Küçük Hücre Dışı Akciğer Kanserinde İzlenen Paraneoplastik Sendromlar
Özet
Paraneoplastik sendromlar, doğrudan tümör invazyonu veya metastazı ile ilişkili olmayan, malignite kaynaklı uzak etkiler olup en sık akciğer kanserinde (%10) görülür. Bu dokuda, küçük hücre dışı akciğer kanseri (KHDAK) başta olmak üzere akciğer kanserlerinde izlenen paraneoplastik sendromlar; endokrin, nörolojik, glomerüler, hematolojik, romatolojik ve dermatolojik olmak üzere altı ana grupta incelenmiştir. Endokrin sendromlar arasında PTHrP salgılanmasına bağlı malignite hiperkalsemisi (HHM), ektopik ADH üretimiyle karakterize uygunsuz ADH sendromu (SIADH), ektopik GHRH kaynaklı akromegali ve pulmoner karsinoid sendrom yer alır. Otoimmün kökenli nörolojik sendromlardan limbik ensefalit; glomerüler hastalıklardan ise nefrotik sendroma yol açan membranöz glomerülopati en yaygın görülenlerdir. Hematolojik grupta, KHDAK'ta ve özellikle adenokanser alt tipinde riski belirgin şekilde artan venöz tromboembolizm (VTE) öne çıkmakta, tedavide DMAH ve DOAC'lar kullanılmaktadır. Romatolojik sendromlar kapsamında çomaklaşma ve periostozla seyreden hipertrofik pulmoner osteoartropati (HPO) ile inflamatuar miyopatiler (polimiyozit/dermatomiyozit) ele alınmıştır. Son olarak, dermatolojik olarak akantozis nigrikans ve "işkembe avuç içi" bulguları açıklanmıştır. Tüm bu sendromların temel yönetim stratejisi, altta yatan primer malignitenin cerrahi, kemoterapi veya radyoterapi ile tedavi edilmesidir.
Paraneoplastic syndromes are malignancy-related distant effects that are not directly caused by primary tumor invasion or metastases, and they are most frequently associated with lung cancer, occurring in approximately 10% of cases. This document reviews the paraneoplastic syndromes observed in lung cancers, particularly non-small cell lung cancer (NSCLC), under six main categories: endocrine, neurological, glomerular, hematological, rheumatological, and dermatological. Endocrine syndromes include humoral hypercalcemia of malignancy (HHM) driven by PTHrP secretion, syndrome of inappropriate ADH (SIADH) characterized by ectopic ADH production, acromegaly caused by ectopic GHRH, and pulmonary carcinoid syndrome. Among the autoimmune neurological syndromes, limbic encephalitis is highlighted, while membranous glomerulopathy is the most common paraneoplastic glomerular disease leading to nephrotic syndrome. In the hematological group, venous thromboembolism (VTE) is prominent, carrying a higher risk in NSCLC and adenocarcinomas, with LMWH and DOACs utilized for its management. Rheumatological manifestations include hypertrophic pulmonary osteoarthropathy (HPO), characterized by digital clubbing and periostosis, as well as inflammatory myopathies like polymyositis and dermatomyositis. Finally, acanthosis nigricans and "tripe palms" are discussed as paraneoplastic dermatological syndromes. The primary treatment strategy for most of these syndromes relies on addressing the underlying malignancy through surgery, chemotherapy, or radiotherapy.
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