REVIEW article
Front. Oncol.
Sec. Pediatric Oncology
Management of ovarian Granulosa Cell Tumor in childhood: a case report and recommendations for a multidisciplinary approach
Provisionally accepted
Sofia Maria Carlotta Arnaboldi1*
Giovanna Gattuso2Maria Laura Nicolosi1Silvia Molinari1Laura Rachele Bettini1
Ernesto Leva3Paolo Passoni1Valentina Chiappa2Cecilia Daolio1
Adriana Balduzzi1
Maura Massimino2Monica Terenziani2
Alessandro Cattoni1- 1Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy
- 2Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
- 3Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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Abstract Juvenile Granulosa Cell Tumor (jGCT) is a rare subtype of pure sex-cord tumors, mostly affecting patients under 20 years of age. As this tumor originates from ovarian cells specialized in sex steroid secretion, jGCT can present with endocrine disorders, including precocious puberty in prepubertal girls or menstrual irregularities in postpubertal patients. In other cases, jGCT may manifest with symptoms related to pelvic mass effects or acute abdomen, prompting urgent gynecologic or surgical evaluation. Most patients are diagnosed with ovarian-confined disease and, for these patients, survival rates exceed 90% following surgery alone. However, advanced and relapsed disease remains a significant concern. As survival rates for cancer continue to improve, addressing survivorship care is essential. Long-term follow-up for patients diagnosed with jGCT in childhood requires a multidisciplinary approach. We hereby describe a clinical case of jGCT diagnosed in an infant girl, for whom a comprehensive multidisciplinary care plan was arranged. Through a comprehensive review of the literature, we developed a clinically applicable flowchart for multidisciplinary management of jGCT at diagnosis and during follow-up, emphasizing the need for patient-centered care that integrates the work of oncologists, endocrinologists, surgeons, gynecologists and geneticists.
Keywords: Sex-cord stromal tumor, Granulosa Cell Tumor, rare tumors management, Ovarian cancers, pediatric gynecologic cancers, cancer predisposing syndromes, precocious puberty, cancer survivorship care plan
Received: 23 May 2025; Accepted: 29 Oct 2025.
Copyright: © 2025 Arnaboldi, Gattuso, Nicolosi, Molinari, Bettini, Leva, Passoni, Chiappa, Daolio, Balduzzi, Massimino, Terenziani and Cattoni. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Sofia Maria Carlotta Arnaboldi, s.arnaboldi5@campus.unimib.it
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