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

Puls, Florian and Pillay, Nischalan and Fagman, Henrik and Palin-Masreliez, Anne and Amary, Fernanda and Hansson, Magnus and Kindblom, Lars-Gunnar and McCulloch, Tom A and Meligonis, George and Muc, Ronald and Rissler, Pehr and Sumathi, Vaiyapuri P and Tirabosco, Roberto and Hofvander, Jakob and Magnusson, Linda and Nilsson, Jenny and Flanagan, Adrienne M and Mertens, Fredrik (2018) PRDM10-rearranged Soft Tissue Tumor: A Clinicopathologic Study of 9 Cases. The American journal of surgical pathology. ISSN 1532-0979. This article is available to all HEFT staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their HEFT Athens login IDs

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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.

Item Type: Article
Additional Information: This article is available to all HEFT staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their HEFT Athens login IDs
Subjects: QZ Pathology. Oncology
Divisions: Clinical Support > Pathology
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Depositing User: Mrs Caroline Tranter
Date Deposited: 21 Dec 2018 14:15
Last Modified: 21 Dec 2018 14:15
URI: http://www.repository.heartofengland.nhs.uk/id/eprint/1801

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