Silindirom
Özet
Silindromlar, ağırlıklı olarak orta yaşlı ve yaşlı kadınların baş-boyun bölgesinde soliter nodüller şeklinde izlenen, mikroskobik olarak mozaik benzeri bir dizilim sergileyen iyi huylu deri eki tümörleridir. Kalıtsal olan ailesel silindromatozis ve Brooke-Spiegler sendromu gibi durumlarda saçlı deride birleşme eğilimi gösteren multipl "türban tümörleri" oluşabilir ve bu olgular CYLD geni mutasyonlarıyla ilişkilidir. Histopatolojik olarak, çevresi eozinofilik hiyalin bantlarla sarılı bazaloid hücre adalarından meydana gelen bu tümörlerde, koyu nükleuslu periferik ve veziküler nükleuslu santral olmak üzere iki belirgin hücre tipi ayırt edilir. İmmünohistokimyasal profilinde apokrin orijini destekleyen CEA, EMA, CK7 ve alfa-düz kas aktin pozitifliği saptanırken, malign transformasyon oldukça nadir olup hiyalin kılıf kaybı ve p53 ekspresyon artışı ile karakterizedir. Tedavisinde cerrahi eksizyon, kriyoterapi ve elektrodesikasyon küretaj tercih edilirken, ayırıcı tanısında hiyalin yapı içermeyen bazal hücreli karsinom ve benzer hücre tiplerine sahip spiradenom titizlikle değerlendirilmelidir.
Cylindromas are benign adnexal tumors presenting microscopically with a mosaic-like pattern, characteristically appearing as solitary nodules in the head and neck region of middle-aged to elderly female patients. In hereditary conditions like familial cylindromatosis and Brooke-Spiegler syndrome, multiple lesions can coalesce on the scalp to form large "turban tumors," driven by mutations in the CYLD tumor suppressor gene that signify a folliculosebaceous-apocrine origin. Histopathologically, these unencapsulated dermal tumors consist of irregular basaloid cell islands surrounded by PAS-positive, eosinophilic hyalin bands composed of collagen types IV and VII. The tumor nests comprise peripheral palisading cells with dark nuclei and larger, centrally located cells with vesicular nuclei. Immunohistochemically, the expression of EMA, CEA, CK7, and alpha-smooth muscle actin supports an apocrine differentiation, whereas local aggressiveness and malignant transformation—marked by the loss of the hyaline sheath—remain extremely rare. Surgical excision, cryotherapy, and electrodesiccation curettage are standard treatments, and precise differentiation from basal cell carcinoma and spiradenoma is crucial during histopathological evaluation.
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.
Crain RC, Helwig EB. Dermal cylindroma (dermal eccrine cylindroma). Am J Clin Pathol 1961;35:504–515.
Patterson, JW. (2021). Tumors of cutaneous appendages. James W. Patterson (Ed.) Weedon’s skin pathology (985-7). 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 (1268-71). Philedelphia; Wolters-Kluwer.
Casado M, Borbujo J. Multiple linear cylindromas. J Am Acad Dermatol. 1992 May 1;26(5):821–4.
Guzzo C, Johnson B. Unusual abdominal location of a dermal cylindroma. Cutis. 1995 Oct 1;56(4):239-40.
Gupta R, Jain R, Sood S, Mohan H. Dermal cylindroma presenting as mass lesion in superomedial orbit. Indian Journal of Ophthalmology. 2003 Jan 1;51(3):257-9.
Sharma HS, Meorkamal MZ, Zainol H, et al. Eccrine cylindroma of the ear canal - report of a case. J Laryngol Otol. 1994;108:706–709.
Saunders H, Tucker P, Saurine T, et al. Pedigree of multiple benign adnexal tumours of Brooke–Spiegler type. Australas J Dermatol 2003;44:144–8.
Uede K, Yamamoto Y, Furukawa F. Brooke-Spiegler Syndrome Associated with Cylindroma, Trichoepithelioma, Spiradenoma, and Syringoma. J Dermatol. 2004;31(1):32–8.
Oiso N, Mizuno N, Fukai K, et al. Mild phenotype of familial cylindromatosis associated with an R758X nonsense mutation in the CYLD tumour suppressor gene. Br J Dermatol 2004;151:1084–6.
Lee MW, Kelly JW. Dermal cylindroma and eccrine spiradenoma. Australas J Dermatol 1996;37:48–9.
Goodlad, J. ve Calonje, E. Tumors of the sweat gland. Eduardo Calonje (Ed.) McKee’s Pathology of the skin with clinical correlation (1657-9). Çin; Elsevier.
Kazakov DV, Soukup R, Mukensnabl P, et al. Brooke-Spiegler syndrome: report of a case with combined lesions containing cylindromatous, spiradenomatous, trichoblastomatous, and sebaceous differentiation. Am J Dermatopathol. 2005;27:27–33.
Lin PY, Fatteh SM, Lloyd KM. Malignant transformation in a solitary dermal cylindroma. Arch Pathol Lab Med 1987;111:765–7.
Penneys NS, Kaiser M. Cylindroma expresses immunohistochemical markers linking it to eccrine coil. J Cutan Pathol 1993;20:40–3.
Weber L, Wick G, Gebhart W, et al. Basement membrane components outline the tumour islands in cylindroma. Br J Dermatol. 1984;111:45–51.
Urbach F, Graham JH, Goldstein J, et al. Dermal eccrine cylindroma: a histochemical, electron microscopic, and therapeutic (X-ray) study. Arch Dermatol 1963;88:880– 894.
Soyer HP, Kerl H, Ott A. Spiradenocylindroma – More than a coincidence? Am J Dermatopathol 1998;20:315–17.
Tellechea O, Reis JP, Ilheu O, et al. Dermal cylindroma: an immunohistochemical study of thirteen cases. Am J Dermatopathol 1995;17:260–5.
Galadari E, Mehregan AH, Lee KC. Malignant transformation of eccrine tumors. J Cutan Pathol 1987;14:15–22.
Kazakov DV, Grossmann P, Spagnolo DV, et al. Expression of p53 and TP53 mutational analysis in malignant neoplasms arising in preexisting spiradenoma, cylindroma, and spiradenocylindroma, sporadic or associated with Brooke–Spiegler syndrome. Am J Dermatopathol 2010;32:215–21.
Rutten A, Hegenbarth W, Kohl PK, et al. Primary cutaneous adenoid cystic carcinoma mimicking dermal cylindroma: histology of the complete surgical excision as the key to diagnosis. J Dtsch Dermatol Ges 2018;16:1016–18.
