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Mediastinal germ cell tumors: clinical aspects and outcomes in 7 children
A De Backer1, G C Madern, F G A J Hakvoort-Cammel
1Section of Pediatric Surgery, Academic Hospital, Free University of Brussels, Laarbeeklaan 101, 1090 Brussels, Belgium. antoine.debacker@az.vub.ac.be
Insights
Mediastinal germ cell tumors are rare in children. Benign tumors treated with surgery have excellent outcomes, while malignant tumors benefit from chemotherapy, improving survival rates.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Tumor Biology
Background:
- Mediastinal germ cell tumors (MGCTs) are exceptionally rare in pediatric populations.
- Limited literature exists on the clinical presentation and management of childhood MGCTs.
Purpose of the Study:
- To report on the clinical presentations, treatment modalities, complications, and outcomes of children diagnosed with mediastinal germ cell tumors.
- To analyze the prognostic factors and treatment effectiveness for pediatric MGCTs.
Main Methods:
- Retrospective chart review of 7 pediatric patients treated for MGCT between 1971 and 2001.
- Analysis of patient demographics, symptoms, surgical procedures, histological findings, chemotherapy regimens, and patient outcomes.
Main Results:
- Four patients with benign mature teratomas achieved complete remission after surgical excision, with no recurrences observed.
- Three patients with malignant tumors (yolk sac tumor, choriocarcinoma, malignant teratoma) received chemotherapy, with one survivor in remission.
- Common presenting symptoms included respiratory distress, persistent cough, chest pain, and weight loss.
Conclusions:
- Childhood MGCTs are rare and often present with severe symptoms.
- Complete surgical excision offers an excellent prognosis for benign mediastinal germ cell tumors.
- Platinum-based chemotherapy has significantly improved survival rates for malignant mediastinal germ cell tumors in children.
Background:
Mediastinal germ cell tumors presenting during childhood are extremely rare. Publications on this entity are very scarce. This paper reports on the clinical presentations, method(s) of treatment, complications, results and outcomes in a series of children with mediastinal germ cell tumors.
Methods:
A retrospective chart review of 7 children treated between 1971 and 2001 for mediastinal germ cell tumor was carried out. Age at diagnosis and symptoms were recorded. Each patient's surgical treatment, peri- and postoperative complications, histological staging and final outcome were analysed.
Results:
The median age of the 4 boys and 3 girls was 3 years (range 21 months-15 years). The most frequent symptoms were respiratory distress, persistent coughing, thoracic pain and anorexia/weight loss. Four patients had histologically benign tumors (mature teratoma). Their sole treatment consisted of complete surgical excision of the tumor and (part of) the thymus using either median sternotomy or left-sided thoracotomy. Recovery was uneventful. No recurrences have been observed. All four are alive with no evidence of disease, between 2.5 and 29 years after treatment. Malignant tumors were observed in three patients (1 yolk sac tumor, 1 choriocarcinoma and 1 malignant teratoma). Treatment consisted of either biopsy or debulking followed by chemotherapy (and radiotherapy in 1 case). Two of them died from uncontrollable metastatic disease. The patient with yolk sac tumor survived; he is now in remission, 4 years after diagnosis.
Conclusions:
Both this study and the literature review testify to the extreme rarity of mediastinal germ cell tumors in childhood. Children with this type of tumor usually are severely symptomatic. Histologically benign tumors carry an excellent prognosis provided surgical excision is complete. Histologically malignant tumors, on the other hand, have a worse prognosis. However, the use of platinum-based combination chemotherapy has considerably increased the survival rates.