Clinical presentation and therapeutic actions
A one year-old boy with Rb suspection was referred to the Department of
Pediatrics, University of Debrecen. Given the bilateral, large primary
extent of the tumor not suitable for local therapy systemic chemotherapy
was immediately introduced. Two cyclophosphamide/vincristine block,
advised for infant neuroblastoma was applied with partial response. Thus
therapy was continued with three more intensive
carboplatin/etoposide/vincristine block, but without further tumor
regression, so three additional VEC
(vincristine/etoposide/cyclophosphamide) was introduced that resulted in
significant tumor regression making possible local therapy. Until the
local intervention two additional VEC was applied with decreased (60%)
dose in view of the patient tolerance. Then a local brachytherapy
(Ruthenium-106 applicator) was applied on the right side, and
cryotherapy on the left side. Five months later a progression was
observed on the right side that extend the local control borders. As a
bridge therapy VEC with cisplatin, then 50 Gy external radiotherapy was
applied for both sides. However, progression was occured five months
later, so enucleation was necessary on the right, and then unfortunately
on the left side three months apart (Sample 1 – S1 and 2 - S2).
Telemetric radiotherapy followed enucleation on both sides in 50 Gy
doses. The proband, at the age of 10 years presented osteosarcoma of the
left orbita that was surgically resected (S3). EURAMOS1 and than EURO
EWING99-VIDE protocol was used for chemotherapy. One year later
osteosarcoma presented on the left tibia as well and was resected 17 cm
of the its proximal region (S4). Resected tibial bone was irradiated
using 100 Gy and EURAMOS1/COSS chemotherapy was applied. At the age of
12 years, left femur and multiple pulmonary metastases were diagnosed.
Up to the upper third, the left femur was amputated and osteosarcoma was
proved by histological examination (S5). According to the bad general
condition of the child, pulmonary tumor was sentenced as inoperable.
Three months later the proband died due to breathing complications.
Progressive metastases of both sides of the lungs were demonstrated as
cause of death during autopsy. Postmortem sampling from the pulmonary
metastases (S6) and from the intact skin (sample S7, for non-tumor
control purposes) was done. Samples provided for the comparative study
and related histological diagnoses are summarized in Table 1 .