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Soft Tissue Pathology

Pathology of Pleomorphic

Hyalinizing Angiectatic Tumour

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Dr Sampurna Roy MD              

 

 

Dermatopathology Quiz Case 155

Diagnosis: Pleomorphic Hyalinizing Angiectatic Tumour

 

                                                                                                                      

 

 

Syn: Pleomorphic Hyalinizing Angiectatic Tumour of Soft Parts

Pleomorphic hyalinizing angiectatic tumour is a rare tumour of uncertain lineage. It was first described in 1996 in a series of 14 cases.

It is a low-grade sarcoma which may recur locally. Metastasis has not been recorded.

Site:  The tumour is seen in the subcutaneous tissue of the distal extremities in adults. It is commonly noted on the ankle and feet.

Gross:  These are circumscribed or infiltrative lesion. Most tumours have a  lobulated appearance with a variegated, tan-brown hemorrhagic cut surface.

Cystic and myxoid changes may be present.

Microscopic features:

1. Clusters of dilated thin-walled vessels with fibrinoid change of their wall with superimposed thrombosis & occasionally hemosiderin-laden macrophage.

2. Intervening stroma contain spindle shaped cells and bizarre polygonal multinucleate cells.

These multinucleate cells show marked nuclear pleomorphism,  hyperchromasia and frequently intranuclear inclusions.

In rare cases psammomatous calcifications may be noted.

3. A variable inflammatory background  mainly composed of lymphocytes, plasma cells, and numerous mast cells.

Immunohistochemistry:   Multinucleate cells are CD34 positive (50% cases)  but negative for S-100 protein, actin and desmin.

Differential diagnosis: 

1. Ancient schwannoma (distinguished from schwannomas by the usual presence of infiltrative margins and the absence of S-100 protein) ;

2. Melanotic schwannoma (presence of hemosiderin, psammomatous calcifications, and intranuclear inclusions).

3. Primary cutaneous myxofibrosarcoma  (Primary cutaneous myxofibrosarcoma mimicking pleomorphic hyalinizing angiectatic tumor (PHAT): a potential diagnostic pitfall.Am J Dermatopathol. 2005 Aug;27(4):322-6.)

4. Hemosiderotic fibrohistiocytic lipomatous lesion: Abstract

5. Some early cases may be mistaken for spindle cell lipoma, benign fibrous histiocytoma or nodular fasciitis.

Local recurrence is common in almost 50% of cases.

 

Further reading

Pleomorphic hyalinizing angiectatic tumor of soft parts: Case Report with unusual ganglion-like cells and review of the literature.

Clinicopathologic features of pleomorphic hyalinizing angiectatic tumor of soft parts.

Pleomorphic hyalinizing angiectatic tumor of soft parts: case report and literature review.

Pleomorphic hyalinizing angiectatic tumor of soft parts: ultrastructural analysis of a case with original features.

Pleomorphic hyalinizing angiectatic tumor: analysis of 41 cases supporting evolution from a distinctive precursor lesion.

Pleomorphic hyalinizing angiectatic tumor of soft parts: report of a case and review of the literature.

May 2016

 

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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