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Table of Contents    
Year : 2011  |  Volume : 59  |  Issue : 6  |  Page : 937-938

Sporadic pituitary stalk hemangioblastoma

Department Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China

Date of Submission02-Oct-2011
Date of Decision28-Oct-2011
Date of Acceptance06-Nov-2011
Date of Web Publication2-Jan-2012

Correspondence Address:
Liwei Zhang
Department Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.91396

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How to cite this article:
Fu H, Hao S, Wu Z, Zhang J, Zhang L. Sporadic pituitary stalk hemangioblastoma. Neurol India 2011;59:937-8

How to cite this URL:
Fu H, Hao S, Wu Z, Zhang J, Zhang L. Sporadic pituitary stalk hemangioblastoma. Neurol India [serial online] 2011 [cited 2020 Feb 22];59:937-8. Available from:


A 49-year-old man was admitted with a 2-year history of progressive worsening headache, episodic vomiting, and incidental polydipsia and hyperuresis. There was no family history of von Hippel-Lindau (VHL) disease. The preoperative endocrinological profile was normal. Magnatic resonance imaging (MRI) revealed a 1.9-cm globular solid tumor in the suprasellar region [Figure 1]a-c. Total resection was performed via a right frontal-temporal craniotomy. The obviously enlarged edematous optic nerve was seen. The top and lateral sides of the tumor were easily peeled from the optic pathway and the wall of the third ventricle. The histological diagnosis was hemangioblastoma (HBL) [Figure 2]. The molestation of the pituitary stalk resulted in panhypopituitarism, which recovered after management with substitutive therapy after 6 months. There was no recurrence during a follow-up period of more than 3.5 years [Figure 1]d.
Figure 1: Preoperative and postoperative magnetic resonance imaging (MRI) of the patient. Preoperative sagittal T1-weighted MRI showing an isointense homogenous suprasellar mass. Note the thickened edematous optic chiasm (arrow) (a). Axial T2-weighted images demonstrating mixed iso- and hyperintense signal. The hypointense areas of the flow void (arrow) (b). Sagittal T1-weighted contrasted image showing a strongly enhanced mass. The pituitary gland is indicated by an arrow (c). Postoperative sagittal T1-MRI demonstrating no recurrence 3.5 years after surgery. The blurry pituitary stalk (arrow) (d)

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Figure 2: Histopathological examination of the tumor. Photomicrograph demonstrating the typical histological features of HBL: A rich vascular network (stars) surrounded by sheets of large, pale, vacuolated stromal cells (arrows). (H and E ×200) (a). The endotheliocytes were positive for CD34. (S and P ×200) (b). The stromal cells were positive for S-100. (S and P×200) (c). The endotheliocytes and the stromal cells were positive for vimentin. (S and P ×200) (d)

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HBL occurs predominantly in the cerebellar hemisphere, spinal cord, and medulla oblongata in adults aged 30-40 years. [1] HBL arising from supratentorial locations is rare, and an origin at the pituitary stalk is even rarer. The most common signs and symptoms in these cases included visual disturbance, irregular menstruation, headache, and polydipsia hyperuresis. For this case, visual disturbance was not present, but headache and occasional polydipsia hyperuresis were reported. The discrete border with the hypothalamus and the optic chiasm might be the reason. MRI is important and necessary for the diagnosis [2],[3],[4] and angiographic study is also useful in revealing a highly vascularized lesion with a persistent blush. On MRI, sellar and suprasellar HBL is consistently isointense on T1-weighted sequences and hyperintense on T2-weighted sequences. Rumboldt et al, observed the signal of flow voids within the tumor on the T2-weighted images and regarded them as a characteristic feature of the tumor. [5] The same feature was also observed in the present patient. These regions are considered to be intratumoral vascular structures. In addition, the tumor was vividly enhanced with contrast administration on T1-weighted images. For most sellar and suprasellar tumors, the optic nerve is thinned as a result of the compression. However, in this patient, the optic nerve is edematous and thickened. It might be the blockage of vein backflow. For sporadic pituitary stalk HBL, it is difficult to distinguish from meningioma, pituitary adenoma and craniopharyngioma. We think that the absence of characteristic dural "tail" signs of meningioma and HBL, which enhance more strongly with contrast than pituitary tumors, is helpful in identifying the nature of these lesions. Additionally, the normal signals from the pituitary gland are also received by pituitary stalk HBL. To the best of our knowledge, calcification was not found in any reported cases, and it is helpful to distinguish the pituitary stalk HBL with craniopharyngioma. The review of the literatures revealed that surgical resection of the pituitary stalk HBL was reported in five patients [2],[3],[6],[7] Excluding Neuman's reported case, which was not reported in detail, resection was completed in three patients and incomplete in one patient. [6] Two of the three patients who underwent complete resection lost all pituitary function after surgery. The preoperative panhypopituitarism persisted after surgery in the third patient. The patient who underwent partial HBL resection maintained normal pituitary function after surgery. The tumor was completely removed for the present case. He recovered from panhypopituitarism, which persisted for 6 months. To conclude, sporadic pituitary stalk HBL is associated with a good outcome when surgical excision is completed. The prognosis of pituitary stalk HBL association with VHL disease will depend mainly on other manifestations of VHL disease.

  References Top

1.Wanebo JE, Lonser RR, Glenn GM, Oldfield EH. The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 2003;98:82-94.  Back to cited text no. 1
2.Cao Y, Gao P, Wang S, Zhao J. Pituitary infundibulum hemangioblastoma detected by dynamic enhancement MRI. Can J Neurol Sci 2010;37:697-9.  Back to cited text no. 2
3.Lonser RR, Butman JA, Kiringoda R, Song D, Oldfield EH. Pituitary stalk hemangioblastomas in von Hippel-Lindau disease. J Neurosurg 2009;110:350-3.  Back to cited text no. 3
4.Prabhu K, Daniel RT, Chacko G, Chacko AG. Optic nerve haemangioblastoma mimicking a planum sphenoidale meningioma. Br J Neurosurg 2009;23:561-3.  Back to cited text no. 4
5.Rumboldt Z, Gnjidic Z, Talan-Hranilovic J, Vrkljan M. Intrasellar hemangioblastoma: Characteristic prominent vessels on MR imaging. AJR Am J Roentgenol 2003;180:1480-1.  Back to cited text no. 5
6.Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P. Hemangioblastomas of the central nervous system: A 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg 1989;70:24-30.  Back to cited text no. 6
7.E, Hernalsteen D, Godfraind C, D'Haens J, Raftopoulos C. Pituitary stalk hemangioblastoma: The fourth case report and review of the literature. Clin Neurol Neurosurg 2007;109:292-8.  Back to cited text no. 7


  [Figure 1], [Figure 2]


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