Pulmonary vein leiomyosarcoma extending into the left atrium

In this paper, the authors report the case of a 28-year-old man with pulmonary vein leiomyosarcoma presenting subacute respiratory distress. Thoracic computed tomography and transoesophagal ultrasonographic examination of the heart suggested the diagnosis of a heart tumour revealed by the obstruction of the mitral valve and pulmonary oedema. Emergency cardiac surgery revealed the mass to be a leiomyosarcoma, probably extending from the right inferior pulmonary vein and extending into the left atrium. The clinical evolution was complicated because of a sudden local relapse. The patient underwent a second cardiac intervention involving lower right lobectomy followed by adjuvant chemotherapy with an ifosfamide-adriamycin combination. This treatment failed to control the disease and a third cardiac intervention was necessary with second-line gemcitabine-paclitaxel adjuvant chemotherapy. Further recurrences were observed with metastases first in the scalp and then in the spine and adrenal glands leading to the death of the patient 2 years after the diagnosis.     

Pulmonary arterial hypertension in women

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a rare condition characterized by sustained elevation in pulmonary arterial resistance leading to right heart failure. BACKGROUND: PAH afflicts predominantly women. Echocardiography is the initial investigation of choice for non-invasive detection of PAH but right-heart catheterization is necessary to confirm the diagnosis. Conventional treatment includes non-specific drugs (warfarin, diuretics, oxygen). The endothelin-1 receptor antagonist bosentan, the phosphodiesterase-5 inhibitor sildenafil, and prostanoids have been shown to improve symptoms, exercise capacity and haemodynamics. Intravenous prostacyclin is the first-line treatment for the most severely affected patients. Despite the most modern treatment the overall mortality rate of pregnant women with severe PAH remains high. Therefore, pregnancy is contraindicated in women with PAH and an effective method of contraception is recommended in women of childbearing age. Therapeutic abortion should be offered, particularly when early deterioration occurs. If this option is not accepted, intravenous prostacyclin should be considered promptly. VIEWPOINTS AND CONCLUSION: Recent advances in the management of PAH have markedly improved prognosis and have resulted in more women of childbearing age considering pregnancy. A multidisciplinary approach should give new insights into cardiopulmonary, obstetric and anaesthetic management during pregnancy, delivery and the post-partum period.     

Early postoperative management and immunosuppressive treatment following lung transplantation

Lung transplantation is now considered a valid option in the management of end-stage respiratory failure. The postoperative period remains a key stage that will influence the average long-term prognosis of the patients. Primary graft failure, postoperative bleeding, infection, acute rejection and complications linked to the surgery, and to vascular or bronchial anastomoses, are risk factors for mortality and morbidity. These must be taken care of quickly via collaboration with the surgical team. The immunosuppressive treatment essential for tolerance induction with regard to the transplanted organ will be introduced during the intraoperative period and continued for life. The combination of a calcineurin inhibitor, an antiproliferative agent and corticosteroids remains the conventional procedure. The role of new molecules as mTor inhibitors remains to be determined.     

Pulmonary epithelioid haemangioendothelioma: two different clinical courses

The first case report concerns a 59-year-old man presenting a chronic cough and the second a 23-year-old woman farmer presenting with worsening dyspnoea associated with cough, expectoration and haemoptysis. In the second case, the woman died 7 months after the onset of the respiratory symptoms. In both cases, chest radiography and thoracic CT scans showed multiple, bilateral pulmonary nodules, bronchial endoscopy was normal and surgical biopsy revealed epithelioid haemangioendothelioma. It is a rare primary pulmonary tumour which is usually found in soft tissue, bone or liver. Epithelioid haemangioendothelioma is a vascular tumour usually affecting women less than 40 years of age. The histological appearance is characteristic and may be confirmed by immunohistochemistry. Chemotherapy and radiotherapy are generally ineffective and surgery is sometimes impossible because of the multifocal lesions. Epithelioid haemangioendothelioma is considered a sarcoma without precise histological prognostic criteria. In its pulmonary location it is responsible for death due to respiratory failure in 50% of cases.     

Intrathoracic lymphadenopathy: an unusual manifestation of rheumatoid arthritis

Lung disease is the most frequent extra-articular manifestation of rheumatoid arthritis. It is detected in nearly 50% of patients with this multisystem affection, his knowledge has benefited from advances in computed tomography (CT). The inflammation can affect the pleura, the airways and the lung parenchyma. Intrathoracic lymphadenopathy complicating rheumatoid lung are not usual, and then pose the problem of differential diagnosis. We report a 51-year-old man, with a history of tobacco intoxication, suffering from rheumatoid arthritis who developed an interstitial lung disease at stage of fibrosis with mediastinal and hilar adenopathy. We will discuss the clinical, paraclinical, evolutionary and therapeutic particularities case.     

Broncholithiasis: six cases

Broncholithiasis is an exceptional condition characterized by the presence of stony formations in the bronchial lumen. We report six cases. Mean age was 41 years. Revealing signs were hemoptysis (n=5), cough (n=5), fever (n=1) and recurrent lower respiratory tract infections (n=1). Physical examination found sonorous rales in two patients and was normal in four. The chest x-ray showed a parenchymal opacity suggestive of calcification in one patient, atelectasia in two, and alveolar images in three. Bronchial endoscopy demonstrated broncholithiasis in one patient, an endobronchial blood clot in one patient with abundant hemoptysis, an endoluminal bud simulating a tumor in two, an inflammatory aspect in one, and was normal in one. Thoracic computed tomography demonstrated broncholithiasis in three patients. Treatment consisted in lobectomy in five patients. The pathology specimen confirmed broncholithiasis in all five and in one revealed caseofollicular lesions of the hillar nodes. Anti-tuberculosis treatment was prescribed for this patient. Therapeutic abstention with regular surveillance was chosen for one patient with an uncomplicated broncholithiasis. Broncholithiasis is an exceptional condition with potentially serious consequences. Certain diagnosis is based on high-resolution computed tomography and endoscopic findings but can nevertheless be a surgical discovery.     

Blast lung injuries

In armed conflicts and during terrorist attacks, explosive devices are a major cause of mortality. The lung is one of the organs most sensitive to blasts. Thus, today it is important that every GP at least knows the basics and practices regarding treatment of blast victims. We suggest, following a review of the explosions and an assessment of the current threats, detailing the lung injuries brought about by the explosions and the main treatments currently recommended.     

Validation of a diagnostic algorithm in non severe pulmonary embolism at the Annecy general hospital. D-dimers,venous lower limb ultra-sound and spiral CT scan

OBJECTIVE AND METHOD: We have evaluated the sensitivity of a diagnostic algorithm for all patients suspected of pulmonary embolism using: D-Dimer, lower limb venous ultrasonography and helical computed tomography. To validate this approach, a lung scan is systematically carried out if the pulmonary embolism diagnosis is not withheld as a result of the algorithm. Clinical tests are organised between the 3rd and 6th month. RESULTS: Two hundred patients were involved between January 1998 and October 1999. One hundred and six pulmonary embolisms were diagnosed. Out of the 200 ultrasonography tests carried out we found: 71 proximal deep-vein thrombosis (popliteal or supra-popliteal), 33 distal thrombosis (infra-popliteal). Ninety-two cases were negative (4 tests non conclusive). We have deduced that a deep-vein thrombosis permits the diagnosis of thrombo-embolic illness without any further diagnostic approach (no computed tomography). Out of the 129 computed tomographies carried out we found: 35 pulmonary embolisms and 23 other diagnoses. Seventy-one lung scans were therefore carried out. We recorded 7 discordances (scans showed high and very high probability for pulmonary embolism whilst computed tomographies did not): pulmonary angiography was negative 4 times and diagnosed pulmonary embolism once and two patients refused to take the test (Table 2). There are two recurrences in the follow-up: proximal thrombosis and a pulmonary embolism. This involved two patients who had refused to undergo pulmonary angiography. CONCLUSION: This diagnostic approach therefore seems satisfactory but would require further investigation on a wider scale.