Bilateral B - cell non - Hodgkin ovarian lymphoma: A case presentation

Sofoudis Chrisostomos1, Koumousidis Antonios2, Vervesou Elisavet3, Sinha Prabha2, Politi Ekaterini4, Salakos Nikolaos1

12nd Department of Obstetrics and Gynecology, University of Athens, Medical school, Aretaieio hospital, Athens, Greece

2Department of Gynecology, Conquest hospital, East Sussex Healthcare NHS Trust, England, United Kingdom

3Department of Hematology, Henry Dynant hospital, Athens, Greece

4Department of Pathology, University of Athens, Medical school, Aretaieio hospital, Athens, Greece

Correspondence: Koumousidis Antonios, 46 Oast House Close, St. Leonards on sea, East Sussex, England, TN377UY, United Kingdom

E - mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

               

Abstract

The correlation between non - Hodgkin lymphoma (NHL) and the genital tract is unquestionable. The ovaries represent the most common anatomic sites. In many cases the clinical spectrum seems identical and misleads the treatment management. The prognosis is often poor due to delayed or inaccurate diagnosis. We present a case of a 63 - year - old female patient with NHL mimicking ovarian carcinoma, early diagnosed and successfully treated.

Keywords: Non - Hodgkin lymphoma; ovarian carcinoma; chemotherapy

 

Lymphomas mimicking ovarian tumors are uncommon and may occur as de novo or secondary as a part of systemic disease1. The difference between primary and secondary lymphomas is extremely important due to differences in the treatment of choice. Prognostic factors include tumor size, staging, and histological type. The most common histological types of non - Hodgkin lymphoma (NHL) are Burkitt lymphoma and diffuse large B - cell lymphoma2. Among them, the B - cell type is correlated with longer survival and better life quality3. Ovarian lymphoma may occur at any age, with a median age of 40 years4,5. The clinical and imaging establishment of the primary ovarian lymphoma remains controversial. Fox et al, suggested three criteria regarding the diagnosis of primary ovarian lymphoma6. Specifically, the tumor must be confined to the ovary regional lymph nodes or adjunctive organs at the time of the diagnosis, the bone marrow and peripheral blood should not contain any abnormal cells, and in case of appearance of extra - ovarian lesion, there must be a few months’ time interval between the occurence of ovarian and extra ovarian lesions. The survival rate of primary ovarian NHL ranges from 0 - 36%7. The average prognosis is poor due to inaccurate or delayed diagnosis. Bilateral involvement, rapid progression of the lesion, advanced stage and signs of systematic disease indicate the severity and poor prognosis of the lesion8.

bilateral1
 
bilateral 2
Figure 1. a) Open biopsy of the right fallopian tube, b) tumor infiltrations on the right adnexa

 

Case Report

We present a case of a 63 - year - old female patient (parity 1, gravida 3) admitted to our department, complaining of distension, abdominal pain, and episodes of tinnitus during the last two months. A brain magnetic resonance imaging (MRI) was performed. The change in signal intensity of bone marrow, the presence of exoskeletal metabolism and the node - like foci in the subcutaneous tissue, were compatible with an hematological - like lymphoma lesion. Computed tomography (CT) of the abdomen revealed the presence of diffuse lymphatic blocks inside the whole retroperitoneal space and free fluid inside the peritoneal cavity, whereas both adnexa were not clearly defined. The picture was compatible with an ovarian neoplastic lesion and lymphoma. Cancer antigen 125 (CA - 125) tumor marker was 764 U/ml (normal range <36 U/ml). Transvaginal ultrasound revealed the presence of several solid, highly vascularised, riveted masses at both ovaries (159 cm3 and 78,5 cm3 in the left and the right ovary, respectively) and infiltrated iliac and paraaortic lymph nodes. Findings were compatible with an ovarian tumor. The gastroscopy and the colonoscopy revealed no signs of malignancy. MRI of the abdomen revealed extended infiltrated lymphatic block inside the paraaortic space, along the iliac vessels. The iliopsoas muscle, the presacral space and the parametrium were not clearly anatomically defined. Presence of free fluid inside the pleural and peritoneal cavity was noted. Papanicolaou smear was negative. Due to the large quantity of free fluid inside the pleural cavity, a pleural catheter was inserted and 1,200 ml of pleural fluid were aspirated. The cytological report revealed the presence of mesothelial cells with reactive changes, histiocytes and many lymphocytes, giving a picture of diffuse lymphatic domination. The patient underwent exploratory laparotomy and open biopsy from both fallopian tubes and the omentum. Large quantity of peritoneal fluid was drained out. There were several enlarged lymph nodes in the retroperitoneal space. The cytological examination revealed middle and large cytological elements, while the immunohistochemical examination reported the presence of B - cells, cluster of differentiation CD20 (+), CD79a (+), CD10 (+), B - cell lymphoma 6 (bcl - 6) protein (+), CD23(+), bcl - 2 protein (-), multiple myeloma oncogene 1 (MUM - 1) (-), CD5 ( -), and a low percentage of T lymphocytes.

 

bil f1a bil f1b
a) Papanicolaou stain X 10 b) Papanicolaou stain X 10
   
bil f1c bilf1d
c) Papanicolaou stain X 20 d) Papanicolaou stain X 40
   
Figure 2. Cytological smears from peritoneal washing dominated by numerous lymphoid cells, lying singly (a) and in clusters (b), some of them with atypical features, suggesting the diagnosis of lymphoma (c,d).

 

Overall, there was infiltration with high malignancy B - cell NHL accompanied with enlarged cells (diffuse large B - cell lymphoma - not otherwise specified, DLBCL - NOS), Epstein - Barr virus (EBV) (-) and a phenotype indicative of germinal cell origin. The bone biopsy revealed hematopoietic cell hyperplasia, increase of the megakaryocytes and limited lymphocyte presence. All these characteristics suggested the diagnosis of an extramedullary lymphoma.

The patient underwent eight cycles of chemotherapy with rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisolone (R - CHOP). The new abdominal CT showed disappearance of the pre - existing tissue between the intestinal helices and the abdominal cavity, and clear improvement of the retroperitoneal lymphatic block. The patient was followed up with CT and tumor markers every six months and was in good clinical condition one year later.

Discussion

Lymphomas mimicking ovarian tumors represent a rare entity, occurring as a regional or a systematic disease. Many studies have been conducted to establish a proper and an accurate diagnosis9. The management of the primary ovarian lymphoma remains controversial and the therapeutic options include surgery, chemotherapy and radiotherapy10. The chemotherapy protocol, regarding diffuse large B - cell lymphomas, consists of the standard R - CHOP regimen. CHOPis the acronym for achemotherapy regimenused in the treatment of NHL: cyclophosphamide, analkylating agentwhich damages DNA by binding to it and causing the formation of cross - links, hydroxydaunorubicin (also called doxorubicin or adriamycin), an intercalating agent which damages DNA by inserting itself between DNA bases, oncovin (vincristine), which prevents cells from duplicating by binding to the protein tubulin, and the corticosteroids prednisone or prednisolone. The above regimen can also be combined with the monoclonal antibody rituximab (R - CHOP). In patients with a history of cardiovascular disease, doxorubicin (which is cardiotoxic) is often omitted. The combination is then referred asCOP(cyclophosphamide, oncovin, and prednisone or prednisolone) orCVP(cyclophosphamide, vincristine, and prednisone or prednisolone). The main advantage of rituximab is its essential role in the treatment management of CD20 (+) B - cell lymphoma.

Conclusion

Ovarian lymphomas are rare entities with specific treatment protocols. The physical examination and preoperative imaging findings help the diagnostic differentiation between primary and secondary lesion, whereas a multidisciplinary approach is the key to the treatment of the disease.

 

Conflict of interest

All authors declare no conflict of interest.

 

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