Bilateral ovarian primary Burkitt's lymphoma in a child: A clinico-radio-pathological evaluation
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0975-2870.153171
Ovarian neoplasms are rare in children. Though ovarian involvement by malignant lymphoma is known to occur in late stages of disseminated nodal disease, primary ovarian lymphoma is a rare entity. We came across a case of a 9-year-old female who came with complaints of dull aching abdominal pain, mainly in the suprapubic region, low grade fever and abdominal distension since 20 days. Ultrasonography revealed two well-defined, mixed echogenic masses in both adnexae. Multiple well-defined mixed echogenic lesions were also seen on the surface of liver with omental thickening and nodularity. Computerized tomography as well as magnetic resonance imaging was done for further evaluation. Ultrasound guided biopsy was performed. The diagnosis of Burkitt's lymphoma involving bilateral ovaries was established after histological, immunohistochemistry, and tumor markers correlation. Keywords: Burkitt′s lymphoma ovary, childhood ovarian tumors, primary ovarian lymphoma
Primary nonHodgkin's lymphoma of the ovary in a child is an extremely rare tumor, which accounts for <0.5% of all nonHodgkin's lymphomas and 1.5% of all ovarian neoplasms. [1] We present a case of bilateral ovarian Burkitt's lymphoma, in a 9-year-old girl. Primary Burkitt's lymphoma of the ovary is a very rare entity in nonendemic countries like India. [2] Patients with abdominal Burkitt's lymphoma usually present late as the symptoms are generally nonspecific until the advanced stage. [3] Distinguishing primary ovarian lymphoma from other pelvic tumors is difficult due to its rarity and it is often misdiagnosed biopsy and immunohistochemistry (IHC) are of utmost value. [4]
A 9-year-old female came with complaints of dull aching abdominal pain, mainly in the suprapubic region, abdominal distension, low grade fever since 20 days and weight loss (about 6 kg). Clinical examination revealed a palpable mass in the pelvic region and hepatomegaly. Hemogram was within normal limits. An ultrasonography of the abdomen and pelvis revealed two closely placed, mixed echogenic bilateral adnexal mass lesions posterior to the urinary bladder and displacing the uterus inferiorly. These measured about 5.1 cm × 3.2 cm and 5.2 cm × 3.5 cm, respectively [Figure 1]. The liver was grossly enlarged (16 cm). Multiple well-defined mixed echogenic lesions were seen involving right and left lobes of liver [Figure 2]. Omental and mesenteric thickening and nodularity were also present. Minimal ascites was noted. For further evaluation, contrast-enhanced computerized tomography and magnetic resonance imaging were done, which confirmed the findings noted on ultrasonography. Two closely placed, lobulated, soft tissue masses were noted in the pelvis posterior to the urinary bladder and extending into respective adnexae [Figure 3] and [Figure 4]. Ovaries were not seen separate from these masses. On contrast study, these masses showed near homogenous enhancement. No calcification or necrosis was seen. Bilateral lower ureters were compressed by these masses resulting in mild bilateral hydroureter and hydronephrosis.
The liver was enlarged. Multiple confluent and few discrete lobulated lesions of various sizes were seen along the surfaces of both lobes of the liver resulting in surface lobulations [Figure 4] and [Figure 5]. Largest lesion measured about 6.3 cm × 6 cm along left lobe. The lesions were hypodense on plain scan and showed mild homogenous contrast enhancement. No calcification or necrosis was seen. The lesions were causing indentations on the underlying liver parenchyma with displacement of the adjacent portal and hepatic venous radicals. The lesions were encasing and compressing the main portal vein and common bile duct at porta. Mild diffuse omental and mesenteric nodularity were seen [Figure 6]. Mild ascites was also noted [Figure 4].
A provisional diagnosis of primary ovarian malignancy with liver metastases was made. Differential diagnosis of pelvic neuroblastoma, primitive neuroectodermal tumor and embryonal rhabdomyosarcoma were considered. Blood investigations showed raised serum lactate dehydrogenase (LDH) levels (350 U/L) and raised serum CA125 levels (168 U/ml). Ultrasound guided biopsy of liver and pelvic lesions were done. Microscopically there were small round cells with hyperchromatic nuclei and scanty cytoplasm arranged in sheets and nests in desmoplastic stroma giving a characteristic starry sky appearance [Figure 7]. IHC was done for further evaluation which showed the presence of atypical lymphoid cells, which were positive for leucocyte common antigen (LCA) and vimentin and negative for chromogranin, synaptophysis, Mic-2, cytokeratin, epithelial membrane antigen, and desmin. The tumor cells also expressed CD20, PAX-5, and CD10 and were negative for CD3, B-cell lymphoma 2 (BCL-2) and terminal deoxynucleotidyl transferase.
Based on above findings a diagnosis of high grade nonHodgkin lymphoma of B-cell type suggestive of Burkitt's lymphoma was made. Our patient is presently receiving first cycle of chemotherapy in a higher cancer institute.
Burkitt's lymphoma is an extranodal disease occurring most often in children before 20 years of age. Burkitt's lymphoma can present in three forms namely endemic, sporadic (nonendemic), and immune deficiency related. In endemic cases jaw, facial bones, mesentery, and gonads are involved, whereas in nonendemic cases intra-abdominal disease is common involving ileum, caecum, mesentery, kidney, liver, and ovary. [4] Primary Burkitt's lymphomas affecting the female genital tract are very rare and are generally misdiagnosed on imaging studies. [5] The ovary is the most frequent site of nonHodgkin's lymphoma in the gynecologic tract accounting for about 0.5-1% of all ovarian neoplasms. [4],[6] Primary ovarian Burkitt's lymphomas are usually bilateral and are usually associated with ascites and peritoneal seedlings. Unilateral ovarian involvement is rare in Burkitt's lymphoma. [4] Clinical features are commonly secondary to compression, obstruction or infiltration of adjacent structures by tumor. Symptoms include abdominal pain, palpable mass, nausea, and vomiting, intestinal obstruction due to bowel compression. [5] Secondaries in the liver are seen in 14% of patients with nonHodkin's lymphoma. [7] Burkitt's lymphoma of the ovary is a rapidly growing tumor, and patients usually have high serum LDH levels. Serum tumor markers of CA125 are also raised. Microscopically it is composed of small noncleaved lymphoblastic cells and show a characteristic starry-sky pattern. [8] On IHC, the presence of positive staining for LCA in the histological specimen distinguishes malignant lymphoma from nonlymphoid neoplasm. Diffuse, large BCLs are positive for CD20 and BCL-6 and/or CD10 and/or BCL-2. [1] It is debatable whether lymphoma can arise de novo in the ovary. Lymphoid aggregates do exist in normal ovarian tissue, which could give rise to such lesions. [9] Imaging findings are nonspecific. Unilateral or bilateral cystic, solid or mixed pelvic masses are seen which are usually hypoechoic on ultrasonography. [8] On computerized tomography (CT) these are usually seen as mildly enhancing hypodense masses. Liver metastases are seen as multiple hypoechoic lesions on ultrasound, whereas on CT liver metastases are usually seen as low attenuation mildly enhancing masses. [8] Ascites and peritoneal seedlings are common findings that occur in 25% of patients with Burkitt's lymphoma. [8] On ultrasound peritoneal involvement appears as hypoechoic mesenteric masses or rarely in Burkitt lymphoma as omental cakes. [10] Computerized tomography findings of peritoneal involvement include nodularity along the peritoneal reflections and liver capsule with peritoneal ascites. [8] CT is the mainstay of lymphoma staging in the chest, abdomen, and pelvis as well as in other nodal lymphomas. Positron emission tomography with 18F-fluorodeoxyglucose has been reported as being a useful method for staging and for assessment of therapeutic response. [1] The tumor can be confused with other primary ovarian tumors as well as other pediatric pelvic masses. [5] The clue to differential diagnosis is the diffuse infiltration of the adjacent Fallopian tube More Details and/or broad ligament is much more common in lymphomas than in most of the other tumors. Burkitt's lymphoma responds well to chemotherapy. [3] The protocol of chemotherapy used in diffuse, large BCL is the standard CHOP regimen. Surgery is reserved for patients with unilateral involvement. [1]
Primary bilateral ovarian Burkitt's lymphoma is a rare tumor in children. It is important to differentiate between primary lymphoma affecting ovaries and secondary ovarian involvement by disseminated lymphoma. This distinction is of utmost importance because primary ovarian lymphomas have a better prognosis and outcome than secondary ovarian involvement by disseminated lymphoma. Primary ovarian lymphoma should be considered when bilateral ovarian involvement, ascites and peritoneal seedlings are present with LCA positivity and serum tumor markers of CA125 and LDH are high.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
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