Introduction: Primary pituitary fibrosarcoma (PPF) in the absence of radiation therapy is an exceedingly rare entity, and a total of 6 cases have been so far reported in the literature only. Patients typically present with subtle symptoms of a sellar mass such as headache, visual compromise, diplopia, and/or symptoms suggestive of endocrinal dysfunction.
Here we report one further case of PPF and we review all reported data on the other cases to illustrate the common clinical scenarios and develop the best management options available for such a rare pituitary tumor.
Methods: A retrospective review of the patient's medical record and the reported cases in the literature.
Results: A 39-year-old female presented with a two-month history of persistent headaches and rapidly progressive unilateral visual loss. MRI of the brain showed a heterogeneously enhancing sellar lesion abutting the optic chiasm. The patient experienced marked improvement in vision and resolution of headaches after microsurgical transsphenoidal resection. Histopathological examination revealed nests of adenoma embedded in fibrotic stroma. 8 months later, she presented again with headaches and further decline in vision due to a recurrent mass. A 2nd microsurgical transsphenoidal debulking was performed urgently for chiasma decompression. Histopathology now revealed a fibrosarcoma grade 2, with an immunohistochemical profile strongly reactive for Vimentin but negative for Keratin, S100, CD34, Chromogranin, EMA, HMB45 and SMA. Two more resections for focally recurrent tumor were undertaken as well as adjuvant radiotherapy and chemotherapy (Vinorelbine, Lipsomal Doxorubicin, and Gemcitabine). However, the lesion progressed and the patient died 21 months after the initial presentation.
Conclusions: In accordance with the literature, radical resection without preservation of the pituitary gland is advocated in combination with subsequent involved field radiation or whole-brain radiotherapy to reduce the chances of local recurrence and in order to improve recurrence-free survival.
Patient Care: By providing a better plan of management including both early radical surgery and radiation in order to avoid or delay the progression of such an aggressive rare pituitary malignancy when encountered by neurosurgeons, ENT surgeons and/or radiation oncologists in clinical practice.
Learning Objectives: By conclusion of this session, participants must be able to 1) understand the reported clinical scenarios of that rare pituitary tumor 2) recognize the natural history of the disease and the histopathological diagnosis including the immunohistochemical profile 3) plan the best available and comprehensive management for those patients with PPF based on the data we will provide so as to have a better outcome in terms of local control and incidence of recurrences or distant spread.
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