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Intraabdominal
desmoplastic small round cell tumour.Pediatr
Surg Int.
2005 Apr;21(4):279-81. Epub 2005 Mar 11
Desmoplastic
small round cell tumour (DSRCT) is an extremely rare neoplasm.
Adolescent males and young adults are most frequently affected. It is
highly malignant, with only 29% of patients surviving up to 3 years.
This paper documents two cases, one of which, at 4 years old, is the
second youngest case documented. Case 1, a 10-year old boy, presented
with a 20-day history of choluria, acholia, asthenia, anorexia, and
right abdominal pain. Laboratory values were altered, and imaging
showed multiples masses in the liver and retroperitoneum. A
minilaparotomy was carried out, and a biopsy showed a stage III DSRCT.
He was treated with chemotherapy but died of hepatic failure. Case 2,
a 4-year-old boy, presented with a 2-month history of abdominal
distension. Several hard masses were palpated in the abdomen, and a
right inguinal mass that compressed the right testis was observed.
Biopsy of the inguinal tumour showed a DSRCT. After treatment with
chemotherapy, two operations were carried out to resect different
intraabdominal masses. The patient died with peritoneal carcinomatosis
2 months after the last operation. The first patient died due to the
advanced stage of the disease, and the second died after chemotherapy,
peripheral blood stem transplantation, and multiple operations. The
occurrence of this type of tumour in the paediatric age group as well
as its high malignancy is noteworthy. Until more effective forms of
treatment are found, we recommend treatment with chemotherapy,
surgery, and radiotherapy, with close monitoring of the patient. |
