Desmoplastic small round cell tumour successfully treated with caffeine-assisted chemotherapy: a case report and review of the literature.

Desmoplastic small round cell tumour (DSRCT) is a rare tumour, usually arising in the abdominal cavity. DSRCT remains an aggressive malignancy, with a poor prognosis despite multi-modality treatments. In the published literature, there has been no patient who lived for three years or more without surgical excision. This report describes a case of DSRCT arising from the brachial plexus and successfully treated with caffeine-assisted chemotherapy. A 29-year-old male presented with pain and numbness in his left forearm. Radiological findings were suggestive of malignant tumour. Histology, immunohistochemical stain and fluorescence in situ hybridisation (FISH) results confirmed the diagnosis of DSRCT. He underwent caffeine-potentiated chemotherapy and the tumour disappeared. The tumour was not removed surgically as it was intertwined in the brachial plexus. Four years after the initial diagnosis, no local relapse and no distant metastases have been observed. Therefore, it is concluded that caffeine-assisted chemotherapy should be one of the treatment options for DSRCT.

Desmoplastic small round cell tumor in children: a new therapeutic approach.

PURPOSE: Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histologic and immunohistochemical features occurring in a young population with a male predominance. The tumor appears to arise as masses in the abdominal cavity without a clear visceral origin. Five patients with DSRCT were treated as usual with combined chemoradiation and surgery. In addition, in our center, patients underwent autologous bone marrow transplant (BMT), which is a novel approach to this disease. METHODS: Charts of 5 patients (4 males, mean age of 11 years) treated between 2000 and 2007 were reviewed. The diagnosis of DSRCT was made on the basis of clinical examination, computed tomographic scan, and explorative laparotomy with biopsy, and biochemical markers were negative. All patients were treated with aggressive chemoradiation and surgery. Three patients also had autologous BMT. RESULTS: Three patients (BMT recipients) responded to treatment. The responding patients had surgery with the intent of removing all disease. Two patients died of their cancer, neither of whom underwent BMT. CONCLUSION: The patients DSRCT are sensitive to an aggressive combination of chemotherapy, surgical debulking, and radiation therapy, followed by autologous BMT. It appears that this new multifaceted treatment offers good palliation, which may prolong survival and a possible cure.

Treatment planning comparison of electron arc therapy and photon intensity modulated radiotherapy for Askin's tumor of chest wall.

BACKGROUND AND PURPOSE: A dosimetric study to quantitatively compare radiotherapy treatment plans for Askin's tumor using Electron Arc (EA) vs. photon Intensity Modulated Radiotherapy (IMRT). MATERIALS AND METHODS: Five patients treated with EA were included in this study. Treatment plans were generated for each patient using EA and IMRT. Plans were compared using dose volume histograms (DVH) of the Planning Target Volume (PTV) and Organs at Risk (OAR). RESULTS: IMRT resulted in superior PTV coverage, and homogeneous dose distribution compared to EA. For EA, 92% of the PTV was covered to 85% of the dose compared to IMRT in which 96% was covered to 95% of the dose. V(107) that represents the hot spot within the PTV was more in IMRT compared to EA: 7.4(+/-2)% vs. 3(+/-0.5)%, respectively. With PTVs located close to the spinal cord (SC), the dose to SC was more with EA, whereas for PTVs located away from the SC, the dose to SC was more with IMRT. The cardiac dose profile was similar to that of SC. Ipsilateral lung received lower doses with IMRT while contralateral lung received higher dose with IMRT compared to EA. For non-OAR normal tissues, IMRT resulted in large volumes of low dose regions. CONCLUSIONS: IMRT resulted in superior PTV coverage and sparing of OAR compared to EA plans. Although IMRT seems to be superior to EA, one needs to keep in mind the volume of low dose regions associated with IMRT, especially while treating young children.

Successful use of intraoperative radiotherapy for local control of an Askin's tumor recurrence.

Askin s tumor is an infrequent disease, with a high tendency to local recurrence. We present the case of a 16-year-old female diagnosed with a new recurrence of this tumor affecting the thoracic wall. There had been a previous 5-year history of 3 local recurrences treated each time by apparently complete surgery. A multidisciplinary approach consisting of chemotherapy, complete chest tumor resection and intraoperative radiotherapy was undertaken. After 2-year follow-up, the patient is alive and free of disease. The role of surgery is still the key to obtaining good survival, but in this case intraoperative radiotherapy proved to be a good adjuvant treatment.

The role of anti-epithelial cell antibodies in the pathogenesis of bilateral radiation pneumonitis caused by unilateral thoracic irradiation.

Two cases of bilateral radiation pneumonitis associated with unilateral thoracic irradiation against lung cancer are described. Both patients died of respiratory failure and autopsy was performed. Histologically, bilateral diffuse alveolar damage was demonstrated in both cases, associated with marked organization of hyaline membrane in one case (case 1). In addition, numerous hyperplastic type II pneumocytes which strongly expressed cytokeratins 8, 18 and 19 were observed. In both patients' sera, antibodies against cytokeratin 8, 18 and 19 were demonstrated by a Western immunoblot. The possible association between autoantibodies to cytokeratins and diffuse alveolar damage observed in patients with bilateral radiation pneumonitis are discussed.

Desmoplastic small round cell tumour in children and adolescents.

BACKGROUND: Desmoplastic small round cell tumour (DSRCT) is a rare highly aggressive neoplasm, and clinical studies are scarce. PROCEDURE: We report six cases of children and adolescents (median age 14 years, range 6.9-17.5) with DSRCT (5 abdominal, 1 paratesticular) registered by the Italian Cooperative Group (ICG) for soft tissue sarcoma over a 9-year period. Patients received a multidisciplinary treatment, including aggressive initial or delayed surgery and radiotherapy. Chemotherapy regimen was based on the use of ifosfamide, vincristine, dactinomycin, and a few doses of antharacyclines (doxorubicin or epirubicin). RESULTS: Complete surgical resection was possible only for the paratesticular tumour. Among the patients with abdominal lesions, macroscopically radical excision was possible in only one case. All patients received multidrug chemotherapy, and tumour reduction was obtained in the 4 evaluable patients. No relapses were evident in the irradiated fields in the 4 patients who received radiotherapy. Two patients remained progression-free 22 and 63 months after diagnosis, one is in third complete remission, whereas three died 10-25 months after diagnosis. CONCLUSIONS: DSRCT is a chemosensitive tumour, but survival rates remain disappointing despite aggressive multimodality therapy. Our results support surgical tumour removal and radiotherapy to achieve local control. Our experience and a review of the literature suggest that patients with localised abdominal tumours or a paratesticular primary may have a better prognosis.