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Ovarian and Testicular Sex Cord-Stromal Tumors

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Book cover Pediatric Germ Cell Tumors

Part of the book series: Pediatric Oncology ((PEDIATRICO,volume 1))

Abstract

Ovarian and testicular stromal tumors are uncommon but important tumors in children and young adults and may be associated with underlying genetic syndromes. Ovarian sex cord-stromal tumors include Sertoli-Leydig cell tumors, juvenile and adult granulosa cell tumors, and gynandroblastoma. Histologic evaluation and surgical staging should guide the management of such tumors; tumor markers play an important role in follow-up evaluations and should be obtained preoperatively when feasible. Testicular stromal tumors often behave in a benign fashion but can be aggressive depending upon age and histopathologic diagnosis. Treatment and outcome data are limited due to the small number of young patients with ovarian and testicular stromal tumors, but international registries have recently been established to collect such data and further our knowledge of these tumors.

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References

  • Acar C, Gurocak S, Sozen S (2009) Current treatment of testicular sex cord-stromal tumors: critical review. Urology 73:1165–1171

    Article  PubMed  Google Scholar 

  • Al-Agha OM, Axiotis CA (2007) An in-depth look at Leydig cell tumor of the testis. Arch Pathol Lab Med 131:311–317

    PubMed  Google Scholar 

  • Ball A, Wenning J, Van Eyk N (2011) Ovarian fibromas in pediatric patients with basal cell nevus (Gorlin) syndrome. J Pediatr Adolesc Gynecol 24:e5–e7

    Article  PubMed  Google Scholar 

  • Beggs AD, Latchford AR, Vasen HF et al (2010) Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut 59:975–986

    Article  CAS  PubMed  Google Scholar 

  • Bertram KA, Bratloff B, Hodges GF et al (1991) Treatment of malignant Leydig cell tumor. Cancer 68:2324–2329

    Article  CAS  PubMed  Google Scholar 

  • Bisogno G (2012) The EXPeRT initiative. In: Schneider DT (ed) Rare tumors in children and adolescents. Springer, Berlin/Heidelberg, pp 327–402

    Google Scholar 

  • Breen JL, Maxson WS (1977) Ovarian tumors in children and adolescents. Clin Obstet Gynecol 20:607–623

    Article  CAS  PubMed  Google Scholar 

  • Brown B, Ram A, Clayton P et al (2007) Conservative management of bilateral sertoli cell tumors of the testicle in association with the Carney complex: a case report. J Pediatr Surg 42:E13–E15

    Article  CAS  PubMed  Google Scholar 

  • Brown J, Shvartsman HS, Deavers MT et al (2005) The activity of taxanes compared with bleomycin, etoposide, and cisplatin in the treatment of sex cord-stromal ovarian tumors. Gynecol Oncol 97:489–496

    Article  CAS  PubMed  Google Scholar 

  • Brown J, Sood AK, Deavers MT et al (2009) Patterns of metastasis in sex cord-stromal tumors of the ovary: Can routine staging lymphadenectomy be omitted? Gynecol Oncol 113:86–90

    Article  PubMed  Google Scholar 

  • Costa MJ, Morris R, Sasano H (1994) Sex steroid biosynthesis enzymes in ovarian sex-cord stromal tumors. Int J Gynecol Pathol 13:109–119

    Article  CAS  PubMed  Google Scholar 

  • Featherstone JM, Fernando HS, Theaker JM et al (2009) Sex cord stromal testicular tumors: a clinical series–uniformly stage I disease. J Urol 181:2090–2096; discussion 2096

    Article  CAS  PubMed  Google Scholar 

  • Fefferman NR, Pinkney LP, Rivera R et al (2003) Sclerosing stromal tumor of the ovary in a premenarchal female. Pediatr Radiol 33:56–58

    Article  PubMed  Google Scholar 

  • Gershenson DM (1994) Management of early ovarian cancer: germ cell and sex cord-stromal tumors. Gynecol Oncol 55:S62–S72

    Article  CAS  PubMed  Google Scholar 

  • Gershenson DM, Morris M, Burke TW et al (1996) Treatment of poor-prognosis sex cord-stromal tumors of the ovary with the combination of bleomycin, etoposide, and cisplatin. Obstet Gynecol 87:527–531

    Article  CAS  PubMed  Google Scholar 

  • Gibbon DG (2005) Conservative management of sex cord tumors with annular tubules of the ovary in women with Peutz-Jeghers syndrome. J Pediatr Hematol Oncol 27:630–632

    Article  PubMed  Google Scholar 

  • Harms D, Kock LR (1997) Testicular juvenile granulosa cell and Sertoli cell tumours: a clinicopathological study of 29 cases from the Kiel Paediatric Tumour Registry. Virchows Arch 430:301–309

    Article  CAS  PubMed  Google Scholar 

  • Hassan E, Creatsas G, Deligeorolgou E et al (1999) Ovarian tumors during childhood and adolescence. A clinicopathological study. Eur J Gynaecol Oncol 20:124–126

    CAS  PubMed  Google Scholar 

  • Heravi-Moussavi A, Anglesio MS, Cheng SW et al (2012) Recurrent somatic DICER1 mutations in nonepithelial ovarian cancers. N Engl J Med 355:234–242

    Article  Google Scholar 

  • Hill DA, Ivanovich J, Priest JR et al (2009) DICER1 mutations in familial pleuropulmonary blastoma. Science 325:965

    Article  CAS  PubMed  Google Scholar 

  • Irving JA, Young RH (2009) Microcystic stromal tumor of the ovary: report of 16 cases of a hitherto uncharacterized distinctive ovarian neoplasm. Am J Surg Pathol 33:367–375

    Article  PubMed  Google Scholar 

  • Jamieson S, Butzow R, Andersson N et al (2010) The FOXL2 C134W mutation is characteristic of adult granulosa cell tumors of the ovary. Mod Pathol 23:1477–1485

    Article  CAS  PubMed  Google Scholar 

  • Kaplan GW, Cromie WJ, Kelalis PP et al (1986) Gonadal stromal tumors: a report of the Prepubertal Testicular Tumor Registry. J Urol 136:300–302

    CAS  PubMed  Google Scholar 

  • Kato N, Romero M, Catasus L et al (2004) The STK11/LKB1 Peutz-Jegher gene is not involved in the pathogenesis of sporadic sex cord-stromal tumors, although loss of heterozygosity at 19p13.3 indicates other gene alteration in these tumors. Hum Pathol 35:1101–1104

    Article  CAS  PubMed  Google Scholar 

  • Kim GJ, Georg I, Scherthan H et al (2010a) Dicer is required for Sertoli cell function and survival. Int J Dev Biol 54:867–875

    Article  CAS  PubMed  Google Scholar 

  • Kim T, Sung CO, Song SY et al (2010b) FOXL2 mutation in granulosa-cell tumours of the ovary. Histopathology 56:408–410

    Article  PubMed  Google Scholar 

  • Koonings PP, Campbell K, Mishell DR Jr et al (1989) Relative frequency of primary ovarian neoplasms: a 10-year review. Obstet Gynecol 74:921–926

    CAS  PubMed  Google Scholar 

  • Kratzer SS, Ulbright TM, Talerman A et al (1997) Large cell calcifying Sertoli cell tumor of the testis: contrasting features of six malignant and six benign tumors and a review of the literature. Am J Surg Pathol 21:1271–1280

    Article  CAS  PubMed  Google Scholar 

  • Merras-Salmio L, Vettenranta K, Mottonen M et al (2002) Ovarian granulosa cell tumors in childhood. Pediatr Hematol Oncol 19:145–156

    Article  PubMed  Google Scholar 

  • Mosharafa AA, Foster RS, Bihrle R et al (2003) Does retroperitoneal lymph node dissection have a curative role for patients with sex cord-stromal testicular tumors? Cancer 98:753–757

    Article  PubMed  Google Scholar 

  • Ozulker T, Ozpacaci T, Ozulker F et al (2010) Incidental detection of Sertoli-Leydig cell tumor by FDG PET/CT imaging in a patient with androgen insensitivity syndrome. Ann Nucl Med 24:35–39

    Article  PubMed  Google Scholar 

  • Ramirez PT, Schmeler KM, Milam MR et al (2008) Efficacy of letrozole in the treatment of recurrent platinum- and taxane-resistant high-grade cancer of the ovary or peritoneum. Gynecol Oncol 110:56–59

    Article  CAS  PubMed  Google Scholar 

  • Rio Frio T, Bahubeshi A, Kanellopoulou C et al (2011) DICER1 mutations in familial multinodular goiter with and without ovarian Sertoli-Leydig cell tumors. JAMA 305:68–77

    Article  CAS  PubMed  Google Scholar 

  • Rishi M, Howard LN, Bratthauer GL et al (1997) Use of monoclonal antibody against human inhibin as a marker for sex cord-stromal tumors of the ovary. Am J Surg Pathol 21:583–589

    Article  CAS  PubMed  Google Scholar 

  • Ross JH, Kay R (2004) Prepubertal testis tumors. Rev Urol 6:11–18

    PubMed  Google Scholar 

  • Ross JH, Rybicki L, Kay R (2002) Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: a summary of the Prepubertal Testis Tumor Registry. J Urol 168:1675–1678; discussion 1678–1679

    Article  PubMed  Google Scholar 

  • Rutgers JK (2011) The case reported as bilateral Sertoli-Leydig cell tumors in a 61-year-old woman with uterine aplasia may instead represent complete androgen insensitivity syndrome. Int J Gynecol Pathol 30:395

    Article  PubMed  Google Scholar 

  • Satoh M (1991) Histogenesis and organogenesis of the gonad in human embryos. J Anat 177:85–107

    CAS  PubMed  Google Scholar 

  • Schneider DT, Calaminus G, Wessalowski R et al (2002) Therapy of advanced ovarian juvenile granulosa cell tumors. Klin Padiatr 214:173–178

    Article  CAS  PubMed  Google Scholar 

  • Schneider DT, Calaminus G, Wessalowski R et al (2003a) Ovarian sex cord-stromal tumors in children and adolescents. J Clin Oncol 21:2357–2363

    Article  CAS  PubMed  Google Scholar 

  • Schneider DT, Janig U, Calaminus G et al (2003b) Ovarian sex cord-stromal tumors–a clinicopathological study of 72 cases from the Kiel Pediatric Tumor Registry. Virchows Arch 443:549–560

    Article  PubMed  Google Scholar 

  • Schneider DT, Terenziani M, Cecchetto G (2012) Gonadal and extragonadal germ cell tumors, sex cord stromal and rare gonadal tumors. In: Schneider DT (ed) Rare tumors in children and adolescents. Springer, Berlin/Heidelberg, pp 327–402

    Chapter  Google Scholar 

  • Schultz KA, Pacheco MC, Yang J et al (2011) Ovarian sex cord-stromal tumors, pleuropulmonary blastoma and DICER1 mutations: a report from the International Pleuropulmonary Blastoma Registry. Gynecol Oncol 122:246–250

    Article  CAS  PubMed  Google Scholar 

  • Schultz KA, Sencer SF, Messinger Y et al (2005) Pediatric ovarian tumors: a review of 67 cases. Pediatr Blood Cancer 44:167–173

    Article  PubMed  Google Scholar 

  • Shah SP, Kobel M, Senz J et al (2009) Mutation of FOXL2 in granulosa-cell tumors of the ovary. N Engl J Med 360:2719–2729

    Article  CAS  PubMed  Google Scholar 

  • Shukla AR, Huff DS, Canning DA et al (2004) Juvenile granulosa cell tumor of the testis: contemporary clinical management and pathological diagnosis. J Urol 171:1900–1902

    Article  PubMed  Google Scholar 

  • Sivathondan Y, Salm R, Hughesdon PE et al (1979) Female adnexal tumour of probable Wolffian origin. J Clin Pathol 32:616–624

    Article  CAS  PubMed  Google Scholar 

  • Slade I, Bacchelli C, Davies H et al (2011) DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome. J Med Genet 48:273–278

    Article  CAS  PubMed  Google Scholar 

  • Stacher E, Pristauz G, Scholz HS et al (2010) Bilateral ovarian well-differentiated Sertoli-Leydig cell tumors with heterologous elements associated with unilateral serous cystadenoma–a case report. Int J Gynecol Pathol 29:419–422

    Article  PubMed  Google Scholar 

  • Sumi T, Ishiko O, Maeda L et al (2002) Adenocarcinoma arising from respiratory ciliated epithelium in mature ovarian cystic teratoma. Arch Gynecol Obstet 267:107–109

    Article  CAS  PubMed  Google Scholar 

  • Tanaka Y, Sasaki Y, Nishihira H et al (1992) Ovarian juvenile granulosa cell tumor associated with Maffucci’s syndrome. Am J Clin Pathol 97:523–527

    CAS  PubMed  Google Scholar 

  • Thomas JC, Ross JH, Kay R (2001) Stromal testis tumors in children: a report from the prepubertal testis tumor registry. J Urol 166:2338–2340

    Article  CAS  PubMed  Google Scholar 

  • Ulbright TM, Amin MB, Young RH (2007) Intratubular large cell hyalinizing sertoli cell neoplasia of the testis: a report of 8 cases of a distinctive lesion of the Peutz-Jeghers syndrome. Am J Surg Pathol 31:827–835

    Article  PubMed  Google Scholar 

  • Vaz RM, Turner C (1986) Ollier disease (enchondromatosis) associated with ovarian juvenile granulosa cell tumor and precocious pseudopuberty. J Pediatr 108:945–947

    Article  CAS  PubMed  Google Scholar 

  • Velasco-Oses A, Alonso-Alvaro A, Blanco-Pozo A et al (1988) Ollier’s disease associated with ovarian juvenile granulosa cell tumor. Cancer 62:222–225

    Article  CAS  PubMed  Google Scholar 

  • White LC, Buchanan KD, O’Leary TD et al (2003) Direct laparoscopic venous sampling to diagnose a small Sertoli-Leydig tumor. Gynecol Oncol 91:254–257

    Article  CAS  PubMed  Google Scholar 

  • Young RH (2005) Sex cord-stromal tumors of the ovary and testis: their similarities and differences with consideration of selected problems. Mod Pathol 18(Suppl 2):S81–S98

    Article  PubMed  Google Scholar 

  • Zheng W, Senturk BZ, Parkash V (2003) Inhibin immunohistochemical staining: a practical approach for the surgical pathologist in the diagnoses of ovarian sex cord-stromal tumors. Adv Anat Pathol 10:27–38

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Hematology and Oncology, Children’s Hospitals and Clinics of Minnesota, Minneapolis, MN, USA

    Kris Ann P. Schultz MD, MS

  2. Department of Pediatric Oncology, Dana Farber Cancer Institute, Boston, MA, USA

    Lindsay Frazier MD

  3. Department of Pediatrics, Klinikum Dortmund, Dortmund, Germany

    Dominik T. Schneider MD

Authors
  1. Kris Ann P. Schultz MD, MS
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  2. Lindsay Frazier MD
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  3. Dominik T. Schneider MD
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Corresponding author

Correspondence to Kris Ann P. Schultz MD, MS .

Editor information

Editors and Affiliations

  1. Pediatric Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA

    A. Lindsay Frazier

  2. Dept. of Pediatrics, MolecularBiology an, UT Southewestern Medical Center, Dallas, Texas, USA

    James F. Amatruda

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Schultz, K.A.P., Frazier, L., Schneider, D.T. (2014). Ovarian and Testicular Sex Cord-Stromal Tumors. In: Frazier, A., Amatruda, J. (eds) Pediatric Germ Cell Tumors. Pediatric Oncology, vol 1. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38971-9_7

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  • DOI: https://doi.org/10.1007/978-3-642-38971-9_7

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  • Online ISBN: 978-3-642-38971-9

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