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 bladder: Dramatic presentation of an unusual tumor

We report a case of an inflammatory myofibroblastic tumor of the bladder (IMT) in an adult male who presented with recurrent hematuria. He required partial cystectomy which revealed perivesical fat infiltration. In spite of this, the tumor was categorized as benign and the patient remained symptom- and tumor-free 18 months post-operatively.     

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     

Inflammatory myofibroblastic tumor of the bladder in a 3-year-old boy

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with unknown malignant potential that has been described in most organ systems. We present the case of a 3-year-old boy who was referred with lower urinary tract symptoms and macroscopic hematuria. An IMT was suspected after clinical, radiological, and surgical work-up, and the diagnosis was confirmed after a partial cystectomy was performed. A bladder-preserving approach is the treatment of choice, but close clinical follow-up is recommended because of the unknown biological behavior of these tumors.     

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 in children: diagnosis and treatment

BACKGROUND/PURPOSE: Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm. Although it is commonly seen in children, the number of childhood cases in the current literature is limited. Furthermore, malignant degeneration or transformation to lymphoma in the recurrent or residual IMT have directed attention to this interesting entity. Herein, the authors present their experience with IMT with special emphasis on diagnosis and treatment. METHODS: All records of children treated with diagnosis of IMT between 1977 and 1999 inclusive were evaluated retrospectively. RESULTS: Seven children were treated for IMT with the mean age of 9.14 +/- 2 years (range, 6 to 12 years). Male to female ratio was 5:2. Respiratory symptoms and clubbing were present in a patient with pulmonary IMT (n = 1). Abdominal pain (n = 3), fever (n = 2), and weight loss (n = 4) were encountered in intraabdominal IMTs. The most frequent physical finding was palpable intraabdominal mass (n = 4). Plain films showed nonspecific findings such as radiodense area in the hemithorax (n = 1), displacement of bowel segments (n = 2), air-fluid levels (n = 1), and amorphous calcification (n = 4). Ultrasonography and CT showed calcified masses in 4 cases. Except the case with intrathoracic IMT, all the tumors were located in the abdomen at various sites such as cardioesophageal junction (n = 1), left hepatic lobe (n = 1), mesentery of the small bowel (n = 2), and antimesenteric wall of the descending colon (n = 1), gastrosplenic region and porta hepatis (n = 1). Tumor sizes ranged from 3 x 2 x 2 cm to 15 x 15 x 13 cm. The masses were excised totally in all but one case. Infiltrated organs (esophagogastric junction, a segment of jejunum, and spleen, stomach wall, and renal capsule) were resected in 3 cases. Total surgical excision of IMT was considered adequate for treatment in 6 cases. One patient with aggressive IMT required further treatments such as immunomodulation and chemotherapy and died of neutropenic sepsis. CONCLUSIONS: IMT is a benign neoplasm rarely presented with malignant features such as local invasiveness, recurrence, distant metastasis, or malignant transformation. IMT can be suspected preoperatively through some hematologic abnormalities and radiologic findings, but precise diagnosis should be made on the basis of histologic findings. Complete surgical resection and close follow-up are all necessary for appropriate treatment to avoid recurrences as well as unnecessary and potentially harmful therapy. The optimal management of locally aggressive and recurrent forms should be decided individually for each patient. J Pediatr Surg 36:908-912.     

Inflammatory myofibroblastic tumor of spermatic cord

INTRODUCTIONInflammatory myofibroblastic tumor (IMT) is a neoplasm of intermediate biologic potential. Only a few cases of IMT in the spermatic cord have been reported. It was earlier included in a wide spectrum of reactive and neoplastic lesions called “inflammatory pseudotumors”. It commonly presents as a painless scrotal mass, usually in children and young adults.PRESENTATION OF CASEWe present a case of IMT in the spermatic cord who based on clinical, radiological and cytological findings underwent surgical exploration of left scrotal sac. The mass was separate from the left testis and left epididymis, and was closely adherent to pampiniform plexus of veins. Wide excision of the mass was done. Histology and immunohistochemistry suggested IMT.DISCUSSIONIMT is a myofibroblastic spindle cell proliferation with chronic inflammatory infiltrate. Surgical exploration is essential as clinically and radiologically benign or malignant nature of mass cannot be distinguished. The diagnosis of IMT is based on the histological features and is substantiated by immunomarkers.CONCLUSIONIn clinically distinct masses, based on frozen section, either tumor excision or radical orchidectomy can be performed. The prognosis is excellent after complete surgical excision of spermatic cord IMT. Careful long-term follow-up is essential, because of the possibility of recurrence, though rare in this site.     

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.     

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 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 mimicking anterior mediastinal malignancy

Inflammatory myofibroblastic tumor arising in the anterior mediastinum is rare. A 58-year-old woman had an anterior mediastinal mass invading the sternum, pericardium, and pleura. It was completely resected and found to be an inflammatory myofibroblastic tumor. The patient was well and had no recurrence 6 months after surgery.     

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.     

Inflammatory myofibroblastic tumor of the urinary bladder in a 27-year-old woman with systemic lupus erythematosus

Abstract:   Spindle cell lesions of the urinary bladder are uncommon tumors, and are most often spindle cell (sarcomatoid) carcinomas, non-neoplastic reactive mesenchymal proliferations, or soft tissue sarcomas. Inflammatory myofibroblastic tumors (IMTs) may also occur in this location. Herein, we report an unusual case of an IMT arising in a previously uninstrumented bladder of a 27-year-old African American female with systemic lupus erythematosus. To our knowledge, IMT has not been reported in the clinical setting of rheumatologic disease.     

Inflammatory (pseudosarcomatous) myofibroblastic tumor of the urinary bladder causing acute abdominal pain.

Inflammatory myofibroblastic tumor is a reactive proliferation of myofibroblasts that rarely involves the urinary bladder. The cause of inflammatory myofibroblastic tumor is unknown but may represent an initial reactive process to an infectious agent or trauma that transforms into neoplastic growth. Cases reported in children, however, often lack any preexisting bladder pathology. The authors present a case in a young child that presented as acute abdominal pain. In general, these tumors follow a benign clinical course after resection, although close monitoring is essential given the rarity of this bladder lesion.     

肾炎症性肌纤维母细胞瘤临床病理分析

目的探讨肾炎症性肌纤维母细胞瘤的病理学特点、临床表现和预后。方法总结1例罕见的肾炎症性肌纤维母细胞瘤,结合文献对其临床表现、组织形态、免疫组化特点、治疗及预后等进行分析和探讨。结果患者女性,58岁,无明显诱因出现排酱油色尿,伴尿频、尿急、尿痛。CT提示＂右肾盂输尿管交界处占位性病变＂,行右侧肾切除术。肾肿物大小为9cm×7cm×6cm,圆形,灰白色,质地较硬,无包膜,与周围界限欠清。肿物切面为灰白色,实性,部分区域粘液变性、出血及坏死。镜下见肿瘤细胞呈梭形,肿瘤细胞呈束状或编织状排列,伴较多淋巴细胞和浆细胞浸润。免疫组化显示Vimentin及SMA呈阳性表达。病理诊断：肾炎症性肌纤维母细胞瘤。结论肾炎症性肌纤维母细胞瘤是非常少见的低度恶性肿瘤,确诊需依靠病理诊断,治疗多采用外科手术切除。