This study gives an elaborate exposition of the opportunity for the timely diagnosis and treatment of ovarian non-Hodgkin's lymphoma (NHL) by providing the observations of more than a decade and to discern the clinicopathologic and immunohistochemical findings of ovarian NHL. We also evaluate the treatment modalities CHOP and rituximab combination chemotherapy and the response to them.

With a slight male preponderance, globally NHL held the 8th and 11th positions in terms of cancer incidence and cancer death respectively, according to the reports of 2013. The most common form of NHL worldwide without having any geographical variation is diffuse large B cell lymphoma, which represents 37% of B-cell cancers worldwide. The presentation of lymphomas as ovarian malignancy is uncommon and what is detected mostly is incidental. The dearth of published reports and reviews on this particular tumor precludes a detailed comprehension of this rare lymphoma. The lion's share among all scientific papers has been individual rare clinical case reports.

Primary ovarian NHL is rare. Patients usually present because of pelvic complaints, but some cases may be detected as an incidental finding. Ann Arbor staging does not describe definitions for primary nodal extranodal because it is initially came into play for Hodgkin's disease which does not have extranodal sites. A recent study defines extranodal NHL as the one that originates from the extranodal site and even though disseminates clinically dominant extranodal site. The neoplasms can be mostly microscopic and are detected only in imaging studies. They are usually unilateral. Both B-cell and T-cell NHL can arise in the ovary. However, B-cell tumors are the ones that are most common and we confronted only B-cell tumors. Among B-cell lineages, DLBCL are common. Immunohistochemistry markers showed CD20+, CD3+, and LCA+.

Patients are well managed with oophorectomy and CHOP or rituximab with CHOP chemotherapy. All three patients responded well to treatment and with long follow-up. Failure-free survival was assured. Patients were followed up till date. Our patients with primary ovarian NHL appear to have a good prognosis. Clinical follow-up in all three cases is relatively long, which is an advantage of our study over other studies in the past.

The significance and novelty of the study is that it pertains to the holistic aspects of this cancer entity including clinical, pathologic, diagnostic, and treatment aura throughout a decade precisely and uninterruptedly. This study describes exposition of the opportunity for the timely diagnosis and treatment of ovarian NHL by providing the observations of more than a decade, and evaluates the treatment modalities CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone), rituximab combination chemotherapy, and the response to them.

Procedures: This is a multicenter retrospective analysis of patients who presented with ovarian mass and on evaluation were diagnosed to have primary NHL of the ovary at multiple tertiary oncology care centers in Mangalore, South India.

Summary: We analyzed 12 years of case records from 2004 to 2016 among which 271 patients had NHL. Among those 271 NHL patients, three patients had primary ovarian NHL. These three cases of ovarian NHL belonged to Ann Arbor stage I AE, IV, and I BE. Patients ranged from 32 to 59 years; mean was 50 years. Fifty percent are premenopausal and the other half attained menopause. This report includes primary ovarian and with ovarian extensive involvement. 2 patients have NHL ovary, one with NHL ovary fallopian tubes involved. Patients presented to the department as referred cases from Obstetrics and Gynecology because their chief complaints were common gynecologic complaints. There were no gross lesions. All were detected in imaging studies. All cases were of B-cell lineage; 50% were DLBCL. All cases are followed up till date and the mean follow-up is 5.8 years. Clinical follow-up is comparatively long, with a maximum up to 8 years in the earliest diagnosed case among these. Three patients were alive, relapse free and disease free, at last follow-up and one is currently on chemotherapy.

A glimpse into 33 primary ovarian NHL cases from various previously published papers is tabulated. Since this is a rarely reported case, there is a dearth of patients and that makes this case important to get into notice. We hereby attempt a comparison with studies conducted in the past with ours. Age has wide range in the previous studies starting from 16 years, whereas our youngest age is 32 years. All 33 patients had pelvic or abdominal symptoms. Almost all cases are unilateral.

Patients had either complete remission or are currently on chemotherapy. An advantage of the study is that we have a long follow-up with patients. Patients sought medical attention first from Obstetrics and Gynecology due to similar presenting complaints of ovarian lymphoma and other diseases of the gynecologic tract. There is indeed a time lag between the commencement of symptoms and the patient presenting to the Oncology department. Since microscopic lesions are not evident on clinical examination, imaging studies are necessary. Only after analyzing the results of the imaging study are patients sent to the Oncology department, and evaluation starts many days after presentation. This must call the attention of all departments to the fact that ovarian lymphomas can present without an obvious abdominal mass or B symptoms.

Conclusion: We conclude that our results corroborate the good prognosis and survival of the patient with primary ovary NHL and with extensive ovarian involvement. Patients are alive and disease free. The advantage of the study is that we have a long follow-up with patients and we have managed the patients without radiotherapy. Our cases have a good survival with oophorectomy and chemotherapy alone unaccompanied by radiotherapy.

Citation Format: Tara Rajendran, Krishna Prasad. Clinicopathologic, immunophenotypic, diagnostic, treatment characteristics of non-Hodgkin's lymphoma: Diffuse large B cell: Primary ovarian and with ovarian extensive involvement [abstract]. In: Proceedings of the Second AACR Conference on Hematologic Malignancies: Translating Discoveries to Novel Therapies; May 6-9, 2017; Boston, MA. Philadelphia (PA): AACR; Clin Cancer Res 2017;23(24_Suppl):Abstract nr 33.