Nasopharyngeal carcinoma (NPC) is a specific entity different from head and neck carcinoma. Incidence is higher in South-East Asia and North Africa. Prognosis, especially for locally advanced stages (IIB - IVB) and metastasis, remains poor: more than third of cases will present local and/or metastatic recurrence. Overall 5-year survival for all NPC stages ranges from 50% to 70%. The role of chemotherapy in metastasis is well established, and remains an important palliative treatment, although no randomized trial has been reported comparing the different chemotherapy regimens. As 1(st)-line treatment, platin-based regimens seems optimal; in 2(nd) line and after progression under platins, there is no consensus: monotherapy with drugs such as gemcitabine, capecitabine or taxanes has been the most widely tested, with acceptable results. Future trials should integrate targeted therapy, in the light of overexpression of EGFR1 and C-kit in NPC. The present study presents a review of the literature concerning the various studies of metastatic NPC.

Background: Bladder small cell carcinoma is an uncommon tumour. Through a retrospective study we will present the evolution of recurrent and metastatic disease and outcome of patients treated at Léon-Bérard Cancer Centre.

No abstract

Desmoplastic small round cell tumors (DSRCT) are rare and aggressive malignant neoplasms that usually occur in young males. They usually present intraabdominally with widespread peritoneal involvement at diagnosis and are sometimes associated with lymphadenopathies and liver metastases. Intraabdominal DSRCT is usually disseminated; no curative outcome has yet been achieved. We report a case of metastatic intraabdominal DSRCT controlled by an anthracycline-based regimen. A literature review of the treatment options, mainly chemotherapy, available for this unusual neoplasm, will be discussed. In patients with unresectable or metastatic disease, symptom control is most important because treatment modalities minimally impact survival. Palliative chemotherapy (mainly monotherapy) is preferable.

Objectives: Only a few case reports and small case series of patients with sarcoidosis or sarcoid-like reaction and testicular germ cell tumors (GCT) have been reported in the literature. We performed a retrospective study of patients with testicular GCT managed at the Centre Léon-Bérard, who presented granulomatosis. Methods: We performed a computerized search to identify all male patients with both a diagnosis of sarcoidosis or granuloma and testicular tumors seen at the Centre Léon-Bérard between 1992 and 2008. Results: A total of 13 patients were identified among the 1,182 patients with testicular tumors. The median age at diagnosis of testicular GCT was 25.5 years. Six patients had stage I disease, 2 patients had stage IIb and 5 patients had stage III. Sarcoid-like granulomatosis was found in 9 patients at the time of initial diagnosis and in 4 patients during follow-up. Sarcoidosis presented mainly as pulmonary disease without severe organ involvement, with a benign evolution. Conclusion: We advise caution in the interpretation of clinical and histological findings in these patients. Sarcoid-like granulomatosis, a condition that can be combined with testicular cancer, should always be considered in the differential diagnosis of metastatic testicular tumors.

No abstract

Wilm's tumor is rare in adulthood. The prognosis is poor when compared with children. Adults more frequently present with advanced disease stages, and chemotherapy has a moderate effect. The various protocols of chemotherapy and indications for surgery and radiotherapy are not yet precisely defined, mainly due to the rarity of the disease. The Society of Pediatric Oncology (SIOP) guidelines suggest that both pediatric and adult patients should have initial chemotherapy followed by surgery. However, the National Wilms' Tumor Stage Group (NWTSG) recommends surgery followed by chemotherapy. The authors present 2 cases of adult nephroblastoma that were highly resistant to presurgical chemotherapy. Nephrectomy was not completed because of rapid disease progression. The poor response of these 2 patients indicates that the alternate NTWSG approach of initial surgery followed by systemic treatment might have better results for this population. The authors review related reports and discuss the diagnosis and management of adult Wilms' tumor.

No abstract