A retrospective review of Mayo Clinic records through 1983 revealed 84 patients (24 male and 10 female; mean age, 41 years) with the diagnosis of pulmonary alveolar phospholipoproteinosis. The major clinical features were dyspnea, cough, fever, and chest pain. Chest roentgenograms usually showed bilateral symmetric alveolar infiltrates, but asymmetric, unilateral, and chronic patchy patterns were also noted. Diagnosis was established by thoracotomy-lung biopsy in 26 patients. Histologic analysis revealed uniform filling of the alveoli by periodic acid-Schiff-positive material and maintenance of normal alveolar architecture. Electron microscopy showed enlarged alveolar macrophages with lamellar osmiophilic inclusions, dense granules, and myeloid bodies. Of the 21 patients who underwent therapeutic bronchoalveolar lavage, 13 had no recurrence of the disease during a mean follow-up of 8.8 years. In patients who underwent pulmonary function testing both before and after lavage, significant restrictive dysfunctions present before the procedure were alleviated afterward. Three deaths occurred among the 34 patients. Pulmonary alveolar phospholipoproteinosis may result from defective clearance of phospholipids by the alveolar macrophages, excessive production of phospholipids by type II pneumocytes, or both. It is likely a nonspecific response to a variety of injuries to the alveolar macrophage or type II pneumocyte or both, including exposure to certain dusts and chemicals and occurrence of hematologic diseases or infections. The uncommon occurrence of this disorder suggests individual susceptibility.

Previously reported from our institution has been a series of 63 patients with pulmonary arteriovenous fistula who were seen from Jan. 1, 1952, through Dec. 31, 1972. Subsequently, we have seen 38 additional patients during the 8 1/2-year period from Jan. 1, 1973, through June 1981. The series includes three patients with hereditary telangiectasia who had bilateral pulmonary arteriovenous fistulas removed at two separate thoracotomies. Our report also includes a brief description of five additional patients with acquired systemic artery-to-pulmonary artery fistula who underwent miniballoon occlusion of the fistula. We are including these five cases because we believe this therapeutic catheterization technique may be of particular value in patients with multiple or bilateral pulmonary arteriovenous malformations and may obviate extensive pulmonary resection and repeat thoracotomy. Pulmonary arteriovenous fistula is believed to occur most often in middle-aged women who have associated Rendu-Osler-Weber syndrome, but most of our patients did not have hereditary hemorrhagic telangiectasia. Twenty-three (36.5%) of the 63 patients in a previous Mayo Clinic series and 18 (47%) of the 38 in the present series had associated Rendu-Osler-Weber syndrome. A logical workup of a patient with pulmonary arteriovenous fistula includes, in sequential fashion based on priority, chest roentgenography and tomography, arterial blood gas measurements, contrast echocardiography with indocyanine green dye, angiography, and measurement of differential pulmonary venous oxygen content.

Thymic tumors are uncommon, and malignant variants of such neoplasms are rare. Hence, the association of two siblings with aggressive tumors of the thymus is not likely to be a coincidence. We report on two brothers with thymic epithelial neoplasms, one being a thymic carcinoma and the other an invasive spindle cell thymoma with associated hypogammaglobulinemia.

Pulmonary lymphangiomyomatosis is a disease of young females, who typically present with progressive dyspnea, hemoptysis, cough, repeated spontaneous pneumothoraces, and chylous effusions. The disease should be suspected when these findings are associated with an obstructive ventilatory impairment and disproportionately abnormal gas exchange. Symptoms may occur in the presence of radiographically normal-appearing pulmonary parenchyma. As the disease progresses, diffuse, prominent interstitial markings, suggestive of pulmonary fibrosis, are seen radiographically. The diagnosis is made by open lung biopsy.

Stage I sarcoidosis usually presents with roentgenographic evidence of hilar adenopathy and the patients are totally asymptomatic. However, five patients were studied at the Mayo Clinic who had stage I sarcoidosis associated with obstructive disease of the airways. Four of the five presented with dyspnea, wheezing, and cough, and they were found to have expiratory slowing on physical examination. One patient was asymptomatic and her physical examination was normal. On pulmonary function testing, she had a decrease in maximal midexpiratory flow, and at fiberoptic bronchoscopy, mucosal changes consistent with noncaseating granuloma of sarcoidosis were seen. All five patients had the classic roentgenographic appearance of sarcoidosis, with hilar and right paratracheal adenopathy. Endobronchial involvement is well known in sarcoidosis, but its significance in stage I disease has not been emphasized in the literature. An awareness of this possibility is important because it may be an indication for bronchoscopy and mucosal biopsy in the patient with stage I sarcoidosis, particularly when the patient presents with dyspnea, wheezing, and cough. Also, corticosteroid, therapy may be indicated in selected patients with stage I sarcoidosis.

To the Editor: I read with interest the editorial by Leroy Hyde, M.D., entitled “Pneumothorax: A Rare Manifestation of Lung Cancer.”1Hyde L Pneumothorax: A rare manifestation of lung cancer (editorial).Chest. 1977; 72: 557Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar I certainly concur that spontaneous pneumothorax is extremely rare in primary bronchogenic carcinoma. Spontaneous pneumothorax is usually seen in young male patients and is usually caused by a rupture of a pleural bleb. When we2Dines DE Clagett OT Payne WS Spontaneous pneumothorax in emphysema.Mayo Clin Proc. 1970; 45: 481-487PubMed Google Scholar reviewed our experience with spontaneous pneumothorax in patients with emphysema, we were amazed that pneumothorax in emphysema was not more common. I am writing because I take issue with Hyde's1Hyde L Pneumothorax: A rare manifestation of lung cancer (editorial).Chest. 1977; 72: 557Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar comments that spontaneous pneumothorax cannot be considered more than a rare manifestation of underlying malignant disease and that when this rare combination occurs, the patient will almost always be more than 40 years of age. I think that spontaneous pneumothorax is not that rare with metastatic sarcomas. We3Dines DE Cortese DA Brennan MD et al.Malignant pulmonary neoplasms predisposing to spontaneous pneumothorax.Mayo Clin Proc. 1973; 48: 541-544PubMed Google Scholar reported the findings in five patients with spontaneous pneumothorax associated with metastatic sarcomas in 1973, and since that time, I have seen six additional patients. I think that this is an important association and can be seen in very young people. Two of the patients whom we3Dines DE Cortese DA Brennan MD et al.Malignant pulmonary neoplasms predisposing to spontaneous pneumothorax.Mayo Clin Proc. 1973; 48: 541-544PubMed Google Scholar described were 18 years of age; one was 20, one was 26, and one was 36 years old. I believe that this is an important point. I think that there is an increased incidence of pneumothorax in people with metastatic sarcomas. Occasionally, as occurred in two of our patients, the pneumothorax preceded the development of the metastatic nodules appearing on the chest x-ray film. I certainly concur with Hyde1Hyde L Pneumothorax: A rare manifestation of lung cancer (editorial).Chest. 1977; 72: 557Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar that pneumothorax should not be considered as a clinical manifestation of primary bronchogenic carcinoma.

Fifty samples of pleural fluid, collected from consecutive patients in a thoracic clinic who had diagnostic thoracentesis, were studied prospectively. Pleural fluid protein was of value in differentiating transudates from exudates. Pleural fluid red cell counts, white blood cell counts, and differential white blood cell counts have no specificity and no usefulness in the differential diagnosis of the origin of the effusion. Pleural fluid cytology was positive in 60% of all the malignancies studied in this series; for the group with metastatic breast carcinoma, there was a 78% positive pleural fluid cytology. Differential white cell counts revealed tumor cells in 45% of malignant effusions. In our experience, the finding of tumor cells is the only useful finding in differential cell counts of the pleural fluid.

Two cases in which infection of the lung by Helminthosporium was characterized by recurrent attacks of asthma with cough productive of sputum and hemoptysis are presented. Resection of the involved lung in each instance demonstrated bronchiectasis with chronic pneumonia and, in one, multiple abscesses. Many septate hyphae, 2.5 to 3 µ in diameter, were detected in the mucous plugs, bronchiolar mucosa and alveoli. Many colonies of Helminthosporium sp. were isolated from the pulmonary tissues removed aseptically at operation. An analogy is drawn between these two cases and cases of allergic aspergillosis reported in the literature. Most likely the mode of entry is inhalation; study of these two cases suggests infection with Helminthosporium superimposed upon asthma, chronic bronchitis, and bronchiectasis.

A case in which sarcoidosis presented as dysphagia caused by compression of the esophagus is reported. In addition, four previously reported cases of sarcoidosis involving the esophagus are summarized. Three of the four previously reported cases presented as dysphagia and one presented as esophageal pain without dysphagia. The present case is reported because it is unusual for sarcoidosis with bilateral hilar adenopathy to compress the esophagus and to present as dysphagia. A case in which sarcoidosis presented as dysphagia caused by compression of the esophagus is reported. In addition, four previously reported cases of sarcoidosis involving the esophagus are summarized. Three of the four previously reported cases presented as dysphagia and one presented as esophageal pain without dysphagia. The present case is reported because it is unusual for sarcoidosis with bilateral hilar adenopathy to compress the esophagus and to present as dysphagia.

Sections of Medicine, Experimental and Anatomic Pathology, and Anesthesiology, Mayo Clinic, Rochester, Minnesota

Nonrandomized data exploring pancreas stereotactic body radiation therapy (SBRT) has demonstrated excellent local control rates and low toxicity. Before commencing a randomized trial investigating pancreas SBRT, standardization of prescription dose, dose constraints, simulation technique, and clinical target volume delineation are required.Specialists in radiation oncology, medical oncology, hepatobiliary surgery, and gastroenterology attended 2 consecutive Australasian Gastrointestinal Trials Group workshops in 2017 and 2018. Sample cases were discussed during workshop contact with specifically invited international speakers highly experienced in pancreas SBRT. Furthermore, sample cases were contoured and planned between workshop contact to finalize dose constraints and clinical target volume delineation.Over 2 separate workshops, consensus was reached on dose and simulation technique. The working group recommended a dose prescription of 40 Gy in 5 fractions. Treatment delivery during end-expiratory breath hold with triple-phase contrast enhanced computed tomography was recommended. In addition, dose constraints, stepwise contouring guidelines, and an anatomic atlas for pancreatic SBRT were developed.Pancreas SBRT is emerging as a promising treatment modality requiring prospective evaluation in randomized studies. This work attempts to standardize dose, simulation technique, and volume delineation to support the delivery of high quality SBRT in a multicenter study.