune-mediated and differ from the side effects you may observe with other types of malignant tumor treatments.

Adverse events that you may experience usually occur in the first 12 weeks of treatment, but can also appear later.

It is very important that you inform your attending physician about any symptoms that appear during treatment with PD-1 blockers. Early recognition of side effects enables your attending physician to immediately initiate appropriate therapy and prevent adverse events from progressing to more severe grades.

If after treatment with PD-1 blocking mAbs (ATC L01FF02, # pembrolizumab** (ATC – L01XC18)) you experience any of the symptoms listed below, please inform your attending physician immediately.

Possible adverse events following PD-1 blocker therapy:

• an increase in the frequency of bowel movements to 2 or more times a day or any nighttime diarrhea, any stool containing mucus and blood;

• abdominal pain or chills, as well as pain requiring medical intervention;

• red, painful eyes or yellowish coloration of the whites of the eyes, blurred vision, inflammation or swelling of the eyes;

• yellow coloration or redness of the skin, itchy rash, skin sensitivity to sunlight;

• newly onset cough or shortness of breath;

• fatigue or drowsiness;

• difficulty concentrating or confusion;

• headache, body aches, or pain at the tumor site;

• fever (body temperature rise up to +38 °C);

• sudden decrease or increase in body weight;

• newly onset erectile dysfunction or loss of interest in intimate life.

Please pay special attention to any changes in bowel habits. Keep a daily log of the number of bowel movements. Diarrhea may not always present as watery stools. An increase in the number of bowel movements per day, even with formed stools, potentially indicates a problem.

Important: side effects may occur from 1 week to several months after starting treatment with PD-1 blockers. If you experience any of the above symptoms, immediately inform your attending physician or nurse.

Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools

Appendix G1. WHO/ECOG patient performance status scale [58]

Russian title: WHO Performance Status Scale / ECOG

Original title: The Eastern Cooperative Oncology Group/World Health Organization Performance Status (ECOG/WHO PS)

Source (official developer website, validated publication): https://ecog-acrin.org/resources/ecog-performance-status

Oken MM, Creech RH, Tormey DC, Horton J, Davis TE, McFadden ET, Carbone PP: Toxicity and response criteria of the Eastern Cooperative Oncology Group. Am J Clin Oncol 1982, 5(6):649-655

Type: rating scale

Purpose: to describe the patient's level of functioning in terms of their ability to care for themselves, daily activities, and physical abilities (walking, work, etc.).

Contents:

Key (interpretation): provided within the scale itself

Appendix G2. Karnofsky scale [59]

Russian title: Karnofsky Performance Scale

Original title (if applicable): KARNOFSKY PERFORMANCE STATUS

Source (official developer website, validated publication): Karnofsky DA, Burchenal JH: The clinical evaluation of chemotherapeutic agents in cancer. In: Evaluation of chemotherapeutic agents. edn. Edited by MacLeod C. New York: Columbia University Press; 1949: 191-205

Type: rating scale

Purpose: to assess the patient's level of functioning in terms of their ability to care for themselves, daily activity, and physical capability (walking, working, etc.).

Content (template):

Key (interpretation): provided within the scale itself