Primary pituitary fibrosarcoma with prior adenoma - a case report of a rare disease.

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Primary pituitary fibrosarcoma with prior adenoma - a case report of a rare disease.

World Neurosurg. 2017 May 27;:

Authors: Bayoumi A, Chen JX, Swiatek PR, Laviv Y, Kasper EM

Abstract
BACKGROUND: Pituitary sarcomas are rare clinical entities most often encountered as secondary neoplasms representing late sequelae of radiation therapy to the sellar region. Primary pituitary fibrosarcomas (PPFS) on the other hand are exceptionally rare tumors with very few cases described in the literature thus far. Herein, we present a case of PFFS and describe it in the context of the existing literature.
CASE DESCRIPTION: A 39 year old female presented with two months of headaches and rapidly progressive vision loss. She was found to have a 2.7 cm pituitary mass and initially underwent transnasal transsphenoidal resection from which pathology confirmed a pituitary adenoma. Eight months after surgery, she represented with vision changes and a recurrent tumor that post-operatively was found to be a primary fibrosarcoma with embedded adenoma remnants. On post-hoc examination of her original pathological specimen, a prominent fibrous tissue component was suspected to be the source of her fibrosarcoma.
CONCLUSIONS: This is a rare case of PFFS that highlights the need for close surveillance in affected patients. Through this case, we review the existing literature and discuss both surgical management and aggressive adjuvant therapy of this rare disease entity.

PMID: 28559065 [PubMed - as supplied by publisher]

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