Program Book

2006 RBp100
RETINOBLASTOMA ASSESSMENT BY DOPPLER
SONOGRAPHY - A FOLLOW - UP STUDY
Maria Teresa B.C. Bonanomi, Osmar C. Saito, Tatiana Tanaka
(mtbonanomi@uol.com.br)
Hospital das Clínicas da Faculdade de Medicina da Universidade de São
Paulo (HCFMUSP) São Paulo - BRAZIL
Purpose. Chistian Andreas Doppler (1803-1853) first described the
physical principle that permits to assess the velocity of an object by
using the change of frequency of waves. This principle was applied in
medicine to study the large vessels in 1957. Color Doppler imaging (CDI)
is an ultrasound technique that combines B-scan images with velocity
information obtained from the Doppler shift of the moving erythrocytes.
The intrinsic circulation of tumors has been reliably imaged in
melanomas before and after treatment. The Purpose of this study is to
assess the retinoblastoma volume and its vascularization by CDI before
and during treatment.
Methods. Retinoblastoma tumors in children with bilateral disease were
studied with the “Toshiba Aplio XG, 16 MHz Transducer. Tumors stage
B or higher were assessed before and after three cycles of the operative
protocol treatment in the HCFMUSP. Tumor volume was calculated
by a multiplication of three manually obtained ultrassonographic
measurements (transversal, longitudinal and antero-posterior in
centimetres) and the mathematical constant 0.52. Vascularization
was indicated as present or absent. Data was compared with the
ophthalmoscopy findings.
Results. There were 14 eligible tumors in 5 children (2 males and 3
female). The mean age of presentation was 10 months (3 to 21months).
Only two lesions were excluded due to unreliable data. The initial mean
volume of the twelve tumors was 0.37cm3 ± 0.68 cm3 (0.004 cm3 to
2.36 cm3). Intrinsic vessels were detected in 9 lesions with mean
volume of 0.49 cm3±0.76 cm3 while in three lesions, mean volume
of 0.01cm3±0.01cm3, tumor vessels could not be demonstrated, the
difference was statistically significant ( p=0.036 Mann-Whitney).The
mean volume at three months was 0.04cm3 ± 0.06cm3 (zero to 0.172
cm3), corresponding to a mean reduction of 86%. The tumor vessels
pattern was exuberant showing variable degrees of turbulence before
treatment. The vascularization at 3 months, considered only in 6 tumors
that were not flat, was still present in 1 (29%) and totally absent in 5.
All 6 tumors showed features of viability at fundus examination in the
third month, meaning opaque tissue sometimes with visible vessels.
Movement of the child and the presence of calcium inside the tumor
hamper the vessel imaging. After treatment, vascularization shrinkage
can be demonstrated by CDI, even in small lesions.
Conclusions. Echodoppler is able to assess size and vascularization of
the tumor in the same session. Intrinsic vessels may be overlooked in
too small tumors. Lack of vascularization in the follow-up does not imply
total involution of the tumor. This diagnostic tool should be considered
in assessing retinoblastoma.
Financial disclosure. None
354 RBp101
PATIENTS WITH UNILATERAL RETINOBLASTOMA AND
HIGH-RISK PATHOLOGIC FEATURES DO NOT REQUIRE
INTENSIVE METASTATIC WORK-UP OR AGGRESSIVE
CHEMOTHERAPY
RETINOBLASTOMA
Posters
48
Ibrahim Qaddoumi1,7, Matthew W. Wilson2,3,6, Catherine Billups4,
Jianrong Wu4, Thomas Merchant5, Barry Shulkin5, Rachel Brennan1,
Barrett G. Haik3,6, Carlos Rodriguez-Galindo1,7, Erin Sullivan1. (ibrahim.
qaddoumi@stjude.org)
Departments of 1. Oncology, 2. Pathology, 3. Surgery, 4. Biostatistics,
5. Radiological Sciences St. Jude Children’s Research Hospital; 6.
Ophthalmology (Hamilton Eye Institute) and 7. Pediatrics, University of
Tennessee Health Sciences Center, Memphis, TN.
Dr. Rodriguez-Galindo is currently at Dana-Farber Cancer Institute,
Boston, MA
Purpose. To describe the work-up, treatment, and outcome of patients
with advanced unilateral intraocular retinoblastoma.
Methods. Patients received up-front enucleation, then were assigned to
Low-Risk (LR), Intermediate-Risk (IR), or High-Risk (HR) groups according
to histology of enucleated eyes. LR patients underwent observation. IR
patients received 4 courses of chemotherapy that included vincristine,
doxorubicin, and cyclophosphamide (VDC). HR patients received 6
courses of chemotherapy: VDC alternated with vincristine, carboplatin,
and etoposide (VCE). The IR and HR groups also received granulocytecolony
stimulating factor.
Results. Fifty patients were treated: LR, 36 patients; IR, 7 patients;
HR, 7 patients. The median age at enrollment was 26 months. All eyes
were classified as Reese-Ellsworth group V. All bone scans (n=79),
lumbar punctures (n=17), and bone marrow aspirates (n=16) were
negative. Chemotherapy-related toxicity was well tolerated. Grades 3
and 4 hematologic toxicities were seen in all patients; grades 3 and 4
nonhematologic toxicities were seen in 7 of 14 patients (in the IR and HR
groups). Chemotherapy dose modifications or delays due to neutropenia
or thrombocytopenia were needed in only 4/67 (6%) courses. Only1
patient in the HR group received radiation therapy.
All patients were alive at the time of analysis; 1 patient was still on active
therapy, and none had experienced recurrence. Median follow-up was
2.5 years (range, 0.2-5.7 years).
Conclusions. Nonmetastatic retinoblastoma with high risk pathologic
features may be cured with a short course of chemotherapy with good
toxicity profile. Thus, intensive metastatic work-up and aggressive
chemotherapy are not required.
Financial disclosure. None
1916 RBp103
TEN YEARS OF EXPERIENCE IN THE TREATMENT OF
INTRA-OCULAR RETINOBLASTOMA IN A SINGLE
INSTITUTION IN BRAZIL
Carla R. Donato Macedo1, Luiz F. Teixeira1,2, Camila H. Hashimoto1, Virginia
L. L Torres1,2, Juliana dos Santos Soares1, Maria T. Seixas1,3, Maria C.
Martin 2, Clelia M. Erwenne1,2 (carladonatomacedo@uol.com.br)
1. Pediatric Oncology Institute/GRAACC/Unifesp
2. Ophthalmology Department/ Unifesp
3. Pathology Department /Unifesp
Purpose. Present clinical and epidemiological features, treatment
modalities and survival of patients with intra-ocular retinoblastoma
Methods. Data as epidemiological features, laterality, international
staging of retinoblastoma, treatment modalities, survival and the
incidence of second malignancies were collected.
The best initial and subsequent treatments were based on whether the
child has unilateral or bilateral disease, the stage of the disease, and
the age of the child.