2D ultrasonography and contrast enhanced ultrasound for the evaluation of cavitating mesenteric lymph node syndrome in a patient with refractory celiac disease and enteropathy T cell lymphoma

Abstract

BackgroundThe cavitating mesenteric lymph node syndrome (CMLNS) is a rare manifestation of celiac disease, with an estimated mortality rate of 50%. Specific infections and malignant lymphoma may complicate its clinical course and contribute to its poor prognosis. Diagnosing the underlying cause of CMLNS can be challenging. This is the first report on contrast enhanced ultrasound (CEUS) findings in enteropathy associated T-cell lymphoma (EATL) complicating CMLNS in a gluten-free compliant patient with persistent symptoms and poor outcome.Case presentationWe present the case of a 51-year old Caucasian male patient, diagnosed with celiac disease and CMLNS. Despite his compliance to the gluten-free diet the symptoms persisted and we eventually considered the possible development of malignancy. No mucosal changes suggestive of lymphoma were identified with capsule endoscopy. Low attenuation mesenteric lymphadenopathy, without enlarged small bowel segments were seen on computed tomography. CEUS revealed arterial rim enhancement around the necrotic mesenteric lymph nodes, without venous wash-out. No malignant cells were identified on laparoscopic mesenteric lymph nodes biopsies. The patient died due to fulminant liver failure 14 months later; the histopathological examination revealed CD3/CD30-positive atypical T-cell lymphocytes in the liver, mesenteric tissue, spleen, gastric wall, kidney, lung and bone marrow samples; no malignant cells were present in the small bowel samples.ConclusionsCEUS findings in EATL complicating CMLNS include arterial rim enhancement of the mesenteric tissue around the cavitating lymph nodes, without venous wash-out. This vascular pattern is not suggestive for neoangiogenesis, as arteriovenous shunts from malignant tissues are responsible for rapid venous wash-out of the contrast agent. CEUS failed to provide a diagnosis in this case.

News in Celiac Disease about Differential Diagnosis from Romania

“…These conclusions are in line with the recommendations of the European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) [48]. A rare manifestation of CD was described, namely: the cavitating mesenteric lymph node syndrome with an estimated mortality rate of 50% [49]. Mosteanu et al proposed spiral enteroscopy for patients who are developing complications during CD follow-up [50].…”

Enteropathy‐associated intestinal T‐cell lymphoma in cavitating mesenteric lymph node syndrome: Fine‐needle aspiration contributes to the diagnosis

“…This, however, may underestimate the true mortality due to limited follow‐up in some reports (median reported follow‐up (months): 12, mean: 17, range 0–72). Lymphoma was diagnosed in 7 patients (24%) including 5 cases reported since 2008 which may reflect a recent increase in awareness for this significant complication and/or increasing use of ancillary techniques for lymphoma diagnosis.…”

Cavitating Mesenteric Lymph Node Syndrome and Enteropathy-Associated T Cell Lymphoma as First Manifestation of Celiac Disease

“…In the first case, the CMLNS and EATL developed in a CD patient with persisting symptoms, despite adherence to the gluten-free diet. 5 The second patient…”