Contribution of Immune-Mediated Paraneoplastic Syndromes to Neurological Manifestations of Neuroendocrine Tumours: A Retrospective Study.

INTRODUCTION: Neurological symptoms associated with neuroendocrine tumours (NETs) may be related to metastatic disease or paraneoplastic syndromes (PNSs); these last are often associated with autoantibodies targeting various onconeural antigens. To better characterize neurological PNSs related to NETs, we report the largest case-series study to date. METHODS: We retrospectively reviewed the charts of all patients diagnosed with NETs of the gastrointestinal tract who presented with neurological symptoms at either of 2 tertiary academic hospitals (Henri Mondor and Beaujon, France) between 1994 and 2016. All patients underwent extensive neurological tests including clinical, laboratory, and radiological investigations. The clinical response to immunomodulating agents was recorded. RESULTS: In the 13 identified patients, the most common presentations were peripheral neuropathy (46.2%) and encephalopathy (26.6%). Of the 6 (53.3%) patients whose serum anti-neuronal antibodies were assayed, 5 had high titres. Short-term oral corticosteroid and immunosuppressant drug therapy was given to 4 of these patients, of whom 3 had a clinical response and 1 no response. Repeated high-dose intravenous immunoglobulin therapy induced a complete clinical response in 1 patient. Encephalopathy resolved fully after hepatectomy or intrahepatic chemoembolization for liver metastases in another 2 patients. DISCUSSION: The neurological symptoms associated with NETs may be due in part to autoimmune PNS. Based on experience at our 2 centres, we estimate that autoimmune PNS occurs in about 1% of patients with NETs. Early symptom recognition allows the initiation of effective treatments including corticosteroids, immunosuppressive drugs, and/or intravenous immunoglobulins.

Interest of Integrated Whole-Body PET/MR Imaging in Gastroenteropancreatic Neuroendocrine Neoplasms: A Retro-Prospective Study.

INTRODUCTION AND AIM: Simultaneous positron emission tomography/magnetic resonance imaging (PET-MRI) combines the high sensitivity of PET with the high specificity of MRI and is a tool for the assessment of gastroenteropancreatic neuroendocrine neoplasms (G-NENs). However, it remains poorly evaluated with no clear recommendations in current guidelines. Thus, we evaluated the prognostic impact of PET-MRI in G-NEN patients. METHODS: From June 2017 to December 2021, 71 G-NEN patients underwent whole-body PET-MRI for staging and/or follow-up purposes. A whole-body emission scan with 18F-6-fluoro-L-dihydroxyphenylalanine (18FDOPA, n = 30), 18F-fluoro-2-deoxy-D-glucose (18FDG, n = 21), or 68Ga-(DOTA(0)-Phe(1)-Tyr(3))-octreotide (68Ga-DOTATOC, n = 20) with the simultaneous acquisition of a T1-Dixon sequence and diffusion-weighed imaging (DWI), followed by a dedicated step of MRI sequences with a Gadolinium contrast was performed. The patients underwent PET-MRI every 6-12 months during the follow-up period until death. Over this period, 50 patients with two or more PET-MRI were evaluated. RESULTS: The mean age was 61 [extremes, 31-92] years. At the baseline, PET-MRI provided new information in 12 cases (17%) as compared to conventional imaging: there were more metastases in eight, an undescribed location (myocardia) in two, and an unknown primary location in two cases. G grading at the baseline influenced overall survival. During the follow-up (7-381 months, mean 194), clinical and therapy managements were influenced by PET-MRI in three (6%) patients due to new metastases findings when neither overall, nor disease-free survivals in these two subgroups (n = 12 vs. n = 59), were different. CONCLUSION: Our study suggests that using PET/MRI with the appropriate radiotracer improves the diagnostic performance with no benefit on survival. Further studies are warranted to evaluate the cost-effectiveness of this procedure.

Surgical treatment of papillary thyroid carcinoma.

Papillary carcinoma accounts for 85% of differentiated thyroid cancers. We witness a yearly increased incidence and this is may be, in part, due to the increasing use of neck ultrasonography. Certain histologic subtypes of papillary carcinoma have a worse prognosis related to vascular invasion, invasion into extrathyroidal tissues, extensive tumor necrosis and/or mitoses. Adequate surgery is the most important treatment variable influencing prognosis, while radioactive iodine treatment, TSH suppression, and external beam irradiation each play adjunctive roles in at least some patients. Numerous schemes have been developed in an effort to achieve more accurate risk factor stratification. Each of the schemes allows accurate identification of the majority (70-85%) of patients at low risk of mortality (T1-3, M0 patients), allowing the follow-up and management of these patients to be less intensive than the higher-risk minority (T4 and M1 patients), who may benefit from a more aggressive management strategy. Overall 5- and 20-year survival in the low-risk group was 100% and 99% respectively. However, the survival in the high-risk group dropped to almost 72% and 57% respectively.

Outcomes after double switching from originator Infliximab to biosimilar CT-P13 and biosimilar SB2 in patients with inflammatory bowel disease: a 12-month prospective cohort study.

BACKGROUND: There are few data regarding multiple switching from the originator Infliximab to its biosimilars. AIM: To assess outcomes and patient perspectives in a prospective manner after double switching from Infliximab to the biosimilars CT-P13 and SB2. METHODS: A total of 158 consecutive patients with inflammatory bowel disease (IBD) receiving CT-P13 maintenance therapy were switched to SB2 and followed for 54 weeks. Patients were stratified according to previous switch from the originator Infliximab to CT-P13 (double switch group) or not (single switch group). RESULTS: The drug persistence was high (94.9%) after 54 weeks. In total, 17 (10.8%) patients experienced loss of response to SB2, including 10 patients who were managed through dose optimisation and continued treatment. No changes were observed in clinical activity scores, fatigue, biological activity and pharmacokinetical parameters after the switch. The safety profile was in line with the current knowledge of Infliximab. According to the Beliefs about Medicines Questionnaire, the patients' perspectives did not change after switching from CT-P13 to SB2. The primary patient concerns remained after the switch, which were focused on effectiveness and safety rather than on the molecular differences between originator and biosimilars or socioeconomic benefits. There were also no differences in the concerns and beliefs between the double and single switch groups. CONCLUSION: Double switching from the originator Infliximab to CT-P13 and then to SB2 was not associated with an impairment in patient beliefs, while the effectiveness, immunogeniity and safety of anti-TNF therapy remained stable after 54 weeks of follow-up.

Effectiveness and Safety of Subcutaneous Rituximab for Patients With Gastric MALT Lymphoma: A Case-Control Comparison With Intravenous Rituximab.

INTRODUCTION: Rituximab is a standard treatment for gastric mucosa-associated lymphoid tissue (MALT) lymphoma (GML). We sought to compare the effectiveness and safety of subcutaneous and intravenous rituximab in a retrospective case-control study. PATIENTS AND METHODS: All consecutive patients with GML treated with subcutaneous rituximab between January 2017 and December 2018 were included and compared to 3 matched control patients (based on Ann Arbor classification, presence of t(11;18) translocation, history of treatment, and type of current treatment) treated with intravenous rituximab between January 2000 and December 2018. Patients with t(11;18) translocation were treated with rituximab in combination with chlorambucil; the other patients were treated with rituximab alone. Effectiveness was assessed at week 52, and safety was assessed through weeks 0 to 52 and compared by the chi-square test. RESULTS: Twenty-five patients were included in the subcutaneous rituximab group and 75 in the intravenous group. There was no difference between the groups in complete remission (78% vs. 76%, P = .99) or overall response rates (91% vs. 89%, P = .99) at week 52. Safety profiles were similar in both groups, with a significant decrease in postinduction grade 2 injection-related reactions and outpatient hospital length of stay in the subcutaneous rituximab group. CONCLUSION: In a small case-control study, we did not find any difference in the effectiveness or safety profiles between subcutaneously and intravenously delivered rituximab for the treatment of patients with GML. We found a decrease in postinduction grade 2 injection-related reactions and outpatient hospital length of stay in the subcutaneous rituximab group.

Determinants of IBD-related disability: a cross-sectional survey from the GETAID.

BACKGROUND: The burden of inflammatory bowel disease (IBD) is rising worldwide. The goal of IBD treatment is to achieve clinical and endoscopic remission but also prevent disability. AIMS: To identify the predictive factors of disability in a large population of patients with IBD. PATIENTS AND METHODS: We conducted a cross-sectional survey in 42 tertiary centres in France and Belgium. A self-administered questionnaire was designed to explore patients and their IBD characteristics. IBD-disk is a validated tool to measure disability in patients with IBD. The IBD-disk score was then calculated for each patient. Based on a previous study, an overall IBD-disk score ≥40 was associated with moderate-to-severe disability. RESULTS: Among the 2011 patients, 1700 were analysed, including 746 (44%) in self-reported clinical remission and 752 (44.2%) declaring clinical activity. The patient global assessment of global remission was missing in 200 (11.8%) of 1700 patients. Moderate-to-severe disability was significantly increased in patients with BMI >25 kg/m2 (OR = 1.66; 95% CI [1.29-2.14]), in those having perception of need for a psychotherapist (OR = 2.24; 95% CI [1.79-3.05]) and social worker (OR = 1.54; 95% CI [1.08-2.21]). Conversely, male gender (OR = 0.83; 95% CI [0.69-0.99]), ulcerative colitis (OR = 0.69; 95% CI [0.53-0.92]), self-reported clinical remission (OR = 0.59; 95% CI [0.46-0.77]) and employed or student occupational status (OR = 0.69; 95% CI [0.52-0.92]) were inversely correlated with disability. Overall, 257 (34.5%) patients who declared being in clinical remission had disability. CONCLUSION: Determinants of IBD-related disability include IBD-related factors but also psychological and social factors. This highlights the importance of a multidisciplinary team in the management of patients with IBD.

Colonic mucosa-associated lymphoid tissue lymphoma: a case series.

Primary colonic mucosa-associated lymphoid tissue (MALT) lymphoma accounts for less than 0.5% of all colon cancers. We report 9 cases of colonic MALT lymphomas (median follow up: 9 [IQR 1-26] years). The disease was multi-focal in 4 patients: the most frequent sites were the cecum in 4 and the rectum in 3 patients. The main endoscopic finding was a polypoid lesion. The treatment modalities were rituximab +/- an alkylating agent (n = 5), an alkylating agent alone (n = 2), surgical resection (n = 1), and endoscopic resection (n = 1). Remission was achieved in 8 cases. Three patients relapsed, and 2 were re-treated. At the end of the study period, 67% of the patients were in remission. All patients were symptom-free. This current series of colonic MALT lymphomas shows the indolent nature of the disease, which may be treated with various modalities.

Patients' perspectives after switching from infliximab to biosimilar CT-P13 in patients with inflammatory bowel disease: A 12-month prospective cohort study.

BACKGROUND: Patients' perspectives after switching from infliximab to a biosimilar have yet to be assessed. AIM: To assess patients' perspectives in a prospective manner after switching from infliximab to CT-P13. METHODS: 113 consecutive patients with inflammatory bowel disease (IBD) on maintenance therapy with infliximab were switched to CT-P13. Patients' perspectives were assessed by questionnaires, including the Beliefs about Medicines Questionnaire (BMQ) and FACIT-F (questionnaire regarding fatigue), and patient-reported outcomes (IBD disability index) at the inclusion and after the fourth CT-P13 infusion. RESULTS: After one year, the patients' perspectives did not change after the switch according to BMQ-general, BMQ-specific necessity and BMQ-specific concerns subscales. No difference was observed in the mean IBD-DI score, while a significant improvement in fatigue was observed according to the FACIT-F questionnaire. Patients' concerns were raised about the use of biosimilars and the risks of switching with a significant improvement after switching (65% vs. 42%, respectively, p = 0.01). Fourteen (12.4%) patients experienced loss of response to CT-P13, including 12 with restoration of steroid-free clinical remission after CT-P13 dose optimization. CONCLUSION: Although some concerns were reported, no difference was observed in patients' perspectives after switching from infliximab to CT-P13.

Splenic artery aneurysm presenting as a submucosal gastric lesion: A case report.

We are reporting the rare case of splenic artery aneurysm of 4 cm of diameter presenting as a sub mucosal lesion on gastro-duodenal endoscopy. This aneurysm was treated by endovascular coil embolization and stent graft implantation. The procedure was uneventful. On day 1, the patient presented an acute severe epigastric pain and cardiovascular arrest. Abdominal computed tomography scan showed an active leak of the intravenous contrast dye in the peritoneum from the splenic aneurysm. We performed an emergent resection of the aneurysm, and peritoneal lavage. Postoperatively, hemorrhagic choc was refractory to large volumes replacement, and intravenous vaso-active drugs. On day 2, he presented massive hematochezia. We performed a total colectomy with splenectomy and cholecystectomy for ischemic colitis, with spleen and gallbladder infarction. Despite vaso-active drugs and aggressive treatment with Factor VIIa, the patient died after uncontrolled disseminated intravascular coagulation.

Omental ischemia.

Omental ischemia is a rare cause of acute abdomen. Clinical diagnosis is usually difficult because clinical signs and symptoms are similar to other common causes of abdominal pain. The most common differential diagnosis is acute appendicitis. Diagnosis is mainly based on ultrasound, and especially computed tomography scan analysis. There is, at present, no standard treatment modality for omental ischemia. When diagnosed by radiological imaging, omental ischemia can be managed conservatively. We hereby review incidence, etiology, pathology, clinical presentation, differential diagnosis, biological anomalies, radiological features, and treatment options of omental ischemia.

Treatment options of inflammatory appendiceal masses in adults.

At present, the treatment of choice for uncomplicated acute appendicitis in adults continues to be surgical. The inflammation in acute appendicitis may sometimes be enclosed by the patient's own defense mechanisms, by the formation of an inflammatory phlegmon or a circumscribed abscess. The management of these patients is controversial. Immediate appendectomy may be technically demanding. The exploration often ends up in an ileocecal resection or a right-sided hemicolectomy. Recently, the conditions for conservative management of these patients have changed due to the development of computed tomography and ultrasound, which has improved the diagnosis of enclosed inflammation and made drainage of intra-abdominal abscesses easier. New efficient antibiotics have also given new opportunities for nonsurgical treatment of complicated appendicitis. The traditional management of these patients is nonsurgical treatment followed by interval appendectomy to prevent recurrence. The need for interval appendectomy after successful nonsurgical treatment has recently been questioned because the risk of recurrence is relatively small. After successful nonsurgical treatment of an appendiceal mass, the true diagnosis is uncertain in some cases and an underlying diagnosis of cancer or Crohn's disease may be delayed. This report aims at reviewing the treatment options of patients with enclosed appendiceal inflammation, with emphasis on the success rate of nonsurgical treatment, the need for drainage of abscesses, the risk of undetected serious disease, and the need for interval appendectomy to prevent recurrence.

Idiopathic sclerosing encapsulating peritonitis: abdominal cocoon.

Abdominal cocoon, the idiopathic form of sclerosing encapsulating peritonitis, is a rare condition of unknown etiology that results in an intestinal obstruction due to total or partial encapsulation of the small bowel by a fibrocollagenous membrane. Preoperative diagnosis requires a high index of clinical suspicion. The early clinical features are nonspecific, are often not recognized and it is difficult to make a definite pre-operative diagnosis. Clinical suspicion may be generated by the recurrent episodes of small intestinal obstruction combined with relevant imaging findings and lack of other plausible etiologies. The radiological diagnosis of abdominal cocoon may now be confidently made on computed tomography scan. Surgery is important in the management of this disease. Careful dissection and excision of the thick sac with the release of the small intestine leads to complete recovery in the vast majority of cases.

Association of parathyroid pathology with well-differentiated thyroid carcinoma.

OBJECTIVE: The objective was to evaluate the association of well-differentiated thyroid carcinoma and parathyroid pathology. METHODS: The medical records of 14 patients with concomitant pathologies were retrospectively reviewed. RESULTS: Parathyroidectomies (1.3%) and thyroidectomies (3.5%) performed for well-differentiated thyroid carcinoma resulted in the diagnosis of concomitant pathologies. Five patients had a primary hyperparathyroidism (PHPT) and nine were operated for thyroidectomy with intraoperative finding of an enlarged parathyroid gland. Patients (64%) were normocalcemic preoperatively. Thirteen had papillary carcinoma. Fifty percent of patients had multiple foci of papillary microcarcinoma. Twenty-nine percent of patients had parathyroid hyperplasia. All patients with preoperative hypercalcemia normalized their serum calcium. During follow-up, thyroglobuline, calcium serum values and cervical ultrasound showed no evidence of recurrence of the diseases. CONCLUSIONS: These observations stress the importance of pre and intraoperative evaluation to detect overt thyroid and parathyroid pathology before performing a parathyroidectomy for hyperparathyroidism or a thyroidectomy for a well-differentiated thyroid carcinoma.

Cervical neck dissection without drains in well-differentiated thyroid carcinoma.

This study aims to review the safety of thyroidectomy combined with cervical neck dissection without drainage in well-differentiated thyroid carcinoma (WDTC). The medical records of consecutive patients who underwent thyroidectomy without drainage for WDTC were retrospectively reviewed. Group 1 included 123 patients who underwent thyroidectomy with central neck dissection and Group 2 included 46 patients who underwent thyroidectomy with central and lateral neck dissection. One hundred twenty-seven patients underwent thyroidectomy without neck dissection and were included in Group 3. Overall, 16 patients (5%) developed postoperative hematoma and/or seroma, seven patients (6%) in the Group 1, three patients (7%) in the Group 2, and six patients (5%) in Group 3. All patients had minor bleeding or seroma not requiring surgical intervention. Overall, 68 patients (23%) had transient postoperative hypocalcaemia, and four patients(1%) had permanent hypoparathyroidism. Seventeen patients (6%) had transient postoperative hoarseness and three had permanent vocal cord paralysis (0.6%). The postoperative stay for all groups was 1 day in 91 per cent of the cases. Patients from Groups 1 and 2 had no increased perioperative local complications or length of stay as compared with Group 3. Cervical neck dissection and thyroidectomy without drains is safe and effective in the treatment of WDTC.