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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 & 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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