[Pulmonary lymphangioleiomyomatosis in a menopaused woman]. / Lymphangioléiomyomatose pulmonaire chez une femme ménopausée.

We report an unusual case of pulmonary lymphangioleiomyomatosis in a menopaused woman who had been taking estrogen hormone replacement therapy for several years. The characteristic feature of this uncommon disease is a proliferation of non-tumoral abnormal smooth muscle cells within the alveolar walls, and around the bronchi, lymph nodes and blood vessels. About twenty cases of pulmonary lymphangioleiomyomatosis have been described in menopaused women, who generally were taking estrogen hormone replacement therapy. This subpopulation does not appear to present any particular clinical, functional or radiographic features.

[Primary malignant mediastinal melanoma. Apropos of a case]. / Mélanome malin primitif médiastinal. A propos d'un cas.

We report the case of a 57-year-old patient with primary malignant melanoma of the mediastinum who survived nine months despite immunotherapy, radiotherapy and chemotherapy. The primary nature of the intrathoracic melanoma was difficult to prove. Definitive diagnosis was based on the uniform morphology of the melanoma which showed junctional anomalies at the histology examination with tracheo-bronchial and esophageal localizations as well as on the absence of other patent or formerly resected melanocyte-rich localizations (skin, mucosa, ocular) at clinical examination and autopsy.

Primary mediastinal germ cell tumors. Results of a French retrospective study.

Eighty-seven patients with primary mediastinal germ cell tumors treated between 1983 and 1990 were studied. Among the 23 patients classified as pure seminoma, eight (35 percent) underwent surgery followed by radiotherapy (n = 6), radiotherapy and/or chemotherapy (n = 2); two patients underwent radiotherapy; 13 patients (57 percent) underwent induction cisplatin-based chemotherapy (ten complete responses) followed by radiotherapy (n = 9), second line chemotherapy (n = 2) and surgical resection of residual tumor (n = 2). On completion of treatment, 22 patients (96 percent) with seminoma were free of disease. The two-year Kaplan-Meier survival rate of these patients was 86 percent. Among the 64 patients with nonseminomatous germ cell tumor, 19 patients (30 percent) underwent surgery as first treatment (ten complete resections) followed by chemotherapy (n = 17) and radiotherapy (n = 5). On completion of treatment, 12 of 19 patients were disease free. Forty-five patients (70 percent) underwent induction cisplatin-based chemotherapy (ten complete responses), and 22 of them underwent resection of residual tumor (19 complete resections). Twenty-three patients were treated with first line chemotherapy without postchemotherapy surgery (three complete responses). In summary, 33 patients (52 percent) with nonseminomatous germ cell tumors became free of disease, and seven patients (21 percent) relapsed after achieving a complete response. The two-year Kaplan-Meier survival rate of the nonseminomatous germ cell tumor patients was 53 percent (87 percent if a complete response), with a median survival of 28 months. Despite a worse prognosis than nonseminomaous tumors from other primary sites, this series of mediastinal germ cell tumors has confirmed the efficacy of therapy.

[Primary malignant germinal tumors of the mediastinum. Results from a national retrospective survey]. / Tumeurs germinales malignes primitives du médiastin. Résultats d'une enquête rétrospective nationale.

The object of this enquiry was to group together a series of patients with a mediastinal germ cell tumour who were diagnosed and treated between 1983-1990 to specify their clinical characters and their prognostic factors and to evaluate the current therapeutic strategies. Out of a total of 87 cases that were analysed 23 had seminomas and 64 malignant dysembryomas. Amongst the 23 patients suffering from seminomas, 8 had first been treated by surgery, 13 by chemotherapy and 2 by radiotherapy. At the end of treatment 22 made a complete response and 4 patients relapsed after 4-13 months following a complete response. 3 patients had metastases at the outset and were alive and in complete response at 1,4 and 7 years respectively. The survival level at 2 years for seminomas was 84%. Of 64 patients suffering from a malignant dysembryoma, 19 were first treated with surgery (10 by complete resection) followed by chemotherapy in 17 cases. At the end of treatment 12 patients were in complete remission. Only one of these patients had a subsequent relapse. 45 patients were first treated by chemotherapy (10 complete responses, 26 partial responses) then secondary surgery was performed in 22 cases, allowing a complete remission in 21. Seven malignant dysembryomas relapsed after an initial complete remission. The patients treated for pure choriocarcinoma were 7 in number: 6 of these received primary chemotherapy. One patient underwent a secondary resection and was living at 26 months. The other 5 died rapidly. The 2 year survival for malignant dysembryomas was 52%. The median survival was 28 months. There was a significant difference in surviyal between dysembryomas in complete remission and those in partial remission.

[Pneumopathy due to sulfasalazopyrine. A case with a challenge test]. / Pneumopathie à la sulfasalazopyrine. Une observation avec test de réintroduction.

The complications of treatment with sulfasalazopyrine(SSP) are rare. We report a case of a 68 year old patient who was hospitalised for a hectic fever with weight loss, chest pain and dyspnoea of effort. This patient had been treated for 2 1/2 months for a haemorrhagic proctocolitis with 3 gms. per day of SSP. There was a leucocytosis of 23 700/mm3 of which there were 3,318 eosinophils/mm3. The pulmonary radiograph showed a predominantly interstitial pattern at the apices in the axillary region and in the costo-phrenic angle. The broncho alveolar lavage (LBA) was normal. A thoracic scan confirmed the interstitial syndrome and the existence of pleural thickening in the upper part of the lungs. As drug induced pulmonary disease was suspected SSP was stopped. The patient became apyrexial in 48 hours without any further treatment. Thirty days later the pulmonary radiograph and the laboratory investigations had returned to 'normal and a challenge test using the drug was given whilst the patient was still in hospital. After 24 hours there was a febrile peak of 38.7 degrees C and 36 hours later there was a leucocytosis once more of 19 700/mm3 of which there were 1,100 eosinophils/mm3. The pulmonary radiograph showed diffuse bilateral interstitial opacities. The treatment was stopped and the fever disappeared within 24 hours. After a review of the literature, two points of this case were discussed in particular: the frequency of pulmonary disease occurring as a complication of SSP and the indication for challenge test.