Naglaa Elkinaai

Childhood Ewing Sarcoma of the Orbit.

In the span of the last 48 years, only 33 cases of children with orbital Ewing sarcoma have been reported. This study is to present 3 cases that were admitted to Children’s Cancer Hospital Egypt 57357, during the period from 2009 to 2013. We have 2 cases treated using the hospital standard Ewing sarcoma treatment protocol, to completion, whereas the third discontinued treatment. All tumors have confirmed CD99 positivity, although translocation (11;22) was positive in 1 patient and negative in the third. With earlier diagnosis and adequate surgical resection and integration of chemotherapy and radiotherapy 1 patient survived for about 4 years, whereas the other 2 cases died due to disease progression or recurrence.

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 Childrens 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.

Survival outcome of intermediate risk neuroblastoma at Children Cancer Hospital Egypt

AIM The study aims to evaluate survival outcome in newly diagnosed pediatric intermediate risk neuroblastoma patients treated at the Children Cancer Hospital - Egypt and their relation to various clinical and pathological factors. METHODS The study included stage 3 patients <1.5 years, children 1.5 years or older with stage 3 disease and favorable histopathological features, infants (<1 year) with International Neuroblastoma Staging System (INSS) stage 4 disease, stage 4 children 1-1.5 years with favorable biology, and infants stage 4 s (with unfavorable biologic features). Patients received systemic chemotherapy, in the form of etoposide and carboplatin alternating with cyclophosphamide, doxorubicin and vincristine, administered at 3-week intervals, with a total of 6 or 8 cycles guided by reaching objective overall response (complete/very good partial/partial response). RESULTS The study included 136 patients, 67 males and 69 females. 101 patients had abdominal primary tumors, 28 had mediastinal masss and 7 with masses in the neck; 68% were stage 3 and the remaining (n = 44) had metastatic disease. The three-year overall survival (OS) and event-free survival (EFS) estimates were 94% ± 2% and 90.9% ± 2.5%, respectively. OS and EFS by gender, age, pathology and INPC were all statistically not significantly different. Moreover, OS for patients having surgery versus no surgery (inoperable residual only) was statistically significant (98.4% ± 1.6% & 88.7% ± 5.3%, respectively, p = .034). CONCLUSION A very high rate of survival is currently achievable in patients with intermediate risk neuroblastoma by chemotherapy or chemotherapy and surgery. In addition to response, our plan is to adopt biologically-based treatment to reduce treatment-induced complications among survivors.

Hepatoblastoma in Developing Countries; Eight Years of Single Centre Experience

Background and objectives: Although hepatoblastoma (HB) is a rare childhood tumor and constitutes only 0.9% of all pediatric cancers, there was an obvious improvement in risk stratification and prognosis over the last two decades. This study aimed to assess the outcome of HB patients treated in our center and to investigate the impact of different prognostic factors on the survival of these patients. Patients and methods: This was a retrospective study included newly diagnosed patients with HB presented to the Children Cancer Hospital Egypt (CCHE 57357), from July 2007 to June 2015. Patient’s data were analyzed for the clinical characteristics and survival outcome of the included patients. Results: One hundred twenty-four children were included during the study period with a median age of 14 months. The tumor was found occupying the entire liver in 25 patients (20%); while it was confined to one lobe in 80% of them, portal vein thrombosis was diagnosed in 10 patients, and there were 30 patients (24%) had metastatic disease at presentation. Only five patients (4%) underwent primary surgical excision, and all of them were grossly excised (stage I); 77/119 (64.7%) experienced delayed surgery after two to six courses (median, four courses) of C5VD and the overall resection rate was 66%. There were 42 patients (35.3%) failed to do surgical excision either because they still had evident metastatic disease with poor chemotherapy response, or because their tumor remained unresectable after six courses of chemotherapy. The 3-year event-free and overall survivals for the whole studied patients were 45.7% (95% CI, 36.9% - 56.7%), and 66.7% (95% CI, 57.1% - 77.8%) respectively. The 3-year EFS and OS were significantly better in those who underwent surgical excision (68.63% and 80.74% respectively, P-value 0.001). Also, the survival rates were significantly affected by the presence of metastatic disease at presentation, tumor stage and initial risk grouping of the studied patients. Conclusion: Surgical excision, tumor stage and COG risk grouping are the main prognostic variables affecting patients’ outcome. Efforts to achieve resectability of the tumor either by advanced surgical techniques or by developing effective preoperative treatment, especially for advanced and metastatic disease, are mandatory.

Surgery in perforated pediatric intestinal lymphoma

BACKGROUND Perforation is the most common surgical complication in pediatric intestinal lymphoma. During operation, many surgical decisions are debatable. AIM To assess the outcome of surgical management of perforated pediatric intestinal lymphoma. PATIENTS AND METHODS This is a retrospective analysis of all pediatric patients (<18 years old) with intestinal lymphoma treated in our hospital between July 2007 and June 2017. Risk factors for perforation, type of management and outcome in cases of intestinal perforation were analyzed. RESULTS The study included 240 patients with intestinal lymphoma. Perforation developed in 16 patients (6.7%) with a median age of 5.3 (range: 2.8-15.7) years. Most of the patients (92.5%) had Burkitt lymphoma. The ileum was the most common site of perforation (n = 10). Perforation occurred at presentation (n = 2), during induction (n = 10), during maintenance chemotherapy (n = 2), or at relapse (n = 2). Primary resection anastomosis was done in 12 patients. The resected specimen showed a viable tumor in ten patients. Wound infection (25%) and dehiscence (12.5%) were the most common postoperative complications. The 5-year overall and event-free survivals of patients with perforation were 78.6% and 71.4%, respectively, compared with 85.5% and 81.2% in non-perforated patients; the difference was not significant (p = 0.374 and p = 0.270, respectively). CONCLUSION Perforation is not an adverse prognostic factor for survival in pediatric intestinal lymphoma patients. Primary resection anastomosis seems to be a safe option if complete tumor resection is feasible.

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.