title: Primary neuroendocrine carcinoma breast: our experience.
abstract: OBJECTIVE
  This retrospective study was designed to present the clinical characteristics and histopathological features of Primary neuroendocrine carcinoma (PNEC) of breast, and to evaluate the impact on outcome following its management on the line of more common primary adenocarcinoma of breast.
  
  
  METHODS
  Records of four patients diagnosed with PNEC of breast were retrospectively reviewed. Data were obtained from medical record from January 2008 to December 2012. Diagnosis of PNEC was confirmed by histopathological examination (HPE) and immunohistochemical (IHC) staining of tissue obtained from Trucut biopsy of the breast lump in all four patients. PNEC of breast was defined by the presence of more than 50% of invasive tumor cells with cytoplasmic immunoreaction for neuroendocrine (NE) markers synaptophysin, chromogranin or neuron specific enolase as per WHO classification. All patients were treated with Modified Radical Mastectomy (MRM), six cycle of Cyclophosphamide, Adriamycin and 5-Flurouracil (CAF) based adjuvant chemotherapy, radiotherapy and hormonal therapy.
  
  
  RESULTS
  There were four female patients. The mean age was 58~years (50-65 years). Breast lump was the presenting complaint in all patients. The result of HPE showed tumor size ranging from 4 to 6.5 cm in diameter. Axillary lymph node metastasis was detected in three (75%) patients. ER and PR expression was positive in four (100%) and three patients (75%) respectively. None of the patients expressed Her-2-neu. IHC staining was positive for NE markers chromogranin in three (75%) patients, synoptophysin in two patients (50%) and Neuron specific enolase three (75%) patients. The mean follow-up time was 27.7 months (range 48-9). All four patients survived without any loco-regional or metastatic recurrence with one patient developing lymphedema of arm.
  
  
  CONCLUSIONS
  Breast lump is the most common presentation of PNEC of the breast with characteristic expression of NE markers by the tumor. Management of this rare tumor may include surgery, chemotherapy, radiotherapy and hormonal therapy depending on the size of the tumor, lymph node and hormone receptor status. However, most appropriate treatment plan has yet to be established.
authors.affiliation: Department of General Surgery, Lady Hardinge Medical College and SSK Hospital, New Delhi, India.
authors.lastname: Suhani
authors.affiliation: Department of General Surgery, Lady Hardinge Medical College and SSK Hospital, New Delhi, India.
authors.forename: Shadan
authors.initials: S
authors.lastname: Ali
authors.affiliation: Department of General Surgery, Lady Hardinge Medical College and SSK Hospital, New Delhi, India.
authors.forename: Gunjan
authors.initials: G
authors.lastname: Desai
authors.affiliation: Department of General Surgery, Lady Hardinge Medical College and SSK Hospital, New Delhi, India.
authors.forename: Shaji
authors.initials: S
authors.lastname: Thomas
authors.affiliation: Department of General Surgery, Lady Hardinge Medical College and SSK Hospital, New Delhi, India.
authors.forename: Lalit
authors.initials: L
authors.lastname: Aggarwal
authors.affiliation: Department of General Surgery, Lady Hardinge Medical College and SSK Hospital, New Delhi, India.
authors.forename: Kusum
authors.initials: K
authors.lastname: Meena
authors.affiliation: Department of General Surgery, Lady Hardinge Medical College and SSK Hospital, New Delhi, India.
authors.forename: Jitendar
authors.initials: J
authors.lastname: Kumar
authors.affiliation: Department of Pathology, Lady Hardinge Medical College and SSK Hospital, New Delhi, India.
authors.forename: Manjula
authors.initials: M
authors.lastname: Jain
authors.affiliation: Department of General Surgery, Lady Hardinge Medical College and SSK Hospital, New Delhi, India.
authors.forename: S K
authors.initials: SK
authors.lastname: Tudu
mesh_terms: D000368:Aged; D001943:Breast Neoplasms / Q000737:chemistry / Q000473:pathology*; D018278:Carcinoma, Neuroendocrine / Q000737:chemistry / Q000473:pathology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local
keywords: Primary neuroendocrine carcinoma breast; breast lump; modified radical mastectomy