Porocarcinoma

Porocarcinoma
Other namesmalignant poroma, eccrine porocarcinoma, malignant eccrine poroma
SpecialtyDermatology, surgery, oncology
SymptomsSuperficial tumor of an eccrine sweat gland virtually anywhere on the skin
ComplicationsInvasion of nearby tissues, recurrence after surgical removal, metastases
CausesUnknown
PrognosisMetastatic porcarcinomas often have a poor prognosis

Porocarcinoma (PCA) (also termed malignant poroma, eccrine porocarcinoma, and malignant eccrine poroma)[1] is a rare form of skin cancer that develops in eccrine sweat glands, i.e. the body's widely distributed major type of sweat glands, as opposed to the apocrine sweat glands which are located primarily in the armpits and perineal area.[2] This cancer typically develops in individuals as a single cutaneous tumor in the intraepidermal spiral part (termed the acrosyringium) of these sweat glands' ducts (i.e. channels) at or near to where they open on the skin's surface.[3] PCA tumors are classified as one form of the cutaneous adnexal tumors;[4] in a study of 2,205 cases, PCA was the most common (11.8%) form of these tumors.[5]

Porocarcinomas are malignant counterparts to the far more common benign tumors of the eccrine sweat gland's acrosyringium, i.e. poromas. As currently viewed, there are 4 poroma variants based on their predominant cell types and extent of their tumor tissues presence in the epidermis and dermis: 1) Hidroacanthoma simplexe poromas are confined to the epidermis, i.e. uppermost layer of the skin. 2) Dermal duct poromas are confined to the dermis, i.e. layer of skin between the epidermis and subcutaneous tissues.[1] 3) Hidradenomas have recently been sub-classified into two groups; 95% are termed clear cell hidradenomas and have features suggesting that they derive from apocrine sweat glands while the remaining 5% are termed poroid hidradenomas and have features suggesting that they derive from eccrine sweat glands.[6] And 4) eccrine poromas are eccrine sweat gland tumors that consist of three cell types (see the histopathology section of Poromas) and are primarily located in the epidermis and superficial dermis. Poromas may have 2 or more of these variants in the same tumor tissue and the variants typically have histopathology findings that are not clearly distinguishable from each other.[7][8] PCA tumors may arise from one of these longstanding poromas[8] (in one study this occurred in 18% of cases[9]) but more commonly appear to develop independently of any precursor poroma.[1]

PCA are locally invasive tumors[1] that have been treated by surgical resection but often recur at the site of their surgical removal and metastasize to distant tissues before or after their removal.[1] Repeatedly recurrent, unresectable, and metastatic PCA have been treated with chemotherapy and/or radiotherapy.[10][11] However, metastatic PCA has responded poorly to these treatments and carries a poor prognosis.[12]

Because of their rarity and lack of distinct clinical features and variable physical and microscopic histological appearances, the diagnosis of porocarcinomas is often challenging.[1] PCA has commonly been either mis-diagnosed or over-diagnosed.[4][9] Under-diagnosis may have been responsible for a recent study conducted in a United Kingdom single center that reported that the number of PCA cases had increased 3-fold over the previous 4 years and was expected to rapidly rise further during the next decade.[9]

  1. ^ a b c d e f Sekine S, Kiyono T, Ryo E, Ogawa R, Wakai S, Ichikawa H, Suzuki K, Arai S, Tsuta K, Ishida M, Sasajima Y, Goshima N, Yamazaki N, Mori T (May 2019). "Recurrent YAP1-MAML2 and YAP1-NUTM1 fusions in poroma and porocarcinoma". The Journal of Clinical Investigation. 129 (9): 3827–3832. doi:10.1172/JCI126185. PMC 6715383. PMID 31145701.
  2. ^ Salih AM, Kakamad FH, Baba HO, Salih RQ, Hawbash MR, Mohammed SH, Othman S, Saeed YA, Habibullah IJ, Muhialdeen AS, Nawroly RO, Hammood ZD, Abdulkarim NH (August 2017). "Porocarcinoma; presentation and management, a meta-analysis of 453 cases". Annals of Medicine and Surgery (2012). 20: 74–79. doi:10.1016/j.amsu.2017.06.027. PMC 5499034. PMID 28721214.
  3. ^ Grieco M, Simonacci F, Grignaffini E, Ricci R, Raposio E (August 2020). "Eccrine porocarcinoma: case report and review of the literature". Giornale Italiano di Dermatologia e Venereologia. 155 (4): 500–504. doi:10.23736/S0392-0488.17.05182-3. PMID 33050682. S2CID 222353389.
  4. ^ a b Kim HJ, Kim A, Moon KC, Seo SH, Kim IH, Kim A, Baek YS (June 2020). "Eccrine Porocarcinoma: A Multicenter Retrospective Study with Review of the Literatures Reported in Korea". Annals of Dermatology. 32 (3): 223–229. doi:10.5021/ad.2020.32.3.223. PMC 7992626. PMID 33911741.
  5. ^ Battistella M, Balme B, Jullie ML, Zimmermann U, Carlotti A, Crinquette M, Frouin E, Macagno N, Ortonne N, Lamant L, de la Fouchardiere A, Aubriot-Lorton MH, Durand L, Josselin N, Franck F, Chatelain D, Lemasson G, Algros MP, Durlach A, Machet MC, Courville P, Osio A, Seris A, Mortier L, Jouary T, Cribier B (January 2022). "Impact of expert pathology review in skin adnexal carcinoma diagnosis: Analysis of 2573 patients from the French CARADERM network". European Journal of Cancer. 163: 211–221. doi:10.1016/j.ejca.2021.11.027. PMID 35090811. S2CID 246388005.
  6. ^ Lim JS, Kwon ES, Myung KB, Cheong SH (June 2021). "Poroid Hidradenoma: A Two-Case Report and Literature Review". Annals of Dermatology. 33 (3): 289–292. doi:10.5021/ad.2021.33.3.289. PMC 8137338. PMID 34079192.
  7. ^ Miller AC, Adjei S, Temiz LA, Gill P, Siller A, Tyring SK (January 2022). "Dermal Duct Tumor: A Diagnostic Dilemma". Dermatopathology. 9 (1): 36–47. doi:10.3390/dermatopathology9010007. PMC 8883970. PMID 35225875.
  8. ^ a b Agaimy A (May 2022). "Fusion-positive skin/adnexal carcinomas". Genes, Chromosomes & Cancer. 61 (5): 274–284. doi:10.1002/gcc.23031. PMID 35167714. S2CID 246864699.
  9. ^ a b c Koh M, Telang G, Fonseca A, Ghanian S, Walker J (September 2021). "Clear Cell Differentiation in Eccrine Porocarcinoma as a High-Risk Feature: Epidemiologic and Pathologic Features of Eccrine Porocarcinoma in a Single-Center Case Series". The American Journal of Dermatopathology. 43 (9): 647–652. doi:10.1097/DAD.0000000000001852. PMID 33867455. S2CID 233298389.
  10. ^ Yazar SK, Serin M (2019). "Results of Surgical Treatment of Patients with Malignant Eccrine Poroma". Sisli Etfal Hastanesi Tip Bulteni. 53 (1): 33–36. doi:10.14744/SEMB.2018.10170. PMC 7847727. PMID 33536823.
  11. ^ Khaja M, Ashraf U, Mehershahi S, Ayyadurai P, Malik S (February 2019). "Recurrent Metastatic Eccrine Porocarcinoma: A Case Report and Review of the Literature". The American Journal of Case Reports. 20: 179–183. doi:10.12659/AJCR.913440. PMC 6380207. PMID 30739904.
  12. ^ McGuire C, Fadel Z, Samargandi O, Williams J (2019). "Primary eccrine porocarcinoma of the thumb with multiple metastases: a case report and review of the literature". Case Reports in Plastic Surgery & Hand Surgery. 6 (1): 88–91. doi:10.1080/23320885.2019.1647108. PMC 6711107. PMID 31489340.