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Year : 2015  |  Volume : 52  |  Issue : 4  |  Page : 636-637

Primary neuroendocrine carcinoma of the breast: A rare and distinct entity

Department of General Surgery, Father Muller Medical College, Mangalore, Karnataka, India

Date of Web Publication10-Mar-2016

Correspondence Address:
K Ashwin Alva
Department of General Surgery, Father Muller Medical College, Mangalore, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.178432

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How to cite this article:
Alva K A, Tauro LF, Shetty P, Saldanha E. Primary neuroendocrine carcinoma of the breast: A rare and distinct entity. Indian J Cancer 2015;52:636-7

How to cite this URL:
Alva K A, Tauro LF, Shetty P, Saldanha E. Primary neuroendocrine carcinoma of the breast: A rare and distinct entity. Indian J Cancer [serial online] 2015 [cited 2020 Jul 7];52:636-7. Available from:


Neuroendocrine tumors are rare, slow growing tumors derived from the neuroendocrine cells that are omnipotent, most commonly arising from the bronchopulmonary and gastrointestinal tract. Neuroendocrine tumors of the breast are rare, accounting for less than 0.1% of all breast cancers and less than 1% of all neuroendocrine tumors.

[1] In a review of literature in 2005, Adegbola et al. have documented that less than 30 cases have been reported in literature.[2] They express synaptophysin and chromogranin tumor markers in more than 50% of the cell population.[3]

A 53 year old post-menopausal female patient gave history of lump in the left breast since 6 months, which was detected incidentally during routine physical examination by the treating physician, which has not progressed in size significantly since then. Patient did not give any history of nausea, vomiting, excessive sweating, palpitations, hot flushes, black outs etc. On examination, a hard non-tender mobile lump measuring 4 by 4 cm in size was felt in the upper inner quadrant of the left breast. The lump moving with the breast, not fixed to the skin or pectoral fascia. Clinically, no axillary nodes were palpable. Mammography showed a 5 by 4 cm lump with irregular borders and distorted architecture [Figure 1]. Fine needle aspiration of the lump showed features of atypical ductal hyperplasia and ductal carcinomainsitu with ?micro-invasion. Patient underwent left modified radical mastectomy.
Figure 1: Mammographic image showing the tumor

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Grossly, on serial section a pale-white solid infiltrating tumor measuring 5 by 4 by 3 cm seen almost involving deep resected margin and 0.2 cm away from the skin [Figure 2]. Ten lymph nodes were identified in the axillary specimen. Microscopy of the multiple sections studied revealed tumor cells in nests, small islands and alveolar and cribriform pattern [Figure 3]. Sections studied from all 10 axillary nodes were free of tumor. Immunohistochemistry confirmed positive staining for synaptophysin [w 4] and chromogranin neuroendocrine markers and negative for estrogen, progesterone and Her 2-neu receptors. Case was signed out as neuroendocrine tumor of the breast. Thorough clinical examination and investigations failed to reveal any primary tumor elsewhere in the body making us declare it as primary neuroendocrine carcinoma of the breast.
Figure 2: Gross specimen photograph

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Figure 3: Histological photograph of the neuroendocrine breast carcinoma

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Figure 4: Synaptophysin staining

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Patient is due for the first follow-up after discharge for adjuvant chemotherapy at the time of reporting.

In 1947, Volger indicated the existence of neuroendocrine cells in mammary tissue. Neuroendocrine tumor of the breast was not recognized as a separate entity until the last WHO classification. WHO defines them as tumors that exhibit morphological features similar to tumors of GI tract and lung and express tumor markers in more than 50% of the cell population. These tumors are usually seen in elderly women around the sixth or seventh decade of life and have no specific clinical or imaging features.[3] The histogenesis of these tumors are unclear, but they are thought to arise from endocrine differentiation of a breast carcinoma rather than from pre-existing endocrine cellsin the breast.[4]

This theoryis also supported by the findings in our case report. Cytology showed features of atypical ductal hyperplasia with ductal carcinomainsitu and biopsy confirmed it to be neuroendocrine carcinoma of the breast. Positive neuroendocrine markers are a must. We report this case in view of rarity of neuroendocrine carcinoma of the breast especially, in this part of the world and also to help in adding one more case to the coveted group of about 50 and odd cases reported so far worldwide and also reaffirming the theory of differentiation of breast cancer to a neuroendocrine lineage.

 » References Top

Ogawa H, Nishio A, Satake H, Naganawa S, Imai T, Sawaki M, et al. Neuroendocrine tumor in the breast. Radiat Med 2008;26:28-32.  Back to cited text no. 1
Adegbola T, Connolly CE, Mortimer G. Small cell neuroendocrine carcinoma of the breast: A report of three cases and review of the literature. J ClinPathol 2005;58:775-8.  Back to cited text no. 2
Ellis IO, Schnitt SJ, Sastre-Garau X. Tumors of the breast, neuroendocrine tumors. World Health Organisation Classification of Tumours. Pathology and Genetics of the Tumours of the Breast and Female Genital Organs. Lyon, (France): International Agency for Research on Cancer (IARC); 2003. p. 13-59.  Back to cited text no. 3
Ajisaka H, Maeda K, Miwa A, Yamamoto K. Breast cancer with endocrine differentiation: Report of two cases showing different histologic patterns. Surg Today 2003;33:909-12.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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