Pelvic Burkitt lymphoma simulating ovarian cancer
Primitive ovarian lymphomas are rare tumours (1.5% of ovarian cancers) that are in the form of a painful pelvic mass that can be perfectly confused with an ovarian epithelial tumor. The average age of occurrence is 40 years. Burkitt lymphomas, sporadic in the west, are classically presented with abdominal localization. Biological analysis and imaging can help with diagnosis. The prognosis depends on the associated medullary and Neuromeningitis. The treatment is based on polychemotherapy. We report a case of abdominal stage III Burkitt lymphoma in a young woman simulating advanced bilateral ovarian cancer.
Abstract
Pelvic Burkitt lymphoma mimicking an ovarian tumor.
Primary malignant lymphoma of the ovary is a rare tumor (1.5% of ovarian tumors). Clinically, it occurs as an abdominal mass with pain which can be confused with an epithelialic ovary tumor. Most tumors are observed during the fourth decade. Burkitt lymphoma, is sporadic in western countries, usually with abdominal involvement. Diagnosis is guided by biological analysis and imaging. Prognosis depends on medullary and neuromeningeal involvement. Polychemotherapy is required. We report a case of a young woman who developed abdominal Burkitt Lymphoma mimicking advanced-stage bilateral ovarian cancer.
Key words: Ovarian lymphoma, Burkitt lymphoma, primary ovarian lymphoma, Burkitt lymphoma treatment, MRI
Keywords: Ovarian lymphoma, Primary Ovarian lymphoma, Ovarian Burkitt lymphoma, treatment of Burkitt's lymphoma, MRI among ovarian lymphomas (1.5% of ovarian cancers), Burkitt lymphomas are even rarer, especially in young women. Their frequency increases in patients carrying the human immunodeficiency virus. The abdominal locations of this type of lymphoma are classic. In sporadic forms, it is essentially a ileocecal-cecal tumor. There is peripheral ganglionic damage in 10 to 20% of cases. In endemic forms, digestive impairment is present only in 60% of cases. It is more diffuse, with peritoneal and retroperitoneal (renal, mesenteric and ovarian) interference. The diagnosis of ovarian localization is often done retrospectively during the Anatomo-pathological examination of the operative part, whereas, of course, the surgery is not the reference treatment.
The diagnosis of Ovarian Burkitt lymphoma imposes a balance in search of neuroméningé (40% of cases) and medullary (15 to 20% of cases) flooding on the prognosis.
Their clinical presentation must therefore be known in order to avoid inappropriate treatment. Their prognosis has improved with the progress of chemotherapy which is the reference treatment.
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Observation
Ms. F., 29 years old, Nulligeste, had pelvic postherpetic that had been evolving for three months. She abruptly presented a pain in the right iliac pit gradually spreading to the rest of the pelvis with abdominal defence. Pelvic ultrasound found the presence of several semi-liquid masses, with a small peritoneal effusion, most likely corresponding to an ovarian lesion up to the level of the umbilicus. The ASP was uncharacteristic. The biological checkup and the tumor markers were normal. The volume of the mass counter-indicating a laparoscopy, the indication of laparotomy was laid. The exploration of the abdomen allowed to find a adherent mass between the cecum and the anterior wall of the bladder leaving the necrotic content deaf; A large right ovarian mass with torsion of the pedicle Annexiel; A mass developed at the expense of the left ovary enclosed in the cul-de-sac of Douglas, the necrotic aspect of which imposed resection before the result of the extemporaneous examination for lymphoma. The remainder of the abdominal exploration did not reveal ascites, peritoneal or hepatic nodules.
The right annex (396 g), the left annex (360 g) and the appendicular mass (60 g) were invaded by a whitish-looking tumor tissue, fish flesh with many hemorrhagic zones. In microscopy, the ovaries and the appendage were invaded by medium-sized cells with an ovoid round-shaped nucleus of a somewhat irregular contour, containing an improperly distributed granular chromatin in which one could spot Sometimes one or more nucleoli. Mitotic activity was important. The cells, which were not cohesive among themselves, associated themselves with many macrophages, thus achieving an aspect said in starry skies. The Immunohistochemical Study: (CD20 +, CD3 –, CD5 –, CD23 –, CD10 +, Bcl2 –, Mib1 +, Bcl6 + Low, LMP1 –) enabled the diagnosis of Burkitt lymphoma.
The search for EBV by in situ hybridization was negative. Serology for Ebstein Bar virus (EBV) and HIV were negative.
Ms. F. Therefore presented abdominal stage III Burkitt lymphoma without meningeal or medullary infiltration. Its treatment consisted of three cycles of CYM type chemotherapy: methotrexate 3 grams per m2 at J1, then Aracytine 100 milligrams/m2 from J2 to J6, with lumbar puncture therapy (methotrexate 15 milligrams, Aracytine 40 milligrams, Dépomédrol 40 milligrams). She was in full remission six months after the end of her treatment.
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Discussion
Ovarian lymphomas are made possible by the presence of lymphocyte aggregates at the ovary level, either physiologically or in response to inflammatory or autoimmune phenomena [1].
Primary ovarian lymphomas account for 0.5% of non-Hodgkin lymphomas and 1.5% of ovarian cancers [2]. Ovarian disease occurs most often in large B-cell lymphomas. The age of occurrence is variable according to the series, with an average age around 40 years [1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11] and thus more early than for epithelial cancers. They are bilateral in 41 to 71% of cases [4]. As with our patient, the clinical signs of call are not specific: pelvic postherpetic, abdominal distension, ascites, digestive occlusion, diffuse peritoneal nodules, and large tumours (5 – 13 cm).
Diagnosis of primary ovarian lymphoma meets specific criteria (localized only to ovaries) explaining their good prognosis compared to disseminated lymphomas reaching the ovaries, with a remission period of more than 60 months after Surgical treatment [6]. Our patient did not meet this definition because it associated a appendicular injury to ovarian damage.
Burkitt lymphomas account for 3 to 5% of the lymphomas in the west. They are due to an anomaly of the C-Myc oncogene. In the West, the EBV genome is found in episomal form only in 10-15% of cases. It was not highlighted in our patient. Burkitt lymphomas have an incidence of 20 to 40% in HIV + subjects [12]. Our patient was seronegative.
Early ovarian Burkitt lymphomas occur in young women and are usually bilateral and associated with ascites [5]. At the biological level, an elevation of CA 125 and LDH is found. In our patient, LDH was 717 U/L 24 hours after the operation and CA 125 had not been completed.
The differential diagnoses to be considered are granulosa tumors, dysacroméliques tumors and metastatic locations. In Anatomo-Pathology, Burkitt lymphomas can be easily distinguished from other non-Hodgkin lymphomas and epithelial ovarian cancers.
[14] imaging can help with differential diagnosis between the two types of tumours [13]. In ultrasound, ovarian lymphomas are nonspecific, homogeneous and hypoéchogènes lesions. The Doppler shows a medium-intensity vascularization.
In the scanner, lymphomas are well demarcated lesions, hypodenses with an average signal enhancement after injection of contrast product.
In MRI, ovarian lymphomas present as homogeneous solid masses, hypo intense in T1, slightly hyper intense in T2, with an average signal enhancement after injection of gadolinium.
These aspects oppose that of ovarian epithelial tumours that present themselves as heterogeneous masses with cystic areas, solids, necrosis ranges and an enhancement after injection of more marked contrast product.
The treatment of Burkitt lymphomas is mainly based on intensive, short-term chemotherapy involving high-dose methotrexate, Cyclophosphamide, and Cytarabine.
In the literature, patients with ovarian lymphoma have undergone surgical treatment because of the similarity of the clinical picture to ovarian epithelial cancer, while the latter has no clear place in this disease Systemic. In our case, the diagnosis was difficult in pre-operative times. There was no NFS anomaly, the LDH had not been dosed, and the right Annex torsion table imposed a surgical sanction. But in the face of the necrotic aspect of the masses linked to the important cellular renewal that we encounter in Burkitt lymphomas, unusual in ovarian cancers, we should have waited for the result of the extemporaneous before we realized The left Salpingo.
Burkitt's lymphoma is considered a pathological entity of high grade malignancy and advances in chemotherapy have improved the prognosis with a cure rate that ranges from 50 to 80% [1]. The prognosis of ovarian lymphomas depends on the clinical stage, the modalities of onset of the disease, the histological type and the phenotype. A medullary, meningeal, HIV + serology, translocation (14 18), incomplete remission after chemotherapy are bad prognosis factors. Patients treated only by chemotherapy for non-Hodgkin ovarian lymphomas retain a hope of pregnancy, especially if the woman is less than 30 years of age at the time of diagnosis. For patients with ovarian lymphoma alone, Dao [6] Describes a case of triple pregnancy after diffuse large-cell B intermediate-grade lymphoma with only one ovary and treated with surgery and chemotherapy. The absence of other cases is probably due to the fact that women were older and had been treated by bilateral castration.
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Conclusion
Ovarian lymphomas are clinically difficult to distinguish from ovarian epithelial cancers. Biology and imaging can help with differential diagnosis. An unusual macroscopic aspect during the surgical exploration must carry out a extemporaneous examination in order to avoid a bilateral castration immediately and thus preserve the chances of reproduction of the young women affected by this pathology However rare.
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