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Primary bilateral ovarian Burkitt lymphoma; a rare issue in gynecologic oncology

Primary bilateral ovarian Burkitt lymphoma; a rare issue in gynecologic oncology We report a rare case of 42-year-old female patient with bilateral primary ovarian Burkitt lymphoma (BL). Primary BLs are rare ovarian oncology neoplasms. Only few cases are reported in English literature, most of them are presented in pediatric and young adult age groups. Rare cases are reported in adult females. Although BL is a rare primary ovarian neoplasm, it is very important to establish an accurate diagnosis as early as possible for therapeutic purposes. immunodeficiency, HIV, and post organ transplant [3]. BL seen INTRODUCTION commonly in boys with average ratio male: female (1.3:1). The In 1958, Dennis Burkitt, a British surgeon, described a childhood average age at presentation is 8 years old (range: 0–20 years) [3]. tumor while working in Uganda characteristically occurs Overall, primary bilateral BL is a rare diagnosis in adults. In this among African children. Back then, the tumors appeared to be article we present a rare case of bilateral primary ovarian BL limited to the warm wet tropics of Africa and Papua New with its histopathology, immunohistochemistry and FISH Guinea [1]. Burkitt lymphoma (BL) is a malignant non-Hodgkin analysis. lymphoma associated with c-MYC gene translocation and hea- vy locus immunoglobulin, resulting in the most common vari- CASE PRESENTATION ant translocation t (8;14) (q24;q32). BL commonly occurs in childhood age group; nevertheless, it is a rare presentation in A 42-year-old lady presented to the emergency department at adults as compared to low-grade B-cell lymphomas, diffuse our hospital complaining of continuous abdominal pain and a large B-cell lymphoma. The World Health Organization (WHO) palpable abdominal mass. No associated symptoms were iden- classification of lymphoid neoplasms sustained the BL subtypes tified. Physical examination demonstrated tender enlarged into three subtypes as follows: endemic BL, sporadic BL and abdomen. Her blood work was unremarkable, except for high immunodeficiency-associated BL [2]. The endemic BL is charac- platelet level (480 × 10 /L). Abdominal and pelvic computed teristically associated with Epstein–Barr virus (EBV) infection. tomography (CT) scan with oral and IV contrast revealed large Sporadic BL occurs in the second and third decades of life, com- bilateral lobulated soft tissue heterogeneous adnexal masses monly in Western Europe and North America, can involve with some cystic components. There was an evidence of vascu- extra-nodal sites such as intestinal tract, mesentery, and more lar enhancement within both adnexal masses. The largest commonly at the ileocecal junction. The latter is frequently mass (9.2 × 11.4 cm ). No radiological evidence of cystic rupture seen in the pediatric population probably due to high lymphoid or hemorrhage were seen within both masses. The uterus was tissue compartment in this area [3]. Immunodeficiency- displaced by both adnexal masses. Both ovaries could not be associated BL mostly detected in patients with primary visualized (Fig. 1A). Bone marrow aspirate showed mild Received: March 8, 2018. Accepted: May 3, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy113/4999379 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 H. Al-Maghrabi and A. Meliti A B C D Figure 1: (A) CT scan with contrast revealed large bilateral lobulated heterogeneous adnexal masses (red arrows), displacing the uterus down (arrow head). (B) Gross photo showing homogenous white tan cut surface, focal areas of hemorrhage and necrosis are seen. (C) Histopathology examination demonstrates sheets of diffuse lymphoid infiltrate with prominent starry-sky appearance (H&E; ×20). (D) Ki67 proliferative index is almost 100% (×20). increase in megakaryocytes with no evidence of infiltrative pro- rearrangement. IGH/BCL2 [t (14; 18)] rearrangement was not cess. Chest x-ray and brain MRI scan were unremarkable for detected. All of the above ancillary studies were consistent with pathologic findings. the diagnosis BL. The patient planned to be treated with multi- The patient underwent total abdominal hysterectomy with agent chemotherapy R-CODOX and R-IVAC chemotherapy. Our bilateral salpingo-oophorectomy. The specimen sent for patho- patient currently is on her third cycle, doing well and alive. logic evaluation. We received two separate adnexal masses, the right adnexal tissue composed of a single solid capsulated mass with smooth tan lobulated cut surface weighing 564 g and meas- DISCUSSION uring 12 × 10 × 6cm . The left adnexal tissue composed of a sin- Primary ovarian lymphoma (POL) is defined as a lymphoma gle solid mass weighing 1040 g and measuring 15 × 10 × 7cm , that originates in the ovary and lacks other manifestations of with homogenous white tan lobulated cut surface, focal areas of lymphoproliferative disorders elsewhere. POL is a very rare hemorrhage and necrosis were seen (Fig. 1B). Microscopic exam- entity in ovary due to absence of lymphoid tissue within the ination of the sections taken from both masses revealed sheets ovary. Primary ovarian non-Hodgkin’s lymphoma accounts of diffuse lymphoid infiltrate composed of medium sized only for 0.5 and 1.5% of all ovarian neoplasms. A number of lymphoid cells with minimal amphophilic cytoplasm, promin- theories looked at the pathogenesis and the origin of POL, some ent basophilic nucleoli, coarse chromatin and thick nuclear of them suggested that it originates from the lymphocytes sur- membrane. Prominent starry sky pattern with numerous rounding the blood vessels in the hilum, and those near the mitosis and apoptosis seen (Fig. 1C). By immunohistochemistry, corpus luteum [4]. Others claim it’s the reactive lymphocytes the tumor cells revealed positive immunoreactivity against secondarily involved in the inflammatory response related to CD79a, CD20, PAX-5, CD10, BCL6 while negative for BCL2, CD3, pelvic inflammatory disease, endometriosis, or autoimmune CD5, CD21, CD23, CD1a, TdT and Cyclin D1. Ki67 proliferative disease that undergo a malignant transformation. There’s only index of 100% expressed in the tumor cells (Fig. 1D). Flow study 50 cases reported of primary non-Hodgkin lymphoma, 21 are result showed small lymphoid population expressing CD19, cases confirmed histopathologically as primary BL with average CD20, CD22 and CD10. In the meantime, FISH interpretation age 6–62-year-old (mean age 27.6 years). revealed positive BCL6 rearrangement and c-MYC (8q24) Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy113/4999379 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Primary bilateral ovarian Burkitt lymphoma 3 Ovarian involvement by lymphoma can be primary or sec- cytogenetically. Early diagnosis and therapy of BL is associated ondary. The secondary involvement either as an early metasta- with better prognosis and survival rate. sis in occult extra-ovarian neoplasms, or as a generalized metastatic diseases [5]. In 1988, Fox et al. [6] and Skodras et al. CONFLICT OF INTEREST STATEMENT [7] suggested a diagnostic criteria for POL as follows: (i) lymph- None declared. oma should be clinically confined to the ovary, adjacent lymph nodes or adjacent ovarian structure caused by direct spread, (ii) preoperative work up like bone marrow aspiration and periph- REFERENCES eral blood smear should be negative, and (iii) metastatic lymph- 1. Stepniak A, Czuczwar P, Szkodziak P, Wozniakowska E, oma should appear at least months after primary ovarian Wozniak S, Paszkowski T. Primary ovarian Burkitt’s lymph- involvement (Paladugu et al. [8] suggested 60 months after the oma: a rare oncological problem in gynaecology: a review of diagnosis established). Top differential diagnoses of BL in literature. Arch Gynecol Obstet 2017;296:653–60. adults would include diffuse large B-cell lymphoma, follicular 2. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert lymphoma, small cell carcinoma, adult granulosa cell tumor R, et al. The 2016 revision of the World Health Organization and dysgerminoma. Histopathology examination stained by classification of lymphoid neoplasms. Blood 2016;127:2375–90. hematoxylin and eosin stain (H&E), and immunohistochemis- 3. Khan WAZ, Deshpande KA, Kurdukar M, Patil P, Mahure H, try studies will show the characteristic features of BL. All of Pawar V. Primary Burkitts’s lymphoma of endometrium these findings were typically expressed in our case as described and bilateral ovaries in a 6‐year‐old female: report of a rare previously. All types of BL show translocation between c-MYC entity and review of the published work. J Obstet Gynaecol oncogene on chromosome 8 and one of the immunoglobulin Res 2013;39:1484–7. genes, commonly the heavy chain gene on chromosome 14. 4. Crawshaw J, Sohaib. SA, Wotherspoon A, Shepherd JH. Less commonly the κ light chain gene on chromosome 2 or the Primary non-Hodgkin’s lymphoma of the ovaries: imaging λ light chain gene on chromosome 22 [9]. findings. Br J Radiol 2007;80:e155–8. It is very important to differentiate primary from secondary 5. Crasta JA, Vallikad E. Ovarian lymphoma. Indian J Med ovarian lymphomas. The prognosis and the survival rate of POL Paediatr Oncol 2009;30:28. are variable depending on the stage at diagnosis. A 5-year sur- 6. Fox H, Langley FA, Govan ADT, Hill AS, Bennett MH. vival rate in POL is 80%, while secondary cases are only 33% Malignant lymphoma presenting as an ovarian tumour: a [10]. POL have a better survival rate probably due to the early clinicopathological analysis of 34 cases. Br J Obstet Gynaecol presentation and early stage at diagnosis. The treatment 1988;95:386–90. depends on the risk-adapted approach by classifying the 7. Skodras G, Fields V, Kragel PJ. Ovarian lymphoma and serous patients into high-risk and low-risk groups depending on the carcinoma of low malignant potential arising in the same following criteria: tumor size (less or more than 10 cm), disease ovary. A case report with literature review of 14 primary stage (bone marrow involvement and CNS infiltration), patient ovarian lymphomas. Arch Pathol Lab Med 1994;118:647–50. performance status, and lactate dehydrogenase level. Multi- 8. Paladugu RR, Bearman RM, Rappaport H. Malignant lymph- agent chemotherapy is considered to be the most suitable oma with primary manifestation in the gonad a clinico- therapeutic option for patients with BL. pathologic study of 38 patients. Cancer 1980;45:561–71. 9. Rosai J, Ackerman S. Surgical Pathology. 8th edn. St. Louis: Mosby, 1996;1917–2020. CONCLUSION 10. Vang R, Medeiros LJ, Warnke RA, Higgins JP, Deavers MT. In conclusion, we present a unique case of bilateral primary Ovarian non-Hodgkin’s lymphoma: a clinicopathologic ovarian BL in a 42-year-old lady confirmed histologically and study of eight primary cases. Mod Pathol 2001;14:1093. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy113/4999379 by Ed 'DeepDyve' Gillespie user on 21 June 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Primary bilateral ovarian Burkitt lymphoma; a rare issue in gynecologic oncology

Journal of Surgical Case Reports , Volume Advance Article (5) – May 18, 2018

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Abstract

We report a rare case of 42-year-old female patient with bilateral primary ovarian Burkitt lymphoma (BL). Primary BLs are rare ovarian oncology neoplasms. Only few cases are reported in English literature, most of them are presented in pediatric and young adult age groups. Rare cases are reported in adult females. Although BL is a rare primary ovarian neoplasm, it is very important to establish an accurate diagnosis as early as possible for therapeutic purposes. immunodeficiency, HIV, and post organ transplant [3]. BL seen INTRODUCTION commonly in boys with average ratio male: female (1.3:1). The In 1958, Dennis Burkitt, a British surgeon, described a childhood average age at presentation is 8 years old (range: 0–20 years) [3]. tumor while working in Uganda characteristically occurs Overall, primary bilateral BL is a rare diagnosis in adults. In this among African children. Back then, the tumors appeared to be article we present a rare case of bilateral primary ovarian BL limited to the warm wet tropics of Africa and Papua New with its histopathology, immunohistochemistry and FISH Guinea [1]. Burkitt lymphoma (BL) is a malignant non-Hodgkin analysis. lymphoma associated with c-MYC gene translocation and hea- vy locus immunoglobulin, resulting in the most common vari- CASE PRESENTATION ant translocation t (8;14) (q24;q32). BL commonly occurs in childhood age group; nevertheless, it is a rare presentation in A 42-year-old lady presented to the emergency department at adults as compared to low-grade B-cell lymphomas, diffuse our hospital complaining of continuous abdominal pain and a large B-cell lymphoma. The World Health Organization (WHO) palpable abdominal mass. No associated symptoms were iden- classification of lymphoid neoplasms sustained the BL subtypes tified. Physical examination demonstrated tender enlarged into three subtypes as follows: endemic BL, sporadic BL and abdomen. Her blood work was unremarkable, except for high immunodeficiency-associated BL [2]. The endemic BL is charac- platelet level (480 × 10 /L). Abdominal and pelvic computed teristically associated with Epstein–Barr virus (EBV) infection. tomography (CT) scan with oral and IV contrast revealed large Sporadic BL occurs in the second and third decades of life, com- bilateral lobulated soft tissue heterogeneous adnexal masses monly in Western Europe and North America, can involve with some cystic components. There was an evidence of vascu- extra-nodal sites such as intestinal tract, mesentery, and more lar enhancement within both adnexal masses. The largest commonly at the ileocecal junction. The latter is frequently mass (9.2 × 11.4 cm ). No radiological evidence of cystic rupture seen in the pediatric population probably due to high lymphoid or hemorrhage were seen within both masses. The uterus was tissue compartment in this area [3]. Immunodeficiency- displaced by both adnexal masses. Both ovaries could not be associated BL mostly detected in patients with primary visualized (Fig. 1A). Bone marrow aspirate showed mild Received: March 8, 2018. Accepted: May 3, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy113/4999379 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 H. Al-Maghrabi and A. Meliti A B C D Figure 1: (A) CT scan with contrast revealed large bilateral lobulated heterogeneous adnexal masses (red arrows), displacing the uterus down (arrow head). (B) Gross photo showing homogenous white tan cut surface, focal areas of hemorrhage and necrosis are seen. (C) Histopathology examination demonstrates sheets of diffuse lymphoid infiltrate with prominent starry-sky appearance (H&E; ×20). (D) Ki67 proliferative index is almost 100% (×20). increase in megakaryocytes with no evidence of infiltrative pro- rearrangement. IGH/BCL2 [t (14; 18)] rearrangement was not cess. Chest x-ray and brain MRI scan were unremarkable for detected. All of the above ancillary studies were consistent with pathologic findings. the diagnosis BL. The patient planned to be treated with multi- The patient underwent total abdominal hysterectomy with agent chemotherapy R-CODOX and R-IVAC chemotherapy. Our bilateral salpingo-oophorectomy. The specimen sent for patho- patient currently is on her third cycle, doing well and alive. logic evaluation. We received two separate adnexal masses, the right adnexal tissue composed of a single solid capsulated mass with smooth tan lobulated cut surface weighing 564 g and meas- DISCUSSION uring 12 × 10 × 6cm . The left adnexal tissue composed of a sin- Primary ovarian lymphoma (POL) is defined as a lymphoma gle solid mass weighing 1040 g and measuring 15 × 10 × 7cm , that originates in the ovary and lacks other manifestations of with homogenous white tan lobulated cut surface, focal areas of lymphoproliferative disorders elsewhere. POL is a very rare hemorrhage and necrosis were seen (Fig. 1B). Microscopic exam- entity in ovary due to absence of lymphoid tissue within the ination of the sections taken from both masses revealed sheets ovary. Primary ovarian non-Hodgkin’s lymphoma accounts of diffuse lymphoid infiltrate composed of medium sized only for 0.5 and 1.5% of all ovarian neoplasms. A number of lymphoid cells with minimal amphophilic cytoplasm, promin- theories looked at the pathogenesis and the origin of POL, some ent basophilic nucleoli, coarse chromatin and thick nuclear of them suggested that it originates from the lymphocytes sur- membrane. Prominent starry sky pattern with numerous rounding the blood vessels in the hilum, and those near the mitosis and apoptosis seen (Fig. 1C). By immunohistochemistry, corpus luteum [4]. Others claim it’s the reactive lymphocytes the tumor cells revealed positive immunoreactivity against secondarily involved in the inflammatory response related to CD79a, CD20, PAX-5, CD10, BCL6 while negative for BCL2, CD3, pelvic inflammatory disease, endometriosis, or autoimmune CD5, CD21, CD23, CD1a, TdT and Cyclin D1. Ki67 proliferative disease that undergo a malignant transformation. There’s only index of 100% expressed in the tumor cells (Fig. 1D). Flow study 50 cases reported of primary non-Hodgkin lymphoma, 21 are result showed small lymphoid population expressing CD19, cases confirmed histopathologically as primary BL with average CD20, CD22 and CD10. In the meantime, FISH interpretation age 6–62-year-old (mean age 27.6 years). revealed positive BCL6 rearrangement and c-MYC (8q24) Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy113/4999379 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Primary bilateral ovarian Burkitt lymphoma 3 Ovarian involvement by lymphoma can be primary or sec- cytogenetically. Early diagnosis and therapy of BL is associated ondary. The secondary involvement either as an early metasta- with better prognosis and survival rate. sis in occult extra-ovarian neoplasms, or as a generalized metastatic diseases [5]. In 1988, Fox et al. [6] and Skodras et al. CONFLICT OF INTEREST STATEMENT [7] suggested a diagnostic criteria for POL as follows: (i) lymph- None declared. oma should be clinically confined to the ovary, adjacent lymph nodes or adjacent ovarian structure caused by direct spread, (ii) preoperative work up like bone marrow aspiration and periph- REFERENCES eral blood smear should be negative, and (iii) metastatic lymph- 1. Stepniak A, Czuczwar P, Szkodziak P, Wozniakowska E, oma should appear at least months after primary ovarian Wozniak S, Paszkowski T. Primary ovarian Burkitt’s lymph- involvement (Paladugu et al. [8] suggested 60 months after the oma: a rare oncological problem in gynaecology: a review of diagnosis established). Top differential diagnoses of BL in literature. Arch Gynecol Obstet 2017;296:653–60. adults would include diffuse large B-cell lymphoma, follicular 2. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert lymphoma, small cell carcinoma, adult granulosa cell tumor R, et al. The 2016 revision of the World Health Organization and dysgerminoma. Histopathology examination stained by classification of lymphoid neoplasms. Blood 2016;127:2375–90. hematoxylin and eosin stain (H&E), and immunohistochemis- 3. Khan WAZ, Deshpande KA, Kurdukar M, Patil P, Mahure H, try studies will show the characteristic features of BL. All of Pawar V. Primary Burkitts’s lymphoma of endometrium these findings were typically expressed in our case as described and bilateral ovaries in a 6‐year‐old female: report of a rare previously. All types of BL show translocation between c-MYC entity and review of the published work. J Obstet Gynaecol oncogene on chromosome 8 and one of the immunoglobulin Res 2013;39:1484–7. genes, commonly the heavy chain gene on chromosome 14. 4. Crawshaw J, Sohaib. SA, Wotherspoon A, Shepherd JH. Less commonly the κ light chain gene on chromosome 2 or the Primary non-Hodgkin’s lymphoma of the ovaries: imaging λ light chain gene on chromosome 22 [9]. findings. Br J Radiol 2007;80:e155–8. It is very important to differentiate primary from secondary 5. Crasta JA, Vallikad E. Ovarian lymphoma. Indian J Med ovarian lymphomas. The prognosis and the survival rate of POL Paediatr Oncol 2009;30:28. are variable depending on the stage at diagnosis. A 5-year sur- 6. Fox H, Langley FA, Govan ADT, Hill AS, Bennett MH. vival rate in POL is 80%, while secondary cases are only 33% Malignant lymphoma presenting as an ovarian tumour: a [10]. POL have a better survival rate probably due to the early clinicopathological analysis of 34 cases. Br J Obstet Gynaecol presentation and early stage at diagnosis. The treatment 1988;95:386–90. depends on the risk-adapted approach by classifying the 7. Skodras G, Fields V, Kragel PJ. Ovarian lymphoma and serous patients into high-risk and low-risk groups depending on the carcinoma of low malignant potential arising in the same following criteria: tumor size (less or more than 10 cm), disease ovary. A case report with literature review of 14 primary stage (bone marrow involvement and CNS infiltration), patient ovarian lymphomas. Arch Pathol Lab Med 1994;118:647–50. performance status, and lactate dehydrogenase level. Multi- 8. Paladugu RR, Bearman RM, Rappaport H. Malignant lymph- agent chemotherapy is considered to be the most suitable oma with primary manifestation in the gonad a clinico- therapeutic option for patients with BL. pathologic study of 38 patients. Cancer 1980;45:561–71. 9. Rosai J, Ackerman S. Surgical Pathology. 8th edn. St. Louis: Mosby, 1996;1917–2020. CONCLUSION 10. Vang R, Medeiros LJ, Warnke RA, Higgins JP, Deavers MT. In conclusion, we present a unique case of bilateral primary Ovarian non-Hodgkin’s lymphoma: a clinicopathologic ovarian BL in a 42-year-old lady confirmed histologically and study of eight primary cases. Mod Pathol 2001;14:1093. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy113/4999379 by Ed 'DeepDyve' Gillespie user on 21 June 2018

Journal

Journal of Surgical Case ReportsOxford University Press

Published: May 18, 2018

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