Purpose: Primary neuroendocrine carcinoma of the breast (NECB) is a rare distinct type of breast carcinoma. There are only some case reports on this topic published in the past. There is still little known on the optimal treatment outcomes, while a wide variety of treatments is proposed by several authors. In this study we searched the literature on NECB in PubMed to clarify its prognosis and possible optimal therapeutic strategies.
Methods: Eighty-six cases of primary NEC, included our case, were collected from PubMed between 1980 and 2013. Initial stage, estrogen receptor (ER)/progesterone receptor (PR)/ human epidermal growth factor receptor 2 (HER-2), surgical procedures, adjuvant treatment and overall survive (OS) were analyzed using the Statistical Package for the Social Sciences ( SPSS, v 16.0 ).
Results: All 86 patients enrolled were eligible. Their mean age at diagnosis was 53.9 years (range 25-83) and 1 case was in a male. Overall survival (OS) at 48 months was 83.5%. Patients with enlarged tumor size (10 patients with tumor size >5.0 cm) or advanced stage (stage III 15 patients, stage IV 2 patients) had poor OS (48-month OS: 51.4 vs 97.1% with tumors >5cm vs ≤2cm, respectively and 0.0%, 68.1%, 72.9% and 95.8% in stage IV, III, II and I, respectively). Patients with positive ER, PR or HER-2 had significantly better OS than did those without (ER, p<0.001; PR, p<0.001; HER-2, p=0.082). Besides, all 60 patients with initial primary surgery and without lymph node dissection (LND) showed better OS than those with initial primary surgery without LND, the difference however being not significant (p=0.133).
Conclusion: Definite diagnosis and clinical stage are prerequisites in the initial approach in NECB. When detected early the disease may have a good prognosis with combined modality treatment such as chemotherapy, surgery, and radiation therapy. An appropriate therapeutic strategy for this group is also important. Our analysis showed that for patients with early localized disease only primary surgery is recommended and LND is optional. In patients with positive steroid receptors postoperative hormonotherapy is suggested.