Some characteristics of pediatric mesothelioma differed from its adult counterpart, including rare exposure to asbestos, different location of the primary tumor, and better prognosis, according to results of a study published in the European Journal of Cancer.1
Pediatric mesothelioma is rare and not well characterized. For adult mesothelioma, long-term survival is limited, and the primary treatment is pemetrexed-based chemotherapy with cytoreductive surgery. The purpose of this study was to characterize pediatric disease.
The retrospective study evaluated data from 33 patients diagnosed with mesothelioma at age 21 years or younger between 1987 and 2018. The median age was 14 years (range, 0.2-21.1 years), and 55% were female. Among patients with available data, 30% were initially misdiagnosed with other conditions malignant or nonmalignant conditions.
The majority of tumors had an epithelial histologic type (64%), followed by biphasic (12%) and unspecified (6%). The primary location of the tumor was peritoneal among 70% of patients, followed by pleural in 15%, multiple sites in 9%, and the vagina in 6%.
Distant metastases were present at diagnosis among 27% of patients.
Asbestos exposure in the home was present for 1 patient, and was unknown for 5 patients.
Among the 27 patients with malignant mesothelioma, the most common symptoms at diagnosis were abdominal enlargement, abdominal or thoracic pain, poor general status, and dyspnea. Among the 6 patients with multicystic mesothelioma of the peritoneum, the most common symptoms at diagnosis was abdominal pain, poor general status, abdominal enlargement, painful scrotal mass, anemia, and fever.
Cisplatin-pemetrexed chemotherapy was administered to 16 patients. Among the 12 evaluable patients, 6 achieved a partial response.
Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) was performed on 20 patients at least once. A complete response was achieved by 17 patients. The 5-year overall survival was 82.3% and the event-free survival was 45.1% during a median follow-up of 6.7 years.
The authors concluded that their results “confirm some clinical differences between childhood and adult mesotheliomas.”
Orbach D, André N, Brecht IB, et al. Mesothelioma in children and adolescents: the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) contribution. Eur J Cancer. 2020;140:63-70. doi:10.1016/j.ejca.2020.09.011