Michele Persico1, Hui-Kuo Shu2, Jim Zhong2
1Neurology, Emory University School of Medicine, 2Radiation Oncology, Winship Cancer Institute at Emory University
Objective:
To present a rare case of a sellar/suprasellar hemangiopericytoma (HPC).
Background:
HPCs are uncommon neoplasms of pericytes, accounting for only 0.4% of primary central nervous system tumors. HPCs are diagnosed more frequently in Caucasians (81.3%) and slightly more frequently in females (53.1%). The mean age of onset is 55.1 years old. Approximately one-third occurs in the head and neck. Among the intracranial hemangiopericytomas, 60% occurs in the skull base and 26% in the falx/parasagittal area. Hemangiopericytomas located in the sella turcica region are extremely rare with only 21 case reports described to date and none presenting as incidentaloma.
Results:
A 62-year-old African-American female with encephalopathy, severe sepsis, and acute kidney injury was transferred from an outside hospital to Emory University for neurosurgical evaluation of a 2.1 x 2.3 x 2.9 cm suprasellar mass with associated mass effect upon the optic chiasm identified on MRI during her hospitalization. Because of her encephalopathy, a review of systems to investigate for headache and vision changes was deferred. An endocrine workup was otherwise unremarkable and a working diagnosis of a clinically silent pituitary adenoma was made. Therefore, a transsphenoidal adenectomy was performed. Gross morphology was of a highly vascular, firm tumor infiltrating the dura and adherent to the sella wall and undersurface of the suprasellar diaphragm. Frozen section evaluation revealed positivity for reticulin and final pathologic diagnosis was anaplastic hemangiopericytoma. Following surgery, the patient developed pituitary insufficiency requiring hormonal replacement and was treated adjuvantly with proton therapy to the dose of 59.4 Gy given in 33 fractions (1.8 Gy/fraction) to the gross disease with a 3 mm clinical target volume margin. Currently, the patient is three years from treatment with no evidence of recurrent disease on serial imaging.
Conclusions:
Suprasellar anaplastic hemangiopericytomas are particularly rare brain tumors that can be misdiagnosed with non-functioning pituitary adenomas.