Gastric calcifying fibrous tumor removed by endoscopic submucosal dissection

The World Health Organization describes calcifying fibrous tumors(CFTs) as rare, benign lesions characterized by hypocellular, densely hyalinized collagenization with lymphoplasmacytic infiltration. These tumors rarely involve the gastrointestinal(GI) tract. A routine endoscopic upper gastrointestinal screen detected a 10-mm submucosal tumor(SMT) in the lesser curvature of the lower corpus of the stomach of an apparently healthy, 37-year-old woman with no history of Helicobacter pylori infection. Endoscopic ultrasonography(EUS) localized the internally isoechoic, homogeneous SMT mainly within the submucosa. Malignancy was ruled out using endoscopic submucosal dissection(ESD). A pathological examination confirmed complete resection of the SMT, and defined a hypocellular, spindle-cell tumor with a densely hyalinized, collagenous matrix, scattered lymphoplasmacytic aggregates as well as a few psammomatous, dystrophic calcified foci. The mass was immunohistochemically positive for vimentin and negative for CD117(c-kit protein), CD34, desmin, smooth muscle actin(SMA) and S100. Therefore, the histological findings were characteristic of a CFT. To date, CFT resection by ESD has not been described. This is the first case report of a gastric calcifying fibrous tumor being completely resected by ESD after endoscopic ultrasonography.     

Gastric Calcifying Fibrous Tumor: A Case of Suspected Immunoglobulin G4-related Gastric Disease

Gastrointestinal lesions resulting from immunoglobulin G4-related disease are classified into two types: One is a gastrointestinal lesion showing marked thickening of the wall, and the other is an IgG4-related pseudotumor. We report the case of a woman with gastric calcifying fibrous tumor undergoing endoscopic resection that contained 62 IgG4+ plasma cells per high-power field and an IgG4-to-IgG ratio of 41% in lesional plasma cells, which shared clinical and histopathological features associated with gastric IgG4-related pseudotumor. So, we postulate that calcifying fibrous tumor as part of the spectrum of IgG4-related disease might be the unifying concept with IgG4-related pseudotumor. Meanwhile, the patient had coexistent autoimmune diseases, including autoimmune atrophic gastritis, Hashimoto''s thyroiditis, and possible primary biliary cirrhosis. The clinical follow-up evaluation was uneventful.     

Inflammatory myofibroblastic tumor of the liver

Inflammatory myofibroblastic tumor is a rare benign entity formerly known as inflammatory pseudotumor. Involvement of the liver is extremely rare. There are controversies about the optimal treatment of this benign entity. Newer reports suggest an association with autoimmune sclerosing pancreatitis and primary sclerosing cholangitis. We present a case of an 18-year-old patient with biliary obstruction from a perihilar mass of the liver requiring hepatic resection. Division of the hepatic bile duct resulted in drainage of yellow, thick, gelatinous material in the presence of benign margins and absence of cholangitis. Histological examination showed a mass with fibroblastic and myofibroblastic cells set in a loose myxoid matrix containing scattered lymphocytes, consistent with an inflammatory myofibroblastic tumor. One-year recovery was uneventful. This report discusses the presentation, diagnosis, and controversies in management of this disease.     

Soft tissue sarcoma as second malignant lesion after therapy for Hodgkin's disease

Summary Two cases of soft tissue sarcoma following treatment of nodular sclerosing Hodgkin's disease are described. One patient developed a malignant schwannoma after radiotherapy, the other was diagnosed as having a malignant fibrous histiocytoma after treatment with radiation and chemotherapy. Both secondary malignancies arose within the irradiated field after a latent period rangig from 9.5 years in the first to 21 years in the second case. A review of the pertinent literature is given and previous reports of malignant tumors and leukemias following therapy for Hodgkin's disease are summarized.     

Primary malignant fibrous histiocytoma of the intestine: Intussusception of a rare neoplasm

Malignant fibrous histiocytoma, a tumor of mesenchymal tissue, is being reported with increased frequency. However, no cases of primary tumors involving the gastrointestinal tract have yet been described. This report documents a case in which the patient clinically manifested a colonic obstruction associated with intussusception. Following radical resection of the mass, close re-evaluation after one year has shown no evidence of tumor recurrence.     

Inflammatory myofibroblastic tumor of the esophagus treated by endoscopy

Inflammatory myofibroblastic tumors are rare lesions that have been described in virtually every organ including the gastrointestinal tract. The esophagus is an extremely unusual site for these tumors, with only a few cases described in the literature. Surgery has been the most common therapeutic approach used for the resection of these lesions. In the present case, a patient diagnosed with an inflammatory myofibroblastic tumor of the upper esophagus was reported, and it was successfully removed by endoscopy with no complications.     

A case of inflammatory malignant fibrous histiocytoma of the colon

Primary malignant fibrous histiocytoma seldom occurs in the alimentary tract. This report documents a case in which a 50-year-old man presented with abdominal fullness and anorexia, together with inflammatory reactions such as fever, leukocytosis, an elevated erythrocyte sedimentation rate and positive CRP. Radiologic and endoscopic studies revealed a large mass in the ascending colon. The histology of the resected tumor revealed inflammatory malignant fibrous histiocytoma, a histologie subtype which is rare among not only soft tissue fibrous but also as gastrointestinal histiocytomas. The inflammatory reactions disappeared following resection of the tumor. Although we suspected that the tumor produced granulocyte colony-stimulating factor because of the slightly elevated serum level and the characteristics of the tumor histology, an immunohistochemical study failed to show the G-CSF production by the tumor cells.     

消化系炎性肌纤维母细胞瘤的研究进展

肝纤维化是多种病因所致慢性肝病的共同病理过程,如不及时控制最终将发展为肝硬化,目前尚无有效的抗肝纤维化的药物.姜黄素是一种酚类化合物,具有抗炎、抗肿瘤、抗脂质过氧化等多种药理作用.大量研究证实,姜黄素可通过多种途径发挥抗肝纤维化作用.此文旨在对近年来国内外有关姜黄素的抗肝纤维化作用及机制的研究进展作一综述.     

An inflammatory myofibroblastic tumor of the ampulla of vater successfully managed with endoscopic papillectomy: report of a case

Inflammatory myofibroblastic tumors (IMTs) are solid neoplastic mesenchymal proliferations composed of myofibroblastic spindle cells admixed with inflammatory infiltrates. The documented sites in the gastrointestinal tract include the esophagus, small intestine, colon, appendix, rectum, pancreas, spleen, liver, and Meckel's diverticulum. Biliary IMTs are rare, and IMTs arising from the ampulla of Vater have not been reported previously. Herein we report the case of a 65-year-old woman with an extrahepatic biliary obstruction due to IMT of the ampulla of Vater, and a successful therapeutic approach using endoscopic ultrasonography and endoscopic papillectomy.     

Inflammatory myofibroblastic tumor of the stomach in an adult female report of a rare case and review of the literature

Inflammatory myofibroblastic tumor is an uncommon mesenchymal neoplasm presenting usually in children and young adults and reported in diverse locations including the lung, abdomen, retroperitoneum, pelvis, and trunk. Only a few cases involving the stomach have been reported, of which only 12 cases have been reported previously in adults. A 35-year-old female presented with complaints of abdominal pain, fever, vomiting, and loss of appetite for one month. Radiologically, a mass was seen along the greater curvature of the stomach, and was excised. Grossly, a well-circumscribed transmural tumor was seen involving the stomach wall. Histology showed a spindle cell lesion with myxoid areas and interspersed inflammatory cell infiltrate, immunopositive for vimentin and smooth muscle actin, and negative for CD34, CD117 and anaplastic lymphoma kinase-1, confirming a diagnosis of inflammatory myofibroblastic tumor. Inflammatory myofibroblastic tumor forms a rare diagnosis in the stomach and is even rarer in adults. We report here an extremely rare case of inflammatory myofibroblastic tumor involving the stomach wall in an adult, and discuss the differential diagnoses at this site.     

Cytohistological and immunohistochemical characteristics of spindle-shaped mesenchymal neoplasms occurring in the gastrointestinal tract

The purpose of the present review is to analyze the cytohistological and immunohistochemical characteristics of spindle-shaped mesenchymal gastrointestinal neoplams (MGNs), a group of unusual neoplastic conditions with different biological behavior. These tumors exhibit clinical pictures strictly related to the site of origin and dimensions, even if they appear generally with an intramural localization. This latter point may suggest an useful application of endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), mainly followed by the cell-block procedure (CBP) in the differential diagnostic approach.

First of all, we discuss the most common entity of MGNs represented by gastrointestinal stromal tumors (GISTs), analyzing the morphologic characteristics and stressing the strength of immunohistochemical algorithm for diagnostic purposes. Successively, we have reported the less common group of spindle-shaped MGNs comprehensive of those arising elsewhere the soft tissues, such as leiomyomas, leiomyosarcomas, schwannomas, inflammatory myofibroblastic tumor and intra-abdominal desmoid fibromatosis. Finally, very uncommon spindle-shaped MGNs, like clear cell, follicular dendritic cell, undifferentiated pleomorphic and radiation-induced sarcomas as well as spindle cell dedifferentiated liposarcomas, have been briefly mentioned.     

Plexiform angiomyxoid myofi broblastic tumor of the stomach

Plexiform angiomyxoid myofibroblastic tumor of the stomach is a unique mesenchymal tumor that we first described in 2007.The tumor is very rare,and to date,only 18 cases confirmed by immunohistochemistry have been reported in the literature.The patients' ages ranged from 7 to 75 years(mean,43 years),and the male-to-female ratio was approximately 1:1.Representative clinical symptoms are ulceration,associated upper gastrointestinal bleeding(hematemesis),and anemia.The tumors are located at the antrum in all c...     

Recurrent inflammatory myofibroblastic tumor with renal, retroperitoneal and lymph node involvement

Inflammatory myofibroblastic tumors are uncommon and benign tumors with unknown aetiology. First reported in the lungs, the inflammatory myofibroblastic tumors have been observed in other locations, especially in the abdomen and the pelvis. We report a 14-year-old adolescent female, who presented sequentially an inflammatory pseudotumor of lymph node, the left kidney and the retroperitoneum. Extrapulmonary inflammatory myofibroblastic tumors are mesenchymal solid tumors. They are frequently circumscribed and confined to a single organ. The recurrence of some inflammatory myofibroblastic tumors and their expression of chromosomal abnormalities found in some types of lymphoma suggest that some of these lesions constitute a true neoplastic process.     

Inflammatory myofibroblastic tumor of the heart

An inflammatory myofibroblastic tumor (IMFT) is recognized as benign tissue proliferative response comprising a variety of inflammatory and mesenchymal cells, and presents commonly at a young age. Although it occurs most frequently in the lung, it has also been observed in other organs and tissues such as the liver, spleen, bladder, and lymph nodes. However, IMFT of the heart is rare, and previously only 38 cases have been reported in the English literature. We herein report the case of a 65-year-old woman with asymptomatic IMFT in the right ventricular outflow tract. Previously reported cases are reviewed.     

Inflammatory Myofibroblastic Tumor of the Lung: Unusual Presentation

Inflammatory myofibroblastic tumor (IMT) of the lung is a rare entity considered to be a benign neoplastic lesion. It often mimics spindle cell sarcoma, fibrous histiocytoma, or fibrosarcoma in histologic appearance. Because of its variable cellular composition, it seldom allows diagnosis preoperatively either through transthoracic fine-needle aspiration or bronchoscopic biopsy. In this report we describe a unique occurrence of a rarely described large inflammatory myofibroblastic tumor of the lung that mimics a posterior mediastinal tumor in a 45-year-old female, detailing clinical presentation, imaging, and management.     

Hepatic manifestations of autoimmune rheumatic diseases

Hepatic manifestations in autoimmune disease include chronic active hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis, and nodular regenerative hyperplasia. These diseases are rare and may occur concomitantly or serially. Clinicians must be aware of the possibility of liver disease so that it can be treated as soon as possible.     

胃肠道间质瘤术后随访综述分析

胃肠道间质瘤(gastrointestinal stromal tumor, GIST)是一类较为少见的胃肠道肿瘤,过去常被误认为平滑肌瘤、平滑肌肉瘤、神经鞘瘤或纤维组织细胞瘤,直到1983年Mazur和Clark才通过电镜观察和免疫组织化学检测结果提出了胃肠道间质瘤的概念。尽管少见,但是,GIST却是胃肠道最为常见的间叶源性肿瘤,约占间叶源性肿瘤的18%,每年发病率约为1~2/10万。其生物学特性与临床表现为潜在恶性。随着近年来对胃肠道间质瘤认识的不断发展以及治疗水平的不断提高,越来越多的GIST患者得到诊断,并且通过手术或是辅以口服分子靶向药物的方式来治疗胃肠道间质瘤,取得了很好的疗效。不论是传统的外科手术治疗还是腹腔镜治疗、内镜下治疗或是腹腔镜与内镜双镜联合治疗,这多种手术治疗方式都为大家所认可。但是,与这多种治疗方式不相匹配的是大家对于术后随访的重视程度。目前对于胃肠道间质瘤术后的随访,各国在各自指南中提出了不同要求,尚未达成统一共识。故现就各个国家和地区对胃肠道间质瘤的随访要求进行综述。     

Giant tumor of malignant fibrous histiocytoma (undifferentiated pleomorphic sarcoma) in the cervical esophagus: a case report

We herein report the case of a 50-year-old man with malignant fibrous histiocytoma (MFH) of the esophagus. The patient was admitted to our hospital because of cough, dysphagia, and weight loss. Esophagography and upper gastrointestinal endoscopy revealed a giant protruding lesion in the cervical esophagus. Total esophagectomy was performed with total laryngectomy and pharyngogastrostomy. The tumor was composed of proliferating spindle cells mixed with pleomorphic giant cells. The histopathological diagnosis was malignant fibrous histiocytoma of the esophagus. Although there have been several case reports of MFH of the alimentary tract, MFH of the esophagus is extremely rare. We recently experienced a case of this disease.     

Inflammatory bowel disease of primary sclerosing cholangitis: A distinct entity?

This is a review of the characteristic findings of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC)and their usefulness in the diagnosis of sclerosing cholangitis.PSC is a chronic inflammatory disease characterized by idiopathic fibrous obstruction and is frequently associated with IBD.IBDassociated with PSC(PSC-IBD)shows an increased incidence of pancolitis,mild symptoms,and colorectal malignancy.Although an increased incidence of pancolitis is a characteristic finding,some cases are endoscopically diagnosed as right-sided ulcerative colitis.Pathological studies have revealed that inflammation occurs more frequently in the right colon than the left colon.The frequency of rectal sparing and backwash ileitis should be investigated in a future study based on the same definition.The cholangiographic findings of immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)are similar to those of PSC.The rare association between IBD and IgG4-SC and the unique characteristics of PSC-IBD are useful findings for distinguishing PSC from IgG4-SC.     

Gastrointestinal stromal tumors: review on morphology, molecular pathology, diagnostics, prognosis and treatment options

Gastrointestinal stromal tumors (GISTs) are the most common non-epithelial mesenchymal tumors of the gastrointestinal tract. GISTs represent a specific group of mesenchymal tumors with uncertain biological behaviors. These tumors are assumed to originate from progenitor cells, usually unable to self-regenerate, which differentiate towards Cajal cells. Apart from common GISTs that occur predominantly in adulthood, a heterogeneous group of tumors has been described that are morphologically identical with GIST, but have a specific clinical presentation and biological properties. Approximately 30% of newly diagnosed GISTs are malignant or have a high potential for malignancy. Currently, GISTs are routinely identified with histological, immunohistochemical, and molecular genetic assays. However, clinical diagnoses, particularly of small or intramural GISTs, might be difficult. The most useful techniques for imaging and monitoring disease progression are endoscopic examinations and fused PET/CT imaging. Surgical treatment is the first-line treatment and the only method that might lead to full remission in patients with a primary GIST. There is currently no consensus on the issues of whether to perform resections in patients with positive margins or resections of metastases. Endoscopic resection could represent a relatively simple and less aggressive alternative as compared to traditional surgery in the treatment of small sized GISTs. Biological therapy with imatinib mesylate is recommended for patients with newly diagnosed, locally advanced, inoperable, or metastasizing gastrointestinal GISTs that express the c-KIT protein. Treatment may reduce a primary tumor to a size small enough for surgical excision. Current research is focusing on the development of new therapies for the treatment of advanced disease and/or disease prophylaxis.