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Pathological Anatomy · Chapter 26. Pediatric Tumors

Hemangioma

Haemangioma

The presented material focuses on childhood malignancies, their histological features, and fundamental differences from adult tumors. Specific small round blue cell tumors, the phenomenon of reversion, and key immunohistochemical markers required for accurate differential diagnosis are discussed.

Common tumorsLeukemias account for 30% of all malignancies in children.
CNS involvementCentral nervous system tumors rank second, with a frequency of 21%.
Rarity of cancerCarcinomas in children are rare, accounting for no more than 6% of cases.
HistogenesisPediatric tumors are often of dysontogenetic origin and develop from tissues.
8 min readFor medical studentsUpdated Sep 25, 2026
Material prepared by WhiteCoat Pro editorial team based on platform study guidesReviewed by medical editor: Aulov A. A., general practitioner · 09/24/2026

Structure of cancer incidence in children

The spectrum of malignancies in childhood is radically different from that in adult patients. The predominant share consists of systemic lesions and tumors originating from embryonic tissues.

As a percentage of all pediatric malignant tumors, the breakdown is as follows:

  • Leukemias are the absolute leaders, accounting for 30% of all cases.
  • Central nervous system (CNS) tumors occur in 21% of cases.
  • Lymphomas (including Hodgkin disease) account for 14%.
  • Neuroblastoma is diagnosed in 6.8% of cases.
  • Rhabdomyosarcoma is detected with a frequency of 6.5%.
  • Wilms tumor accounts for 5.2%.
  • Retinoblastoma occurs in 2.7% of cases.
  • Hepatoblastoma is the rarest of the listed ones, accounting for only 0.9%.

Key features of pediatric neoplasms

Pediatric malignant tumors possess a number of unique biological and clinical characteristics that require a special approach to classification and treatment.

The main features include:

  1. Organ nonspecificity. Unlike adult patients, in whom tumors most frequently affect specific organs (e.g., lungs, breast, prostate, colon), in children neoplasms are largely not tethered to a specific organ. A typical example is rhabdomyosarcomas — the most common soft tissue sarcomas in childhood, which do not have an exclusive predisposition to arise solely in striated muscle tissue.
  2. Rarity of carcinomas. True cancer (tumors of epithelial tissue) in children is extremely rare, making up no more than 6% of all cases. In adults, carcinomas are the dominant type.
  3. Age dependence. The incidence rate and tumor types vary significantly depending on the child's age.
  4. Difficulties in classification. The standard histogenetic principle of classification, which works exceptionally well in adults, is not always applicable in pediatric practice. Dysontogenetic tumors may include elements of several germ layers simultaneously.
  5. Prognosis and survival. In general, the prognosis for malignant tumors in children is more favorable. Five-year survival in children exceeds 60%, whereas in adults this figure is less than 50%.
  6. Sensitivity to therapy. Pediatric tumors demonstrate high sensitivity to chemotherapy, whereas adult carcinomas are often poorly sensitive or entirely resistant to it.
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Tumor reversion phenomenon

One of the most striking properties of certain pediatric neoplasms is tumor reversion — the ability for spontaneous or induced transition (maturation) from a malignant form to a benign one.

This phenomenon is explained by the fact that such tumors originate either from stem undifferentiated cells or from embryonic tissues that lagged in their development compared to the surrounding normal tissues of the child.

Known examples of reversion:

  • Malignant neuroblastoma is capable of maturing into benign ganglioneuroma.
  • Malignant hepatoblastoma can transform into benign adenoma.
  • Immature teratoma transitions into mature teratoma via differentiation.

Histological picture and IHC diagnostics

Under standard hematoxylin and eosin staining, the microscopic picture of many pediatric tumors is characterized by the predominance of primitive (embryonic) features over pleomorphic-anaplastic ones. The histological structure often mimics the processes of specific organogenesis of the organ where the tumor arose. To designate such embryonic tumors, the suffix "-blastoma" is frequently used (e.g., nephroblastoma, retinoblastoma).

Many neoplasms consist of primitive cells possessing small hyperchromatic nuclei and extremely scanty cytoplasm. The stroma in such tumors is virtually absent, and they are represented by dense sheets of cells. In pathomorphology, the terms solid tumors or small round blue cell tumors are used to designate them.

The differential diagnostic range of such neoplasms includes lymphoma, neuroblastoma, rhabdomyosarcoma, and primitive neuroectodermal tumor. For an accurate diagnosis, the use of immunohistochemical (IHC) markers is critically important:

  • CD45 (leukocyte common antigen) and other cluster of differentiation (CD) markers are used to identify hematopoietic cells.
  • Neuron-specific enolase and synaptophysin serve as neuronal markers.
  • Myogenin, desmin, actin are expressed in muscle differentiation.
  • Vimentin acts as a universal marker of mesenchymal differentiation.
  • CD99 is a characteristic marker for primitive neuroectodermal tumors.

Comparative characteristics: children vs. adults

The differences between pediatric and adult oncology are systemic in nature. This concerns both the biology of the tumor itself and the approaches to diagnosis and prevention.

SignIn childrenIn adults
Target of lesionPredominantly tissues (lymphoid, nervous, hematopoietic)Predominantly organs
Histological typeMostly sarcomas87% are carcinomas
Stage at diagnosisAbout 80% are already disseminatedOften localized or regional
Screening and preventionScreening not developed, prevention unlikelyScreening well-established, prevention possible in 80% of cases
Early diagnosisOften possible only by chanceConstantly improving

Briefly: answers to topical questions

What are the main histological types of hemangiomas?

The main histological types of hemangiomas are classified into several groups according to their structure. The following variants are distinguished:

  • Capillary (simple, flat, senile) — consists of small capillary-type vessels surrounded by connective tissue.
  • Racemose (cirsoid, plexiform) — represented by a tangle of wide and tortuous arterial and venous vessels.
  • Cavernous — consists of dilated vascular cavities lined with a single layer of endothelium.
  • Mixed — different areas of the tumor exhibit various structural patterns.

Also, a separate classification distinguishes 4 main types: capillary, cavernous, sclerosing hemangiomas, and telangiectasias.

What are the microscopic features of capillary hemangioma?

Microscopically, capillary hemangioma consists of differentiated small capillary-type vessels.

  • Vascular structures — densely packed thin-walled capillaries and small cavities filled with blood.
  • Endothelium — the cavities are lined with endothelium, and the capillary walls may be formed by markedly swollen (hypertrophic) endothelium.
  • Stroma — poorly developed, represented by connective tissue surrounding the vessels.

What are the histological features of cavernous hemangioma?

Histologically, cavernous hemangioma is a vascular lesion built of large, thin-walled caverns.

  • Vascular cavities — dilated caverns of various sizes communicating with each other through anastomoses.
  • Lining and contents — large thin-walled cavities lined with a single layer of endothelium and filled with blood or thrombotic masses.
  • Stroma — the walls of the cavities vary in thickness and are separated by thin stromal layers (connective tissue septa).
  • Capsule — the tumor has a fibrous capsule.

Where are cavernous hemangiomas most commonly localized?

Cavernous hemangiomas are most frequently localized in the following areas:

  • Skin and mucous membranes — a frequent external localization.
  • Internal organs — the liver is affected especially often, where cavernous hemangioma is the most common benign tumor (often located subcapsularly).
  • Soft tissues and submucosal layer — also potential sites of development.

In bone tissue, this tumor is extremely rare.

What immunohistochemical markers are used to confirm the endothelial origin of a hemangioma?

For the immunohistochemical study of juvenile hemangiomas, the GLUT-1 marker is used: a positive reaction with the GLUT-1 antibody is noted. In routine hematoxylin and eosin staining during the involution stage, vascular cavities are visualized.

What is the phenomenon of spontaneous regression in juvenile (infantile) hemangioma?

The phenomenon of spontaneous regression (involution) of infantile hemangioma consists of a predictable sequence of its developmental phases.

  • Growth phase — in the first weeks and months of life, there is an initial period of rapid growth and an increase in tumor size.
  • Stabilization phase — at the age of 1–3 years, tumor growth stops.
  • Involution phase — thereafter (usually by age 5), the neoplasm undergoes subsequent slow involution (regression).

As part of which hereditary syndrome do multiple hemangiomas of the cerebellum and retina occur?

Multiple vascular neoplasms of the cerebellum and retina occur as part of von Hippel-Lindau syndrome.

  • Hippel-Lindau syndrome — is a hereditary autosomal dominant disorder, one of the manifestations of which is the presence of hemangiomas. Within this syndrome, patients develop multiple hemangioblastomas of the cerebellum and/or spinal cord, as well as retinal angiomatosis.

What fatal complications can cavernous hemangioma of the liver cause?

Cavernous hemangioma of the liver can lead to life-threatening conditions, especially when giant in size.

  • Intraperitoneal hemorrhage — occurs as a result of spontaneous rupture of the hemangioma, which is the most dangerous complication.
  • Kasabach-Merritt phenomenon — the development of consumption coagulopathy with thrombocytopenia due to platelet sequestration within a giant tumor, which can lead to severe hemorrhagic diathesis.

What is a glomus tumor (glomangioma) and where is it typically located?

A glomus tumor (glomus angioma, Masson tumor) is a benign vascular neoplasm originating from glomus-type arteriovenous anastomoses in the skin (neuromyoarterial receptors involved in thermoregulation). Key characteristics:

  • Localization — predominantly the distal phalanges of the fingers and toes, as well as the subungual space.
  • Macroscopy — a bluish-red nodule ranging from 0.3 to 1 cm in size.
  • Clinical presentation — characterized by pain and potential vasomotor disturbances.

What is the Kasabach-Merritt syndrome in the context of giant vascular tumors?

Kasabach-Merritt syndrome (giant hemangioma with thrombocytopenia) is a severe complication occurring with large capillary or cavernous hemangiomas (more than 5–6 cm in diameter). The pathogenesis of the syndrome is driven by widespread blood clotting (vascular thrombosis) within the tumor itself. This leads to platelet sequestration, consumption coagulopathy, thrombocytopenia, and, consequently, hemorrhagic diathesis accompanied by thrombocytopenic purpura.

How to memorize

To memorize IHC markers of myogenic differentiation, use the MDA abbreviation: Myogenin, Desmin, Actin.

Class and exam questions

What does the tumor reversion phenomenon consist of?

Reversion is the unique ability of certain malignant embryonal neoplasms in children to mature and transform into benign tumors (for example, the transition of a neuroblastoma into a ganglioneuroma).

Why are pediatric tumors called organ-nonspecific?

Because they often develop not from the epithelium of specific organs, but from embryonic tissues or stem cells, which can be located in various parts of the body. Rhabdomyosarcoma is a characteristic example.

What is the main histological pattern of small round blue cell tumors?

On hematoxylin and eosin staining, they appear as clusters of primitive cells with hyperchromatic small nuclei, extremely scant cytoplasm, and an almost complete absence of stroma.

Which marker is used to confirm primitive neuroectodermal tumor?

For the differential diagnosis of primitive neuroectodermal tumors, CD99 is a specific immunohistochemical marker.

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What else to study on this topic

  • Detailed analysis of the morphology of small round blue cell tumors
  • Pathogenesis of dysembryoplastic neoplasms
  • Specific IHC markers in pediatric oncology
  • Clinical and morphological features of rhabdomyosarcoma
  • Mechanisms of tumor reversion at the cellular level

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