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Kronik Öksürüğün Nadir Bir Sebebi: Diffüz İdiyopatik Pulmoner Nöroendokrin Hücre Hiperplazisi ve Karsinoid Tümör Birlikteliği
A Rare Cause of Chronic Cough: Coexistence of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Carcinoid Tumor
Turkiye Klinikleri Arch Lung. 2021;20(1):30-4
doi: 10.5336/archlung.2020-79759
Makale Dili: TR
Tam Metin
ÖZET
Diffüz idiyopatik pulmoner nöroendokrin hücre hiperplazisi [diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)], bronş duvarındaki nöroendokrin hücrelerin proliferasyonu ile karakterize nadir bir hastalıktır. İlk olarak Aguayo tarafından tanımlanan DIPNECH'nin, akciğer karsinoid tümörlerinin preinvaziv lezyonu olduğu düşünülmektedir. Hastaların ortalama tanı yaşı 66 olup, çoğu 'nonsmoker' kadınlardır. Hastalık hakkındaki tanı ve tedavi stratejileri kısıtlıdır. Uzun süredir var olan ve açıklanamayan nefes darlığı, öksürük gibi nonspesifik semptomlar ile kendini gösteren DIPNECH için bugüne değin histolojik, radyolojik ve/veya patolojik kriterlerin olduğu bir konsensüs yoktur. Bu sebeple geç ya da yanlış tanı alan bir hastalık olabilmektedir. Bu yazıda, 73 yaşında bilinen hiçbir hastalığı olmayan ve kronik öksürüğü olan bir hastanın video aracılı torakoskopik cerrahi biyopsi ile DIPNECH ve karsinoid tümör tanısı alması sunulmuştur.
Anahtar Kelimeler: Nöroendokrin tümörler; karsinoid tümörü; kronik öksürük
Diffüz idiyopatik pulmoner nöroendokrin hücre hiperplazisi [diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)], bronş duvarındaki nöroendokrin hücrelerin proliferasyonu ile karakterize nadir bir hastalıktır. İlk olarak Aguayo tarafından tanımlanan DIPNECH'nin, akciğer karsinoid tümörlerinin preinvaziv lezyonu olduğu düşünülmektedir. Hastaların ortalama tanı yaşı 66 olup, çoğu 'nonsmoker' kadınlardır. Hastalık hakkındaki tanı ve tedavi stratejileri kısıtlıdır. Uzun süredir var olan ve açıklanamayan nefes darlığı, öksürük gibi nonspesifik semptomlar ile kendini gösteren DIPNECH için bugüne değin histolojik, radyolojik ve/veya patolojik kriterlerin olduğu bir konsensüs yoktur. Bu sebeple geç ya da yanlış tanı alan bir hastalık olabilmektedir. Bu yazıda, 73 yaşında bilinen hiçbir hastalığı olmayan ve kronik öksürüğü olan bir hastanın video aracılı torakoskopik cerrahi biyopsi ile DIPNECH ve karsinoid tümör tanısı alması sunulmuştur.
Anahtar Kelimeler: Nöroendokrin tümörler; karsinoid tümörü; kronik öksürük
ABSTRACT
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare condition charactarized by profileration of neuroendocrine cells in bronchial wall. DIPNECH, first defined by Aguayo, is thought to be a pre-invasive lesion of lung carcinoid tumors. The average age of patients diagnosed is 66, most of whom are non-smoker women. Diagnostic and therapeutic strategies for the disease are limited. There is no consensus regarding the clinical, histological, radiological and/or pathological criteria for DIPNECH presented by long-standing and nonspecific symptoms such as unexplained shortness of breath and cough therefore DIPNECH might be a misdiagnosed or late-diagnosed disease. In this article a 73 year old patient is presented with no undelying disease and chronic cough who was diagnosed with DIPNECH and carcinoid tumor by video assisted thoracoscopic surgery biopsy.
Keywords: Neuroendocrine tumors; carcinoid tumor; chronic cough
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare condition charactarized by profileration of neuroendocrine cells in bronchial wall. DIPNECH, first defined by Aguayo, is thought to be a pre-invasive lesion of lung carcinoid tumors. The average age of patients diagnosed is 66, most of whom are non-smoker women. Diagnostic and therapeutic strategies for the disease are limited. There is no consensus regarding the clinical, histological, radiological and/or pathological criteria for DIPNECH presented by long-standing and nonspecific symptoms such as unexplained shortness of breath and cough therefore DIPNECH might be a misdiagnosed or late-diagnosed disease. In this article a 73 year old patient is presented with no undelying disease and chronic cough who was diagnosed with DIPNECH and carcinoid tumor by video assisted thoracoscopic surgery biopsy.
Keywords: Neuroendocrine tumors; carcinoid tumor; chronic cough
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