Juvenile Granulosa Cell Tumor in a 13-Year-Old Girl With Enchondromatosis (Oilier's Disease): A Case Report

doi:10.1016/S1083-3188(98)70135-4

Journal of Pediatric and Adolescent Gynecology

Volume 11, Issue 3, August 1998, Pages 147-150

https://doi.org/10.1016/S1083-3188(98)70135-4 Get rights and content

Abstract

Juvenile granulosa cell tumor (JGCT) of the ovary has been reported to occur rarely in conjunction with Ollier's disease. We report a case of a 13-year-old girl who was noted to have a large abdominal mass at the time of excision of a symptomatic enchondroma of the right femur. Subsequent laparotomy at a tertiary care children's medical center identified a JGCT confined to the right ovary. Review of the literature reveals eight previous cases of JGCT in patients with Ollier's disease. The pathophysiology and possible common causes of both disorders is discussed.

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Cited by (31)

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Citation Excerpt :
In addition to the risk of developing skeletal malignant lesions, Ollier patients also seem to have an increased risk for the development of nonskeletal malignancies, especially intracranial tumors of glial origin and juvenile granulosa cell tumor [3]. This brief report of a case of juvenile granulosa cell tumor associated with Ollier disease is only the 11th such case to be documented in the literature so far (Table 1) [6–15]. Moreover, to our best knowledge this is the second case published in literature where malignant transformation of enchondroma was documented in a patient with Ollier disease associated with granulosa cell tumor (GCT).
Ovarian juvenile granulosa cell tumor has an interesting association with multiple enchondromatosis (Ollier disease and Maffucci syndrome) and should be considered a leading diagnosis when an ovarian mass is found in young patients with these conditions. Besides the association with nonskeletal malignancies, there is a high risk of malignant transformation of enchondroma in chondrosarcoma as was also the case in this instance.
The report uses multiple images to document the representative and characteristic markers of multiple enchondromas in a 22-year-old patient with Ollier disease complicated by malignant transformation of chondrosarcoma and in whom the disease is associated with ovarian juvenile granulosa cell tumor of the right ovary.
It is important to recognize that when the female patient presents with enchondromatosis and a large unilateral multilocular-solid ovarian mass, the specific diagnosis of granulosa cell tumor can be made with high accuracy.
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Citation Excerpt :
Implicated genes in these syndromes include a receptor for parathyroid hormone and parathyroid hormone-related protein (PTHR1)81 and IDH1 and IDH2.82 An association between these enchondromatoses and juvenile granulosa cell tumors (JGCTs) of the ovary has been described.83–88 Most of the patients who develop JGCTs in the setting of MS/OD do so in the first decade of life and frequently present with precocious puberty due to estrogen production of the tumor.88
This review covers gynecologic manifestations that may occur in rare hereditary syndromes. Recent advances in disorders, such as hereditary leiomyomatosis, renal cell carcinoma syndrome and tuberous sclerosis complex, are discussed as well as lesions that occur in von Hippel-Lindau syndrome, nevoid basal cell carcinoma syndrome, Cowden syndrome, Ollier disease/Maffucci syndrome, and Carney complex. Characteristic clinicopathologic features of each of these syndromes are discussed with an emphasis on the key features that enable pathologists to identify patients at highest risk for these diseases.
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We report the second known case of Maffucci syndrome associated with an adrenal cortex tumor. Endocrine tumors have been reported in patients with multiple enchondromas, although the underlying mechanism of this combination is unknown. Maffucci syndrome is characterized by abnormalities of several mesodermal derivates. Therefore, routine evaluation for involvement of the adrenal cortex may be warranted to improve our knowledge of this syndrome and of its pathophysiology.
Maffucci syndrome and adrenal cortex tumor
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Juvenile Granulosa Cell Tumor in a 13-Year-Old Girl With Enchondromatosis (Oilier's Disease): A Case Report

Abstract

J Paediatr

Dyschondroplasie et tumeur de l’ovaire

Ann Pediatr

Enchondromatosis (Oilier's disease) and ovarian juvenile granulosa cell tumor

Cancer

Bilateral juvenile granulosa cell tumours associated with skeletal enchondromas

Aust NZ J Obstet Gynaecol

Juvenile granulosa cell tumor of the ovary in children: a clinical study of 15 cases

J Clin Oncol