Multicentre phase II study of cisplatin-etoposide chemotherapy for advanced large-cell neuroendocrine lung carcinoma: the GFPC 0302 study.

BACKGROUND: The optimal treatment of large-cell neuroendocrine carcinoma (LCNEC) of the lung remains unclear. Here, our primary objective was to assess the efficacy of cisplatin-etoposide doublet chemotherapy in advanced LCNEC. Accuracy of the pathological diagnosis and treatment toxicity were assessed as secondary objectives. PATIENTS AND METHODS: Prospective, multicentre, single-arm, phase II study with a centralised review of treatment-response and pathological data. Patients had untreated performance status (PS) 0/1 stage IV/IIIB LCNEC and received cisplatin (80 mg/m22 d1) and etoposide (100 mg/m22 d1-3) every 21 days. RESULTS: Eighteen centres included 42 patients (mean age, 59 ± 9 years; 69% men; median of four cycles/patient). At least one grade-3/4 toxicity occurred in 59% of patients (neutropaenia, thrombocytopaenia, and anaemia in 32%, 17%, and 12%, respectively). The median progression-free survival (PFS) and overall survival (OS) were 5.2 months (95% confidence interval, CI, 3.1-6.6) and 7.7 months (95% CI, 6.0-9.6), respectively. The centralised pathologist review reclassified 11 of 40 (27.5%) patients: 9 as small-cell lung cancer, 1 as undifferentiated non-small-cell lung cancer, and 1 as atypical carcinoid. Survival data were not significantly changed by excluding the reclassified patients. CONCLUSIONS: The pathological diagnosis of LCNEC is difficult. The outcomes of advanced LCNEC treated with cisplatin-etoposide doublets are poor, similar to those of patients with advanced small-cell lung carcinoma (SCLC).

[Epidermoid cyst of the testis. Conservative surgery in 3 cases]. / Kyste épidermoïde du testicule. Chirurgie conservatrice à propos de 3 cas.

The authors report 3 cases of epidermoid cyst of the testis. Clinical, laboratory and ultrasound data were relatively nonspecific. Precise histological criteria were defined by PRICE in 1969. The diagnosis is based on frozen section examination after transnnguinal testicular exploration. The authors describe the most conservative therapeutic approach for a constantly benign testicular tumour.

[Interstitial pneumopathy revealing type B Niemann Pick disease in an adult]. / Pneumopathie interstitielle révélatrice d'une maladie de Niemann-Pick de type B chez un adulte.

INTRODUCTION: Niemann-Pick disease is an autosomal recessive disorder due to partial or total deficit in sphingomyelinase. EXEGESIS: We report a case of type B Niemann-Pick disease revealed by pneumonia and splenomegaly associated with blue histiocyte syndrome. Investigations that were done 2 years prior to diagnosis had shown the existence of isolated chronic lipid pneumonia which is specific of overloading. CONCLUSION: Though diagnosis is based on biochemistry, the morphology of alveolar histiocytes after simple bronchoalveolar lavage is of value.

Prognostic value of nuclear grade of renal cell carcinoma.

BACKGROUND: The authors assessed the interest and the value of Fuhrman's nuclear grade as a possible prognostic factor for renal cell carcinoma (RCC). METHODS: An 11-year retrospective study of 190 patients with RCC treated by radical nephrectomy was performed. The distribution by grade was: Grade I, 54 patients; Grade II, 58; Grade III, 58; and Grade IV, 20. The distribution of the patients by tumor stage according to the TNM15 classification was: pT1, 56 patients; pT2, 41; pT3a, 55; pT3b, 25; pT3c + pT3d + pT4b, 5; and pT4a, 8. Significant correlations with other prognostic parameters were noted. Survival curves by grade were evaluated by the Kaplan-Meier method. RESULTS: Nuclear grade was correlated with tumor stage (P = 0.0001), synchronous metastases (P = 0.003), lymph node involvement (P = 0.0001), renal vein involvement (P = 0.0001), tumor size (P = 0.0001), and perirenal fat involvement (P = 0.001). No correlation was found between nuclear grade and tumor multicentricity (P = 0.14) and cell type (P = 0.2). Nuclear grade was an effective parameter in predicting development of distant metastases after nephrectomy. Among the 54 patients who presented with Grade I tumors, only one tumor did metastasize during the 5-year follow-up, whereas 17% of the Grade III and 30% of the Grade IV tumors metastasized. The 5-year actuarial survival rates of the patients with Grade I, II, III, and IV tumors was 76%, 72%, 51%, and 35%, respectively. The comparison of the survival curves by grade showed a statistically significant difference between the curves when Grade I and II tumors were compared with Grade III and IV tumors (P = 0.001). CONCLUSION: In this study, nuclear grade was found to have prognostic significance and seems to be an important criterion when considering the outcome of patients with RCC.

[Infantile polycystic hepatorenal disease. 2 consecutive cases in a patient. Ultrasonic diagnosis and indication for elective pregnancy termination]. / Maladie polykystique hépatorénale infantile. Deux cas consécutifs chez une patiente. Diagnostic échographique et indication d'interruption médicale de grossesse.

We report a case of infantile polycystic kidney disease, during the course of two consecutive pregnancies in the same woman. Observed rates of recurrence in families at risk is higher than theoretical rates (25%). Antenatal ultrasound can show signs of bilateral involvement, which is always lethal and generally leads to elective termination of pregnancy. Diagnosis can rarely be made before 24 weeks of pregnancy.

[Why and how to grade renal carcinomas?]. / Pourquoi et comment grader les carcinomes rénaux?

The prognosis of renal cell carcinomas has been a controversial subject for pathologists for a long time. At the present time, in addition to stage, the nuclear grade defined by Führman represents an essential element in the prognostic assessment. A renal cell carcinoma can be easily characterized by its stage and its grade, defining a precise prognosis. Specific entities such as tubulopapillary tumors, oncocytomas and chromophobe cell carcinomas must also be graded until the results of complementary studies become available allowing a better assessment of their autonomy among renal cell carcinomas.

[The multifocal form of bronchocentric granulomatosis]. / Granulomatose bronchocentrique à forme multifocale.

Bronchocentric granulomatosis is a rare entity and has only recently been characterised. We report a case of a forty-three year old man presenting with multifocal bronchocentric granulomatosis which was revealed following some oto-rhino-laryngological and bronchopulmonary symptoms. The open lung biopsy confirmed the diagnosis. Treatment was by bolus doses of corticosteroids (30 mg/kg) and of methotrexate in low dosage (7.5 mg/week, then 15 mg/week) which checked the initially unfavourable outcome.

Metastatic prostatic adenocarcinoma-diagnosed by bronchoalveolar lavage and tumour marker determination.

We report a patient who presented with a diffuse interstitial lung disease in whom clinical and radiologic investigation led to the suspicion of lymphangitis carcinomatosa. Bronchoalveolar lavage (BAL) was performed and revealed the presence of prostatic specific antigen (PSA) positive cells. A prostatic needle aspiration confirmed the diagnosis of adenocarcinoma. This case demonstrates the value of tumour marker determination in BAL.

[Leiomyosarcoma of the superior vena cava. Case report]. / Leiomyosarcome de la veine cave supérieure. A propos d'un cas.

Leiomyosarcoma of the blood vessels is a rare malignant tumor, and its localization in the superior vena cava is exceptional. We had the opportunity to study such a case and we compared our clinical, histological and ultrastructural results with those of 5 other reported cases.

[Mediastinal adenopathy disclosing Castleman's disease. Apropos of a case]. / Adénopathies médiastinales révélatrices d'une maladie de Castleman. A propos d'une observation.

Castleman's disease or pseudo-tumoral lymphoid hyperplasia is a rare entity. We report a case of Castleman's tumour in the antero-superior mediastinum which was revealed by a radiological examination in a young woman of 26. We also report immunohistochemical studies. There are two clinical presentations of Castleman's disease; a localised form (mediastinal or extrathoracic) and the multicentric variety. The localised form may be asymptomatic or can be accompanied by general features of inflammatory disease. Surgical excision of these localised forms both confirms the diagnosis and assures a cure. The multicentric variety has various clinical peculiarities (the patients are older and/or immunosuppressed and suffering from multiple enlarged glands also in the viscera) and the disease in above all progressive. There are associations with Kaposi sarcoma and lymphoproliferative complications are described in this multicentric form and their prognosis remains poor.

Subpleural micronodules in diffuse infiltrative lung diseases: evaluation with thin-section CT scans.

Thin-section computed tomography (CT) was performed in 244 patients with infiltrative lung diseases and 29 healthy control subjects to evaluate the frequency, profusion, and diagnostic value of subpleural parenchymal micronodules. These areas of increased attenuation (less than 7 mm in diameter) were analyzed in four groups: coal miners with chest radiographic findings of coal worker's pneumoconiosis (n = 61), coal miners with no radiographic evidence of pneumoconiosis (n = 73), patients with nonoccupational chronic infiltrative lung disease (n = 110), and healthy adults (n = 29). Subpleural parenchymal micronodules were observed with high frequency in pulmonary lymphangitic carcinomatosis, coal worker's pneumoconiosis, and sarcoidosis but were also seen in 14% of control subjects. Predominant sites of lesions were the posterior subpleural areas in the upper lobes. Subpleural parenchymal micronodules have no diagnostic value when observed as an isolated CT finding but may suggest that diagnosis of pneumoconiosis, sarcoidosis, or pulmonary lymphangitic carcinomatosis when observed in association with mild parenchymal lesions.

Congenital cystic adenomatoid malformation of the lung.

Twenty cases of cystic adenomatoid malformation of the lung were observed: 2 had died in utero; the diagnosis was made at birth in 13 infants of which 3 were premature. A Bochdalek's hernia had been diagnosed before birth in 2 cases by echo-tomography. The correct antenatal diagnosis had been made in 2 cases. Two infants had no symptoms, 3 were dyspneic, 8 were in respiratory distress and had to be intubated and ventilated. Two had a prune belly syndrome. Eight infants had a thoracotomy during their first week of life. Pulmonary resections concerning those 13 patients comprised 10 lower lobectomies, 1 of which was associated with a lingulectomy, and 2 upper lobectomies, 1 of which was associated with a middle lobe resection. Five patients were diagnosed and operated upon between 10 months and 8 years of age; 4 had recurrent bronchitis and 1 was diagnosed during the treatment of a gastroenteritis. They had 3 lower and 2 upper lobectomies. Recovery was uneventful in all patients except for 1 who was reoperated upon for intestinal obstruction. Antenatal diagnosis of cystic adenomatoid malformation should become standard. The malformation may be mistaken for a pulmonary sequestration or bronchogenic cyst. Differential diagnosis of a congenital hernia is important.

[Magnetic resonance imaging in the evaluation in utero of Siamese twins]. / Imagerie par résonance magnétique (IRM) dans le bilan in utero des jumeaux siamois.

The authors report a case of twins conjoined at the umbilicus and diagnosed by ultrasound after 19 weeks of amenorrhoea in whom an assessment in utero was carried out using magnetic resonance imaging after the patient had been curarized. A review of the literature on this very difficult problem of conjoined twins has given us the possibility to assess the diagnostic measures as well as the prognosis of this pathology. In particular we point out the results that can be obtained using MRI in utero during the second and third trimesters of the pregnancy.

[Leiomyoma of the esophagus and pulmonary metastasis]. / Léiomyome de l'oesophage et métastase pulmonaire.

An important and symptomatic esophageal leiomyoma is treated by enucleation. A secondary metastasis, also important and symptomatic, is resected 3 years later. The follow-up is now 2 years, without any sign of relapse. This case allows for discussion of the malignancy of leiomyoma and of its microscopic criteria.

[The lung in brain death. Anatomo-biological correlations for pulmonary transplantation]. / Le poumon des sujets en état de mort cérébrale. Corrélations anatomo-biologiques en vue de la transplantation pulmonaire.

Histopathological studies of the lung conducted during multiple organ procurement from 25 subjects in a state of brain death showed that 19 of them had lung lesions pre-existent to coma, viz. alveolitis and lung invasion by dusts increasing with the subject's age. Alveolar or interstitial emphysema and subpleural bullae caused by mechanical ventilation were present in 19 cases; signs of bronchial inertia were found in 6 cases and established superinfection in 3 cases. Finally, 17 subjects showed interstitial and vascular alterations induced by oedema and shock which were related to the time spent in intensive care units, to the cause of the coma and to the quantity and quality of intravenous received while in intensive care unit. There were histological correlations between these various factors, but precise criteria concerning the functional prognosis of the transplant could not be defined. However, this study seemed to confirm the need for a new approach to the intensive care of brain-dead subjects during lung procurement: the time elapsed before procurement should be shortened, the amount of medicinal solutions administered should be reduced, Ringer lactate and packed cells should be used in preference to albumin derivatives, and arterial blood pressure, rather than diuresis, should be maintained.

[Congenital cysts of the esophageal wall with a respiratory type mucosa]. / Kystes congénitaux de la paroi oesophagienne à revêtement muqueux de type respiratoire.

Eight patients with congenital esophageal cysts are described. They presented with respiratory or gastrointestinal symptoms or an abnormal mediastinal profile on a standard chest film. All cysts were contained in the middle or inferior portions of the thoracic esophagus. They were excised in a submucosal plane. Their coat was composed of a ciliated, respiratory type epithelium. In the embryo and after separation of the tracheal diverticulum, the esophagus is lined with ciliated cells which are able cover a "cystic duplication", which is surrounded by an esophageal muscular coat or can be "included" in a common mesenchyma, which explains the possible presence of bronchial elements in the wall of an esophageal cyst. In this eventuality the cyst may be defined as being "bronchogenic".

[2 cases of villous tumor of the appendix]. / Deux observations de tumeur villeuse de l'appendice.

Villous tumors of the vermiform appendix are uncommon with two cases and a review of the literature, the authors indicate the lack in specific clinical sign, and lay stress on the importance of the pathologic study on the therapeutic management.

[Morphologic diagnosis of pulmonary hemorrhage]. / Diagnostic morphologique de l'hémorragie pulmonaire.

The gross and histologic morphologic features of early and advanced pulmonary hemorrhages are easy to recognize. Although there is no close correlation between the hemorrhagic appearance of the bronchoalveolar lavage fluid, the histologic features of hemorrhage, and the score of hemosiderin in alveolar macrophages, studies of bronchoalveolar lavage fluid samples are valuable for diagnosing and monitoring pulmonary hemorrhages.

Acute monoblastic leukemia with osteosclerosis and extensive myelofibrosis.

A 4-month-old infant was admitted with a monoblastic infiltration of the skin associated with osteosclerosis. Both lesions spontaneously disappeared within a few months, but 2 years later, a monoblastic leukemia occurred that was associated with marked skin erythema and myelofibrosis. Skin and bone marrow specimens showed a monoblastic infiltration with numerous intermingled mast cells of normal appearance. Whether myelofibrosis was a feature of a systemic mastocytosis or of the leukemic process is discussed in this case.