List of Abbreviations
CT – computed tomography
ICD-10 – International Classification of Diseases, 10th Revision
MRI – magnetic resonance imaging
PNS – paranasal sinuses
Terms and Definitions
Benign tumors are pathological formations arising from impaired control mechanisms of cell division, growth, and differentiation. Clinically, tumors present as foci of pathological (abnormal) tissue growth in various organs and body structures.
The distinguishing characteristics of a benign tumor are:
· identity, or minimal differences in structure between the neoplasm cells and the tissue cells from which the tumor grows;
· expansive growth;
· absence of metastases.
An analysis of the structure of benign neoplasms of the nose and paranasal sinuses indicates a relatively high frequency of osteogenic and chondrogenic tumors in the sinonasal region.
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Osteoma is a benign, slow-growing (average rate 0.44–6.0 mm per year) tumor developing from bone tissue. Not a single case of osteoma malignant transformation has been described in the literature.
Osteoblastoma is a rare benign bone tissue tumor producing reticulofibrous trabeculae surrounded by large osteoblasts. First described in 1956.
Fibrous dysplasia is a tumor-like bone lesion based on the destruction of bone tissue followed by deformation and replacement of the bone marrow cavity with fibrous tissue. Synonyms: cystic osteodystrophy, fibrous osteitis, osteodystrophy deformans, focal fibrosis, Lichtenstein disease, Lichtenstein-Jaffe disease, Lichtenstein-Bryce disease (more than 30 different terms in total).
Aneurysmal bone cyst is a rare benign bone disease characterized by local destructive and expansive growth. The name is due to the radiological similarity of the changes to an aneurysm and the presence of cystic cavities; first described in 1942.
Ossifying fibroma is a benign tumor consisting of calcifications, cementum, bone, and fibrous tissues. First described in 1927 by Montgomery [1]. Synonyms include fibro-osteoma, cemento-ossifying fibroma, cementing fibroma, and psammomatoid ossifying fibroma; in addition, juvenile active (aggressive) ossifying fibroma is distinguished, which is characterized by a more aggressive clinical course.
Giant cell tumor is a rare benign bone tissue neoplasm with a locally aggressive course and destructive growth. It may be combined with ossifying fibrous dysplasia, cherubism, or Paget's disease. Synonym: osteoclastoma.
Chondroma is a true tumor developing from cartilage tissue, characterized by a prolonged development period ranging from 3 months to 10 years.
Chondromyxoid fibroma is a rare benign tumor consisting of spindle or stellate cells located in a myxoid or chondroid stroma. First described in 1948.
Chondroblastoma is a rare benign tumor originating from chondroblasts. First described by Codman in 1931 as "epiphyseal chondromatous giant cell tumor". Later, Jaffe proposed the term "chondroblastoma" and included the tumor in the group of cartilage-derived neoplasms.
Nasal chondromesenchymal hamartoma is a benign tumor formed by spindle cells, cartilage tissue, and hemorrhagic cysts; first described by McDermott et al. in 1998.
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
The exact causes of benign osteogenic and chondrogenic neoplasms of the nose and PNS have not been definitively established. Certain risk factors whose influence may provoke tumor development can be stated with a degree of probability: embryogenesis disorders, infection, and trauma.
The embryonic origin of osteoma is associated with ossification disorders in the area where the ethmoid and frontal bones join, which is confirmed by the frequent occurrence of osteomas, as well as anomalies and malformations of intranasal structures in this anatomical zone [1, 2]; however, this does not explain the existence of osteomas in other locations. According to other data, the formation of osteomas of the nose and PNS may be caused by bone tissue remodeling processes against the background of infectious-inflammatory changes and trauma to the nasofacial region [3, 4]. At the same time, the aforementioned changes do not precede the emergence of osteomas in all cases.
The etiology of osteoblastoma has not been definitively established. Genetically determined conditions, trauma, metabolic and endocrine disorders, and chronic inflammatory diseases may play a certain role in the occurrence of this tumor, as well as ossifying fibroma and giant cell tumor.
In the development of fibrous dysplasia, the primary role is played by a mutation in the GNAS1 gene, which is responsible for the synthesis of protein G [5].
Despite the lack of reliable data on the causes of aneurysmal bone cyst development, its formation is associated with trauma (hematoma resorption) and local vascular disorders (venous thrombosis, arteriovenous aneurysm, etc.), which lead to increased venous pressure and, consequently, dilation and engorgement of the vascular bed [6, 7]. Furthermore, a neoplastic origin of aneurysmal bone cyst cannot be ruled out; aberrations in the 17p13 and 16q22 genes have been described in this condition [8].
Potential risk factors for the development of chondrogenic tumors—chondroma, chondromyxoid fibroma, chondroblastoma—include trauma, inflammatory processes, heredity, genetically determined ossification disorders, and endocrinopathies.
1.3 Epidemiology of the Disease or Condition
The proportion of osteomas in the structure of skeletal neoplasms reaches up to 10% [9]. Osteoma is more frequently detected in males aged 20–50 years (mean age around 40 years) [10]. The most frequent sinonasal localization of osteomas is the frontal sinus (30 to 52–71.8% of cases), less frequently the ethmoid labyrinth cells (16.9–22%), maxillary (1.7–6.3%), sphenoid (up to 4.9%) sinuses, and the nasal cavity (0.6%) [11, 12]. Observations of osteomas of the inferior [13] and middle [14] nasal conchae, nasal septum [11], and nasal bone [15] have been described. An osteoma can be either an independent disease or a manifestation of others—for example, Gardner's syndrome, characterized by multiple osteomas of the maxillofacial region combined with colon polyps and other soft tissue tumors.
Osteoblastoma accounts for less than 1% of primary bone tumors [16]. The mean age of patients with osteoblastoma is 20.4 years, and the disease is twice as common in males [17, 18]. Localization in the maxillofacial region accounts for 15–20% of all osteoblastoma cases [19]. The nose and PNS are affected extremely rarely. Isolated cases of osteoblastoma of the nasal septum [20], maxillary [21], sphenoid [22], and frontal [23] sinuses, ethmoid labyrinth cells [24], and middle [25] and inferior [26] nasal conchae have been described.
Fibrous dysplasia accounts for about 2% of all bone tumors. It is more common in females [27], predominantly aged 10 to 20 years [3, 5, 6]. In otorhinolaryngological practice, fibrous dysplasia is relatively rare—usually affecting the PNS [28], and less commonly the nasal bones [29], and the inferior and middle nasal conchae [30].
The annual incidence of aneurysmal bone cyst is 0.14 new cases per 100 population [31]. It is slightly more frequent in females. The majority of aneurysmal bone cyst cases occur in the first two decades of life. Localization of an aneurysmal bone cyst in the maxillofacial region accounts for less than 5% of all cases of the disease [32]. In sinonasal localization, the process most frequently affects the bones of the orbitomaxillary-ethmoid complex. A primary aneurysmal bone cyst is predominantly localized in the ethmoid labyrinth cells with extension to the orbital walls, and less frequently in the maxillary, frontal, and sphenoid sinuses [33]. Cases involving the middle nasal concha have also been described [34].
Exact data on the prevalence of ossifying fibroma are lacking. According to some data, it occurs 5 times more frequently in women. Juvenile ossifying fibroma is more commonly detected in boys aged 6–11 years. The mean age of patients is about 20 years [35, 36]. The preferred localization of ossifying fibroma is the maxillofacial region, particularly the mandible. Localization in the nasal cavity and PNS accounts for about 15% of all cases of the disease, with the maxillary sinus and ethmoid bone cells being most frequently affected, and the sphenoid and frontal sinuses, as well as the middle and inferior nasal conchae, being affected less frequently [8, 9, 10, 11].
Giant cell tumors account for 5% of all primary bone tumors and 20% of benign primary bone tumors. Patients are predominantly aged 20 to 50 years, mostly female [37, 38, 39]. In the maxillofacial region, it occurs in less than 1% of all giant cell tumor cases; isolated cases of this neoplasm localized in the ethmoid cells, frontal, and sphenoid sinuses have been described [37].
The proportion of chondroma among benign bone tumors ranges from 4.2 to 36.4% [40, 41, 42]. The nasal cavity and paranasal sinuses are a relatively rare localization for chondroma. Since the first description of nasal chondroma in 1842, about 140–150 cases of this disease have been reported in the English-language literature [43, 44]. In the structure of benign neoplasms of the nasal cavity and paranasal sinuses, chondroma accounts for 2.95–6% [45, 46]. According to various data, sinonasal chondroma most commonly develops from the ethmoid labyrinth, the lateral wall of the nasal cavity, and the sphenoethmoidal region [47, 48, 49].
Chondromyxoid fibroma accounts for about 1–5% of facial skeleton bone lesions [50, 51]. There is no clear gender predilection, although some studies note a higher predisposition in females. It is believed that chondromyxoid fibroma most frequently occurs in young individuals aged 20–30 years, whereas other observations note a predominance of patients in the fifth decade of life [52]. In the head region, the tumor most commonly affects the mandible, and less frequently the nasal cavity and paranasal sinuses. A high frequency of chondromyxoid fibroma localization is noted in the frontal, sphenoid, occipital, and temporal bones, as well as the clivus. Cases of chondromyxoid fibroma of the ethmoid labyrinth cells and nasal septum have been published [53, 54].
Chondroblastoma accounts for less than 1% of all primary bone tumors [55]. The mean age of patients with chondroblastoma is about 44 years, and it is slightly more common in males [56]. In the head and neck region, the tumor accounts for 16% of cases; cases of chondroblastoma of the nasal bones [8], as well as the maxillary and sphenoid sinuses, have been described [57, 58, 59, 60, 61].
1.4 Features of Coding the Disease or Condition
D14.0 Benign neoplasm of middle ear and respiratory system.
1.5 Classification of the disease or condition
There is no universally accepted classification of benign osteogenic and chondrogenic tumors of the nose and paranasal sinuses. Classifications of individual tumors are working classifications and are based on morphological features, extent, and localization of the neoplasm, which are taken into account when planning treatment tactics.
Osteoma: histologically, compact osteomas (represented by very dense cortical bone), cancellous osteomas (containing spaces filled with fibrous tissue), and mixed forms (a combination of the first two types of osteomas) are differentiated [62].
Osteoblastoma: depending on the growth rate of the tumor, three types of osteoblastoma are distinguished: latent (asymptomatic and usually an incidental finding during patient evaluation for other conditions), active (characterized by moderate growth dynamics and the appearance/progression of symptoms as the tumor enlarges), and aggressive (distinguished by rapid and expansive growth, destruction of the sinus walls, and a tendency to spread beyond the sinus) [63].
Fibrous dysplasia: monostotic fibrous dysplasia (involvement of a single bone, 70–85% of cases) and polyostotic fibrous dysplasia (involvement of multiple bones, 15–30% of cases) are distinguished [64].
Aneurysmal bone cyst: 3 phases of cyst development are distinguished – the osteolysis phase, the demarcation phase, and the repair phase.
Chondroma: two groups of tumors are differentiated: enchondroma – a tumor developing inside the bone, and exchondroma – a tumor localized on the outer surface of the bone.
1.6 Clinical presentation of the disease or condition
The onset of symptoms in benign osteogenic and chondrogenic tumors of the nose and paranasal sinuses is generally associated with an increase in tumor size, its compression of the paranasal sinus walls, and/or its spread to adjacent anatomical structures. Small tumors are usually asymptomatic and may be an incidental finding on radiological examination of the nose and paranasal sinuses.
Frequently, the leading symptom of a benign bone or cartilage tumor of the nose and paranasal sinuses is headache in the nasofacial region or the projection of the corresponding sinus, nasal congestion, and anosmia. The onset of these complaints may be associated with tumor obstruction of the natural ostium of the affected sinus, leading to secondary sinusitis or mucocele. In aneurysmal bone cysts, patients may present with recurrent epistaxis.
Extension of the tumor into the orbit is accompanied by deformity of the nasoorbital region, proptosis, periorbital pain, chemosis, diplopia, epiphora in case of nasolacrimal duct compression, or periorbital emphysema.
Less commonly, compression of the optic nerve may result in decreased visual acuity, and intracranial tumor spread can lead to generalized and/or focal neurological symptoms, meningitis, or brain abscess.
In lesions of the sphenoid bone, patients with giant cell tumors present with headaches, visual disturbances, trigeminal hyperesthesia, and cranial nerve palsies affecting eye muscles.
A frequent symptom of fibrous dysplasia of the nose and paranasal sinuses is asymmetry and painless swelling of the facial region. When pathological changes are localized in the clivus and sphenoid sinus, a prolonged asymptomatic period of the disease is possible.
2. Diagnostics, medical indications and contraindications for diagnostic methods
Subjective manifestations and clinical symptoms of sinonasal tumors are generally nonspecific and minimal, especially in the early stages of the process. In some tumors, symptoms may be completely absent for a prolonged period (several years). For example, a prominent clinical picture is absent in 4–10% of paranasal sinus osteoma cases [65, 66], and the incidence of incidental detection of this tumor on computed tomography of the nose and paranasal sinuses reaches 1–3% [65]. This requires careful attention to the patient's complaints and history.
Criteria for diagnosing benign osteochondrogenic tumors of the nose and paranasal sinuses: the diagnosis is established based on complaints, medical history, physical examination data, and the results of instrumental examinations (endoscopic endonasal examination of the nasal cavity and nasopharynx, computed tomography of the nose and paranasal sinuses).
2.1 Complaints and Anamnesis
It is recommended to clarify the timing, sequence of onset, and duration of pathological subjective sensations; the dynamics of subjective symptoms from their onset to the current medical visit; and prior treatment for the reported complaints and its efficacy.
When the tumor is localized in the nasal cavity, symptoms of progressive nasal obstruction (unilateral or bilateral) usually dominate. Subsequently, as the neoplasm enlarges, impaired ventilation and drainage of the paranasal sinuses may occur due to occlusion of their natural ostia, resulting in secondary rhinosinusitis or mucocele [65].
One of the frequent complaints presented by such patients is headache. When the tumor extends beyond the sinus, patients may note deformity of the nasofacial region, proptosis, diplopia; epiphora upon compression of the nasolacrimal duct; decreased visual acuity upon compression of the optic nerve; and intracranial complications such as meningitis, brain abscess, or pneumocephalus upon destruction of the cerebral wall of the sinus [66].
It is recommended to clarify the nature of the patient's complaints and subjective sensations that prompted the medical visit, their duration, severity, dynamics, and potential cause; previous trauma and diseases of the nose and paranasal sinuses, their treatment (medical, surgical) and its efficacy; carefully review the patient's available results from previous examinations and consultations (laboratory tests, CT, MRI, consultant reports; results of histological examination of biopsy material, surgical protocols of previous interventions in the nasal cavity and paranasal sinuses); and determine the presence of similar complaints in close relatives of the patient [98].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
2.2 Physical Examination
It is recommended to perform a general physical examination, palpation/percussion of the nasofacial region (walls of the paranasal sinuses), and palpation of the cervical lymph nodes (submandibular, submental, and deep cervical lymph nodes, the latter group including retropharyngeal lymph nodes) [67, 99]. Strength of recommendation C (level of evidence – 4)
Comments: during the physical examination, attention should be paid to any deformity of the nasofacial region, tenderness (sensitivity) upon palpation/percussion of the paranasal sinus (PNS) walls, and the exit points of the trigeminal nerve branches. An important diagnostic procedure is the assessment of the cervical lymph nodes, which drain lymph from the nasal cavity and paranasal sinuses. Generally, this involves evaluating the size, consistency, shape, tenderness, and mobility of the palpated lymph nodes. Changes in regional lymph nodes are not characteristic of benign neoplasms of the nose and paranasal sinuses.
2.3 Laboratory Diagnostics
A biopsy of the neoplasm and pathomorphological examination of the biopsy (surgical) material using immunohistochemical methods are recommended (if indicated) for the differential diagnosis of benign and malignant tumors of the nose and PNS [68, 100].
GCP CLE 4Grade of Recommendation C (Level of Evidence 4)
Comments: most often, tissue sampling of a neoplasm in the nasal cavity or PNS is performed via targeted biopsy, usually under visual control using endoscopes. Biopsy forceps (punch biopsy) can be used for this purpose. The material for examination should be taken at the border between the tumor and unchanged tissues. For small tumors, an excisional biopsy is advisable, in which the neoplasm is removed within healthy tissue margins followed by histological examination. If a cystic lesion or solid tumor is present, an aspiration biopsy is performed (using a needle and syringe). In certain cases, material for cytologic examination can be obtained by making an imprint on a glass slide applied to the pathologically altered area of the mucous membrane, or using an imprint smear—where material harvested from the mucosal surface using a scraper or cytological brush is transferred onto a glass slide.
2.4 Instrumental Diagnostics
Anterior rhinoscopy is recommended for all patients as the most common preliminary examination method for the nasal cavity, allowing assessment of the nasal vestibule, anterior nasal mucosa, inferior and middle nasal conchae, nasal septum, presence/absence and nature of pathological discharge in the anterior parts of the inferior, middle, and common nasal meatuses; and detection of a neoplasm located in the anterior nasal cavity or extending into these areas from deeper regions of the nose, paranasal sinuses, and partially or completely obstructing the nasal cavity [69].
GCR CLOCE 5Grade of Recommendation C (Level of Evidence 5)
Videorinoscopy is recommended for all patients – performed using a rhinoscope or nasopharyngolaryngofiberscope. It is advisable to perform the initial examination without prior preparation of the nasal cavity and without anesthesia. This provides an adequate assessment of the true state of the mucosa, the nature, quantity [112], and localization of pathologically altered tissues, and other changes [70].
GCR CLOCE 4Grade of Recommendation C (Level of Evidence 4)
Computed tomography (CT) of the nose and PNS is recommended when a tumor of the nose and paranasal sinuses is suspected, using step-by-step scanning from the hard palate plane (lower scanning boundary) to the upper boundary of the frontal sinus (upper scanning boundary) with a 2 mm slice thickness. It is characterized by high sensitivity and specificity in diagnosing benign neoplasms. CT helps clarify the localization, extent, size, structure of the neoplasm, presence/absence of calcification, and remodeling of the adjacent bone tissue [71].
GCR CLOCE 5Grade of Recommendation C (Level of Evidence 5)
Osteoma – CT allows not only detecting the presence of a paranasal sinus osteoma, but also determining its structural type and establishing the site of origin. The radiographic appearance of an osteoma corresponds to its structure. Compact osteomas are spherical, rounded tumors that appear as a dense, almost structureless neoplasm (Figure 1).
Fig. 1. CT of the paranasal sinuses, coronal (A) and axial (B) planes: A compact osteoma of the right ethmoid labyrinth presented as a rounded, high-density lesion with clear, smooth margins.
Spongy and spongio-compact osteomas have a coarse trabecular structure arranged in a specific pattern – distinct margins of compact bone transitioning into the cortical bone layer (Figure 2).
Fig. 2. Axial CT of the paranasal sinuses. Spongy osteoma of the right frontal sinus.
Osteomas of mixed structure have different bone tissue structures at the site of attachment to the bone wall and at the periphery. In the area of the stalk, the bone tissue is soft, while its density gradually increases toward the periphery.
According to computed tomography, such osteomas consist of two zones: a zone with a density of 300–600 HU, having a spongy structure, and a zone with a density of over 800 HU, having a compact bone structure. A clear boundary between these zones is not always observed; sometimes there is a gradual increase in bone tissue absorption coefficients (Figure 3).
Fig. 3. CT of the paranasal sinuses: axial (A) and coronal (B) planes. Mixed-type osteoma of the left frontal sinus originating from the intersinus septum. Labels: compact bone (CB), tumor stalk (S). Spongy bone is present in the stalk area, while more mature compact bone is found at the tumor periphery.
Osteoblastoma on CT typically presents as a combination of dense bone and areas of opacification (Figure 4).
Fig. 4. CT of the skull, axial plane. Giant osteoblastoma extending into the right orbit, nasal cavity, and anterior cranial fossa.
CT data make it possible to determine signs of locally aggressive growth of the osteoblastoma, its site of origin, and the presence/absence of tumor extension beyond the paranasal sinuses.
Fibrous dysplasia on CT is characterized by a relatively homogeneous, ground-glass-like tissue with a density ranging approximately from 300 to 450 HU, which is lower than the usual densitometric values for the spongy bone layer and corresponds to the density of fibrous tissue (Figure 5).
Fig. 5. CT of the paranasal sinuses, axial plane. Fibrous dysplasia of the maxillary (A) and frontal (B) sinuses.
The focus of fibrous dysplasia is unencapsulated and can therefore spread to adjacent anatomical structures. The cystic (pseudocystic) form of fibrous dysplasia (cystic osteodystrophy) is characterized by a combination of areas with a relatively homogeneous bone structure and a density typical of fibrous tissue (300–450 HU) and areas of lower density (sometimes approaching fluid density) that are rather clearly demarcated from the surrounding tissue. These areas of pseudocystic degeneration are usually arranged haphazardly, sometimes in the center of the zone and sometimes at its periphery (Figure 6).
Fig. 6. CT of the paranasal sinuses, axial plane. Pseudocystic form of fibrous dysplasia of the right frontal sinus and anterior ethmoid air cells. Areas of pseudocystic degeneration (arrows) against the background of sclerotically altered bone tissue.
In fibrous dysplasia of the sphenoid body, the sphenoid sinus is affected first, after which the process usually spreads to the greater wing of the sphenoid bone, pterygoid process, lesser wing, planum sphenoidale, and other anatomical structures. The resulting deformation and narrowing of the foramen ovale, foramen rotundum, inferior orbital fissure, optic canal, and vidian canal (Figure 7), along with the compression of closely adjacent cranial nerves and major vessels, are accompanied by corresponding clinical symptoms, which is the direct reason for such a patient's visit to the doctor.
Fig. 7. CT of the paranasal sinuses, axial (A) and coronal (B) views. Fibrous dysplasia of the sphenoid bone. B – the pathological process spreads to the greater wing of the sphenoid bone (arrow) and the pterygoid process; asymmetry of the vidian canals – it is markedly narrowed on the affected side (arrows).
In fibrous dysplasia of the maxilla, the pathological process relatively quickly spreads to the maxillary sinus, alveolar, zygomatic, and frontal processes. When the medial wall of the maxillary sinus is involved, narrowing or obliteration of the nasolacrimal duct and nasal meatuses, and nasal septum deviation may be observed (Figure 8).
Fig. 8. CT of the paranasal sinuses, axial view (A) and coronal reconstruction (B). Fibrous dysplasia of both maxillary sinuses with involvement of the zygomatic processes (osteosementodysplasia).
The affected maxillary bone and zygomatic processes have a heterogeneous structure, against which extensive high-density zones (cementicles) are identified around the tooth roots.
Chondroma appears on CT as a mass of heterogeneous density with well-defined borders and foci of mineralization (Figure 9).
Fig. 9. CT of the nose and paranasal sinuses, coronal (A) and axial (B) planes. Chondroma of the nasal cavity. The tumor originates from the nasal septum, obturates the right nasal cavity, and has clear margins. Displacement of the medial wall of the right maxillary sinus and secondary inflammatory changes in the right maxillary and frontal sinuses are detected.
Unlike chondroma, osteochondroma is characterized by the presence of bone tissue of heterogeneous structure and density in the central parts and cartilage-density tissue at the periphery of the tumor (Figure 10).
Fig. 10. CT of the paranasal sinuses, axial plane (A) and coronal reconstruction (B). Osteochondroma of the nasal septum with extension into the left maxillary sinus
Comment: due to the high probability of osteogenic and chondrogenic benign tumors of the nose and paranasal sinuses spreading to adjacent anatomical zones — the upper jaw, orbit, and cranial cavity — to diagnose/exclude these conditions and coordinate treatment tactics in relevant cases, it is recommended to perform an MRI, consult a maxillofacial surgeon, ophthalmologist, and neurosurgeon. Such a comprehensive examination is strictly indicated if the patient has changes in the dentition and jaw system (displacement, loosening, tooth loss, maxillofacial deformity), eyes (proptosis and periorbital pain, chemosis and diplopia in case of extraocular muscle damage, lacrimation), as well as meningeal and focal neurological symptoms.
2.5 Other Procedures
Not required
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
Surgical treatment of benign osteogenic and chondrogenic tumors of the nose and paranasal sinuses is recommended, performed using endonasal, external (extranasal), and combined approaches. Certain limitations of endoscopic interventions are usually associated with large tumor size and/or difficult-to-reach location. With significant dissemination of the neoplasm, extranasal approaches are used — transpalatal, lateral rhinotomy, craniofacial resection, midfacial degloving, etc. The choice of surgical technique is determined by the surgeon's training level and the availability of appropriate instruments and equipment. The efficacy and safety of the intervention in these cases are enhanced by the use of navigation equipment [28, 72].
GCR CLeE 4 Grade of Recommendation C (Level of Evidence 4)
Osteoma. It is recommended to use endonasal (endoscopic), external, and combined approaches to remove an osteoma. The surgical technique depends on the size and site of tumor attachment to the sinus wall. The external approach is known to be traumatic and may be accompanied by postoperative aesthetic defects, paresthesias, and prosopalgia [73, 113].
GCP CLE 4Grade of Recommendation C (Level of Evidence 4)
Comment. The determination of indications for endoscopic removal of frontal sinus osteoma, depending on localization, extent of tumor spread, and anatomical features of the frontal sinus, has been systematized by A.G. Chiu et al., 2005 (Table 1) [72].
Grade 1 and 2 osteomas are preferred for endoscopic removal. Later clinical observations have demonstrated the feasibility of successful endoscopic removal of Grade 3 and 4 tumors [39, 40, 68, 69]. The combined approach combines the advantages of endoscopic and external techniques.
Table 1
Classification of frontal sinus osteomas (according to A.G. Chiu et al., 2005)
Grade 1 | The tumor is attached posteroinferiorly and located medially to the imaginary sagittal plane passing through the lamina papyracea. The anteroposterior diameter of the tumor is less than 75% of the anteroposterior diameter of the frontal recess |
|---|---|
Grade 2 | The tumor is attached posteroinferiorly, located medially to the imaginary sagittal plane passing through the lamina papyracea. The anteroposterior diameter of the lesion is greater than 75% of the anteroposterior diameter of the frontal recess |
Grade 3 | The tumor is attached anteriorly or superiorly and/or extends beyond the imaginary sagittal plane passing through the lamina papyracea |
Grade 4 | The tumor completely obturates the frontal sinus |
A relative limitation of endoscopic osteoma removal, mainly related to the duration of the procedure, is large osteomas localized in the posterosuperior parts of the frontal sinus. The endoscopic approach is not indicated when the osteoma is localized on the anterior wall or the inferior wall of the lateral parts of the frontal sinus.
Intraorbital and intracranial extension of an osteoma is not considered an absolute contraindication to endoscopic tumor removal [28].
Osteoma recurrence is rare, and the period of its occurrence is usually quite long (5–8 years) [75].
For small asymptomatic osteomas, a watchful waiting strategy is recommended. According to some sources, surgery should be resorted to for osteomas occupying more than 50% of the sinus volume [76].
Osteoblastoma. Surgical removal of the tumor is recommended. As with osteoma surgeries, when choosing the method for surgical removal of an osteoblastoma — endonasal (endoscopic), external, or combined — one should take into account the localization, size, and site of tumor attachment to the sinus wall. Radical removal of osteoblastoma generally leads to a complete cure, with no recurrence of the disease noted during 5–15 years of follow-up after surgery [19, 101].
GCP CLE 4Grade of Recommendation C (Level of Evidence 4)
Comment. The difficulties of surgical treatment of osteoblastoma, as well as other benign osteogenic tumors, in pediatric practice are due to limitations associated with the ongoing development of skull bones in childhood. According to some data, in children (mean patient age 14 years), the recurrence rate of osteoblastoma can reach 32% [77]. A case of osteoblastoma malignant transformation after radiation therapy has been described [78].
Fibrous dysplasia. Indications for surgery include the severity of bone deformity, the patient's age, and the limited extent of the lesions. Due to the likelihood of disease regression, a watchful waiting strategy with continuous monitoring and periodic CT scans is recommended [79, 102, 103]. Drug therapy with bisphosphonates can be recommended for the prevention of pathological fractures and pain, although there is a risk of jaw osteonecrosis [80, 114]. In McCune-Albright syndrome, drug therapy is also possible to manage the corresponding endocrine disorders [81].
GCR CLOCE 5Grade of Recommendation C (Level of Evidence 5)
Comment. Several series of successful endoscopic removals of fibrous dysplasia of the nose and paranasal sinuses have been described in the literature. Endoscopic surgery is indicated to relieve symptoms of rhinosinusitis associated with fibrous dysplasia, as well as for optic nerve decompression. The use of decortication of the maxillary sinus bone walls in fibrous dysplasia has been reported [82], but the efficacy of this treatment requires evidence due to the small number of patients operated on using this method.
Aneurysmal bone cyst. Surgical removal of the tumor is recommended. The scope and method of the intervention depend on the localization and extent of the aneurysmal bone cyst and can range from cyst cavity puncture to curettage and resection of the affected bone area [83, 104, 105, 115].
Comments: Taking into account the anatomical and topographical features of localization (at the skull base, within the paranasal sinuses), as well as the intense blood supply of the tumor, preoperative embolization of the feeding vessels is recommended [106]. Radiation therapy is possible, but undesirable due to the risk of malignant transformation of the aneurysmal bone cyst (transformation into sarcoma) [84]. Disease recurrences may occur in 10–30% of cases [85], most often within the first two years after treatment [86].
GCR CLeE 4Grade of Recommendation C (Level of Evidence 4)
Ossifying fibroma. Surgical removal of the tumor is recommended. An important condition for effective treatment and recurrence prevention is the earliest and most complete removal of the ossifying fibroma possible. Depending on the tumor localization and size, craniofacial resection, lateral rhinotomy, midfacial degloving, and endoscopic tumor dissection are used for its removal [87].
GCP CLE 4Grade of Recommendation C (Level of Evidence 4)
Comment. The recurrence rate of juvenile ossifying fibroma, even with complete removal of the neoplasm, reaches 30%. The risk of recurrence may persist for a long period—18 years after the removal of the ossifying fibroma—indicating the need for prolonged postoperative follow-up. Attempts at radiation therapy for ossifying fibroma have proven ineffective and potentially dangerous due to the likelihood of tumor malignant transformation [88].
Giant cell tumor. Surgical removal of the tumor is recommended [89].
GCP CLE 4Grade of Recommendation C (Level of Evidence 4)
Comment. The recurrence rate depends on the extent of the process. Due to the relatively small number of observed giant cell tumors of the nose and paranasal sinuses, data on the recurrence rate of the disease at this site are absent; nevertheless, such patients require prolonged follow-up. Radiation therapy may be recommended for localizations that limit the possibility of radical tumor removal or require disabling interventions [90, 107].
Chondroma. Surgical removal of the tumor is recommended. This is due to the potential risk of tumor malignant transformation, as well as progressive compression and destruction of the walls of the nasal cavity, paranasal sinuses, orbit, and skull base. Given these circumstances, preference may be given to endoscopic removal of the nasal chondroma, or, if the tumor spreads into the maxillary sinus and orbit, to Denker's maxillectomy. Radical removal of an extensive chondroma of the nose and paranasal sinuses may be complicated by intraoperative complications, in particular bleeding. In some cases, transpalatal or external (lateral rhinotomy) approaches are used for chondromas of this localization. Chondromas of the nasal bones can be successfully removed using the approach employed in external rhinoplasty [92, 93].
GCP CLE 4Grade of Recommendation C (Level of Evidence 4)
Chondromyxoid fibroma. Surgical removal is recommended. Tumor curettage has a positive effect in 80% of cases; recurrence is usually associated with insufficiently radical removal of the neoplasm [94, 108]. However, cases of multiple recurrences of chondromyxoid fibroma have been described even after radical surgery. A lower recurrence rate is observed with en bloc excision of the tumor, although such surgery is traumatic [95, 109]. Most authors agree that wide excision—either endoscopic or using a midface degloving approach—is preferable [96, 110].
GCR CLOCE 4Grade of Recommendation C (Level of Evidence 4)
Chondroblastoma. Surgical treatment—radical excision of the tumor—is recommended, but due to localization features, complete removal of the neoplasm may be difficult. With curettage of chondroblastoma alone, the recurrence rate can reach as high as 55% [97, 110].
GCP CLE 4Grade of Recommendation C (Level of Evidence 4)
4. Medical Rehabilitation and Sanatorium-Resort Treatment
A restricted regimen is recommended for patients after surgical removal of a neoplasm for 10-14 days or more postoperatively (depending on the localization and extent of the intervention) [111].
GCR CLOCE 5Grade of Recommendation C (Level of Evidence 5)
5. Prevention and Dispensary Observation
Specific prevention of benign osteogenic and chondrogenic neoplasms of the nose and paranasal sinuses is absent. For small, asymptomatic paranasal sinus osteomas, an expectant management strategy is recommended.
Considering the probability of recurrence and the risk of malignant transformation of some benign neoplasms, follow-up monitoring of the patient is recommended after surgery. The timing is determined individually and depends on the nature and localization of the tumor, as well as the extent and radicality of the surgical intervention—the first examination using CT should be performed 3–6 months after surgery, and subsequently at 1 year or more, depending on the specific clinical situation [111].
GCR CLOCE 5Grade of Recommendation C (Level of Evidence 5)
6. Organization of Medical Care
Medical care is organized according to the patient management algorithm (Appendix B):
1) the indication for hospitalization is the presence of a benign osteogenic or chondrogenic neoplasm of the nose and paranasal sinuses in the patient and the absence of contraindications to elective surgical treatment (tumor removal);
2) the indications for discharging the patient from the medical organization after surgical intervention are the absence of complications and a satisfactory general condition of the patient.
7. Additional Information
The prognosis depends on the histological structure, localization, and extent of the neoplasm of the nose and paranasal sinuses.
Considering the difficulty of diagnosing osteogenic and chondrogenic benign tumors of the nose and paranasal sinuses—caused, in particular, by the relatively long period of asymptomatic course of the neoplasm and the nonspecific nature of clinical manifestations, as well as the high probability of the association of tumors of this localization with infectious and inflammatory diseases of the paranasal sinuses—patients with a persistent or progressive inflammatory process in the nasal cavity and paranasal sinuses against the background of ongoing therapy, especially with a unilateral localization, should always be evaluated regarding the presence/exclusion of a tumor.
Radical removal of a benign tumor generally leads to a complete cure of the disease; however, due to the potential tendency of some such neoplasms to recur, and in some cases the probability of malignant transformation, follow-up monitoring of the patient is necessary.
For the purpose of timely diagnosis and treatment of such patients, their examination should include a CT scan of the nose and paranasal sinuses and, when necessary, a biopsy followed by histological tissue examination.
Criteria for Evaluating the Quality of Medical Care
Type of medical care | Specialized Medical Care |
|---|---|
Age group | Adults |
Conditions of medical care | Inpatient |
Form of medical care | Elective |
Quality criteria | Level of certainty evidence | Level convincingness of recommendations |
|---|---|---|
Collection of complaints and anamnesis was performed | ||
A general physical examination of the patient was performed, including palpation/percussion of the nasofacial region (paranasal sinus walls) and palpation of the cervical (submandibular, submental, and deep cervical) lymph nodes | ||
Frontal rhinoscopy and/or (if indicated) videorhinoscopy was performed | C | 2 |
A CT scan of the nose and paranasal sinuses was performed | A | 1 |
Surgical removal of the neoplasm was performed | A | 4 |
A restricted regimen was prescribed for 10-14 days or more postoperatively | C | 2 |
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Appendix A1. Working Group Composition
Kryukov A.I., MD, Professor, is a member of a professional association;
Nosulya E.V., MD, Professor, is a member of a professional association;
Kim I.A., MD, is a member of a professional association;
Piskunov I.S., MD, Professor, is not a member of a professional association;
Zelikovich E.I., MD, Professor, is not a member of a professional association.
There is no conflict of interest.
Appendix A2. Methodology for Developing Clinical Guidelines
The methodology for developing clinical guidelines is based on a comprehensive analysis of the current state of the problem, literature data, and regulatory legal acts, followed by discussion within the professional community and amendments based on the results of resolutions of conferences and national congresses, provided they are approved by the Ministry of Health.
The clinical guidelines summarize the authors' experience in the diagnosis and treatment of patients with benign osteogenic and chondrogenic tumors of the nose and paranasal sinuses.
The clinical guidelines contain information necessary for the diagnosis, differential diagnosis, and selection of treatment methods for patients with benign osteogenic and chondrogenic tumors of the nose and paranasal sinuses.
Methods used for evidence collection/selection:
· electronic database search.
· the evidence base for the recommendations includes publications in the Cochrane Database of Systematic Reviews, as well as the EMBASE and MEDLINE databases. The search depth was 10 years.
Methods used to assess the quality and strength of evidence:
· expert consensus;
· assessment of significance in accordance with the rating scheme.
Target audience of the clinical guidelines:
1. otorhinolaryngologists.
2. medical students, residents, postgraduate students.
Table 1. Level of Evidence (LoE) grading scale for diagnostic methods (diagnostic interventions)
LoE | Definition |
|---|---|
1 | Systematic reviews of studies with reference standard control or systematic reviews of randomized controlled trials with meta-analysis |
2 | Individual studies with reference standard control or individual randomized controlled trials and systematic reviews of studies of any design, except randomized controlled trials, with meta-analysis |
3 | Studies without consecutive reference standard control or studies with a reference standard not independent of the test method, or non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case report |
5 | Only justification of mechanism of action or expert opinion is available |
Table 2. Level of evidence (LoE) grading scale for prevention, treatment, and rehabilitation methods (preventive, therapeutic, and rehabilitative interventions)
LoE | Definitions |
|---|---|
1 | Systematic review of RCTs with meta-analysis |
2 | Individual RCTs and systematic reviews of studies of any design except RCTs, with meta-analysis |
3 | Non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case reports or case series, case-control studies |
5 | Only justification of the mechanism of action of the intervention (preclinical studies) or expert opinion is available |
Table 3. Grade of Recommendation (GoR) scale for prevention, diagnostic, treatment, and rehabilitation methods (preventive, diagnostic, therapeutic, and rehabilitative interventions)
GoR | Definition |
|---|---|
A | Strong recommendation (all considered efficacy criteria (outcomes) are important, all studies are of high or acceptable methodological quality, and their conclusions on the outcomes of interest are consistent) |
B | Conditional recommendation (not all considered efficacy criteria (outcomes) are important, not all studies are of high or acceptable methodological quality, and/or their conclusions on the outcomes of interest are inconsistent) |
C | Weak recommendation (lack of adequate quality evidence (all considered efficacy criteria (outcomes) are unimportant, all studies are of low methodological quality, and their conclusions on the outcomes of interest are inconsistent) |
Procedure for updating clinical guidelines.
The mechanism for updating the clinical guidelines provides for their systematic revision—at least once every three years, as well as upon the emergence of new proven data on the diagnosis, treatment, prevention of osteogenic and chondrogenic benign tumors of the nose and paranasal sinuses, and rehabilitation of patients with these diseases, or in the presence of justified additions/comments to the previously approved clinical guidelines, but no more frequently than once every 6 months.
Appendix A3. Reference Materials
These clinical guidelines have been developed taking into account the following regulatory and legal documents:
Federal Law No. 323-FZ dated November 21, 2011 "On the Fundamentals of Protecting the Health of Citizens in the Russian Federation"
Order of the Russian Ministry of Health No. 905n dated November 12, 2012, “On Approval of the Procedure for Providing Medical Care to the Population in the Profile of 'Otorhinolaryngology'”
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”
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 Practice Guidelines, Their Revision, the Standard Template of Clinical Practice Guidelines, and Requirements for Their Structure, Composition, and Scientific Validity of Information Included in Clinical Practice Guidelines”
Order of the Ministry of Health of the Russian Federation dated October 13, 2017, No. 804n, "On approval of the nomenclature of medical services"
Appendix B. Physician Action Algorithms
Appendix C. Patient Information
Benign tumors of the nose and paranasal sinuses are characterized by slow growth and, as they increase in size, can exert pressure on the adjacent walls of the nose, paranasal sinuses, and orbit, causing their destruction in certain cases. One of the frequent complaints in tumors of the nose and paranasal sinuses is headache, as well as progressive nasal breathing difficulty and, subsequently, the development of secondary sinusitis.
When a tumor spreads beyond the nose and paranasal sinuses, craniofacial deformity, double vision, decreased vision, and in some cases, the development of meningitis and other complications may occur.
Treatment of benign tumors of the nose and paranasal sinuses is surgical. Given the probability of tumor recurrence, follow-up dispensary monitoring is necessary in the postoperative period, the duration of which is determined by the physician.
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
None.