上顎洞腺癌の肺動脈腫瘍塞栓によって呼吸不全を呈したvon Recklinghausen病

A Case of von Recklinghausen Disease Showing Respiratory Failure due to Metastatic Pulmonary Vascular Tumor Embolism of an Upper Nasal Cavity Adenocarcinoma

要旨　von Recklinghausen病を基礎にもつ63歳，男性．右上顎洞腺癌に対し，根治切除術，および放射線療法70グレイを施行されていた．1年8カ月後，下肢の浮腫および咳嗽と呼吸困難が出現した．著明な低酸素血症，胸部X線写真にてびまん性の間質性陰影，CEAおよびCYFRAの上昇を認め，胸部HRCTでは巣状にスリガラス様陰影を呈し，脈管の肥厚を認めた．気管支肺胞洗浄液は血性で，経気管支肺生検では血管内に腺癌の腫瘍塞栓を認め，上顎洞癌の肺転移と判明した．von Recklinghausen病には神経原性腫瘍の合併が有名であるが，近年，上皮性腫瘍，特に腺癌の合併報告が増えている．今回，われわれはvon Recklinghausen病に組織型が腺癌であった上顎洞癌を合併した症例を報告する．

Summary

　The case is a 63-year-old man who had been treated complete resection and radiation therapy(70Gy) for mucous duct-type adenocarcinoma of right upper nasal cavity. His first chief complain was cough and edema of low extremities twelve months after operation, and he presented dyspnea. His laboratory findings showed severe hypoxia, elevated titer of serum CEA and CYFRA, chest X-ray revealed diffuse interstitial shadow, and chest HRCT revealed focal ground-glass opacities and thickness of bronchovascular-bandle. He had a metastatic lung cancer from the paranasal sinus. The speciemen of transbronchial lung biopsy presented tumor embolism of adenocarcinoma similar to the pri-mary tumor. It is famous that the patient in von Reckling-hausen disease complicates neurogenic tumor, but it is increased complicating epitherial type malignant tumor recently, especially adenocarcinoma. We report a case of von Recklinghausen disease complicating adenocarcinoma of upper respiratory tract.