Background. Sweat gland carcinomas with clear cell features are extrem
ely rare neoplasms, with few well documented cases reported in the lit
erature. Methods. Data on nine patients with malignant eccrine adnexal
neoplasms characterized by a prominent clear cell neoplastic componen
t were studied. Immunohistochemical stains with a panel of antibodies
against epithelial, stromal, and neural antigens were performed on fiv
e tumors and electron microscopic examination of one. Results. The tum
ors showed a spectrum of histologic features and growth patterns that
ranged from well differentiated, low grade malignant neoplasms to poor
ly differentiated, highly aggressive, recurrent, and metastasizing tum
ors. All tumors contained a varied proportion of cells with abundant c
lear cytoplasm, similar to those seen in a group of benign eccrine adn
exal neoplasms that have been variously designated as clear cell hidra
denoma, nodular hidradenoma, clear cell myoepithelioma, and eccrine ac
rospiroma. Immunohistochemical stains on five tumors and ultrastructur
al examination in one were consistent with eccrine differentiation. Cl
inical follow-up of eight patients showed local recurrence in six, fol
lowed by metastases in three, despite local excision, radiation, and c
hemotherapy. Criteria for differentiating these tumors from their beni
gn counterparts and from other types of malignant adnexal neoplasms an
d metastatic lesions are presented. Conclusions. The findings indicate
that clear cell eccrine carcinomas comprise a heterogeneous group of
lesions that may range from locally recurring, low grade well differen
tiated tumors to highly aggressive, high grade tumors with a definite
potential for uncontrollable local recurrence and metastasis. Wide sur
gical excision is recommended as the primary treatment for such neopla
sms.