Manifestation of a sellar hemangioblastoma due to pituitary apoplexy: a case report

Abstract

IntroductionHemangioblastomas are rare, benign tumors occurring in any part of the nervous system. Most are found as sporadic tumors in the cerebellum or spinal cord. However, these neoplasms are also associated with von Hippel-Lindau disease. We report a rare case of a sporadic sellar hemangioblastoma that became symptomatic due to pituitary apoplexy.Case presentationAn 80-year-old, otherwise healthy Caucasian woman presented to our facility with severe headache attacks, hypocortisolism and blurred vision. A magnetic resonance imaging scan showed an acute hemorrhage of a known, stable and asymptomatic sellar mass lesion with chiasmatic compression accounting for our patient's acute visual impairment. The tumor was resected by a transnasal, transsphenoidal approach and histological examination revealed a capillary hemangioblastoma (World Health Organization grade I). Our patient recovered well and substitutional therapy was started for panhypopituitarism. A follow-up magnetic resonance imaging scan performed 16 months postoperatively showed good chiasmatic decompression with no tumor recurrence.ConclusionsA review of the literature confirmed supratentorial locations of hemangioblastomas to be very unusual, especially within the sellar region. However, intrasellar hemangioblastoma must be considered in the differential diagnosis of pituitary apoplexy.

Spontaneous Hemorrhage from Central Nervous System Hemangioblastomas

“…Although all of our hemorrhagic presentations occurred in posterior fossa tumors, several case reports exist of supratentorial (8,28) or intraspinal hemorrhagic hemangioblastomas (8,14,19,26), thus mandating inclusion of hemorrhagic hemangioblastoma on the differential diagnosis of any hemorrhagic lesion.…”

Supratentorial haemangioblastoma without von Hippel–Lindau syndrome in an adult: A rare tumor with review of literature

“…[ 16 ] On reviewing the literature from 1902 to 2014, we found 57 cases of supratentorial HBL without VHL as shown in Table 1 . [ 6 8 9 15 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 ] On reviewing the literature, we found that supratentorial HBL affected patients ranging from 3 months to 80 years with maximum cases belonging to 20–30 years and 40–50 years age group. Among the infants, only one case was found, thus making it a rare tumor in infancy.…”

Recurrent hemorrhage in hemangioblastoma involving the posterior fossa: Case report

“…[ 9 ] To this date, 56 cases of acute hemorrhage have been described with an underlying HGB. [ 1 2 3 5 6 7 10 11 15 16 17 18 20 22 23 24 25 31 33 34 35 37 41 ] A total of 19 cases have been described in the posterior fossa. Large hemorrhages related to HGBs have been described more commonly in spinal locations.…”