Case Report Bilateral Ovarian Burkitt's Lymphoma: Successful Treatment with Preservation of Ovarian Function Anselm Chi-wai Lee MBBS, FRCPCH 1 , *, Chan Hon Chui MBBS, FRCS 2 1 Children's Haematology & Cancer Centre, Mount Elizabeth Hospital, Singapore 2 Surgery Centre for Children, Mount Elizabeth Medical Centre, Singapore abstract Background: Bilateral ovarian Burkitt's lymphoma was diagnosed in a 10-year-old girl who presented with abdominal pain, constipation, and signs of ovarian torsion. Case: Left salpingo-oopherectomy and biopsy of the right ovary were carried out as initial surgical intervention. She remained well in first complete remission at 25 months after multiagent chemotherapy, with pubertal development on clinical examination, hormonal mea- surements, and pelvic sonography. Summary and Conclusion: Although rare, Burkitt's lymphoma should be included in the differential diagnosis of bilateral ovarian tumors in childhood, and more conservative surgical management is indicated to preserve ovarian function and fertility. Key Words: Burkitt's lymphoma, Fertility preservation, Non-Hodgkin lymphoma, Ovarian neoplasms Introduction Sporadic Burkitt's lymphoma involving both ovaries is an uncommon clinical entity but is repeatedly reported in the medical literature. 1e8 It is a diagnostic and therapeutic challenge, especially in the setting of an acute abdomen. A lymphomatous diagnosis is often not suspected in ovarian tumors, and there is a tendency for complete resection of the diseased organs during exploratory laparotomy. Given the highly successful outcomes of contemporary chemo- therapy for childhood B-cell non-Hodgkin lymphoma, 9 pe- diatric surgical teams should consider the possibility of non-Hodgkin lymphoma in children presenting with bilat- eral ovarian masses and resort to less mutilating surgery to preserve ovarian function. To the best of our knowledge, the girl described in the following report is the first child who has recovered with an intact and functioning ovary. Case A 10-year-old girl of Chinese descent presented with acute abdominal pain associated with increasing con- stipation. A lower abdominal mass was detected at the local hospital, and computed tomography revealed multiple masses on both sides of the pelvis (Fig. 1A and B). Physical examination at our institution showed a prepubertal child in distress with abdominal pain, suggestive of ovarian tor- sion. At emergency laparotomy, bilateral adnexal masses were found (Fig. 1C). A left salpingo-oopherectomy was first carried out. Following the frozen section, which showed the presence of a small round cell tumor, a biopsy sample was taken of the left adnexal mass. There was no tumor spillage during the operation. The final histopathologic features and immunocytochemistry confirmed the diagnosis of Burkitt's lymphoma. Positron emission tomography showed meta- bolically avid lesions in para-aortic lymph nodes and in the right maxilla. The bone marrow and cerebrospinal fluid were clear of malignancy. Chemotherapy based on an LMB89 protocol 9 was commenced 10 days postoperatively (Table 1). She completed the treatment uneventfully in 13 weeks. Repeat positron emission tomography at 6 weeks and after the completion of treatment showed the disease in continuous remission. At 25 months of follow-up, she remained disease free with bilateral breast development, increased volume in the remaining ovary with follicles on sonography, and serum estradiol (211.0 pmol/L) and gonadotropin levels (follicular stimulating hormone, 5.0 IU/L, luteinizing hormone, 0.67 IU/L) in the pubertal ranges. Summary and Conclusion Lymphomas account for 10% of childhood malignancies and are one of the most curable cancers. In Nigeria and probably parts of Africa where the endemic form of Burkitt's lymphoma prevails, about half of the ovarian tumors seen in patients under 20 years of age are Burkitt's lymphoma. 10 In other parts of the world, non-Hodgkin lymphoma that primarily affects the ovary is rare. 11 Osborne and Robboy 12 studied 42 cases of ovarian lymphoma and noted 16 pa- tients were younger than 20 years. Among them, 10 (63%) had Burkitt's or Burkitt-like lymphoma. Interestingly, lym- phoma affecting the ovaries tended to be bilateral (55%) in their series. The authors indicate no conflicts of interest. * Address correspondence to: Dr. Anselm Chi-wai Lee, MBBS, FRCPCH, Children's Haematology & Cancer Centre, Mount Elizabeth Hospital, Level 4, 3 Mount Elizabeth, Singapore 228510; Phone: þ65-67312673; fax: þ65-62354924 E-mail address: anselm.cw.lee@gmail.com (A.-w. Lee). 1083-3188/$ - see front matter Ó 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2014.09.013