Abstract
Background: Intraabdominal desmoplastic small round cell tumors (IDSRCT) are rare in children
and predominantly affect male adolescents and young adults. We present our experience
in the management of five children with diffuse IDSRCT, managed with aggressive chemotherapy,
surgery, radiotherapy and peripheral blood stem cell transplantation. Material and Methods: During the last decade five patients, four males and one female (mean age 9.6 years),
with diffuse IDSRCT were managed in our department. The main symptoms were abdominal
distention, vague abdominal pain, and vomiting. Three patients with inoperable tumor
on admission were submitted initially to open biopsy followed by aggressive chemotherapy.
Regression of the tumor was followed by a second laparotomy and radical excision of
any macroscopically distinguishable masses, followed by chemotherapy. In the remaining
two patients a debulking procedure was done initially, followed by chemotherapy. The
accurate diagnosis of the disease was established by immunohistochemistry, additionally
confirmed in the last two patients by molecular analysis. Results: Three patients who had radical excision of the tumor and adjuvant chemotherapy had
recurrence after two to six months. In the remaining two patients, recurrence was
evident after two and eighteen months, respectively, following debulking. In addition,
one patient with recurrence received radiotherapy and two others underwent peripheral
blood stem cell transplantation. All but one patient died within three years from
diagnosis. The last patient, who was submitted to a debulking procedure, is still
alive eight months after the operation. Conclusions: Intrabdominal desmoplastic small round cell tumor is a highly aggressive malignancy
with a very poor prognosis. Multiagent chemotherapy usually leads initially to a temporary
regression of the tumor, but recurrence is the rule. Radical surgical excision, radiotherapy
and peripheral blood stem cell transplantation does not seem to improve prognosis
significantly. Despite all therapeutic modalities the outcome is dismal and surgical
efforts can be considered only as palliative.
Key words
Desmoplastic small round cell tumor - childhood
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M.D. George Mavridis
“Aghia Sophia” Children's Hospital
79 Alevizatou str.
15669 Papagou, Athens
Greece
eMail: mavridisg@hotmail.com
