Agaimy, Abbas and Gaumann, Andreas and Schroeder, Josef and Dietmaier, Wolfgang and Hartmann, Arndt and Hofstaedter, Ferdinand and Wuensch, Peter H. and Mentzel, Thomas (2007) Primary and metastatic high-grade pleomorphic sarcoma/malignant fibrous histiocytoma of the gastrointestinal tract: an approach to the differential diagnosis in a series of five cases with emphasis on myofibroblastic differentiation. VIRCHOWS ARCHIV, 451 (5). pp. 949-957. ISSN 0945-6317,
Full text not available from this repository. (Request a copy)Abstract
Primary and metastatic so-called malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma (MFH) is rare in the gastrointestinal (GI) tract with approximate to 50 primary and five metastatic cases reported so far. We evaluated two primary gastric and three metastatic intestinal high-grade pleomorphic sarcomas with features of storiform-pleomorphic MFH. Gastric tumours occurred in a 79-year-old man and a 68-year-old woman. One patient died post-operatively, and the other was disease-free at 6 months. Three patients presented with GI metastasis 24, 60 and 0 months after diagnosis of MFH of the heart (n=1) and the thigh (n=2). Metastases were located in the small (n=1) and large bowel (n=2) and were characteristically pedunculated and polypoid with oedematous haemorrhagic stroma. Concurrent metastases (brain, lung, bone) were present in all three cases. Tumours expressed alpha-smooth muscle actin (four of five), platelet-derived growth factor receptor (PDGFR) alpha (three of three) and PDGFR beta (two of three) but were negative for CD117, CD34 and other lineage-specific markers. Ultrastructural examination revealed myo/fibroblastic features. Both gastric MFH were wild type for KIT and PDGFR alpha. In conclusion, primary and metastatic MFH of the GI tract commonly express PDGFR alpha and show a myo/fibroblastic phenotype. They should be distinguished from a variety of primary and metastatic pleomorphic neoplasms, in particular high-grade sarcomatous GI stromal tumours (GIST), pleomorphic leiomyosarcoma, sarcomatoid carcinoma and other mimics.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | SOFT-TISSUE TUMORS; STROMAL TUMORS; ASCENDING COLON; GIANT-CELLS; H-CALDESMON; LEIOMYOSARCOMA; SARCOMA; MYOFIBROSARCOMA; CLASSIFICATION; EXPRESSION; MFH; gastrointestinal; GIST; peritoneum; PDGFR alpha; PDGFR beta |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Pathologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 01 Dec 2020 12:24 |
| Last Modified: | 01 Dec 2020 12:24 |
| URI: | https://pred.uni-regensburg.de/id/eprint/32055 |
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