A case of long-term survival of metastatic desmoplastic small round cell tumor treated with multimodal therapy

Abstract

Abstract. Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive and malignant tumor that predominantly affects young males. No standard therapy is currently available for patients with DSRCT and the prognosis remains extremely poor. In this study, we report a thought-provoking DSRCT case. A 24-year-old male was admitted to our hospital with a chief complaint of hematemesis. Computed tomography revealed a retrovesical mass with a splenic hilar tumor, multiple lung and liver tumors and marked lymph node swellings. The source of hematemesis was gastric varices caused by the compression of the splenic vein by a splenic hilar tumor. The patient was provided with a histological diagnosis of DSRCT based on needle biopsy from the liver tumors and the pelvic mass was thought to be the primary lesion. This is a long-term survival case of metastatic DSRCT treated with multimodal therapy including 15 courses of multiagent chemotherapy, radiation therapy for the hepatic portal region using 42.5 Gy, and four instances of therapeutic endoscopy. The prolonged progression-free survival period (15 months) obtained following chemotherapy suggests the chemosensitive feature of the disease. We used a modified P6 regimen (cyclophosphamide, pirarubicin, vincristine, ifosfamide and etoposide) and a modified PAVEP regimen (cyclophosphamide, pirarubicin, etoposide and cisplatin) to decrease severe adverse events and to improve the completion rate of chemotherapy. DSRCT is an aggressive but chemo-sensitive disease, and continuous chemotherapy using an appropriate regimen with possible supportive care is essential for long-term survival. This case report may represent a treatment option for this rare disease.

Abdominal Desmoplastic Small Round Cell Tumor: Multimodal Treatment Combining Chemotherapy, Surgery, and Radiotherapy is the Best Option

“…ifosfamide based regimens, n = 5, API, n = 1; or other (cyclophosphamide based or etoposidecisplatin, n = 5), for a median of 4 cycles (range 3-10 cycles). 22,23 Partial radiologic response and stable disease were observed in 68 and 23 % of the patients, respectively. There was no complete radiologic response.…”

Desmoplastic Small Round Blue Cell Tumor: A Review of Treatment and Potential Therapeutic Genomic Alterations

“…A modified P6 regimen and a modified PAVEP regimen [ 63 , 68 ] (cyclophosphamide, pirarubicin, etoposide, and cisplatin) have been employed to decrease severe adverse events and to improve the completion rate of chemotherapy. These modified regimens use Cytoxan of 4 g/m 2 and replace Adriamycin with pirarubicin.…”

Two autopsy cases of desmoplastic small round cell tumor

“…A clinical report of 20 abdominal DSRCT cases showed that the median overall survival (OS) was 22 months (interquartile range: 12–28 months), and that the 5‐year OS rate was 20% . However, cases of long‐term survival of metastatic DSRCT have recently been reported . Here we present two autopsy cases of DSRCT, that is, an aggressive case (survival after initial symptoms was only 12 months) and a long‐term survival case (survival after primary hospitalization was 42 months).…”

“…5 However, cases of long-term survival of metastatic DSRCT have recently been reported. 6 Here we present two autopsy cases of DSRCT, that is, an aggressive case (survival after initial symptoms was only 12 months) and a long-term survival case (survival after primary hospitalization was 42 months). Table 1 shows the clinical summary of both cases including patient characteristics, clinical and imaging findings, initial diagnosis (clinical/histological), treatments, and outcomes.…”