The neuroendocrine tumors (NETs) originate from the neuroendocrine (NE) cells that produce various types of amines and peptides. 1 NETs of the breast are rare slow-growing tumors and accounts for less than 1% of all NETs and less than 0.1% of all breast cancer. Focal NE differentiation can be associated with different histologic subtypes of breast carcinomas like in situ carcinoma and invasive ductal, lobular, colloid, or papillary carcinoma. 2 Sapino et al proposed the first diagnostic criteria in 2001 according to which the term Neuroendocrine Carcinoma of Breast (NECB) was entitled to those tumors expressing NE markers-Synaptophysin and/or Chromogranin in more than 50% of the tumor. 3 However, in the 2012 World Health Organization (WHO) classification, it was concluded that a 50% threshold for NE marker expression was arbitrary, hence it was removed. 4 Diagnostic requirements for primary NECB includes the histological presence of an in situ component and exclusion of any evidence of metastatic NEC. 2 According to WHO classification 2012, NECs were subdivided into three categories-well-differentiated NET, poorly differentiated NE carcinoma/small cell carcinoma, and invasive breast carcinoma with NE differentiation. 4 However, in a recently proposed consensus statement by the WHO and International Agency for Research on Cancer (IARC), the term "neuroendocrine neoplasm (NEN)" was adopted harmonizing with those of other organ systems to provide a uniform classification framework. NENs encompasses both welldifferentiated NETs corresponding to Grade 1 (carcinoid-like) and Grade 2 (atypical carcinoid-like) tumors (regarded as carcinomas in the breast) and poorly differentiated NECs, represented by small-cell and large-cell carcinoma. 5,6 A 78-year-old female presented to the Surgery Outpatient Department with the complaint of a left-sided breast lump for 2 months. On examination, a hard, tender, 3 × 3 cm size lump was palpated above the nipple and areola of the left breast. There was no history of nipple discharge or nipple retraction. No history of breast trauma or any other significant family history was elicited.
Primary sarcoma of the breast comprises less than 1% of all the breast malignancies. It is a locally aggressive neoplasm with only a few hundred cases published till date. The index case is being reported due to rarity of this entity. Breast sarcoma should always be considered whenever spindle cells are seen in the histological sections. The main diagnostic confusion arises with malignant phyllodes tumor and metaplastic carcinoma from which distinction is important from prognosis as well as treatment point of view. Herein, we report a case of a 47-yr-old female, who presented with a right-sided breast lump which was diagnosed as suspicious of carcinoma breast on fine needle aspiration (FNAC). Thereafter, modified radical mastectomy was performed, and on histopathology, a possibility of fibro-histiocytic tumor of intermediate malignancy was rendered, which was subsequently confirmed by immunohistochemistry (IHC).
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