J Pathol Transl Med.  2017 Mar;51(2):171-175. 10.4132/jptm.2016.08.29.

Malignant Solitary Fibrous Tumor with Heterologous Rhabdomyosarcomatous Differentiation: A Case Report

Affiliations
  • 1Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. songjs@amc.seoul.kr
  • 2Department of Radiology, Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
  • 3Department of Orthopedic Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Abstract

Malignant solitary fibrous tumor (MSFT) is a well-described entity, from which heterologous differentiation is extremely rare. We encountered a case of MSFT with rhabdomyosarcomatous differentiation in a 56-year-old man. This patient presented with a large mass in his posterior thigh. He had been treated with chemoradiation for sarcoma involving the cervical spine, right femoral head, and both lungs 6 months earlier. A wide excision was performed. The mass measured 10.6 cm and showed a fish-flesh cut surface with necrotic foci. Microscopically, the tumor showed heterogeneous cellularity with a hemangiopericytic vascular pattern. A hypercellular area showed spindle cells or epithelioid cells with high mitotic activity (63/10 high-power fields) and immunoreactivity for CD34 and CD99. A hypocellular area and a cystic area showed pleomorphic rhabdoid cells with immunoreactivity for desmin and myogenin. This is a report of a rare case of MSFT with rhabdomyosarcomatous differentiation and presents new histologic features of MSFT.

Keyword

Solitary fibrous tumors; Malignant; Rhabdomyosarcoma

Figure

  • Fig. 1. Magnetic resonance image revealing a well-defined enhancing mass in the postero-medial compartment of the left thigh (arrow) (A), with metastasis in the C2 vertebral body (arrow) (B). (C) Chest computed tomography showing multiple nodules in both lungs. (D) Grossly, a large lobulated mass is observed in the left thigh. The cut surface of the mass is yellow-tan and firm with necrotic foci.

  • Fig. 2. Microscopic findings. (A) The mass consists of a hypercellular area (H), a hypocellular area (L), and a cystic area (C). (B) The hypercellular area is composed of short spindle or epithelioid cells with a hemangiopericytic vasculature. (C) The hypocellular area shows plump epithelioid cells with inflammatory cells. (D) The cystic area is unilocular and contacted both the hypercellular and the hypocellular areas.

  • Fig. 3. Morphologic and immunohistochemical findings. (A) The hypercellular area is composed of short spindle or epithelioid cells with prominent nucleoli and high mitotic activity (63/10 highpower fields) and shows immunoreactivity for CD34 (B) and immunonegativity for desmin (C) and myogenin (D). (E) The hypocellular area is composed of plump epithelioid cells and shows immunoreactivity for CD34 (F) and strong immunopositivity for desmin (G) and myogenin (H). (I) The cystic component is composed of pleomorphic rhabdoid cells and shows focal immunoreactivity for CD34 (J) and strong immunopositivity for desmin (K) and myogenin (L).


Cited by  2 articles

Frozen Cytology of Meningeal Malignant Solitary Fibrous Tumor/Hemangiopericytoma
Myunghee Kang, Na Rae Kim, Dong Hae Chung, Gie-Taek Yie
J Pathol Transl Med. 2019;53(3):192-197.    doi: 10.4132/jptm.2019.03.20.

Recurrent malignant solitary fibrous tumor of the scalp: a case report and literature review
Ahmed Rabie, Abdulkarim Hasan, Yasein Mohammed, Ayman Abdelmaksoud, Ali A. Rabaan
J Pathol Transl Med. 2022;56(2):103-108.    doi: 10.4132/jptm.2021.10.29.


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