Added predictive value of 18F-FDG PET/CT for pediatric rhabdomyosarcoma.

OBJECTIVE: To determine the prognostic value of quantitative fluorine-18-fluorodeoxyglucose (F-FDG) standardized uptake value (SUV) in patients with pediatric rhabdomyosarcoma (RMS). PATIENTS AND METHODS: Consecutive 98 (50 males and 48 females) (age range: 4 months to 17.5 years, mean age: 5.8 ± 4.5) patients with pathologically proven RMS who underwent PET/computed tomography for initial staging were retrospectively assessed for whether primary SUVmax and the primary/liver SUVmax ratio could predict event-free survival (EFS) and overall survival (OS) for 36 months using receiver operating characteristic curve analysis. Univariate and multivariate analyses were used to determine the reliability of these metabolic parameters and various clinical factors. RESULTS: Higher SUVmax was significantly related to the presence of regional or distant metastasis with worse prognosis. With receiver operating characteristic curve marked cut-off values of 3.6 and 2.1 for primary SUVmax and the primary/liver SUVmax ratio, respectively, both EFS and OS proved to be higher in patients with SUVmax ranked below the determinate values. Patients with a primary/liver SUVmax ratio below the cut-off value of 2.1 had OS (60.8%) and EFS (48.1%) compared with 44.5 and 14.8% for patients with lesions exceeding the cut-off point of uptake; however, this failed to achieve statistical significance. In the evaluation of primary SUVmax, similar results were obtained with P values of 0.76 and 0.62, respectively. High SUVmax was more prevalent among patients with less favorable clinical and pathological features including unfavorable primary site, alveolar pathology, and high-risk group. CONCLUSION: F-FDG PET/computed tomography may be considered an additional prognostic predictor of outcome in RMS patients, where higher F-FDG uptake seems to be linked to lower survival and correlated to different unfavorable parameters.

Clinical presentation of intraocular retinoblastoma; 5-year hospital-based registry in Egypt.

PURPOSE: To study the presenting signs of Retinoblastoma in Egypt at Egypt's main pediatric oncology referral center. METHODS: This is a prospective descriptive study (hospital-based registry) conducted at Children's Cancer Hospital Egypt between July 2007 and December 2012. RESULTS: Out of 262 patients diagnosed with retinoblastoma, 244 were suffering from intra-ocular disease at presentation. One hundred thirty-nine (57%) patients presented with unilateral disease, while 105 (43%) suffered bilateral disease. The mean age at presentation was 20.6 ± 17 months, averaging 18.87 ± 11.76 months for bilateral and 25.72 ± 18.78 months for unilateral disease. The most common clinical presentation was leukocoria in 180 (73.8%) patients, strabismus in 32 (13.1%) patients and decreased visual acuity in 12 (4.9%) patients. Group D and E disease represented 62% of all affected eyes. Patients with advanced disease (Group C-E) had longer duration of symptoms. CONCLUSION: In Egypt, retinoblastoma patients present more frequently with advanced disease. There is an ever-increasing need to develop a national team dedicated to studying disease significance and formulating a national awareness program.

Corrigendum to "Outcome of Rhabdomyosarcoma in First Year of Life: Children's Cancer Hospital 57357 Egypt".

[This corrects the article DOI: 10.1155/2013/439213.].

Clinical significance of anaplasia in childhood rhabdomyosarcoma.

BACKGROUND: The presence of anaplastic features has been known to correlate with poor clinical outcome in various pediatric malignancies, including Wilms tumor and medulloblastoma but not in rhabdomyosarcoma. AIM: Aim was to study the frequency of anaplasia at presentation in childhood rhabdomyosarcoma and its relationship to clinical and pathological characteristics as well as to outcome. PATIENTS AND METHODS: Anaplasia was retrospectively assessed in 105 consecutive pediatric rhabdomyosarcoma patients who were registered at the Children's Cancer Hospital in Egypt (CCHE) during the period from July 2007 till the end of May 2010. RESULTS: Anaplasia was diagnosed in 18 patients (17.1%), focal in 10 (9.5%) and diffuse in 8 (7.6%). The distribution of anaplasia was found to be more common in older patients having age⩾10 years. Also it was more likely to occur in the high risk group and in tumors with unfavorable histology (alveolar subtype), and stage IV. The 3-year failure free survival rates for patients with and without anaplasia were 27.8±10.6% and 53.4±5.8%, respectively (p=0.014) and the 3-year overall survival rates were 35.3±11.6% and 61±6%, respectively (p=0.019). CONCLUSIONS: The frequency of anaplasia in pediatric patients with rhabdomyosarcoma in our study was 17.1%. The presence of anaplasia had statistically significant worse clinical outcome.

Phthisis bulbi: clinical and pathologic findings in retinoblastoma.

PURPOSE: Phthisis bulbi represents an ocular end-stage disease characterized by shrinkage and disorganization of the eye. We aim at identifying the pathologic changes of phthisis bulbi associated with retinoblastoma. DESIGN: Retrospective observational case series study. METHODS: 16 enucleated eyes were enrolled retrospectively between 2007 and 2012. Pathologic gross and microscopic findings were assessed. RESULTS: Cases showed grossly shrunken eyes with a mean volume of 4.3 cc. Sclera was markedly thickened in the majority of cases with mean of 2272.8 µ. Choroid showed an average thickness of 1029 µ. Necrosis, dystrophic calcification, ossification, gliosis, residual viable tumor was identified in many cases. Pathologic high risk factors were seen in three specimens. CONCLUSIONS: We conclude that retinoblastoma must be considered in the differential diagnosis of phthisis bulbi in the pediatric patients and active tumor was present in half of the patients.

Outcome of Rhabdomyosarcoma in First Year of Life: Children's Cancer Hospital 57357 Egypt.

Background. Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children. Fifty percent of RMS cases occur in the first 10 years of life and less commonly in infants younger than one-year old. These infants require adapted multimodality treatment approaches. Patients and Methods. We analyzed patients' characteristics, treatment modalities, and the outcome for RMS infants treated at Children's Cancer Hospital Egypt (CCHE) between July 2007 and December 2010 and compared them to patients above one year treated on the same protocol. Results. Out of the 126 RMS treated during this period, 18 were below the age of one year. The male: female ratio was 1.25 : 1. The median age at diagnosis was 0.7 ± 0.2 years. Most of the cases (27.8%) were presented in head and neck regions. The estimated 4-years failure-free survival and overall survival for infants were 49 ± 12% and 70 ± 12%, respectively. These failure-free survival rate and overall survival rate did not differ from those for older patients (P = 0.2). Conclusion. Infants with RMS are a unique group of RMS who needs special concerns in tailoring treatment in addition to concerns regarding toxicity and morbidity in infants.