PEDIATRIC SURGERY UPDATE ©

VOLUME 55 NO 01 JULY 2020

Ovarian Immature Teratoma

Ovarian teratomas in children are the most common germ cell tumors. Can be mature, immature and malignant. The vast majority of ovarian teratoma are mature. Ovarian immature teratomas (OIT) represent 1% of ovarian tumors graded according to the proportion of tissue containing immature neural elements. More than 80% of immature teratoma has elevated levels of alpha-fetoprotein. Tumors with higher AFP levels exhibit additional foci of malignant germ cell components. Peak incidence occurs between 15 and 19 years of age presenting as pelvic mass, abnormal uterine bleeding, abdominal pain or abdominal distension. US shows a complex ovarian lesion (solid and cystic components) or a heterogenous lesion in CT-Scan. Fat and scattered calcifications can also be present. Staging represents findings at surgery whether the tumor is confined to the ovary, peritoneum, pelvis, lymph node, adjacent organs, bilateral or has malignant ascites. Grade refers to pathologic presence of immature tissue in lower power field. Immature teratomas behave in a malignant fashion only if foci of malignant germ cell elements (yolk sac tumor) are present or if they are resected incompletely giving rise to the growing teratoma syndrome. Grade at diagnosis is the most important risk factor for relapse across all age groups. In children with grade 1 and 2 tumors there are no relapse regardless of stage. The majority of relapses (20%) occur in children with grade 3 tumors. Grade 3 with stage I/II disease have excellent free survival in comparison with stage III/IV. Completeness of resection influences free survival. Most children with OIT will not need chemotherapy. Grade, stage and completeness of resection are important risk factors for relapse. Recurrent disease occurs within the pelvis at the site of the original tumor. Tumor size does not correlate with tumor grade. The management of ovarian immature teratoma is unilateral salpingo-oophorectomy plus comprehensive staging. Complete resection is a key factor in avoiding tumor relapse.  Routine biopsy of the unaffected ovary is unnecessary because immature teratoma is almost always unilateral. Lymphadenectomy does not provide any significant benefit to the survival of patients affected by immature teratoma. The reason to remove the tube with the tumor is to reduce an ectopic pregnancy risk. Initial adjuvant chemotherapy does not reduce future relapse or progression in OIT. Ovarian-sparing surgery during tumorectomy is an option being studied and depends on the anatomic feasibility of each case. Adjuvant chemotherapy is use for residual or recurrent disease though it may cause growing teratoma syndrome. Children with OIT should be follow-up with serial US and AFP levels.

References:
1- Zhao T, Liu Y, Wang X, Zhang H, Lu Y: Ovarian cystectomy in the treatment of apparent early-stage immature teratoma. J Int Med Res. 45(2):771-780, 2017
2- Pashankar F, Hale JP, Dang H, et al: Is adjuvant chemotherapy indicated in ovarian immature teratomas? A combined data analysis from the Malignant Germ Cell Tumor International Collaborative. Cancer. 122(2):230-7, 2016
3- Luczak J, Baglaj M: Ovarian teratoma in children: a plea for collaborative clinical study. J Ovarian Res. 11(1):75, 2018
4- Frazer JL, Hook CE, Addley HC, et al: Recurrent ovarian immature teratoma in a 12-year-old girl: Implications for management. Gynecologic Oncology 154: 259-265, 2019
5- Shinkai T, Masumoto K, Chiba F, et al: Pediatric ovarian immature teratoma: histological grading and clinical characteristics. J Pediatr Surg 55: 707-710, 2020
6- Li S, Liu Z, Dong C, Long F, et al: Growing Teratoma Syndrome Secondary to Ovarian Giant Immature Teratoma in an Adolescent Girl: A Case Report and Literature Review. Medicine (Baltimore). 95(7):e2647, 2016

   Hepatic Hemangioendothelioma Revisited

*Edited by: Humberto Lugo-Vicente, MD, FACS, FAAP
P.O. Box 10426, Caparra Heights Station, San Juan, Puerto Rico 00922-0426
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Pediatric Surgery Update ISSN 1089-7739
Last updated: August 2020