"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series.

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State: Public
Version: Final published version
License: All rights reserved
Serval ID
serval:BIB_D5DCACB3D0F6
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series.
Journal
The American journal of surgical pathology
Author(s)
Guillou L., Wadden C., Coindre J.M., Krausz T., Fletcher C.D.
ISSN
0147-5185 (Print)
ISSN-L
0147-5185
Publication state
Published
Issued date
02/1997
Peer-reviewed
Oui
Volume
21
Number
2
Pages
130-146
Language
english
Notes
Publication types: Case Reports ; Journal Article ; Review
Publication Status: ppublish
Abstract
Eighteen examples of an unusual malignant soft-tissue neoplasm, the morphology of which ranged from that of "atypical" epithelioid sarcoma to that of a rhabdoid tumor or undifferentiated carcinoma (with transitional forms) are described. Patients included 11 males and seven females; their median age was 35.5 years with most patients aged 20 to 40 years. Development of a mass was the main presenting symptom. Six tumors developed in the pelvis and perineal region, four in the pubic region and vulva, three in the buttocks, one in the deep soft tissues of the left hip, one on the penis, one in left forearm, one in left axilla, and one on the occiput. Tumor size ranged from 1 to 20 cm (median, 4 cm). On microscopic examination, the tumor cells invaded the subcutaneous or deep soft tissues, had prominent epithelioid or rhabdoid features, had marked cytologic atypia, and grew in a multinodular pattern in half of the cases. Areas of necrosis were often seen. A granuloma-like pattern reminiscent of that observed in classic epithelioid sarcoma was observed in only two cases. Immunohistochemically, positivity for cytokeratin, epithelial membrane antigen, and vimentin was seen in all but one of the cases. Of 16 cases, 10 and eight tumors reacted with desmin and CD34, respectively; five of 15 reacted at least focally with smooth-muscle actin, whereas three of 13 and one of 10 reacted for HMB-45 and carcinoembryonic antigen, respectively. S-100 protein and CD31 yielded negative results. Seven tumors were investigated at the ultrastructural level, four of which showed prominent intracytoplasmic intermediate filament aggregates, often accumulating into paranuclear whorls, which is in keeping with the rhabdoid phenotype. Five tumors showed features of epithelial differentiation (i.e., tonofilament-like structures or desmosomes or both), whereas one tumor displayed features of myofibroblastic differentiation. Differential diagnoses include mainly conventional epithelioid sarcoma, extrarenal malignant rhabdoid tumor, epithelioid malignant peripheral nerve sheath tumor, melanoma, rhabdomyosarcoma, and undifferentiated carcinoma. Follow-up information on 14 patients (range, 4 months to 8 years; median, 19 months) revealed local recurrence in one case and metastatic dissemination in six patients, leading to death in five. In our opinion, the above-described neoplasms represent a usually "proximal-type" of epithelioid sarcoma. In contrast to the conventional, "distal-type" epithelioid sarcoma, the proximal variant is characterized by a predominantly large-cell, epithelioid cytomorphology, marked cytologic atypia, frequent occurrence of rhabdoid features, and lack of a granuloma-like pattern in most cases. It appears to be somewhat more aggressive (or at least metastasizes earlier) than usual epithelioid sarcoma.
Keywords
Adolescent, Adult, Biomarkers, Tumor, Carcinoma/chemistry, Carcinoma/pathology, Carcinoma/therapy, Child, Diagnosis, Differential, Female, Humans, Immunohistochemistry, Male, Microscopy, Electron, Middle Aged, Retrospective Studies, Rhabdoid Tumor/chemistry, Rhabdoid Tumor/pathology, Rhabdoid Tumor/therapy, Sarcoma/chemistry, Sarcoma/pathology, Sarcoma/therapy, Soft Tissue Neoplasms/chemistry, Soft Tissue Neoplasms/pathology, Soft Tissue Neoplasms/therapy
Pubmed
Web of science
Create date
29/01/2008 19:34
Last modification date
16/07/2020 9:42
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