肝脏炎性肌纤维母细胞瘤研究进展

炎性肌纤维母细胞瘤,是指由分化的肌纤维母细胞性梭形细胞组成,常伴大量炎细胞和/或淋巴细胞的一种间叶性肿瘤.炎性肌纤维母细胞瘤病因未明,可见于身体各个部位,发病年龄多见于儿童和青少年.肝脏炎性肌纤维母细胞瘤多发于肝右叶,组织活检、CT和MRI有助于诊断和鉴别诊断,且免疫组化常表达波形蛋白(Vimentin),平滑肌肌动蛋白(SMA),肌特异性肌动蛋白(MSA),大部分患者经手术治疗后预后良好.本文复习国内外文献对发生于肝脏的炎性肌纤维母细胞瘤的病因及发病机制、临床表现、影像学特点、组织学形态、免疫组织化学及电镜特征、诊断及鉴别诊断、治疗等问题作系统综述,以期提高对肝脏炎性肌纤维母细胞瘤的认识.     

Inflammatory myofibroblastic tumor of the liver

Hepatic inflammatory myofibroblastic tumors are uncommon low grade malignant neoplasms. They can be confused clinically and by imaging studies with abscess.     

罕见的肝脏炎性肌纤维母细胞瘤的临床病理分析

目的研究肝脏炎性肌纤维母细胞瘤(hepatic inflammatory myofibroblastic tumor,hepatic IMT)的临床特点,以提高对该病的认识和诊疗水平。方法收集5例患者的临床资料,分析和总结其主要的临床病理特点。结果肝脏单发者4例,多部位受累者1例。临床症状包括发热、乏力、纳差、肿块局部疼痛等。可伴有贫血、纤维蛋白原和肝酶增高。病理检查示梭形肿瘤细胞排列成束状或编织状分布于胶原性基质中,伴淋巴细胞、浆细胞浸润,肿瘤细胞表达波形蛋白Vim(5/5)、平滑肌肌动蛋白SMA(5/5)和肌特异性蛋白MSA(5/5)。全部患者行肿瘤完整切除。随访11~65个月,无复发、转移、死亡。结论肝脏IMT的临床表现无特异性,病理学检查和免疫组化对诊断意义较大,手术完整切除是其有效的治疗方法。     

Intra-abdominal inflammatory myofibroblastic tumor: Spontaneous regression

Inflammatory myofibroblastic tumors are usually treated by surgical resection. We herein report two cases of intra-abdominal inflammatory myofibroblastic tumors that were unresectable and underwent spontaneous regression without any treatment. Our case report and literature review show that regression is more common in the middle-aged and older male populations. Abdominal discomfort and fever were the most common symptoms, but the majority of patients had no obvious physical signs. There was no specific indicator for diagnosis. The majority of the lesions regressed within 3 mo and nearly all of the masses completely resolved within 1 year. We conclude that the clinical characteristics of inflammatory myofibroblastic tumors are variable and, accordingly, the disease needs to be subdivided and treated on an individual basis. Surgery is always the first-line treatment; however, for those masses assessed as unresectable, conservative therapy with intense follow-up should be considered.     

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.     

消化系统和腹膜腔炎性肌纤维母细胞瘤的CT表现与病理对照

目的探讨消化系统和腹膜腔炎性肌纤维母细胞瘤(IMT)的临床病理特点及CT影像特征,以提高对该病的认识。 方法回顾性收集并分析2012年2月至2021年2月郑州大学第一附属医院14例经病理组织学确诊的IMT患者的临床、病理及CT影像资料,观察并评估病灶的部位、形态、生长方式、最长直径、边缘、密度、包膜是否完整、表面有无溃疡,分析病灶增强方式及强化程度等,结合文献分析总结IMT的CT特征并与病理结果相对照。 结果14例IMT位于肠系膜3例,胃窦2例,结肠2例,胃体、回肠、回盲部、腹膜后、胆囊、肝脏、胰腺各1例。病灶最长径为2~9(4.90±2.20)cm,6例形态不规则呈分叶状,7例为单发类圆形,1例为多发类圆形;9例有完整包膜,5例包膜不完整;8例瘤内见迂曲血管,11例瘤周见供血血管;12例病灶内见坏死囊变,3例内见钙化,2例内见脂肪,1例合并出血。增强后13例强化不均,7例为全瘤填充样强化,6例为边缘环形强化,1例为中心分隔样强化;9例呈明显强化,4例呈轻度强化,1例呈中度强化。 结论IMT可来源于消化道和腹膜腔各区域,临床表现不典型,CT影像表现有一定特征性,对提示IMT的诊断有一定的价值,可辅助临床制定手术方案和治疗后随访,确诊依赖于病理及免疫组织化学检查。     

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.     

Huge pelvi-abdominal malignant inflammatory myofibroblastic tumor with rapid recurrence in a 14-year-old boy

Inflammatory myofibroblastic tumor(IMT) is an uncommon benign neoplasm with locally aggressive behavior but malignant change is rare.We report an unusual case of pelvic-abdominal inflammatory myofibroblastic tumor with malignant transformation in a 14-year-old boy presenting with abdominal pain and 9 kg body weight loss in one month.Computed tomography revealed a huge pelvi-abdominal mass(30 cm),possibly originating from the pelvic extraperitoneal space,protruding into the abdomen leading to upward displace...     

Rectal inflammatory myofibroblastic tumor: Case report and literature review

Rationale:Rectal inflammatory myofibroblastic tumor (IMT) is an extremely rare mesenchymal tumor characterized by a mixture of spindle-shaped myofibroblasts or fibroblasts and inflammatory infiltration of lymphocytes and plasma cells. To date, only 8 cases of rectal IMT have been reported. Herein, we report an additional case of rectal IMT in a 28-year-old woman.Patient concerns:A 28-year-old woman presented with abdominal pain and hematochezia.Diagnoses:Colonoscopy showed a 3.0-cm subepithelial tumor with central ulceration, covered by white exudate in the rectum. Rectal magnetic resonance imaging revealed a 4.0 × 3.0-cm-sized well-defined subepithelial tumor in the right wall of the rectum, with suspicious right perirectal fat infiltration.Interventions:Laparoscopic anterior resection was performed. Microscopic examination of the surgical specimen revealed bland-looking spindle cells intermingled with lymphoplasma cells. Immunohistochemistry and fluorescence in situ hybridization showed anaplastic lymphoma kinase positivity and anaplastic lymphoma kinase positivity rearrangement. Rectal IMT was confirmed based on histological, immunohistochemical, and fluorescence in situ hybridization findings. The patient was doing well without evidence of tumor recurrence 1 year after the surgery.Lessons:Rectal IMT, despite its rarity, should be considered in the differential diagnosis of rectal cancer. Second, an accurate histopathologic diagnosis and complete surgical resection can be the most important approaches to offer a chance for the cure of rectal IMT.     

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 trachea

Inflammatory myofibroblastic tumor of the trachea is a rare benign tumor in children. We describe a 9-year-old girl who presented with recurrent episodes of wheeze and severe respiratory distress requiring mechanical ventilation. She had recurrent collapses of the right lung and a chest CT and bronchoscopy confirmed the presence of an obstructing mass lesion at the carina. The lesion, 1.5 cm x 1.5 cm in size, was debulked by rigid bronchoscopy and histopathology revealed features of inflammatory myofibroblastic tumor. A repeat bronchoscopy at 1 month of follow up did not reveal any residual lesion.     

Novel treatment of endobronchial inflammatory myofibroblastic tumor in a child

Isolated endobronchial inflammatory myofibroblastic tumors (IMT) are rare, accounting for about 1% of primary endobronchial tumors in children. The mainstay of treatment for this tumor has been surgical resection. Recently, the identification of anaplastic lymphoma kinase (ALK) gene mutations in half of IMTs and promising results of treatment with ALK inhibitors in other ALK-positive tumors have opened the possibility of alternative approaches. We present a 4-year-old child with an ALK-positive endobronchial IMT, treated with endoscopic resection and neoadjuvant therapy with crizotinib, without evidence of tumor recurrence 2 years after the initial resection.     

Hepatic inflammatory pseudotumor presenting in an 8-year-old boy: A case report and review of literature

Hepatic inflammatory pseudotumors are uncommon benign lesions. Accurately diagnosing hepatic inflammatory pseudotumor can be very challenging because the clinical presentation and radiological appearances are nonspecific and cannot be certainly distinguished from malignant neoplastic processes. Herein, we present a case of hepatic IPT in an 8-year-old boy who presented to clinic with a 3-mo history of a tender hepatic mass, fever of unknown origin, and 9-kg weight loss. The physical examination was notable for tender hepatomegaly. Laboratory investigations were notable for a normal hepatic profile and elevated erythrocyte sedimentation rate and C-reactive protein. A T2-attenuated magnetic resonance imaging scan of the abdomen showed a 4.7 cm × 4.7 cm × 6.6 cm, contrast-enhancing, hyper-intense, well-defined lesion involving the right hepatic lobe. In view of the unremitting symptoms, tender hepatomegaly, thrombosed right hepatic vein, nonspecific radiological findings, and high suspicion of a deep-seated underlying infection or malignancy, a right hepatic lobectomy was recommended. Microscopically, the hepatic lesion exhibited a mixture of inflammatory cells (histiocytes, plasma cells, mature lymphocytes, and occasional multinucleated giant cells) in a background of dense fibrous tissue. Immunohistochemically, the cells stained negative for SMA, ALK-1, CD-21 and CD-23, diffusely positive for CD-68, and focally positive for IgG4. The final histopathological diagnosis was consistent with hepatic IPT. At the postoperative 4-mo follow-up, the patient was asymptomatic without radiological evidence of recurrence.     

Inflammatory myofibroblastic tumor successfully treated with chemotherapy and nonsteroidals: A case report

Inflammatory myofibroblastic tumor (IMT) occurring at retroperitoneal sites has rarely been reported. We report the case of a previously well 14-year-old girl with no history of abdominal disease whose past medical history and family tumor history were unremarkable. She complained of intermittent abdominal pain for one month. An abdominal mass was found on physical examination and abdominal contrast-enhanced computed tomography (CT) showed a hypodense soft mass, the size and location of which suggested a well delineated retroperitoneal tumor surrounding the superior mesenteric vessels measuring 3.3 cm × 4.5 cm × 4.5 cm with enlarged lymph nodes. The patient underwent an exploratory laparotomy followed by biopsy and was subsequently diagnosed with retroperitoneal IMT. She was successfully treated with postoperative chemotherapy and oral diclofenac sodium. Following completion of therapy the mass was no longer palpable and no longer visible on CT scanning. The use of methotrexate and cisplatin for aggressive myofibroblastic tumors is also reviewed.     

Inflammatory myofibroblastic tumor (IMT) of the pancreas

Inflammatory myofibroblastic tumor (IMT) is an uncommon mass lesion composed of a variety of inflammatory and other mesenchymal cells. Although IMT was originally reported in the lung, it is now recognized that IMT can occur in a variety of organs. The occurrence of IMT in the pancreas, however, is rare. Here, we report a case of IMT arising from the head of the pancreas in a 55‐year‐old man. He underwent pancreaticoduodenectomy, with the diagnosis of carcinoma of the pancreas; the pathological diagnosis of the tumor was IMT. By referring to previously reported cases, we conclude that, in the management of IMT in the pancreas, surgical excision is the primary choice, in order to obtain a definitive diagnosis as well as to relieve symptoms, and strict follow‐up after surgery is required.     

“Atypical steroid response” in a pulmonary inflammatory myofibroblastic tumor

A 6‐year‐old girl was evaluated for the presence of a paratracheal mass with right upper lobe atelectasis due to an endobronchial mass. Bronchoscopic biopsy established a diagnosis of inflammatory myofibroblastic tumor (IMT) and prednisone initially led to a significant reduction of the endobronchial lesion. However, 8 weeks later, when still on prednisone, the mediastinal mass enlarged dramatically. At thoracotomy, a well‐circumscribed, multilobulated mass was partially resected and a diagnosis of IMT confirmed. Immunosuppression by corticosteroids may have favored the rapid progression of this apparently benign, indolent tumor. Pediatr Pulmonol. 2010; 45:721–726. © 2010 Wiley‐Liss, Inc.