Pleomorphic hyalinizing angiectatic tumor of soft parts: ultrastructural analysis of a case with original features.
Pleomorphic hyalinizing angiectatic tumor, a rare neoplasm of uncertain lineage resembling malignant fibrous histiocytoma and schwannoma, was first described in 1996 by M. E. F. Smith et al. (Am Surg Pathol. 20:21-29). To date, less than 100 cases have been reported in the international literature. It occurs in subcutaneous and intramuscular soft tissues of extremities or trunk in adults without sex predilection. All lesions are composed of sheets and fascicles of spindled and pleomorphic cells associated with clusters of thick-walled ectatic vessels surrounded by a perivascular hyaline material and inflammatory cells such as mast cells. About one-half of these neoplasms express CD34. No patient has developed metastases but occasional local recurrences are possible. This tumor of uncertain lineage is suggested to be an aggressive locally growing low-grade sarcoma. Only 3 cases were previously studied by electron microscopy and appeared to consist of primitive fibroblastic cells. The authors report histological and ultrastructural characteristics of a new case of PHAT excised from the right buttock of a 66-year-old man with the presence of ganglion-like cells, a feature that has not been previously reported, and unusual central ischemic necrosis. The features of this case are suggestive of a fibroblastic origin.
Capovilla
Mathieu
M
Laboratoire Pol-Bouin, Hôpital Maison-Blanche, Reims, France. mat_cap@caramail.com
Birembaut
Philippe
P
Cucherousset
Joël
J
Ploton
Dominique
D
de Saint-Maur
Paul P
PP
Fléjou
Jean-François
JF
Lesec
Guy
G
D000368:Aged; D018322:Angiofibroma / Q000175:diagnosis; D014408:Biomarkers, Tumor / Q000378:metabolism; D001808:Blood Vessels / Q000473:pathology; D003937:Diagnosis, Differential; D005350:Fibroma / Q000175:diagnosis; D015726:Giant Cells / Q000473:pathology; D051677:Histiocytoma, Malignant Fibrous / Q000175:diagnosis; D006801:Humans; D006818:Hyalin / Q000378:metabolism; D007124:Immunoenzyme Techniques; D008297:Male; D009442:Neurilemmoma / Q000175:diagnosis; D012509:Sarcoma / Q000378:metabolism / Q000601:surgery / Q000648:ultrastructure*; D012983:Soft Tissue Neoplasms / Q000378:metabolism / Q000601:surgery / Q000648:ultrastructure*
D014408:Biomarkers, Tumor