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Nodular Lymphocyte predominant Hodgkin lymphoma (NLPHL) are rare lymphomas in pediatric patients comprising less than 10 % of all Hodgkin lymphoma (HL). They are for the most part diagnosed at stage I or II and indolent with lymphadenopathy often preceding the diagnosis by many months/years. Survival is excellent. Historically, patients were treated according to classical HL protocols. Due to high toxicity and excellent prognosis, management of NLPHL shifted to de-escalation protocol with good results. No treatment beyond surgical resection was proposed for localized unique nodal disease completely resected. The closed European protocol (EuroNet PHL LP1) evaluated the efficacy of low intensity chemotherapy protocol based on CVP courses (cyclophosphamide vinblastine prednisone) for stage IA/IIA not fully resected. Final results are not yet available. Advanced stage NLPHL are rare and there is no clinical trial and no consensus treatment in children. The SFCE lymphoma committee recently established recommendations for staging and treatment of limited and advanced NLPHL in children based on current practices and published results. The goal was to allow homogeneous practice on a national scale. If incomplete resection for patients with stage I/IIA combination of low intensity chemotherapy (CVP) and rituximab is recommended. For intermediary and advanced stage intensification with AVD (adriamycine vinblastine dacarbazine) or CHOP courses (cyclophosphamide doxorubicine vincristine prednisone) combined with rituximab are advocated. In children, there is no indication for first-line local treatment with radiotherapy., (Copyright © 2023 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Thymic tumors. Thymic epithelial tumors are rare malignancies, that may be aggressive and difficult to treat, with variable prognosis. The histopathological classification distinguishes two major tumor types: thymomas and thymic carcinomas. Autoimmune manifestations are observed in nearly one third of patients at diagnosis; myasthenia gravis is the most common, followed by pure red cell aplasia and hypogammaglobulinemia. Assessing the resectability of the tumor represents the first stage of the therapeutic strategy, as complete resection is the most significant prognostic factor on patient survival. For unresectable thymic tumors, treatment is then based on induction chemotherapy followed by surgical resection or radiotherapy. Following a call of the French National Cancer Institute, a network of expert centers for the management of thymic malignancies started in in 2012: RYTHMIC., Competing Interests: N. Girard déclare des interventions ponctuelles pour les entreprises BMS, MSD, AstraZeneca, Boehringer Ingelheim, Pfizer, Roche, Novartis et Teva. C. Merveilleux du Vignaux déclare n’avoir aucun lien d’intérêts. B. Besse et T. Molina n’ont pas transmis de déclaration d'intérêts.

The Massive Open Online Course (or MOOC) "Diagnostic Strategies Cancers", was hosted in autumn 2016 on the platform "France Université Numérique" and had two levels of learners: students in the field of health and biology and the general public. Of the 5285 learners in 81 different countries, 1237 (23%) were successfully certified. This MOOC was also integrated into the teaching program of medical students of Paris Diderot University and Paris 13 University. Using anonymous questionnaires before and after MOOC, it has been shown that pathology is less known than other medical specialties. Participation in this MOOC led to a marked improvement in participants' knowledge of the place and role of the pathologist in the diagnosis of cancers. Regarding the students who have followed the MOOC as part of their university course, their comments were very positive, but it is necessary to make substantial adjustments in the amounts and contents of the campus-based courses., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Overall survival of patients with Hodgkin lymphoma has dramatically increased in recent years, with an increased rate of malignant complications. We report here a 54-year-old man who presented with concomitant relapse of classical Hodgkin's lymphoma and lung adenocarcinoma 20 years after initial treatment with chemotherapy and mantle radiotherapy. Pathogenic mechanisms of these malignant complications are discussed. A prolonged follow-up of patients with Hodgkin's lymphoma is required., (Copyright © 2010 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Hematogones are bone marrow precursors of B-lymphoid cells which are morphologically difficult to distinguish from blasts and/or from small lymphocytes. We report the case of a patient presenting idiopathic myelofibrosis with minimal myeloid blastic transformation causing severe pancytopenia, treated by allograft and showing in a bone marrow biopsy, a hyperplasia of B-lymphoid cells. Histopathology and immunohistochemistry identified these cells as hyperplasia of hematogones and not a transformation into lymphoblastic acute leukaemia. The cytology of a myelogram confirmed the diagnosis.

We report a case of primary plasma cell leukaemia, with an absolute count of plasma cells of 53 Giga/L, diagnosed in a 83-year-old woman. The patient's condition improved, with no circulating plasma cells after 3 weeks of treatment, in response to the combination of thalidomide and dexamethasone administered for 5 days followed by thalidomide alone. The clinical presentation, the morphological, flow cytometric and pathophysiological characteristics of the plasma cell leukaemia and the treatment are summarised in this paper.

In preclinical tumor model and in human cancer, tumor antigen specific T lymphocytes play a key role in the control of tumor development. Nevertheless in numerous cases, the infiltrating tumor T cells do not seem to influence the clinical progression of the tumor. A better phenotypic and functional characterization of T cells in close contact with tumor associated with a comprehensive analysis of tumor evasion mechanism to the host response should lead to an optimization of cancer immunotherapy protocols.

Context: The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of French Cancer Centers (FNCLCC), the 20 French cancer centers and specialists from French public university and general hospitals and private clinics. Its main objective is the development of clinical practice guidelines to improve the quality of health care and outcome for cancer patients. The methodology is based on literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery., Objectives: To develop, according to the definitions of the Standards, Options and Recommendations, clinical practice guidelines for the management of non small cell lung carcinoma patients., Methods: Data were identified by searching Medline and the personal reference lists of members of the expert groups, then submitted for review to independent reviewers. This is a short version of the SOR guideline covering diagnosis, treatment and follow-up and includes the algorithms for the management of patients with non-small cell lung cancer.

Context: The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of French cancer centers (FNCLCC), the 20 French cancer centers, and specialists from French public universities, general hospitals and private clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and the outcome of cancer patients. The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery., Objectives: To develop clinical practice guidelines for non small cell lung cancer patients according to the definitions of the Standards, Options and Recommendations project., Methods: Data were identified by searching Medline , web sites, and using the personal reference lists of members of the expert groups. Once the guidelines were defined, the document was submitted for review to independent reviewers., Results: This article presents the chapter "Prognosis significance of oncogenes and tumor suppressor genes" from the full report "Standards, Options and Recommendation for non small cell lung cancer" validated in August 2000. The main recommendations are: 1) No clear clinical prognostic value of oncogenes and tumor suppressor genes (p53, bcl-2, Ki-ras, c-erbB-2, Rb, p16) in non small cell lung cancer, can be established from the available evidences (standard, level of evidence C). 2) Prospective multicenter studies should be performed to assess prognostic significance of oncogenes and tumor suppressor genes in non small cell lung cancer.

A new classification of lymphoma has been published in 2001 under the direction of the World Health Organisation. This classification is based on a consensus between experts in haematopathology, haematology and oncology involved in management of lymphoma. Around 40 entities are described on the basis of morphology, immunophenotype, genetic and clinical presentation. Lymphomas and lymphoid leukaemias are gathered because tumour masses and leukaemic phases are present in numerous entities. This classification differentiates B-cell lymphomas from T/NK (natural killer) cell lymphomas. Grading the different lymphomas into low grade or high grade is no more required in this classification.

WHEN THE PRIMARY CANCER IS KNOWN: Secondary tumoral pleurisy can develop at any stage in the evolution of a neoplastic disease and its diagnosis is easy when the primitive cancer is known. Cytological analysis of the pleural liquid and/or biopsy often provides material and the slides can then be compared with those of the primitive cancer and a relationship established. However, a probabilistic diagnosis should not be in haste, without anatomopathological confirmation, since the causes of pleurisy in a cancer patient are varied. WHEN THE PRIMARY CANCER IS NOT KNOWN: The most difficult situation is that when the pleurisy is revelatory of a neoplastic disease. In this case, explorations should be limited to a few essential examinations guided by the clinical profile, without being tempted to carrying out exhaustive explorations of the primitive cancer, which would not change the prognosis. Before such explorations, it is essential to obtain a precise pleural diagnosis, and for this, the indication for a pleuroscopy must be easily and rapidly accessible. FROM A THERAPEUTIC POINT OF VIEW: Once the diagnosis of pleurisy has be established, treatment is in two parts, associated or not: local treatment consisting in pleural sympysis and general treatment (chemotherapy or even hormone therapy, adapted to the primitive neoplasia). Pleural sympysis is often performed using talc, either during the pleuroscopy (talc insufflated under visual control), or during thoracic draining (talc slurry).

We report an unusual case of iterative cutaneous manifestations of B-cell chronic lymphocytic leukaemia, masked by a florid epithelioid granulomatous response. These cutaneous lesions appeared without blood hyperlymphocytosis or lymph node enlargement. The diagnosis of cutaneous tumorous infiltrates was morphologically very difficult and required immunohistochemistry as well as PCR. This observation stresses the diagnostic difficulties encountered in lymphoma, with intense stroma reaction and the need for complementary techniques to reach the right diagnosis and thus deliver appropriate therapy.

The authors report the case of a 35 year-old polynesian male admitted to hospital with a diagnosis of myeloma. Bone marrow study showed an intense plasmocytosis, mature, Marshalko type. A new physical examination disclosed polyadenopathies and the lymph node biopsy showed lymph node modifications typical of plasma cell type Castleman's disease. At the same time, the polytypic profile of the bone marrow plasmacytosis and of the hypergammaglobulinemia was demonstrated, thus confirming the reactive state of the plasmacytosis, due to Castleman's disease. Such massive, reactive plasmacytosis belong to one of the various pathological features of multicentric Castleman's disease. Thus, this disease should belong to the etiologic diagnosis of intense bone marrow plasmacytosis and be distinguished from multiple myeloma, sometimes associated to Castleman's disease, especially in case of POEMS syndrome.

Epstein-Barr virus is associated with many diseases. Today, the pathologist may study either by immunohistochemistry or in situ hybridization on tissue sections: EBV genome, EBV messenger RNA, EBV latent and replicative proteins. Several technics can be performed on fixed paraffin-embedded tissue to demonstrate the presence of EBV DNA, EBER-1 RNA, LMP-1 protein. Frozen tissues are required for the study of EBNA-2, ZEBRA and replicating proteins expression. The results, obtained during the study of benign and malignant proliferations always or often associated with EBV, such as infectious mononucleosis, Burkitt's lymphomas, AIDS associated lymphomas, lymphoproliferations in immunocompromised patients, Hodgkin's disease, and some epithelial proliferations, are summarized.

The incidence of non-Hodgkin's malignant lymphoma is known to be increased in patients who are seropositive for the human immunodeficiency virus (HIV). We report here a multicentre retrospective study of 21 HIV-positive patients with non-Hodgkin's lymphoma seen between 1985 and 1987. All phenotype B lymphomas of intermediate or high malignancy grade according to the Working Formulation are difficult to classify histologically. Because of this problem, reexamination of the specimens by several pathologists and perhaps also the use of other morphological prognostic criteria, such as mitotic index, seem to be desirable. In more than one-third of our patients the presence of a lymphoma led to the finding of HIV seropositivity in subjects who were all issued from populations at risk. Median age was 39 years. Spread evaluation showed stage III or IV in most cases with, in 4 out of 5 patients, extranodal sites, notably the neuromeningeal system, liver, gastrointestinal tract and bone marrow. The median overall survival was 5 months, but in April 1989 2 patients had survived for more than 30 months. Obtaining complete remission (11/21 cases) was imperative for a 10 months' survival. Eight of the 11 patients in whom complete remission was obtained had received the heavy induction chemotherapy required by the degree of malignancy, but no death due to drug toxicity was recorded. 17 patients died, with active lymphoma (12 cases) and/or infection (8 cases) being documented at the time of death. The finding of more than 500/sq. mm CD4 lymphocytes in peripheral blood in 10 cases while the lymphoma was developing, and the heterogeneity of the Epstein-Barr virus (EBV) profile in serum raise the question of the role played by T-cell immunodeficiency and by EBV infection in the physiopathogenesis of these lymphomas.

The clinical and morphological features of 29 cases of nodular Hodgkin's disease, lymphocyte predominance type (NLPHD) are reported. This disease must be distinguished from other forms of Hodgkin's disease due to its nodular pattern with predominance of small lymphocytes, without sclerosis. Its main feature is the presence of rare lymphocytic histiocytic type Reed-Sternberg cells (LHRS). From a clinical point of view, we observed that the primary lymph nodes involvement are mostly seen in cervical and axillary sites, with a clinical stage I or II at diagnosis, without biological signs of activity and with a good prognosis and long duration of the disease. Two main aspects may be underlined: the frequent relapses after biopsy in lack of treatment, and a possible transformation into a large B cell lymphoma. Immunohistological stainings suggest that LH-RS cells are transformed B cells. NLPHD is a clinicopathological entity which must be currently considered and treated as Hodgkin' disease.