Journal of Research in Medical Sciences

: 2022  |  Volume : 27  |  Issue : 1  |  Page : 41-

Ollier's disease associated with ovarian juvenile granulosa cell tumor and triple X syndrome: A letter to the editor

Shamim Shafieyoon1, Mohammad Ali Tahririan2, Ali Hekmatnia1, Andrew Parviz Zarei3, Farzaneh Hekmatnia4, Ghazaleh Jamalipour Soufi1,  
1 Department of Radiology, Isfahan University of Medical Sciences, Isfahan, Iran
2 Department of Orthopedics, Isfahan University of Medical Sciences, Isfahan, Iran
3 Department of Medicine, The Princes Alexandra Hospital, London, UK
4 Department of Radiology, Lister Hospital, London, UK

Correspondence Address:
Dr. Ghazaleh Jamalipour Soufi
Department of Radiology, Isfahan University of Medical Sciences, Isfahan

How to cite this article:
Shafieyoon S, Tahririan MA, Hekmatnia A, Zarei AP, Hekmatnia F, Soufi GJ. Ollier's disease associated with ovarian juvenile granulosa cell tumor and triple X syndrome: A letter to the editor.J Res Med Sci 2022;27:41-41

How to cite this URL:
Shafieyoon S, Tahririan MA, Hekmatnia A, Zarei AP, Hekmatnia F, Soufi GJ. Ollier's disease associated with ovarian juvenile granulosa cell tumor and triple X syndrome: A letter to the editor. J Res Med Sci [serial online] 2022 [cited 2022 Jun 30 ];27:41-41
Available from:

Full Text

Dear Editor,

Ollier's disease, known as multiple enchondromatosis, is a rare nonhereditary benign bony lesion, which can be found in early childhood. Enchondromas frequently are developed in long tubular bones and flat bones. The lesions may affect multiple bones and are usually asymmetrically distributed, predominantly affecting one side of the body.[1] Radiologic features demonstrate multiple radiolucent, homogeneous density lesions with an oval or elongated shape and well-defined slightly thickened bony margins.[2],[3] The association of Ollier's disease and malignant transformation to chondrosarcoma has been described in 30%–50% of cases. Pancreatitis, anaplastic astrocytoma, and liver cancer have been reported, with less commonly.[4],[5] Malignant ovarian neoplasms and their association with Ollier's disease have rarely been described in the literature.

A 22-month-old female infant was referred to the orthopedic department of our hospital with left leg deformity and discrepancy in lower limb length. Radiologically, multiple lower limb and right upper limb enchondromas were found, and a diagnosis of Ollier's disease was established [Figure 1]. She was preterm, delivered at 32 weeks' gestation. A karyotype study was performed during the fetal period and triple X syndrome could be detected. At the age of 18 months, sudden progressive ascites were developed. Sonography revealed a large solid cystic mass with multilocular appearance and heterogeneous echogenicity, as well as internal vascularity. Furthermore, abdominopelvic computed tomography scan imaging revealed an enhanceable heterogeneous mass lesion in the right and central aspect of the abdominal cavity. Laparotomy and histopathological evaluations illustrated irregular-shaped cells in myxoid background, with basophilic secretions and high mitotic count in cell nuclei. Tumor cells expressed cytokeratin, calretinin, ki-67, inhibin, and WT1; a diagnosis of ovarian juvenile granulosa cell tumor (JGCT) was established.{Figure 1}

The association between two rare entities, Ollier's disease and JGCT, was reported. Ollier's disease affects unilateral limbs; however, we noted that left lower and right upper limbs could be affected by Ollier's disease. Another interesting aspect of this case report was triple X syndrome (Karyotype 47 XXX), and its association with Ollier's disease and JGCT was established for the first time. The fact that Ollier's disease can increase the risk of various neoplasms should be considered in managing these patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


The authors would like to thank the Departments of Radiology and Orthopedics, Kashani Hospital, Isfahan University of Medical Sciences.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


1Silve C, Jüppner H. Ollier disease. Orphanet J Rare Dis 2006;1:37.
2Suster D, Hung YP, Nielsen GP. Differential diagnosis of cartilaginous lesions of bone. Arch Pathol Lab Med 2020;144:71-82.
3Burgetova A, Matejovsky Z, Zikan M, Slama J, Dundr P, Skapa P, et al. The association of enchondromatosis with malignant transformed chondrosarcoma and ovarian juvenile granulosa cell tumor (Ollier disease). Taiwan J Obstet Gynecol 2017;56:253-7.
4Gajavelli S, Nakhla J, Nasser R, Yassari R, Weidenheim KM, Graber J. Ollier disease with anaplastic astrocytoma: A review of the literature and a unique case. Surg Neurol Int 2016;7:S607-11.
5Bonnet C, Thomas L, Psimaras D, Bielle F, Vauléon E, Loiseau H, et al. Characteristics of gliomas in patients with somatic IDH mosaicism. Acta Neuropathol Commun 2016;4:31.