Andrea Burgetova1, Zdenek Matejovsky2, Michal Zikan3, Jiri Slama3, Pavel Dundr4, Petr Skapa5, Kamila Benkova6, David Cibula3, Daniela Fischerova7. 1. Department of Radiology, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic. 2. Orthopedic Clinic, Bulovka Hospital, First Faculty of Medicine, Charles University, Czech Republic. 3. Gynecologic Oncology Center, Department of Obstetrics and Gynecology, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic. 4. Department of Pathology, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic. 5. Department of Pathology, Second Faculty of Medicine, Charles University, Czech Republic. 6. Department of Pathology, Bulovka Hospital, First Faculty of Medicine, Charles University, Czech Republic. 7. Gynecologic Oncology Center, Department of Obstetrics and Gynecology, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic. Electronic address: daniela.fischerova@seznam.cz.
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.
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.
Authors: Jad M El Abiad; Sarah M Robbins; Bernard Cohen; Adam S Levin; David L Valle; Carol D Morris; Nara L de Macena Sobreira Journal: Am J Med Genet A Date: 2020-03-07 Impact factor: 2.802
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