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 Download (3MB) | Preview |
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 |
| Open access status: | An open access version is available from UCL Discovery |
| 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 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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