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Management of the Retroperitoneum In Children and Adolescents with Malignant Germ Cell Tumors of the Testis

Saturday, October 20, 2012: 11:51 AM
Grand Ballroom A/B (Hilton Riverside)
Jonathan H. Ross, MD, FAAP, Pediatric Urology, University Hospitals Rainbow Babies and Children's Hospital, Cleveland, OH, Deborah Billmire, MD, FAAP, Pediatric Surgery, JW Riley Hospital for Children, Indianapolis, IN, Frederick J. Rescorla, MD, FAAP, Pediatric Surgery, Indiana University, Indianapolis, IN, Thomas A. Olson, MD, Pediatric Hematology/Oncology, Emory University School of Medicine, Atlanta, GA, Marc G. Schlatter, MD, FACS, Pediatric Surgery, Helen DeVos Children's Hospital, Grand Rapids, MI and A. Lindsay Frazier, MD, ScM, Pediatric Hematology/Oncology, Dana-Farber Cancer Institute, Boston, MA

Purpose: To assess the current management of the retroperitoneum (RP) in children and adolescents with malignant testicular germ cell tumors (GCT). 

Methods: We reviewed the records of 98 boys with testicular GCT enrolled in the intergroup POG/ CCSG protocols from 1990-1996 regarding management of the RP, histologic findings, and outcome. 

Results: The age distribution for the patients was bimodal with all pure yolk sac tumors (YST) (n=57) occurring in boys under 10 years of age and all embryonal, seminoma, and mixed germ cell tumors (ESMGCT) (n=36) occurring in boys over 10 years old.

14 RP operations were performed in patients with YST before chemotherapy.  Available pathology revealed metastatic YST in 10 patients (lymph node sizes ranging from 1.8 – 6.2cm) and benign lymph nodes in 3 patients (all <=2.3cm).  In 3 RP operations performed in patients with YST following chemotherapy, 2 excised residual masses revealed necrosis and 1 lymph node sampling in a patient with a CR revealed benign lymph nodes.  AFP was available for 2 of these patients and was normal.  No patient with YST died.

11 RP operations were performed in patients with ESMGCT before chemotherapy all of which were positive for malignant GCT.  Lymph nodes measured 0.8 – 14cm in size.  14 ESMGCT patients had RP surgery following chemotherapy for a residual mass revealing 2 GCT, 5 teratomas, 6 necrosis, and 1 neurofibrosarcoma. 

 4 patients in the study died.  1 patient with neurofibromatosis developed a RP neurofibrosarcoma.  The other 3 had stage 4 ESMGCT.  1 of these patients received chemotherapy followed by biopsy of residual liver and RP masses.  Pathology revealed teratoma and 1 year later he had a total RPLND for a growing mass which revealed teratoma with undifferentiated blastic and sarcomatous elements . 

Conclusion: For prepubertal patients with YST, pre-chemotherapy RP surgery may be unnecessary as large nodes (> 2.3 cm in this study) can be assumed to be positive and small nodes presumed negative that progress can be salvaged with chemotherapy.  For adolescents with malignant GCT this limited data suggests that neither pre-chemotherapy biopsy of large nodes nor staging RPLND for clinical stage 1 disease are crucial for survival.  Surgical resection or biopsy of a post-chemotherapy RP mass is appropriate in adolescent patients because of the possibility of finding malignant GCT or teratoma.  One of the patient deaths suggests that a full RPLND may be appropriate for patient with RP teratoma.  Whether biopsy or observation is best for post-chemotherapy masses in YST patients is unknown – both patients in this study had necrotic tumor.  Consistent surgical approaches based on clearer guidelines in future protocols will allow better evaluation of how surgical management impacts outcomes and  the role of tumor markers and lymph node size in stratifying RP management.