Successful treatment of inflammatory myofibroblastic tumor with malignant transformation by surgical resection and chemotherapy

Inflammatory myofibroblastic tumor (IMT) is a tumor composed of myofibroblasts and a mixed inflammatory infiltrate that rarely undergoes malignant transformation. The authors present the case of a 7-year-old boy with an abdominal mass diagnosed as IMT with malignant transformation. The tumor recurred twice after attempts at resection and was initially treated with vincristine and etoposide. After a third recurrence and incomplete resection, he was treated with cisplatin, Adriamycin, and methotrexate. He is disease-free after 2 years, representing successful combined surgery and chemotherapy in the treatment of malignant IMT. The use of chemotherapy for aggressive myofibroblastic tumors is reviewed.     

小儿炎性肌纤维母细胞瘤诊断与治疗的探讨

目的探讨小儿炎性肌纤维母细胞瘤(IMT)的诊断与治疗方法。方法分析和总结我院收治的5例手术病理确诊为IMT患儿的临床表现、实验室检查、影像学检查、病理学诊断、外科治疗方法及随访情况等。结果 5例IMT患儿中,2例肿瘤位于膀胱,3例肿瘤分别位于颈部、腹部及右下肺,行肿瘤完整切除术,其中膀胱及右下肺肿瘤患儿术后随访2～8个月未见肿瘤复发,腹部肿瘤患儿未能随访;1例膀胱肿瘤活检,明确诊断后家长放弃治疗;1例颈部肿瘤复发,初期经化疗肿瘤消失,后出现颈部肿瘤转移及复发,再次手术及化疗,后期无法有效控制肿瘤增长,最终家长放弃治疗。结论小儿IMT是一种罕见的软组织肿瘤,无特异性表现,确诊依靠病理学检查。手术切除是有效的治疗方法,但存在复发和转移可能,术后需长期随访,对于复发者化疗有一定疗效,反复复发者预后不良。     

Inflammatory myofibroblastic tumor of the trachea

This report described a 2-year-old boy who was presented with severe respiratory distress and stridor. Bronchoscopy and CT revealed a mass in the left anterolateral tracheal wall and histopathology showed a tracheal inflammatory myofibroblastic tumor. Initial removal by rigid bronchoscopy resulted in prompt recurrence of the tumor. Therefore, he underwent tracheal surgical resection. A bronchoscopy at 12 months after surgery did not show any recurrence sign.     

A case of an inflammatory myofibroblastic tumor in the lung which expressed TPM3–ALK gene fusion

We herein describe a 4-year-old boy who after being treated for pneumonia showed an abnormal shadow at the hilus of the right lung on chest X-rays with continued inflammatory findings in his laboratory data. CT and MR investigations suggested the existence of a neoplasm at that site. An open biopsy was thus performed for a definite diagnosis. The histological findings and the expression of TPM3–ALK fusion gene confirmed a diagnosis of an inflammatory myofibroblastic tumor. A right upper and middle lobectomy including the tumor was thus performed for a complete resection. In addition to the histological diagnosis, the detection of the tumor specific fusion gene provided objective evidence in making a diagnosis.     

儿童输尿管肿瘤的诊治分析

目的 总结儿童输尿管肿瘤的病理类型、临床表现、诊断、治疗和预后,提高对该病的认识.方法 回顾性分析2011年1月至2015年10月我院收治的5例输尿管肿瘤患儿的临床资料,结合文献总结儿童输尿管肿瘤的病理类型、临床表现、诊断、治疗及预后.5例患儿均为男性,输尿管炎性肌纤维母细胞瘤3例,2例以腹痛就诊,分别为6岁和10岁,肿瘤位于输尿管下段,均行输尿管下段肿物切除+输尿管端端(输尿管膀胱)吻合,1例以尿痛并血尿就诊,年龄1岁9月龄,肿物来源于下段输尿管,经输尿管膀胱连接部长入膀胱,继发膀胱输尿管积水,经输尿管和膀胱联合切除肿物+输尿管膀胱再植术.结果 术后随访5~48个月,未见肿瘤复发及转移.恶性横纹肌样瘤1例6岁,以左下腹痛2周就诊,输尿管肿瘤边界不清与腰大肌肉和后腹膜粘连,切除肿物行输尿管端端吻合,术后ICE方案化疗一疗程后局部复发,放弃治疗,术后233d死亡.尤文/原始神经外胚层瘤1例12岁,以右侧腰痛10d就诊,肿瘤位于输尿管髂血管水平,行输尿管肿瘤切除术+输尿管端端吻合术,术后予CAV+IE化疗12个月,随访56个月,未见转移及复发.结论 输尿管肿瘤在儿童发病率极低,文献曾报道的病理类型包括炎性肌纤维母细胞瘤、恶性横纹肌样瘤、尤文/原始神经外胚层瘤、横纹肌肉瘤,术前无特异方法诊断,手术完整切除肿瘤并重建输尿管是治疗的主要方法,确诊需要结合病理检查,根据病理类型决定是否化疗,治疗后均需要长期随访.     

Clinicopathologic conference: Pulmonary tumor in a thirteen-year-old child

An inflammatory myofibroblastic tumor as a distinct clinicopathologic entity in the lung has gained wide acceptance. The majority of these tumors are either contained within a single segment or pulmonary lobe and may cross the interlobar fissure and/or extend into the mediastinum, especially if arising in a perihilar location. We describe the case of a 13-year-old male with a 4-year history of an unresponsive lung mass in the left hemithorax. The resected tumor showed the typical morphology of a fasciculated fibroblastic/myofibroblastic lesion.     

Liver transplantation for a hilar inflammatory myofibroblastic tumor

A 7-yr-old boy presented with obstructive jaundice secondary to an inflammatory myofibroblastic tumor centered on the hepatic hilum and extending into the liver. The tumor was further complicated by portal vein phlebitis and occlusion. Attempted resection of the tumor with portal vein reconstruction and bilioenteric drainage was unsuccessful and he required urgent orthotopic liver transplantation. In contrast to more peripheral inflammatory myofibroblastic tumors in the liver, hilar lesions are locally aggressive, causing occlusive portal phlebitis and biliary obstruction. Successful management may include the need for liver transplantation.     

Inflammatory myofibroblastic tumour of the liver in a child: CT and MR findings

Inflammatory myofibroblastic tumour of the liver is a rare disease in children and is characterized by an inflammatory mass of uncertain aetiology which simulates a true neoplasm, often resulting in a diagnostic dilemma. We report a pathologically proven case of inflammatory myofibroblastic tumour of the liver in a 14-month-old boy with jaundice. CT and MRI showed an ill-defined, homogeneously enhancing mass located in the hilar portion of the liver with biliary obstruction. He underwent percutaneous transhepatic biliary drainage to relieve obstructive jaundice, followed by lobectomy and, finally, liver transplantation.     

Recurrent gastric inflammatory myofibroblastic tumour in a 13-year-old male

A 13-year-old boy who had epigastric pain and pallor for 2 months and found to have an ulcerative mass in the stomach and underwent partial gastrectomy. A diagnosis of inflammatory myofibroblastic tumour (IMT) of the stomach was made on histological examination. Three years later, recurrence in the stomach, with invasion into the pancreas and hilum of the spleen was noted and was managed by wide wedge resection of the stomach, distal pancreatectomy and splenectomy. The patient has been recurrence-free for the past 2 years. Gastric IMT is an uncommon tumour in children with unpredictable prognosis.     

Successful sleeve lobectomy of pediatric inflammatory myofibroblastic tumor

Inflammatory myofibroblastic tumor (IMT) is an uncommon solid tumor that was originally described in the lung. A 4‐year‐old girl was admitted to hospital with urticarial rash. On chest radiographs, an opacity was seen in the inferior zone of the left lung, and computed tomography showed a mass in the left lower lobe. Left lower sleeve lobectomy was performed, and the diagnosis was confirmed as IMT. Sleeve resection is the best option in lesions located in the mainstem bronchus or secondary carina. Herein, we present a rare case of IMT of the lung that was successfully treated with sleeve lobectomy. There have been fewer than 15 childhood cases of IMT reported in the literature, and the present 4‐year‐old patient is one of the youngest.     

Pediatric inflammatory myofibroblastic tumor: anaplastic lymphoma kinase (ALK) expression and prognosis

BACKGROUND: Pediatric inflammatory myofibroblastic tumor (IMT) is rare, with unpredictable clinical behavior. Recently, it has been associated with anaplastic lymphoma kinase (ALK) expression. METHODS: Patients under age 16, treated for IMT between 1976 and 2000 were reviewed. Mean follow-up was 8 years (range 1 month-22 years). RESULTS: Eight children had IMT, with a mean age of 6 years (range, 11 months-14 years) and female to male ratio of 3:1. Tumor location was lung (four patients), abdomen (two patients), lung and abdomen (one patient), and abdomen, head, and neck (one patient). Presenting symptoms included anemia (seven patients), fever (six patients), and dyspnea (four patients). Laboratory results included thrombocytosis (six patients), hypergammaglobulinemia (four patients), elevated sedimentation rate (four patients), and leukocytosis (three patients). Immunohistochemistry revealed ALK expression in four of eight tumors. Four children had complete resection and are alive. Two of these children had ALK-positive tumors. Four patients had incomplete resection, and two had recurrences treated successfully with resection and radiotherapy; the other two died of disease. For the incomplete resection patients, those that were ALK-positive lived, and those that were ALK-negative did not. CONCLUSIONS: Eight children were treated for IMT over a 15-year period. ALK expression was found in half the tumors. Prognosis was improved with ALK expression and complete surgical resection.     

Inflammatory pseudotumor of the mesentery and small intestine

Three cases of abdominal inflammatory pseudotumor or inflammatory myofibroblastic tumor of the small intestinal mesentery are presented with additional examples from the literature. An abdominal mass was detected in three preadolescent children who presented with fever, growth failure, hypochromic anemia, thrombocytosis and polyclonal hypergammaglobulinemia. The radiographic findings confirmed the presence of a mass. Following surgical resection, the systemic manifestations dramatically resolved. The solid mesenteric mass was composed of spindle cells with the features of myofibroblasts and mature plasma cells and small lymphocytes. Clinical follow-up confirmed the benign nature of this lesion with no signs of recurrence in any patient from 3 to 6 years after the initial surgery.     

Complete resection of hepatoblastoma originating in the caudate lobe: case report and literature review

Hepatoblastoma originating in the caudate lobe is extremely rare, and resection is technically challenging. The aim of this study was to report our experience of complete resection of hepatoblastoma originating in the caudate lobe and review the literature. A 12-month-old boy was admitted for investigation of an upper abdominal mass. Imaging studies showed an irregularly lobulated 8-cm solid tumor in the left lobe of the liver. Serum α-fetoprotein was 67,700 ng/mL. This tumor was diagnosed as hepatoblastoma (PRETEXT II) and chemotherapy (3 courses of CDDP and ADR) was performed. After chemotherapy, the tumor had shrunk to a resectable size and its origin in the caudate lobe could be determined. Left hepatic lobectomy with en bloc resection of the caudate lobe was performed. Total blood loss was 10 mL, and the operation lasted 9 h. His postoperative course was uneventful. Preoperative chemotherapy facilitated complete resection by left hepatic lobectomy with total excision of the caudate lobe.     

Inflammatory myofibroblastic tumor of the bladder in children: what can be expected?

Inflammatory myofibroblastic tumor of the bladder is an uncommon condition of unknown neoplastic potential. In adults the tumor is seen in association with instrumentation of the lower genitourinary tract, while in children it appears to run an idiopathic course. Its clinical and radiological presentation in children resembles sarcoma. The case of a 10-year-old girl with inflammatory myofibroblastic tumor is presented, outlining the histological and immunhistochemical features to allow differentiation between sarcomas, the most important differential diagnosis. An outcome meta-analysis of the literature identified 35 cases of inflammatory myofibroblastic tumor in the bladder of children. Conservative surgery is the strategy of choice. There is no evidence of recurrence or metastasis at a median follow up of 1.5 years.     

MEGADOSE METHYLPREDNISOLONE FOR EVANS SYNDROME

Endobronchial involvement in non-Hodgkin lymphoma is rare even in the presence of advanced disease. A 15-year-old boy presented with progressively worsening dyspnea with occasional hemoptysis for 1 week prior to admission. Three days later, he was intubated due severe dyspnea with complete atelectasis of the right lung. Fiberoptic bronchoscopy disclosed an endobronchial mass almost occupying the right main bronchus. He underwent partial resection of the endobronchial tumor with rigid bronchoscopy. An airway stenting was used in this patient because he had severe tracheal obstruction from the tumor. The compromised airway was alleviated by combined chemotherapy and tracheobronchial stenting.     

Inflammatory myofibroblastic tumor of the midesophagus

An inflammatory myofibroblastic tumor (IMFT) is a rare entity that can arise in a multiplicity of organs including the lung, liver, and at any location within the gastrointestinal tract. Typically, an IMFT presents as a localized mass with clinical symptoms dependent upon its site of origin. IMFTs pathologically resemble a neoplastic process but are theorized to arise from an unknown inflammatory event. We present a case of a midesophageal IMFT in a 12-year-old female.     

Combined chemotherapy and tracheobronchial stenting for life-threatening airway obstruction in a child with endobronchial non-Hodgkin lymphoma

Endobronchial involvement in non-Hodgkin lymphoma is rare even in the presence of advanced disease. A 15-year-old boy presented with progressively worsening dyspnea with occasional hemoptysis for 1 week prior to admission. Three days later, he was intubated due severe dyspnea with complete atelectasis of the right lung. Fiberoptic bronchoscopy disclosed an endobronchial mass almost occupying the right main bronchus. He underwent partial resection of the endobronchial tumor with rigid bronchoscopy. An airway stenting was used in this patient because he had severe tracheal obstruction from the tumor. The compromised airway was alleviated by combined chemotherapy and tracheobronchial stenting.     

INFLAMMATORY MYOFIBROBLASTIC TUMOR OF THE MIDESOPHAGUS

An inflammatory myofibroblastic tumor (IMFT) is a rare entity that can arise in a multiplicity of organs including the lung, liver, and at any location within the gastrointestinal tract. Typically, an IMFT presents as a localized mass with clinical symptoms dependent upon its site of origin. IMFTs pathologically resemble a neoplastic process but are theorized to arise from an unknown inflammatory event. We present a case of a midesophageal IMFT in a 12-year-old female.     

Inflammatory pseudotumor of the lung manifesting as a posterior mediastinal mass

A case of inflammatory pseudotumor of the lung in a 6-year-old boy is presented. The respiratory illness presented as a mycoplasma pneumonia and there had been a similar episode of mycoplasma pneumonia one and a half years previously. Computed tomography revealed a large, calcified right posterior mediastinal mass and exploratory thoracotomy revealed a large posterior mediastinal mass that had an endobronchial component and grew exophytically from the lung. The present case suggests that inflammatory pseudotumor of the lung can manifest as a mediastinal mass and could be associated with mycoplasma pneumonia infection.     

Abdominal Inflammatory Myofibroblastic Tumor: Report on Four Cases and Review of Literature

Background

The Abdominal Inflammatory Myofibroblastic Tumor (AIMT) is a rare tumor with unknown etiology which usually occurs in children and adolescents. It is composed of myofibroblastic spindle cells intermixed with inflammatory cells. We present four cases of AIMT.

Cases Presentation

We herein present four cases of AIMT in different ages (range: 3.5 to 13 years) and in different organs (stomach, periduodenal, mesenteric, and colon). There were two females and two males. The main symptoms were abdominal pain/mass/obstruction, vomiting, and weight loss. In all four patients, diagnosis was made by laparatomy and pathologic examination of excised mass lesion. Three patients underwent complete excision and no residual disease was present, one patient received chemotherapy due to tumor recurrences. The patients were followed up in average for four years.

Conclusion

As the imaging and laboratory tests are non-specific, the diagnosis of AIMT is rarely made before surgery. AIMT should, therefore, be considered when a mass arises in an unusual location in the pediatric age group. Complete surgical resection should be performed whenever possible and the child should be kept on long-term follow-up.