Rare Coexistence of a Cerebellar Hemangioblastoma and Angiomatosis of the Breast without Underlying Phakomatosis

摘要

Angiomatosis of the breast is an unusual benign vascular process which may affect middle aged women and simulate carcinoma. We report a unique case of a female patient with cerebellar hemangioblastoma and coexisting breast angiomatosis. We discuss the neuroradiology and breast imaging, illustrating the diagnostic pearls and pitfalls in the setting of this extremely uncommon combination. 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.