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      Ovarian Non-Hodgkin Lymphoma Revealed by Bone Metastasis: A Rare Pediatric Case

      case-report

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          Abstract

          Ovary involvement of non-Hodgkin lymphoma (NHL) is rare. We report a rare case of ovarian NHL during adolescence revealed by bone metastasis. The diagnosis of malignant lymphoma was established after adnexectomy and histological study of the excised tissue. The tumor was classified as a diffuse large B-cell lymphoma. The patient has been treated according to the LMB French protocol with good outcome after two years. Although NHL is a rare ovarian neoplasm, it is essential to establish an accurate diagnosis as early as possible for therapeutic purposes.

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          The Société Française d'Oncologie Pédiatrique LMB89 protocol: highly effective multiagent chemotherapy tailored to the tumor burden and initial response in 561 unselected children with B-cell lymphomas and L3 leukemia.

          C Coze, Catherine Patte, M J Terrier … (2001)
          This study was undertaken to show that a high survival rate can be obtained in B-cell (Burkitt and large B-cell) lymphoma and L3 leukemia with multiagent chemotherapy adapted to the tumor burden (stage, resection status, percentage of blasts in bone marrow, and central nervous system [CNS] involvement) and early response to chemotherapy, to investigate actual prognostic factors, and to see if large B-cell lymphoma can be treated with the same regimen as Burkitt lymphoma. Patients were classified into 3 risk groups. Group A (resected stage I and abdominal stage II) received 2 courses of vincristine, cyclophosphamide, doxorubicin, and prednisone. Group B (patients not eligible for groups A or C) received 5 courses of chemotherapy with, in addition, high-dose methotrexate, 3 g/m(2) over 3 hours; infusional cytarabine; and intrathecal (IT) methotrexate. Group C (patients with CNS involvement and acute lymphoblastic leukemia with at least 70% of blasts in bone marrow) received 8 courses with, in addition, high-dose methotrexate, 8 g/m(2); high-dose cytarabine; etoposide; and triple IT. Except in group A, treatment started with a prephase (COP, low-dose vincristine and cyclophosphamide). It was intensified for patients who did not respond to COP in group B and any patient with residual viable cells after the consolidation phase. A total of 561 patients were enrolled in the SFOP LMB89 protocol (July 1989-June 1996). Five-year survival is 92.5% (95% confidence interval [CI], 90%-94%) and event-free survival (EFS) 91% (95% CI, 89%-93%). EFS is 98% (95% CI, 90%-100%), 92% (95% CI, 89%-95%), and 84% (95% CI, 77%-90%) for group A, B, and C, respectively. In group B, multivariate analysis of prognostic factors showed that a lactate dehydrogenase level more than 2-fold the normal value, no response after COP, and age of at least 15 years were associated with a lower EFS. CNS involvement was the only prognostic factor in group C. (Blood. 2001;97:3370-3379)
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            Malignant lymphoma presenting as an ovarian tumour: a clinicopathological analysis of 34 cases.

            H. Fox, J. Hill, Thomas Bennett … (1988)
            The clinicopathological features of 34 patients with malignant lymphoma or leukaemia where the initial presentation was as an ovarian tumour are described. Nineteen patients died, usually within 1 year of diagnosis, whilst 15 survived, some for long periods. Factors indicating a poor prognosis were rapid onset of abdominal symptoms, the presence of systemic symptoms, bilateral ovarian tumours, advanced stage and a histological pattern other than that of a B-cell lymphoma. For staging purposes the Ann Arbor method appeared a more sensitive prognostic indicator than did the FIGO staging system. It is suggested that primary malignant lymphomas of the ovary are extremely rare and that for therapeutic purposes all lymphomas of the ovary should be regarded as local manifestations of systemic disease.
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              Ovarian non-Hodgkin's lymphoma: a clinicopathologic study of eight primary cases.

              R Vang, L. Jeffrey Medeiros, R Warnke … (2001)
              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 I(E). 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.
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                Author and article information

                Contributors
                M. Elbaz:
                ORCID: https://orcid.org/0000-0003-3084-7715
                Journal
                Journal ID (nlm-ta): Case Rep Pediatr
                Journal ID (iso-abbrev): Case Rep Pediatr
                Journal ID (publisher-id): CRIPE
                Title: Case Reports in Pediatrics
                Publisher: Hindawi
                ISSN (Print): 2090-6803
                ISSN (Electronic): 2090-6811
                Publication date Collection: 2021
                Publication date (Electronic): 20 April 2021
                Volume: 2021
                Electronic Location Identifier: 8826688
                Affiliations
                1Pediatric Hematology and Oncology Department, Mohamed VI University Hospital, Caddi Ayyad University, Marrakesh, Morocco
                2Nuclear Medicine Department, Mohamed VI University Hospital, Caddi Ayyad University, Marrakesh, Morocco
                3Anatomo-pathology Department, Mohamed VI University Hospital, Caddi Ayyad University, Marrakesh, Morocco
                Author notes

                Academic Editor: Pannee Visrutaratna

                Author information
                https://orcid.org/0000-0003-3084-7715
                Article
                DOI: 10.1155/2021/8826688
                PMC ID: 8079212
                PubMed ID: 33986965
                SO-VID: c9aad3cc-40fa-4ad5-a349-dff041bb3465
                Copyright © Copyright © 2021 M. Elbaz et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 23 July 2020
                Date revision received : 3 April 2021
                Date accepted : 15 April 2021
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Pediatrics
                Data availability:
                ScienceOpen disciplines: Pediatrics

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