PRDM10 -rearranged Soft Tissue Tumor : A Clinicopathologic Study of 9 Cases
(2019) In American Journal of Surgical Pathology 43(4). p.504-513- 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... (More)
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.
(Less)
- author
- organization
- publishing date
- 2019
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- immunohistochemistry, PRDM10, sarcoma, superficial CD34-positive fibroblastic tumor, translocation-associated sarcoma
- in
- American Journal of Surgical Pathology
- volume
- 43
- issue
- 4
- pages
- 504 - 513
- publisher
- Lippincott Williams & Wilkins
- external identifiers
-
- scopus:85059131040
- pmid:30570551
- ISSN
- 0147-5185
- DOI
- 10.1097/PAS.0000000000001207
- language
- English
- LU publication?
- yes
- id
- 4cd9c793-dbe4-49ff-a388-b04b69a45f53
- date added to LUP
- 2019-01-18 10:44:20
- date last changed
- 2024-06-11 02:43:08
@article{4cd9c793-dbe4-49ff-a388-b04b69a45f53,
abstract = {{<p>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.</p>}},
author = {{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}},
issn = {{0147-5185}},
keywords = {{immunohistochemistry; PRDM10; sarcoma; superficial CD34-positive fibroblastic tumor; translocation-associated sarcoma}},
language = {{eng}},
number = {{4}},
pages = {{504--513}},
publisher = {{Lippincott Williams & Wilkins}},
series = {{American Journal of Surgical Pathology}},
title = {{PRDM10 -rearranged Soft Tissue Tumor : A Clinicopathologic Study of 9 Cases}},
url = {{http://dx.doi.org/10.1097/PAS.0000000000001207}},
doi = {{10.1097/PAS.0000000000001207}},
volume = {{43}},
year = {{2019}},
}