Respiratory Failure due to Pulmonary Lymphangitis Carcinomatosis* ]in, Fufita,M.D., Ph.D., EC.C.E; Yoshifumi Yomagishi, M . D.; Akihito Kubo. M. D.; Keiichi Takigawa. M.D.; YoaufumiYamaji,M.D.. Ph.D.;and]iwToJurra. M.D., Ph.D. A man with primary lung cancer developed respiratory failure due to lymphangitis carcinomatosis that was diag- nosed by transbmnchial lung biopsy specimen. AAer comb i i chemotherapy with high& etoposide and cisplatin (CDDP), he was able to cease oxygen therapy and s h e d impmvement of his lymphangitis carcinomatosis. He received a total dose of 13,400 mg/ms of etoposide. This case suggested that respiratory failure due to lymphangitis carcinomatosis can be a treatable condition. (Chest 1993; 103:967-68) L ymphangitis carcinomatosis is a welldefined pathologic entity that is characterized by diffuse permeation of the pulmonary lymphatics with metastatic tumor, and it generally has a very poor prognosis. Mk describe herein a patient with respiratory failure due to the lymphatic spread of pulmonary adenocarcinoma; this was improved by combination chemotherapy consisting of highdose etoposide and cisplatin (CDDP). *Fmm the First Depnrtment of Internal Medicine. Kagawa Medical w a .Japan. School. m Reptint requests. Dr lir' , 1st Department of lnternal Medicrne. Kagaw Medkd' S c 1d7 S 1 Miki-cho, Kita-gun. Kagaum 7610 7 s lopnn A 55-yearold man was admitted to our institution with a fourweek history of hemoptysis and progressively increasing dyspnea associated with a nonproductive cough. The medical history was unremarkable. Physical examination showed a palpable lymph node in the right supraclavicularregion. The chest roentgenogram showed a coin lesion of the right middle lobe, generalized interstitial and alveolar opacities, and small bilateral pleural ehsions. Roentgenographic features of a coin lesion of the right middle lobe appeared to be pulmonary in origin, and transbronchial biopsy specimen obtained from this lesion showed a poorly differentiated adenocarcinoma. Significant hypoxemia was shown by arterial blood gas studies with the patient breathing room air (Pa0,=51.6 mm Hg; PaCOa=37.1 mm Hg; and pH =7.46). The cause of his respiratory distress was shown to be lymphangitic spread of a poorly differentiated adenocarcinoma as the result of transbronchial lung biopsy. He was treated with four courses of combination chemotherapy using CDDP (80 mg/ma, day I), fluomuracil (800 mdm2, days 1 to 3), and etoposide (70 mg/mg,days 1to 5). His respiratory symptoms decreased after combination chemothera~v. .,. but his condition deteriorated again immediately after the fourth course of chemotherapyWe then decided to treat him with high-dose etoposide (500 m d ma, days 1to 3, total: 1,500m@ma)and CDDE After the first course of high-dose combination chemotherapy, his respiratory symptoms were relieved. High-resolution computed tomography also demonstrated improvement of the thickening of the bronchovascular bundles (Fig 1). His PaQ improved from 56.6 mm Hg to 67 mm Hg. However, his respiratory symptoms gradually deteriorated during the intervals between courses of chemotherapy. The highdose combination chemotherapy was finally repeated for a total of eight courses to control his symptoms. Table 1showed the changes of PaQ before and after the combination chemotherapy. There was no other interval process that had impacted on the blond gas values (infection or pulmonary infarction, for example). The total cnmulative dose of etoposide administered to this patient was 13.400 mg/ mg. After eight courses of high-dose chemotherapy, his condition again deteriorated and he died of respiratory failure 14 months from the original onset of his symptoms. FIGURE1. High-resolution computed tomographic (q image of the patient before (A) and after (B) chemotherapy. Thickening of the b m n c h d bundles was markedly improved after chemotherapy. CHEST 1 103 1 3 1 MARCH, 1993 Downloaded From: http://publications.chestnet.org/ on 09/09/2014 %7 Table 1-RaO, Values before and after Cornbindion Chemotherapy with High-Dose E t o p o . d e and CDPP High-Dose Chemotherapy, Course PaO, before, mm Hg (Condition) 56.6 61.6 61.3 53.4 53.3 48.7 60.1 57.5 (room air) (room air) (roomair) (nasal 2 L) (room air) (room air) (room air) (mask 10 L) PaO, after, mm Hg (Condition) 67 (room air) 80.3 (room air) 61 (room air) 69.1 (room air) 62.2 (room air) 60.1 (roomair) 68.1 (mask 2 L) 167.9 (mask 10 L) Diffuse involvement of the pulmonary lymphatics, often referred to as lymphangitis carcinomatosis, gives rise to a very striking clinical and pathologic picture.' Many different varieties of primary cancer may give rise to this form of spread within the lungs, notably cancers of the stomach, breast, and lung. The chest roentgenogram typically shows an interstitial pattern with streaky micronodular mottling, which sometimes coexists with alveolar opacities, and often with pleural effusions. Rapid deterioration marked by progressive respiratory impairment is the usual course, and the mean survival after presentation has been reported to b e only two m0nths.l The value of chemotherapy in the management of lymphangitis carcinomatosis has been disputed, and more purely palliative measures, such as the appropriate use of opiates, and the control of pleural effusions, have been r e c o ~ n m e n d e d . ~ However, Fernandez e t a14 suggested that lymphangitis carcinomatosis due to ovarian carcinoma could b e reversed by chemotherapy. In this case, the patient was relieved from progressive dyspnea by combination chemotherapy consisting of high-dose etoposide and C D D P To control his respiratory symptoms, we were impelled to continue combination chemotherapy and finally he received 13,400 mgl m2 of etoposide and 960 mglm2 of C D D P Although costeffectiveness should also be considered, our case suggests that combination chemotherapy can have a role in the treatment of patients with lymphangitis carcinomatosis. 1 Spencer H. Pathology of the lung, 3rd ed. Belfast: Pergamon Press 1981. 2 Hauser TE, Steer A. Lymphangitis carcinomatosa of the lungs: six case reports and a review of the literature. Ann Intern Med 1951; 34:881 3 Streeton JA. Lymphangitis carcinomatosa. Med J Aust 1981; 4:380 4 Fernandez K, O'Hanlan KA, Rodriguez-Rodriguez L, Marino WD. Respiratory failure due to interstitial lung metastases of ovarian carcinoma reversed by chemotherapy. Chest 1991; 99;1533-34 Aortic Stenosis Associated with Scheie's Syndrome* Report of Successful Valve Replacement Hirvshi Masudo, M.D.; Yasuo Morishita, M.D.; Akira Taira, M.D.; and Masclru Kuriymu, M.D. A 62-year-old man who had aortic stenosis associated with Scheie's syndrome (mucopolysaccharidosis [MPS], type I-S) successfully underwent aortic valve replacement. T h e composition of acidic glycosaminoglycans (acid muwpolysaccharides) of the excised aortic valve analyzed by highperformance liquid chromatography (HPLC) supported the diagnosis of Scheie's syndrome. This article reviews the literature on aortic stewsis in MPS, a rare inherited metabolic disorder, and discusses biochemical features and ( C h t 1993; 103:968-70) surgical repair. I ACAC = acidic glycosaminoglycans, HPLC = high- rformance liquid chromatography; MPS = mucopolysaaehariGis M ucopolysaccharidosis (MPS) is a group of rare inherited metabolic diseases subdivided into several syndromes according to clinical, genetic, and biochemical features. The basic characteristic is excessive accumulation of acidic glycosaminoglycans (AGAG) in various tissues. Cardiac abnormality, especially a valvular lesion, is relatively common and is the most life-threatening complication. However, reports of aortic stenosis associated with Scheie's syndrome (MPS, type I-S) are very rare. We recently saw a patient with aortic stenosis associated with Scheie's syndrome, in which aortic valve replacement gave a good long-term result. The purpose of this article is to present the clinical, histologic, and biochemical features of this very rare combination and to evaluate the significance of prosthetic valve replacement. A 62-yearold man came to our hospital for evaluation of general fatigue. At the time of hospital admission, he was 150 cm tall and weighed 46 kg. He had a flat nasal bridge, thick lips, and mild macroglossia. His neck was short, and shoulders were narrow and rounded. Corneal clouding was noted bilaterally. The external rotation of arms and forearms was slightly limited. A grade 416 aortic systolic ejection murmur and a grade 3/6 aortic diastolic blowing murmur were heard. Routine hematologic and biochemical examinations gave normal values. Urinary analysis showed excessive excretion of dermatan sulfate. In both lymphocyte and fibroblast cultures, lysosomal alpha-L-iduronidase was deficient, strongly indicating Scheie's syndrome. Cardiac catheterization revealed severe stenosis and slight regurgitation in the aortic valve. His parents, who were first cousins, did not have obvious abnormalities However, the younger brother had similar clinical features: flat nasal bridge, macroglossia, short neck, bilateral corneal clouding, limited flexion ofarms and forearms, umbilical hernia. And lysosomal alphaL-iduronidase was deficient. Echocardiography revealed slight k *From the Second Department of Surgery Dn. Masuda, Morishita, and Taira) and the Third Department o Internal Medicine (Dr. Kuriyarna), Kagoshima University Faculty of Medicine, Kagoshima, Japan. Reprint requests: Dr. Masuda, 2nd Department of Surgery, Kagoshim Unioersity of Medicine, 8-35-1 Sakuragaoka, Kagoshimu 890, lapan Aortic Stenosis Associated with Schem's S y n d m (Masuda eta/) Downloaded From: http://publications.chestnet.org/ on 09/09/2014