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MEDIASTINAL GERM CELL TUMORS IN CHILDREN AND ADOLESCENTS. OUTCOMES AT A COMPREHENSIVE CANCER CENTER IN MEXICO
Abstract
Background and Aims
Mediastinal germ cell tumors (MGCT) represent the second most common location for extragonadal germ cell tumors. They are a rare disease, and the initial diagnosis require a high index of suspicion. The clinical picture may vary from an incidental finding, or cough, thoracic deformation, to the extreme of a compressive emergency. The diagnosis may be done with cross sectional imaging studies and serum tumor markers. A biopsy may be obviated, which is of particular importance if the anesthetic risk is high. Neoadjuvant chemotherapy may be indicated if the tumor volume is high and the serum tumor markers are elevated. This case series focuses on the surgical approach and outcomes.
Methods
Methods: the span of our study was of 10 years, and we reviewed the medical records of all patients with mediastinal germ cell tumors at our hospital, which is a comprehensive cancer center. The variables included age, sex, symptoms at presentation, course of disease and tumor volume, histology, as well as multimodal treatment strategies and outcomes.
Results
Results: 47 patients with mediastinal tumors were treated during the study period, and 20 of them were MGCT. We included 18 patients, 16 were male, with average age of 10.2 + 4.9 years. 60% presented with compressive symptoms. The tumor occupied more than 50% of the thoracic cavity in 14 cases. Neoadjuvant chemotherapy (4 cycles) was given in 15 patients. Surgical resection was possible in 16, and the most frequent approach was a clamshell thoracotomy. Three patients died of disease progression or complications. The survival rate for this cohort at 80 months was of 56%.
Conclusions
Surgical resection with adequate exposure is the cornerstone to achieve long term survival.
FUNCTIONAL FECAL AND URINARY OUTCOMES AFTER SACROCOCCYGEAL TUMOR RESECTION IN PEDIATRIC PATIENTS
- Hira Ahmad, United States of America
- Devin Halleran, United States of America
- Alessandra Gasior, United States of America
- Ihab Halaweish, United States of America
- Mark Ranalli, United States of America
- Marc A. Levitt, United States of America
- Richard J. Wood, United States of America
- Jennifer H. Aldrink, United States of America
Abstract
Background and Aims
Sacrococcygeal masses are uncommon tumors in infants and children. The most common histopathology is mature sacrococcygeal teratoma (SCT), occurring in 1/40,000 births. These tumors are often challenging to approach given the proximity to and often displacement of pelvic functional organs, including bladder, rectum, and anal sphincter complex. The aim of this study is to describe functional bladder and bowel outcomes for patients who underwent resection of a sacrococcygeal mass.
Methods
A retrospective review was performed of patients who underwent sacrococcygeal mass resection between 1999 and 2019 and were followed long-term in surgical oncology and colorectal clinics at our institution. Baylor Social Continence Scale and Vancouver Symptom Score surveys were employed to assess fecal and urinary continence. Age at the time of the operation, tumor characteristics, histopathology, type of anorectal malformation (ARM) if present were also recorded. At each follow-up visit, bowel and urinary continence, and urinary tract infections were assessed.
Results
74 patients were included in our study. 51 (69%) patients were females. 23 (31%) patients had an associated ARM. The median age at resection was 7 months [IQR 0-25]. 39 (53%) patients were clean for stool at most recent follow-up, 15 (20%) were diapered, and 4 (5%) were diverted. 28 (38%) were dry for urine, 18 (24%) on intermittent catheterizations, and 14 (19%) diapered. Post-operatively, 23 of the 58 patients over age 5 years (40%) formally attended a bowel management program. The mean Baylor continence scale was 15 and the Vancouver Symptom Score was 8 at most recent follow-up.
Conclusions
Sacrococcygeal masses are uncommon anomalies that may have long-term urinary and fecal functional outcomes. A multidisciplinary approach to the care of these complex patients with bowel and bladder management programs may optimize outcomes and patient quality of life.
MANAGEMENT AND OUTCOMES OF PAEDIATRIC EXTRA-CRANIAL GERM CELL TUMOURS AT CHRIS HANI BARAGWANATH ACADEMIC HOSPITAL
Abstract
Background and Aims
Paediatric germ cell tumours (GCTs) encompass a wide variety of neoplasms and account for 1 – 3% of childhood tumours. Diagnosis and staging require a combination of imaging, tumour markers and tissue biopsy. Management varies due to their heterogeneous nature, with surgical resection being a major determinant. Current US survival rates are at 90% for children presenting with stage I or II, 87% with stage III, and 82% with stage IV. However, South African national statistics regarding incidence and survival rates of GCTs are lacking, hence, the necessity for research on GCTs in a South African setting.The aim is to critically review the management practices and outcomes of children with GCTs at Chris Hani Baragwanath Academic Hospital (CHBAH).
Methods
A retrospective review of records for patients younger than 16 years of age presenting with extra-cranial GCT (gonadal and extra-gonadal) between 1 January 2008 and 31 December 2017.
Results
Forty one patients were identified. Thirty two Extra-gonadal GCTs accounted for 78.05% of the patients, of which 27 were Sacrococcygeal Teratomas (SCTs), 2 thyroid, 1 adrenal, 1 uterine and 1 unspecified. Nine gonadal GCTs accounted for the remaining 21.95% , 7 ovarian and 2 testicular. The overall survival was 78.05%, with 25 patients (60.98%) being disease free, five patients (12.20%) having disease recurrence, two patients (4.88%) having residual disease, and four patients (9.76%) having died. The deaths included 3 patients with ovarian tumours older than 10 years, and 1 SCT who was 1 year 8 months. Recurrence was noted in the SCTs, and residual disease in 1 ovarian and 1 SCT.
Conclusions
The overall survival rate of 78.05% is lower than in the US, and mortalities occurred in the older females with ovarian tumours, and a late presenting SCT. The reason for the high rate of recurrence in SCTs may be due to incomplete resection.