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La mesenteritis esclerosante es una enfermedad no neoplásica poco común que afecta el mesenterio del intestino delgado con inflamación fibrosante crónica, hay pocos datos sobre la historia natural y las opciones terapéuticas para esta afección La mesenteritis esclerosante forma parte de un espectro de condiciones idiopáticas, de rara presentación, caracterizada por inflamación crónica del mesenterio y fibrosis, y fue descrita por primera vez en 1924. El método empleado para desarrollar el estudio, es la presentación de un caso clínico. El objetivo propuesto de este escrito, es conocer las diferentes formas de presentación de un cuadro clínico de mesenteritis. Entre los resultados importantes encontrados se describen los siguientes: La localización más frecuente es el mesenterio del intestino delgado, pero en algunos casos puede afectar al mesocolon (sigmoides). La evolución histológica progresa en 3 estadios. El primero es la lipodistrofia mesentérica en la que los macrófagos invaden el tejido mesentérico. En este estadio el cuadro es oligosintomático y de progreso benigno. El segundo es la PM en la que se observa la presencia de infiltrado de células inflamatorias plasmáticas y algunos polimorfonucleares, células gigantes de cuerpo extraño y macrófagos. Las principales conclusiones sobre el estudio, la SM es un trastorno idiopático poco común que afecta el mesenterio del intestino delgado con diversos grados de fibrosis, inflamación y necrosis grasa. Su presentación clínica es bastante variable y varía de asintomática a enfermedad debilitante. Aproximadamente el 50% de los pacientes podría no necesitar ningún tratamiento.

Ein 71-jahriger Mann stellte sich zur Abklarung eines unklaren abdominellen Tumors vor. Er berichtete uber abdominelle Schmerzen, Motilitatsstorungen und Gewichtsverlust seit einigen Wochen. Die Bildgebung zeigte eine grose mesenteriale Raumforderung mit begleitender Lymphadenopathie, geringen Aszitesansammlungen sowie venoser Kongestion. Zur Sicherung der Verdachtsdiagnose einer sklerosierenden Mesenteritis sowie zum Ausschluss eines Lymphoms erfolgte eine Laparoskopie mit Gewebeentnahme. Das Material war nicht ausreichend reprasentativ, sodass eine Laparotomie mit erneuter Gewebeentnahme durchgefuhrt wurde. Anhand des Materials wurde die seltene Diagnose einer sklerosierenden Mesenteritis bestatigt und aufgrund der Beschwerden eine medikamentose Therapie mit Tamoxifen und Prednison eingeleitet.

espanolEl termino mesenteritis esclerosante, hace referencia a una enfermedad idiopatica, rara, de curso cronico, caracterizada por cambios inflamatorios y fibroticos en el tejido adiposo del mesenterio del intestino. Se desconoce su etiologia, pero se cree que puede ser el resultado de una respuesta inflamatoria inespecifica del mesenterio a una agresion alergica, quimica, termica, infecciosa, autoinmune o quirurgica, aunque no existe evidencia cientifica que sostenga esta hipotesis. El presente articulo describe y compara diferentes literaturas sobre Sintomas, causas y tratamiento de una mesenteritis esclerosante. Para ello, se recolecta esta informacion de diferentes fuentes bibliograficas adquiridas de bases de datos (SCOPUS, PubMed, Biblioteca Cochrane, Google Scholar) valorando la calidad y veracidad de la informacion recopilada, asi como la actualidad del contenido. Los dos casos de estudio aqui presentados, confirman que esta afeccion se presenta principalmente en hombres y que el diagnostico para determinarla puede ser confuso por la similitud de otras patologias, lo que conlleva a la realizacion de multiples estudios (incluidos procedimientos laparoscopicos). En el contexto del tratamiento a menos que haya una obstruccion intestinal en donde se requiere un procedimiento quirurgico (derivaciones, resecciones parciales o colostomia) no existe un tratamiento especifico, aunque hay investigaciones que sugieren farmacos inmunosupresores e inmunomoduladores. EnglishThe term sclerosing mesenteritis refers to an idiopathic, rare, chronic disease characterized by inflammatory and fibrotic changes in the adipose tissue of the mesentery of the intestine. Its etiology is unknown, but it is believed that it may be the result of a non-specific inflammatory response of the mesentery to an allergic, chemical, thermal, infectious, autoimmune or surgical attack, although there is no scientific evidence to support this hypothesis. This article describes and compares different literatures on Symptoms, causes and treatment of sclerosing mesenteritis. To do this, this information is collected from different bibliographic sources acquired from databases (SCOPUS, PubMed, Cochrane Library, Google Scholar), assessing the quality and veracity of the information collected, as well as the timeliness of the content. The two case studies presented here confirm that this condition occurs mainly in men and that the diagnosis to determine it can be confusing due to the similarity of other pathologies, which leads to the performance of multiple studies (including laparoscopic procedures). In the context of treatment, unless there is an intestinal obstruction where a surgical procedure is required (diversions, partial resections, or colostomy), there is no specific treatment, although there is research that suggests immunosuppressive and immunomodulatory drugs.

Epiploic appendagitis is a condition resulting from ischemia or necrosis involving the appendage epiploica either due to torsion or spontaneous thrombosis of the venous outflow. It is one of the myriad causes of acute abdominal pain and can masquerade clinically as appendicitis, omental infarction, sclerosing mesenteritis and even diverticulitis. Epiploic appendagitis of the vermiform appendix is a rare entity, clinically indistinguishable from appendicitis. We present a 45-year-old male patient with 4-day duration of right iliac fossa pain and tenderness, with strong clinical suspicion of acute appendicitis. CT scan of the abdomen and pelvis demonstrated Epiploic appendagitis of the vermiform appendix, whilst the appendix remained uninflamed. The patient was thus discharged with conservative management without having to go undergo needless surgery, thereby avoiding the potential complications thereof.

Sclerosing mesenteritis (SM) is an idiopathic disorder affecting mesentery, characterized by fat necrosis, chronic inflammation and fibrosis. The clinical presentation varies from asymptomatic cases to acute abdomen. The diagnosis is suggested by imaging but can be definitely established only by biopsies. In this paper, we discuss ultrasonography-based management of SM.

Calcifying fibrous tumor (CFT) is a rare mesenchymal lesion that has been documented throughout the gastrointestinal tract. Gastrointestinal CFTs may occur at virtually any age, with a predilection for adults and for females. They occur most commonly in the stomach and the small and large intestines. CFTs are most often found incidentally, cured by local resection, and have a low risk of recurrence. Histology shows three characteristic features: Spindle cell proliferations within a densely hyalinized stroma, scattered calcifications, and lymphoplasmacytic inflammation. CFTs are immunoreactive for CD34, vimentin and factor XIIIa, helping to distinguish them from other benign mesenchymal neoplasms. The differential diagnosis of CFTs includes sclerosing gastrointestinal stromal tumor, leiomyoma, schwannoma, solitary fibrous tumor, inflammatory myofibroblastic tumor, plexiform fibromyxoma, fibromatosis, sclerosing mesenteritis, and reactive nodular fibrous pseudotumor. The pathogenesis of CFTs remains unclear, but some have hypothesized that they may be linked to IgG4-related disease, inflammatory myofibroblastic lesions, hyaline vascular type Castleman disease, sclerosing angiomatoid nodular transformation of the spleen, or trauma.

Mesenteric diseases are broadly separated into 2 groups: non-neoplastic and neoplastic. Common non-neoplastic mesenteric diseases include those involving the mesenteric vasculature and those of inflammatory processes. Mesenteric inflammatory processes can mimic a neoplastic process. Neoplastic diseases of the mesentery are rare. Generally, the morphology, behavior and diagnostic criteria for mesenteric tumors are similar to their soft tissue or organ-specific counterparts. Their recognition can be challenging because they sometimes are overlooked in differential diagnoses.

Highlights • Mesenteric lipodystrophy is a rare fibroinflammatory disease of unknown origin. • The detection of mesenteric lipodystrophy is challenging and requires early clinical suspicion. • Clinical and imaging findings can mimick other pathological conditions affecting the mesenteric fat tissue. • Contrast enhanced CT is the most accurate imaging technique for diagnosing mesenteric lipodystrophy., Introduction Mesenteric lipodystrophy is a rare fibroinflammatory disease of unknown origin with clinical and radiological non specific findings. Presentation of the case The case of a 42-years-old man affected by a pedunculated mesenteric lipodystrophy mimicking Meckel’s diverticulum is reported. Clinical, imaging and histological findings are discussed. Discussion Mesenteric lipodystrophy affects the mesenteric fat of the abdomen with a typical diffuse thickening of the mesentery, nodular thickening of the mesenteric root and presence of mass-like lesions. Ultrasound (US) and Multiphasic Computed Tomography (CT) represent the main imaging tools used for diagnosis. Clinical and imaging findings can mimick other pathological conditions affecting the mesenteric fat tissue. Contrast enhanced CT is the most accurate imaging technique for diagnosing mesenteric lipodystrophy due to the high panoramicity and accuracy with multiplanar imaging. Multiphasic technique helps to characterize the lesion and to recognize vascular anatomy. Oral administration of iodinated contrast medium may help to assess the relationship with bowel loops. All these diagnostic elements are crucial for the surgical timing and approach. Conclusions Due to the heterogeneous mesenteric involvement, the nonspecific CT findings and the high number of diseases for differential diagnosis, the detection of mesenteric lipodystrophy is challenging and requires early clinical suspicion. An histological examination is always necessary.

A 72-year-old man was followed as an outpatient at our hospital for 6 years after surgery for small cell carcinoma of left adrenal gland origin. Follow-up abdominal computed tomography showed a 6-cm mass in the left lower mesentery. The patient underwent open laparotomy. The histological diagnosis was sclerosing mesenteritis. The previous specimens of the left adrenal mass were then re-examined with a microscope, and panniculitis was found around the small cell carcinoma. Both lesions were histologically similar to IgG4-related disease (RD), but they did not completely meet the diagnostic criteria of IgG4-RD clinically or histologically.

Patient: Female, 83-year-old Final Diagnosis: Sclerosing mesenteritis Symptoms: Abdominal pain • intestinal obstruction • vomiting Medication: — Clinical Procedure: — Specialty: Surgery Objective: Rare disease Background: The term “sclerosing mesenteritis” includes a spectrum of rare idiopathic diseases involving the small and/or large bowel. It appears as a diffuse, localized, or multinodular thickening of the mesentery, with a variable degree of chronic non-specific inflammation, fat necrosis, and fibrosis. Case Report: Here, we report a case of 83-year-old woman with symptoms of intestinal occlusion, vomiting, and abdominal pain. Radiographic examinations showed air fluid levels in right and left quadrants and in the mesogastric site, while computed tomography (CT) documented a strangulated inguinal hernia with ileal obstruction. Based on clinical examination and radiologic findings, the patient underwent surgery for inguinal hernia reduction. The examination of viscera revealed 2 tracts of ileum with ischemic signs and covered by fibrin; thus, the 2 intestinal loops were resected. Histological examination revealed chronic non-specific inflammation of the whole intestinal wall, including the subserosa in the resected tract of proximal ileum, while the distal ileal loop (not herniated tract) showed a subserosal fibrous nodule of 2 cm in greatest diameter, composed of a proliferation of spindle cells haphazardly arranged in a collagenized stroma. The diagnosis of sclerosing mesenteritis was rendered. Conclusions: The present case shows the possibility of an incidental diagnosis during another intervention such as hernia surgery. Pathologists should be aware of this disease to avoid confusion with aggressive tumors such as intra-abdominal desmoid-type fibromatosis and gastrointestinal stromal tumor.

Sclerosing mesenteritis (SM), a benign chronic fibrosing inflammatory disease of the mesentery, is a rare disease discovered in 1924. The prevalence of the disease is less than 1%. The exact etiology of the disease is not clear. It is thought that the integrity of the gastrointestinal lumen may be altered from chronic inflammatory effects. SM may be associated with autoimmune diseases, trauma, malignancy, or surgery. The most common clinical presentation is abdominal pain. Obstructive symptoms may occur. Diagnosis is made by CT abdomen and biopsy. Treatment includes surgical and immunosuppressive medications.

Sclerosing mesenteritis (SM) is a rare fibroinflammatory disorder that involves mesenteric adipose tissue, more frequently localized in the small intestine, with an insidious clinical presentation having symptoms related to mass effect, usually resulting in bowel obstruction, mesenteric ischemia, as well as rapid weight loss. We report a case of a 23-year-old male presenting with palpable abdominal mass, mesogastric pain, and a history of rapid weight loss, who underwent exploratory laparoscopy. A hemorrhagic and gelatinous nodular tumor mass of the mesentery was identified and the surgical procedure was converted to a laparotomic approach. Histologically, the mass was composed of a proliferation of bland-looking spindle cells with slightly eosinophilic cytoplasm and elongated normochromatic nuclei with mild nuclear atypia, haphazardly set in a collagenized stroma; fat necrosis and inflammatory cells (lymphocytes, plasma-cells, and histiocytes) were also evident. The diagnosis of sclerosing mesenteritis was made. Our case emphasizes that histology remains pre-eminent for a correct diagnosis of SM, as pre-operative radiological-based diagnosis is non-specific.

Patient: Male, 42-year-old Final Diagnosis: Neuroendocrine tumor Symptoms: Abdominal pain Medication:— Clinical Procedure: — Specialty: Surgery Objective: Unusual clinical course Background: Jejunoileal neuroendocrine tumors (JI-NETs) are rare tumors that can be associated with mesenteric fibrosis. This case report is of an incidental finding of a JI-NET in a patient who was previously misdiagnosed with sclerosing mesenteritis. Case Report: A 42-year-old man was admitted to our institution with diffuse abdominal pain and clinical and radiographic signs of bowel obstruction. He had a previous diagnosis of sclerosing mesenteritis, which had been histologically diagnosed after an exploratory laparoscopy performed in 2009 for recurrent acute abdominal pain. He was also annually monitored through computed tomography scans for an incidentally discovered, gradually enlarging mesenteric mass for which a “wait and watch” management approach was adopted. After a period of fasting and observation, the patient underwent an urgent exploratory laparotomy because of his worsening condition. Intraoperatively, an ileocecal resection was performed, along with excision of the known mesenteric mass. The pathology report revealed an ileal NET with nodal metastases within the mesentery and mesenteric tumor deposits (pT3N1). Conclusions: JI-NETs are rare entities, which are usually encountered as incidental findings or in patients with unspecific abdominal pain. Our case represents a probable delayed diagnosis of JI-NET in the context of sclerosing mesenteritis; therefore, a possible association between these 2 conditions should be investigated.

Sclerosing mesenteritis (SM) is a chronic nonspecific mesenteric inflammation. I report a case of a 72-year-old male treated with etanercept for psoriatic arthritis for 7 years who developed abdominal discomfort, urinary retention, acute kidney injury, and bilateral ureteric obstruction. CT abdomen revealed retroperitoneal mass. Biopsy showed sclerosing mesenteritis. One year later, after discontinuation of etanercept, CT abdomen showed regression of the mass. To my knowledge, this is first case report of reversible sclerosing mesenteritis associated with etanercept therapy.

Sclerosing mesenteritis is a rare benign disease with a prevalence of 0.16-3.4% and is characterized by chronic nonspecific inflammation and extensive fibrosis in the adipose tissue of the mesentery although the exact pathogenesis is still elusive. A 65-year-old woman was referred with suspicion of an abdominal mass and biliary stones on abdominal ultrasonography and CT. Bile duct stones were confirmed by endoscopic ultrasonography and successfully treated by endoscopic retrograde cholangiography with stone removal. Furthermore, a 4.7 cm conglomerated mass on small intestinal mesentery was suspected as sclerosing mesenteritis based on the features on abdominal MRI. However, because it could not be differentiated from malignancy without histologic examination, laparoscopic excisional biopsy was performed; it showed only inflammatory cells with extensive fibrosis. Therefore, the abdominal mass was confirmed as sclerosing fibrosis and the patient was followed-up without any treatments because no mass-related symptoms accompanied the findings. Six months later, abdominal CT showed no significant change in the mass. Herein, we report a rare case of incidentally found idiopathic sclerosing mesenteritis.

ObjectivePeritoneal or mesenteric tumours may correspond to several tumour types or tumour-like conditions, some of them being represented by histiocytosis. This rare condition often poses diagnostic difficulties that can lead to important time delay in targeted therapies. Our aim was to describe main features of histiocytoses with mesenteric localisation that can improve the diagnostic process.DesignWe performed a retrospective study on 22 patients, whose peritoneal/mesenteric biopsies were infiltrated by histiocytes.ResultsAbdominal pain was the revealing symptom in 10 cases, and 19 patients underwent surgical biopsies. The diagnosis of histiocytosis was proposed by initial pathologists in 41% of patients. The other initial diagnoses were inflammation (n=7), sclerosing mesenteritis (n=4) and liposarcoma (n=1). The CD163/CD68+CD1a- histiocytes infiltrated subserosa and/or deeper adipose tissues in 16 and 14 cases, respectively. A BRAFV600E mutation was detected within the biopsies in 11 cases, and two others were MAP2K1 mutated. The final diagnosis was histiocytosis in 18 patients, 15 of whom had Erdheim-Chester disease. The median diagnostic delay of histiocytosis was 9 months. Patients treated with BRAF or MEK inhibitors showed a partial response or a stable disease. One patient died soon after surgery, and five died by the progression of the disease.ConclusionDiagnosis of masses arising in the mesentery should be carefully explored as one of the possibilities in histiocytosis. This diagnosis is frequently missed on mesenteric biopsies. Molecular biology for detecting the mutations in BRAF or in genes of the MAP kinase pathway is a critical diagnostic tool.

Patient: Male, 57-year-old Final Diagnosis: Sclerosing mesenteritis Symptoms: Abdominal pain Medication: Prednisone Clinical Procedure: CT scan • MRI Specialty: Endocrinology and Metabolic Objective: Rare disease Background: Sclerosing mesenteritis is a rare disease characterized by chronic inflammation of mesenteric adipose tissue. To our knowledge, this is the first case report that presents the effects of glucocorticoid therapy on metabolic control in diabetes mellitus, aggravated by sclerosing mesenteritis. We want to show the significance of this rare disease, which could be underestimated as a cause of decompensation of diabetes mellitus. Case Report: A 57-year-old man with diabetes type 2 was admitted to the hospital to obtain better metabolic control of this disease. In addition, he reported persistent pain in the left side of his abdomen. Sclerosing mesenteritis was diagnosed based on the CT and MRI images. Prednisone was administered. The treatment resulted in better glycemic control and abdominal pain reduction. On follow-up after 1 year, the patient reported a decrease in the abdominal pain and an MRI showed a significant reduction of abnormalities in the mesentery. Conclusions: It is known that glucocorticoids exacerbate hyperglycemia, particularly in patients with diabetes mellitus. However, we noticed contrary effects in the case of our patient. We suggest that the inflammatory process occurring in sclerosing mesenteritis was one of the main causes of metabolic decompensation in our patient. The effect of reduction of inflammation with glucocorticoids was stronger than the hyperglycemic effect of this treatment. That is why, in the presence of this autoimmune disease, the use of glucocorticoids can paradoxically lead to better glycemic control.

Sclerosing mesenteritis is a rare condition characterized by chronic inflammation and fibrotic changes of the mesentery. To determine the long-term management and outcomes of patients with sclerosing mesenteritis. Patients with biopsy-proven sclerosing mesenteritis at the Mayo Clinic between January 2006 and December 2016 were identified. Clinical data were collected retrospectively. One hundred and three patients were identified, median age 68.0 years (range 35.0–85.3). Most patients were symptomatic (87.4%) at presentation. Patients received no treatment (52.4%), medical therapy (42.7%) or surgery (4.9%) on initial diagnosis. The most common initial regimens were prednisone plus tamoxifen (41.9%), prednisone alone (23.3%), and prednisone plus colchicine (11.6%) with 55.6%, 57.2%, and 60% of patients improving, respectively, p = 0.85 for a difference in response rates. At least half of the patients responded to prednisone plus tamoxifen, prednisone plus colchicine, or prednisone alone at 6.0, 7.2, and 8.4 months, respectively. At a median follow-up of 45.6 months (95% CI 24.1–69.7), 65.4% of patients were receiving medical therapy. Of those receiving tamoxifen-based, steroid-based, or steroid-sparing regimens, 100%, 87.5%, and 77.8% had improved by their last follow-up appointment respectively, p = 0.15. Prednisone plus colchicine has a similar efficacy to prednisone plus tamoxifen for the initial and long-term treatment of sclerosing mesenteritis. The majority of patients were initiated on medical therapy over the long term with most reporting symptomatic improvement within a year. Death from SM was rare.

A 64-year-old man presented to our hospital with abdominal pain and 4–5 episodes of watery diarrhea per day for 2 months. Abdominal ultrasound examination revealed a mass in the peritoneal cavity, and computed tomography showed a 13.4 cm mass in the mesentery and a 3 cm mass in the mesocolon. The patient underwent laparoscopic partial resection for diagnosis. Microscopically, abundant fibrosis and numerous immunoglobulin (Ig) G4-positive plasma cells were observed. The serum level of IgG4 was 665 mg/dl postoperatively. These findings suggested that the lesion was consistent with IgG4-related sclerosing mesenteritis. Oral steroids resulted in rapid disappearance of symptoms and a decrease in masses. Recently, sclerosing mesenteritis are reported as IgG4-related disease or mimicking IgG4-related disease but multiple lesions rarely occur in the same organ. We report a case of IgG4-related sclerosing mesenteritis with multiple lesions without involvement of other organs, such as the pancreas and salivary glands.

Sclerosing Mesenteritis (SM) is a rare fibroinflammatory disease of unknown etiology in which intestinal obstruction results from encasement of variable lengths of bowel by a dense fibrocollagenous membrane that gives the appearance of a cocoon. To our knowledge no cases of SM have been reported in patients with compensated cirrhosis without ascites. We report a case with SM diagnosed at laparotomy due to rapidly worsening small bowel obstruction, in a patient with compensated alcoholic cirrhosis. A 50-yearsold Caucasian male was evaluated because of cramping abdominal pain and discomfort associated with diarrhea, occasional post-prandial vomiting, fatigue and a significant weight loss. Compensated alcoholic cirrhosis had been diagnosed, two months before current presentation. Physical examination revealed abdominal distention with highpitched metallic sounds and bilateral peripheral pedal edema. Computed tomography of the abdomen demonstrated severe dilated small bowel loops as well as fluid into the peritoneal cavity. Based on the imaging findings, a surgical procedure was carried out. During laparotomy, an extra fibrous layer fully surrounding the small intestine was divided and a side-to-side functional ileo-cecum anastomosis was performed. Histopathological examination revealed fibroconnective tissue proliferation and inflammatory infiltration. Combined with the characteristic surgical findings a diagnosis of SM was made in a patient with compensated alcoholic cirrhosis. The patient recovered uneventfully and at 6-month follow-up he had no symptoms or signs of SM recurrence. The insidious onset of bowel obstruction symptoms in patients with even compensated cirrhosis, should include SM in the differential diagnosis.

Sclerosing mesenteritis (SM) is a rare inflammatory condition with unknown etiology that affects the mesenteric adipose tissue. We present a case of a 49-year-old male with severe abdominal pain who underwent abdominal biopsy confirming the presence of adipose inflammation and necrosis. The diagnosis of SM was made, and the patient was treated with prednisone and tamoxifen. As this condition is rare, there are no standard guidelines for management. This case aims to outline a possible treatment plan.

Sclerosing mesenteritis is a rare and often benign condition characterized as a fibrotic disease consisting of non-suppurative inflammation of adipose tissue. Through mass effect, sclerosing mesenteritis can compromise the gastrointestinal lumen as well as mesenteric vessel integrity. There is a poor understanding of this disorder and its pathogenesis, which presents with various symptomatology and often without identification of inciting factors. Patients with sclerosing mesenteritis exhibit gastrointestinal and systemic manifestations including weight loss, fever, nausea, vomiting, diarrhea, and abdominal pain. This case presents a patient with a seven-month history of chronic, epigastric abdominal pain following laparoscopic surgery for acute uncomplicated appendicitis. The patient underwent work-up with computed tomography and magnetic resonance enterography that confirmed the presence of a mesenteric mass of unknown etiology located in the mid-epigastrium. Due to the inability to safely sample the mass, the patient underwent diagnostic laparoscopy, which was subsequently converted to an open procedure where excision of the mesenteric lesion was performed. Surgical pathology revealed fat necrosis with fibrosis, granulomatous inflammation, and dystrophic calcifications consistent with sclerosing mesenteritis. The patient was seen in follow-up with the resolution of her epigastric abdominal pain. This case report demonstrates a unique presentation of a symptomatic patient with a mesenteric mass not amenable to non-invasive biopsy. Complete excision of this lesser sac mass revealed sclerosis mesenteritis as the pathological cause.

IgG4 is increasingly recognized as a pathophysiologic component of a variety of diseases. These include type 1 autoimmune pancreatitis, IgG4-related sclerosing cholangitis, retroperitoneal fibrosis, chronic sclerosing aortitis and periaortitis. Some cases of sclerosing mesenteritis, a fibrosing form of mesenteric panniculitis has been classified as IgG4-related. IgG4-related disease can affect a large number of organ systems. In most cases, more than one organ system is involved. Clinical manifestations of these conditions are dependent on the organ systems that are involved and the duration of illness. These can range from the subacute development of painless organ enlargement to severe conditions such as obstructive renal failure in patients with retroperitoneal fibrosis. Treatment consists of high doses of glucocorticoids, followed by prolonged tapering. A variety of immunosuppressive agents, (including rituximab) either alone or in combination are treatment options in patients who fail or relapse on glucocorticoid therapy. Maintenance immunosuppressive therapy is considered in patients with severe organ damage or relapsing disease.

Patient: Male, 33-year-old Final Diagnosis: Sclerosing mesenteritis Symptoms: Abdominal pain • diaphoresis • nausea • tachycardia Medication:— Clinical Procedure: Exploratory laparotomy Specialty: Surgery Objective: Rare co-existance of disease or pathology Background: Sclerosing mesenteritis is an inflammatory and fibrotic disease that affects the mesentery of the small intestine. This condition is non-neoplastic, although it is frequently associated with underlying malignancies. The overall etiology is unclear because of the limited number of cases available for review, yet a number of possible mechanisms have been described, including ischemia. Factor V (FV) Leiden is a hereditary condition causing hyper-coagulability, thrombosis, and ischemia. Because ischemia is one of the proposed mechanisms for the fibrosis and sclerotic findings of sclerosing mesenteritis, this case explores a possible association between FV Leiden and sclerosing mesenteritis. Case Report: Herein, we describe a case of sclerosing mesenteritis in a patient heterozygous for FV Leiden, with a strong personal and family history of venous thromboembolism. This patient presented with acute worsening of chronic abdominal pain and was found to have a small bowel obstruction requiring acute surgical intervention. Imaging findings and pathologic examination of the ileum and mesentery conclusively diagnosed sclerosing mesenteritis. Conclusions: This case serves to highlight a possible association between mesenteric ischemia secondary to chronic thrombotic activity and sclerosing mesenteritis. This patient’s virgin abdomen and lack of additional risk factors for sclerosing mesenteritis make this case a unique presentation of the disorder. This case serves to update the literature at large, as only one prior case in a FV Leiden patient has been described, in which the patient had the additional risk factor of previous abdominal surgery.

Introduction: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a rare autoimmune disorder involving 1 or multiple organs, most commonly the pancreas, lacrimal glands, and salivary glands. However, IgG4-related sclerosing mesenteritis (SM) involving the small-bowel mesentery is rare. Given that IgG4-related SM usually mimics the imaging characteristics of mesenteric malignancies, its preoperative diagnosis remains challenging. In addition, no specific consensus has been reached regarding the treatment of IgG4-related SM. Therefore, a better understanding of the characteristics, treatment, and prognosis of IgG-related SM is urgently needed. Herein, we report a rare case of IgG-related SM. Patient concerns: A 67-year-old man was admitted to our hospital after incidental detection of an abdominal mass on ultrasound imaging, although he reported being generally well. The findings on triple-phase abdominal computed tomography were highly consistent with a malignant mesenteric tumor. Diagnoses: The hallmark histopathological features along with elevated levels of IgG4 (145 mg/dL) and imaging findings were indicative of IgG-related SM. Interventions: The patient was treated surgically. Postoperative histopathological examinations exhibited tissue infiltration with lymphocytes and IgG4-positive plasma cells, as well as fibrosis. Outcomes: Ten days after surgery, the patient was discharged from the hospital, and did not show any clinical sign of IgG-related SM within 1-year follow-up. Conclusion: This case highlights the mesentery as an uncommon site of involvement as well as how early IgG-related SM can be completely asymptomatic. Thus, this study has advanced our knowledge of IgG-related SM and may improve treatments for similar conditions.

IgG4-related disease (IgG4-RD) is a systemic, autoimmune, fibroinflammatory disease that can cause multi-organ damage. Although there have been many trials and studies since its recognition in 2003, there is still much that is unknown. Furthermore, IgG4-RD can affect any organ in the body and often has many mimics and alternative diagnoses, which can make for a challenging workup. Imaging plays a substantial role in the diagnosis of IgG4-RD and is often the first occasion where IgG4-RD comes into consideration. Thus, knowledge about the imaging findings of various manifestations of IgG4-RD can aid in the diagnosis and have a significant impact on patient management. In this article, we review the wide array of imaging findings, both typical and atypical, as well as possible mimics of IgG4-RD in the abdomen and pelvis.

Mesenteric tumour deposits frequently occur in small-intestine neuroendocrine tumours. In many instances, these mesenteric tumour deposits are surrounded by a dense fibrotic stroma and have associated lymphoplasmacytic inflammation. The aim of this study was to examine whether mesenteric tumour deposits in patients with small-intestine NETs neuroendocrine tumours show histological and immunophenotypic overlap with IgG4-related sclerosing mesenteritis.Sixty-six mesenteric tumour deposits from 66 patients with small-intestine neuroendocrine tumours with blocks available for further studies were identified from our archives. Cases were assessed for clinicopathological features and the presence of IgG4-positive and IgG-positive plasma cells by immunohistochemistry. Ratios of IgG4-positive to IgG-positive plasma cells were calculated. Seventeen mesenteric tumour deposits (26%) showed40 IgG4-positive plasma cells per high-power field, and the majority of cases (68%) showed at least some staining of IgG4-positive plasma cells. Mesenteric tumour deposits with20 IgG4-positive plasma cells per high-power field tended to be larger (25.9 ± 13.0 mm versus 18.6 ± 15.8 mm; P = 0.07), and had more IgG-positive plasma cells (88 ± 24 versus 36 ± 37; P 0.01) and a higher IgG4-positive/IgG-positive plasma cell ratio (0.66 ± 0.18 versus 0.17 ± 0.25; P 0.01). All but one mesenteric tumour deposit with20 IgG4-positve plasma cells had a ratio of40%.IgG4 expression is frequent in mesenteric tumour deposits from small-intestine neuroendocrine tumours. Undersampling of tumour on biopsies of mesenteric tumour deposits could potentially cause diagnostic confusion with IgG4-related sclerosing mesenteritis.

Intra-abdominal panniculitis is a rare condition characterized by intraperitoneal lipodystrophy that may manifest as a necrotic adipose lump causing symptoms due to inflammation or mass effect. Although this condition tends to primarily affect the root of the mesentery, it may affect any part of the peritoneum, including the omentum. However, isolated omental panniculitis, in which no other site is affected, is a very rare form of intra-abdominal panniculitis and only eight cases have been reported in the literature so far. Mesenteric panniculitis, especially in the late fibrous stage of retractile mesenteritis, is known to occasionally cause intestinal obstruction by virtue of its relation to the bowel. However, omental panniculitis has only been reported to cause nonspecific inflammatory symptoms. We present a case of isolated omental panniculitis of indeterminate etiology. To the best of our knowledge, this is the first such case reported to present with intestinal obstruction.

Objective: Mesenteric panniculitis (MP) and sclerosing mesenteritis is an umbrella term used to describe a rare, chronic, and frequently benign fibrosing inflammatory disease that affects the adipose tissue of the mesentery. The diagnosis is usually based on imaging (computed tomography [CT] or magnetic resonance imaging) findings and is confirmed by biopsy. We report the unusual case of a patient with MP with typical CT findings and diabetes mellitus (DM).Methods: A 48-year-old male presented for symptomatic new onset DM. An abdominal CT scan revealed a well-defined mesenteric mass compatible with MP. The combination of insulin degludec and sitagliptin/metformin resulted in a decrease of HbA1c; however, this was followed by a subsequent gradual increase in HbA1c and positive glutamic acid decarboxylase auto-antibodies.Results: The patient developed auto-immune DM that was confirmed by the presence of auto-antibodies.Conclusion: Panniculitis has been infrequently reported in patients with type 2 DM. This is the first report of a patient with MP correlated with auto-immune DM. The possible pathophysiologic mechanisms are discussed.Abbreviations: CT computed tomography DM diabetes mellitus HbA1c hemoglobin A1c MP mesenteric panniculitis SM sclerosing mesenteritis

Background Sclerosing mesenteritis (SM) and mesenteric panniculitis are rare processes of the mesentery which pose a major clinical and imaging challenge. This review article attempts to introduce a systematic nomenclature to address typical symptoms, imaging and major differential diagnoses. Results SM with its subtypes-mesenteric lipodystrophy, panniculitis and retractile mesenteritis-is a chronic process with a very diverse and heterogeneous clinical appearance. The typical radiological characteristics ("misty mesentery", increased lymph nodes, pseudocapsule and "fat halo") are also not very specific. A link between SM and malignant diseases is not proven, but there is controversial discussion. Therefore, if there are doubts about the diagnosis, further examinations and a biopsy are desired. Conclusion When diagnosing SM, "red flags", i. e. characteristics that are atypical for SM in imaging, should be considered. In case of ambiguous imaging, differential diagnostics should also consider malignant diseases and exclude them.

Introduction/Objective Sclerosing Mesenteritis is an uncommon, idiopathic, localized inflammatory syndrome involving the small intestine and colonic mesentery. It is considered a benign condition that commonly occurs in elderly with a gender predilection for males, and its etiology remains unknown. Small Bowel Obstruction (SBO) is a rare, unexpected, but detrimental complication of progressive Sclerosing Mesenteritis. Herein, we present a case of an enlarging, progressive Sclerosing Mesenteritis with extensive involvement of the small bowel and mesentery requiring two consecutive major surgical interventions. Methods/Case Report A 72-year-old male with Myelodysplastic Syndrome (MDS) and recent history of loop ileostomy due to distal intestinal obstruction secondary to enlarging Sclerosing Mesenteritis, presented to our institution with non-specific symptoms of worsening abdominal pain and multiple episodes of gastrointestinal bleeding. Radiographic investigation revealed SBO and he subsequently underwent exploratory laparotomy resulting in total enterectomy with excision of mesenteric mass, extended right colectomy, Whipple procedure, and gastrostomy. The specimens were sent to pathology for histopathological evaluation and gross examination revealed several bosselated, tan-white, firm and rubbery, fibrotic lesions with associated lobulated fibroadipose tissue. Microscopic examination showed extensive mesenteric fibrosis with dense bundles of collagen fibers, areas of fat necrosis, mucosal ischemia and calcification involving the small bowel and serosal surface of large intestine and peritoneum. The lesional cells showed minimal atypia, mitoses, and lacked the Beta-catenin nuclear staining seen in mesenteric fibromatosis. Given the clinical history and histopathological findings of the lesion, we favored the diagnosis of Sclerosing Mesenteritis. Results (if a Case Study enter NA) N/A Conclusion The etiology of Sclerosing Mesenteritis is not well-understood and there are cases of Sclerosing Mesenteritis reported in the literature in association with trauma, surgery, malignancy, and IgG4-related disease. Our patient’s post-operative history was complicated by short gut syndrome, and he is currently requiring small bowel transplant. We report this case for its unusual and aggressive clinical presentation, and to heighten clinical awareness for detrimental consequences of this seemingly benign condition.

Here we report a case of sclerosing mesenteritis that we diagnosed with needle biopsy under the guidance of computed tomography (CT) and ultrasound (US) observation. An 82-year-old woman presented with appetite loss, weight loss and epigastric pain. CT of the abdomen and pelvis revealed increased density of the mesentery adjacent to the small bowel and enlarged lymph nodes. Sclerosing mesenteritis was suspected, but malignancies, such as lymphoma, were also considered. We performed CT and US-guided needle biopsy with the coaxial technique. An introducer needle was inserted, its correct location was documented with CT, and multiple specimens were taken with a finer needle passed through the introducer without incident. Adequate specimens were obtained, and the histological diagnosis of sclerosing mesenteritis was made. We treated the patient with corticosteroids and her symptoms and the radiographic findings improved. The coaxial technique was a useful and minimally invasive tool for making the diagnosis of sclerosing mesenteritis.

Sclerosing mesenteritis (SM) is a rare disease with non-specific clinical manifestations and should be supported by radiological examination and confirmed by histopathological evaluation. Its relationship with cancer especially caecal adenocarcinoma is still unclear. This case report describes a young man who was diagnosed as having SM and poorly-differentiated caecal adenocarcinoma.

A 75-year-old man presented with abdominal distension, hypoproteinemia, ascites and a 35-mm mass in the small bowel mesentery. Laparotomy was performed, and he was diagnosed with sclerosing mesenteritis. His clinical condition improved, with computed tomography (CT) showing tumor shrinkage and decreasing ascites after administration of prednisolone; however, on drug withdrawal, abdominal fullness recurred and CT revealed an enlarging tumor and increasing ascites. Capsule endoscopy (CE) and double-balloon enteroscopy (DBE) were performed to further investigate hypoalbuminemia, which revealed white villi, white nodules, white debris, and mucosal edema in the jejunum. Biopsies from the jejunal mucosa demonstrated infiltration by chronic inflammatory cells consisting mostly of lymphocytes and plasma cells, with marked lymphangiectasia of the lamina propria and submucosa. A fecal alpha-1-antitrypsin clearance test revealed abnormal leakage from the gastrointestinal tract, confirming that hypoalbuminemia was secondary to protein-losing enteropathy (PLE). The incidence of sclerosing mesenteritis accompanied by PLE is very rare. Only six cases have been reported so far. CE and DBE were helpful for diagnosing this condition, and should be performed in patients in whom the cause of hypoalbuminemia is unknown, and in those with PLE.

Patient: Male, 80 Final Diagnosis: Sclerosing mesenteritis Symptoms: Abdominal distension • abdominal tenderness Medication: — Clinical Procedure: Paracentesis Specialty: Gastroenterology and Hepatology Objective: Rare disease Background: Sclerosing mesenteritis (SM) is a rare idiopathic inflammation and fibrosis of the mesentery. Its etiology and pathogenesis remain unclear. It has been linked to abdominal trauma related to peritoneal dialysis, recent abdominal surgery, idiopathic inflammatory disorder, paraneoplastic syndrome, ischemia/infection, and autoimmunity. Abdominal CT is the most sensitive imaging modality, and diagnosis is usually confirmed by surgical biopsy. Patients most often present with abdominal pain, nausea, vomiting, diarrhea, and weight loss, and less commonly with chylous ascites and small bowel obstruction. Treatment is usually supportive; surgical intervention may be attempted for life-threatening complications such as bowel obstruction or perforation. Case Report: This report describes an 80-year-old man with hypertension and end-stage renal disease (ESRD) presenting with increasing abdominal pain and tenderness over the past 5 months. Abdominal enhanced computed tomography (CT) revealed a fat-ring sign and peritoneal calcifications along the serosa surface of small bowel consistent with sclerosing mesenteritis. His hospital course was complicated by increasing ascites requiring multiple ultrasound-guided paracentesis, worsening leukocytosis, and persistent hypotension after dialysis, requiring pressor support. Ascitic fluid analysis was consistent with chylous ascites. The patient subsequently developed small bowel obstruction causing focal perforation, leading to the death of our patient. In this report, we review the clinical presentation, radiographic findings, treatment, and outcome in our patient and review the relevant literature. Conclusions: Diagnosis of sclerosing mesenteritis is challenging due to its nonspecific clinical features. Sclerosing mesenteritis is a debilitating albeit self-limiting disorder that can rarely become fulminant, largely due to its complications.

Background Sclerosing mesenteritis (SM) is sometimes used as an umbrella-term for idiopathic inflammatory conditions in the mesentery. Mesenteric panniculitis (MP) is a radiological finding and its relation to clinical SM is not fully understood. The aims of this study were to determine whether any correlation could be found between the radiological findings and the clinical disease course. Methods Patients observed due to idiopathic inflammation of the mesentery were identified. If SM could be verified histologically or MP radiologically, the patients were included in this descriptive retro perspective study. Results Typical radiological changes were observed in 27 patients. A majority (23/27) of these patients had mild to moderate symptoms. This group with typical radiology was labelled MP. Four patients were included due to histologically verified disease but had uncharacteristic radiology involving multiple compartments of the abdomen. All four had marked systemic inflammation, fever and fluctuating radiologic findings. Three had severe disease with multiple hospitalisations and complications but responded promptly to corticosteroids. This group was denoted SM. Conclusions We have identified two subgroups of patients; firstly, MP with stable and characteristic radiologic changes and secondly SM with atypical radiology and a more aggressive clinical course. We propose that the term SM should be reserved for this latter condition.

Sclerosing mesenteritis includes a spectrum of inflammatory disorders involving the adipose tissue of the bowel mesentery. To perform a systematic review of previously reported cases of sclerosing mesenteritis (SM) to determine the epidemiology, risk factors, methods of diagnosis, treatment patterns and outcomes for this disease. Medline, PubMed, Google Scholar and Cochrane database were searched using keywords mesenteric panniculitis, retractile mesenteritis, mesenteric lipodystrophy and sclerosing mesenteritis. Data was collated into a single excel database, transferred into SPSS (Version 21.0) and analyzed. Patients diagnosed with SM were between ages of 3 and 88 with a mean age of 55 ± 19.2 years. SM was more common in Caucasians (n = 28, 60.8% of those reporting ethnicity) and men (n = 133, 69.3%) with a male to female ratio of 2.3:1. 28.6% (n = 55) of patients reported a prior abdominal surgery or abdominal trauma, 8.9% (n = 17) had a history of malignancy, and 5.7% (n = 11) of autoimmune disease. 85.4% (n = 164) underwent surgical abdominal exploration (open or laparoscopic); 41.7% (n = 80) had surgery with resection of the involved bowel and mesentery. 34.9% (n = 67) of patients received medical treatment with the majority of them receiving steroids (n = 56, 83.5%). Symptom duration of more than a month (66.7% vs 40.4%, p

Sclerosing mesenteritis (SM) is an extremely rare disease characterized by chronic non-specific inflammation, fat necrosis and fibrosis of the mesentery. We presented a 77-year-old man with progressive dyschezia, abdominal pain and mass in left lower quadrant. Computed tomography (CT) exhibited a thickened mesentery, enlarged lymph nodes and strand-like densities around the mesenteric vessels. However, laboratory investigation, colonoscopy and positron emission tomography did not provide any specific results for diagnosis. Because of the exacerbating abdominal pain, partial colectomy was performed and SM was diagnosed based on the pathological changes of mesentery including fat necrosis, multifocal lipid-filled macrophages, lymphocytes and multifocal fibrosis. Although SM is difficult to diagnose and often found by incident, progressive deterioration of abdominal symptoms and general status alteration are indicators of SM. Some typical imaging and pathologic manifestations are also helpful to SM diagnosis. There is no standard treatment for SM. Operation is preferred in those at the stage of fibrosis and particularly combined with intestinal obstruction. Therefore, a multidisciplinary collaboration is essential to diagnose and manage this rare disease, with combined approaches in gastroenterology, colorectal surgery, pathology and radiology. Gastroenterol Res. 2017;10(1):50-55 doi: https://doi.org/10.14740/gr732w