Invasive cribriform carcinoma of the breast (ICCB), also termed invasive cribriform carcinoma, is a rare type of breast cancer that accounts for 0.3% to 0.6% of all carcinomas (i.e. cancers that develop from epithelial cells) in the breast. It originates in a lactiferous duct as opposed to the lobules that form the alveoli in the breasts' mammary glands (lobules make the milk which the ducts channel to the breast's nipple). ICCB was first described by Dixon and colleagues in 1983 as a tumor that on microscopic histopathological inspection had a cribriform pattern, i.e. a tissue pattern consisting of numerous "Swiss cheese"-like open spaces and/or sieve-like small holes (see adjacent Figure). The latest edition (2019) of the World Health Organization (2019) termed these lesions invasive cribriform carcinomas indicating that by definition they must have a component that invades out of their ducts of origin into adjacent tissues. In situ ductal cancers (i.e. cancers localized entirely within their tissues of origin) that have a cribriform histopathology are regarded as belonging to the group of ductal carcinoma in situ tumors. Rarely, cancers with the histopathological cribriform pattern develop in other organs such as the prostate gland (termed invasive cribriform prostate cancer or cribriform prostate cancer), salivary gland (termed cribriform adenocarcinoma of salivary glands), sweat glands (termed primary cutaneous cribriform apocrine carcinoma), thyroid gland (termed cribriform-morular thyroid carcinoma), colon (termed cribriform colon cancer), and lung (termed cribriform pattern in lung adenocarcinoma and considered to be a rare variant of acinar adenocrcinoma of the lung). Here, the term invasive cribriform carcinoma of the breast rather than invasive cribriform carcinoma is used in order to clearly distinguish it from these other cribriform carcinomas. ICCB, while clearly a cancerous tumor that occurs predominantly in older females and in rare cases males, has many favorable clinical and pathological features including a low rate of metastasizing to distant tissues and an excellent prognosis. Earlier editions of the World Health Organization divided ICCB into two forms, the "pure" form (sometimes termed "classical"), i.e. ICCB tumors that had >90% cribriform areas, and the "mixed" form, i.e. ICCB tumors that had >50% cribriform areas with the remaining areas having a tubular histological pattern. However, many earlier and more recent studies have regarded mixed ICCB as consisting of >50% cribriform areas with the remaining areas containing tubular or certain other histopathological patterns. The latter definition of mixed ICCB is used here.
Presentation Individuals presenting with ICCB in one large study were on average 61 years/old post-menopausal females and similarly aged males (average age 65.4) with the males representing 1.7% of all cases. However, individuals as young as 14 and 18 years have been diagnosed with the disease. ICCB tumors are small (i.e. 58%-75.4% less than 2-5 centimeters and 22%-38% more than 2-5 centimeters in largest diameters), asymptomatic masses first detected by palpation or screening methods such as mammography or medical ultrasound. Screening methods often (i.e. 32% of cases) detect areas of microcalcification (i.e. tiny deposits of calcium) in the masses. In a relatively small percentage of cases (e.g. 15.9 to 25.5% in most studies), the workup of these individuals finds that their tumor has metastasized (i.e. spread) to the lymph nodes in the nearby axilla (i.e. armpit). Very rarely, individuals have presented with: an ulcerated skin lesions or inverted nipple, with recurrences at or near to the site were a ICCB tumor had been surgically removed, or with metastases to distant tissues such as the vertebral bones.
Pathology
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