Case report of primary small cell neuroendocrine breast cancer
Abstract
Primary neuroendocrine cancer of the breast (NECB) is an extremely rare
tumor. In 2003, the World Health Organization (WHO) recognized this category with
three well-described subtypes: small cell, large cell, and carcinoid-like carcinoma;
very few peer-review publications based on the WHO definition were encountered
in the literature, and we conducted a literature search to investigate the reported
incidence, diagnosis, prognosis, hormone receptor status, and treatment options for
this rare tumor. Confirming the breast as an origin of neuroendocrine tumor repre-
sents a challenge. The diagnosis is mainly dependent on the exclusion of other extra-
mammary organs based on clinical, radiological, and pathological data.
Primary neuroendocrine carcinoma of the breast is rare - only about 30
cases have been reported in literature. Immunohistochemical examination showing
expression of chromogranin and/or synaptophysin confirms evidence of neuro-
endocrine differentiation. Usually foci of neuroendocrine differentiation can be seen
in breast carcinoma and are reported to be present in about 2-5% of breast cancer
cases. Here, we report a case of breast carcinoma in which most of the areas studied
on the tissue section showed neuroendocrine differentiation.
Primary neuroendocrine carcinoma of the breast is a group that exhibits
morphological features similar to those of neuroendocrine tumors of both the
gastrointestinal tract and the lung.
Case presentation
We report the case of a 50-year-old Caucasian woman with primary small
cell neuroendocrine cancer of the breast, which we characterized with immunohisto-
chemical techniques. A palpable and mobile 3.0 cm tumor was located in the upper-
outer quadrant of her right breast. After pathological confirmation the patient
underwent 8 cycles of chemotherapy, and subsequent radical mastectomy with
axillary lymph node resection were performed. Microscopically, the tumor consisted
predominantly of a diffuse proliferation of small oat cells. The tumor cells were posi-
tive for neuroendocrine markers; 21 of 30 lymph nodes were metastatic.
Local recurrence with multiple lung metastases developed only 5 weeks after
surgery, despite of the transient tumor regression achieved by chemotherapy. This case
reinforces the importance of an early correct diagnosis and the standardization of a
treatment regimen for this very rare tumor.
A correct treatment needs to be chosen.
tumor. In 2003, the World Health Organization (WHO) recognized this category with
three well-described subtypes: small cell, large cell, and carcinoid-like carcinoma;
very few peer-review publications based on the WHO definition were encountered
in the literature, and we conducted a literature search to investigate the reported
incidence, diagnosis, prognosis, hormone receptor status, and treatment options for
this rare tumor. Confirming the breast as an origin of neuroendocrine tumor repre-
sents a challenge. The diagnosis is mainly dependent on the exclusion of other extra-
mammary organs based on clinical, radiological, and pathological data.
Primary neuroendocrine carcinoma of the breast is rare - only about 30
cases have been reported in literature. Immunohistochemical examination showing
expression of chromogranin and/or synaptophysin confirms evidence of neuro-
endocrine differentiation. Usually foci of neuroendocrine differentiation can be seen
in breast carcinoma and are reported to be present in about 2-5% of breast cancer
cases. Here, we report a case of breast carcinoma in which most of the areas studied
on the tissue section showed neuroendocrine differentiation.
Primary neuroendocrine carcinoma of the breast is a group that exhibits
morphological features similar to those of neuroendocrine tumors of both the
gastrointestinal tract and the lung.
Case presentation
We report the case of a 50-year-old Caucasian woman with primary small
cell neuroendocrine cancer of the breast, which we characterized with immunohisto-
chemical techniques. A palpable and mobile 3.0 cm tumor was located in the upper-
outer quadrant of her right breast. After pathological confirmation the patient
underwent 8 cycles of chemotherapy, and subsequent radical mastectomy with
axillary lymph node resection were performed. Microscopically, the tumor consisted
predominantly of a diffuse proliferation of small oat cells. The tumor cells were posi-
tive for neuroendocrine markers; 21 of 30 lymph nodes were metastatic.
Local recurrence with multiple lung metastases developed only 5 weeks after
surgery, despite of the transient tumor regression achieved by chemotherapy. This case
reinforces the importance of an early correct diagnosis and the standardization of a
treatment regimen for this very rare tumor.
A correct treatment needs to be chosen.
Keywords
breast cancer, primary small cell neuroendocrine breast cancer, treatment, dissemination