Pulmonary Artery Sarcoma Mimicking Pulmonary Embolism

Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease.The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival.     

Pulmonary Artery Sarcoma Masquerading as Chronic Pulmonary Thromboembolism

We describe the case of a 60-year-old woman who presented with pulmonary artery sarcoma, a very rare tumor of the cardiovascular system. Her tumor was initially misdiagnosed as chronic pulmonary thromboembolism, and she underwent pulmonary endarterectomy.Early diagnosis of primary pulmonary artery sarcoma is crucial. That alternative should always be considered before settling on a diagnosis of pulmonary embolism. Suspicion should be aroused by the failure of anticoagulant treatment to alleviate pulmonary perfusion abnormalities and systemic symptoms. Surgical resection of the tumor—preferably by pulmonary endarterectomy, followed by reconstruction as needed—is currently the most promising treatment for pulmonary artery sarcoma.     

Pulmonary blastomycosis

Blastomycosis is a rare but important fungal infection diagnosed primarily in the south central and midwestern United States but also in the American and Canadian borders of the Great Lakes. Epidemics of infection related to point-source exposure include patients of all ages and both sexes, but endemic cases are usually in young to middle-aged adults, with more men than women reported. Pneumonia is the most common manifestation and the lung is almost always the organ initially infected. The lung manifestations range from illness that mimics acute bacterial pneumonia to chronic, destructive lung disease appearing like tuberculosis or lung cancer. Extrapulmonary disease can occur with or without concomitant lung disease. In descending order, cutaneous, osseous, prostatic, and central nervous system involvements are the most frequent manifestations of extrapulmonary blastomycosis. Amphotericin B is curative, but, because of toxicity, oral azole agents have replaced amphotericin B as therapy for less than overwhelming blastomycosis. Itraconazole is now considered to be the agent of choice with fluconazole, voriconazole, and posaconazole having a role in selected patients. In a patient with life-threatening or central nervous system blastomycosis amphotericin B should be given, at least initially.     

Pulmonary endometriosis

Pulmonary endometriosis is manifested as either asymptomatic pulmonary nodules or as pneumothorax, hemothorax, or hemoptysis during menses. We review 84 cases of pulmonary endometriosis in the English literature and report 3 additional patients. One of our patients is the first reported to have hemoneumothorax. Catamenial pneumothorax usually involved the right chest, and occurred in young nulliparous women without pelvic endometrosis. Pleuroscopy, laparoscopy with pneumoperitoneum, and thoracotomy produced a tissue diagnosis infrequently. Hormonal suppression of ovulation and pleurodesis usually corrected this disorder. Catamenial hemothorax only affected the right chest, but occurred in older multiparous women with pelvic endometriosis. While thoracotomy or laparotomy produced a tissue diagnosis, these procedures were not curative. In contrast, our patient with this disorder was treated successfully with pleurectomy. Catamenial hemoptysis occurred in multiparous women without pelvic endometriosis. Bronchoscopy localized bleeding but never produced a tissue diagnosis. Thoracotomy produced endometrial tissue. Endometrial pulmonary nodules require a diagnosis but do not otherwise produce problems.     

Pulmonary capillaritis

Vasculitis, inflammation, and necrosis of blood vessels can involve any size or type of vessel in the pulmonary vasculature, including the capillaries, so-called capillaritis. Although pulmonary capillaritis is a histopathologic diagnosis that is not pathognomonic of a specific disorder, it usually signals the presence of an underlying systemic vasculitis or collagen vascular disease. Patients with pulmonary capillaritis usually present with bilateral infiltrates on chest radiographs and can be acutely ill with diffuse alveolar hemorrhage that may be life threatening. Therapy depends on diagnosis of the underlying disease that gave rise to the capillaritis. Since many of the disorders leading to capillaritis are treated by immunosuppression with corticosteroids and cyclophosphamide or azathioprine, infection must be excluded early in the course of therapy.     

Pulmonary aspiration

In addition to acute respiratory failure associated with chronic obstructive lung disease the most serious and frequent problems encountered in an intensive care setting are those of pulmonary aspiration. The young patient with drug abuse, the elderly shock patient with pulmonary aspiration are now seen with increasing frequency. Young persons with drug abuse who have been burned present a new and serious challenge to the various medical teams who must take care of them. The special acute respiratory failure due to inhalation of hot smoke is often fatal and usually unsuspected. The common denominator of such types of acute respiratory failure is aspiration into the lungs of foreign material—be it food, solid objects, gastric contents or hot smoke. In this review of pulmonary aspiration we have included a short review of the problems of drowning.     

Pulmonary cryptococcosis

Cryptococcosis is a common opportunistic infection in acquired immunodeficiency syndrome (AIDS) patients, also occurring in other immunosuppressed patients and occasionally those with no apparent immunocompromise. The majority of cases are caused by the ubiquitous encapsulated yeast, Cryptococcus neoformans, whereas Cryptococcus GATTII accounts for a smaller proportion of cases, often in immunocompetent patients. Severe meningoencephalitis is the commonest presentation; however, pulmonary cryptococcosis in human immunodeficiency virus (HIV)-seropositive individuals is underdiagnosed and without appropriate treatment leads to severe disseminated disease. The natural history of pulmonary cryptococcal infection in other immunosuppressed patients is also of dissemination and progression in the majority of cases, whereas immunocompetent patients may present with more localized, self-limiting disease. The presentation is usually with nonspecific respiratory symptoms, although severe respiratory failure has been reported in both immunocompromised and immunocompetent patients. Radiological presentations are varied and nonspecific, influenced by the underlying immune status of the patient. Diagnosis is based on isolation of Cryptococcus from, or detection of cryptococcal antigen in, a pulmonary specimen, coupled with appropriate clinical, radiological, and histopathological findings. Antifungal treatment with amphotericin B +/- flucytosine is recommended for severe disease, whereas fluconazole is the treatment of choice for mild and localized infections.     

Pulmonary telangiectasia

Two patients with pulmonary telangiectasia are described. One of these is by far the oldest yet reported. The diagnosis and prognosis of this condition are discussed.