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.


METHODS
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.
Bisogno
G
G
Division of Haematology/Oncology, Department of Paediatrics, University of Padova, Padova, Italy. gbisogno@oncopedipd.org
Roganovich
J
J
Sotti
G
G
Ninfo
V
V
di Montezemolo
L C
LC
Donfrancesco
A
A
Mascarin
M
M
Carli
M
M
D000008:Abdominal Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D000293:Adolescent; D000903:Antibiotics, Antineoplastic / Q000008:administration & dosage; D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage; D000972:Antineoplastic Agents, Phytogenic / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D003131:Combined Modality Therapy; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male; D009364:Neoplasm Recurrence, Local; D011379:Prognosis; D012074:Remission Induction; D016879:Salvage Therapy; D018228:Sarcoma, Small Cell / Q000188:drug therapy / Q000532:radiotherapy / Q000556:secondary / Q000601:surgery*; D015996:Survival Rate; D013736:Testicular Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D014750:Vincristine / Q000008:administration & dosage
D000903:Antibiotics, Antineoplastic; D018906:Antineoplastic Agents, Alkylating; D000972:Antineoplastic Agents, Phytogenic; D003609:Dactinomycin; D014750:Vincristine; D007069:Ifosfamide