[Malignant paraganglioma of the uterus]. / Paragangliome malin de l'utérus.

We report a malignant uterine paraganglioma in a 41-year-old woman who underwent a hysterectomy for meno-metrorrhagia. It was initially thought to be a leiomyoma in necrobiosis. The clinical outcome was characterized by an early regional recurrence (in the left Fallopian tube). Later, vertebral and lung metastasis occurred, leading to death 22 months after the initial diagnosis. Paragangliomas are uncommon neuroendocrine tumors, related to pheochromocytomas. They are mainly found in the para-aortic and retroperitoneal region, and less commonly in the pelvic area. Location in the uterus is extremely rare: 5 cases were previously reported and only one malignant.

Oxaliplatin and paclitaxel combination in patients with platinum-pretreated ovarian carcinoma: an investigator-originated compassionate-use experience.

PURPOSE: Compassionate-use oxaliplatin paclitaxel was assessed for toxicity and efficacy according to clinical platinum resistance status in cisplatin-carboplatin-pretreated advanced ovarian cancer patients. PATIENTS AND METHODS: Thirty-seven patients, retrospectively grouped into four oxaliplatin-paclitaxel dose levels (mg/m2): (DL1: 100/135; DL2: 130-135/135; DL3: 100/160-175; DL4: 130-135/160-175), received oxaliplatin and paclitaxel every three to four weeks. RESULTS: Thirty-one of thirty-seven treated patients were evaluable for activity, with 1 complete and 14 partial responses, (objective response rate: 48%, 95% CI: 31-66). Of 18 platinum-resistant patients 6 responded, and of 13 platinum-sensitive patients, 9 responded. One patient (3%) had two febrile neutropenia episodes, and eight (22%) and eleven patients (30%) had grades 3 and 4 neutropenia, respectively. Six patients (16%) experienced grade 3 peripheral neuropathy. The median response duration was 10.8 months, with a 23-month (range 8-54) median follow-up. Median progression-free and overall survivals were 9 months (95% CI: 7-12), and 25.2 months (95% CI: 12-39), respectively. CONCLUSIONS: The antitumour activity of oxaliplatin-paclitaxel in platinum-resistant ovarian cancer patients accords with experimental data on the agents' lack of cross-resistance. Time-related progression parameters confirm it as a promising salvage treatment option.

Giant cell tumor of the sternum. Report of a case with a 17q isochromosome.

We report a case of giant cell tumor of the manubrium with radiological evidence of aggressiveness. Few cases of giant cell tumor of the sternum have been published. Extensive surgery offers the best hopes of recovery. Cytogenetic studies evidenced several clonal abnormalities including a 17q isochromosome. The TP53 suppressor gene is located at 17q13.1. Whether loss of one of the TP53 alleles may have contributed to the aggressive behavior of our patient's tumor is discussed.

Outpatient treatment with subcutaneous interleukin-2 and interferon alfa administration in combination with fluorouracil in patients with metastatic renal cell carcinoma: results of a sequential nonrandomized phase II study. Subcutaneous Administration Propeukin Program Cooperative Group.

PURPOSE: We report the results of the Subcutaneous Administration Propeukin Program (SCAPP) II trial of an outpatient treatment in renal cell carcinoma using interleukin-2 (IL-2) and interferon alfa-2a (IFN-alpha) administered subcutaneously in combination with fluorouracil (5-FU). The objective of this multicenter trial was to confirm that the combination of IL-2, IFN-alpha, and 5-FU leads to a response rate greater than 20%. PATIENTS AND METHODS: Patients with metastatic renal cell carcinoma were included in this study. During the induction phase of the treatment, which lasted 10 weeks, IL-2 and IFN-alpha were administered subcutaneously three times a week for 8 weeks at doses of 18 MIU and 9 MIU, respectively. During these 8 weeks, every Monday, 5-FU was administered at a dose of 750 mg by intravenous infusion over 30 minutes. After evaluation, responding patients or patients with stable disease (SD) were given maintenance treatment, until disease progression (PD) or the appearance of unacceptable toxicity. Each maintenance cycle consisted of a 2-week treatment followed by a three-week rest period. During treatment, IL-2 and IFN-alpha were administered subcutaneously three times a week at doses of 18 MIU and 9 MIU, respectively. Every Monday, 5-FU was administered at a dose of 750 mg by intravenous infusion over 30 minutes. RESULTS: This trial was closed when the sixth sequential analysis showed the lack of benefit from this combination. At the end of the induction period, of 62 patients, 12 (19%; 95% confidence interval [CI], 10% to 31%) reached an objective response, including one complete response (CR), 16 presented with SD, and 27 showed PD. Twenty-seven patients (43%) developed severe toxicity that required reduction of the planned doses (13 patients), delayed treatment (eight patients), or treatment termination (six patients). Seventeen patients were given maintenance treatment. One- and 2-year survival rates were estimated at 55% and 33%, respectively. The 2-year survival rate was 15% in 11 patients who presented with three poor-prognosis factors and 41% in 51 patients who initially presented with no, one, or two poor-prognosis factors (P = .04). CONCLUSION: As in other recently published studies that used 5-FU, IL-2, and IFN-alpha, the multicenter SCAPP II trial in patients with metastatic renal cell carcinoma generated severe toxicity. This sequential trial failed to confirm the favorable results previously obtained by Atzpodien and Sella with this combination of three drugs. Its efficacy, assessed on the response and survival rates, is near to the results observed in programs that used IL-2 alone given subcutaneously.

[Retroperitoneal extragonadal germ cell tumors: apropos of 4 cases. The importance of research of an occult testicular lesion]. / Les tumeurs germinales extragonadiques rétropéritonéales: à propos de 4 observations. Intérêt de la recherche d'une lésion testiculaire occulte.

In the absence of palpable testicular tumor one often retains the primitive character of an extragonadal germ-cell tumor with retroperitoneal localization. This motion must be reconsidered in front of the occasionally late discovery of the scrotal origin of those lesions. The usefulness of testicular sonography in the revelation of infra clinical lesions is now perfectly well admitted. Concurrently, testicular biopsies can provide the notion of carcinoma in situ and its links with the retroperitoneal germ-cell tumor must be indicated precisely. The four related observations allow us to stress on the seriousness of this affection which often remains tardily diagnosed. The specific treatment of the testicular lesion, when it is put into evidence, turns out to be absolutely necessary.

[Treatment of hypereosinophilic syndromes of myeloproliferative expression with the combination of hydroxyurea and interferon alpha. Apropos of 7 cases]. / Traitement des syndromes hyperéosinophiliques à expression myéloproliférative par l'association hydroxyurée-interféron alpha. A propos de 7 observations.

Seven patients suffering from hypereosinophilic syndrome with clinical and/or hematological symptoms of a myeloproliferative syndrome were treated with a combination of hydroxyurea and interferon-alpha. To date, 6 of them have been followed for more than 1 year. In all cases, this therapy decreased circulating hypereosinophilia to under 1,500/mm3 and obtained normal eosinophil levels in 5 patients with a parallel regression of visceral complications. None of the patients experienced treatment-associated side effects, particularly hematological ones. Hypereosinophilic syndrome has a poor prognosis due, for the most part, to the development of visceral, cardiac and neurological complications, and, more secondarily, to the risk of progression towards acute leukemia. Conventional treatments, corticosteroids and hydroxyurea, have greatly improved the prognosis, but failure of these therapeutics remains common in the myeloproliferative form of the disease. Because interferon-alpha was proven to effectively treat chronic myeloid leukemia, it has been proposed for the treatment of hypereosinophilic syndromes, and encouraging results have been obtained despite the high daily doses required to control the disease. In the future, its association with hydroxyurea could represent an alternative therapy capable of controlling the disease at low doses thereby limiting the risk of side effects.

[Bone localizations disclosing Hodgkin's disease. Apropos of 11 cases]. / Localisations osseuses révélatrices de la maladie de Hodgkin. A propos de 11 observations.

The authors report 11 cases of Hodgkin's disease in which the first sign was due to bone involvement. Such observations are rare and raise diagnosis delays (Means: 5, 6 months in this series). Modern medical imaging techniques (scintigraphy, CT scan, magnetic resonance imaging) are very useful to an earlier diagnosis which will be better confirmed by lymph node biopsy given the poor information yielded by osseous cytology and/or histology. The elective localisation in bones belonging to the axial skeleton seems to support the hypothesis of a contiguous osseous involvement from lymphoïd organs in contact with bones. Evolution with chemotherapy and radiotherapy is very similar to that observed in patients without bone involvement.

[Richter's syndrome with testicular localization]. / Syndrome de Richter à localisation testiculaire.

Based on a case showing testicular localisation of sarcomatous degeneration in the context of the progression of chronic lymphoid leukemia (Richter's syndrome), the authors discuss the classification of intrascrotal lymphomatous conditions.

[Pseudolymphomatous aspects of mesenteric lymphadenopathies in Whipple's disease. Value of x-ray computed tomography monitoring]. / Aspects pseudo-lymphomateux des adénopathies mésentériques dans la maladie de Whipple. Intérêt de la surveillance tomodensitométrique.

Lymphadenopathy is a very common finding during the course of Whipple's disease (W.D.). Abdominal (mesenteric and retroperitoneal) tumoral lymphnodes may mimick malignant neoplasm, specially a lymphoma. The authors report the case of a patient who had an abdominal computer-assisted tomography (C.T.) at the diagnosis and during an eleven month survey for W.D. General C.T. findings can help to differenciate mesenteric lymphadenopathy in W.D. from those found in malignant lymphomas: low density (10-20 Hounsfield Units) because of the high fatty charge of Whipple's lymphnodes, lymphadenopathy in the lower mediastinum, progressive but slow improvement with antibiotic therapy.

[Immunologic thrombopenic purpura and HIV infection in heroin addicts]. / Purpura thrombopénique immunologique et infection HIV chez les héroïnomanes.

The authors have studied the clinical aspects and mechanism of the thrombocytopenia observed in heroin addicts in the light of recently published data. This relatively frequent complication of intravenous drug addiction must be related to the immunological disturbances caused by carriage of the HIV virus. The evolutive characteristics of thrombocytopenia in such cases and the therapeutic implications of this association between thrombocytopenic purpura and acquired immunodeficiency are discussed.

[Esophageal localization revealing Hodgkin's disease]. / Localisation oesophagienne révélatrice d'une maladie de Hodgkin.

The authors report the case of a 28 year old man with Stage III E Hodgkin's disease presenting with massive upper oesophageal involvement with cervical and mediastinal lymphadenopathy. The diagnosis was confirmed by lymph node and endoscopic biopsies (mixed cellularity). A complete remission was obtained by chemotherapy (ABVD) followed by extended radiotherapy. Then other cases have been previously reported. Upper oesophageal involvement with mediastinal lymphadenopathy often occurring in young patients raises the differential diagnosis of a carcinoma. The efficacy of chemotherapy of first intention is underlined.