Wael Zekri

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.

Does primary tumor volume predict the outcome of pediatric nasopharyngeal carcinoma?: A prospective single-arm study using neoadjuvant chemotherapy and concomitant chemotherapy with intensity modulated radiotherapy.

Nasopharyngeal carcinoma is rare in children and adolescents; however, it has a better prognosis than in adults. Nevertheless, its prognostic factors are not clearly documented.

Nonfunctioning Adrenocortical Carcinoma in Pediatric Acute Lymphoblastic Leukemia: A Case Report of a Rare Multiple Primaries Combination

Childhood adrenocortical carcinoma (ACC) is a rare tumor and its association with acute lymphoblastic leukemia (ALL) is even rarer. One such case is discussed in this case report. A 3-year-old patient was concomitantly diagnosed with ALL and an initially nonmetastatic ACC. Management started by following the Total XV protocol without a window phase. Left adrenalectomy was conducted after the consolidation phase. Recurrence of a mass at the tumor bed was discovered at week 33 of the continuation phase. Reexcision was conducted, followed by the administration of an ACC protocol including cisplatin, etoposide, and doxirubicin. Mitotane was added when a pulmonary metastasis was discovered and then stopped after the patient suffered from an arachnoid cyst and speech difficulties. The ALL protocol was resumed from week 34 of the continuation phase. Progression of pulmonary nodules was noted after week 45. A pulmonary metastectomy was performed. The ALL protocol was resumed up to week 51 with a good response as proven by assessment of minimal residual disease. A further recurrence was diagnosed at the abdominal tumor bed with a paravertebral mass and a pulmonary nodule. The patient was assigned to palliative treatment and died after a 32-month survival. Such rare associations need more extensive discussions of the best possible management in scientific literature.

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.].

Bilateral clear cell sarcoma of the kidney

Clear cell sarcoma of the kidney (CCSK) accounts for 2-5% of all pediatric renal malignancies, and is known for its propensity to metastasize to bone and other sites. We are reporting two cases with bilateral CCSK that were diagnosed at our institution. One patient initially presented with bilateral renal masses, as well as pulmonary, hepatic and bone metastasis; while other present only with bilateral masses with no evident distant metastasis. Both patients received aggressive neo-adjuvant chemotherapy to decrease tumor size. One patient completed his designated treatment and initially showed complete remission (CR); eventually suffering from relapse. The other patients tumor progressed during the course of chemotherapy. Both cases manifested brain dissemination at the time of relapse or progression. This emphasizes the importance of staging stratification in CCSK. This also illustrates CCSKs ability to metastasize to bone and other sites including the brain (a primary relapse site in our cases).

Clear cell sarcoma of the kidney: patients' characteristics and improved outcome in developing countries.

Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive tumor accounting for 5% of pediatric renal tumors with an incidence of 20 patients per year in the USA. It is bone metastasizing with poor prognosis. Our aim was to show characteristics of patients in relation to improved outcome in one of the developing countries.

Histopathology and prognosos of clear cell sarcoma of the kidney

Background: Clear cell sarcoma of the kidney (CCSK) is an uncommon renal neoplasm of childhood. CCSK has a wide histologic spectrum that mimics other pediatric renal neoplasms with considerable diagnostic difficulty. Our aim is to show various histopathologic CCSK patterns, pitfalls, diagnostic utilities and outcome in one of the developing countries. Methods: We included all patients diagnosed as CCSK in the period between July 2007 and May 2013 at Childrens Cancer Hospital, Egypt. Patient demographics, clinical presentation, pathology and management were reviewed. Follow up was continued until March 2013. Results: Thirty-two patients were found within that interval, accounting for 6.4% of all renal tumors. Median age was 28.5 months. Stages I, II, III, IV and V represented 11 (34.4%), 3 (9.4%), 13 (40.6%), 3 (9.4%) and 2 (6.3%), respectively. The majority of tumors showed classic pattern either solely or focally. Other patterns were myxoid, spindle, cellular, epithelioid, haemangiopericytomatous, palisading and anaplastic. Wide immunohistochemical panels were done including vimentin, INI-1, WT1, CK, CD99, Synaptophysin, Desmin, LCA, CD34, P53 and EGFR. Fluorescence in situ hybridization (FISH) for EWS break apart translocation was done for 5 cases (suspected PNET) and synovial sarcoma breakapart translocation in 4 cases. Overall survival and event-free survival was 93% (95% CI 92.06–93.94%) for 16 months. Conclusion: CCSK has many histologic patterns that highlight the diagnostic pathologic dilemma. In addition, our institution showed increased survival of CCSK.