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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2021; 40(02): e190-e194
DOI: 10.1055/s-0040-1718999
Case Report | Relato de Caso

Co-Occurrence of Pineal-Region and Pituitary-Stalk Hemangioblastomas in a Patient Presenting with Von Hippel-Lindau Disease – A Case Report

Ocorrência simultânea de hemangioblastomas na região pineal e na haste hipofisária em paciente com doença de Von Hippel-Lindau – Relato de Caso
Gustavo Rassier Isolan 
1   Centro Avançado de Neurocirurgia e Neurologia (CEANNE), Porto Alegre, Rio Grande do Sul, Brazil
,
Gerson Evandro Perondi 
2   Hospital Cristo Redentor, Porto Alegre, Rio Grande do Sul, Brazil
,
Amauri Dalla-Corte 
3   Universidade do Vale do Rio dos Sinos (UNISINOS), São Leopoldo, Rio Grande do Sul, Brazil
,
Allan Fernando Giovanini 
4   Experimental Pathology, Mestrado Profissional em Odontologia Clínica, Universidade Positivo, Curitiba, Paraná, Brazil
,
João Paulo Mota Telles 
5   Department of Neurology, Division of Neurosurgery, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, Brazil
,
Nícollas Nunes Rabelo 
5   Department of Neurology, Division of Neurosurgery, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, Brazil
,
Eberval Gadelha Figueiredo 
5   Department of Neurology, Division of Neurosurgery, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, Brazil
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Abstract

Introduction Hemangioblastomas of the pineal region or pituitary stalk are extremely rare. Only two cases of hemangioblastomas involving the pineal region have been reported, and four involving the pituitary stalk. The purpose of the present manuscript is to describe an unusual case of supposed hemangioblastoma found concomitantly in the pineal region and pituitary stalk of a patient diagnosed with Von Hippel-Lindau (VHL) disease.

Case Report A 35-year-old female patient with a previous diagnosis of VHL complaining of occipital headaches and balance disturbances for three weeks, who previously had a cerebellar hemangioblastoma resected. The visual characteristics of the tumor suggested a friable vascular lesion with a reddish-brown surface, and an incisional biopsy was performed. The tumor consisted of a dense vascular network surrounded by fibrous stroma abundant in reticulin and composed by both fusiform and dispersed xanthomatous cells; the immunohistochemistry was immunopositive for neuron-specific enolase and immunonegative for epithelial membranous antigen. The patient has been monitored closely for 2 years, and the supratentorial masses have not presented any volume alteration.

Conclusion This rare association must be taken into account in patients with VHL disease, or at least be suspected in patients who present a thickening of the pituitary stalk and a pineal-region mass. We believe a biopsy of our asymptomatic patient could have been dangerous due to inherent complications like intraoperative bleeding. We recommend close observation of asymptomatic lesions with MRIs every six months or until the lesions become symptomatic. If the pineal-region tumor does become symptomatic, gross resection via a transcallosal approach would be ideal.

Resumo

Introdução Hemangioblastomas da região pineal ou da haste hipofisária são raros. Apenas 2 casos foram reportados para a pineal e 4, para a haste hipofisária. O objetivo deste artigo é relatar um caso incomum de supostos hemangioblastomas encontrados concomitantemente na pineal e na haste hipofisária em paciente com Doença de Von Hippel-Lindau (VHL).

Relato de Caso Mulher de 35 anos, com diagnóstico de VHL e histórico de ressecção de hemangioblastoma cerebelar, apresentou cefaleia occipital e queixas relacionadas ao equilíbrio por 3 semanas. As características visuais do tumor sugeriam lesão vascular friável com superfície vermelho-amarronzada, sendo realizada biópsia incisional. O tumor consistia de rede vascular densa rodeada de estroma fibrótico abundante em reticulina e composto de células xantomatosas. Imunohistoquímica positive para enolase específica de neurônios (NSE) e negative para antígeno membranoso epitelial (EMA). Paciente monitorada por 2 anos, sem alteração nas massas.

Conclusão Essa associação rara deve ser suspeitada em pacientes com VHL, na presença de espessamento da haste hipofisária e massa pineal. Acreditamos que uma biópsia, para a paciente assintomática, seria perigosa devido a complicações inerentes como hemorragia intra-operatória. Recomendamos observação de lesões assintomáticas com RM a cada 6 meses ou até que as lesões se tornem sintomáticas. Caso o tumor da região pineal se torne sintomático, ressecção macroscópica por via transcalosa seria ideal.

Keywords

tumor - von hippel-lindau - hemangioblastoma

Palavras-chave

tumor - Von Hippel-Lindau - hemangioblastoma


Publication History

Received: 12 June 2020

Accepted: 24 August 2020

Article published online:
26 November 2020

© 2020. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Acikalin MF, Oner U, Tel N, Paşaoğlu O, Altinel F. Supratentorial hemangioblastoma: a case report and review of the literature. Arch Pathol Lab Med 2003; 127 (09) e382-e384
    CrossrefPubMedGoogle Scholar
  • 2 Filling-Katz MR, Choyke PL, Oldfield E. et al. Central nervous system involvement in Von Hippel-Lindau disease. Neurology 1991; 41 (01) 41-46
    CrossrefPubMedGoogle Scholar
  • 3 Fujisawa I, Asato R, Okumura R. et al. Magnetic resonance imaging of neurohypophyseal germinomas. Cancer 1991; 68 (05) 1009-1014
    CrossrefPubMedGoogle Scholar
  • 4 Isaka T, Horibe K, Nakatani S, Maruno M, Yoshimine T. Hemangioblastoma of the third ventricle. Neurosurg Rev 1999; 22 (2-3): 140-144
    CrossrefPubMedGoogle Scholar
  • 5 Isolan GR, Krayenbühl N, Mahmoud M, Al-Mefty O. A hemangioblastoma in the pineal region: case report. Neurosurgery 2007; 61 (02) E423 , discussion E423
    CrossrefPubMedGoogle Scholar
  • 6 Leger J, Velasquez A, Garel C, Hassan M, Czernichow P. Thickened pituitary stalk on magnetic resonance imaging in children with central diabetes insipidus. J Clin Endocrinol Metab 1999; 84 (06) 1954-1960
    PubMedGoogle Scholar
  • 7 Lonser RR, Glenn GM, Walther M. et al. von Hippel-Lindau disease. Lancet 2003; 361 (9374): 2059-2067
    CrossrefPubMedGoogle Scholar
  • 8 Lonser RR, Weil RJ, Wanebo JE, DeVroom HL, Oldfield EH. Surgical management of spinal cord hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 2003; 98 (01) 106-116
    CrossrefPubMedGoogle Scholar
  • 9 Lundin P, Pedersen F. Volume of pituitary macroadenomas: assessment by MRI. J Comput Assist Tomogr 1992; 16 (04) 519-528
    CrossrefPubMedGoogle Scholar
  • 10 Prieto R, Roda JM. Hemangioblastoma of the lateral ventricle: case report and review of the literature. Neurocirugia (Astur) 2005; 16 (01) 58-62
    CrossrefPubMedGoogle Scholar
  • 11 Richard S, David P, Marsot-Dupuch K, Giraud S, Béroud C, Resche F. Central nervous system hemangioblastomas, endolymphatic sac tumors, and von Hippel-Lindau disease. Neurosurg Rev 2000; 23 (01) 1-22 , discussion 23–24
    CrossrefPubMedGoogle Scholar
  • 12 Wanebo JE, Lonser RR, Glenn GM, Oldfield EH. The natural history of central nervous system hemangioblastomas in patients with von Hippel–Lindau disease. J Neurosurg 2003; 98: 82-94
    CrossrefPubMedGoogle Scholar
  • 13 Wasenko JJ, Rodziewicz GS. Suprasellar hemangioblastoma in Von Hippel-Lindau disease: a case report. Clin Imaging 2003; 27 (01) 18-22
    CrossrefPubMedGoogle Scholar
  • 14 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
    CrossrefPubMedGoogle Scholar
  • 15 Weil RJ, Lonser RR, DeVroom HL, Wanebo JE, Oldfield EH. Surgical management of brainstem hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 2003; 98 (01) 95-105
    CrossrefPubMedGoogle Scholar
  • 16 Van Velthoven V, Reinacher PC, Klisch J, Neumann HP, Gläsker S. Treatment of intramedullary hemangioblastomas, with special attention to von Hippel-Lindau disease. Neurosurgery 2003; 53 (06) 1306-1313 , discussion 1313–1314
    CrossrefPubMedGoogle Scholar
  • 17 Sajadi A, de Tribolet N. Unusual locations of hemangioblastomas. Case illustration. J Neurosurg 2002; 97 (03) 727
    CrossrefPubMedGoogle Scholar
  • 18 Egeler RM, D'Angio GJ. Langerhans cell histiocytosis. J Pediatr 1995; 127 (01) 1-11
    CrossrefPubMedGoogle Scholar
  • 19 Kilpatrick SE, Wenger DE, Gilchrist GS, Shives TC, Wollan PC, Unni KK. Langerhans' cell histiocytosis (histiocytosis X) of bone. A clinicopathologic analysis of 263 pediatric and adult cases. Cancer 1995; 76 (12) 2471-2484
    CrossrefPubMedGoogle Scholar
  • 20 Pomarede R, Czernichow P, Finidori J. et al. Endocrine aspects and tumoral markers in intracranial germinoma: an attempt to delineate the diagnostic procedure in 14 patients. J Pediatr 1982; 101 (03) 374-378
    CrossrefPubMedGoogle Scholar