Malignant acrospiroma, also known as nodular hidradenocarcinoma, malignant nodular clear-cell hidradenoma, mucoepidermoid hidradenocarcinoma, clear-cell hidradenocarcinoma, malignant clear-cell acrospiroma, and clear-cell eccrine carcinoma,[1] is a rare and aggressive tumour of the eccrine sweat glands.[2] It was first documented by Keasbey and Hadley in 1954.[3]
Lesions manifest as slow growing 0.5 to 10 cm nodules that may drain or ulcerate. Malignant acrospiroma can develop de novo or from a pre-existing benign lesion.
Malignant acrospiroma is treated with wide surgical excision. It has a 50% chance of recurrence and 60% chance of metastasis in the first 2 years.
Malignant acrospiroma usually presents around the age of 50 and has no strong gender preference. Malignant acrospiromas account for around 6% of all malignant eccrine tumors.
Signs and symptoms
Usually, lesions appear as slow-growing nodules that have the potential to drain and ulcerate. Their sizes vary from 0.5 to 10 cm.[4][5] Typically, they affect the head, neck, and limbs;[2] the breasts and chest are less frequently affected.[6][7]
Causes
Malignant acrospiromas can develop spontaneously or evolve from a preexisting benign lesion.[2]
Diagnosis
The diagnosis of malignant acrospiroma is primarily based on histological, immunohistochemical, or ultrastructural characteristics.[1]
Histological traits include a pronounced nodular (lobular) pattern. Normally, there is no link between the epidermis and the tumour, but the superficial epithelium may become ulcerated.[1] The tumour may have enhanced mitotic activity and localized regions of necrosis.[8][9]
Within the first two years, the tumour has a 50% chance of local recurrence and a 60% chance of metastatic spread. Disease-free 5-year survival rates have been reported to be less than 30%.[14]
Epidemiology
Malignant acrospiromas account for around 6% of all malignant eccrine tumours.[15] The disease typically appears in middle ages.[16] There does not appear to be a clear gender preponderance, despite some reports suggesting that malignant acrospiromas are more common in women.[4]
^ abKauderer, C; Clarke, HD; Fatone, CT (1995). "Malignant eccrine acrospiroma. A case study". Journal of the American Podiatric Medical Association. 85 (2). American Podiatric Medical Association: 116–117. doi:10.7547/87507315-85-2-116. ISSN8750-7315. PMID7877107.
^Ogilvie, J W (June 1982). "Malignant eccrine acrospiroma. A case report". The Journal of Bone and Joint Surgery. American Volume. 64 (5): 780–782. PMID6282887.
^Ko, Christine J.; Cochran, Alistair J.; Eng, William; Binder, Scott W. (2006). "Hidradenocarcinoma: a histological and immunohistochemical study*". Journal of Cutaneous Pathology. 33 (11). Wiley: 726–730. doi:10.1111/j.1600-0560.2006.00536.x. ISSN0303-6987. PMID17083691.
^Andreoli, Michael T.; Itani, Kamal M.F. (2011). "Malignant eccrine spiradenoma: a meta-analysis of reported cases". The American Journal of Surgery. 201 (5). Elsevier BV: 695–699. doi:10.1016/j.amjsurg.2010.04.015. ISSN0002-9610. PMID20851376.
^Ohta, Masayoshi; Hiramoto, Michiaki; Fujii, Miki; Togo, Takeshi (2004). "Nodular Hidradenocarcinoma on the Scalp of a Young Woman: Case Report and Review of Literature". Dermatologic Surgery. 30 (9). Ovid Technologies (Wolters Kluwer Health): 1265–1268. doi:10.1111/j.1524-4725.2004.30390.x. ISSN1076-0512. PMID15355375.