Primary Neuroendocrine Carcinoma of the Breast Metastatic to the Bones, Which Chemotherapy?

Abstract

The primary neuroendocrine carcinoma (NEC) of the breast is defined as immunohistochemical expression of neuroendocrine markers (chromogranin and synoptophysin) in more than 50% of the neoplastic cells according to World Health Organization (WHO) classification of tumors in 2003 (Tumours of the Breast and Female Genital Organs, 2003, Lyon: IARC Press). It accounts for less than 5% of all cancers arising from the breast (Tumours of the Breast and Female Genital Organs, 2003, Lyon, France: IARC Press). However, based on the study conducted by Wang et al., the primary NEC of breast comprises less than 0.1% of all mammary carcinomas (Frankf Z Pathol, 73, 1963, 24). Because of the rarity of the disease and absence of the prospective trials, there is no standard treatment for primary NEC of the breast. Herein, we report the case of a middle age woman with primary NEC with bone metastasis.

Primary neuroendocrine carcinoma of the breast with leptomeninges metastasis: A case report and literature review

“…A search of the literature published in PubMed V R from 1980 to 2021 using the terms ("neuroendocrine cancer of breast" or "small cell breast cancer" or "oat cell carcinoma of the breast") and ("advanced" or "metastatic") identified 12 case reports related to metastatic NEBC. [5][6][7][8][9][10][11][12][13][14][15][16] The following data were extracted from each article: first author name, year of publication, patient sex, age, tumour size, family history of breast cancer, status of ER, PR and HER2, Ki-67 index, metastatic sites, disease-free survival, overall survival and treatment options (Table 2). NECB is a rare breast cancer and the median age of onset is 63 years old.…”

“…Some researchers believe that NECB originates from the divergent differentiation of a neoplastic stem cell into epithelial and neuroendocrine cells, 26,27 while others think that NECB stems from neural crest cells that migrate to the mammary glands or it originates from neuroendocrine cells present in the breast tissue. 28 Neuroendocrine tumours are composed of densely cellular, solid nests and trabeculae of cells that differ from spindle to plasmacytoid and large clear cells separated by delicate fibrovascular stroma 16 and all undifferentiated NECB cells express neuroendocrine markers. [29][30][31] Neuron-specific enolase is recognized as the most common but not specific neuroendocrine indicator, while CgA and Syn are reliable neuroendocrine markers.…”

“…41 For advanced NECB, platinum-based regimens are the most common treatment choice, such as etoposide combined with cisplatin or carboplatin (EP/EC). 16 In the literature reviewed, EP and EC were the most frequently used formulas for first-line chemotherapy. 10,11,14,16 Moreover, when metastatic disease is diagnosed, the treatment strategy should be individualized depending on the general conditions and comorbidities of the patient, the extent and aggressiveness of the disease, and the biological features of the tumour.…”

“…16 In the literature reviewed, EP and EC were the most frequently used formulas for first-line chemotherapy. 10,11,14,16 Moreover, when metastatic disease is diagnosed, the treatment strategy should be individualized depending on the general conditions and comorbidities of the patient, the extent and aggressiveness of the disease, and the biological features of the tumour. 36 The current case was diagnosed with LM, which is a type of aggressive and malignant metastasis of NECB, with a reported overall survival of 4-6 weeks.…”

Head to head: do neuroendocrine tumours in the breast truly exist?

“…Numerous papers have been published through the years describing NE breast tumours (Table 1). 13,20,30,32–69 There is variation in how these tumours were defined and diagnosed, with some equating invasive breast carcinoma with NE differentiation as NEN/NET while others conform to the recently endorsed terminology for NEN, 9 with only rare reports of ‘carcinoids’. NECs form a significant proportion of these publications.…”