Malignant acrospiroma explained

Malignant acrospiroma
Synonyms:Nodular hidradenocarcinoma, malignant nodular clear-cell hidradenoma, mucoepidermoid hidradenocarcinoma, clear-cell hidradenocarcinoma, malignant clear-cell acrospiroma, and clear-cell eccrine carcinoma.

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, is a rare and aggressive tumour of the eccrine sweat glands. It was first documented by Keasbey and Hadley in 1954.[1]

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.

The diagnosis of malignant acrospiroma is primarily based on histological, immunohistochemical, or ultrastructural characteristics. The differential diagnosis includes trichilemmal carcinoma, sebaceous carcinoma, clear cell changes in squamous cell carcinoma, basal cell carcinoma, balloon cell melanoma, porocarcinoma, and metastatic renal clear cell carcinoma.

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.[2] [3] Typically, they affect the head, neck, and limbs;[4] the breasts and chest are less frequently affected.[5] [6]

Causes

Malignant acrospiromas can develop spontaneously or evolve from a preexisting benign lesion.[4]

Diagnosis

The diagnosis of malignant acrospiroma is primarily based on histological, immunohistochemical, or ultrastructural characteristics.[7]

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.[7] The tumour may have enhanced mitotic activity and localized regions of necrosis.[8] [9]

The differential diagnosis for malignant acrospiroma includes additional cancers with clear cell changes, such as trichilemmal carcinoma, sebaceous carcinoma, clear cell changes in squamous cell carcinoma, basal cell carcinoma, balloon cell melanoma, porocarcinoma, and metastatic renal clear cell carcinoma.[7]

Treatment

The primary therapeutic method is wide surgical excision.[10] Because of the aggressive nature and proclivity for lymphatic invasion, several authors suggest prophylactic lymph node dissection.[11] Adjuvant chemotherapy's effectiveness is not well established.[12] [13]

Outlook

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.[2]

See also

Further reading

Notes and References

  1. Le . Keasbey . Clearcell hidradenoma; report of three cases with widespread metastases . Cancer . 1954 . 7 . 5 . 934–952 . 0008-543X . 13199772 . 10.1002/1097-0142(195409)7:5<934::aid-cncr2820070519>3.0.co;2-5 . 2024-04-08 .
  2. Kauderer . C . Clarke . HD . Fatone . CT . Malignant eccrine acrospiroma. A case study . Journal of the American Podiatric Medical Association . American Podiatric Medical Association . 85 . 2 . 1995 . 8750-7315 . 10.7547/87507315-85-2-116 . 116–117. 7877107 .
  3. Ogilvie . J W . Malignant eccrine acrospiroma. A case report . The Journal of Bone and Joint Surgery. American Volume . June 1982 . 64 . 5 . 780–782 . 6282887.
  4. Diab . Maria . Gabali . Ali . Kittaneh . Muaiad . Malignant acrospiroma: a case report in the era of next generation sequencing . BMC Cancer . Springer Science and Business Media LLC . 17 . 1 . 2017-03-27 . 1471-2407 . 10.1186/s12885-017-3217-5 . free . 221. 28347286 . 5368941 .
  5. Long . William P. . Dupin . Charles . Levine . Edward A. . Recurrent Malignant Acrospiroma: Treatment by Chest Wall Excision . Dermatologic Surgery . 24 . 8 . 1998 . 1076-0512 . 10.1111/j.1524-4725.1998.tb04273.x . 908–912. 9723060 .
  6. CYRLAK . DVORA . BARR . RONALD J. . WILE . ALAN G. . Malignant Eccrine Acrospiroma of the Breast . International Journal of Dermatology . Wiley . 34 . 4 . 1995 . 0011-9059 . 10.1111/j.1365-4362.1995.tb01595.x . 271–273. 7790144 .
  7. Pandey . Pinki . Dixit . Alok . Chandra . Subrat . Tanwar . Aparna . Cytological features of malignant eccrine acrospiroma presenting as a soft tissue mass axilla: A rare sweat gland tumor with histologic correlation . International Journal of Applied and Basic Medical Research . Medknow . 5 . 2 . 2015 . 145–148 . 2229-516X . 10.4103/2229-516x.157173 . free . 26097826 . 4456892 .
  8. Amel . Trabelsi . Olfa . Gharbi . Faten . Hammedi . Makrem . Hochlef . Slim . Ben Ahmed . Moncef . Mokni . Metastatic hidradenocarcinoma: Surgery and chemotherapy . North American Journal of Medical Sciences . Wolters Kluwer -- Medknow Publications . 1 . 7 . 2024-03-14 . 22666726 . 3364684 .
  9. Ko . Christine J. . Cochran . Alistair J. . Eng . William . Binder . Scott W. . Hidradenocarcinoma: a histological and immunohistochemical study* . Journal of Cutaneous Pathology . Wiley . 33 . 11 . 2006 . 0303-6987 . 10.1111/j.1600-0560.2006.00536.x . 726–730. 17083691 .
  10. Andreoli . Michael T. . Itani . Kamal M.F. . Malignant eccrine spiradenoma: a meta-analysis of reported cases . The American Journal of Surgery . Elsevier BV . 201 . 5 . 2011 . 0002-9610 . 10.1016/j.amjsurg.2010.04.015 . 695–699. 20851376 .
  11. EL-DOMEIRI . ALI A. . BRASFIELD . RICHARD D. . HUVOS . ANDREW G. . STRONG . ELLIOT W. . Sweat Gland Carcinoma . Annals of Surgery . Ovid Technologies (Wolters Kluwer Health) . 173 . 2 . 1971 . 0003-4932 . 10.1097/00000658-197102000-00014 . 270–274. 5100098 . 1397621 .
  12. KERSTING . DAVID W. . Clear Cell Hidradenoma and Hidradenocarcinoma . Archives of Dermatology . American Medical Association (AMA) . 87 . 3 . 1963-03-01 . 323–333 . 0003-987X . 10.1001/archderm.1963.01590150039007 . 14032110 .
  13. Lopez-Burbano . Luis F. . Cimorra . Gustavo A. . Gonzalez-Peirona . Elias . Alfaro . Jorge . Malignant Clear-Cell Hidradenoma . Plastic and Reconstructive Surgery . Ovid Technologies (Wolters Kluwer Health) . 80 . 2 . 1987 . 0032-1052 . 10.1097/00006534-198708000-00027 . 300–303. 3037576 .
  14. Ohta . Masayoshi . Hiramoto . Michiaki . Fujii . Miki . Togo . Takeshi . Nodular Hidradenocarcinoma on the Scalp of a Young Woman: Case Report and Review of Literature . Dermatologic Surgery . Ovid Technologies (Wolters Kluwer Health) . 30 . 9 . 2004 . 1076-0512 . 10.1111/j.1524-4725.2004.30390.x . 1265–1268. 15355375 .
  15. Mehregan . Amir H. . Eccrine Adenocarcinoma . Archives of Dermatology . American Medical Association (AMA) . 119 . 2 . 1983-02-01 . 0003-987X . 10.1001/archderm.1983.01650260012008 . 104.
  16. Görtler . I . Köppl . H . Stark . G.B . Horch . R.E . Metastatic malignant acrospiroma of the hand . European Journal of Surgical Oncology (EJSO) . Elsevier BV . 27 . 4 . 2001 . 0748-7983 . 10.1053/ejso.2000.1067 . 431–435. 11417993 .