Management challenges of ovarian adult-type granulosa cell tumors at a tertiary facility in resource-limited setting: A case series of three patients and review of the current literature

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Abstract

Introduction and importance

Adult-type granulosa cell tumors (AGCTs) are rare, low-grade malignant ovarian sex-cord stromal tumors, accounting for approximately 5 % of all ovarian tumors. These tumors are characterized by their potential for late recurrence and complex management, which presents significant challenges, particularly in resource-limited settings where access to advanced diagnostic tools and treatment options is restricted. This study highlights a series of rare AGCT cases diagnosed in premenopausal and postmenopausal women in Northern Tanzania. Given the global rarity of AGCTs and the limited literature on the condition, especially in this region, this study aims to address a critical knowledge gap.

Case series

This report presents a series of three patients diagnosed with ovarian adult-type granulosa cell tumors (AGCTs) based on histological findings. The series underscores the variability in clinical presentations and highlights the significance of individualized treatment approaches in managing these rare tumors.

Clinical discussion

Managing AGCTs in resource-limited settings requires balancing optimal care with available resources. Surgery remains the cornerstone of treatment; however, the lack of advanced diagnostic tools and limited access to adjuvant therapies necessitate a focus on achieving the best possible surgical outcomes and prioritizing rigorous clinical follow-up. This report explores the rarity of AGCTs, reviews existing literature on similar cases, and examines the numerous challenges associated with their management in such settings.

Conclusion

Adult granulosa cell tumors are rare, low-grade malignant neoplasms of the ovarian sex-cord stromal origin. These tumors are notable for their potential to recur even years after initial treatment. Consequently, regular follow-up with clinical examinations and tumor marker assessments is recommended. While chemotherapy can provide palliative benefits and manage recurrence, surgery remains the cornerstone of treatment, as illustrated in our case report.

Highlights

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Granulosa cell tumor (GCT) of the ovary is a rare indolent malignant neoplasm, often diagnosed at early stages.
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GCT should be considered in the differential diagnosis of solid / cystic and hemorrhagic ovarian mass in postmenopausal patients.
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Complete tumor removal is essential, as any remaining disease is associated with a worse prognosis.

Related collections

Most cited references 16

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Is Open Access

Preferred Reporting Of Case Series in Surgery (PROCESS) 2023 guidelines

Ginimol Mathew, Catrin Sohrabi, Thomas Franchi … (2023)

Introduction: The Preferred Reporting Of CasE Series in Surgery (PROCESS) guidelines were developed in 2016 in order to improve the reporting quality of surgical case series. Since its inception, it has been updated twice, in 2018 and 2020, and has been cited over 1000 times. PROCESS guidelines have enjoyed great acceptance within the surgical research community. Our aim is to update the PROCESS guidelines in order to maintain its applicability in the field of surgical research. Methods: A PROCESS 2023 steering group was created. By working in collaboration, members of this group came up with proposals to update the PROCESS 2020 guidelines. These proposals were presented to an expert panel of researchers, who in turn scrutinised these proposals and decided whether they should become part of PROCESS 2023 guidelines or not, through a Delphi consensus exercise. Results: A total of 38 people participated in the development of PROCESS 2023 guidelines. The majority of items received a score between 7 and 9 from greater than 70% of the participants, indicating consensus with the proposed changes to those items. However, two items (3c and 6a) received a score between 7 and 9 from less than 70% of the participants, indicating a lack of consensus with the proposed changes to those items. Those items will remain unchanged. Discussion: The updated PROCESS 2023 guidelines are presented with an aim to continue improving the reporting quality of case series in surgery.

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Granulosa cell tumor of the ovary.

S T Schumer, Stephen A. Cannistra (2003)

Adult granulosa cell tumor (GCT) of the ovary is oftentimes a hormonally active, stromal cell neoplasm that is distinguished by its ability to secrete sex steroids such as estrogen. Patients may present with vaginal bleeding caused by endometrial hyperplasia or uterine cancer as a result of prolonged exposure to tumor-derived estrogen. In addition, GCT is a vascular tumor that may occasionally rupture and result in abdominal pain, hemoperitoneum, and hypotension, mimicking an ectopic pregnancy in younger patients. GCT is usually associated with a mass on pelvic examination that is subsequently confirmed on ultrasonography. Surgery is required for definitive tissue diagnosis, staging, and tumor debulking. In older women, a total abdominal hysterectomy and bilateral salpingooophorectomy are typically performed. In women of childbearing age, a more conservative unilateral salpingo-oophorectomy may be performed, assuming that careful staging reveals that the disease has not extended outside of the involved ovary and that a concomitant uterine cancer has been excluded. Survival of patients with GCT is generally excellent because most patients present with early-stage disease, although certain high-risk patient groups may be identified. Stage is the most important prognostic factor, with a higher risk of relapse being associated with stages II through IV disease. In addition, patients with stage I disease associated with features such as large tumor size, high mitotic index, or tumor rupture may also be at higher risk in some series. The value of postoperative adjuvant therapy for high-risk patients has not been investigated by prospective randomized trials, which are difficult to perform because of the rarity of this tumor. Nonetheless, the use of adjuvant chemotherapy or radiation has sometimes been associated with prolonged disease-free survival in patients with high-risk features. Because of the propensity of GCT to recur years after initial diagnosis, prolonged surveillance with serial physical examination and serum tumor markers such as estradiol and inhibin is reasonable.

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Molecular pathogenesis of granulosa cell tumors of the ovary.

Stacey Jamieson, Peter J Fuller (2012)

Granulosa cell tumors of the ovary (GCT) comprise a distinct subset of ovarian cancers that account for approximately 5% of all ovarian malignancies. They are thought to arise from normal proliferating granulosa cells of the late preovulatory follicle and exhibit many morphological and biochemical features of these cells. GCT are distinct from other ovarian carcinomas in their hormonal activity; their ability to secrete estrogen, inhibin, and Müllerian inhibiting substance accounts for some of the clinical manifestations of the disease and also provides useful tumor markers for disease surveillance. Although considered to be of low malignant potential, GCT are commonly associated with slow, indolent disease progression, and frequent yet long delays to tumor recurrence are characteristic of this disease. Unlike the more intensely investigated epithelial ovarian tumors, relatively little is known about the molecular and genetic changes that give rise to GCT. To date, many investigations have centered around pathways known to be involved in normal granulosa cell proliferation, including those activated by FSH receptor stimulation. Most recently, the finding that approximately 97% of adult GCT harbor a somatic missense mutation in the FOXL2 gene (c.402C→G; p.C134W) represents an exciting advancement in the field of GCT research. The high frequency with which the mutation occurs in adult GCT, along with its absence from juvenile GCT and other human malignancies is suggestive of an oncogenic or gain-of-function mutation and, indeed, that the mutation is pathognomonic for adult GCT. In this review, we explore the implications of this finding and the most recent work characterizing molecular pathways of potential pathogenetic significance in GCT. Copyright © 2012 by The Endocrine Society

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Author and article information

Contributors

John Lugata

Journal

Journal ID (nlm-ta): Int J Surg Case Rep

Journal ID (iso-abbrev): Int J Surg Case Rep

Title: International Journal of Surgery Case Reports

Publisher: Elsevier

ISSN (Electronic): 2210-2612

Publication date PMC-release: 10 December 2024

Publication date Collection: January 2025

Publication date (Electronic): 10 December 2024

Volume: 126

Electronic Location Identifier: 110729

Affiliations

[a ]Department of Obstetrics and Gynecology, Kilimanjaro Christian Medical Centre, Moshi, Tanzania

[b ]Faculty of Medicine, Kilimanjaro Christian Medical University College, Moshi, Tanzania

[c ]Department of Pathology, Kilimanjaro Christian Medical Centre, Moshi, Tanzania

[d ]School of Clinical Medicine, University of Cambridge, Cambridge, United Kingdom

[e ]Kilimanjaro Clinical Research Institute, Moshi, Tanzania

Author notes

[* ]Corresponding author at: Department of Obstetrics and Gynecology, Kilimanjaro Christian Medical Centre, Box 3010, Moshi, Tanzania. lugataj06@ 123456gmail.com

Article

Publisher Item ID: S2210-2612(24)01510-4 Publisher ID: 110729

DOI: 10.1016/j.ijscr.2024.110729

PMC ID: 11697405

PubMed ID: 39667123

SO-VID: 87b68465-5ffd-4462-8cf1-152bb86979d7

License:

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

History

Date received : 13 November 2024

Date revision received : 2 December 2024

Date accepted : 9 December 2024