Juvenile granulosa cell tumor associated with Ollier disease

Abstract

Juvenile granulosa cell tumor (JGCT) is a rare neoplasm of childhood. Interestingly, it is known to be associated with Ollier disease, which is a rare bone disease characterized by multiple enchondromatosis. There is paucity of literature about the co-occurence of these two conditions. However, this association is noteworthy because these two conditions share a common pathogenesis. We report a case of JGCT in a 2.5-year-old female child in which multiple enchondromas mimicking bony metastasis were an incidental finding during routine workup for tumor staging, thus leading to a diagnosis of Ollier disease.

Keywords: Enchondroma; Maffucci syndrome; Ollier disease; juvenile granulosa cell tumor.

Figure 1

Ovarian tumor with multiple small lobulated foci of increased signal intensity in the right femur

Figure 2

Lytic lesions in the right femur in the X-ray

Figure 3

Surgical removal of the ovarian tumor

Figure 4

High-power view of the granulosa cells with call–exner bodies (granulosa cells tend to form primitive follicle seen as empty spaces between granulosa cells)

Figure 5

Enchondromas