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¹Departments of Medical Oncology, İstanbul University Oncology Institute, İstanbul
²Departments of Radiation Oncology, İstanbul University Oncology Institute, İstanbul
³Departments of Nuclear Medicine, İstanbul University İstanbul Faculty of Medicine, İstanbul
⁴Departments of Pathology, İstanbul University İstanbul Faculty of Medicine, İstanbul Primary neuroendocrine carcinoma of the breast is very rare and has been revealed as a result of the widespread use of sensitive techniques for the detection of neuroendocrine markers. Published cases number less than 30, all presenting with a lump in the breast as the first sign. We report a case of neuroendocrine carcinoma of the breast that presented first with skin metastases. A 60-year-old woman with coin skin lesions, 2-3 cm, on the back, belly, and side of the hip was admitted to Istanbul University Oncology Institute. Histopathological results of a punch biopsy revealed lymphangitis carcinomatosa. Mammography and sonography performed as part of the systemic evaluation demonstrated a 6 mm solid lesion. Subsequent evaluation of the biopsy specimen revealed primary neuroendocrine carcinoma. First-line hormonotherapy with letrozole, to inhibit the estrogen receptor, together with monthly injections of long-acting 30 mg octreotide, for the skin lesions, were initiated. The patient was followed-up for 20 months, during which time no progression was observed. Then relaps was seen in the lungs and died six months later. Diagnosis, therapy, and follow-up of the case are presented. Keywords : Octreoscan; neuroendocrine carcinoma of breast