Journal of Pathology and Translational Medicine

Review

Neuropathologic features of central nervous system hemangioblastoma: Rebecca A. Yoda, Patrick J. Cimino; J Pathol Transl Med. 2022;56(3):115-125. Published online May 3, 2022; DOI: https://doi.org/10.4132/jptm.2022.04.13

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Hemangioblastoma is a benign, highly vascularized neoplasm of the central nervous system (CNS). This tumor is associated with loss of function of the VHL gene and demonstrates frequent occurrence in von Hippel-Lindau (VHL) disease. While this entity is designated CNS World Health Organization grade 1, due to its predilection for the cerebellum, brainstem, and spinal cord, it is still an important cause of morbidity and mortality in affected patients. Recognition and accurate diagnosis of hemangioblastoma is essential for the practice of surgical neuropathology. Other CNS neoplasms, including several tumors associated with VHL disease, may present as histologic mimics, making diagnosis challenging. We outline key clinical and radiologic features, pathophysiology, treatment modalities, and prognostic information for hemangioblastoma, and provide a thorough review of the gross, microscopic, immunophenotypic, and molecular features used to guide diagnosis.

Citations

Citations to this article as recorded by

Immunohistochemical Expression of PAX8 in Central Nervous System Hemangioblastomas: A Potential Diagnostic Pitfall for Neuropathologists
Giuseppe Broggi, Jessica Farina, Valeria Barresi, Francesco Certo, Giuseppe Maria Vincenzo Barbagallo, Gaetano Magro, Rosario Caltabiano
Applied Immunohistochemistry & Molecular Morphology.2025;[Epub] CrossRef
Endolymphatic Sac Tumor. Post-Radiosurgery Evaluation Using Time-Resolved Imaging of Contrast Kinetics MR Angiography
Antonella Blandino, Allegra Romano, Chiara Filippi, Sofia Pizzolante, Andrea Romano, Giulia Moltoni, Edoardo Covelli, Maurizio Barbara, Alessandro Bozzao
Ear, Nose & Throat Journal.2025;[Epub] CrossRef
Stereotactic radiosurgery in the management of central nervous system hemangioblastomas: a systematic review and meta-analysis
Amirhossein Zare, Amirhessam Zare, Alireza Soltani Khaboushan, Bardia Hajikarimloo, Jason P. Sheehan
Neurosurgical Review.2025;[Epub] CrossRef
Characterization of spinal hemangioblastomas in patients with and without von Hippel-Lindau, and YAP expression
Ana-Laura Calderón-Garcidueñas, Steven-Andrés Piña-Ballantyne, Eunice-Jazmín Espinosa-Aguilar, Rebeca de Jesús Ramos-Sánchez
Revista Española de Patología.2024; 57(3): 160. CrossRef
Patients With Hemangioblastoma: Mood Disorders and Sleep Quality
Ali Riazi, Yaser Emaeillou, Nima Najafi, Mohammad Hoseinimanesh, Mohammad Ibrahim Ashkaran, Donya Sheibani Tehrani
Brain Tumor Research and Treatment.2024; 12(2): 87. CrossRef
Radiosurgically Treated Recurrent Cerebellar Hemangioblastoma: A Case Report and Literature Review
François Fabi, Ève Chamberland, Myreille D’Astous, Karine Michaud, Martin Côté, Isabelle Thibault
Current Oncology.2024; 31(7): 3968. CrossRef
Dual manifestations: spinal and cerebellar hemangioblastomas indicative of von Hippel-Lindau syndrome
Nurhuda Hendra Setyawan, Rachmat Andi Hartanto, Rusdy Ghazali Malueka, Ery Kus Dwianingsih, Dito Pondra Dharma
Radiology Case Reports.2024; 19(11): 5000. CrossRef
Phenotypic and Genotypic Features of a Chinese Cohort with Retinal Hemangioblastoma
Liqin Gao, Feng Zhang, J. Fielding Hejtmancik, Xiaodong Jiao, Liyun Jia, Xiaoyan Peng, Kai Ma, Qian Li
Genes.2024; 15(9): 1192. CrossRef
Case report: Hemangioblastoma in the brainstem of a dog
Kirsten Landsgaard, Samantha St. Jean, Stephanie Lovell, Jonathan Levine, Christine Gremillion, Brian Summers, Raquel R. Rech
Frontiers in Veterinary Science.2023;[Epub] CrossRef
Intramedullary hemangioblastoma of the thoracic cord with a microsurgical approach: A case report and literature review
Eduardo Cattapan Piovesan, Werner Petry Silva, Adroaldo Baseggio Mallmann, Felipe Severo Lanzini, Bruna Zanatta de Freitas, Francisco Costa Beber Lemanski, Charles André Carazzo
Surgical Neurology International.2023; 14: 137. CrossRef
Secondary Holocord Syringomyelia Associated With Spinal Hemangioblastoma in a 29-Year-Old Female
Eric Chun-Pu Chu, Edouard Sabourdy, Benjamin Cheong
Cureus.2023;[Epub] CrossRef
Belzutifan in adults with VHL-associated central nervous system hemangioblastoma: a single-center experience
Bryan J. Neth, Mason J. Webb, Jessica White, Joon H. Uhm, Pavel N. Pichurin, Ugur Sener
Journal of Neuro-Oncology.2023; 164(1): 239. CrossRef
Resection of Intramedullary Hemangioblastoma: Timing of Surgery and Its Impact on Neurological Outcome and Quality of Life
Michael Schwake, Sarah Ricchizzi, Sophia Krahwinkel, Emanuele Maragno, Stephanie Schipmann, Walter Stummer, Marco Gallus, Markus Holling
Medicina.2023; 59(9): 1611. CrossRef

Case Study

Adrenal hemangioblastoma: Joo-Yeon Koo, Kyung-Hwa Lee, Joon Hyuk Choi, Ho Seok Chung, Chan Choi; J Pathol Transl Med. 2022;56(3):161-166. Published online February 28, 2022; DOI: https://doi.org/10.4132/jptm.2021.12.28

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Abstract PDF: Hemangioblastoma (HB) is a rare benign tumor that most commonly occurs in the cerebellum. HB is composed of neoplastic stromal cells and abundant small vessels. However, the exact origin of stromal cells is controversial. Extraneural HBs have been reported in a small series, and peripheral HBs arising in the adrenal gland are extremely rare. Herein, we report a case of sporadic adrenal HB in a 54-year-old woman. The tumor was a well-circumscribed, yellow mass measuring 4.2 cm in diameter. Histologically, the tumor was composed of small blood vessels and vacuolated stromal cells with clear cytoplasm. On immunohistochemical stain, the stromal cells were positive for S-100 protein, neuron-specific enolase, and synaptophysin. The tumor did not reveal mutation of VHL alleles. We herein present a case of HB of the adrenal gland and review of the literature.

Original Articles

Neoplastic Stromal Cells of Intracranial Hemangioblastomas Disclose Pericyte-derived Mesenchymal Stromal Cells-like Phenotype.: Yong Han Jung, Jeong Kim, Bo Mi Kim, Eun Kyoung Kim, Mi Seon Kang, Soojin Jung, Young Il Yang, Shin Kwang Khang; Korean J Pathol. 2011;45(6):564-572.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.6.564

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Abstract PDF

BACKGROUND
Stromal cells (SCs) of hemangioblastomas (HBs) have been regarded as true neoplastic components, but their ontogeny remains unclear. Convincing evidence suggests that embryonic mesenchymal cells may be the cells of origin of HBs. The aim of the present study was to investigate the immunophenotypic characteristics of neoplastic SCs using a set of markers against endothelial cells (ECs), vascular smooth muscle cells (vSMCs), mesenchymal stromal cells (MSCs), and pericytes.
METHODS
Intracranial HBs (n=46), angiolipoma (n=9), and pyogenic granuloma (n=11) were retrieved and the immunophenotypic profile of SCs was determined by immune stainings.
RESULTS
The MIB-1 labeling index was significantly higher in SCs compared to that of ECs and vSMCs, regardless of the type of lesion. The neoplastic SCs of HBs consistently expressed both MSC and pericyte markers, but did not express markers of ECs and vSMCs. Double immunofluorescent staining demonstrated that the neoplastic SCs of HBs expressing MSC or pericyte markers directly abutted onto the ECs of capillaries/venules.
CONCLUSIONS
The results suggest that the neoplastic SCs of HBs share the immunophenotypic profile and distribution with those of pericyte-derived MSCs. Thus, HBs might originate from a distinctive population of pericyte-derived MSCs in the central nervous system.

Citations

Citations to this article as recorded by

Role of Endothelial-to-Mesenchymal Transition in the Pathogenesis of Central Nervous System Hemangioblastomas
Shigeki Takada, Masato Hojo, Noriyoshi Takebe, Kenji Tanigaki, Susumu Miyamoto
World Neurosurgery.2018; 117: e187. CrossRef
Endogenous Gastric-Resident Mesenchymal Stem Cells Contribute to Formation of Cancer Stroma and Progression of Gastric Cancer
Eun-Kyung Kim, Hye-Jung Kim, Young-Il Yang, Jong Tae Kim, Min-Young Choi, Chang Soo Choi, Kwang-Hee Kim, Jeong-Han Lee, Won-Hee Jang, Soon-Ho Cheong
Korean Journal of Pathology.2013; 47(6): 507. CrossRef

Multiple Hemangioblastomas in Cerebellum, Medulla Oblongata and Spinal Cord.: Won Ae Lee, Hye Jae Cho, III Hyang Ko, Sang Jin Kim; Korean J Pathol. 1995;29(3):403-406.

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Abstract PDF: Hemangioblastomas comprise 1 to 2% of all intracranial neoplasm, and 8 to 12% of tumors within the posterior fossa. They are composed of admixtures of three different cell types; endothelial cells, pericytes and stromal cells. Although most hemangioblastomas arise sporadically, they are associated with von Hippel-Lindau disease in about 20% cases. We have experienced a case of multiple hemangioblastomas occuiing in the cerebellum, medulla oblongata and cervical spinal cord simultaneously in a 55-year-old male. He had complained of headache, dizziness, generalized weakness and gait disturbance for 2 weeks. The patient had neither specific family history nor increased hematocrit. MRI showed a nonenhancing cystic lesion with an enhancing mural nodule in the right cerebellar hemisphere and two separate enhancing nodules in the medulla oblongata and dorsal cervical spinal cord at the 5-6th. Grossly, the excised mass of the cerebellum, 2.5 x 2 x 1.8cm, was solid to partly cystic, and that of spinal cord, lcm in diameter, was mostly solid. Microscopically, the tumor was composed of thin-walled blood vessels in variable size and interspersed stromal cells. The stromal cells revealed dimorphic cytoplasm that were either homogeneous and eosinophilic, or clear and vacuolated. Immunohistochemically, the endothelial cells reacted positively for glial fibrfllaty acidic protein(GFAP) and vimentin. The stromal cells reacted diffusely positively for vimentin, focally positively for GFAP and S-100 protein near the periphery of the tumor, focally positivel for neuro specipic enolase(NSE), and negatively for lysozyme, desmin and chromogranin. Ultrastructurally, the stromal cells contained numerous microfilaments and lipid droplets.

Nature of Stromal Cells in Cerebellar Capillary Hemangioblastoma: Immunohistochemical analysis.: Soon Won Hong, Tai Seung Kim, Ji Young Han; Korean J Pathol. 1995;29(5):584-589.

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Abstract: The origin of the stromal cell of cerebellar hemangioblastoma has long been studied electron microscopically and immunohistochemically. The results and theories about the stromal cell origin are variable and plentiful. However, the exact origin of the stromal cell remains controversial. The present study is aimed to elucidate the nature of the stromal cell of cerebellar hemangioblastoma. Ten cases of hemangioblastoma in Severance Hospital were used for immunohistochemical analysis of the stromal cell. The immunohistochemical staining of GFAP, S-100 protein, NSE, alpha-l-antichymottypsin, cytokeratin, CD 68, factor VIII related antigen, and synaptophysin were performed. The results were as follows; GFAP and S-100 protein were stained mainly but weakly in bellar capillary spindle cell and cellular process. NSE was stained mainly in foam cells, and 6 cases among them revealed strong reaction. ct-l-antichymotrypsin was stained in a few foam cells of 5 cases. Cytokeratin, CD 68, factor VIII related antigen, and synaptophysin showed negative reaction. Based on these results, it is considered that the origin of the stromal cell is histiocytic or neurogenic rather than glial. The weak positivity of GFAP and S-100 protein may support the neurogeriic origin but ct-l-antichymotrypsin positivity does not support the possibility. The positivity of GAP and S-100 protein supports the phagocytic action of histiocytic cell and suggests histiocytic origin rather than neurogenic.

Case Report

The Cytology of a Cellular Variant of Cerebellar Hemangioblastoma in Squash Preparation : Pitfalls in Diagnosis.: Young Lyun Oh, Yeon Lim Suh; Korean J Cytopathol. 2006;17(2):148-152.

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Abstract PDF: Due to its nuclear pleomorphism, knowledge regarding the cytological findings of cerebellar hemangioblastoma can lead to misdiagnosis when using squash specimens, which in other circumstances serves as a useful adjunct in the diagnosis of brain tumors on frozen section. We recently experienced the cytological findings of a cellular variant of cerebellar hemangioblastoma in a 51-year-old man. Squash specimens revealed scattered single tumor cells, with pleomorphic nuclei and cytoplasmic vacuoles, on a hemorrhagic background. The cellular clusters were composed of spindle-shaped endothelial cellsin addition to densely clustered stromal cells. Intranuclear inclusions were frequently seen. The nuclear pleomorphism, bubbly cytoplasmic vacuoles and presence of intranuclear inclusions, seen in the squash specimen, may increase the difficulty of frozen section diagnosis of cerebellar hemangioblastoma. Awareness of the cytologicalfindings of hemangioblastoma is needed to avoid the pitfalls in the intraoperative diagnosis of cerebellar hemangioblastomas.

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