A 68-year-old white man presented to the inpatient service at Columbia
-Presbyterian Hospital with a chief complaint of a progressively enlar
ging tumor of the right infraorbital region for many years and multipl
e, pink papulonodules on his head, neck, and trunk, A biopsy specimen
of the right infraorbital and back lesions demonstrated an infiltratin
g adenocarcinoma with prominent signet ring cells, and small lumen for
mation, Results of an extensive workup revealed bone marrow metastases
and no evidence of a primary malignancy. We present a case of primary
sweat gland carcinoma with metastases to the skin based upon history,
clinical presentation, results of physical examination, histopatholog
ic examination, immunohistochemical studies, and response to 5-fluorou
racil chemotherapy. Due to the rarity of the tumor, the diagnosis is u
sually not made until the tumor(s) is invasive. An excellent response
to systemic chemotherapy with 5-fluorouracil in metastatic sweat gland
carcinoma was noted in this case.