title: Locally-advanced primary neuroendocrine carcinoma of the breast: case report and review of the literature.
abstract: BACKGROUND
  Primary neuroendocrine carcinoma of the breast is a heterogeneous group of rare tumors with positive immunoreactivity to neuroendocrine markers in at least 50% of cells. Diagnosis also requires that other primary sites be ruled out and that the same tumor show histological evidence of a breast in situ component. Primary neuroendocrine carcinoma of the breast rarely presents as locally advanced disease and less frequently with such widespread metastatic disease as described herein. The review accompanying this case report is the first to provide an overview of all the cases of primary neuroendocrine carcinoma of the breast published in the literature and encompasses detailed information regarding epidemiology, histogenesis, clinical and histologic diagnosis criteria, classification, surgical and adjuvant treatment, as well as prognosis. We also provide recommendations for common clinical and histologic pitfalls associated with this tumor.
  
  
  METHODS
  We describe a case of a 51-year-old Hispanic woman initially diagnosed with locally-advanced invasive ductal carcinoma that did not respond to neoadjuvant treatment. After undergoing modified radical mastectomy the final surgical pathology showed evidence of alveolar-type primary neuroendocrine carcinoma of the breast. The patient was treated with cisplatin/etoposide followed by paclitaxel/carboplatinum. Thirteen months after surgery the patient is alive, but developed pulmonary, bone, and hepatic metastasis.
  
  
  CONCLUSIONS
  The breast in situ component of primary neuroendocrine carcinoma of the breast may prevail on a core biopsy samples increasing the probability of underdiagnosing this tumor preoperatively. Being aware of the existence of this disease allows for timely diagnosis and management. Optimal treatment requires simultaneous consideration of both the neuroendocrine and breast in situ tumor features.
authors.affiliation: Department of Surgery, Hospital Universitario San Ignacio, Pontificia Universidad Javeriana, Bogotá, Colombia.
authors.forename: Fernando A
authors.initials: FA
authors.lastname: Angarita
authors.forename: Jorge L
authors.initials: JL
authors.lastname: Rodríguez
authors.forename: Eugenio
authors.initials: E
authors.lastname: Meek
authors.forename: Jesus O
authors.initials: JO
authors.lastname: Sánchez
authors.forename: Mauricio
authors.initials: M
authors.lastname: Tawil
authors.forename: Lilian
authors.initials: L
authors.lastname: Torregrosa
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000556:secondary / Q000628:therapy; D001943:Breast Neoplasms / Q000175:diagnosis* / Q000628:therapy; D016190:Carboplatin / Q000008:administration & dosage; D018270:Carcinoma, Ductal, Breast / Q000175:diagnosis* / Q000628:therapy; D018278:Carcinoma, Neuroendocrine / Q000175:diagnosis* / Q000628:therapy; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary / Q000628:therapy; D008175:Lung Neoplasms / Q000556:secondary / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008408:Mastectomy; D008875:Middle Aged; D020360:Neoadjuvant Therapy; D009367:Neoplasm Staging; D017239:Paclitaxel / Q000008:administration & dosage; D011379:Prognosis; D012196:Review Literature as Topic
chemical_list: D005047:Etoposide; D016190:Carboplatin; D017239:Paclitaxel; D002945:Cisplatin