Digital Papiller Adenokarsinom
Özet
Dijital papiller adenokarsinom (DPA), ekrin ter bezlerinden köken alan, akral bölgelerde ağrısız ve yavaş büyüyen nodüllerle karakterize, nadir ve agresif bir malign deri eki tümörüdür. Genellikle erkeklerde el parmaklarında görülen bu neoplazm, yüksek lokal nüks (%5-21) ve özellikle akciğere metastaz (%26-50) yapma potansiyeline sahiptir. Histopatolojik olarak, epidermis ile ilişkisiz dermiste yerleşen tümör; solid, kistik ve kribriform yapılar ile karakterize olup, luminal küboidal ve dış miyoepitelyal hücrelerden oluşan çift sıralı epitel dizilimi gösterir. Ayırıcı tanıda hidradenom ve papiller ekrin adenom gibi benign lezyonlardan ayrılması, infiltratif büyüme paterni, atipi ve mitoz varlığı ile sağlanır. İmmunhistokimyasal olarak luminal tabakada Pansitokeratin, CK7, EMA ve CEA pozitifliği; dış tabakada ise p63 ve SMA gibi miyoepitelyal belirteçler izlenir. Son genetik araştırmalar tümörde BRAF-V600E mutasyonunun varlığını ortaya koymuştur. Erken tanı, lezyonların atlanmaması adına kritik öneme sahip olup, ana tedavi seçeneği nüks ve metastaz riskini önemli ölçüde azaltan geniş cerrahi eksizyon veya ampütasyondur.
Digital papillary adenocarcinoma (DPA) is a rare, aggressive malignant cutaneous adnexal tumor originating from eccrine sweat glands, characterized by painless, slow-growing subcutaneous nodules on acral sites. Predominantly affecting males on the palmar aspect of digits, it harbors a high potential for local recurrence (5-21%) and metastasis (26-50%), primarily targeting the lungs. Histopathologically, the tumor resides in the dermis without epidermal connection, displaying solid, cystic, and pseudopapillary areas with a typical cribriform pattern. The glandular structures feature a distinct double-layered epithelium composed of inner cuboidal cells and outer myoepithelial cells. Differentiating DPA from benign mimics like hidradenoma and papillary eccrine adenoma relies on identifying infiltrative growth, atypia, and mitotic activity. Immunohistochemically, luminal cells express Pancytokeratin, CK7, EMA, and CEA, while outer cells react with p63 and SMA. Recent genetic analyses via next-generation sequencing have identified BRAF-V600E mutations in these tumors. Given its high recurrence and metastatic rates, prompt diagnosis and complete wide surgical excision or amputation remain the gold standard treatment to optimize patient outcomes.
Referanslar
Kazakov DV, Michal M, Kacerovska D, McKee PH.2012.Cutaneous Adnexal Tumors. 1th. ed Philadelphia: Wolters Kluwer-Lippincott Williams &Wilkins.
Elder DE, Massi D, Scolyer RA, Willemze R.2018. WHO Classification of Skin Tumours. 4th ed. Lyon: IARCH
Jabbour AJ, Tangoren IA, Kanik AB. Digital Papillary Adenocarcinoma: Case of a Rare Malignant Cutaneous Tumor of the Eccrine Sweat Gland. Case Rep Dermatol. 2021 Aug 6;13(2):422-427.doi: 10.1159/000518442
Chi Chi C, Kuo T T ,Wang S S, Aggressive Digital Papillary Adenocarcinoma A Silent Malignancy Masquerading as Acquired Digital Fibrokeratoma. Am J Clin Dermatol 2007; 8 (4): 243
Carter K.Yao J.J.Melton S.D.Lopez J.Huerta S.Digital papillary adenocarcinoma: presentation, natural history and management.Rare Tumors. 2015; 7: 92-95
Chisholm C, Jessup C, Vandergrif T, Motaparthi K, Elston D M.2017 Dıagnostıc Pathology: Nonneoplastıc Dermatopathology, Second Edıtıon.Elsevier
Patterson JW, editor. Tumors of cutaneous appendages. Weedon’’s Skin Pathology. 5th ed. Philedephia: Elsevier; 2021. p. 970–971.
Duke WH , Sherrod TT , Lupton GP. Aggressive digital papillary adenocarcinoma (aggressive digital papillary adenoma and adenocarcinoma revisited) . Am J Surg Pathol. 2000 ; 24 : 775 – 784
Wiedemeyer, K, Gill, P, Schneider, M et al. Clinicopathologic Characterization of Hidradenoma on Acral Sites.A Diagnostic Pitfall With Digital Papillary Adenocarcinoma..The American Journal of Surgical Pathology: May 2020 - Volume 44 - Issue 5 - p 711-717 doi: 10.1097/PAS.0000000000001426
Kao G.F.Helwig E.B.Graham J.H. Aggressive digital papillary adenoma and adenocarcinoma. A clinicopathological study of 57 patients, with histochemical, immunopathological, and ultrastructural observations..J Cutan Pathol. 1987; 14: 129-146
Weingertner N, Gressel A , Battistella M et al. Aggressive digital papillary adenocarcinoma: A clinicopathological study of 19 cases. J Am Acad Dermatol. 2017 Sep;77(3):549-558.e1. doi: 10.1016/j.jaad.2017.02.028
Suchak R, Wang W L, . Prieto V G et al. Cutaneous digital papillary adenocarcinoma: A clinicopathologic study of 31 cases of a rare neoplasm with new observations. American Journal of Surgical Pathology, 36(12), 1883-1891. https://doi.org/10.1097/PAS.0b013e31826320ec
Bell D , Aung P , Prieto P V et al. Next-generation sequencing reveals rare genomic alterations in aggressive digital papillary adenocarcinoma AnnDiagnPathol. 2015 Dec;19(6):381-4. doi: 10.1016/j.anndiagpath.2015.08.002
Jih D M Elenitsas R , Vittorio C C, Aggressive digital papillary adenocarcinoma: a case report and review of the literature. Am J Dermatopathol. 2001 Apr;23(2):154-7. doi: 10.1097/00000372-200104000-00014
Haynes D.Thompson C.Leitenberger J.Vetto J.Mohs micrographic surgery as a digit-sparing treatment for aggressive digital papillary adenocarcinoma.Dermatol Surg. 2017; 43: 1487-148.
Rismiller K Knackstedt T J Aggressive Digital Papillary Adenocarcinoma: Population-Based Analysis of Incidence, Demographics, Treatment, and Outcomes. . 2018 Jul;44(7):911-917. doi: 10.1097/DSS.0000000000001483.
Dhillon P , Powell B, Mehdi S. Aggressive digital papillary adenocarcinoma and sentinel node biopsy: A case report and literature review. JPRAS Open Volume 24, June 2020, Pages 43-46. doi.org/10.1016/j.jpra.2020.03.001
