Abstract
Primary pituitary sarcoma in the absence of previous radiation therapy is very rare, only eight cases having been reported. We present the first reported case of sclerosing epithelioid fibrosarcoma of the pituitary, a 56-year-old woman presenting with diplopia and panhypopituitarism. Magnetic resonance imaging showed a 1.2 × 0.4 × 0.5-cm sellar mass invading the right cavernous sinus. Despite surgery and radiation therapy, our patient developed intracranial metastases and died 2 years after the initial diagnosis. Sclerosing epithelioid fibrosarcoma is an infrequently occurring, distinct variant of soft tissue fibrosarcoma. Its immunophenotype includes vimentin and occasionally CD-34, B cell lymphoma-2, S-100 protein, or keratin expression. Although initially thought to be a low-grade lesion, sclerosing epithelioid fibrosarcomas may also behave in a high-grade manner.
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Acknowledgment
The authors acknowledge the diagnostic assistance of Dr. Antonio G. Nascimento of the Bone and Soft Tissue Working Group, the photographic expertise of Mr. James Hopfenspirger, and the secretarial service of Mrs. Denise Chase, all of Mayo Clinic. Appreciation is likewise extended to Willie Hernandez for manuscript and figure preparation and JoLynne Marsh for secretarial assistance.
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Massier, A., Scheithauer, B.W., Taylor, H.C. et al. Sclerosing Epithelioid Fibrosarcoma of the Pituitary. Endocr Pathol 18, 233–238 (2007). https://doi.org/10.1007/s12022-007-9010-2
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DOI: https://doi.org/10.1007/s12022-007-9010-2