Inflammatory myofibroblastic tumor of the trachea; report of a case

A 45-year-old female with a complaint of dyspnea was admitted to our hospital. Computed tomography (CT) and bronchoscopy revealed a 10 mm intraluminal polypoid tumor with a smooth surface and hemorrhagic tendency located in the tracheal wall 2.0 cm distal from the vocal cords. For pathological diagnosis, airway management and hemorrhage control, tumor resection was performed using a rigid bronchoscope and neodymium yttrium aluminum garnet (Nd-YAG) laser under general anesthesia. Histologically, the tumor was diagnosed as an inflammatory myofibroblastic tumor of the trachea. Inflammatory myofibroblastic tumor is typically a benign tumor that develops in such sites as the lung, stomach, and mesentery. To the best of our knowledge, this is the first reported case of an inflammatory myofibroblastic tumor arising in the trachea.     

肝门部胆管炎性肌纤维母细胞瘤1例

患者女性,54岁,因"中上腹部疼痛1 d"入院。全腹部增强CT：胆总管上段、肝内胆管扩张,胆总管上段软组织影,考虑肿瘤。门诊拟"胆管肿瘤"收入院。患者入院后于全麻下行腹腔镜肝门部胆管肿瘤切除+肝门部胆管整形+肝胆管空肠Roux-en-Y吻合+肝血管瘤切除术。术后诊断：肝门部胆管炎性肌纤维母细胞瘤。患者术后恢复尚可,于术...     

Inflammatory myofibroblastic tumor of the cerebellar hemisphere--case report

A 60-year-old man presented with a rare cerebellar inflammatory myofibroblastic tumor (IMT) manifesting as gait disturbance and dysarthria. Brain magnetic resonance imaging demonstrated an intra-axial round-shaped isointense mass homogeneously enhanced with gadolinium in the right cerebellar hemisphere, as well as perifocal edema extending to the brain stem and right thalamus. The tumor was elastic hard and was resected en bloc with a clear margin. Histological examination revealed IMT with spindle cells and collagen, but negative for anaplastic lymphoma kinase expression. IMT most commonly affects the lung, but may involve many other parts of the body. There is some debate regarding the disease entity of IMT in the central nervous system (IMT-CNS) because of its rarity and high frequency of recurrence. IMT-CNS is an important differential diagnosis among tumor-like intracranial lesions and total resection is required.     

Inflammatory myofibroblastic tumor of the rectum in a 13-month-old girl: a case report

Inflammatory myofibroblastic tumor is a rare benign neoplasm. It is common in children and has been reported in various locations throughout the body but rarely in the rectum. A 13-month-old girl presented with a short history of a painless anal mass and no hematochezia. The mass was completely excised, and histologic examination of the initial biopsy showed fascicles of spindle cells in a mixed inflammatory background with predominance of plasma cells, typical of an inflammatory pseudotumor. The spindle cells were positive for smooth muscle actin and anaplastic lymphoma kinase staining. There is no evidence of recurrence or metastasis after a follow-up of 4.5 years.     

前列腺炎症性肌纤维母细胞瘤1例报告并文献复习

目的:提高前列腺炎症性肌纤维母细胞瘤(IMT)的诊治水平. 方法:回顾性分析l例前列腺IMT患者的临床资料并复习相关文献.患者,男性,62岁,因反复排尿困难曾在当地医院诊断为“前列腺增生症”,前后行两次前列腺电切术(TURP).术后症状仍反复发作,故患者第1次来我院就诊,行TURP加经尿道膀胱肿瘤电切术(TUR-BT)...     

膀胱炎性肌纤维母细胞瘤一例报告并文献复习

目的探讨膀胱炎性肌纤维母细胞瘤的临床、病理、组织化学特性和诊治方法及预后。方法总结1例膀胱炎性肌纤维母细胞瘤患者的临床资料。患者,男,54岁,肉眼血尿10 d入院。盆腔B超及CT增强扫描示膀胱前壁占位性病变。膀胱镜检可见顶壁4 cm×4 cm×5 cm肿物,表面缺血呈暗红色,病理活检疑为膀胱尿路上皮恶性肿瘤。结果行膀胱部分切除术。组织学表现为增生的梭形或长梭形肿瘤细胞囊状分布,间质小血管增生,伴淋巴细胞、嗜酸性粒细胞及浆细胞浸润,诊断为膀胱炎性肌纤维母细胞瘤。免疫组织化学染色结果为瘤细胞平滑肌特异性肌动蛋白（＋）、波形蛋白（＋）、间变性淋巴瘤激酶（＋）。随访6个月,患者无瘤存活。结论膀胱炎性肌纤维母细胞瘤确诊依据病理学检查,应与横纹肌肉瘤、梭形细胞肉瘤等鉴别,治疗以膀胱部分切除或经尿道切除为主。     

Inflammatory myofibroblastic tumor of the epididymis: case report and review of the literature

The inflammatory myofibroblastic tumor (IMT) is a rare benign tumor, known by several names and found in several locations throughout the body, including the lungs and genitourinary system (mostly the bladder). We report a case of a paratesticular IMT in a 17-year-old boy. Although the initial clinical diagnosis was rhabdomyosarcoma, the histopathologic examination revealed it to be an IMT. The preoperative diagnosis of a scrotal mass should differentiate between benign and malignant tumors. A definitive diagnosis of a scrotal mass should be established by surgical exploration and resection. It is important to counsel patients with scrotal masses regarding the possibility of orchiectomy, even in the case of a benign tumor such as IMT.     

淋巴结炎症性肌纤维母细胞瘤1例

<正>患者男,42岁,主因"右下腹疼痛5天"急诊入院。专科检查:腹部平软,右侧腹股沟区压痛、无反跳痛及腹肌紧张。血常规及生化检查均正常。CT(图1)及MRI(图2):右侧髂窝占位性病变,平扫CT呈等密度、内有小点状钙化灶,MRI呈等T1混杂T2信号;增强扫描均呈渐进性明显强化,中央有星芒状瘢痕样无强化区,考虑巨淋巴结增生症可能性大。CTA:右侧髂窝肿块由髂内动脉分支供血,引流静脉为髂静脉,下腔静脉早显,考虑血管畸形可能     

成人双侧输尿管炎症性肌纤维母细胞瘤1例报告并文献复习

目的介绍成人双侧输尿管炎症性肌纤维母细胞瘤（IMT）的病因、临床、病理特点和诊治方法及预后,提高对该病的认识。方法报告1例成人双侧输尿管炎症性肌纤维母细胞瘤病例。结合文献探讨本病病因、临床表现、诊断、治疗及预后。结果患者男性,59岁,双侧腰痛半月人院。影像学检查提示双侧输尿管下段占位性病变。手术完整切除双侧肿瘤,行输尿管膀胱再植术。术后病理诊断为双侧输尿管炎症性肌纤维母细胞瘤,免疫组化显示ALK（灶状4-）,SMA（＋）,vimentin（＋）,CD34（-）,desmin（-）,S-100（-）。随访3月未见肿瘤复发及转移。结论成人双侧输尿管IMT罕见。临床难与输尿管恶性肿瘤鉴别,确诊需依靠病理,手术完整切除肿瘤是目前有效治疗方法。     

膀胱炎性肌纤维母细胞瘤的诊断与治疗

目的 提高膀胱炎性肌纤维母细胞瘤的诊治水平.方法 膀胱炎性肌纤维母细胞瘤患者7例.男3例,女4例.平均年龄38(10～54)岁.临床主要表现为尿痛20～60 d(平均33 d)、血尿3～20 d(平均9 d),肿瘤位于膀胱左侧壁3例,顶底部4例.B超检查均提示低回声实质性团块,大小1.4 cm×1.2 cm～4.7 cm×4.5 cm.CT检查表现为不规则或类圆形软组织低密度影.膀胱镜检查提示1.5 cm×1.5 cm～5.0 cm×5.0 cm大小菜花状肿物,基底部较宽,表面有坏死组织及血块附着2例.膀胱镜下取活检3例,2例报告移行细胞癌Ⅰ级,1例报告膀胱移行细胞乳头状增生,小灶性区域呈恶性潜能未定,乳头状肿瘤改变.术中行冰冻病理切片2例,1例报告膀胱肉瘤,中低度恶性;1例报告膀胱间叶组织肿瘤,低度恶性.结果 7例均行膀胱部分切除术.病理诊断膀胱炎性肌纤维母细胞瘤.肿瘤1.5 cm×1.5 cm～5.0 cm×5.0 cm大小,多为灰褐色,质软,有光泽,水肿明显.镜下可见明显黏液变区和富于细胞区,梭形、短梭形及星芒状肌纤维母细胞散在于黏液样基质中,以梭形最常见,胞质嗜伊红,并伴大量浆细胞、嗜酸性粒细胞、中性粒细胞及淋巴细胞浸润.免疫组织化学染色7例平滑肌肌动蛋白、波形蛋白、AE1/AE3阳性,间变性淋巴瘤激酶阳性6例,钙结合蛋白阳性4例,结蛋白阳性6例,CD_(34)、CD_(117)及S-100蛋白均阴性.6例平均随访35(1～60)个月,均存活,无复发.结论 膀胱炎性肌纤维母细胞瘤是一种中间型并具恶性潜能的肿瘤.临床表现、影像学检查、膀胱镜检查无特异性,病理检查及免疫组化染色可确诊,治疗以手术切除为主.     

Inflammatory myofibroblastic tumor of the larynx

BACKGROUND: Inflammatory myofibroblastic tumor, composed of myofibroblastic spindle cells with acute and chronic inflammatory cells, is an unusual, benign solid mass that mimics a neoplastic process. METHODS: We report a rare case of a patient with a laryngeal inflammatory myofibroblastic tumor. Laryngoscopy demonstrated a submucosal mass involving the right false cord. The mass was a well-enhanced supraglottic lesion on CT scan. It showed medially high signal intensity and peripherally low signal intensity on T2-weighted MR images, and it displayed a high magnetization transfer ratio; before surgery, it was believed to be a malignant tumor. Laryngoscopic biopsy was performed. Pathologic features of the specimen were diagnostic for inflammatory myofibroblastic tumor. RESULTS: Steroid therapy was chosen for further treatment. No recurrence was observed for 4 years. CONCLUSION: In patients with chronic hoarseness who have a malignant-looking submucosal laryngeal mass, inflammatory myofibroblastic tumor should be considered. Conservative surgery and steroid treatment are advocated because of laryngeal preservation.     

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.     

肝脏炎性肌纤维母细胞瘤

目的探讨肝脏炎性肌纤维母细胞瘤的临床病理特征、诊治和预后。方法总结了一例肝脏炎性肌纤维母细胞瘤病人的临床资料,并复习了国内外的文献,分析和归纳其临床表现、诊断和治疗。结果肝脏炎性肌纤维母细胞瘤的临床病理特征和预后如下:①常见于儿童和青少年;②临床症状常表现为贫血、发热、上腹部隐胀痛、体重下降等;③物理检查可见肝脏包块;④病理特征:该瘤由分化的肌纤维母细胞性梭形细胞组成,常伴大量浆细胞和(或)淋巴细胞浸润;⑤外科切除或肝移植后,预后佳,无复发。结论肝脏炎性肌纤维母细胞瘤有其特征性的临床表现,经手术切除后可治愈。     

肝脏炎性肌纤维母细胞瘤

目的 探讨肝脏炎性肌纤维母细胞瘤的临床病理特征、诊治和预后。方法总结了一例肝脏炎性肌纤维母细胞瘤病人的临床资料，并复习了国内外的文献，分析和归纳其临床表现、诊断和治疗。结果肝脏炎性肌纤维母细胞瘤的临床病理特征和预后如下：①常见于儿童和青少年；②临床症状常表现为贫血、发热、上腹部隐胀痛、体重下降等；③物理检查可见肝脏包块；①病理特征：该瘤由分化的肌纤维母细胞性俊形细胞组成．常伴大量浆细胞和（或）淋巴细胞浸润；⑤外科切除或肝移植后，预后佳，尤复发。结论肝脏炎性肌纤维母细胞瘤有其特征性的临床表现，经手术切除后可治愈。     

Inflammatory myofibroblastic tumor of the renal pelvis

Inflammatory myofibroblastic tumor (IMT) of the genitourinary tract is rare and has been classified into separate groups based on their anatomical site and postulated aetiology. Herein, we present a case of IMT of the renal pelvis. A 44-year-old man presented with gross hematuria. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) revealed a tumor of the left renal pelvis. Under the diagnosis of left renal pelvic tumor, he underwent left nephrectomy and ureterectomy. Microscopic examination revealed fascicular spindle cell proliferation in an oedematous myxoid background with an infiltrate of plasma cells. The spindle cells were strongly positive for smooth muscle actin (SMA) and vimentin, and negative for desmin and anaplastic lymphoma kinase (ALK). Diagnosis of the lesion was IMT of the renal pelvis.     

Inflammatory myofibroblastic tumor of the lung.

OBJECTIVE: Inflammatory myofibroblastic tumor (IMT) is a rare disease that usually occurs in the lung. Recently, several reports have suggested that IMT is a true neoplasm rather than a reactive lesion. In this retrospective study, we reviewed clinicopathological characteristics and prognoses for all patients with surgically resected IMT of the lung at our institute. METHODS: From January 1985 to December 2002, nine patients had surgical intervention for IMT of the lung at the National Cancer Center Hospital, Tokyo. The resected lesions were studied histologically, immunohistochemically, and ultrastructurally. Follow-up was complete in all patients and varied from 3 months to 16 years 2 months (median, 6 years 2 months). RESULTS: These nine patients included five men and four women. They ranged in age from 25 to 66 years. Seven patients were asymptomatic. The two symptomatic patients had problems including cough, hemoptysis, and dyspnea. For all these patients, the diagnostic procedure was surgical excision. The resected tumor size ranged from 1.0 to 4.0 cm in diameter. Histologically, a variety of inflammatory and spindle cells were observed. The spindle cells corresponded ultrastructurally to myofibroblasts or fibroblasts. With the exception of one patient who had spontaneous resolution of a recurrent tumor, there was no recurrence in these patients, and all of them are in good health. CONCLUSIONS: Histopathologically, IMT is characterized by myofibroblasts that are mixed with chronic inflammatory cells, including plasma cells, lymphocytes, and histiocytes. Surgical resection, when possible, can be chosen as the treatment. Complete resection leads to excellent survival.