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Management of neuroendocrine carcinomas of the breast: A rare entity
Yesim Yildirim1, Sahende Elagoz, Ayhan Koyuncu
1Department of Medical Oncology, Acibadem University, Soyak Yenisehir Selale Evleri, 34770 Umraniye, Istanbul, Turkey.
Abstract:
Neuroendocrine (NE) carcinoma of the breast is extremely rare and constitutes less than 0.1% of all breast tumors. Only a few studies are currently available in the literature and a standard approach to treating this tumor has yet to be established. The aim of this study was to apply pathological treatment modalities in clinical practice and to select the most appropriate treatment accordingly. Six female patients were diagnosed with primary NE carcinoma of the breast. The patients underwent modified radical mastectomy with axillary dissection. Pathological specimens were stained with hematoxylin and eosin and an immunohistochemical panel of antibodies [neuron-specific enolase (NSE), chromogranin, synoptophysin, estrogen and progesterone receptor, c-erbB2 and Ki-67]. The results showed that tumor size ranged from 2 to 4.5 cm in diameter. Lymph node metastasis was detected in 4 (67%) patients. Estrogen and progesterone receptor expression was found in 5 (83%) patients. None of the patients expressed c-erbB2. Chromogranin was found to be positive in 5 (83%) patients. Synoptophysin expression was detected in 5 (83%) patients. NSE was stained in 4 (67%) patients. An intraductal component was found in 5 (83%) patients. Lymphovascular invasion was found in 5 (83%) patients. Adjuvant chemotherapy was administered to patients with a Ki-67 index of ≥10%. Radiotherapy was administered to 4 (67%) patients, and 4 (67%) patients received hormonal therapy. The mean follow-up time was 31.1 months (range 12-52). All 6 patients survived, although following chemotherapy and tamoxifen, the disease progressed in 1 patient who received second-line hormonal therapy. In conclusion, NE carcinoma of the breast is a distinct entity. Management of this rare tumor may include surgery and radiotherapy depending on the size of the tumor and lymph node status. However, the exact role of chemotherapy and hormonal therapy has yet to be established. Adjuvant chemotherapy is recommended for patients with a Ki-67 index of ≥10%, and hormonal treatment appears to be feasible in patients who are positive for estrogen and/or progesterone receptor.
Insights
Neuroendocrine breast carcinoma is rare, accounting for <0.1% of breast tumors. This study suggests surgery and radiotherapy are key, with chemotherapy and hormonal therapy potentially beneficial based on specific markers.
Area of Science:
- Oncology
- Pathology
Background:
- Neuroendocrine (NE) carcinoma of the breast is an extremely rare malignancy, representing less than 0.1% of all breast tumors.
- Limited research exists, and a standardized treatment protocol is not yet established for this rare breast cancer subtype.
Purpose of the Study:
- To investigate pathological treatment modalities for primary NE breast carcinoma in clinical practice.
- To determine the most appropriate treatment strategies based on observed patient outcomes and tumor characteristics.
Main Methods:
- Six female patients with primary NE breast carcinoma underwent modified radical mastectomy and axillary dissection.
- Immunohistochemical analysis was performed using neuron-specific enolase (NSE), chromogranin, synoptophysin, estrogen/progesterone receptors, c-erbB2, and Ki-67.
- Adjuvant chemotherapy, radiotherapy, and hormonal therapy were administered based on tumor characteristics and patient status.
Main Results:
- Tumor size ranged from 2-4.5 cm, with lymph node metastasis in 67% of patients.
- Estrogen/progesterone receptor and chromogranin positivity were observed in 83% of patients; synoptophysin was also positive in 83%.
- Lymphovascular invasion and an intraductal component were present in 83% of cases. Adjuvant chemotherapy was given for Ki-67 index ≥10%.
Conclusions:
- NE breast carcinoma is a distinct clinicopathological entity requiring tailored management.
- Surgery and radiotherapy are integral, with their use dependent on tumor size and lymph node involvement.
- The roles of chemotherapy and hormonal therapy require further investigation, though chemotherapy is recommended for Ki-67 ≥10% and hormonal therapy for receptor-positive cases.