British Journal of Radiology (2007) 80, e155-e158
© 2007 British Institute of Radiology
doi: 10.1259/bjr/35049074
Primary non-Hodgkin's lymphoma of the ovaries: imaging findings
J Crawshaw, FRCR
1
S A Sohaib, FRCR
1
A Wotherspoon, MRCPath
2 and
J H Shepherd, FRCS, FRCOG
3
Departments of 1 Radiology, 2 Histopathology and 3 Gynaecological Oncology, The Royal Marsden Hospital, London, UK
Correspondence: Dr James Crawshaw, Radiology Department, The Royal Marsden NHS Foundation Trust, Downs Road, Sutton, Surrey SM2 5PT, UK. E-mail: jameswcrawshaw{at}hotmail.com
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Abstract
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Primary ovarian lymphoma is very rare. We report a case of primary ovarian Burkitt-type non-Hodgkin's lymphoma, describing the imaging appearance on ultrasound, CT and MRI, and review the literature.
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Case report
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A 28-year-old nulliparous woman presented to her general practitioner with a 6-week history of worsening abdominal distension, lower abdominal pressure and frequency of micturition. She had no relevant past medical or family history. Physical examination revealed a mass arising from the pelvis and extending up to just above the pubic symphysis. 3 weeks later the mass had grown to reach the level of the xiphisternum. There was no lymphadenopathy or hepatosplenomegaly. She was not immunocompromised. Further investigation at this stage showed raised tumour markers, with a CA-125 level of 1111 U ml–1. Lactate dehydrogenase (LDH) was also raised at 905 U L–1 (normal range 130–250 U L–1). Other tumour markers, including 
-human chorionic gonadotropin (beta-HCG), 
-fetoprotein (AFP) and carcinoembryonic antigen (CEA), were normal.
Ultrasound revealed two large masses, one in the right side of the pelvis and the other in the left iliac fossa. The masses were homogenous and slightly echogenic with small cystic areas around the periphery (Figure 1
). There was through-transmission of sound and a small amount of free fluid in the pelvis. MRI was performed to further characterize the lesions. MRI showed that the masses were separate from the uterus and a normal ovary could not be identified. The masses, approximately 13 cm in diameter, were of low signal intensity on T1 weighted images and enhanced avidly following intravenous gadolinium. On the T2 weighted images the masses were of intermediate signal intensity (Figure 2) with well-defined, round, 1 cm, high signal intensity lesions in the periphery, consistent with follicles. These appearances in conjunction with a history of a rapid increase in size of the lesions raised the possibility of ovarian lymphoma as a diagnosis. A staging CT of the chest abdomen and pelvis showed the two contiguous heterogeneously enhancing solid masses (Figure 3). The small cysts at the periphery of the masses identified on ultrasound and MRI were not seen. There were a few small, 1 cm retroperitoneal lymph nodes. There was no other adenopathy and the spleen was normal. The bone marrow aspirate was normal with no evidence of lymphoma.
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Figure 1. Transverse ultrasound shows a well-defined homogeneous hyperechoic mass with through-transmission of sound, focal cysts (white arrows) in the periphery and a small amount of free fluid (black arrow).
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Figure 2. (a) Axial T1 weighted MR image before and (b) coronal image after intravenous gadolinium shows a low signal intensity mass, which avidly enhances. Note that there is also some septal enhancement (black arrow). The second mass (white arrow) is seen extending into the left upper quadrant. (c) Sagittal T2 weighted MR image of the pelvis shows an heterogeneous intermediate-to-high signal intensity ovarian mass posterior to the uterus with high signal follicles (arrowheads) in the periphery and free fluid (asterisk) within the pouch of Douglas.
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Figure 3. Contrast-enhanced axial CT image shows two masses (arrowheads), one in the pelvis (a) and the other in the left iliac fossa (b). The masses displace surrounding organs and free fluid is seen in the right flank. Of note there are small retroperitoneal lymph nodes (black arrow) in the left para-aortic region.
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An ultrasound-guided core biopsy was taken from the right ovarian mass. The pathological assessment was consistent with primary ovarian non-endemic Burkitt-type non-Hodgkin's lymphoma (NHL). The core of tissue showed diffuse infiltration by lymphoid cells of intermediate size with scanty cytoplasm and round nuclei with a mild degree of pleomorphism. The nuclei contained multiple small nucleoli and there were frequent mitoses. The cells expressed CD20, CD10, bcl-6 and immunoglobulin M but were negative for bcl-2 protein. Proliferation was assessed as approaching 100% by staining for Ki67. Cytogenetics showed the presence of a t(8;14) translocation (Figure 4).
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Figure 4. Haematoxylin- and eosin-stained section showing a diffuse population of intermediate-sized cells with scattered macrophages. Abundant apoptosis is seen and frequent mitoses are present.
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The patient was started on chemotherapy and a follow-up CT scan at 6 weeks showed a dramatic reduction in the size of the masses (Figure 5).
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Figure 5. Contrast-enhanced CT through the pelvis after 6 weeks, following two cycles of chemotherapy. The tumours (arrowheads) have markedly reduced in size.
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Discussion
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Involvement of the ovary in NHL is well recognized [1]; however, primary ovarian lymphoma is a rare manifestation of NHL. Primary ovarian lymphoma accounts for 0.5% of NHL and 1.5% of ovarian tumours [2]. This is partly because of the fact that there is no lymphoid tissue within the ovary. It has been suggested that the tumour originates from lymphocytes in the ovaries, surrounding blood vessels at the hilum and related to the corpus luteum. Most authors are in agreement that this is a local manifestation of a systemic disease. Fox et al [3] suggested the following criteria for the diagnosis of primary ovarian lymphoma:
- 1. At the time of diagnosis, the lymphoma is clinically confined to the ovary and a full investigation fails to reveal evidence of lymphoma elsewhere. A lymphoma can still, however, be considered as primary if spread has occurred to immediately adjacent lymph nodes or if there has been direct spread to infiltrate immediately adjacent structures.
- 2. The peripheral blood and bone marrow should not contain abnormal cells.
- 3. If further lymphomatous lesions occur at sites remote from the ovary then at least several months should have elapsed between the appearance of the ovarian and extra-ovarian lesions.
Many of the clinical findings in ovarian lymphoma mimic ovarian carcinoma with elevated tumour markers and ascites, as in this case. Other features that have been described include omental cake and pleural effusions. This case emphasises the need for specialist referral of suspicious masses, particularly if there are unusual features. Premature exploratory laparotomy would clearly be inappropriate in this case.
Burkitt's lymphoma has been described in HIV/AIDS patients, particularly paediatric cases, and represents up to 20% of AIDS-related lymphoma. With the improved survival and increased prevalence of patients with HIV/AIDS, the incidence of AIDS-related NHL may increase. The ovary is a relatively rare site of disease, with the mesentery being the more common site. In this case the tumour grew significantly over the 10-day period between referral and diagnosis. Ovarian cancer does not grow this rapidly; however, germ cell tumours and lymphoma can. Lymphoma has the fastest doubling time of any tumour and it can be as low as 24 h. To our knowledge this is the first case to report imaging findings of primary Burkitt's lymphoma of the ovary.
Ferrozzi et al [4] described the MRI findings in NHL of the ovaries. They reported five cases, only one of which was a primary lymphoma. The MRI findings included solid bilateral masses, which were low signal intensity on T1 weighted images and mildly high signal intensity on T2 weighted images. Lesions were homogeneous with mild to moderate contrast enhancement with intravenous gadolinium. The authors did not describe the imaging findings in the case of primary lymphoma of the ovary [4].
Mitsumori [5] presented a case of Burkitt-type NHL in a 12-year-old child with MRI findings of intermediate signal mass on T2 weighted images and several small cysts at the periphery in a linear arrangement. There was also septal enhancement with intravenous gadolinium. To the best of our knowledge, there are no descriptions of the CT and ultrasound findings in Burkitt-type NHL.
In the assessment of adnexal mass lesions MRI provides a better characterization than ultrasound [6]. This is mainly because MRI is better than ultrasound in distinguishing solid from complex cystic fluid components and also in identifying the organ of origin. In this case MRI confirmed that the lesions were predominantly solid bilateral ovarian masses. Because of the rapid growth of the tumour, the main differential diagnoses in this case were between a lymphoma, an immature germ cell tumour and a granulocytic sarcoma. The tumour markers AFP and -HCG are occasionally not elevated in immature-type germ cell tumours. Granulocytic sarcoma (chloroma) is a mass of malignant myeloid precursor cells in an extramedullary site [7]. It is less likely to be bilateral. Other predominantly solid ovarian tumours such as fibroma, thecoma and Brenner cell tumour were excluded in this case because of the rapid growth of the masses. There was no fibrous component on MRI to suggest a diagnosis of fibroma or thecoma. Brenner cell tumours are frequently small, seldom bilateral and show extensive calcification. Malignant epithelial ovarian cancers are rarely predominantly solid. It is also important to realize that small amounts of ascites and the concomitant raised CA-125 levels are non-specific for the diagnosis of ovarian epithelial tumours. Sex cord–stromal tumours such as granulosa cell tumours and Sertoli–Leydig cell tumours are often functional. Metastases to the ovaries should also be considered, accounting for approximately 30% of ovarian malignancy; however, a primary lesion or other sites of metastases were not seen in this case. Metastases can be solid or cystic and bilateral, with MRI characteristics depending on the primary tumour.
In summary, primary ovarian Burkitt-type NHL is a rare cause of an ovarian mass or masses. An important feature is the extremely rapid growth rate of these tumours. Imaging may show solid lesions with preservation of follicles at the periphery of the ovary. The importance of considering lymphoma in the differential diagnosis is that the management of such patients is different from that of patients with other types of ovarian tumour, as urgent chemotherapy is the initial treatment of choice.
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Acknowledgments
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We would like to thank Janet MacDonald for assistance with the illustrations.
Received for publication September 26, 2005.
Revision received December 20, 2005.
Accepted for publication January 12, 2006.
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References
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- Chorlton I, Norris HJ, King FM. Malignant reticuloendothelial disease involving the ovary as a primary manifestation: a series of 19 lymphomas and 1 granulocytic sarcoma. Cancer 1974;34:397–407.[CrossRef][Medline]
- Dimopoulos MA, Daliani D, Pugh W, Gershenson D, Cabanillas F, Sarris AH. Primary ovarian non-Hodgkin's lymphoma: outcome after treatment with combination chemotherapy. Gynecol Oncol 1997;64:446–50.[CrossRef][Medline]
- Fox H, Langley FA, Govan ADT, Hill AS, Bennett MH. Malignant lymphoma presenting as an ovarian tumour: a clinicopathological analysis of 34 cases. Br J Obstet Gynaecol 1998;95:386–90.
- Ferrozzi F, Tognini G, Bova D, Zuccoli G. Non-Hodgkin's lymphomas of the ovaries: MR findings. J Comput Assist Tomogr 2000;24:416–20.[CrossRef][Medline]
- Mitsumori A. MR appearance of non-Hodgkin's lymphoma of the ovary. AJR Am J Roentgenol 1999;173:245[Free Full Text]
- Sohaib S, Mills T, Sahdev A, Webb J, Van Trappen P, Jacobs I, et al. The role of magnetic resonance imaging and ultrasound in patients with adnexal masses. Clin Radiol 2005;60:340–8.[CrossRef][Medline]
- Jung S, Chun K, Park S, Lee E. MR findings in ovarian granulocytic sarcoma. Br J Radiol 1999;72:301–3.[Abstract]
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Abstract
Case report
