|LETTER TO EDITOR
|Year : 2009 | Volume
| Issue : 4 | Page : 352-354
Primary neuroendocrine carcinoma of the breast, which chemotherapy?
LK El Hassani1, Y Bensouda1, H M'Rabti1, S Boutayeb1, M Kamouni2, L Gamra2, H Errihani1
1 Department of Medical Oncology, National Institute of Oncology, Rabat, Morocco
2 Department of Pathology, National Institute of Oncology, Rabat, Morocco
|Date of Web Publication||9-Sep-2009|
L K El Hassani
Department of Medical Oncology, National Institute of Oncology, Rabat
|How to cite this article:|
El Hassani L K, Bensouda Y, M'Rabti H, Boutayeb S, Kamouni M, Gamra L, Errihani H. Primary neuroendocrine carcinoma of the breast, which chemotherapy?. Indian J Cancer 2009;46:352-4
|How to cite this URL:|
El Hassani L K, Bensouda Y, M'Rabti H, Boutayeb S, Kamouni M, Gamra L, Errihani H. Primary neuroendocrine carcinoma of the breast, which chemotherapy?. Indian J Cancer [serial online] 2009 [cited 2013 Jun 20];46:352-4. Available from: http://www.indianjcancer.com/text.asp?2009/46/4/352/55566
Primary neuroendocrine carcinomas of the breast are extremely uncommon, with only very few cases published in English literature. Their best treatment is still unknown and various modes of management have been employed in treating this disease.
A 40-year-old woman presented with a lump in her right breast with pain and rapidly progressing tender, firm, enlarged breast [Figure 1]. Mammography revealed an irregular bulky mass of 10 cm. A biopsy was performed, and neuroendocrine carcinoma was diagnosed by histological examination. Immunohistochemistry was positive for neuron specific enolase [Figure 2], synaptophysine and chromogranine. While the progesterone receptor was positive in more than 50%, the oestrogen receptor was negative. The radiological investigations (Computed tomography (CT) scan of the chest, abdominal CT scan, brain CT scan, and bone scintigraphy) were negative for both metastatic and other primary diseases. The evaluation after three cycles of FAC (5 fluorouracil, adriamycin, cyclophospahmide) showed progression [Figure 3] with the development of cerebral metastasis. She died after one cycle of VP16-CDDP.
Neuroendocrine tumors of the breast are a rare malignancy in general, their prevalence is about 0.5% in a series of 1368 histopathologically proven breast cancers. 
Their clinical and imaging features mimic those of breast carcinomas in many ways without any specificity.  Our patient had an irregular lump of 10/15 cm, with erythematous and oedematous skin, and its mammography revealed an irregular bulky mass.
To diagnose a primary neuroendocrine carcinoma of the breast, a nonmammary site has to be excluded clinically and / or an in situ component should be demonstrated histologically. The imaging procedures required to exclude other primary tumors or distant metastases are: chest, abdominal and pelvic CT scan, bone scan, and positron emission tomography (PET) scanning.  All these radiological investigations were carried out in our case except PET scanning.
Most patients are treated as for adenocarcinoma of the breast.  The majority of information that guides management consists of retrospective chart reviews, anecdotal experience, and case reports. However, it seems acceptable that the initial management of patients with localized neuroendocrine tumor disease should be similar to the treatment of the other tumor types arising in the same site, with the exception of pulmonary neuroendocrine tumors. 
Regarding systemic management, there is no standard treatment protocol and a large variety of chemotherapy protocols have been employed in treating this disease. Systemic therapy principles have been derived from small retrospective case reviews of primary neuroendocrine breast carcinomas and extrapolated from studies of nonbreast neuroendocrine carcinomas, since the clinical behavior and histology are similar.
All data suggest that chemotherapy should be similar to their pulmonary counterpart and the most used chemotherapeutic regimen is VP16-CDDP.  Our patient was treated as for adenocarcinoma of the breast; she received FAC as neoadjuvant chemotherapy. Anthracyclines were ineffective in our case, and VP16-CDDP was probably the most appropriate regimen in this situation.
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[Figure 1], [Figure 2], [Figure 3]
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