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  Table of Contents  
Year : 2015  |  Volume : 52  |  Issue : 1  |  Page : 85-86

Primary neuroendocrine carcinoma of breast: A rare tumor

1 Department of Pathology, Command Hospital (SC), Pune, India
2 Department of Surgery, AFMC, Pune, India
3 Department of Endocrinology, Command Hospital (SC), Pune, India

Date of Web Publication3-Feb-2016

Correspondence Address:
V Rana
Department of Pathology, Command Hospital (SC), Pune
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.175563

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How to cite this article:
Rana V, Kakkar S, Anand S, Singh Y. Primary neuroendocrine carcinoma of breast: A rare tumor. Indian J Cancer 2015;52:85-6

How to cite this URL:
Rana V, Kakkar S, Anand S, Singh Y. Primary neuroendocrine carcinoma of breast: A rare tumor. Indian J Cancer [serial online] 2015 [cited 2022 May 17];52:85-6. Available from:


A 65-year-old woman presented at our hospital with gradually enlarging lump in the right breast over last 6 months. On examination, a well-defined, firm mass of 6 ×

4 cm, with irregular surface was felt in the upper-outer quadrant of right breast. She had no palpable axillary lymphadenopathy. The trucut biopsy of the lump done outside our center was reported as invasive ductal carcinoma. Computed tomography scan of the lung, abdomen and bone scan was normal. The patient underwent a right Modified radical mastectomy

(MRM) at our center. On histopathologic examination, the infiltrating tumor revealed features of neuroendocrine differentiation in the form of nested solid islands of tumor cells separated by thin fibro vascular stroma [Figure 1]. The nuclei of tumor cells had stippled chromatin. Adjacent breast parenchyma had foci of Solid type of intraductal carcinoma [Figure 2]. Focal mucin production was also present. Modified Bloom Richardson score was 3 + 2 + 1 = 6; grade II. Axillary lymph nodes showed metastatic deposits with identical histomorphology in four of 20 nodes. The path stage was pT3N2. Immunohistochemistry (IHC) studies with antibodies directed against Neuron-specific enolase (NSE), chromogranin, synaptophysin, estrogen receptor (ER), progesterone receptor (PR) and Her2 neu were carried out. More than 90% of tumor cells in breast and lymph nodes were positive for NSE, Chromogranin, synaptophysin, and ER/PR [Figure 3]a,[Figure 3]b,[Figure 3]c,[Figure 3]d. Tumor cells were negative for Her2 neu. Diagnosis made was primary neuroendocrine carcinoma of breast (PNECB); solid type. PNECB is extremely rare and accounts for less than 5% of all cancers arising in the breast.[1] The diagnosis of PNECB is based on the WHO classification criteria which states that IHC expression of neuroendocrine markers must be demonstrated in more than 50% of the tumor cells.[1] This distinguishes PNECB from other breast carcinomas which may show only neuroendocrine morphological features or focal positivity on IHC. The diagnosis of primary tumor is made only if non-mammary sites are excluded or if an in situ component can be found in breast.[1]
Figure 1: Microphotograph showing tumor with solid architecture. Inset high power view (b) reveal stippled nuclear chromatin

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Figure 2: Microphotograph showing tumor with adjacent breast parenchyma having component of solid intraductal carcinoma

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Figure 3: IHC stains: (a) Estrogen receptor, (b) Neuron-.specific enolase, (c) Synaptophysin, (d) Chromogranin

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The biological behavior and the treatment of PNECB have not been well studied. In past most PNECB apart from small-cell undifferentiated type were considered low-grade malignancies and were associated with a favorable outcome.[2] In literature only five small series are reported using the current WHO criteria and none of them was case controlled, all had relatively small numbers of patients.[3] However, the first largest case-controlled study at the university of Texas M. D Anderson Cancer center has revealed that despite similar age and disease stages at presentation, NECB showed a more aggressive course than invasive ductal carcinoma, with a higher propensity for local and distant recurrence and poorer overall survival.[4] More statistically significant studies are required in this field. Endocrine therapy and radiation therapy has showed a trend toward improved survival. This letter aims to highlight that PNECB is a distinct tumor, but is under diagnosed and for that increased awareness is warranted amongst both pathologists and oncologists.

  References Top

Tavassoli FA, Devilee P, editors. Tumours of the breast. In: Pathology and Genetics of Tumors of the Breast and Female Genital Organs. WHO Classification of Tumours. Lyon, France: IARC Press; 2003. p. 32-4.  Back to cited text no. 1
Akhtar K, Zaheer S, Ahmad SS, Hassan MJ. Primary neuroendocrine carcinoma of the breast. Indian J Pathol Microbiol 2009;52:71-3.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
Ghanem S, Kabaj H, Naciri S, Glaoui M, Ismaili N, Benjaafar N, et al. Primary neuroendocrine carcinoma of the breast: A rare and distinct entity. J Cancer Res Exp Oncol 2011;3:50-4.  Back to cited text no. 3
Wei B, Ding T, Xing Y, Wei W, Tian Z, Tang F, et al. Invasive neuroendocrine carcinoma of the breast: A distinctive subtype of aggressive mammary carcinoma. Cancer 2010;116:4463-73.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3]

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