Management strategy for children with ovarian immature teratoma: results from a tertiary pediatric oncology center.

OBJECTIVES: We present an Egyptian study on pediatric ovarian immature teratomas (ITs), aiming to clarify our treatment strategy selection. METHODS: A retrospective review of all children with pure ovarian ITs who were treated at our institution between 2008 and 2023. The analysis included clinical characteristics, tumor staging according to Children's Oncology Group (COG), grading based on the Norris system, management, and outcomes. RESULTS: Thirty-two patients were included, with a median age of 9 years. All patients underwent primary surgery. Unilateral salpingo-oophorectomy was performed in 31 patients. Surgical staging was completed in all patients. Based on COG staging, there were 28 patients (87.5%) stage I, 1 (3%) stage II, and 3 (9.5%) stage III. According to Norris classification, 16 patients (50%) were classified as grade I, 9 (28%) grade II, and 7 (22%) grade III. All patients in stage I were treated using surgery-alone approach, whereas the remaining four (12.5%) received adjuvant chemotherapy. Five patients in stage I had gliomatosis peritonei (GP), and none of them underwent extensive surgery. At a median follow-up of 86 months, two patients had events. The first patient (stage III/grade I) developed IT relapse on the operative bed, and the second (stage I/grade I) had a metachronous IT on the contralateral ovary. Both patients were successfully managed with surgery followed by second-line chemotherapy. Five-year overall survival and event-free survival for all patients were 100% and 93.4%, respectively. CONCLUSIONS: Surgery-alone strategy with close follow-up achieves excellent outcomes for localized ovarian ITs in children, irrespective of the Norris grading or the presence of GP. However, adjuvant chemotherapy is questionable for patients with incompletely resected or locally advanced tumors, and its role requires further evaluation through prospective multicentric studies with a larger sample size.

Scoliosis After Resection of Primary Pediatric Chest Wall Tumors.

BACKGROUND: Scoliosis is one of the long-term consequences of surgical resection of pediatric chest wall tumors. This study aimed to identify the risk factors associated with scoliosis development following the resection of chest wall tumors. METHODS: Retrospective cohort study of 64 children who underwent resection of malignant chest wall tumors from 2009 to 2022. Univariate and multivariate analyses were used to investigate factors associated with scoliosis development. RESULTS: The median age at the time of surgery was 7 years (range, 3-21), with 33 (51.6%) patients undergoing surgery before the age of 10 years. The most common histology was Ewing sarcoma (n = 57). A median of 3 (range, 1-5) contiguous ribs were resected. A total of 34 (53.1%) patients had anterior CWTs and 30 (46.9%) had posterior CWTs. Concomitant partial lung and diaphragmatic resection were performed in 12 patients (lung, n = 7; diaphragm, n = 5). Scoliosis convex towards the resection side developed in 21 (32.8%). The primary risk factors for scoliosis were resecting 3 or more ribs (OR 6.44) and resection of the posterior rib segment (OR 5.49). Patients with a tumor resection below 10 years old were not associated with a higher risk of scoliosis. CONCLUSIONS: Scoliosis following resection of a primary malignant pediatric chest wall tumor is associated with resection involving three or more ribs and resection of the posterior rib sector. TYPE OF STUDY: Retrospective observational. LEVEL OF EVIDENCE: IV.

Prognostic significance of the ratio of surgically resected to radiologically detected lung nodules in patients with metastatic osteosarcoma.

The factors that affect the prognosis of patients' metastatic osteosarcoma are still poorly understood. In this study, we investigated a new prognostic factor, the ratio of surgically resected to radiologically detected osteosarcoma lung nodules (SR/RD), which may have predictive value. PATIENTS AND METHODS: Data from patients with metastatic osteosarcoma who underwent metastasectomy between January 2009 and December 2020, in a single center, were reviewed. The relationships between survival and the SR/RD ratio, timing of lung metastases, number of nodules, laterality, and presence of tumor necrosis at first metastasectomy were investigated. RESULTS: Among the 125 metastatic osteosarcoma patients, 80 patients had an SR/RD ratio ≤1. The median duration of follow-up was 72 months, ranging from 6 to 118 months. The five-year overall survival (OS) and postmetastasectomy event-free survival (EFS) for all patients were 36.5% and 18.1%, respectively. The five-year OS of patients with a low SR/RD ratio was 49.6% and that of patients with a high SR/RD ratio was 11.8 (P = 0.001). The two-year postmetastasectomy EFS rates of the high and low ratio groups were 24.1% and 9.4%, respectively (P = 0.001). The SR/RD ratio, number of nodules, and tumor necrosis had significant effects on OS and postmetastasectomy EFS in univariate analysis. A Cox proportional hazard model demonstrated that tumor necrosis and an SR/RD ratio >1 were associated with OS (HR = 1.8 and 2.01) and postmetastasectomy EFS (HR = 1,69 and 1.97). CONCLUSIONS: A high SR/RD ratio of greater than 1 and poor tumor necrosis were significantly associated with poor survival among patients with metastatic osteosarcoma who had lung metastasectomy. The high SR/RD ratio may be a surrogate outcome for incomplete metastatic tumor resection.

Effect of timing of pulmonary metastasis occurrence on the outcome of metastasectomy in osteosarcoma patients.

BACKGROUND: Complete metastasectomy is the best predictor of survival in patients with osteosarcoma pulmonary metastases. There has been some controversy in the literature regarding the prognostic significance of the timing of occurrence of lung metastasis. METHODS: We reviewed the clinical course of all osteosarcoma patients with pulmonary metastases treated by metastasectomy in our hospital from January 2008 through December 2016. Each patient who underwent metastasectomy was placed into one of three groups based on whether lung metastases were present at initial presentation (Group 1), developed during chemotherapy (Group 2), or appeared after completion of chemotherapy (Group 3). Data were obtained retrospectively and follow-up was obtained until the end of June 2017. RESULTS: We identified 170 patients with pulmonary nodules of whom 99 (58.2%) underwent at least one metastasectomy (149 thoracotomies). Eleven patients had benign pulmonary nodules and were excluded. The other 88 patients were classified as Group 1 (37), Group 2 (18) or Group 3 (33). The median follow-up was 35 months (range 8 to 99). Postmetastasis 5-year overall survival (OS) was 38.1 ±â€¯6.4%; event-free survival (EFS) was 25 ±â€¯5.3%. By group, postmetastasis 5-year OS and EFS were 34.3 ±â€¯13% and 18 ±â€¯9.3% in Group 1, 8 ±â€¯6.5% and 6.5 ±â€¯5% in Group 2, and 52 ±â€¯11.4% and 25 ±â€¯9% in Group 3 (P < 0.001). In univariate analysis, the only significant factors associated with survival were timing of occurrence of lung metastasis and the number of lung nodules found. CONCLUSION: The timing of occurrence of lung metastasis is an important prognostic factor among osteosarcoma patients eligible for metastasectomy. Patients whose metastases occurred during chemotherapy had the worst survival. LEVEL OF EVIDENCE: Level II.

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.

Surgical approaches, anaesthetic management and outcome in pediatric superior mediastinal tumors.

BACKGROUND: Pediatric superior mediastinal tumors are a heterogeneous group of tumors with marked variation in pathology and extension. We reviewed our experience with different surgical approaches to tumors originating from or extending to superior mediastinum in pediatrics. PATIENTS AND METHODS: The medical records of all patients who had undergone resection for superior mediastinal tumors in Children's Cancer Hospital - Egypt, between January 2008 to December 2015, were reviewed for demographic data, clinico-pathological features, radiologic findings, operative techniques and outcome. RESULTS: The study included 20 patients. Diagnosis included: germ cell tumors (n=8), neuroblastoma (n=4), soft tissue sarcoma (n=3), thymolipoma (n=2), infantile fibromatosis (n=1), calcifying fibrous tumor (n=1), and thymic carcinoma (n=1). Tumor extension was divided into tumors extending unilaterally to one hemithorax (n=9), tumors extending bilaterally to both hemithoraces (n=4), and cervico thoracic junction tumors (n=7). Extended lateral thoracotomy was used in 8 patients. Other approaches included trapdoor (n=5), clamshell (n=4), cervical approach (n=2) and double level lateral thoracotomy (n=1). There was no perioperative mortality, and postoperative morbidity was 20%. At the end of December 2016, 15 patients were alive free of disease, 5 patients developed local and/ or distant relapse. CONCLUSION: Pediatric superior mediastinal tumors could be divided into 3 groups according to tumor extension. Each group has an optimum surgical approach that achieves the best exposure for adequate resection. However, further research is needed to confirm the conclusion as this was a descriptive study and the sample size was too small for valid statistical analysis.

Outcome of resectable pediatric Ewing sarcoma of the ribs.

PURPOSE: Was to evaluate the outcome of multimodality treatment in resectable primary Ewing sarcoma/primitive neuroectodermal tumor ES/PNET of the ribs and role of thoracoscopy in facilitating resection of these tumors. PATIENTS AND METHODS: This was a retrospective study including 22 patients with primary ES/PNET of the ribs surgically treated at Children's Cancer Hospital Egypt (CCHE) between January 2008 until the end of December 2014. RESULTS: Median age was 8.5years (range 5months to 16years.). All patients received neoadjuvant chemotherapy. Thoracoscopic exploration was performed in 15 (68%) patients. Resection included 1,2,3 and 4 ribs in (7,4,8 and 3 patients) respectively, parts of the diaphragm (3 patients), wedge resection of the lung (10 patients) and pleural nodules (2 patients). Primary closure was feasible in 11 patients and rib transposition was done in one patient. Reconstruction by proline mesh covered by muscle flap was done in 10 patients. Margins were microscopically positive in 3 patients and close in 2 patients. Postoperative radiotherapy was given in 8 patients. With a median follow-up of 38.5months, the 3-year event -free survival (EFS) and overall survival(OS) rates were 31.6% and 55.6%, respectively. CONCLUSION: Multimodality treatment is essential in the management of ES-PNET of the ribs. Neoadjuvant chemotherapy facilitates adequate resection. The role of thoracoscopy and the indications of postoperative radiotherapy need further evaluation.

Epidemiology and management of breast carcinoma in Egyptian males: experience of a single Cancer Institute.

OBJECTIVE: To assess the epidemiological and clinico-pathological features, surgical and reconstructive techniques, adjuvant treatments and clinical outcome of breast carcinoma in males (BCM) at the Egyptian National Cancer Institute (NCI). PATIENTS AND METHODS: Thirty-two males with breast carcinoma presented to NCI between January 2000 and December 2002. They were evaluated by complete history, physical examination, laboratory and radiological investigations. RESULTS: Median age was 59 years. Left sided and retroareolar breast lumps were the commonest presentations. Grade II tumors positive for hormone receptors were very common. Stages I, II, III and IV of the disease were encountered in 6.2%, 34.4%, 34.4% and 25.0% of patients, respectively. Curative surgery was done in 22 patients; they received adjuvant hormonal therapy, chemotherapy and radiotherapy in 22, 16 and 10 patients, respectively. Eight metastatic patients were treated with palliative measures. Surgery was done in 25 patients; the most common procedure was modified radical mastectomy (40.6%). Primary closure was feasible in 17 patients (68%), local flaps were needed in 4 cases (16%), while myocutaneous flap was done in 3 cases (12%). The commonest complication was the development of seroma (9 cases). The overall survival (OS) at 5 years was 65.4%. The disease free survival (DFS) at 5 years was 53.9%. Stage and curative surgery significantly affected OS, while type of surgery was the only variable significantly affecting DFS. CONCLUSION: Male breast carcinoma occurs at older ages than females, usually in advanced stage. This necessitates directing attention of males and awareness on the prevalence and risk factors for this disease.

Continuous validity of pedicled myocutaneous and myofascial flaps in reconstruction after surgery for head and neck cancer.

PURPOSE: The aim of this study was to reevaluate the role and effectiveness of pedicled myocutanous and myofascial flaps in reconstruction after resection of head and neck cancer. PATIENTS AND METHODS: This study represents the authors own experience using pedicled myocutanous and myofascial flaps in reconstruction after resection of malignant tumors of different sites in the head and neck. The study included 121 patients with head and neck cancer operated upon at the National Cancer Institute, Cairo University and Alminia Cancer Center over 3 years duration, between July 2005 and the end of July 2008. Four types of flaps were used namely the Pectoralis major (Group I), lower trapezius (Group II), Latissimus dorsi (Group III), and the temporalis ((Group IV) flaps. Utility of the different types of these flaps was reevaluated in terms of indications, advantages, and postoperative complications. RESULTS: This study included 121 patients, 83 males and 38 females. The mean age was 56 years (range, 14- 65 years). Oral malignancies represented most of the cases (71 cases). Pectoralis major myocutaneous (PMMC) flap was the most commonly used flap (84 cases) and its main indication was oral and pharyngeal defects. Lower trapezius and Latissimus dorsi myocutaneous flaps were used in 14 and 12 cases respectively. Their main indications were tumors in the occiput, ear pinna, parotid and neck regions. The Latissimus dorsi myocutaneous flap was also used for reconstruction of oral and pharyngeal defects in 7 female patients with large breasts and for salvage reconstruction after failure of reconstruction by (PMMC) flap in one patient and for reconstruction after excision of local recurrence on top of previous (PMMC) flap in another patient. Temporalis myofascial flap was used in 12 cases and the main indication was orbital defects. The overall postoperative complications was 19.8% (24/121). It was 20% (17/84) in group I, 28.6% (4/14) in group II, and 25% (3/12) in group III. No flap related complications were reported in group IV. All complications were successfully managed except for one case in (Group I), in which a major flap loss developed and was successfully salvaged by pedicled latissimus dorsi myocutaneous flap. CONCLUSION: Pedicled myocutanous and myofascial flaps are still indicated in composite head and neck reconstruction. Their reliability, safety, and high degree of resistance to infection make them essential specially the (PMMC) flap previously described as the spare wheel of head and neck reconstructive surgery. It is suitable for lateral mandibular defects in edentulous patients and in partial pharyngeal defects in irradiated patients. Other pedicled myocutaneous flaps are still valid in certain occasions and sites. KEY WORDS: Head and neck cancer - Pectoralis major - Trapezius - Latissimus dorsi - Temporalis myocutaneous - Myofascial flaps - Reconstruction.