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
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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
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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 Article

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.

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