Secretory carcinoma was once used exclusively as a term for rare, slowly growing breast tumors without reference to their location in the breast. It is now termed mammary secretory carcinoma because secretory carcinoma has sometimes been used to name tumors which develop in non-breast tissues but have the microscopic appearance of, and a critical gene abnormality found in, mammary secretory carcinoma. This genetic abnormality is a balanced genetic translocation that forms a '' ETV6-NTRK3 fusion gene'' which appears involved in promoting the development and/or progression of all these tumors. The formerly termed secretory carcinomas include:

Mammary secretory carcinoma

Mammary secretory carcinoma, also termed secretory carcinoma of the breast, is a slow growing breast cancer that develops in female adults and, in a significant percentage of cases, males and children. While rare, it is the most common type of breast cancer that develops in children. (Mammary secretory carcinoma, termed secretory carcinoma at the time, was first described in 1966.)

Mammary analogue secretory carcinoma

Mammary analogue secretory carcinoma, also termed MASC_SG, is a slow growing

salivary gland neoplasm Salivary gland tumours, or neoplasms, are tumours that form in the tissues of salivary glands. The salivary glands are classified as major or minor. The major salivary glands consist of the parotid, submandibular gland, submandibular, and subling ...

(the SG in MASC_SG stands for salivary gland) that develops in adults and, in a significant number of cases, children. MASC_SG tumors are located most often in a

parotid salivary gland The parotid gland is a major salivary gland in many animals. In humans, the two parotid glands are present on either side of the mouth and in front of both ears. They are the largest of the salivary glands. Each parotid is wrapped around the ma ...

or infrequently a buccal mucosa salivary gland, submandibular salivary gland, or small salivary gland in the lower lip, upper lip, or

hard palate The hard palate is a thin horizontal bony plate made up of two bones of the facial skeleton, located in the roof of the mouth. The bones are the palatine process of the maxilla and the horizontal plate of palatine bone. The hard palate spans ...

. (MASC_SG was first described in 2010.)

Secretory carcinoma of the skin

As of 2021, 26 cases of secretory carcinoma of the skin had been reported. In a review of 25 cases, the skin tumors occurred in 16 females and 9 males aged 13 to 98 years (mean: 51.8 years). These tumors most common locations were the axilla (10 cases), neck (3), and lip (3 cases) with single cases occurring in other skin sites such as a cheek, eyelid, thigh, or skin at the base of a breast nipple. The tumors were treated primarily by surgical removal plus in some cases nearby

lymph node A lymph node, or lymph gland, is a kidney-shaped organ of the lymphatic system and the adaptive immune system. A large number of lymph nodes are linked throughout the body by the lymphatic vessels. They are major sites of lymphocytes that includ ...

radiotherapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a treatment using ionizing radiation, generally provided as part of cancer therapy to either kill or control the growth of malignant cells. It is normally delivered by a linear particle ...

. Although these patients need to be followed for longer periods, at the times of their last follow-up reports, all of these patients' tumors had taken indolent courses without recurring or metastasizing to distant tissues. However, in a recent report not included in the series of 25 cases, a 31-year-old female was diagnosed with an axillary secretory carcinoma of the skin and treated with surgical removal of the tumor and

to nearby lymph nodes; 4 years later, the patient developed metastasizes in both lungs. Most of these tumors consisted of cells that expressed the ''NTRK3-ETV6'' fusion gene. (Secretory carcinoma in the skin was first described in 2009.)

Salivary gland–type carcinoma of the thyroid

Salivary gland–type carcinoma of the thyroid, also termed intrathyroidal mammary analog secretory carcinoma, has been reported in 12 cases in the English language. In a review of 11 cases, salivary gland–type carcinomas of the thyroid were diagnosed in adult females (9 cases) and males (2 cases) with an average age 61.5 years (range: 36–74 years). These individuals presented with a thyroid mass with two individuals complaining of shortness of breath and one individual complaining of hoarseness. Their tumors' microscopic

histopathology Histopathology (compound of three Greek words: 'tissue', 'suffering', and '' -logia'' 'study of') is the microscopic examination of tissue in order to study the manifestations of disease. Specifically, in clinical medicine, histopatholog ...

resembled that described in the pathology section of mammary secretory carcinoma and consisted of tumor cells that expressed the ''NTRK3-ETV6'' fusion gene. All cases were treated by thyroidectomy (removal of the thyroid gland) plus

in 6 cases and/or

chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...

in 3 cases. Six of 10 individuals experienced local recurrences (n = 3) or one or more metastases (n = 3) after initial treatment; their tumors had invaded nearby normal thyroid tissues,

trachea The trachea (: tracheae or tracheas), also known as the windpipe, is a cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all animals' lungs. The trachea extends from ...

, mediastinum, and/or and cartilage and metastasized to the liver, lung, kidney, bone, and/or soft tissues. Salivary gland-type carcinoma of the thyroid appears to be a more aggressive disease than mammary secretory carcinoma or MASC_SG. (Salivary gland-type carcinoma of the thyroid was first described in 2015.)

Secretory carcinomas located in other sites

Tumors with the microscopic histopathology of secretory carcinomas and consisting of cells that express the ''NTRK3-ETV6'' fusion gene have been reported to occur in the nasal cavity of a 51 year old (tumor size: 1.5×1.5×0.4 cm) and 62 (tumor size: 4×4×1.5) year old woman, an

ethmoid sinus The ethmoid sinuses or ethmoid air cells of the ethmoid bone are one of the four paired paranasal sinuses. Unlike the other three pairs of paranasal sinuses which consist of one or two large cavities, the ethmoidal sinuses entail a number of small ...

of a 67-year-old female, lung of a 62-year-old female (8.5 cm in largest dimension), and

vulva In mammals, the vulva (: vulvas or vulvae) comprises mostly external, visible structures of the female sex organ, genitalia leading into the interior of the female reproductive tract. For humans, it includes the mons pubis, labia majora, lab ...

of a 51-year-old female.

References

{{reflist Breast cancer Salivary gland neoplasia Thyroid cancer Integumentary neoplasia Rare cancers Oncogenes