33 Special Types of Invasive Breast Carcinoma: Diagnostic Criteria ...

adults and, thus, the term “secretory” is preferable to “juvenile”. The microscopic
appearance of the lesion is the same regardless of patient age. No clinical hormonal
abnormality has been found to explain the secretory activity of the tumor and there is
no association with pregnancy.
Secretory carcinoma has an excellent prognosis. Although secretory carcinomas have
been reported to be more aggressive in adults, Rosen et al. (3) failed to find any
clinicopathologic difference with age, except for a greater delay in diagnosis in
younger patients. Axillary metastases occur, but they rarely involve more than three
lymph nodes (6-11); and, the risk of nodal involvement is as great in children as it is
in adults. Local excision is usually the initial treatment in children, and consideration
should be given to retaining the breast bud in prepubertal patients. In postmenarchal
children, wide local excision is adequate for small lesions, but quadrantectomy may be
needed for negative margins around larger tumors. Axillary dissection is indicated, if
clinical examination suggests nodal metastases. The value of radiation and/or
chemotherapy remain unclear (10,11). Clearly, secretory carcinoma should not be
confused with more common and more aggressive breast carcinomas such as
InvDC,NST with apocrine features. Familiarity with its histologic features should
allow its easy recognition.
Clinicopathologic features: In a review, Rosen et al. (3) found that 37% of patients
were less than 20 years old, 31% were in their twenties, and the remainders were over
30. The median age of 19 patients studied at the Armed Forces Institute of Pathology
(4) was 25 years (range 9 to 69 years); 14 were older than 20 years while one was a 9year-old
boy. Affected boys are usually under 10 years of age, although it was
reported in a 24-year-old man (10). Secretory carcinoma is uncommon in perimenarchal
girls 10 to 15 years of age. Secretory carcinomas are usually well
circumscribed, white or brown, and measure from 0.6 to 12 cm. The tumor is
characterized by large amounts of extra- and intracellular secretions, which are
strongly PAS and mucicarmine positive. The abundant secretion is usually pale pink
or amphophilic, often with a vacuolated or bubbly appearance. Tumor cells have
granular, clear, signet-ring, and/or vacuolated cytoplasm, sometimes with an apocrine
appearance. Indeed, secretory carcinomas are negative for gross cystic disease-fluid
protein-15 [GCDFP-15], an apocrine marker (3,4). But, Lamovec et al. (12) report that
two of four cases they studied were GCDFP-15 positive. All four cases were strongly
positive for S100 protein and alpha-lactalbumin; two of the four were negative for
CEA. These groups of tumors were diploid or near diploid and all had low S-phase
fractions by flow cytometry. A control group of 13 InvDC,NSTs, which showed
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