Sweat gland carcinoma was first reported in 1865 but remains poorly understood due to its rarity, heterogeneity of subtypes, and confusing nomenclature.
A 72-year-old man presented with a 15-cm ulcerated, firm, pedunculated lesion of his left axilla that had recently doubled in size after 20 years of relative indolence (Fig. 1). He reported no systemic signs of illness, weight loss, or functional impairment. The results of his neurovascular examination were normal and he had palpable axillary lymph nodes. A computed tomography scan showed no chest wall involvement or pulmonary metastasis. The core needle biopsy demonstrated a malignant epithelial neoplasm.
The mass was excised with 1-cm margins, and axillary lymphadenectomy was performed, followed by a pedicled latissimus dorsi flap and skin graft reconstruction. Pathologic analysis confirmed “hidradenocarcinoma with preexisting hidradenoma” with negative margins. All 35 lymph nodes were negative for malignancy.
Sweat gland carcinomas are rare, accounting for 0.005 percent of all malignant epithelial neoplasms.1 Lesions are typically a violaceous nodule, papule, or plaque, although other colors have been reported. Most occur in the head and neck, followed by the extremities; only occasionally do they appear on the trunk.
Age at presentation ranges from 1 to 86 years, with most cases occurring in the fifth to seventh decades of life. These tumors overall occur equally in both sexes, although certain subtypes appear to have a predilection for males or females. Most sweat gland carcinomas grow slowly, with patients frequently having delayed presentation of 5 years or more. Rapid growth often occurs in a previously slow-growing lesion, possibly due to malignant degeneration of an originally benign tumor.
Lymphatic metastasis typically occurs at a rate of 20 to 24 percent. Regional cutaneous metastasis and hematogenous spread have also been noted.2 Local recurrence occurs in 14 to 20 percent of cases.2 Most patients survive beyond 10 years and usually die of another cause, although notable exceptions of death within 6 months of diagnosis due to metastasis (to lymph node and lungs) have been reported.3
Controversy remains as to the most appropriate classification system. Table 1 outlines a system reported by Cruz that most readily accommodates the variety of names used in the literature.4
Wide local excision and evaluation of lymph node status via sentinel lymph node biopsy or regional lymphadenectomy are recommended. Radiotherapy is reserved for recurrence or metastatic lymph node involvement.2 Chemotherapy does not currently have a role in management, although administration of trastuzumab has been reported in a metastatic tumor with Her-2/neu gene amplification.5
As is typical for sweat gland carcinomas and for hidradenocarcinoma (malignant acrospiroma) in particular, this tumor manifested rapid growth and ulceration after two decades as an indolent, small lesion. Management of these tumors should include wide excision and evaluation for lymph node and pulmonary metastasis. Adjuvant radiation therapy is likely appropriate for cases with lymph node metastasis or high-grade tumors.
The authors have no commercial associations or financial disclosures with regard to the article.
The authors thank Tiffany Sun, B.S., for her assistance in procurement of data for the article.
J. Alan Chambers, M.D., M.P.H.
Avron H. Lipschitz, M.D.
Daniel Petrisor, D.M.D., M.D.
Department of Surgery
C. Alejandra Garcia-Mitchell, M.D.
Department of Plastic Surgery
Shelly Stepenaskie-Wolz, M.D.
Department of Pathology
University of Texas Southwestern Medical Center
1. Wick W, Coffin M. Adnexal carcinomas of the skin: I. Eccrine carcinomas. Cancer
2. Hirsh L, Enterline H, Rosato E, et al. Sweat gland carcinoma. Ann Surg.
3. Hernandez-Perez E, Cestoni-Parducci R. Nodular hidradenoma and hidradenocarcinoma: A 10-year review. J Am Acad Dermatol.
4. Cruz DJ. Sweat gland carcinomas: A comprehensive review. Semin Diagn Pathol.
5. Nash JW, Barrett TL, Kies M, et al. Metastatic hidradenocarcinoma with demonstration of Her-2/neu gene amplification by fluorescence in situ hybridization: Potential treatment implications. J Cutan Pathol.
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