Extraneural capillary haemangioblastoma
Definition
A tumour occurring outside the central nervous system, identical top that more commonly found within the brain, in the posterior fossa, spinal cord and retina. The histogenesis is uncertain.
Epidemiology
This is a tumour of adults. There is an association with von Hippel-Lindau disease1,5,9.
Clinical features
Extraneural capillary haemangioblastoma may occur at a range of sites including in association with peripheral nerves3,5, in liver6,9, lung6, pancreas, retroperitoneum4, kidney, urinary bladder, lower extremities7,8 and nasal skin2.
Histopathology
The tumour is highly vascular, comprising capillaries surrounded by stromal cells with clear or vacuolated cytoplasm. These stromal cells may resemble lipoblasts. Necrosis, pleomorphism and mitotic activity are lacking. There are areas of paucicellular sclerotic stroma.
Immunohistochemistry
| |
AE1/AE3
|
0/41
|
|
|
Cam5.2
|
0/31, 0/14
|
|
EMA
|
0/31, 0/17
|
|
SMA
|
1/3(focal positivity)1
|
|
MSA
|
1/4(focal positivity)1
|
|
Desmin
|
0/41, 0/14, 0/17
|
|
Calponin
|
2/3(focal in one case)1, 1/14, 1/17
|
|
CD31
|
0/41, 0/17
|
|
CD34
|
0/51, 0/14, 0/17
|
|
CD57
|
1/14
|
|
Factor VIII
|
0/31
|
|
S-100
|
5/5(focal in one case)1, 1/14,
1/3(focal)5, 1/17
|
|
HMB-45
|
0/31, 0/14, 0/17
|
|
GFAP
|
0/31, 0/14, 0/17
|
|
Vimentin
|
4/4(focal in one case)1, 1/14, 3/35
|
|
Calretinin
|
0/31
|
|
WT1
|
0/31
|
|
Inhibin
|
1/51
|
|
Chromogranin
|
1/21
|
|
NSE
|
1/14, 3/35, 1/17
|
| |
|
Differential diagnosis
Prognosis
The tumour does not recur after surgical resection5.
References
1 Nonaka D, Rodriguez J,Rosai J Extraneural hemangioblastoma: a report of 5 cases. Am J Surg Pathol 2007; 31:1545-51
2 Boyd AS,Zhang J. Hemangioblastoma arising in the skin. Am J Dermatopathol 2001; 23:482-4
3 Brodkey JA, Buchignani JA,O'Brien TF. Hemangioblastoma of the radial nerve: case report. Neurosurgery 1995; 36:198-200; discussion 200-1
4 Fanburg-Smith JC, Gyure KA, Michal M, et al. Retroperitoneal peripheral hemangioblastoma: a case report and review of the literature. Ann Diagn Pathol 2000; 4:81-7
5 Giannini C, Scheithauer BW, Hellbusch LC, et al. Peripheral nerve hemangioblastoma. Mod Pathol 1998; 11:999-1004
6 McGrath FP, Gibney RG, Morris DC, et al. Case report: multiple hepatic and pulmonary haemangioblastomas--a new manifestation of von Hippel-Lindau disease. Clin Radiol 1992; 45:37-9
7 Michal M, Vanecek T, Sima R, et al. Primary capillary hemangioblastoma of peripheral soft tissues. Am J Surg Pathol 2004; 28:962-6
8 Patton KT, Satcher RL, Jr.,Laskin WB. Capillary hemangioblastoma of soft tissue: report of a case and review of the literature. Hum Pathol 2005; 36:1135-9
9 Rojiani AM, Owen DA, Berry K, et al. Hepatic hemangioblastoma. An unusual presentation in a patient with von Hippel-Lindau disease. Am J Surg Pathol 1991; 15:81-6
This page last revised 14.2.2008
©SMUHT/PW Bishop