Malignant eccrine spiradenoma of the vulva: a case report and review of the literature

Abstract

Malignant eccrine spiradenoma is a rare skin tumor of sweat gland origin. We present the first reported case of this tumor in the female genitalia. Due to the rarity of this tumor, there has yet to be an established standard of care. The present case is that of a 41-year-old woman with malignant eccrine spiradenoma of the periclitoral region. She had an 18-month history of a recurrent, painful mass adjacent to the clitoris. Her diagnosis was made after excision of the cystic tumor. The patient then underwent a partial radical vulvectomy with bilateral sentinel lymph node sampling. As malignant eccrine spiradenoma is a rare tumor, no standard care exists for treatment and postoperative management. Based on our review of the literature, wide local excision appears to be the preferred initial treatment. Furthermore, adjuvant chemotherapy and/or radiation does not seem to improve survival in patients with advanced or recurrent cancer. Although lymph node sampling and/or lymphadenectomy is frequently reported in the treatment of this tumor, hematogenous metastasis can also occur. Therefore, these patients require close postoperative follow-up for recurrent disease.

A case of malignant eccrine spiradenoma metastatic to intramammary lymph node

“…Longstanding lesions rarely enlarge rapidly with ulceration or change of color to progress to a malignant eccrine spiradenoma (MES). MES is rare, with only 98 cases reported to date [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]. Here we describe a unique case of MES that metastasized to an intramammary lymph node (IMLN).…”

“…Two types of epithelial cells are present: cells with small and dark nuclei, and cells with large and pale nuclei [8]. Malignant transformation is very rare and has been reported in only 98 patients since the first report by Dabska in 1972 [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]. Multiple synonymous terms, including MES, eccrine spiradenocarcinoma, and sweat gland carcinoma ex-ES, have been used to designate these aggressive lesions [3].…”

Malignant eccrine spiradenoma: case report and review of the literature, including 15 Japanese cases

“…MES is rare, with only 68 cases reported in the English literature, including 2 Japanese cases, 1 Korean, 1 Taiwanese and 1 African American case 2–8 . In addition, 15 other cases have been reported in the Japanese literature (Table 1).…”

“…Several secondary therapies have been reported, 2 but their effectiveness is doubtful. There was a disappointing outcome for 6/10 patients (60%) who underwent radiation, 3/6 (50%) who underwent chemotherapy and the single patient who underwent immunotherapy.…”

Malignant eccrine spiradenoma: a new case report

“…Only 23 cases of MES have been reported in the California Cancer Registry since 1988. Furthermore, there are 62 cases including small case series of MES in the literature 3 . To further attest the rarity of this tumor, MES was not encountered in a large series of 60 sweat gland carcinomas recently reported by Urso et al 4 MES equally affects men and women.…”