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BMJ Open Gastroenterology May 2021Peritoneal or mesenteric tumours may correspond to several tumour types or tumour-like conditions, some of them being represented by histiocytosis. This rare condition...
OBJECTIVE
Peritoneal 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.
DESIGN
We performed a retrospective study on 22 patients, whose peritoneal/mesenteric biopsies were infiltrated by histiocytes.
RESULTS
Abdominal 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 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.
CONCLUSION
Diagnosis 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.
Topics: Delayed Diagnosis; Histiocytosis; Humans; Neoplasms; Proto-Oncogene Proteins B-raf; Retrospective Studies
PubMed: 34020934
DOI: 10.1136/bmjgast-2021-000622 -
World Journal of Clinical Cases Oct 2014Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or... (Review)
Review
Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.
PubMed: 25325063
DOI: 10.12998/wjcc.v2.i10.528 -
International Journal of Hyperthermia :... 2023This study assessed the improvement of symptoms and pregnancy outcomes in infertile patients with various types of adenomyosis who were treated with high-intensity... (Review)
Review
OBJECTIVE
This study assessed the improvement of symptoms and pregnancy outcomes in infertile patients with various types of adenomyosis who were treated with high-intensity focused ultrasound (HIFU).
MATERIALS AND METHODS
Between October 2017 and January 2022, 129 infertile patients with adenomyosis who wished to conceive were treated with HIFU. Based on the relationship between the adenomyotic lesion, the endometrium, and the subserosa of the uterus on magnetic resonance imaging, the adenomyotic lesions were divided into internal, external, intramural, and full-thickness types. Menstruation pain score, menstruation blood volume score, anti-Müllerian hormone (AMH) levels, reproductive results, pregnancy and delivery complications, and other clinical variables were compared among these four groups.
RESULTS
Patients with external adenomyosis had the greatest menstrual distress, whereas patients with internal adenomyosis had the greatest menstrual blood volume. Dysmenorrhea and heavy menstruation were significantly improved after HIFU treatment in all groups. AMH levels were not significantly different before and six months after HIFU. Of the 129 patients, 50 (38.7%) became pregnant after HIFU, and patients with internal adenomyosis had the highest pregnancy rate. Patients with adenomyotic lesions located in the posterior wall of the uterus had a higher pregnancy rate than those with lesions located in the fundus of the uterus.
CONCLUSIONS
The classification of adenomyosis is closely related to distinctions in clinical symptoms and pregnancy outcomes. Infertile patients with different types of adenomyosis could be effectively treated with HIFU. HIFU can be considered as an option for infertile patients with adenomyosis who want to maintain their fertility.
Topics: Pregnancy; Female; Humans; Adenomyosis; Pregnancy Outcome; Treatment Outcome; High-Intensity Focused Ultrasound Ablation; Infertility; Dysmenorrhea
PubMed: 37495217
DOI: 10.1080/02656736.2023.2238140 -
Zhonghua Wei Chang Wai Ke Za Zhi =... Nov 2020Pneumatosis cystoides intestinalis (PCI) is a rare disease, which is characterized by the accumulation of gas cysts located in the submucosa or subserosa of the... (Review)
Review
Pneumatosis cystoides intestinalis (PCI) is a rare disease, which is characterized by the accumulation of gas cysts located in the submucosa or subserosa of the gastrointestinal tract. It can occur in the whole or part of the gastrointestinal tract from the esophagus to the rectum, but clinically the main involved sites are the colon and small intestine. PCI can also appear in other sites such as mesentery, the greater omentum and the hepatogastric ligament. In recent years, with the renewal of imaging method, the detection rate of PCI has been on the rise. Most patients with PCI have no obvious symptoms or only non-specific symptoms of the digestive tract like abdominal distension, abdominal pain, diarrhea, hematochezia, etc. The atypical clinical symptoms of PCI can easily lead to missed diagnosis or misdiagnosis. A small amount of patients would have complications like peritonitis and even perforation of the digestive tract. The therapeutic principle for these patients is different from that for patients with acute abdomen. The prognosis of PCI depends on its severity and comorbidities. In this article, a literature review would be conducted on the epidemiological characteristics, etiology and pathogenesis, clinical manifestations, diagnosis and treatment of PCI, which might help clinical doctors with diagnosis and treatment of the disease.
Topics: Humans; Pneumatosis Cystoides Intestinalis
PubMed: 33212565
DOI: 10.3760/cma.j.cn.441530-20191010-00446 -
Romanian Journal of Morphology and... 2020Abdominal elastotic deposits are uncommon lesions that often presents as polyps. They show three histological patterns: fibroelastosis, angioelastosis, and... (Review)
Review
Abdominal elastotic deposits are uncommon lesions that often presents as polyps. They show three histological patterns: fibroelastosis, angioelastosis, and elastofibroma. We describe 23 cases including rare locations, such as mesentery, greater omentum, hernia sac, spleen, peripancreatic fat, and hypodermal fat. The age of the patients ranged from 49 to 93 years (mean, 76.8 years). Most lesions were discovered incidentally in the microscopic study. The most frequent locations were peritoneal subserosa (43.5%) and mesentery∕mesocolon∕greater omentum (39.1%). The most common pattern was fibroelastosis (69.6%) followed by angioelastosis (26.1%). We observed one case of omental elastofibroma. A review of the 14 abdominal elastofibromas described including our case revealed that the age of the patients ranged from 45 to 88 years (mean, 68.5 years). Female predominance is striking (M:F, 1:12). The most common site was the stomach (50%). The greater omentum (14.3%), small intestine (7.1%), and pancreas (7.1%) are very rare sites for this lesion. Only one case before ours has been published in the greater omentum. The size of the lesions ranged from 0.7 cm to 8 cm (mean 3.2 cm). In 36.4% of the cases located in the digestive tract, the mucosa did not show alterations. Ulcerations (36.4%) or polypoid excrescences (18.2%) were mostly observed. Six (42.9%) cases were asymptomatic and six (42.9%) cases simulated a neoplasm. Two cases were associated with elastofibromas in other locations. Differential diagnosis includes amyloidoma, elastofibrolipoma, mesenteric elastic vascular sclerosis in neuroendocrine tumors, diverticular disease elastosis, pseudoxanthoma elasticum, pulse granuloma, and digestive lesions in patients treated with D-Penicillamine.
Topics: Aged; Aged, 80 and over; Female; Gastrointestinal Tract; Humans; Intestine, Small; Mesentery; Middle Aged; Omentum; Soft Tissue Neoplasms
PubMed: 33817725
DOI: 10.47162/RJME.61.3.22 -
Journal of Medical Ultrasonics (2001) Apr 2021The major role of conventional ultrasonography (US) can be divided into three processes: cancer screening, differential diagnosis, and assessment of the depth of tumor... (Review)
Review
The major role of conventional ultrasonography (US) can be divided into three processes: cancer screening, differential diagnosis, and assessment of the depth of tumor invasion. As US is a simple and minimally invasive modality, it is widely used for cancer screening and health checkups. Both gallbladder (GB) polyps and thickened wall of the GB are common US findings. On the contrary, US is prone to interference from gas echoes, and its diagnostic accuracy depends on both the US technology and the ability of sonographers. It is also important to be well acquainted with characteristic artifacts and how to manage their influence. Furthermore, magnified images acquired using high-resolution US (HRUS) are strongly helpful to pick up small lesions. As for differential diagnosis, classification of GB polypoid lesions (GPLs) into pedunculated or sessile (broad-based) types is very important. Cholesterol polyps in pedunculated lesions and localized adenomyomatosis (ADM) in sessile lesions are the most important targets to be differentiated. Furthermore, significant findings including number, size, growth rate, shape, internal echo, surface contour, and internal structure should be evaluated and judged as a whole. Usually, US delineates the GB wall as a two- or three-layer structure. However, as the inner hypoechoic layer includes not only the mucosa and muscularis propria but also the fibrous layer of subserosa, the differentiation between T1 (confined to the mucosa or muscularis propria) and T2 (invading the subserosa) based on the layer structure is difficult. Shape, size, and internal echo structure may be helpful for further assessment.
Topics: Adult; Aged; Diagnosis, Differential; Female; Gallbladder; Gallbladder Neoplasms; Humans; Male; Middle Aged; Polyps; Ultrasonography
PubMed: 31828545
DOI: 10.1007/s10396-019-00989-5 -
Parasitology Research Jan 2021Despite significant advances in understanding the role of the immune response in Opisthorchis viverrini-associated carcinogenesis, little is known about how infection...
Despite significant advances in understanding the role of the immune response in Opisthorchis viverrini-associated carcinogenesis, little is known about how infection induces gall bladder disease. This study investigated whether mast cells are activated in cholecystitis associated with O. viverrini, gall bladder specimens from ninety-two patients who had undergone cholecystectomy at the Khon Kaen Regional Hospital, Khon Kaen, Thailand. Two representative sections from the body of fresh gall bladder tissue were fixed in Carnoy's solution and embedded in paraffin wax. The paraffin sections were stained for mast cells and IgE plasma cells by the double histochemical and immunohistochemical method. The cells in the epithelium, lamina propria, muscular layer, and subserosa were counted and expressed as cells per square millimeter. The gall bladder bile was examined for the presence of O. viverrini eggs. Significantly higher mean mast cell numbers were found in the lamina propria (221.41 ± 16.01 vs 116.97 ± 14.61 cells per mm; P < 0.005) of egg positive compared to egg negative groups, respectively. No comparable differences in mast cell number were observed in other layers. IgE plasma cells were rarely seen. The results suggest that mast cell hyperplasia occurs during cholecystitis in association with opisthorchiasis and may play a role in the pathogenesis of the disease.
Topics: Adult; Animals; Bile; Cholecystitis; Feces; Female; Humans; Hyperplasia; Immunoglobulin E; Male; Mast Cells; Middle Aged; Mucous Membrane; Opisthorchiasis; Opisthorchis; Thailand
PubMed: 33155102
DOI: 10.1007/s00436-020-06937-4 -
Esophagus : Official Journal of the... Oct 2023Classification of extramural invasion of esophagogastric junction carcinoma (EGJC) is not yet established. The anatomy surrounding the EGJ alters between the mediastinum... (Review)
Review
Classification of extramural invasion of esophagogastric junction carcinoma (EGJC) is not yet established. The anatomy surrounding the EGJ alters between the mediastinum and the abdominal cavity. This review proposed a T3 classification of EGJC based on anatomical continuity. Analysis of endoscopic ultrasound images, review of intraoperative images, and detailed observation of surgical specimens were followed by a review of the literature. In the EGJ, the muscularis propria of the esophagus is enclosed in mediastinal adipose tissue called the adventitia, which is surrounded by the diaphragmatic crus and contains the paraesophageal lymph nodes (LNs). After passing through the esophageal hiatus along with the vagus nerves and blood vessels, the adventitia joins the adipose tissue containing the paracardial LNs, which is covered by the peritoneum, and then further divides into the lesser and greater omentum. The connective tissue outside the muscularis propria of the stomach, including the adipose tissue of the omentum, is called the subserosa. According to the TNM classification, T3 esophageal and gastric cancer is defined as invasion of the adventitia and subserosa, respectively. Given that the adventitia is anatomically continuous with the subserosa, T3 tumors of the EGJ can be described as those that extend through the muscularis propria but do not invade the peritoneum or diaphragmatic crus. We propose classifying T3 EGJC as "tumor extends through muscularis propria" rather than using the separate terms "adventitia" and "submucosa". T4 could be "tumor perforates serosa or invades adjacent structures", as per the current gastric cancer classification.
Topics: Humans; Esophageal Neoplasms; Stomach Neoplasms; Carcinoma; Esophagogastric Junction
PubMed: 37470882
DOI: 10.1007/s10388-023-01023-x -
Frontiers in Medicine 2020Despite significant progress in the research of fibrosis in various organs, fibrosis remains a poorly understood complication of Crohn's disease (CD). We analyzed...
Despite significant progress in the research of fibrosis in various organs, fibrosis remains a poorly understood complication of Crohn's disease (CD). We analyzed pathologic features of fibrosis and inflammation in CD and compared them with the normal bowel, aiming to clarify whether fibrosis in CD pathogenetically resembles fibrosis in other organs. Resection specimens from 30 patients with CD were included. Normal bowel from resection specimens of colorectal carcinoma was used for comparison. Trichrome Masson staining, immunohistochemistry for α-smooth muscle actin, fibroblast activation protein, CD34 and erg, hybridization for TGF-β1 and analysis of selected fibrosis-related microRNAs were performed. In normal bowel, CD34-positive fibroblasts/pericytes were detected in the submucosa and subserosa, particularly around blood vessels. In CD, fibrosis prevailed in the submucosa and subserosa, together with proliferation of myofibroblasts and disappearance of CD34-positive fibroblasts/pericytes. TGF-β1 was present in the lamina propria in normal bowel and CD, and in deeper parts of the bowel wall in CD. MicroRNAs , and , which have been demonstrated to contribute to fibrosis in various organs, showed significant deregulation in CD. Distribution of fibroblasts/pericytes in the submucosa and subserosa of normal bowel, their disappearance in fibrosis in CD, together with the appearance of myofibroblasts, suggest that fibroblasts/pericytes are the most likely source of myofibroblasts in CD. Furthemore, fibrosis-related microRNAs showed deregulation in fibrotic areas. Pathogenesis of fibrosis in CD is thus comparable to fibrosis in other organs, in which myofibroblasts are the key effector cells, and pericytes have emerged as the main origin of myofibroblasts. Fibrosis in CD should be regarded as a result of (over)response of the bowel wall to the presence of inflammation in deep structures of the bowel wall, presenting another example of a common pathogenetic pathway of fibrosis development.
PubMed: 32432120
DOI: 10.3389/fmed.2020.00167