9150/1 Hemangiopericytoma, NOS



Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72

A mesenchymal tumor composed of dilated vascular spaces (some with a staghorn or bifed shape) throughout. The individual tumor cells are surrounded by reticulin everywhere, are negative for muscle, nerve sheath, and epithelial markers, and are positive for CD34 and CD99. The biologic behavior of hemangiopericytoma cannot always be predicted from the morphologic features. The 5- and 10-year survival rates of malignant hemangiopericytomas have been reported to be 40% and 29% respectively 1
 
Mills SE, Carter D, Greenson JK, Oberman HA, Reuter VE
Sternberg's Diagnostic Surgical Pathology
4th Edition
Lippincott, Williams & Wilkins: Philadelphia 2004



.
Hemangiopericytomas closely resemble cellular areas of solitary fibrous tumour (SFT) and and are closely related to, if not synonymous with, SFT
2
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



. Lesions formerly known as infantile HPC fall within the spectrum of infantile myofibromatosis.



Breast
ICD-O-3 topography code: C50

Haemangiopericytoma (HPC) is a mesenchymal tumour histologically characterized by dilated vascular spaces, often with a staghorn or bifed shape. Individual tumour cells are surrounded by a reticulin network and are positive for CD34 and CD99. Haemangiopericytomas closely resemble cellular areas of solitary fibrous tumour (SFT) and and are closely related to, if not synonymous with, SFT. Lesions formerly known as infantile HPC fall within the spectrum of infantile myofibromatosis
3
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Haemangiopericytoma of the breast is a rare tumour with only around 20 reported primary cases. It affects predominantly adult women, but occasional cases occur in children and in men
4
Click to access Pubmed
Tavassoli FA, Weiss S (1981)
Hemangiopericytoma of the breast.
Am J Surg Pathol 5: 745-52



5
Click to access Pubmed
Arias-Stella J, Rosen PP (1988)
Hemangiopericytoma of the breast.
Mod Pathol 1: 98-103



6
Click to access Pubmed
Talwar S, Prasad N, Gandhi S, Prasad P (1999)
Haemangiopericytoma of the adult male breast.
Int J Clin Pract 53: 485-6



. The tumour typically presents as a round to oval, well circumscribed mass measuring 1-19 cm. Myxoid areas alternating with small cysts filled with a watery fluid can be observed macroscopically. Larger tumours may contain areas of haemorrhage and necrosis. Histologically, haemangiopericytoma is characterized by a compact proliferation of bland ovoid to spindle-shaped cells around branching vessels. Focally trapped mammary ducts and ductules are often found at the periphery of the lesion.
The majority of mammary haemangiopericytoma are benign; recurrence or metastases have not been reported. Wide local excision is often sufficient
7
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31

A mesenchymal tumor composed of dilated vascular spaces (some with a staghorn or bifed shape) throughout. The individual tumor cells are surrounded by reticulin everywhere, are negative for muscle, nerve sheath, and epithelial markers, and are positive for CD34 and CD99. The biologic behavior of hemangiopericytoma cannot always be predicted from the morphologic features. The 5- and 10-year survival rates of malignant hemangiopericytomas have been reported to be 40% and 29% respectively
8
 
Mills SE, Carter D, Greenson JK, Oberman HA, Reuter VE
Sternberg's Diagnostic Surgical Pathology
4th Edition
Lippincott, Williams & Wilkins: Philadelphia 2004



.
Hemangiopericytomas closely resemble cellular areas of solitary fibrous tumour (SFT) and and are closely related to, if not synonymous with, SFT
9
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



. Lesions formerly known as infantile HPC fall within the spectrum of infantile myofibromatosis.



Prostate gland
ICD-O-3 topography code: C61

A mesenchymal tumor composed of dilated vascular spaces (some with a staghorn or bifed shape) throughout. The individual tumor cells are surrounded by reticulin everywhere, are negative for muscle, nerve sheath, and epithelial markers, and are positive for CD34 and CD99. The biologic behavior of hemangiopericytoma cannot always be predicted from the morphologic features. The 5- and 10-year survival rates of malignant hemangiopericytomas have been reported to be 40% and 29% respectively
10
 
Mills SE, Carter D, Greenson JK, Oberman HA, Reuter VE
Sternberg's Diagnostic Surgical Pathology
4th Edition
Lippincott, Williams & Wilkins: Philadelphia 2004



.
Hemangiopericytomas closely resemble cellular areas of solitary fibrous tumour (SFT) and and are closely related to, if not synonymous with, SFT
11
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



. Lesions formerly known as infantile HPC fall within the spectrum of infantile myofibromatosis.



Kidney
ICD-O-3 topography code: C64

Haemangiopericytoma (HPC) is a mesenchymal tumour histologically characterized by dilated vascular spaces, often with a staghorn or bifed shape. Individual tumour cells are surrounded by a reticulin network and are positive for CD34 and CD99. Haemangiopericytomas closely resemble cellular areas of solitary fibrous tumour (SFT) and and are closely related to, if not synonymous with, SFT. Lesions formerly known as infantile HPC fall within the spectrum of infantile myofibromatosis
12
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Primary renal haemangiopericytomas are extremely rare. Most of them arise in the renal sinus and in perirenal tissue. Tumours are large, firm, and histologically composed of fusiform pericytes separated by capillaries with the typical staghorn configuration
13
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



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