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

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskrift

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.

Detaljer

Författare
  • Florian Puls
  • Nischalan Pillay
  • Henrik Fagman
  • Anne Palin-Masreliez
  • Fernanda Amary
  • Magnus Hansson
  • Lars Gunnar Kindblom
  • Tom A. McCulloch
  • George Meligonis
  • Ronald Muc
  • Pehr Rissler
  • Vaiyapuri P. Sumathi
  • Roberto Tirabosco
  • Jakob Hofvander
  • Linda Magnusson
  • Jenny Nilsson
  • Adrienne M. Flanagan
  • Fredrik Mertens
Enheter & grupper
Externa organisationer
  • Sahlgrenska University Hospital
  • University College London
  • Nottingham City Hospital
  • Addenbrooke's Hospital
  • Birmingham Heartlands Hospital
  • Skåne University Hospital
  • Royal National Orthopedic Hospital
Forskningsområden

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Medicinsk genetik

Nyckelord

Originalspråkengelska
Sidor (från-till)504-513
TidskriftAmerican Journal of Surgical Pathology
Volym43
Utgivningsnummer4
Tidigt onlinedatum2018
StatusPublished - 2019
PublikationskategoriForskning
Peer review utfördJa