Primary Pituitary Fibrosarcoma with Previous Adenoma

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), in contrast, are exceptionally rare tumors with very few cases described in the literature thus fa...

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Bibliographic Details
Published in:World neurosurgery 2017-09, Vol.105, p.1032.e7-1032.e11
Main Authors: Bayoumi, Ahmed B., Chen, Jenny X., Swiatek, Peter R., Laviv, Yosef, Kasper, Ekkehard M.
Format: Article
Language:English
Subjects:
Adenoma - diagnostic imaging
Adenoma - pathology
Adenoma - surgery
Adult
Decompression, Surgical - methods
Diagnosis, Differential
Female
Fibrosarcoma - diagnostic imaging
Fibrosarcoma - secondary
Fibrosarcoma - surgery
Gadolinium - pharmacokinetics
Humans
Magnetic Resonance Imaging
Pituitary
Pituitary Gland - diagnostic imaging
Pituitary Gland - metabolism
Pituitary Gland - pathology
Pituitary Neoplasms - diagnostic imaging
Pituitary Neoplasms - surgery
Sarcoma
Sella turcica
Skull base
Transsphenoidal surgery
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Summary: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), in contrast, 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. A 39-year-old woman presented with 2 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 postoperatively was found to be a primary fibrosarcoma with embedded adenoma remnants. On post-hoc examination of her original pathologic specimen, a prominent fibrous tissue component was suspected to be the source of her fibrosarcoma. 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.
ISSN:1878-8750
1878-8769
DOI:10.1016/j.wneu.2017.05.096