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A rare case of peripheral nerve hemangioblastoma—case report and literature review

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Abstract

Hemangioblastoma is a rare benign tumor that consists of neoplastic vacuolated stromal cells and highly developed capillary blood vessels. These tumors are almost exclusively found in the central nervous system, but in extremely rare cases, it can arise in peripheral nerves. In the majority of cases, it occurs sporadically (60 to 75 %), but in about 25 % of cases, it is associated with von Hippel-Lindau disease. We present the first case of ulnar nerve hemangioblastoma in a 70-year-old male. The patient presented with a slow-growing palpable mass on the front side of the right upper arm. Macroscopically, the tumor was round shaped, encapsulated, reddish-orange in color, very well vascularized, and about 3 cm in diameter; one nerve fascicle was entering the tumor tissue, so it was resected with the tumor. The rest of the nerve fascicles were intact. Postoperative course was uneventful. Histopathological analysis with immunohistochemical analysis confirmed that the tumor was a peripheral nerve hemangioblastoma WHO grade I. Physical treatment was conducted, and there was no motor neurological deficit on follow-up after 3 months, only hypoesthesia of the fourth and fifth finger. These lesions are so rarely found arising from peripheral nerves that only four published cases exist in literature today. There is very little data about these tumors in world literature, so we consider our paper a valuable contribution to global knowledge on these tumors.

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Correspondence to Lukas Rasulic.

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Comments

Stefano Ferraresi, Rovigo, Italy

“Going on eating oysters one finally will find a pearl”: this paper is the result of the total commitment to the cause of peripheral nerves by Lukas Rasulic and his team. The paper is very interesting and casts a light on a very rare entity that, for instance, in spite of our great experience on peripheral nerve tumors, we never encountered. Also, from the review of the literature, one cannot determine with certainty if a hemangioblastoma of a peripheral nerve can be removed, saving the function of the main trunk, as it commonly happens for schwannomas. This obviously raises a question whether to operate or not on these patients, since the preservation of nerve function must be the primary goal in a PNS benign tumor. The last two cases (the one by D. Kim and this by L. Rasulic) seem to confirm the feasibility of a neat surgery in this rare entity.

For this reason, we thank the authors in the name of the scientific community for this nice report.

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Rasulic, L., Samardzic, M., Bascarevic, V. et al. A rare case of peripheral nerve hemangioblastoma—case report and literature review. Neurosurg Rev 38, 205–209 (2015). https://doi.org/10.1007/s10143-014-0587-x

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  • DOI: https://doi.org/10.1007/s10143-014-0587-x

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