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Soft Tissue Pathology 

Pathology of Hemangiopericytoma

Dr Sampurna Roy MD             

 

                                                                                                                      

 

 

Hemangiopericytoma was first described by Stout and Murray as a less organoid form of glomus tumour arising from pericytes. HEMANGIOPERICYTOMA: A VASCULAR TUMOR FEATURING ZIMMERMANN'S PERICYTES.

Enzinger and Smith (Hemangiopericytoma. An analysis of 106 cases. Hum Pathol. 1976 Jan;7(1):61-82.) divided this tumour into two groups:

I. Adult  

II. Infantile or Congenital Hemangiopericytoma.

The concept of 'Hemangiopericytoma' has been questioned my many authors due to absence of reproducible  diagnostic criteria.

(Hemangiopericytoma: a dying breed? Reappraisal of an ‘entity’ and its variants: a hypothesis. Curr Diagn Pathol 1994;1:19–23.)

Many soft tissue tumours focally display 'hemangiopericytoma- like' pattern.

Differential diagnosis:  Solitary fibrous tumour ; Synovial Sarcoma ; Infantile Myofibromatosis ; Infantile fibrosarcoma ; Malignant peripheral nerve sheath tumour ; Low-grade endometrial stromal sarcoma. (Mod Pathol. 2005 Jan;18(1):40-7) ;  Juxtaglomerular  tumour ;  (Am J Clin Pathol 2001 Dec;116(6):854-63).

Mesenchymal chondrosarcoma ;  Phosphaturic mesenchymal tumour;  Osteosarcoma;   Malignant mesotheliomas ; Thymomas ; Sarcomatoid carcinomas ; Melanoma.

Adult Hemangiopericytoma

 

Occurs in  elderly patients and is usually located in deep soft tissue, lower extremities, pelvis and retroperitoneum.

It is rarely present within the ceentral nervous system.

Gross:  Solitary well circumscribed solid tumour with a smooth surface.

Sectioning reveals greyish white to reddish brown areas.

Areas of hemorrhage, necrosis and cystic degeneration are often present.

Microscopic features Histopathology Image1 ; Histopathology Image2 .

The tumour is characterized by tightly packed cellular areas surrounding thin walled branching blood vessels.

The tumour cells are small, ovoid to spindle shaped with ill-defined cell boundaries.

The collapsed  blood vessels, lined by flat endothelial cells, are arranged in a 'stag-horn' pattern.

The spindle cells are each surrounded by a reticulin  sheath and are separated from the endothelial cells by a basement membrane.

Silver stain demonstrates extravascular location of the tumour cells and individual cells display pericellular reticulin pattern.

Immunohistochemistry:   Actin and other myoid markers are negative.

Vimentin and CD34 shows nonspecific positivity.

The features indicating poor prognosis include increased cellularity, necrosis , hemorrhage, more than 4 mitotic figures per 10 high power fields.

Visit: Lipomatous hemagiopericytoma ; Sinonasal Hemangiopericytoma ;

 

Further reading:

Optic nerve meningeal hemangiopericytoma: a clinicopathologic case report.

Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. Ge

A case of hemangiopericytoma of the soft palate with articulate disorder and dysphagia.

Central nervous system hemangiopericytoma, retrospective four year pathology case series.

Intraventricular hemangio-pericytoma.

True hemangiopericytoma of the nasal cavity.

Proliferation index as a prognostic marker in hemangiopericytoma of the head and neck.

Hemangiopericytoma: a 20-year single-institution experience.

Myofibromatosis in adults, glomangiopericytoma, and myopericytoma: a spectrum of tumors showing perivascular myoid differentiation. 

The histopathologic spectrum of hemangiopericytoma: application of immuno- histochemical analysis including proliferative markers to facilitate diagnosis and predict prognosis. 

 
Infantile Hemangiopericytoma

Usually present at birth or noted soon after.

This tumour has a benign course.

Site:   Mostly solitary lesion, located on the head and neck area, extremities or the trunk.

Microscopic features:  The multilobulated tumour displays polymorphic population of cells consisting of spindle cells with myofibroblastic features and a more primitive round cells (both are smooth muscle actin positive).

There are distinct intravascular and perivascular satellite nodules outside the main tumour mass.  There is evidence of intravascular endothelial cell proliferation. 

Mitotic figures are noted together with focal necrosis and vascular invasion (this is not related to bad prognosis).

It has been suggested that infantile myofibromatosis and infantile hemangiopericytoma represent different stages of maturation  of the same lesion.

 

Further reading:

Childhood hemangiopericytoma: review of St Jude Children's Research Hospital.

Splenic hemangiopericytoma in a one-month-old infant.

Multicentric infantile hemangiopericytoma: case report and review of the literature.

Infantile hemangiopericytoma: a clinico-morphological study of 10 cases.

Infantile hemangiopericytoma--case report and literature review.

Fetal hemangiopericytoma with an associated cerebral anomaly.

Infantile hemangiopericytoma versus infantile myofibromatosis. Study of a series suggesting a continuous spectrum of infantile myofibroblastic lesions. 

Hemangiopericytoma in pediatric ages: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group.Cancer.

Hemangiopericytoma in children and infants.

Childhood splenic hemangiopericytoma: a previously unreported entity.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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