Primary ovarian lymphoma is a rare malignancy with <1% of incidence. Diffuse lymphoma and a B-cell phenotype are the most common histological type and phenotype, respectively.[2,3] Primary and secondary ovarian lymphomas differ in terms of prognosis. As compared to primary ovarian lymphoma, patients with nodal lymphomas presenting as an ovarian mass have the worst outcome. The literature shows the presence of synchronous carcinomas, carcinomas with sarcomas, and a few case reports or series of carcinoma with lymphomas. The association of low-grade non-Hodgkin lymphoma (NHL) lymphomas with carcinomas has been reported as case reports or series, with lung, stomach, or colon primaries. Plasmablastic subtype in primary ovarian lymphoma is extremely rare with two reported cases so far.[6,7]
We report here a case of fluorodeoxyglucose positron emission tomography–computed tomography (FDG PET-CT) study depicting a subtype of plasmablastic lymphoma primarily involving the ovary associated with a synchronous lung malignancy.
A 46-year-old female patient evaluated with ultrasonography for symptoms of oral ulcers, dysphagia, and epigastric pain for the last 2 months was identified to have a large right adnexal mass with ovary not separately delineated. Blood parameters showed an elevated LDH of 348 U/with minimally increased tumor markers – CA125 level of 86.51 U/ml and CA19-9 level of 238.17 U/ml. A suspicion of metastases from unknown primary was raised, and the patient was referred for a whole-body PET-CT scan. The CT component of the scan showed a large heterogeneous right adnexal mass, having multiple solid-enhancing nodules within showing intense FDG uptake, with enlarged para-aortic nodes [Figure 1]. An irregular thick-walled cavitating lesion with FDG concentration was detected in the lower lobe of the left lung. Histological findings of the right adnexal mass revealed plasma cell neoplasm consistent with plasmablastic lymphoma. In view of an unusual presentation of associated cavitating lesion in the left lung, a biopsy was advised which identified the presence of adenosquamous carcinoma cells [Figure 2]. In view of the association of plasmablastic lymphomas with retroviral disease, blood investigations were done, which confirmed the presence of HIV antigen.
The patient was planned for the treatment of both the malignancies simultaneously: external beam radiotherapy to the left lung lower lobe mass and six cycles of cyclophosphamide, vorinostat, etoposide, and prednisone chemotherapy followed by involved site radiotherapy to the right adnexal region.
Primary ovarian lymphoma constitutes 0.5% of NHL and 1% of all ovarian tumors. It is difficult to distinguish ovarian lymphoma from primary ovarian malignancies clinically, as it does not show any characteristic signs or symptoms. FDG PET-CT might be helpful in this case since most of the primary ovarian malignancies (which are mucin-secreting tumors) usually show low-grade FDG uptake, while most of the lymphomas are FDG avid and show intense FDG uptake. Fox et al. proposed the following criteria to consider a diagnosis of primary ovarian lymphoma: (i) at the time of diagnosis, the lymphoma is clinically confined to the ovary and other investigations fail to reveal evidence of lymphoma elsewhere. (ii) The peripheral blood and bone marrow should not contain any abnormal cells. (iii) 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. The cells of origin for tumor are lymphocytes of both B- and T-cell lineage occurring within cortical granulomas, in the ovarian stroma, and within ovarian follicles and corpora lutea. The absence of other sites of lymphomatous involvement fulfilled the criteria of ovarian involvement being a primary lymphomatous involvement in our case.
Plasmablastic lymphomas are highly aggressive lymphomas, predominantly associated with immune-compromised patients, and usually involve the mucosa of the oral cavity.
Our patient too had oral ulcers which could favor mucosal involvement by the lymphoma, though no significant FDG uptake was noted in this region.
Plasmablastic lymphoma primarily involving the ovary is extremely rare, with two case studies reported in the literature: plasmablastic lymphomatous involvement of an ovarian teratoma and another of plasmablastic variant of B-cell lymphoma involving bilateral ovaries.
The intense uptake in adnexal lesion suggested an aggressive pathology, unlike primary ovarian malignancies which usually have a paucity of cells with rich mucin background leading to poor FDG concentration.
The presence of HIV infection is a predisposing factor for both, aggressive lymphomas and adenocarcinomas of the lung, which could explain the dual malignancy in this patient. The case presented here is unique for two reasons – one: a rare case of plasmablastic variant of lymphoma involving the ovary, and second, synchronous presentation of adenocarcinoma of the lung with plasmablastic lymphoma of the ovary.
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