Siringofibroadenom
Özet
Siringofibroadenom (Ekrin siringofibroadenom; ESFA), duktal diferansiyasyon gösteren epitelyal hücrelerin fibrovasküler bir stroma içerisinde anastomozlaşan kordonlar halinde proliferasyonu ile karakterize, genellikle benign ve soliter seyreden nadir bir deri eki tümörüdür. Literatürde neoplazm, varyant veya reaktif bir süreç olup olmadığına dair tartışmalar devam etmekte ve klinik prezentasyonlarına göre Starink tarafından 4, Weedon tarafından ise 5 farklı alt tipe ayrılmaktadır. Sıklıkla ileri yaştaki bireylerin ekstremitelerinde büyük hiperkeratotik plaklar olarak ortaya çıkan bu lezyonlar, nadiren Schöpf sendromu gibi kalıtsal hastalıklarla veya inflamatuar kutanöz bozukluklarla ilişkili reaktif süreçler olarak da görülebilir. Mikroskopik incelemede epidermise bağlı kafes benzeri anastomozlaşan ince epitel kordonları, bol musin ve yoğun lenfoplazmositer infiltrat içeren gevşek bir stroma dikkati çeker. İmmünhistokimyasal olarak duktal ve akrosiringeal farklılaşmayı destekleyen EMA, CEA, CK19 ve filagrin ekspresyonu izlenir. Ayırıcı tanısında poroma, siringofibrokarsinom ve fibroepitelyal bazal hücreli karsinom yer alan bu iyi huylu antitelerin tedavisinde kriyoterapi, radyoterapi ve lazer gibi yöntemler başarıyla uygulanmaktadır.
Syringofibroadenoma (Eccrine syringofibroadenoma; ESFA) is a rare, benign, and usually solitary skin adnexal tumor characterized by the proliferation of epithelial cells showing ductal differentiation in the form of anastomosing cords within a fibrovascular stroma. Contradictions persist in the literature regarding whether it represents a true neoplasm, a variant, or a reactive process; it is categorized into 4 subtypes by Starink and 5 by Weedon based on clinical presentations. Frequently manifesting as large hyperkeratotic plaques on the extremities of elderly individuals, these lesions can also occur as multiple neoplasms associated with hereditary disorders like Schöpf syndrome or as reactive processes linked to inflammatory cutaneous conditions. Microscopic evaluation reveals thin, interconnected epithelial cords attached to the epidermis forming a mesh-like pattern, accompanied by a loose stroma containing abundant mucin and a dense lymphoplasmacytic infiltrate. Immunohistochemically, expressions of EMA, CEA, CK19, and filagrin support ductal and acrosyringeal differentiation. Differential diagnosis includes poroma, syringofibrocarcinoma, and fibroepithelial basal cell carcinoma, and these benign adnexal lesions are successfully managed with cryotherapy, radiotherapy, or laser ablation.
Referanslar
Sangüeza, OP., Cassariono, DS., Glusac, EJ. ve ark. (2017) Cylindroma. Elder DE, Massi D, Scolyer RA ve Willemze R (Ed.) WHO Classification of skin tumors (189) Lyon; IARC
Takeda H, Mitsuhashi Y, Hayashi M, Kondo S. Eccrine syringofibroadenoma: case report and review of the literature. Journal of the European Academy of Dermatology and Venereology. 2001 Mar;15(2):147-9.
Mascaro JM. Considerations sur les tumeurs fibro-epitheliales. Le syringofibroadenome eccrine. Ann Dermatol Syphilol. 1963;90:146-53.
WEEDON D, LEWIS J. Acrosyringeal nevus. Journal of Cutaneous Pathology. 1977 Jun;4(3):166-8.
Weedon D. Eccrine syringofibroadenoma versus acrosyringeal nevus. Journal of the American Academy of Dermatology. 1987 Mar 1;16(3 Pt 1):622-3.
Mehregan AH, Marufi M, Medenica M. Eccrine syringofibroadenoma (Mascaro). Report of two cases. Journal of the American Academy of Dermatology. 1985 Sep 1;13(3):433-6.
Hashimoto K, Mehregan A, Kumakiri M. Tumors of Skin Appendages . In Boston: Butterworths,; 1987. p. 18–25.
Starink TM. Eccrine syringofibroadenoma: Multiple lesions representing a new cutaneous marker of the Schöpf syndrome, and solitary nonhereditary tumors. J Am Acad Dermatol. 1997 Apr 1;36(4):569–76.
Patterson, JW. (2021). Tumors of cutaneous appendages. James W. Patterson (Ed.) Weedon’s skin pathology (984-85). Çin; Elsevier.
Stewart, CL., Novoa, ROA. ve Seykora JT. (2015). Tumors of the epidermal appendages. David E. Elder (Ed.) Lever’s Histopathology of the skin (1274-76). Philedelphia; Wolters-Kluwer
Fouilloux B, Perrin C, Dutoit M, Cambazard F. Clear cell syringofibroadenoma (of Mascaro) of the nail. British Journal of Dermatology. 2001 Mar;144(3):625-7.
Noguchi M. Eccrine syringofibroadenoma developing in a sebaceous naevus. Br. J. Dermatol.. 2000;142:1050-1.
Billson VR, Dyall-Smith DJ. Eccrine syringofibroadenoma: a report of 2 cases. Pathology. 1991 Jan 1;23(3):259-62.
Nomura K, Kogawa T, Hashimoto I, Katabira Y. Eccrine syringofibroadenomatous hyperplasia in a patient with bullous pemphigoid: a case report and review of the literature. Dermatology. 1991;182(1):59-62.
Requena L, Sangüeza O. Mixed Tumors of the Skin. In Cutaneous Adnexal Neoplasms 2017. pp. 105-125. Springer, Cham.
Hu S, Bakshandeh H, Kerdel FA, Rongioletti F, Romanelli P. Eccrine syringofibroadenoma of clear cell variant: An immunohistochemical study. The American journal of dermatopathology. 2005 Jun 1;27(3):228-31.
González-Serva A, Pró-Rísquez MA, Oliver M, Caruso MG. Syringofibrocarcinoma versus squamous cell carcinoma involving syringofibroadenoma: is there a malignant counterpart of Mascaro's syringofibroadenoma? The American journal of dermatopathology. 1997 Feb 1;19(1): 58-65.
Katane M, Akiyama M, Ohnishi T, Watanabe S, Matsuo I. Carcinomatous transformation of eccrine syringofibroadenoma. Journal of cutaneous pathology. 2003 Mar;30(3):211-4.
Bjarke T, Ternesten‐Bratel A, Hedblad M, Rausing A. Carcinoma and eccrine syringofibroadenoma: a report of five cases. Journal of cutaneous pathology. 2003 Jul;30(6):382-92.
