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Primary vascular tumors of the lung are rare. 

                          Image of Kaposi's  sarcoma

Kaposi's sarcoma is usually seen in patients with HIV infection.

        Visit: Kaposi's sarcoma of the Soft Tissue

Pulmonary Kaposi's sarcoma in patients with acquired immune deficiency syndrome: a clinicopathological study. Thorax. 1987 Apr;42(4):262-8.

Pulmonary Kaposi's sarcoma may contribute to respiratory dysfunction in patients with acquired immune deficiency syndrome (AIDS) and features of pneumonitis. Opportunistic infections are readily recognised in endoscopic material, but pulmonary Kaposi's sarcoma is easily missed, so that patients are deprived of specific treatment. The clinical and pathological findings from nine cases of pulmonary Kaposi's sarcoma have been reviewed; these were found among 84 patients with AIDS and pneumonitis undergoing fibreoptic bronchoscopy and bronchoalveolar lavage. Diagnosis was established before death in eight patients (in five by bronchial biopsy and in three by open lung biopsy). Examination of lavage fluid showed alveolar haemorrhage in six patients. It is concluded that: (1) fibreoptic bronchoscopy may be useful in the diagnosis of endobronchial lesions of Kaposi's sarcoma; (2) alveolar haemorrhage in patients with AIDS is suggestive of pulmonary Kaposi's sarcoma. Factors that may cause difficulties in diagnosis include the focal nature of some lesions and the pleural or parenchymatous location of others. In addition, in the lung as in the skin, the early stages of Kaposi's sarcoma resemble granulation tissue. Such lesions are far more difficult to recognise than is the late nodular stage.

                          Image of Angiosarcoma

Primary pulmonary angiosarcoma is a very rare tumour. It usually occurs as a metastatic tumour from a cardiac primary or some other distant primary.

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Primary pulmonary angiosarcoma.Rev Mal Respir. 2007 Jan;24(1):73-6.

INTRODUCTION: Primary pulmonary angiosarcoma (PPA) is a very rare tumour with a grave and short prognosis and whose primary site is difficult to confirm. The diagnosis is essentially histological and immunohistochemical because there are no specific clinical or radiological signs. OBSERVATION: We report a case of a man with PPA, discovered during the investigation of chest pain. Imaging (x-ray and CT) identified a right apical mass. The diagnosis was made by histological and immunohistochemical analysis of CT guided percutaneous biopsies. CONCLUSION: As a result of this observation we propose further studies on this extremely rare and grave condition.

Primary pulmonary angiosarcoma.Rev Pneumol Clin. 2005 Apr;61(2):115-8

Pulmonary angiosarcoma is an uncommon vascular and usually secondary tumor. Only a few primary cases of pulmonary angiosarcoma have been described. We report a case of primary pulmonary angiosarcoma in an adult man who presented with hemoptysis. Chest x-ray film and chest CT showed a right para-cardiac opacity associated with diffuse alveolar consolidation of the right basal lobe. Right inferior lobectomy has been performed. Pulmonary angiosarcoma was diagnosed on histological and immuno-histochemical studies of the operative specimen. The primitive character was retained after ruling out all other tumor localizations. The clinical outcome was rapidly fatal. This observation is added to the other rare cases published of primitive pulmonary angiosarcoma. It confirms the poor prognosis and the extremely hemorrhagic nature of this tumor. Isolated necrotic parenchymatous mass was an original radiological pattern observed in this patient.

Primary angiosarcoma of the lung and pleura. Monaldi Arch Chest Dis. 2004 Oct-Dec;61(4):234-6.

A 46 year old male smoker was admitted for severe continuing hemoptysis. Chest-X-rays and chest computed tomography revealed nodular infiltrates and bilateral hemothorax. Fiberoptic bronchoscopy resulted to non-diagnostic cytological and microbiological findings. Open lung and pleural biopsies after right thoracotomy revealed epithelioid angiosarcoma and further staging assessment showed secondary brain and liver foci. The patient received several courses of chemotherapy but he died one month later. The clinical, radiological, pathological, histochemical and therapeutic aspects of the disease are discussed. Angiosarcoma, a rare tumour with poor prognosis should be taken into consideration in the differential diagnosis of hemoptysis.

Primary angiosarcoma of the lung. Ann Diagn Pathol. 2005;9(5):302-4.

Primary angiosarcoma of the lung is a rare disorder with few cases reported in the literature [Patel AM, Ryu JH. Angiosarcoma in the lung. Chest 1993;103:1531-35]. We present a case of primary angiosarcoma of the lung in a 79-year-old woman. Preoperative computed tomographic scan revealed soft tissue nodules surrounded by a halo of ground-glass attenuation, an appearance consistent with hemorrhagic pulmonary nodules [Primack S, Hartman T, Lee KS. Pulmonary nodules and the CT halo sign. Radiology 1994;190:513-15]. Lung biopsy revealed a multifocal malignant neoplasm with areas of hemorrhage. The histologic, cytologic, and immunophenotypic features were characteristic of epithelioid angiosarcoma. This case report contributes to the sparse literature on this disease and provides computed tomographic and pathologic correlation in a patient with hemorrhagic pulmonary nodules.

Metastatic angiosarcoma masquerading as diffuse pulmonary hemorrhage: clinicopathologic analysis of 7 new patients.Arch Pathol Lab Med. 2001 Dec;125(12):1562-5

CONTEXT: Diffuse pulmonary hemorrhage is an uncommon presenting manifestation of angiosarcoma. OBJECTIVE: To review the clinical, radiologic, and pathologic findings of patients with metastatic angiosarcoma who presented with diffuse pulmonary hemorrhage. DESIGN: Patients fulfilling inclusion criteria were identified from the consultation files. Clinical and radiologic data were obtained from referring pathologists. Histologic slides were reviewed in all patients. RESULTS: Our patients included 6 men and 1 woman, aged 31 to 73 years; 4 patients were younger than 40 years. Six patients presented with hemoptysis, and all had diffuse abnormalities on radiographic studies. Clinical considerations prior to biopsy included pulmonary hemorrhage syndrome (n = 2), acute respiratory failure (n = 1), and infection (n = 1). Metastatic disease was included in the differential diagnosis in only 1 patient. None had a diagnosis of malignancy prior to lung biopsy. All biopsies showed hemorrhage associated with atypical epithelioid and spindle cells, forming anastomosing vascular channels, distributed along and within lymphatics and arteries. Neoplastic cells were immunoreactive for factor VIII-related protein or CD31 in all cases for which special stains were available. Three patients with complete follow-up died of their disease. Primary sites were discovered in all 3 patients who underwent autopsy examination. Two tumors arose in the heart and 1 in the pelvic soft tissues. One additional patient had a likely primary site identified in the right atrium by cardiac ultrasound and was subsequently lost to follow-up. CONCLUSION: Angiosarcoma should be included in the differential diagnosis of diffuse pulmonary hemorrhage, especially in young adults.

                  

Pulmonary Kaposi sarcoma in the era of highly active antiretroviral therapy. HIV Med. 2006 Jul;7(5):291-3.                  

OBJECTIVE: Since the introduction of highly active antiretroviral therapy (HAART) there has been a dramatic reduction in the incidence of Kaposi sarcoma (KS) and an improvement in survival. We wished to examine whether the outcome in pulmonary KS (pKS) has also altered. METHODS: In a single-institution cohort of 1140 HIV-positive patients with KS, 305 patients were diagnosed in the HAART era (1996-2004). We examined the clinicopathological features and outcomes of these patients, of whom 25 had pKS and 280 did not. RESULTS: Patients with pKS had lower CD4 cell counts at the time of KS diagnosis (Mann-Whitney U-test P=0.005). The incidence of pKS was higher in African patients than in non-African patients in this sample (Fisher's test, P=0.001). There were no significant differences in age, gender, plasma HIV-1 viral load or prior HAART treatment at the time of KS diagnosis. Five-year overall survival in the pKS group was 49% [95% confidence interval (CI) 26-73%] as compared with 82% (95% CI 76-87%) for the non-pKS group (log rank, P<0.0001). CONCLUSION: PKS remains an ominous diagnosis in the era of HAART, with a median survival of just 1.6 years.

Pulmonary Kaposi's sarcoma in pregnancy.Am J Perinatol. 2004 Aug; 21(6):355-63.

Kaposi's sarcoma in human immunodeficiency virus (HIV) -infected women, often misdiagnosed, has an aggressive clinical course, with high rates of visceral involvement and decreased survival. We describe the first case of isolated pulmonary Kaposi's sarcoma in pregnancy. A nulliparous woman was diagnosed with AIDS after presenting at 25 weeks gestation with a cough and multiple pulmonary nodules. Extensive pulmonary evaluation was nondiagnostic until thorascopic lung biopsy revealed Kaposi's sarcoma. Despite combination antiretroviral therapy, her malignancy progressed. Labor was induced at 33.5 weeks gestation for nonreassuring fetal testing. She received chemotherapy postpartum and remains in remission. Pulmonary Kaposi's sarcoma should be considered in the differential diagnosis of HIV-infected obstetric patients with respiratory compromise. Definitive diagnosis is necessary given the aggressive clinical course that is potentially responsive to therapy.

Pulmonary Kaposi's sarcoma revealed by a solitary nodule in a patient with acquired immunodeficiency syndrome. Am J Respir Crit Care Med. 1994 Apr;149(4 Pt 1):1041-3

Kaposi's sarcoma is very common in patients with AIDS. Usually, skin lesions are associated with various visceral involvements. A homosexual patient with AIDS presented with cough and dyspnea, which were followed months later by hemoptysis. He had no skin lesions or endobronchial Kaposi's sarcoma at any time. His chest radiograph showed only an irregular solitary nodule. It exhibited very slow development over time. Surgery was performed, and this solitary nodule proved to be pulmonary Kaposi's sarcoma. Pulmonary Kaposi was the sole manifestation of this associated AIDS sarcoma. This very unusual case report of pulmonary Kaposi sarcoma indicates that this diagnosis should be considered in patients with AIDS presenting with a solitary pulmonary nodule.

Bronchopulmonary Kaposi's sarcoma in patients with AIDS.Thorax. 1992 Sep;47(9):721-5

BACKGROUND: Kaposi's sarcoma in HIV antibody positive patients may affect the lungs. This study describes the presentation, chest radiographic appearances, and pulmonary function test abnormalities in patients with AIDS who had tracheobronchial Kaposi's sarcoma. METHODS AND RESULTS: Twenty nine (8%) of 361 consecutive HIV antibody positive patients undergoing bronchoscopy for respiratory symptoms had tracheobronchial Kaposi's sarcoma. Eight patients had intercurrent infections and one had previously received chemotherapy for cutaneous Kaposi's sarcoma; these patients were excluded. Seven of the remaining 20 patients had localised Kaposi's sarcoma (lesions confined to the trachea or the subsegments of one lobe) and 13 had widespread Kaposi's sarcoma (affecting the trachea and one lobe or the subsegments of more than one lobe); 19 patients also had cutaneous and palatal Kaposi's sarcoma. Seven patients, four with widespread disease, had a normal radiograph. All patients had reduced transfer factor (TLCO) and transfer coefficient (KCO) but only those with widespread disease had reductions in forced expiratory volume in one second (FEV1), forced vital capacity (FVC), and peak expiratory flow (PEF). Follow up pulmonary function testing in seven patients (median three months later) showed further reductions in TLCO. All four patients who received no treatment had progressive radiographic abnormalities; bronchoscopy in two patients showed progressive tracheobronchial disease, and two patients had further reductions in FEV1 and FVC. In three patients treated with chemotherapy palliation of symptoms was achieved but two had further reductions in FEV1 and FVC and the radiograph deteriorated. Bronchoscopy showed regression of disease in only one patient. CONCLUSION: Pulmonary Kaposi's sarcoma produces abnormalities of TLCO even in patients with localised disease; airflow obstruction may occur in patients with widespread disease. Bronchoscopic reassessment of the extent of disease may not accurately reflect response to chemotherapy.

Pulmonary Kaposi's sarcoma. Premortem histologic diagnosis. Am J Surg Pathol. 1986 May;10(5):301-11

Nine open lung biopsies and nine transbronchial biopsies from 10 patients with pulmonary Kaposi's sarcoma were reviewed to define the pattern of involvement in the lung by Kaposi's sarcoma and to determine the usefulness of transbronchial biopsy in making the diagnosis. There were nine patients with acquired immune deficiency syndrome (AIDS) and one patient with sporadic pulmonary Kaposi's sarcoma. A lymphatic distribution was seen in all cases. A spectrum ranging from distinctive polymorphous cellular infiltrates ultimately interpreted as Kaposi's sarcoma to "classic" Kaposi's sarcoma was found. Recognition of the former enabled retrospective recognition of Kaposi's sarcoma in four of eight transbronchial bronchial biopsies. The diagnosis of pulmonary Kaposi's sarcoma in one other patient was made solely on the basis of transbronchial biopsy. Eight patients died from pulmonary Kaposi's sarcoma; two patients are alive with extensive pulmonary Kaposi's at last follow-up. We believe that transbronchial biopsy may be useful in establishing a diagnosis of pulmonary Kaposi's sarcoma in many more patients than is generally appreciated.

Pulmonary Kaposi's sarcoma in the acquired immune deficiency syndrome. Clinical, radiographic, and pathologic manifestations. Am J Med. 1986 Jul;81(1):11-8

Pulmonary Kaposi's sarcoma related to the acquired immune deficiency syndrome (AIDS) has not been well characterized. To define the clinical, radiographic, and pathologic features of this entity, 11 autopsy-proved cases of pulmonary Kaposi's sarcoma were reviewed. The most common clinical symptoms were dyspnea and cough, but hemoptysis and stridor were also found. Nodular infiltrates and pleural effusions were the most commonly found radiographic abnormalities. Pulmonary function tests were sensitive in detecting the pulmonary abnormalities due to Kaposi's sarcoma. A low diffusion capacity, lack of arterial desaturation with exercise, and obstruction to airflow were suggestive of pulmonary involvement with this malignancy. Although endobronchial Kaposi's sarcoma was visualized at bronchoscopy as cherry-red, slightly raised lesions, bronchial biopsy specimens always showed no abnormalities. Transbronchial brushings and biopsy specimens and analysis of pleural fluid were also not helpful in establishing a diagnosis. In the seven subjects with extensive parenchymal Kaposi's sarcoma at autopsy, the pleura was always involved. Eight subjects had involvement of the tracheobronchial tree. In all of the subjects, pulmonary Kaposi's sarcoma was a significant cause of morbidity, and in three of 11 subjects (27 percent) it was the direct cause of death.

 
 November  2009 
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