Juvenile granulosa cell tumor and high blood ca-125 levels in children with pseudo-meigs syndrome
Çocuklarda over kist ve tümörleri nadirdir. Yaklaşık olarak solit tümörlerin %1-2 kadarını oluşturur. Over tümörleri üç gruba ayrılır: epitelyal, embrionik ve sex kord stromal tumor. Sex kord stromal tümörler overin central parçasında primordial hücrel- erden oluşur. Biz iki yaşında meme büyümesi şikayeti ile gelen juvenile granüloza hücreli tümörü(JGHT) olan olguyu tespit ettik. Olguda büyük intraabdominal kitle, asit ve sağ hemitoraksta plevral efüzyon mevcuttu. Bu birliktelik Pseudo-Meigs sendromu idi. JGHT ile beraber olan Pseudo-Meigs sendromu oldukça nadir görülmektedir. Bu kadar küçük yaşta literatürde bulunmamaktadır. Sonuç olarak küçük çocukta olsa JGHT ile Pseudo-Meigs sendromu birlikteliği unutulmamalıdır.
Juvenile granüloza hücreli tümörü ve psödo meig sendromlu çocukda yüksek kan ca-125 düzeyi
Ovarian tumors and cysts are rarely seen in children. They constitute 1-2% of solid tumors among children. Over tumors are categorized into three main groups: epithelial, embrionic, and sex cord-stromal tumors. Sex cord-stromal tumors arise from primordial cell cords that are located in the center of the ovary during embriological development. We have detected a patient who was diagnosed with a juvenile granulosa cell tumor (JGCT) at two years of age after presenting with excessive breast enlarge- ment during the past month. She had a large mass in the abdomen, ascites, and marked pleural effusion in the right hemithorax. This association was Pseudo-Meigs syndrome. The association of JGCT and Pseudo-Meigs syndrome is a considerably rare event. Moreover, since the patient was the youngest among all previously reported cases in existing literature, our observations indicate that a juvenile granulosa cell tumor may occur at an early age.In conclusion, Pseudo-Meigs syndrome with JGCT shouldn't forget, even in young children.
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- 1. Raafat F, Klys H, Rylance G. Juvenile granulosa cell tumor. Pediatr Pathol 1990;10(4):617-23.
- 2.Powell JL, Johnson NA, Bailey CL, Otis CN. Management of advanced juvenile granulosa cell tumor of the ovary. Gynecol Oncol 1993;48(1):119-123.
- 3.Vassal G, Flamant F, Caillaud JM, Demeocq F, Nihoul- Fekete C, Lemerle J. Juvenile granulosa cell tumor of the ovary in children: a clinical study of 15 cases. J Clin Oncol 1988;6(6):990-5.
- 4.Meigs JV, Cass JW. Fibroma of the ovary with ascites and hydrothorax. Am J Obstet Gynecol 1937;33:249-67.
- 5.Meigs JV. Fibroma of the ovary with ascites and hydro- thorax: Meigs syndrome. Am J Obstet Gynecol 1954; 67: 962-85.
- 6. Dunn JS Jr, Anderson CD, Method MW. Hydropic degen- erating leiomyoma presenting as pseudo-Meigs syndrome with elevated CA 125. Obstet Gynecol 1998;92(4 Pt 2): 648-9
- 7. Chourmouzi D, Papadopoulou E, Drevelegas A. Magnetic resonance imaging findings in pseudo-Meigs' syndrome as- sociated with a large uterine leiomyoma: a case report. J Med Case Reports 2010 28;4:120.
- 8. Bridgewater JA, Rustin GJS. Pseudo-Meigs syndrome secondary to an ovarian germ cell tumor. Gynecol Oncol 1997;66:539-41.
- 9.Cotran RS, Kumar V, Collins T. The female genital tract. In: Cotran RS, Kumar V, Collins T, editors. Robbins patho- logic basis of disease. Philadelphia7 WB Saunders Co; 1999. p. 1035-91.
- 10. Lin JY, Angel C, Sickel JZ. Meigs syndrome with elevated serum CA 125. Obstet Gynecol 1992;80:5636.
- 11. Tae Soo Hahm, Ji Sun Ham, and Ji Yeong Kang. Unilateral massive hydrothorax in a gynecologic patient with pseu- do-Meigs syndrome. Korean J Anesthesiol 2010; 58(2): 202-6.
- 12. Jones III OW, Surwit EA. Meigs syndrome and elevated CA 125. Obstet Gynecol 1989;73:5201.
- 13. Timmerman D, Moerman P, Vergote I. Meigs syndrome with elevated serum CA 125 levels: Two case reports and review of the literature. Gynecol Oncol 1995;59:4058.
- 14. Leyva-Carmona M, Vázquez-López MA, Lendinez-Molinos F. Ovarian juvenile granulosa cell tumors in infants. J Pediatr Hematol Oncol 2009;31(4):304-6.
- 15. Capito C, Flechtner I, Thibaud E, et al. Neonatal bilateral ovarian sex cord stromal tumors. Pediatr Blood Cancer 2009;52(3):401-3.
- 16. Young RH, Dickersin GR, Scully RE. Juvenile granulosa cell tumor of the ovary. A clinicopathological analysis of 125 cases. Am J Surg Pathol 1984;8(8):575-96.
- 17. Kaur H, Bagga R, Saha SC, et al. Juvenile granulosa cell tumor of the ovary presenting with pleural effusion and ascites. Int J Clin Oncol 2009;14(1):78-81.
- 18. Papathanasiou K, Papageorgiou C, Tsonoglou D. A case of Meigs' syndrome with a gigantic granulosa ovarian tumor. Clin Exp Obstet Gynecol 1998;25(1-2):61-3.
- 19. Choi K, Lee HJ, Pae JC, et al. Ovarian granulosa cell tu- mor presenting as Meigs' syndrome with elevated CA125. Korean J Intern Med 2005;20(1):105-9.
- 20. Wolff AL, Ladd AP, Kumar M, Gunderman RB, Stevens J. Pseudo-Meigs syndrome secondary to ovarian germ cell tumor. J Pediatr Surg 2005;40(4):737-9.