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Secretory carcinoma is a very rare, slow-growing breast cancer first described in children, whose cells make milk-like secretions. It is usually triple-negative but almost always carries the ETV6-NTRK3 gene fusion, so the rare patient whose tumour spreads can be treated with an NTRK inhibitor tablet, and most need no chemotherapy.
Secretory breast carcinoma is a rare subtype of infiltrating ductal-origin carcinoma that expresses the ETV6-NTRK3 gene fusion, first cloned in paediatric mesenchymal cancers; the fusion was confirmed in 12 of 13 cases (92 percent) but in no other ductal carcinomas, and its expression transformed mammary epithelial cells into gland-forming tumours in mice, establishing it as a primary oncogenic event (Tognon 2002). Histologically the tumour shows microcystic, solid and tubular patterns with abundant intracellular and extracellular secretion, is usually low grade and often triple-negative or weakly ER-positive with a basal-like immunophenotype (Cserni 2021; Thomas 2023). The European Working Group for Breast Screening Pathology places secretory carcinoma among the triple-negative special types with an indolent course whose patients are unlikely to benefit from systemic chemotherapy, favouring surgery with or without radiotherapy (Cserni 2021). Because the ETV6-NTRK3 fusion is an NTRK fusion, the tumour-agnostic approvals of larotrectinib and entrectinib apply to the rare metastatic case.
Very rare (under 0.15 percent of breast cancers in most series; the review literature counts it among the 5 to 10 percent of triple-negative cancers that are rare subtypes); it can occur in children and young adults as well as adults (Thomas 2023).
Most cancers start in the ducts and drain first to the axillary nodes, which is why the armpit is checked and a sentinel node is sampled.
Same organ: Triple-negative breast cancer (TNBC), Breast cancer (all types), HR-positive / HER2-negative breast cancer, HER2-positive breast cancer, Male breast cancer, Ductal carcinoma in situ (DCIS), High-risk early HR-positive breast cancer, HR-positive metastatic breast cancer after CDK4/6 inhibitors, HER2-low and HER2-ultralow metastatic breast cancer, Early HER2-positive breast cancer, HER2-positive breast cancer with brain metastases, Early triple-negative breast cancer, Metastatic triple-negative breast cancer, Basal-like 1 triple-negative breast cancer (BL1), Basal-like 2 triple-negative breast cancer (BL2), Mesenchymal triple-negative breast cancer (M), Mesenchymal stem-like triple-negative breast cancer (MSL), Luminal androgen receptor triple-negative breast cancer (LAR), Immunomodulatory triple-negative breast cancer (IM), Metaplastic breast carcinoma, Carcinoma with medullary pattern (medullary breast cancer), Adenoid cystic carcinoma of the breast, Apocrine carcinoma of the breast, BRCA-associated triple-negative breast cancer, Inflammatory breast cancer, Paget disease of the nipple, Phyllodes tumour of the breast, Invasive lobular carcinoma of the breast, Invasive breast carcinoma of no special type (invasive ductal carcinoma), Tubular carcinoma of the breast, Mucinous carcinoma of the breast, Papillary carcinomas of the breast (encapsulated, solid and invasive papillary), Invasive cribriform carcinoma of the breast, Invasive breast carcinoma with medullary pattern (medullary carcinoma), Invasive micropapillary carcinoma of the breast, Neuroendocrine neoplasms of the breast, Lobular carcinoma in situ (LCIS)
Surgery with clear margins, radiotherapy after breast conservation; chemotherapy is usually not indicated for this indolent type (European working group consensus).
NTRK inhibitors (larotrectinib, entrectinib) under their tumour-agnostic approvals for NTRK fusion-positive solid tumours.
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Query for this cancer: (TITLE:"Secretory carcinoma of the breast" OR ABSTRACT:"Secretory carcinoma of the breast" OR TITLE:"Juvenile carcinoma of the breast" OR ABSTRACT:"Juvenile carcinoma of the breast" OR TITLE:"Secretory breast carcinoma" OR ABSTRACT:"Secretory breast carcinoma" OR TITLE:"SBC" OR ABSTRACT:"SBC" OR TITLE:"ETV6-NTRK3 fusion-positive breast carcinoma" OR ABSTRACT:"ETV6-NTRK3 fusion-positive breast carcinoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Secretory carcinoma of the breast, not a curated reading list.
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Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Avoid grapefruit.
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
See all on the product pages:Entrectinib·Printable cards in the navigator
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