BACKGROUND: Signet ring cell carcinoma of the eyelid is a rare variant of eccrine sweat gland carcinoma and has been reported previously in only five patients. METHODS: The authors report the clinical findings of a 55-year-old man with a signet ring cell carcinoma in the left eyelid as well as a clinical follow-up of 4.5 years. Several biopsies and the exenteration specimen were analyzed by routine light microscopy, electron microscopy, and comprehensive immunohistochemical stains on paraffin sections. RESULTS: Histologically, the tumor was shown to be a rare type of eccrine sweat gland carcinoma with signet ring cells and Indian file growth pattern reminiscent of invasive lobular carcinoma of the breast. Estrogen and progesterone receptors were identified immunohistochemically. On electron microscopy, intracytoplasmic pseudolumina with microvilli were positive for anti-human milk fat globulin and the lectin peanut agglutinin. Clinically, the tumor followed a malignant course with orbital invasion and lymph node metastases. CONCLUSIONS: Histologic recognition of this variant of eccrine sweat gland carcinoma is important because of its aggressive and malignant behavior and the wide range of differential diagnoses. Primarily, metastatic mammary carcinoma must be excluded. The treatment is primary excision with histologic control of the excision margins. In more advanced stages, radiation therapy, neck dissection, and anti-estrogen therapy should be considered.
BACKGROUND: Signet ring cell carcinoma of the eyelid is a rare variant of eccrine sweat gland carcinoma and has been reported previously in only five patients. METHODS: The authors report the clinical findings of a 55-year-old man with a signet ring cell carcinoma in the left eyelid as well as a clinical follow-up of 4.5 years. Several biopsies and the exenteration specimen were analyzed by routine light microscopy, electron microscopy, and comprehensive immunohistochemical stains on paraffin sections. RESULTS: Histologically, the tumor was shown to be a rare type of eccrine sweat gland carcinoma with signet ring cells and Indian file growth pattern reminiscent of invasive lobular carcinoma of the breast. Estrogen and progesterone receptors were identified immunohistochemically. On electron microscopy, intracytoplasmic pseudolumina with microvilli were positive for anti-human milk fat globulin and the lectin peanut agglutinin. Clinically, the tumor followed a malignant course with orbital invasion and lymph node metastases. CONCLUSIONS: Histologic recognition of this variant of eccrine sweat gland carcinoma is important because of its aggressive and malignant behavior and the wide range of differential diagnoses. Primarily, metastatic mammary carcinoma must be excluded. The treatment is primary excision with histologic control of the excision margins. In more advanced stages, radiation therapy, neck dissection, and anti-estrogen therapy should be considered.