Primary Ovarian non-Hodgkin Lymphoma Research Articles

Primary Ovarian Non-Hodgkin's Lymphoma: An 18-year Retrospective Institutional Study and Review of Literature from India.

Primary lymphomas of the female reproductive tract are rare and the ovarian extranodal presentation of non-Hodgkin's lymphoma (NHL) accounts for only 0.5% of all NHLs and 1.5% of all ovarian malignancies. We retrospectively reviewed the institutional medical oncology database for newly diagnosed NHL cases between 1999 and 2017. We aimed to study the clinical characteristics, pathology, and outcome of primary ovarian non-Hodgkin's lymphoma (NHL) cases presented to our institution. Weidentified three patients (3.7% of extranodal NHLs and 0.85% of all NHL patients) with primary ovarian NHL from 350 NHL patient records. They underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by six to eight cycles of (rituximab, adriamycin, cyclophosphamide, vincristine, prednisolone (R-CHOP/CHOP), and they attained complete remission. Given the heterogeneity of cancer incidence in India and the absence of state-wise cancer registries, our study argues a pressing need to develop a national representative registry for NHL for accurate incidence, mortality, and survival data. Additionally, fertility preservation is an important issue that must be discussed with women of fertile age and the parents of children.

Tumor characteristics and survival in patients with extranodal non-Hodgkin lymphoma of the female reproductive tract

<h3>Objectives:</h3> Primary extranodal non-Hodgkin lymphoma (NHL) of the female reproductive tract is rare, comprising approximately 1% of all NHL. Studies to date have been limited to case reports without longitudinal follow-up. The objective of this study is to describe tumor characteristics and survival in patients with primary vulvar, vaginal, cervical, uterine and ovarian NHL using the Surveillance, Epidemiology and End Results (SEER) cancer database. <h3>Methods:</h3> Adults with a histologic diagnosis of non-Hodgkin lymphoma (ICD-O-3 9590-9823) and a primary site coded as vulva (ICD-10-CM C51.0-51.9), vagina (C52.9), cervix (C53.0-53.9), uterus (C54.0-C55.9), or ovary (C56.9-C57.4) diagnosed between 1973 and 2015 were included in the analysis. Data was abstracted on variables of interest including age at diagnosis, year of diagnosis, demographics, Ann Arbor stage, histologic subtype, treatment regimens and disease-specific survival (DSS). Descriptive statistics were used to compare characteristics between primary sites. Kaplan-Meier survival curves and multivariable Cox proportional hazards survival analysis were performed using SPSS. <h3>Results:</h3> A total of 793 patients with primary gynecologic NHL were identified, including vulva (n=60), vagina (n=101), cervix (n=187), uterus (n=142) and ovary (n=303). The median age at diagnosis was 55 years old and ranged from 18 to 94 years. Vulvar, vaginal and cervical primary cancers were more likely to be diagnosed at an early stage compared to uterus and ovary (p<0.001) and receive radiation (p<0.001). Ovarian primary NHL was most likely to be diagnosed at an advanced stage (65% stage III or IV) and 88% of patients were treated with surgery. There was no significant difference in chemotherapy administration between the primary sites. The median DSS was not reached. The 5-year disease-specific survival rate was 83.2% for cervical, 81.8% for vaginal, 72.3% for ovarian, 70.9% for vulvar and 69.1% for uterine primary sites (p=0.019). On multivariate analysis, compared to primary ovarian NHL, DSS was significantly improved if the primary site was cervix (hazard ratio 0.61, 95% CI 0.39-0.97) or vagina (HR 0.52, 95% CI 0.29-0.92) with no difference in survival with primary vulvar NHL (HR 0.76, 95% CI 0.43-1.35) or primary uterine NHL (HR 0.90, 95% CI 0.59-1.38). Age at diagnosis (HR 1.04, 95% CI 1.03-1.05), histologic subtype (Burkitt's lymphoma vs other NHL, HR 3.26, 95% CI 1.82-5.84), and advanced stage (Stage II vs I: HR 2.16, 95% CI 1.42-3.28; III vs I: 1.34, 0.60-2.98; IV vs I: 3.50, 2.43-5.06) were associated with worse survival and more recent diagnosis (HR 0.98, 95% CI 0.96-1.00) and treatment with surgery (HR 0.52, 95% CI 0.36-0.73)) were associated with improved survival. <h3>Conclusions:</h3> Overall, extranodal NHL of the female reproductive tract has an excellent prognosis. The clinical characteristics, treatment regimens and survival differ between primary sites.

Ovarian dlbcl presenting as a very large ovarian mass in a young woman – a case report (and potential frozen section pitfall)

While ovarian involvement as a late manifestation of disseminated non-Hodgkin lymphoma (NHL) is not uncommon, primary ovarian NHL, or NHL presenting for the first time as an ovarian mass, is rare, having a reported incidence of around 0.5% of all untreated NHL and 1.5% of untreated ovarian neoplasms. This case study reports a 27-year-old woman who underwent a unilateral salpingo-ophorectomy and staging for a very large, rapidly enlarging, right ovarian mass, suspected clinically of being a dysgerminoma. On frozen section, the tumour was favoured to be a germ cell tumour, with juvenile granulosa cell tumour also not excluded. On permanent section, however, the tumour more clearly demonstrated morphological features and immunological features in keeping with a large B-cell non-Hodgkin lymphoma. Coupled with FISH analysis showing no evidence of a MYC gene rearrangement, a diagnosis of DLBCL, NOS, double expressor phenotype, was ultimately made. This case serves as a reminder that, although rare as an initial presentation in the ovary, lymphoma should be considered by pathologists in the differential diagnosis of ovarian tumours, including at frozen section, where consideration should also be given to obtaining fresh tissue for flow cytometry. 1.Dimopoulos MA, Daliani D, Pugh W, et al. Primary ovarian non-Hodgkin's lymphoma: outcome after treatment with combination chemotherapy. Gynecol Oncol 1997; 64: 446–50.

Primary Ovarian Non-Hodgkin Lymphoma- A Diagnostic Challenge with Clinicopathological Study of Eight Cases

Introduction: The involvement of the Female Genital Tract (FGT) by lymphoma is extremely rare, with ovaries being most commonly affected. Less than 1% of lymphomas present with ovarian involvement and less than 1.5% of ovarian neoplasms are of lymphoid origin. Secondary involvement of ovary by systemic lymphoma is more common than Primary Ovarian Lymphomas (POL) which is usually Primary Ovarian Non-Hodgkin lymphoma (PONHL) of B-cell lineage. Aim: To understand the clinicopathological and immuno- morphological features of Primary Ovarian Non-Hodgkin lymphoma. Materials and Methods: This was a descriptive retrospective study conducted at Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka. India, for a duration of 14 years between July 2006 to June 2020. Eight cases of PONHL were identified from departmental archives and clinicopathological and Immunohistochemistry (IHC) findings of these tumours were analysed. Results: The PONHL constituted 0.4% of all Non-Hodgkin lymphoma (NHL) reported during the study period. The patients age ranged from 13-60 years with a mean age of 34 years. Among eight cases of PONHL, two cases were of Diffuse Large B-Cell Lymphoma (DLBCL), followed by one case each of High- Grade B-Cell Lymphoma, Not Otherwise Specified (HGBL, NOS), Follicular Lymphoma (FL), Burkitt Lymphoma (BL), Plasmablastic Lymphoma (PBL), Precursor B-Lymphoblastic Lymphoma (B-LBL), and precursor T-Lymphoblastic Lymphoma (T-LBL). Seven cases were staged IE (Ann Arbor staging system) while one case was designated as stage IIE. Conclusion: This was probably the first study on PONHL from India. The diagnosis of PONHL is challenging unless there is a high index of suspicion as these patients present with non specific pelvic symptoms and can be misdiagnosed as other epithelial, stromal or germ cell ovarian neoplasm which differs in treatment and prognosis. Histological examination with IHC and molecular testing are essential to establish a diagnosis.

Abstract 33: Clinicopathologic, immunophenotypic, diagnostic, treatment characteristics of non-Hodgkin's lymphoma: Diffuse large B cell: Primary ovarian and with ovarian extensive involvement

Abstract 33: Clinicopathologic, immunophenotypic, diagnostic, treatment characteristics of non-Hodgkin's lymphoma: Diffuse large B cell: Primary ovarian and with ovarian extensive involvement

Primary Ovarian Non-Hodgkin’s Lymphoma: Retrospective Study of 16 Patients

Background: Primary non-Hodgkin’s lymphoma (PONHL) of the ovary is rare disease. Ovarian involvement by NHL is usually secondary, as a part of systemic disease. It is frequently diagnosed as ovarian carcinoma which causes a significant delay in diagnosis and management. Objective: To analyze, to report and to better understand the clinico-pathologic features and results of treatment, and prognostic factors of these tumors. Material & methods: This was a retrospective single institutional work that included 16 cases of primary ovarian non-Hodgkin lymphoma (PONHL) treated in National Cancer Institute-Cairo University from January 2010 till January 2015. All available medical data including the clinical and pathological characteristics, treatment, and outcomes of patients with PONHL are analyzed. Results: Data from 16 patients are obtained. The patient’s age ranges from 14 to 55 years (mean 28 years). Ascites is the most common manifestation (75%). Tumor size ranges from 5 to 24 cm (mean 13.1). LDH is elevated in all cases (mean 644 U/L) and CA-125 is elevated in only 4 cases (25%) especially when there is an extensive peritoneal irritation. Ten cases (62.5%) are bilateral with stage IV-E according to the Ann Arbor staging system. The remaining six cases (37.5%) are unilateral with Ann Arbor stage I-E. There are no stage II-E or III-E in the current study. Tumors are classified according to the World Health Organization as follows: diffuse large B-cell lymphoma (10 cases) (62.5%), Burkitt’s lymphoma (5 cases) (31%) and only one case of B-lymphoblastic lymphoma/leukemia. All the tumors are of B-cell lineage and are all CD20 positive. All Burkitt’s lymphoma cases show higher Ki67 index (4 cases are 100% and one is 88%). The case of B-lymphoblastic lymphoma/leukemia is positive for TDT & CD 10. Surgery is the main treatment modality for primary diagnosis and for staging, although chemotherapy should have been the primary treatment because it is one of the most chemosensitive tumors. Follow-up period ranges from 3 months to 5 years (mean 33 months). Ten patients are alive without disease. Two cases experienced relapse and one case died during chemotherapy treatment. The remaining three cases died from other causes than disease. The median overall survival time was not reached yet; however, the mean overall survival was 46.8 monthes; median progression free survival was 36 months. Conclusion: Most patients with PONHL present with symptoms attributable to an ovarian mass which necessitates extensive surgical staging that is not mandatory for lymphomas. More studies will be needed to better define and treat this rare entity.

Clinical characteristics and treatment of primary ovarian malignant lymphoma

Objective: To investigate the clinical features and prognostic factors of primary ovarian lymphoma(POL). Methods: A retrospective review was performed based on the clinical records of 14 POL cases treated at Tianjin Medical University Oncology institute Hospital verity from 2000-6 to 2010-5. Results: The median age of patients was 47 years at presentation(range 28~62years). Abdominal pain was the most common initial symptom. The majority of histological subtype was B cell lymphoma, above 50% of which is diffuse large B-cell non-Hodgkin's lymphoma. A R0 resection was carried out in 2 patients who were lost to follow-up soon after surgery. 11 patients were treated by CHOP, FC or CHOPE chemotherapy. Chemotherapy combined with rituximab were given to 5 patients. Until now, 5 patients were alive, 6 patients died, and only one patient was lost to follow up after therapy. Conclusion: POL is an extremely rare lymphoma. The ultimate diagnosis depends on histopathologic examination. Primary ovarian non-Hodgkin lymphoma should be treated with multi-modality strategies. Treatment with doxorubicin-based chemotherapy after oophorotomy is recommended. B cell lymphoma preferred to use rituximab combination chemotherapy. Abdominal cavity chemotherapy prophylaxis and irradiation can decrease the probability for recurrence .

Primary Ovarian Lymphoma: A Case Report and Review of Literature

Primary Ovarian Lymphoma: A Case Report and Review of Literature

Clinical features and treatment outcomes of 14 cases of primary ovarian non-Hodgkin’s lymphoma: a single-center experience

To analyze the clinical characteristics, results of treatment, and prognostic factors of patients diagnosed as primary ovarian non-Hodgkin's lymphoma (PONHL). Fourteen cases of PONHL treated in Fudan University Cancer Center during a 10-year period were retrospectively reviewed, and the clinical data of the patients were analyzed for correlation between KPS, clinical stage, tumor size, IPI, ovary involvement, treatment and prognosis. The median age was 45 years, and thirteen patients were diagnosed of diffuse large B-cell lymphoma and one patient lymphoblastic lymphoma. Four patients were stage IE, three stage IIE, and seven stage IVE. The median tumor size was 8 cm (range, 4.0-15.0 cm). The median overall survival (OS) of the 14 patients was 23.0 months (range 11.5-71.2 months). Thirty-six percentage of patients with bilateral ovary involvement had a shorter survival time than those with unilateral ovary involvement (median OS: 19.0 vs. 37.2 months, P=0.014). The OS of Stage IVE was worse than stage IE and stage IIE (median OS: 18.75 vs. 37.5 months, P=0.047). Patients with IPI>2 had worse prognosis than those with IPI≤2 (median OS: 19.0 vs. 42.1 months, P=0.03). PONHL patient with larger tumor mass had worse prognosis (median OS: 19.1 vs. 37.2 months, P=0.019). R-CHOP regimens had a tendency to improve the OS but was not shown to be statistically significant (median OS: 22.4 vs. 37.2 months, P=0.436). The management of PONHL should be based on multi-modality treatment including surgery and chemotherapy. The significant prognostic factors of survival are tumor size, Ann Arbor staging, and IPI.

HIV-associated primary non-Hodgkin's lymphoma of ovary: A case report

Background. Although there have been few case series of primary non-Hodgkin's lymphoma (NHL) of ovary, no patient with AIDS-related primary ovarian lymphoma has been described in the literature. Case. We report a case of human immunodeficiency virus infected female with primary ovarian NHL for which she could not receive standard NHL combination chemotherapy and standard HIV highly active antiretroviral therapy (HAART) and succumbed to death within 7 months after diagnosis. Conclusions. Primary ovarian lymphoma is rare as compared with other ovarian tumors and has similar presentation to other common ovarian malignancies. The patients from developing countries cannot afford standard NHL combination chemotherapy and HAART as these drugs are expensive and are not provided freely to all patients, in state government run public hospitals.

Ovarian Non-Hodgkin's Lymphoma: A Clinicopathologic Study of Eight Primary Cases

Primary (localized) non-Hodgkin's lymphoma (NHL) of the ovary is rare. We studied eight cases of primary ovarian NHL to better understand the clinicopathologic and immunophenotypic features of these tumors. The patients ranged in age from 29 to 62 years (mean 47 years). Pelvic complaints were the most common symptoms; however, three of eight neoplasms were discovered incidentally. All tumors were unilateral and Ann Arbor stage IE. The three incidental NHL were microscopic (largest 1.2 cm), whereas the grossly evident lesions ranged from 7.5 to 20 cm (mean 13.3). Each tumor was classified according to the World Health Organization Classification as follows: diffuse large B-cell lymphoma (three cases), follicular lymphoma (two cases), Burkitt lymphoma (one case), T-cell anaplastic large cell lymphoma (one case), and precursor T-lymphoblastic lymphoma (one case). Six tumors were of B-cell lineage, and two tumors were of T-cell lineage. All three diffuse large B-cell lymphomas were positive for BCL-6, two were positive for CD10, and two were positive for BCL-2. Estrogen and progesterone receptors were negative in all NHLs assessed. Patients were treated by various combinations of surgery, chemotherapy, and radiotherapy. Clinical follow-up ranged from 1.3 to 11.7 years (mean 5.2) and all patients were alive without disease at last follow-up. We conclude that most patients with primary ovarian NHL present with symptoms attributable to an ovarian mass, but in a subset of patients ovarian NHL may be detected incidentally. With appropriate therapy, patients appear to have a favorable prognosis although follow-up is short for some patients in this study.

Primary Ovarian Non-Hodgkin's Lymphoma: Outcome after Treatment with Combination Chemotherapy

Because the outcome of patients with primary ovarian non-Hodgkin's lymphoma (NHL) is controversial, we retrospectively analyzed experience with adults seen at the University of Texas M.D. Anderson Cancer Center from 1974 to 1993. Patients were included if at least one ovary was pathologically involved, and if combination chemotherapy was used that must have included doxorubicin for intermediate grade histologies. We identified 15 patients who constituted 0.5% of all untreated NHL and 1.5% of untreated ovarian neoplasms that presented to our instutition during this time. One patient refused therapy, leaving 14 assessable for response. Nine patients had intermediate-grade, 5 had high-grade, and none had low-grade NHL. One ovary was involved in 4 patients, and both in 10, in 7 of whom additional sites were involved, including supradiaphragmatic nodes in 2. Four patients had AAS I and 10 had AAS IV. Favorable (0 or 1) and unfavorable (>1) IPI scores were seen in 5 and 9 patients, respectively. The complete remission rate for all patients was 64%, and 5-year survival and FFS for all assessable patients were 57 and 46%, respectively. We conclude that the complete remission rate and FFS of patients with ovarian NHL treated with appropriate chemotherapy appear to be similar to that of patients with other nodal NHLs. Further work is required to determine prognostic factors in ovarian NHL.

Primary Ovarian Non-Hodgkin Lymphoma

Primary Ovarian Non-Hodgkin Lymphoma