Gastrointestinal Tümörler ve Paraneoplastik Sendromlar

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Özet

Paraneoplastik sendromlar, malign tümörlerin tetiklediği immün yanıtlar ve salgılanan sitokin, peptid ya da hormonlar sonucu gelişen, doğrudan tümör kitlesine bağlı olmayan sistemik klinik tablolardır. Genellikle akciğer ve meme tümörlerine eşlik etseler de, gastrointestinal sistem (GİS) tümörlerinde de sıklıkla görülürler ve en çok akantozis nigrikans, seboreik dermatoz gibi dermatolojik bulgularla ortaya çıkarlar. GİS malignitelerinden küçük hücreli özefagus kanserinde sensori-motor nöropati ve vaskülit izlenebilirken; mide kanserinde Trousseau bulgusu (tromboflebit), mikroanjiyopatik hemolitik anemi, membranöz glomerülonefrit ve Lesser-Trelat bulgusu dikkat çeker. Hepatosellüler karsinomda (HSK) ciddi seyreden Tip B hipoglisemi, eritropoietin kaynaklı eritrositoz, hiperkalsemi ve hiperkolesterolemi en sık saptanan paraneoplastik sendromlardır. Pankreatik nöroendokrin tümörler ise Cushing sendromu ile dirençli diyareye yol açabilir. Bu sendromların patofizyolojisinde vücudun ürettiği Anti-Hu, Anti-Ri, Anti-Yo gibi otoantikorlar ile tümör kaynaklı medyatörler rol oynar. Tedavide ana strateji cerrahi, kemoterapi veya radyoterapi ile primer tümör yükünü ortadan kaldırmaktır; ayrıca otoantikorları hedefleyen immünsupresyon, intravenöz immünoglobulin ve plazma değişimi uygulanır. Gizli tümörlerin erken tespitini sağlayan bu süreçler, multidisipliner bir yaklaşım gerektirir.

Paraneoplastic syndromes are clinical manifestations occurring secondary to altered immune responses triggered by malignant tumors, leading to the secretion of various cytokines, peptides, or hormones rather than direct tumor invasion. Although commonly associated with lung and breast cancers, their incidence in gastrointestinal (GI) tract tumors is significant, with dermatological manifestations such as acanthosis nigricans and seborheic dermatosis being the most frequent. In specific GI malignancies, small cell esophageal cancer can present with sensorimotor neuropathy and vasculitis, while gastric cancer is often associated with Trousseau's sign (thrombophlebitis), microangiopathic hemolytic anemia, and Lesser-Trelat sign. In hepatocellular carcinoma (HCC), severe Type B hypoglycemia, erythrocytosis due to erythropoietin secretion, hypercalcemia caused by parathyroid hormone-related peptide, and hypercholesterolemia represent characteristic paraneoplastic features. Additionally, pancreatic neuroendocrine tumors can induce Cushing's syndrome and severe diarrhea. The complex pathophysiology involves tumor-secreted substances or autoantibodies such as Anti-Hu, Anti-Ri, and Anti-Yo that inadvertently target normal tissues. The primary treatment strategy focuses on eradicating the underlying tumor through surgery, chemotherapy, or radiotherapy; secondary methods utilize immunosuppressive agents, intravenous immunoglobulin, or plasmapheresis to clear harmful autoantibodies. Requiring a multidisciplinary approach, early detection of these syndromes is vital for uncovering occult tumors and improving patient survival.

Referanslar

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199-204

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14 Ekim 2022

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