The association of enchondromatosis with malignant transformed chondrosarcoma and ovarian juvenile granulosa cell tumor (Ollier disease)

Taiwan J Obstet Gynecol. 2017 Apr;56(2):253-257. doi: 10.1016/j.tjog.2017.02.002.

Abstract

Objective: 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.

Case report: 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.

Conclusion: 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.

Keywords: Ollier disease; chondrosarcoma; enchondromatosis; juvenile granulosa cell tumor; ultrasound.

Publication types

  • Case Reports

MeSH terms

  • Abdominal Neoplasms / complications*
  • Abdominal Neoplasms / diagnostic imaging
  • Chondrosarcoma / complications*
  • Chondrosarcoma / diagnostic imaging
  • Enchondromatosis / complications*
  • Enchondromatosis / diagnostic imaging
  • Female
  • Granulosa Cell Tumor / complications*
  • Granulosa Cell Tumor / diagnostic imaging
  • Humans
  • Ovarian Neoplasms / complications*
  • Ovarian Neoplasms / diagnostic imaging
  • Tomography, X-Ray Computed
  • Ultrasonography
  • Young Adult