In:
Archives of Pathology & Laboratory Medicine, Archives of Pathology and Laboratory Medicine, Vol. 130, No. 8 ( 2006-08-01), p. 1219-1222
Abstract:
Neoplasms of perivascular epithelioid cells (PEComas) have in common the coexpression of muscle and melanocytic immunohistochemical markers. Although this group includes entities with distinct clinical features, such as angiomyolipoma, clear cell sugar tumor of the lung, and lymphangioleiomyomatosis, similar tumors have been documented in an increasing diversity of locations. The term PEComa is now generally used in reference to these lesions that are not angiomyolipomas, clear cell sugar tumors, or lymphangioleiomyomatoses. While most reported PEComas have behaved in a benign fashion, malignant PEComas have occasionally been documented. We present a case of hepatic PEComa with benign histologic features, which nonetheless presented with metastases to multiple sites nearly 9 years later. This case represents the second documented malignant PEComa of the liver, as well as the longest follow-up of a surviving patient with a malignant PEComa, emphasizing both the need for criteria that more accurately predict the behavior of PEComas and the necessity of long-term follow-up of patients with PEComas.
Type of Medium:
Online Resource
ISSN:
1543-2165
,
0003-9985
DOI:
10.5858/2006-130-1219-MNOPEC
Language:
English
Publisher:
Archives of Pathology and Laboratory Medicine
Publication Date:
2006
detail.hit.zdb_id:
2028916-9
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