Breast Cancer Associated with Von Recklinghausen's Disease: Case Report and Review of Literature.

Breast cancer in association with type- 1 neurofibromatosis (NF1) is a rare clinical entity. Here, we present the case of a 45-year-old woman with typical clinical manifestations of NF1, who presented with a right breast lump, which she had first noticed 8 months back but had disregarded, believing it to be another manifestation of her Von Recklinghausen's disease. Clinical examination and diagnostic workup were suggestive of a breast carcinoma. Subsequently, she underwent modified radical mastectomy, followed by postoperative chemotherapy. Histopathological examination revealed high grade invasive duct carcinoma and multiple neurofibromas. After 7.5 months, she developed recurrence at the drain site with pulmonary and bony metastasis and died 9 months after surgery. Delayed presentation of the patient was the result of her mistaken identification for the breast tumor as a manifestation of NF-1. Early onset, high grade tumor and early recurrence with metastasis place strong emphasis on clinical alertness and to opt for an aggressive treatment option.