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PRDM10-rearranged Soft Tissue Tumor A Clinicopathologic Study of 9 Cases

Puls, F; Pillay, N; Fagman, H; Palin-Masreliez, A; Amary, F; Hansson, M; Kindblom, L-G; ... Mertens, F; + view all (2019) PRDM10-rearranged Soft Tissue Tumor A Clinicopathologic Study of 9 Cases. The American Journal of Surgical Pathology , 43 (4) pp. 504-513. 10.1097/PAS.0000000000001207.

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Flanagan AMF edit PRDM10 manuscript 23.08.2018.pdf - Accepted version
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Abstract

Gene fusion transcripts containing PRDM10 were recently identified in low-grade undifferentiated pleomorphic sarcomas (UPS). Here, we describe the morphologic and clinical features of 9 such tumors from 5 men and 4 women (age: 20 to 61 y). Three cases had previously been diagnosed as UPS, 3 as superficial CD34-positive fibroblastic tumor (SCD34FT), 2 as pleomorphic liposarcoma, and 1 as pleomorphic hyalinizing angiectatic tumor. The tumors were located in the superficial and deep soft tissues of the thigh/knee region (4 cases), shoulder (2 cases), foot, trunk, and perineum (1 case each) ranging in size from 1 to 6 cm. All showed poorly defined cellular fascicles of pleomorphic cells within a fibrous stroma with frequent myxoid change and a prominent inflammatory infiltrate. All displayed highly pleomorphic nuclear features, but a low mitotic count. Most tumors were well circumscribed. One of 9 tumors recurred locally, but none metastasized. Immunohistochemically, all were CD34+ and showed nuclear positivity for PRDM10; focal positivity for cytokeratins was seen in 5/6 cases. PRDM10 immunoreactivity was evaluated in 50 soft tissue tumors that could mimic PRDM10-rearranged tumors, including 4 cases exhibiting histologic features within the spectrum of SCD34FT. Except for 2/6 pleomorphic liposarcomas and 1/4 myxofibrosarcomas, other tumors did not show nuclear positivity but displayed weak to moderate cytoplasmic immunoreactivity. In conclusion, PRDM10-rearranged soft tissue tumor is characterized by pleomorphic morphology and a low mitotic count. Its morphologic spectrum overlaps with SCD34FT. Clinical features of this small series suggest an indolent behavior, justifying its distinction from UPS and other sarcomas.

Type: Article
Title: PRDM10-rearranged Soft Tissue Tumor A Clinicopathologic Study of 9 Cases
DOI: 10.1097/PAS.0000000000001207
Publisher version: https://doi.org/10.1097/PAS.0000000000001207
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Science & Technology, Life Sciences & Biomedicine, Pathology, Surgery, PRDM10, sarcoma, superficial CD34-positive fibroblastic tumor, translocation-associated sarcoma, immunohistochemistry, MYXOINFLAMMATORY FIBROBLASTIC SARCOMA, PLEOMORPHIC LIPOSARCOMA, FIBROUS HISTIOCYTOMA, GENE FUSIONS, CANCER, EXPRESSION, EMPHASIS, NEOPLASM, FEATURES, PROTEIN
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Pathology
URI: https://discovery.ucl.ac.uk/id/eprint/10072104
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