Primary gastric amyloidosis associated with linitis plastica and delayed progression to systemic amyloidosis and multiple myeloma.

We report the case of a woman in her 50s who underwent, 5 years prior, a total gastrectomy after neoadjuvant chemotherapy for diffuse-type gastric cancer diagnosed during a workup for isolated gastric primary light chain (AL) amyloidosis. At the time of diagnosis, immunoglobulins light chain measurements and bone marrow biopsy were performed to rule out multiple myeloma and came back normal. Three years later, the patient developed systemic amyloidosis involving the heart and the lungs, after which she developed multiple myeloma. Isolated amyloid deposits in the stomach are a rare finding. While AL amyloidosis is frequently found in concomitance with multiple myeloma, late progression of primary AL amyloidosis to systemic amyloidosis and multiple myeloma is uncommon.

pCLE detects mucosal neoplastic vascular pattern in gastric linitis plastica.

Linitis plastica (LP) is a very aggressive and rare carcinoma with a scirrhous stroma that affects the submucosal and muscular layers of the stomach even without mucosal alterations. Lack of timely diagnosis is a crucial problem related to its prognosis and treatment. In this study, we investigated the LP-associated vascular pattern as a possible means to improve the diagnosis of these patients. During standard endoscopy, mucosal architecture, tortuosity and enlargement of vessels, as well as the presence of vascular leakage and efficiency of the blood flow were assessed in six LP patients using probe-based Confocal Laser Endomicroscopy (pCLE). In all LP patients, we detected abnormal changes in vasculature. The aberrant features of the vascular network were common to all LP patients examined and consisted of vessel enlargement, tortuosity, and leakage associated with the affected submucosal layer. This is the first study to highlight the presence of marked vascularization associated with LP, characterized by the presence of abnormal and non-functional vessels, similar to what is observed in neoplastic tissues. Therefore, the analysis of LP by pCLE may provide a new endoscopic approach and strategy to better define these patients.

Incomplete resection and linitis plastica are factors for poor survival after extended multiorgan resection in gastric cancer patients.

The aim of this retrospective study was to analyze the morbidity, mortality, and survival rates of extended multiorgan resection (EMR) for locally advanced gastric cancer patients compared to gastrectomy alone and a palliative operation. 893 locally advanced gastric cancer patients without distant metastasis had surgery including gastrectomy alone (GA group, n = 798), EMR resection (EMR group, n = 75), and palliative operation (palliative gastrectomy or gastrojejunostomy (PO group, n = 20)). Postoperative mortality and complication rates in the EMR group were significantly higher than in the GA group (2.7% vs 0.4%, P = 0.010 and 25.3% vs 8.1%, P < 0.001, respectively), but similar in the PO group. The median survival time of the EMR group was significantly longer than in the PO group (27 months vs 11 months, P = 0.020), but significantly worse (P = 0.020) than in the GA group (44 months). Incompleteness of resection (R1) and linitis plastica were independent prognostic factors for survival in the EMR group. Three different gastric cancer surgeries led to different postoperative mortality and complication rates. EMR had a better survival rate compared with PO while GA had the longest survival time with the lowest mortality and complication rates.

[Lymphedema of the lower limbs: Initial manifestation of gastric linitis plastica]. / Lymphœdème des membres inférieurs révélant une linite gastrique.

BACKGROUND: Primary lymphedemas are constitutional abnormalities of the lymphatic system. Secondary lymphedemas occur after damage to the lymphatic system, mainly after cancer treatments or tumour mass compression. There are many other causes, including filariasis, which is nonetheless very rare in France. PATIENTS AND METHODS: A 52-year-old man presented with a two-month history of increased size of the left leg. He was asymptomatic and in good general condition. Clinical examination revealed non-pitting lymphedema and ipsilateral hydrocele without loco-regional compressive lymph node. Initial extensive explorations were unremarkable. Lymphoscintigraphy revealed low tracer fixation in the left leg. The symptoms continued to worsen, with exacerbation and bilateralization of the lymphedema. Two months later, axillary lymph nodes appeared corresponding to metastasis from a signet-ring cell carcinoma. Despite two lines of chemotherapy, the patient died 8 months later due to multiple metastatic disease. DISCUSSION: Our case is remarkable because the lymphedema was not related to extrinsic compression and was the first symptom of gastric cancer. In the absence of compression, endo-lymphatic micro-metastases could constitute the causative process. Acquired lymphedema of the lower limbs must be recognized as a potential early symptom of gastric carcinoma and should therefore prompt further investigations.

Advanced gastric cancer linitis plastica presented with disseminated intravascular coagulation.

An old man was found unconscious; on admission found to have disseminated intravascular coagulation with concern of upper gastrointestinal bleed after he was found to have melena. Esophagogastroduodenoscopy on admission showed diffuse thickened gastric folds, and biopsy showed mucosal oedema. Bone marrow biopsy concerning for lymphoma was obtained showed adenocarcinoma. MRI of the abdomen was significant for diffuse gastric wall thickening. A repeat endoscopic ultrasound showed a diffuse gastric wall thickening of 15 mm and submucosal tunneling technique biopsy suggested high-grade, invasive, signet ring adenocarcinoma of the stomach. Oncology was consulted to initiate palliative chemotherapy. In retrospect, the patient was questioned regarding gastrointestinal symptoms; he reported gradual early satiety, dysphagia and unintentional weight loss over the course of 4 months.

[Wernicke encephalopathy accompanying linitis plastica]. / Wernicke-encephalopathia linitis plasticában.

Wernicke encephalopathy (or Wernicke-Korsakoff encephalopathy) is a rarely diagnosed neurological disorder, which is caused by vitamin B1 deficiency. In the classical form it is characterized by a typical triad (confusion, oculomotor disturbance and ataxia), however, in the majority of the cases only confusion is present. It can be frequently observed in subjects with chronic alcohol consumption, but it may accompany different pathological states of which end stage malignant diseases are the most importants, where confusion may have different backgrounds. The authors present the case of an old male patient with advanced gastric cancer recognised and treated vitamin B1 deficiency, and they draw attention to difficulties of the diagnosis of Wernicke's disease.

[From retroperitoneal fibrosis to gastric linitis]. / De la fibrose rétropéritonéale à la linite gastrique.

Retroperitoneal fibrosis may reveal many diseases, including neoplasias. An 83-year-old man presented with an acute renal failure due to compressive retroperitoneal fibrosis. Clinically the only abnormal feature was a cutaneous subclavicular infiltrated lesion and laboratory features included hypereosinophilia, anemia and elevated acute phase reactants. A thoracic CT-scan showed pleuritis and para-esophageal lymph node and the 18FDG-PET-scan an hypermetabolism lesion of the oesophagus. Gastroscopy and colonoscopy found a gastric linitis, already multi-metastatic at diagnosis. The gastric linitis can present with many decepting clinical forms, increasing the risk of delayed diagnosis.

Facing the challenge of managing linitis plastica--review of the literature.

Linitis Plastica (LP) denotes a diffuse, intra-murally infiltrating, anaplastic carcinoma in a hollow structure resulting in a shrunken organ with thickened walls. The neoplasm is encountered in every segment of gastrointestinal tract with the gastric localization being the most frequent and simultaneous or successive affection of different digestive sites rarely reported. Symptoms often associated with LP of the stomach include pain and lump in the epigastrium, weight loss, anorexia, dysphagia, nausea and vomiting. Diagnosis of LP-type adenocarcinomas of the stomach has traditionally been achieved by brush cytology and mucosal biopsy. Nevertheless, these techniques may yield false negative results due to potential submucosal location of the lesion. Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) contributes to the detection of abundant, independent malignant cells indicative of an anaplastic neoplasm. The characteristic histopathological feature of this entity is cellular spread to the submucosa and stroma with minimal mucosal alterations accompanied by an excessive desmoplastic reaction. Despite recent research on alternative therapies, surgical resection appears the only potentially curative approach. The aim of this review was to evaluate the results of current diagnostic modalities and surgical interventions for LP with special reference to the extent of its histological dissemination and to present the recent literature in order to provide an update on the contemporary concepts of therapeutic management of the disease.