Fibrosarcoma of the Pulmonary Artery, Masquerading as a Pheochromocytoma

Wolf, Paul L.; Dickenman, R. C.; Langston, John D.

doi:10.1093/ajcp/34.2.146

Cited by 31 publications

(11 citation statements)

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“…This specific paraneoplastic syndrome with predominant malaise and anaemia has not, to our knowledge, been described in this context previously. Only one patient has been reported with a paraneoplastic picture similar to the clinical presentation of a pheochromocytoma [6]. The peripheral location of the tumour in our patient is rather exceptional and contributed to the uncomplicated surgery and the ongoing favourable outcome after 20 months of follow-up.…”

Section: Discussionsupporting

confidence: 50%

Rapidly growing intrathoracic mass with paraneoplastic syndrome

Lehmann¹,

Solèr²,

Bubendorf³

et al. 1996

European Respiratory Journal

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A 56 year old nonsmoker presented with a 6 month history of dry cough, increasing malaise, night sweats, loss of appetite and weight loss of 10 kg. His past medical history was unremarkable. The patient was in a clearly reduced general state, his temperature was 37.5°C, blood pressure 120/80 mmHg and heart rate 78 beats·min -1 . Physical examination including cardiopulmonary and neurological systems was normal.Laboratory analyses demonstrated an anaemia with a haemoglobin level of 8.5 g·dL -1 but with normal erythrocyte indices, a normal differential white blood cell count but a thrombocytosis of 500×10 9 platelets·L -1 . Anaemia quickly recurred after transfusion of 2 units of blood. Erythrocyte sedimentation rate (ESR) (110 mm·h -1 ) and C-reactive protein (CRP) (134 mg·L -1 ; normal value <10 mg·L -1 ) were elevated. Liver function tests showed significant elevations of gamma-glutamyltransferase (172 U·L -1 ; normal value 8-66 U·L -1 ) and of alkaline phosphatase (674 U·L -1 ; normal value 31-108 U·L -1 ). Other laboratory tests, including urine, three stool specimens for occult blood, parameters of haemolysis, vasculitis and tumour markers (carcinoembryonic antigen (CEA), neuron-specific enolase (NSE), β 2 -microglobulin and markers for germ cell tumours) were negative, as was a tuberculin skin test. Results of pulmonary function tests were also normal.Chest radiograph and computed tomography (CT) scan of the thorax are shown in figures 1 and 2.On fibreoptic bronchoscopy, no endobronchial pathology was seen. Cytological analyses of bronchial washings and transbronchial needle aspiration material from the middle lobe segmental carina showed no evidence of malignancy. A search for metastases, including CT-scan of the brain and bone scintigraphy was negative. As it seemed unlikely that the small hilar mass was responsible for the clinical symptoms and the anaemia, which rather suggested a systemic disease, we first ruled out occult bleeding, infection or a lymphoma. Endoscopy of the upper gastrointestinal tract, colonoscopy, bone marrow biopsy and aspiration, CT-scan of the abdomen and scintigraphy of the bone marrow were normal.A second bronchoscopy and transbronchial needle cytology in the middle lobe showed a few malignant cells, which could not be further classified. In order to proceed to a percutaneous, CT-guided needle-biopsy, another CTscan of the thorax was performed, 4 weeks after the first one ( fig. 3). Tru-cut biopsy (with a 18-gauge needle) revealed an undifferentiated large-cell tumour with necrotic areas.Immunohistochemically, the tumour was positive for the soft tissue marker vimentin but not for the epithelial marker LU-5. Three days later, a thoracotomy was performed. A central tumour in the middle and lower lobe was found, in the bifurcation between the middle and lower lobe arteries. A right pneumonectomy was performed. Pathology findings are given in figures 4-6.

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Section: Discussionsupporting

confidence: 50%

Rapidly growing intrathoracic mass with paraneoplastic syndrome

Lehmann¹,

Solèr²,

Bubendorf³

et al. 1996

European Respiratory Journal

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“…In the case of Elphinstone and Spector (1959) the pericardial sac contained 200 ml of straw-coloured fluid, which was not diagnosed before death. Extravascular extension of the tumour has been reported several times (van der Linde, 1942;Lowell and Tuhy, 1949;Jacques and Barclay, 1960;Wolf, Dickenman, and Langston, 1960).…”

Section: Discussionmentioning

confidence: 99%

“…However, a few cases with a different clinical picture were described. Wolf et al (1960) reported a patient with a fibrosarcoma of the pulmonary artery with symptoms suggesting a phaeochromocytomaattacks of palpitations, sweating, and hypertension. Another unusual clinical history was reported by Green, Crevasse, and Shanklin (1964).…”

Section: Discussionmentioning

confidence: 99%

Leiomyosarcoma of the pulmonary trunk associated with pericardial effusion

Thijs

Kroon

Leeuwen

1974

Thorax

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Thijs, L. G., Kroon, T. A. J., and van Leeuwen, Th. M. (1974). Thorax, 29,[490][491][492][493][494]. Leiomyosarcoma of the pulmonary trunk associated with pericardial effusion. The case history of a patient with a leiomyosarcoma of the pulmonary trunk is presented. The clinical picture was dominated by unexplained shortness of breath, episodes of cough and haemoptysis, general weakness, and weight loss. Signs of a pericardial effusion and pulmonary stenosis developed a few weeks before death. Thoracotomy revealed an inoperable tumour arising from the pulmonary trunk and a large pericardial effusion. Microscopy of the tumour removed at necropsy showed a leiomyosarcoma.Primary sarcomata of the pulmonary trunk are rare. In 1969 Wackers, van der Schoot, and Hampe collected 21 cases from the literature, including their own case, and presented a review of the reported case histories. McConnell (1970) called attention to primary neoplasms of the heart and great vessels containing bony and cartilaginous elements. He found eight patients described in the literature, three of whom had tumours located in the pulmonary artery, and reported another patient with an osteosarcoma of the pulmonary artery arising from the area of the pulmonary valves.The patient to be presented here had a leiomyosarcoma of the pulmonary trunk complicated by a pericardial effusion. CASE REPORTA 48-year-old white woman visited the outpatients' department on 31 August 1971 because of a cough of two months' duration, slight shortage of breath, tiredness, palpitations, and weight loss. A week before the first examination she had developed fever with pain in the right chest and haemoptysis. Abnormal physical findings were confined to the respiratory system and consisted of an impaired percussion note and weak breath sounds at the right lung base. A chest radiograph showed a few infiltrative lesions and a small pleural effusion at the right base.A diagnosis of bronchopneumonia associated with 'Present address:

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“…The tumor may be localized on the valves [10,25] or extends into the outflow tract or the right ventricle [13], The usual pattern of growth of this malignant process is characterized by the invasion of the intima with a localized penetration of the media and the surrounding structures [4,36], Dis tance propagation is due to fragmentation and emboli to the branches of the pulmon ary artery [39], Although hematogenous spread is uncommon, distant metastases have been discovered in about 50°/o of pa tients and were found in the lungs [15,29], pleura, pericardium and mediastinum [36] [38] and disseminated intravascular coa gulopathy. It is assumed that tumor parti cles, rich in tissue thromboplastin, enter the pulmonary circulation and initiate intravas cular coagulation in the pulmonary vessels [36].…”

Section: Discussionmentioning

confidence: 99%

“…In the differential diagnosis of primary leiomyosarcoma of the pulmonary artery, the following possibilities should be consid ered: massive pulmonary artery emboli with chronic cor pulmonale [11,17] [39] reported a pa tient with fibrosarcoma of the pulmonary artery masquerading as a pheochromocytoma, due to the secretion of an epinephrineor norepinephrine-like substance secreted by the tumor.…”

Section: Discussionmentioning

confidence: 99%