Histiocytoid eccrine sweat gland carcinoma of the eyelid: report of a case

Abstract

This report describes the 12th documented case of histiocytoid, diffuse or signet-ring cell eccrine sweat gland carcinoma of the eyelid, a rare neoplasm that characteristically affects older men and mimics distant metastasis. Clinically, the patient was thought to have a colonic metastasis. Histologically, the neoplasm comprised sheets and cords of polygonal, eosinophilic cells, with dispersed larger, rounded single cells resembling histiocytes, and cells with cytoplasmic lumina or a signet-ring appearance. Occasional duct formation was also seen. The features were reminiscent of both the histiocytoid and lobular variants of breast carcinoma. Ultrastructurally, the presence of nonintestinal type microvilli with a length : width ratio in excess of 16 were consistent with an eccrine origin, but also raised the possibility of metastatic mesothelioma. However, a metastatic lesion was excluded by clinical and imaging investigations. There has been no recurrence 3 years after primary excision.

A Case of Primary Signet-Ring Cell/Histiocytoid Carcinoma of the Eyelid

“…The tumor cells show signet-ring cell or histiocytoid appearance and diffuse infiltration into the dermis and subcutis, without epidermal involvement. The tumor is considered to originate from sweat glands in the eyelid: [1][2][3][4][5] eccrine and apocrine sweat glands (Moll gland); however, there is much debate on whether the tumor expresses the phenotype of eccrine or apocrine sweat glands. The tumor mimics metastasizing signet ring cell adenocarcinoma, especially from the gastrointestinal tract, breast, and urinary bladder.…”

“…The tumor mimics metastasizing signet ring cell adenocarcinoma, especially from the gastrointestinal tract, breast, and urinary bladder. [1][2][3][4][5] Here, we report a patient with primary signet-ring cell/histiocytoid carcinoma of the eyelid who showed a phenotype of protein expression similar to that of Moll gland and investigate its histogenesis by immunohistochemical comparison of the tumor with normal apocrine and eccrine sweat glands of the perineal and axillary region.…”

Signet-Ring Cell/Histiocytoid Carcinoma of the Eyelid

“…Consistent ultrastructural findings for signet-ring cell/histiocytoid carcinoma are intracytoplasmic lumen and microvilli. 2,6,8,10,11,[14][15][16]19 The other ultrastructural findings have been frequently reported are desmosomes, 8,12,16 mucin granules, 2,12,14,15 tonofilaments, 2,8,12,19 abundant golgi complex and mitochondria, 2,8,19 and membrane-bound vacuoles. 8,10,15 Immunohistochemical studies that usually positive in signet-ring cell/histiocytoid carcinoma are pancytokeratin, 4,9,10,12,13,15,19 AE1/AE3, 4,9,16 EMA, 13,14,16,19 CK7,4,5,12,14,17,20 CAM5.2,4,9,16 34BE12,4,12,15 BerEP-4, 4,16 CEA, …”

“…21 However, because of its rarity, there are not many studies for these antibodies in signet-ring cell/histiocytoid carcinoma. 4,5,14 In the literature, only 2 reported cases of signet-ring cell/histiocytoid carcinoma show positivity to podoplanin 4 and 2 cases show negativity to p63. 5,14 Mammaglobin-positive cells in this case cannot distinguish primary skin tumor from metastatic breast cancer, 4 but metastasis from occult breast carcinoma is excluded by thorough clinical examination and negative immunostaining for ER.…”

“…4,5,14 In the literature, only 2 reported cases of signet-ring cell/histiocytoid carcinoma show positivity to podoplanin 4 and 2 cases show negativity to p63. 5,14 Mammaglobin-positive cells in this case cannot distinguish primary skin tumor from metastatic breast cancer, 4 but metastasis from occult breast carcinoma is excluded by thorough clinical examination and negative immunostaining for ER. In addition, strong membrane positivity for E-cadherin is not consistent with metastatic lobular carcinoma of the breast.…”