Inflammatory Myofibroblastic Tumor of the Liver: A Diagnostic Challenge

Inflammatory myofibroblastic tumor (IMT) is an uncommon myofibroblastic neoplasm that was formerly included within the broad category of inflammatory pseudotumor (IPT). IMT is rarely encountered in the liver. Similar to IMT of other organs, the interchangeable use of the terms IMT and IPT in liver has made the analysis of these lesions difficult. In this review, clinical and pathological features of IMT of the liver are reviewed and the differential diagnosis of IMT is discussed, with emphasis on IPT and the other entities included in this large category. IMT can mimic malignant tumors. There are no known unique diagnostic clinical, laboratory, or radiological features. The definitive diagnosis of IMT depends on careful pathological examination. The histopathological evaluation of hepatic IMT reveals that, the myxoid/vascular pattern is the most frequently observed, followed by, in decreasing frequency, fibrous histiocytoma-like pattern and hypocellular fibrous pattern. In IMT of the liver, anaplastic lymphoma kinase (ALK) expression reliably predicts the presence of an ALK gene rearrangement. The diagnosis of hepatic IMT depends on the dominant histopathological pattern, and the management of the disease is still controversial. IMT of the liver is a distinctive neoplasm of intermediate biological potential, and should be distinguished from the variety of lesions that are included under the broad category of IPT. Therefore, to avoid confusion regarding the true incidence and behavior of hepatic IMT, the term IPT should not be used interchangeably with IMT. The rarity of IMT in liver should not minimize its consideration in the differential diagnosis of liver tumors, especially in patients with tumor markers in normal range.     

Inflammatory Myofibroblastic Tumor Presenting with Diabetes Insipidus in an Eight-Year-Old Boy: A Case Report

Inflammatory myofibroblastic tumors (IMT) develop as a non-neoplastic proliferation of myofibroblasts in a myxoid to collagenous stroma admixed with inflammatory cells. The symptoms depend on the specific location of the tumor, which can be anywhere, but is particularly in the respiratory system. Thus, patients with IMT can present with a variety of findings. A pediatric patient with IMT who presented with cough, breathlessness, polyuria-polydipsia, and convulsions is described in this report.     

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.     

An Unexpected Cause of Respiratory Distress and Cyanosis: Cardiac Inflammatory Myofibroblastic Tumor

Inflammatory myofibroblastic tumor is an uncommon spindle cell tumor, occurring mainly in children and young adults. It is an extremely rare cardiac tumor especially patients under 1 year. Although it is benign, the tumor may be very aggressive locally. The diagnosis of this unusual pediatric cardiac tumor without pathologic specimens is difficult. We report a rare case of inflammatory myofibroblastic tumors of the right ventricle in a 7‐month‐old girl presenting with respiratory distress and cyanosis.     

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.     

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 pseudotumor of the spleen in a patient with human immunodeficiency virus infection: a case report and review of the literature

Inflammatory pseudotumor of the spleen (IPS) is a rarely described benign tumoral lesion of unknown etiology and pathogenesis. Diagnosis is complex as clinical manifestations and imaging features are indistinguishable from lymphoproliferative disorders and other malignancies of the spleen. Human immunodeficiency virus (HIV) infection is often combined with several malignancies including non-Hodgkin's lymphoma and Kaposi's sarcoma. However, no HIV infection-associated IPS has been reported so far. We report and discuss a case of IPS in an HIV-infected woman who presented with abdominal pain and multiple lesions in the spleen.     

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.     

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.     

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 pseudotumour of the lung

Inflammatory pseudotumours of the lung are extremely rare. Their pathogenesis is controversial, their diagnosis is often difficult and their clinical behaviour may be unpredictable – ranging from benign to locally invasive, to metastatic in spite of an apparently ‘benign’ histology. A patient who presented with multiple recurrent lesions in the contralateral lung almost two years after the resection of a large primary tumour of the left upper lobe is reported.     

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.     

肿瘤标记物联合检测在肺癌诊断中的临床应用

目的 评价癌胚抗原(CEA)、血清细胞角蛋白19片段(CY211)、糖类抗原125(CA125)及神经元特异烯醇化酶(NSE)水平对肺癌早期诊断、判断病理类型中的意义.方法 检测89例肺癌患者和82例肺良性疾病患者血清CEA、CY211、CA125及NSE水平并进行比较分析.结果 肺癌患者血清CEA、CY211、CA125及NSE水平均高于肺良性疾病组,前三者差异有统计学意义(P＜0.05);其中CY211的敏感性优于其余3项指标,4项联合检测的敏感性优于单个指标,差异有统计学意义(P＜0.01),NSE在小细胞癌中敏感性最高,CEA在腺癌中敏感性最高,CY211在鳞癌中最高.结论 血清CEA、CY211、NSE及CA125均可作为肺癌筛查的指标;其中CY211最敏感.另外,不同的肿瘤标记物升高对不同的病理分型有一定帮助;4项联合检测可提高肺癌诊断的灵敏度、准确率.     

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.     

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。