Pituitary stalk hemangioblastoma: The fourth case report and review of the literature

Fomekong, Edward; Hernalsteen, Daniëlle; Godfraind, Catherine; D’Haens, Jean; Raftopoulos, Christian

doi:10.1016/j.clineuro.2006.11.007

Cited by 24 publications

(25 citation statements)

References 31 publications

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“…No more than 27 reported cases to date (including our patient's case) describe HBLs originating in the sellar and suprasellar region (see [1] and references therein, and [2,8-11]) of which 18 were confirmed with histopathology (Table 1). Of the 27 cases, only seven (26%) were sporadic.…”

Section: Discussionmentioning

confidence: 67%

“…Hemangioblastomas (HBLs) are benign, slowly growing and highly vascular tumors of the central nervous system (CNS), accounting for just 1% to 2.5% of all intracranial neoplasms, and 7% to 12% of primary tumors located in the posterior fossa [1]. In up to one in four cases of HBL there is an association with von Hippel-Lindau (VHL) disease [2], a rare autosomal dominant condition that predisposes patients to multisystemic neoplastic disorders such as HBLs of the CNS, retinal angiomas, renal cell carcinoma, pheochromocytomas, serous cystadenomas and neuroendocrine tumors of the pancreas.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2011

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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.

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Section: Discussionmentioning

confidence: 67%

Section: Introductionmentioning

confidence: 99%

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

et al. 2011

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“…When associated with VHL, the mean age at diagnosis is third decade; however, sporadic cases are seen a decade later. It is commonly seen in cerebellum6–8; however, they can be found in brain stem and spinal cord 3 9. On the basis of macroscopic examination, they can be divided into simple cystic, macrocystic, solid and microcystic type.…”

Section: Discussionmentioning

confidence: 99%

Cerebellar haemangioblastoma: a rare entity

Aziz

Alam²,

Varshney³

et al. 2011

Case Reports

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“…Either mutations or deletions in other chromosomes or epigenetic or non-genetic phenomena may be required for tumor formation in specific organs of patients with VHL syndrome [Gordeuk et al, 2004;Glasker et al, 2006]. Cerebellar or supratentorial HBL can be managed with excision, with or without conventional radiation or stereotactic radiosurgery [Niemela et al, 1996;Fomekong et al, 2007]. Retinal HBL is usually treated with laser photocoagulation, cryotherapy, or excision [Singh et al, 2002;Liang et al, 2007].…”

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confidence: 99%

“…The early age of onset of her pituitary dysfunction and the relatively large size of her intrasellar HBL resemble features of the present case. Other VHL patients had HBL that involved the structures adjacent to the sella turcica (pituitary stalk, sphenoid sinus, optic chiasm, floor of third ventricle); many of them had hypopituitarism or vision loss [Fomekong et al, 2007].…”

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confidence: 99%

Hypogonadotrophic hypogonadism due to intrasellar hemangioblastoma in von Hippel–Lindau syndrome

Barton

Goncalves²,

Santani

et al. 2009

American J of Med Genetics Pt A

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TO THE EDITOR:Intrasellar hemangioblastoma (HBL) has been described in few patients with von Hippel-Lindau (VHL) syndrome [Rho 1969;Dan and Smith, 1975;Sawin et al., 1996]. We report on a 26-year-old white man who presented with intermittent headache and dizziness and also complained of infertility. He did not have anosmia. His father died at age 62 of heart disease; his mother, two full brothers, and two half-brothers reported that they were healthy. He was small (height 160 cm, weight 43.6 kg) and had markedly decreased secondary sexual characteristics, including small testicles ($15 cm 3 each). Neurologic examination was normal. Funduscopic examination revealed a small HBL in the left superior retina.MRI scanning revealed a cystic mass in the left cerebellar hemisphere; there was deviation of the brain stem and moderate compression of the fourth ventricle (Fig. 1). A homogeneous 1.5 cm mass filled the sella turcica and extended slightly into the suprasellar cistern; the posterior pituitary was displaced posteriorly and compressed against the dorsum sellae. There was marked enhancement of the intrasellar mass after injection of gadolinium diethyltriaminepentacetic acid (Gd-DTPA) (Fig. 2). CT scans of the abdomen and pelvis were normal. Hemoglobin was 150 g/L.Serum hormone levels were: total testosterone 1.42 nmol/mL (reference 8.36-28.70 nmol/mL), free (direct) testosterone 0.03 nmol/L (reference 0.17-0.73 nmol/L), luteinizing hormone <0

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Pituitary stalk hemangioblastoma: The fourth case report and review of the literature

Cited by 24 publications

References 31 publications

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

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

Cerebellar haemangioblastoma: a rare entity

Hypogonadotrophic hypogonadism due to intrasellar hemangioblastoma in von Hippel–Lindau syndrome

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