Shwachman-Diamond syndrome and solid tumors: Three new patients from the French Registry for Severe Chronic Neutropenia and literature review.

Shwachman-Diamond syndrome with Shwachman-Bodian-Diamond syndrome (SBDS) biallelic variants is a rare disorder that predisposes the carrier to malignant hemopathies but solid-cancer predisposition is poorly known. Among 155 cases entered in the French Registry for Severe Chronic Neutropenia, three were identified with malignant solid tumors (ovary, breast, and esophagus). All cancers occurred during the fifth decade and, despite being localized at diagnosis, were rapidly fatal thereafter. No cancer was observed post transplantation in the 14 HSCT survivors. Based on the literature and our patient data, we can merely advance that this complication is predominantly diagnosed in adults.

Role of Unit Physicians and Challenges Encountered in the Follow-up of Military Personnel with Cancer.

INTRODUCTION: General practitioners (GPs) play a central role in the management and coordination of care of patients with malignant tumors and blood diseases. Civilian GPs encounter certain difficulties during the care of such patients. The practice of unit medicine in a military environment differs from that in a civilian context through expertise in fitness to serve and to deployment and the target population. We identified the difficulties encountered by "unit" physicians during and after cancer treatment. MATERIALS AND METHODS: We conducted a multicenter cross-sectional descriptive study from July 2, 2021, to September 30, 2022, targeting all military GPs belonging to the French Armed Forces Health Service. We sent a questionnaire consisting of 1 open- and 16 closed multiple-choice questions describing the population of unit physicians and their patients (questions 1-5), the difficulties encountered by physicians in the follow-up of military personnel with cancer (Questions 6, 7, 11, 12, and 13), and the potential information networks accessible to physicians (questions 8-10, 14, and 17). RESULTS: Three hundred and ninety physicians completed the questionnaires. Among the 700 military GPs, 390 physicians responded to the questionnaire and 327 completed it exhaustively. The questionnaire response rate was 55%. Of the responding physicians, 49% and 70% reported following patients with an "active" malignant tumor and a malignant tumor pathology in remission, respectively. Thirty-one percent of the physicians encountered difficulties with these patients as follows: 26% concerning fitness for duty, 17% in medical follow-up, 14% in addressing the psychological aspect, 11% concerning specialist accessibility for advice, 10% in managing deconditioning to effort, 9% in addressing the social aspect, 7% in medical management, and 6% concerning other issues. CONCLUSIONS: Difficulties in the follow-up of patients with cancer affect military doctors. They mainly concern fitness for duty and medical follow-up.

Role of Cancer Surgery in the Improvement of the Operative Skills of Military Surgeons During Deployment: A Single-Center Study.

INTRODUCTION: The maintenance of military surgeons' operative skills is challenging. Different and specific training strategies have been implemented in this context; however, little has been evaluated with regard to their effectiveness. Cancer surgery is a part of military surgeons' activities in their home hospitals. This study aimed to assess the role of oncological surgery in the improvement of military surgeons' operative skills. METHODS: Between January and June 2019, the surgical activities of the departments of visceral, ear, nose, and throat, urological, and thoracic surgery were retrospectively reviewed and assessed in terms of the operative time (OT). All surgeons working at the Sainte Anne Military Teaching Hospital were sent a survey to rate on a 5-point scale the current surgical practices on their usefulness in improving surgical skills required for treating war injuries during deployment (primary endpoint) and to compare on a 10-point visual analog scale the influence of cancer surgery and specific training on surgical fluency (secondary endpoint). RESULTS: Over the study period, 2,571 hours of OT was analyzed. Oncological surgery represented 52.5% of the surgical activity and almost 1,350 hours of cumulative OT. Considering the primary endpoint, the mean rating allocated to cancer surgery was 4.53 ± 0.84, which was not statistically different than that allocated to trauma surgery (4.42 ± 1.02, P = 0.98) but higher than other surgery (2.47 ± 1.00, P < 0.001). Considering the secondary endpoint, cancer surgery was rated higher than specific training by all surgeons, without statistically significant difference (positive mean score of + 2.00; 95% IC: 0.85-3.14). CONCLUSION: This study demonstrates the usefulness of cancer surgery in improving the operative skills of military surgeons.

Ophthalmic manifestations in IgG4-related disease: Clinical presentation and response to treatment in a French case-series.

IgG4-related disease (IgG4-RD) is characterized by variable tissue or organ involvements sharing common pathological findings. Orbital or orbital adnexa involvement of the disease has been reported in a few case series. The aim of our study was to characterize and analyze ophthalmic manifestations from a nationwide French case-series.Patients with IgG4-RD and orbital or orbital adnexa involvement included in the French multicentric IgG4-RD case-registry were identified. Only patients fulfilling "modified" comprehensive diagnostic criteria with pathological documentation were retained for the study. Clinical, biological, pathological, radiological findings and data regarding the response to treatment were retrospectively analyzed.According to our data registry, the frequency of IgG4-related ophthalmic disease (IgG4-ROD) was 17%. Mean age at diagnosis was 55.1 ±â€Š7.1 years with a male/female ratio of 2.2. The 19 cases of IgG4-ROD consisted of lacrimal gland (68.4%), soft tissue (57.9%), extra-ocular muscles (36.8%), palpebral (21.1%), optical nerve (10.5%), orbital bone (10.5%), and mononeuritis (V1 and/or V2, 10.5%) involvements. IgG4-ROD was bilateral in 57.9% of cases. Extra-ophthalmic manifestations were reported in 78.9% of cases. All patients responded to prednisone but two-thirds of patients relapsed within a mean (SD) of 9.8 (3.5) months and 72.2% required long-term glucocorticoids and/or immunosuppressive agents. Eight patients were treated by rituximab with a favorable response in 87.5% of cases.Lacrimal involvement is the most frequent ophthalmic manifestation of IgG4-RD and is frequently associated with extra-orbital manifestations. Despite initial favorable response to steroids, the long-term management of relapsing patients needs to be improved.