Suprasellar Hemangioblastoma Unrelated to von Hippel-Lindau Disease Successfully Treated through Extended Transsphenoidal Approach: Diagnostic Value of Von Hippel-Lindau Disease Gene-Derived Protein

Jia Wenting; Yoshikazu Ogawa; Jun Ito; Teiji Tominaga

doi:10.1055/s-0036-1582016

Journal of Neurological Surgery Part A: Central European Neurosurgery, Table of Contents

J Neurol Surg A Cent Eur Neurosurg 2017; 78(03): 296-301
DOI: 10.1055/s-0036-1582016

Case Report

Georg Thieme Verlag KG Stuttgart · New York

Suprasellar Hemangioblastoma Unrelated to von Hippel-Lindau Disease Successfully Treated through Extended Transsphenoidal Approach: Diagnostic Value of Von Hippel-Lindau Disease Gene-Derived Protein

Authors

Jia Wenting

¹Department of Neurosurgery, Kohnan Hospital, Sendai, Miyagi, Japan
Yoshikazu Ogawa

¹Department of Neurosurgery, Kohnan Hospital, Sendai, Miyagi, Japan
Jun Ito

²Department of Neurosurgery, Hachinohe City Hospital, Hachinohe, Aomori, Japan
Teiji Tominaga

³Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan

Abstract

Hemangioblastoma tends to occur in the infratentorial regions and rarely in the supratentorial regions. This tumor is strongly associated with von Hippel-Lindau disease (VHLD), especially in the supratentorial regions, with only 12 cases of suprasellar hemangioblastoma unrelated to VHLD. However, all these cases were diagnosed based on clinical screening and confirmation of family history. We report a case of suprasellar hemangioblastoma that was successfully removed through an extended transsphenoidal approach and diagnosed as a sporadic case unrelated to VHLD by immunohistochemical examination.

A 67-year-old woman had mild diabetes insipidus and a visual field defect. Head magnetic resonance imaging revealed a suprasellar tumor compressing the optic chiasm upward. The tumor was totally removed without complication through an extended transsphenoidal approach. Postoperative histologic examination disclosed large vacuolated stromal cells and rich capillary networks, and the diagnosis was established as hemangioblastoma. Clinical screening and confirmation of family history revealed no specific results, and additional immunohistochemical staining showed diffuse cytoplasmic expression of anti-VHLD gene-derived protein (pVHL).

We emphasize that pVHL immunohistochemical evaluation would be wise to adopt, especially for patients with a high risk of VHLD related to younger age and supratentorial lesions.

Keywords

hemangioblastoma - pVHL - sporadic - suprasellar tumor - transsphenoidal surgery

Full Text

References

References
1 Hussein MR. Central nervous system capillary haemangioblastoma: the pathologist's viewpoint. Int J Exp Pathol 2007; 88 (5) 311-324
2 Louis DN, Ohgaki H, Wiestler OD , et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 2007; 114 (2) 97-109
3 Zamzuri I, Ghazali MM, Zainuddin N , et al. Molecular genetic analysis of a supratentorial haemangioblastoma in a non-Von Hippel Lindau patient. Med J Malaysia 2005; 60 (3) 360-363
4 Mills SA, Oh MC, Rutkowski MJ, Sughrue ME, Barani IJ, Parsa AT. Supratentorial hemangioblastoma: clinical features, prognosis, and predictive value of location for von Hippel-Lindau disease. Neuro Oncol 2012; 14 (8) 1097-1104
5 Bladowska J, Zimny A, Guziński M , et al. Usefulness of perfusion weighted magnetic resonance imaging with signal-intensity curves analysis in the differential diagnosis of sellar and parasellar tumors: preliminary report. Eur J Radiol 2013; 82 (8) 1292-1298
6 Fu H, Hao S, Wu Z, Zhang J, Zhang L. Sporadic pituitary stalk hemangioblastoma. Neurol India 2011; 59 (6) 937-938
7 Grisoli F, Gambarelli D, Raybaud C, Guibout M, Leclercq T. Suprasellar hemangioblastoma. Surg Neurol 1984; 22 (3) 257-262
8 Ikeda M, Asada M, Yamashita H, Ishikawa A, Tamaki N. A case of suprasellar hemangioblastoma with thoracic meningioma [in Japanese]. No Shinkei Geka 2001; 29 (7) 679-683
9 Kachhara R, Nair S, Radhakrishnan VV. Sellar-sphenoid sinus hemangioblastoma: case report. Surg Neurol 1998; 50 (5) 461-463 ; discussion 463–464
10 Miyata S, Mikami T, Minamida Y, Akiyama Y, Houkin K. Suprasellar hemangioblastoma. J Neuroophthalmol 2008; 28 (4) 325-326
11 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 (1) 24-30
12 Peker S, Kurtkaya-Yapicier O, Sun I, Sav A, Pamir MN. Suprasellar haemangioblastoma. Report of two cases and review of the literature. J Clin Neurosci 2005; 12 (1) 85-89
13 Prabhu K, Daniel RT, Chacko G, Chacko AG. Optic nerve haemangioblastoma mimicking a planum sphenoidale meningioma. Br J Neurosurg 2009; 23 (5) 561-563
14 Rumboldt Z, Gnjidić Z, Talan-Hranilović J, Vrkljan M. Intrasellar hemangioblastoma: characteristic prominent vessels on MR imaging. AJR Am J Roentgenol 2003; 180 (5) 1480-1481
15 Xie T, Zhang X, Hu F , et al. Suprasellar hemangioblastoma mimicking a craniopharyngioma: result of extended endoscopic transsphenoidal approach—case report. Neurol Med Chir (Tokyo) 2013; 53 (10) 735-739
16 Lee GI, Kim JM, Choi KS, Kim CH. Sporadic hemangioblastoma in the pituitary stalk: a case report and review of the literature. J Korean Neurosurg Soc 2015; 57 (6) 465-468
17 Shimoda Y, Ogawa Y, Endo H, Watanabe M, Tominaga T. Coexistence of sporadic cerebellar hemangioblastoma and pituitary null cell adenoma: simultaneous expression of von Hippel-Lindau gene product. Case report. Neurol Med Chir (Tokyo) 2012; 52 (8) 591-594
18 Shimoda Y, Ogawa Y, Watanabe M, Tominaga T. Clinicopathological investigation of vascular endothelial growth factor and von Hippel-Lindau gene-related protein expression in immunohistochemically negative pituitary adenoma—possible involvement in tumor aggressiveness. Endocr Res 2013; 38 (4) 242-250
19 Maher ER, Neumann HP, Richard S. von Hippel-Lindau disease: a clinical and scientific review. Eur J Hum Genet 2011; 19 (6) 617-623
20 Wordinger RJ, Dickey JF, Hill Jr JR. Influence of a progestogen on the cytophysiologic character of the bovine adenohypophysis. Am J Vet Res 1977; 38 (4) 449-454
21 Nishizawa S, Ohta S, Oki Y. Spontaneous resolution of diabetes insipidus after pituitary stalk sectioning during surgery for large craniopharyngioma. Endocrinological evaluation and clinical implications for surgical strategy. Neurol Med Chir (Tokyo) 2006; 46 (3) 126-134 ; discussion 134–135
22 Ogawa Y, Kawaguchi T, Tominaga T. Outcome and mid-term prognosis after maximum and radical removal of craniopharyngiomas with the priority to the extended transsphenoidal approach—a single center experience. Clin Neurol Neurosurg 2014; 125: 41-46
23 Peyre M, David P, Van Effenterre R , et al; French NCI Network VHL Disease and Inherited Predisposition to Kidney Cancer. Natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease. Neurosurgery 2010; 67 (3) 577-587 ; discussion 587
24 Trepanier A, Ahrens M, McKinnon W , et al; National Society of Genetic Counselors. Genetic cancer risk assessment and counseling: recommendations of the National Society of Genetic Counselors. J Genet Couns 2004; 13 (2) 83-114
25 Woodward ER, Wall K, Forsyth J, Macdonald F, Maher ER. VHL mutation analysis in patients with isolated central nervous system haemangioblastoma. Brain 2007; 130 (Pt 3): 836-842