[Severe complications of systemic treatment in thoracic oncology]. / Complications sévères des traitements systémiques du patient cancéreux thoracique (complications hors infections et urgences respiratoires).

Primary thoracic cancers affect a large number of patients, mainly those with lung cancer and to a lesser extent those with pleural mesothelioma and thymic tumours. Given their frequency and associated comorbidities, in patients whose mean age is high, these diseases are associated with multiple complications. This article, the last of a series dedicated to emergencies in onco-haematological patients, aims to present a clinical picture of the severe complications (side effects, immune-related adverse events) associated with systemic treatments, excluding infections and respiratory emergencies, with which general practitioners and specialists can be confronted. New toxicities are to be expected with the implementation of innovative therapeutic approaches, such as CAR-T cells, along with immunomodulators and antibody-drug conjugates.

Impact of adjuvant radiotherapy and chemotherapy on thymoma.

PURPOSE: Thymoma is a rare tumour. The most common treatment for thymoma is surgical resection, while the use of radiotherapy and chemotherapy remains controversial. PATIENTS AND METHODS: We conducted a monocentric observational study of 31 patients diagnosed with thymoma from June 2004 to July 2020 at cancer centre in Strasbourg, France. We analysed the outcomes of the patients. RESULTS: The 2- and 5- year locoregional relapse-free survival rates were 96.3% (95% confidence interval [CI]: 76.5-99.5%) and 68.0% (95% CI: 43.8-83.5%), respectively. Radiotherapy and chemotherapy significantly improved local tumour control (P=0.0008 and 0.04, respectively), while a larger initial tumour size significantly worsened local control rates (P=0.04). The 5- and 10-year overall survival rates were 87.1% (95% CI: 69.2-95%) and 81.7% (95% CI: 60.3-92.2%), respectively. The median overall survival was not reached, and no favourable factor was retrieved. For relapsed patients, the median overall survival after relapse was 115 months. CONCLUSION: Despite the inherent limitations of retrospective studies with a limited patient sample size, we demonstrated that chemotherapy and radiotherapy in addition to surgery were effective in achieving local control and contributed to improving patient outcomes in thymoma. Notably, an aggressive treatment strategy at the time of relapse resulted in favourable outcomes for retreated patients.

[Thymoma and squamous thymic carcinoma diagnosis; experience from the RYTHMIC network]. / Diagnostic des thymomes et carcinomes thymiques malpighiens ; expérience du réseau RYTHMIC.

The RYTHMIC network, supported by the French National Cancer Institute is dedicated to the management of patients with thymic epithelial tumors through regional and national multidisciplinary tumor boards. Tumor board decisions are based on the initial pathology diagnoses. However, following clinical inclusion in the network, a central pathology review is organized, implicating a panel of pathologists, for histotype and stage classification, which is different from a classical second opinion from pathologist to pathologist for a difficult case. Thanks to the participation of all French pathologists, more than 1000 cases have been reviewed by the panel. The aim of this review is to share with the French pathology community, the experience of the group. It underlines the importance of macroscopy and surgeon-pathologist involvement to allow a good central review, the main histopathological and immunophenotypical patterns of the most frequent thymomas and thymic carcinoma types, the differential diagnoses, as well as the difficulties for the panel to reproducibly assess on slides, stage, for some cases.

[Ileum and brain, unusual metastatic sites for a thymoma]. / Iléon et encéphale, des sites métastatiques inhabituels pour un thymome.

INTRODUCTION: Thymomas are epithelial neoplasms of thymic origin, preferentially localized in the anterior mediastinum. Recurrences after surgery are uncommon and usually occur in the intrathoracic area. The occurrence of extra-thoracic metastases is an unusual phenomenon. CASE REPORT: Here we report the case of a 61-year-old man with no special medical history. He smoked about 40 pack years but stopped in 1999. Initially he presented with a mediastinal thymoma and underwent surgical resection. One year later the development of abdominal pain and bowel disorders lead to the discovery of an ileal ulcero-necrotic tumour. After surgical resection, histological examination revealed secondary thymoma. A few months later he underwent cerebral MRI because of neurological symptoms. This revealed a second metastasis located in the brain. Stereotactic radiotherapy led to an improvement. After more than one year of follow-up the patient developed a papillary thyroid carcinoma but there were no signs of recurrence of the thymoma. CONCLUSIONS: Extra-thoracic metastases of thymoma are exceptional but their existence should not be overlooked. Their management is not standardised because of lack of data in the literature. Though surgical excision in oligo-metastatic subjects is a frequently reported therapeutic option, a radiotherapeutic approach, particularly in cerebral situations, could be a credible alternative.

[Thymoma and autoimmune diseases]. / Thymomes et maladies auto-immunes.

The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Autoimmune diseases occur most often before thymectomy, but they can be discovered at the time of surgery or later. Two situations require the systematic investigation of a thymoma: the occurrence of myasthenia gravis or autoimmune erythroblastopenia. Nevertheless, the late onset of systemic lupus erythematosus or the association of several autoimmune manifestations should lead to look for a thymoma. Neither the characteristics of the patients nor the pathological data can predict the occurrence of an autoimmune disease after thymectomy. Thus, thymectomy usefulness in the course of the autoimmune disease, except myasthenia gravis, has not been demonstrated. This seems to indicate the preponderant role of self-reactive T lymphocytes distributed in the peripheral immune system prior to surgery. Given the high infectious morbidity in patients with thymoma, immunoglobulin replacement therapy should be considered in patients with hypogammaglobulinemia who receive immunosuppressive therapy, even in the absence of prior infection.

[Thymomectomy by minimally invasive surgery. Comparative study videosurgery versus robot-assisted surgery]. / Traitement des thymomes par chirurgie mini-invasive. Étude comparative vidéochirurgie versus voie robot-assistée.

OBJECTIVES: To report the results of minimally invasive surgery in patients with stage I or II thymoma in the Masaoka classification. The reference technique is partial or complete thymectomy by sternotonomy. METHODS: A retrospective single-center study of a prospective database including all cases of thymoma operated from April 2009 to February 2015 by minimally invasive techniques: either videosurgery (VATS) or robot-assisted surgery (RATS). The surgical technique, type of resection, length of hospital stay, postoperative complications and recurrences were analysed. RESULTS: Our series consisted of 22 patients (15 women and 7 men). The average age was 53 years. Myasthenia gravis was present in 12 patients. Eight patients were operated on by VATS and 14 patiens by RATS. There were no conversions to sternotomy and no perioperative deaths. The mean operating time was 92min for VATS and 137min for RATS (P<0.001). The average hospital stay was 5 days. The mean weight of the specimen for the VATS group was 13.2 and 45.7mg for the RATS group. Twelve patients were classified Masaoka stage I and 10 were stage II. According to the WHO classification there were 7 patients type A, 5 type AB, 4 type B1, 4 type B2 4 and 2 type B3. As proposed by the Group ITMIG-IASLC in 2015 all patients corresponded to group I. The mean follow-up period was 36 months. We noted 3 major perioperative complications according to the Clavien-Dindo classification: one pneumonia, one phrenic nerve paralysis and one recurrent laryngeal nerve palsy. We observed one case of local recurrence at 22 months. Following surgery 4 patients were treated with radiotherapy and 2 patients with chemotherapy. CONCLUSIONS: The minimally invasive route is safe, relatively atraumatic and may be incorporated in the therapeutic arsenal for the treatment of Masaoka stage I and II thymoma as an alternative to conventional sternotomy. RATS and VATS are two minimally invasive techniques and the results in the short and medium term are acceptable. The clinical advantages of one over the other are sifficult to establish. RATS could handle larger and more complex lesions in view of the weight and size of the operating instrument.

[Thymic disease associated with nephrotic syndrome: a new case with membranous nephropathy and literature review]. / Les syndromes néphrotiques para-thymiques : revue de la littérature à propos d'une observation.

INTRODUCTION: Malignant thymoma or thymic hyperplasia is associated with various autoimmune diseases. Renal disease has rarely been reported in this condition. We report a new case with improvement of renal disease after thymectomy. CASE REPORT: A 77-year-old-women with nephritic syndrome was found to have associated thymic mass. Renal pathology showed membranous nephropathy. The thymic mass pathology showed a B2 type thymoma. After thymectomy the nephrotic syndrome improved. CONCLUSION: Glomerulopathy can be secondary to an acquired thymic disease. Membranous nephropathy but also other glomerular diseases can be observed often presenting with nephritic syndrome. Despite the rarity of this association this clinical observation underlines that a thymoma should be searched in the presence of a glomerulopathy. The glomerulopathy can be improved by the treatment of the thymoma.

[Rythmic-pathology: the French national pathology network for thymic epithelial tumours]. / Rythmic-Pathologie : le groupe français de relecture des tumeurs épithéliales thymiques du réseau clinique Rythmic.

Epithelial thymic tumours are rare and sometimes difficult to classify. Since 2010, the French National Cancer Institute supports a French national network, called Rythmic, devoted to the treatment of these tumours through regional and national multidisciplinary conferences using the web. All the tumours are secondarily reviewed by a French pathology national network for classification and staging. This review focuses on the presentation of the Rythmic network, and mainly to the Pathology review process.

[Myasthenia, from the internist's point of view]. / La myasthénie du côté de l'interniste.

Myasthenia gravis is an autoimmune disease due to specific antibodies inducing a neuromuscular transmission defect causing muscle fatigability. If onset of the disease may be at any age, myasthenia gravis concerns mostly young adults, in majority females. The disease characteristic features are the following: ocular symptoms (ptosis or diplopia) as main initial manifestation, extension to other muscles in 80 % of the cases, variability of the deficit, effort induced worsening, successive periods of exacerbation during the disease course, severity depending on respiratory and swallowing impairment (if rapid worsening, a myasthenic crisis is to be suspected), association with thymoma in 20 % of patients and with other various autoimmune diseases, most commonly hyperthyroidism and Hashimoto's disease. Diagnosis relies on the clinical features, improvement with cholinesterase inhibitors, detection of specific autoantibodies (anti-AChR or anti-MuSK), and significant decrement evidenced by electrophysiological tests. The points concerning specifically the internist have been highlighted in this article: diagnostic traps, associated autoimmune diseases, including inflammatory myopathies that may mimic myasthenia gravis, adverse effects of medications commonly used in internal medicine, some of them inducing myasthenic syndromes. The treatment is well codified: the treatment is well codified: (1) respect of adverse drugs contra-indications, systematically use of cholinesterase inhibitors, (2) thymectomy if thymoma completed with radiotherapy if malignant, (3) corticosteroids or immunosuppressive agent in severe or disabling form, (4) intensive care unit monitoring, plasmapheresis or intravenous immunoglobulins for patients with myasthenic crisis.

[Thymoma-associated myasthenia gravis: Clinical features and surgical results]. / La myasthénie associée aux thymomes : particularités cliniques et résultats chirurgicaux.

OBJECTIVE: The aim of this study was to compare the characteristics of myasthenic patients with and without thymoma, and the results of thymectomy in both types of patients. MATERIAL AND METHODS: A retrospective study was conducted among 66 patients who underwent thymectomy for myasthenia gravis in our department over a 10-year period (2000-2010). The surgical approach was sternotomy or anterolateral thoracotomy. Patients were divided into two groups according to the presence of thymoma: with (T-MG) and without (NT-MG) thymoma. Complete stable remission (CSR) was the primary endpoint. RESULTS: Median age was 35.09±9.89 years. The NT-MG group had 38 patients (57.57%) and the T-MG group 28 patients (42.43%). There was no difference between the two groups regarding the surgical approach (P=0.52). T-MG patients were older (40.54±15.16 vs. 31.37±9.46) (P=0.008) and predominantly male. There were more generalized forms (P=0.01) and more bulbar involvement (P=0.02) in the T-MG group. The rate of CSR at 5 years was 7% and 17% in the T-MG and NT-MG patients respectively (P=0.70). At 10 years, it was 36% and 94.73% respectively (P=0.03). CONCLUSION: Thymomatous myasthenia gravis is characterized by the severity of its clinical features. Remission rate at 10 years was significantly lower in the myasthenia with thymoma group.