Cerebellar hemangioblastoma case reports

A 50-year old patient with a history of right-sided cerebellar hemangioblastoma resection 10 years previously presented with a recurrent left sided palpable breast mass. She was referred for triple breast assessment and subsequent ultrasound-guided biopsy. On physical examination, the lesion was hypoechoic, ill-defined and located in the upper outer quadrant as are most breast malignancies. Ultrasound and mammography showed suspicious features. The ipsilateral axilla was normal. Histopathology revealed a diagnosis of breast angiomatosis with no evidence of associated malignancy. Vascular tumors of the breast are very rare, present diagnostic challenges and are prone to local recurrence. Complete excision with clear margins is recommended. Mastectomy is a consideration for diffuse disease that cannot be fully cleared with lumpectomy or Wide local excision. Cerebellar hemangioblastoma and breast angiomatosis is a very unique combination, particularly in the absence of an underlying phacomatosis. Radiological features of angiomatosis mimicking malignancy without pathognomonic imaging signs have been observed. Knowledge of these rare vascular breast tumors is key to making this unusual diagnosis and helps to reduce the number of radical surgical procedures 1).

A patient treated for sporadic cerebellar HB relapsed 12 years post-surgery. She developed disseminated disease throughout the CNS, including leptomeningeal manifestations. Repeat surgery and craniospinal radiation therapy were unsuccessful.

This case is in line with previous publications on disseminated non-VHL HB. Available treatment options are inefficient, emphasizing the need for improved understanding of HB biology to identify therapeutic targets 2)

A 10-year-old child who presented with a large hematoma in the left cerebellar hemisphere. Hemangioblastomas was not expected preoperatively to be the cause. An emergency suboccipital craniotomy was performed. Histopathological examination confirmed the diagnosis of hemangioblastoma with massive hemorrhage 3).

Wegner U, Balschat S, Decker T, Ryan AG. Rare Coexistence of a Cerebellar Hemangioblastoma and Angiomatosis of the Breast without Underlying Phakomatosis. J Clin Imaging Sci. 2019 Mar 28;9:8. doi: 10.25259/JCIS-9-8. eCollection 2019. PubMed PMID: 31448159; PubMed Central PMCID: PMC6702855.
Bains SJ, Niehusmann PF, Meling TR, Saxhaug C, Züchner M, Brandal P. Disseminated central nervous system hemangioblastoma in a patient with no clinical or genetic evidence of von Hippel-Lindau disease-a case report and literature review. Acta Neurochir (Wien). 2019 Feb;161(2):343-349. doi: 10.1007/s00701-019-03800-z. Epub 2019 Jan 17. Review. PubMed PMID: 30652202.
Wang Q, Cheng J, Zhang W, Ju Y. Spontaneous massive intracystic hemorrhage due to cystic hemangioblastoma in a pediatric patient. Br J Neurosurg. 2019 Jul 10:1-2. doi: 10.1080/02688697.2019.1639618. [Epub ahead of print] PubMed PMID: 31290349.
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