Hemangioblastoma

Classification according to ICD-10
D48.1	Hemangioblastoma
ICD-10 online (WHO version 2019)

Histology of the hemangioblastoma with tightly packed capillary networks and stromal cells (reticular subtype) in between.

Immunohistochemistry: In the staining for CD31, the walls of the numerous blood vessels are marked brown.

The hemangioblastoma is an extremely vascular tumor that can occur in the area of ​​the central nervous system, but also in the soft tissue .

Hemangioblastomas are typically associated with von Hippel-Lindau syndrome (VHL) , but can also occur sporadically. Hemangioblastomas of the central nervous system are classified as benign tumors according to the WHO classification of tumors of the central nervous system as grade I.

localization

Hemangioblastomas are often located in the cerebellum , brain stem, or spinal cord , but rarely occur in the cerebrum . Hemangioblastomas occurring in the area of ​​the retina of the eye are often incorrectly referred to as retinal angiomas.

Symptoms

Depending on the location of the tumor, cerebellar symptoms (e.g. disorders of movement coordination ( ataxia ) or speech disorders ( dysarthria )), a spinal paraplegic syndrome or a root compression syndrome can occur. Since some hemangioblastomas produce the growth factor erythropoietin , a secondary increase in red blood cells ( polycythemia ) can occur.

Diagnosis

In radiological imaging, hemangioblastomas appear as contrast-enhancing, often pseudocystic masses.

pathology

Histologically it is an extremely vascular tumor. In the more common reticular subtype, between densely packed capillary blood vessels, one sees individual larger cells located in the tumor stroma with light or clear, often vacuolated cytoplasms, which are referred to as stromal cells, the histogenesis of which remains unexplained. The cellular subtype already described by Harvey Williams Cushing and Percival Bailey , in which the stromal cells are grouped in cell clusters and whose histological picture is reminiscent of that of paragangliomas, is rarer . When differentiating between metastases from renal cell carcinomas (which also occur more frequently in patients with von Hippel-Lindau syndrome), which is important in the differential diagnosis, the lack of staining of the stromal cells for the epithelial membrane antigen (EMA) and the evidence of low proliferative activity in the staining for Ki67 / MIB-1 will be helpful.

Therapy and prognosis

Therapy is carried out by surgically removing the tumor. After complete removal of the tumor (including the cyst wall), the prognosis is generally favorable. Recurrences occur in about 10% of patients and appear to affect the cellular subtype of hemangioblastoma in particular. Differentiating it from a newly developed second tumor can be difficult in patients with von Hippel-Lindau disease.

literature

Individual evidence

1. ^ P. Bailey, HW Cushing: Tumors arising from the blood vessels of the brain. Springfield 1928.
2. ↑ TJ Hufnagel et al .: Immunohistochemistry of capillary hemangioblastoma. Immunoperoxidase-labeled antibody staining resolves the differential diagnosis with metastatic renal cell carcinoma, but does not explain the histogenesis of the capillary hemangioblastoma. In: The American Journal of Surgical Pathology . 1989; 13 (3), pp. 207-216. PMID 2465700
3. ↑ PC Burger et al: The use of the monoclonal antibody Ki-67 in the identification of proliferating cells: application to surgical neuropathology. In: The American Journal of Surgical Pathology. 1986; 10 (9), pp. 611-617. PMID 2428262
4. ↑ M. Hasselblatt et al .: Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases. In: Neuropathology and Applied Neurobiology . 2005; 31 (6), pp. 618-622. PMID 16281910 .

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