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Retroperitoneal germ cell tumors: a clinical study of 12 patients
Antoine De Backer1, Gerard C Madern, Frans W J Hazebroek
1Department of Pediatric Surgery, Academic Hospital, Free University of Brussels, B-1090 Brussels, Belgium. antoine.debacker@az.vub.ac.be
Insights
Retroperitoneal germ cell tumors (GCTs) are rare in infants. Surgical removal can be complex, but outcomes are generally good for benign GCTs and malignant yolk sac tumors (YSTs) with appropriate treatment.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Developmental Biology
Background:
- Retroperitoneal germ cell tumors (GCTs) are rare in newborns and infants.
- Understanding their clinical presentation, treatment, and outcomes is crucial for effective management.
Observation:
- A retrospective review of 12 infants with retroperitoneal GCTs between 1974-2002.
- Diagnosis occurred antenatally, at birth, or in early infancy, often presenting as large abdominal masses.
- Associated chromosomal anomalies were noted in 3 patients.
Findings:
- Surgical removal of retroperitoneal GCTs presented significant challenges due to tumor size and adherence, leading to perioperative complications in 4 patients.
- Histologically, tumors included mature teratomas, immature teratomas, and malignant yolk sac tumors (YSTs).
- Nine survivors with benign tumors remain disease-free after a mean follow-up of 12 years; two patients with YSTs achieved remission.
Implications:
- Despite surgical risks, retroperitoneal GCTs in infants have favorable long-term outcomes with timely and appropriate treatment.
- Early diagnosis and management are key to improving survival rates for these rare pediatric malignancies.
- Further research into optimizing surgical techniques and adjuvant therapies for GCTs is warranted.
Purpose:
The aim of the study was to examine the clinical presentation, method(s) of treatment, complications, and results in newborns and infants with retroperitoneal germ cell tumors (GCTs).
Methods:
A retrospective chart review of all patients treated between 1974 and 2002 for GCT located in the retroperitoneum in 2 institutions identified 12 patients with histologically proven retroperitoneal GCT. Vital data concerning pregnancy and delivery were analyzed. Age at diagnosis and symptoms were recorded, as well as possibly associated anomalies. Data concerning surgical treatment, perioperative and postoperative complications, histological staging, and final outcome were all analyzed.
Results:
In 3 patients, the diagnosis had been made antenatally between 31 and 35 weeks of gestation. In 1 patient, the diagnosis was made at birth, and in 8 later in life (ages 3, 5, 7, 8, 8, 11, 18, and 24 months). Symptoms in these 8 boys and 4 girls were abdominal distension and a palpable upper abdominal mass, right-sided in 5, left-sided in 5, and central in 2; the tumor was usually big. Associated anomalies were noted in 4 patients and were chromosomal in 3 (Down syndrome in 2 and Klinefelter syndrome in 1). One baby died of uncontrollable bleeding during an emergency operation immediately after traumatic birth. The other 11 infants survived. Four other patients showed serious perioperative complications (1 caval vein tear, 1 choledochal tear, 1 cyst rupture, and 1 esophagogastric tear) which were managed without further consequences. Histologically, 4 tumors were mature teratomas, 6 were immature teratomas (grade I in 4, grade II in 1, and grade II-III in 1), and 2 were malignant yolk sac tumors (YSTs). The patients with YSTs underwent surgical biopsy, followed by chemotherapy and excision of the remaining tumor and of the metastases. No adjuvant treatment was administered in the patients with benign disease. Nine survivors with benign tumor are disease-free between 1 and 30 years after surgery. Two patients with YST have now been in remission for 6 and 5 years, respectively.
Conclusions:
Both this study and the literature review performed testify to the extreme rarity of GCTs in the retroperitoneum. Surgical removal of the tumors appeared to be hazardous because of the extent of the tumor, the displacement and elongation of adjacent structures and organs, and/or the adhesion of the tumor to surrounding tissues; this resulted in several perioperative complications. The long-term results are good, however, with 9 of 10 patients with benign tumors in good health after a mean follow-up of 12 years, and with the 2 patients with YST in remission for 6 and 5 years, respectively.