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Presented by Dr. Andres Matoso and prepared by Dr. Harsimar Kaur
Adult male with inguinal lymphadenopathy.
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1. Question
Adult male with inguinal lymphadenopathy.
Correct
Correct: B.
Histology: This lymph node is almost entirely replaced by a tumor composed of nests and sheets of cells with eosinophilic cytoplasm and large nuclei with prominent nucleoli. There are occasional glandular lumina and intracytoplasmic mucin vacuoles.
Discussion: The skin adnexal structures have a similar histogenesis to mammary glands. Accordingly, neoplasms arising from adnexal sweat glands and mammary glands look very similar histologically, as well as immunophenotypically. Apocrine sweat glands are concentrated in areas such as the axilla, eyelid, anogenital regions, and ear canal. Apocrine carcinoma is a rare form of malignancy that originates in sweat apocrine glands and, therefore, it is more common in regions with high density of such glands including the axilla and anogenital area. The definition of apocrine carcinoma varies in the literature. Some experts would require morphologic evidence of apocrine differentiation including tumor cells with granular eosinophilic cytoplasm and evidence of decapitation, while others would accept tumors with a wider range of morphologic features but with expression of GCDFP15 and occurring in the axillary or anogenital regions that are known to harbor apocrine glands. A lesion that is more common in men and shows the same immunophenotype is extramammary Paget disease (EMPD). Histologically, extramammary Paget disease is predominantly an intraepithelial (in-situ) lesion with superficial invasion in a minority of the cases. Clinically, patients with EMPD present with an insidious rash or erythema that is suspected to be a fungal infection or a dermatitis. In contrast, patients with apocrine carcinoma present with a nodule or mass. Lymph node metastases is rare in EMPD, and most patients have a favorable prognosis, with the main clinical challenge being to obtain negative margins to avoid local recurrence. Most patients with apocrine carcinoma of the scrotum present with metastatic disease. Similar to EMPD and breast carcinomas, apocrine carcinomas are positive for GATA3, GCDFP15, CK7 and mammaglobin. AR could also be positive, and tumors can occasionally show positive staining for NKX3.1 and PSA, as also seen in EMPD.
References:
Shabihkhani M et al. Appl Immunohistochem Mol Morphol. 2020 Aug;28(7):524-531.
Kamanda S et al. Am J Dermatopathol. 2022 Apr 1;44(4):260-266.Incorrect
Correct: B.
Histology: This lymph node is almost entirely replaced by a tumor composed of nests and sheets of cells with eosinophilic cytoplasm and large nuclei with prominent nucleoli. There are occasional glandular lumina and intracytoplasmic mucin vacuoles.
Discussion: The skin adnexal structures have a similar histogenesis to mammary glands. Accordingly, neoplasms arising from adnexal sweat glands and mammary glands look very similar histologically, as well as immunophenotypically. Apocrine sweat glands are concentrated in areas such as the axilla, eyelid, anogenital regions, and ear canal. Apocrine carcinoma is a rare form of malignancy that originates in sweat apocrine glands and, therefore, it is more common in regions with high density of such glands including the axilla and anogenital area. The definition of apocrine carcinoma varies in the literature. Some experts would require morphologic evidence of apocrine differentiation including tumor cells with granular eosinophilic cytoplasm and evidence of decapitation, while others would accept tumors with a wider range of morphologic features but with expression of GCDFP15 and occurring in the axillary or anogenital regions that are known to harbor apocrine glands. A lesion that is more common in men and shows the same immunophenotype is extramammary Paget disease (EMPD). Histologically, extramammary Paget disease is predominantly an intraepithelial (in-situ) lesion with superficial invasion in a minority of the cases. Clinically, patients with EMPD present with an insidious rash or erythema that is suspected to be a fungal infection or a dermatitis. In contrast, patients with apocrine carcinoma present with a nodule or mass. Lymph node metastases is rare in EMPD, and most patients have a favorable prognosis, with the main clinical challenge being to obtain negative margins to avoid local recurrence. Most patients with apocrine carcinoma of the scrotum present with metastatic disease. Similar to EMPD and breast carcinomas, apocrine carcinomas are positive for GATA3, GCDFP15, CK7 and mammaglobin. AR could also be positive, and tumors can occasionally show positive staining for NKX3.1 and PSA, as also seen in EMPD.
References:
Shabihkhani M et al. Appl Immunohistochem Mol Morphol. 2020 Aug;28(7):524-531.
Kamanda S et al. Am J Dermatopathol. 2022 Apr 1;44(4):260-266.
