INFLAMMATORY MYOFIBROBLASTIC TUMOR OF THE GASTROESOPHAGEAL JUNCTION IN CHILDHOOD

We report an unusual case of an inflammatory myofibroblastic tumor arising at the gastroesophageal junction in a 14-year-old girl. The bland histologic appearance with concurrent infiltration into adjacent structures made diagnostic interpretation difficult, but suggested a neoplastic process. A literature review was undertaken to address diagnostic and management issues raised in this case. Although the anatomic location was unusual, clinical, gross, histopathologic, and immunohistochemical data substantiated the diagnosis of inflammatory myofibroblastic tumor. The bland histologic appearance was consistent with the most widely accepted view of inflammatory myofibroblastic tumor as a low-grade neoplasm. Wide surgical excision was performed. This is considered the preferred treatment given the potential risk of recurrence and aggressive behavior, most frequently noted with extrapulmonary disease. Although inflammatory myofibroblastic tumor represents an heterologous spectrum of benign to malignant neoplastic proliferations, the prognosis is good in cases with benign histologic features.     

Inflammatory myofibroblastic tumour of the extrahepatic bile ducts: an unusual cause of obstructive jaundice in children.

Inflammatory myofibroblastic tumour is a rare entity in children with few reported series. The extrahepatic bile duct is an unusual location for this tumour. The authors report here the case of an 8-year-old girl presenting with obstructive jaundice due to inflammatory myofibroblastic tumour of the extrahepatic bile ducts with differential diagnosis of obstructive jaundice in children.     

Inflammatory myofibroblastic tumour of the lung manifesting as progressive atelectasis

A 6-year-old girl with an inflammatory myofibroblastic tumour of the lung is presented. The radiological features of this lesion are often atypical, as in this case, presenting as progressive atelectasis. The differential diagnosis is extensive and can be difficult, despite modern imaging techniques. Received: 25 November 1998 Accepted: 19 May 1999     

Inflammatory myofibroblastic tumor of the lung: a rare cause of atelectasis in children

Although rare, inflammatory myofibroblastic tumor is the most common primary lung mass in children. We report the case of an 11-year-old boy investigated for persistent cough and dyspnea with complete left lung atelectasis mimicking pneumonia. CT and MRI showed an endobronchial mass of the left main bronchus. The boy underwent endoscopic resection of the tumor and histology was in favor of an inflammatory myofibroblastic tumor of the lung. This diagnosis should be suspected in children with recurrent pneumonia. The prognosis is good after complete resection.     

Congenital peribronchial myofibroblastic tumor: prenatal imaging clues to differentiate from other fetal chest lesions

We present a prenatal case of congenital peribronchial myofibroblastic tumor referred as a congenital pulmonary airway malformation (CPAM) with hydrops and polyhydramnios at 30 weeks’ gestational age. US and fetal MRI findings did not fit with the referral diagnosis, raising the possibility of intrinsic lung tumor. Fetal hydrops worsened and the baby was successfully delivered by ex utero intrapartum treatment (EXIT) to resection at 31 weeks’ gestational age. To the best of our knowledge, this is a unique case of congenital peribronchial myofibroblastic tumor that underwent comprehensive prenatal evaluation and EXIT procedure with good outcome.     

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.     

Intracranial and mediastinal inflammatory myofibroblastic tumour

Inflammatory myofibroblastic tumour (IMT) is a rare neoplastic condition, commonly known as inflammatory pseudotumour, which is the most frequent primary tumour of the lung in childhood. IMT can have an extrapulmonary location, but is infrequent in the brain and mediastinum, and it is extremely unusual that both lesions appear simultaneously as in our patient. The definitive diagnosis is established by biopsy, but there are some MRI findings that can be useful to identify the lesion. Received: 13 January 2000 Revised: 3 October 2000 Accepted: 20 October 2000     

Intravascular Inflammatory Myofibroblastic Tumors in Infancy

Inflammatory myofibroblastic tumor (IMT), previously described as inflammatory pseudotumor, can occur at any age but is a recognized soft tissue tumor of childhood. Less than 10 previous cases have been described of IMT affecting the heart, in patients ranging from 5 months to 17 years of age. We present three unusual, but similar, cases of IMT in infants, which were all predominantly intravascular in location, one of which was associated with death due to angiodestructive lesions of the coronary and cerebral arteries. These cases demonstrate an apparently distinct phenotype, with a predominant intravascular location of the tumor. Furthermore, this series highlights the difficulty in categorizing such lesions as benign versus malignant on histological grounds alone. IMT should be considered in the differential diagnosis of unusual pediatric intravascular spindle cell lesions.     

Pseudosarcoma of the bladder associated with residual urachus in a 3-year-old girl

Bladder pseudosarcoma or pseudosarcomatous myofibroblastic proliferation (PMP) is a rare but nevertheless very important differential diagnosis of any bladder mass. In childhood most such masses are usually malignant soft-tissue sarcomas, and distinction from these is therapeutically crucial. To date no aetiological factor for PMP has been identified in children, and treatment is limited to surgical intervention with no role for chemotherapy or radiotherapy. We report clinical, anatomical, and histological findings in a 3-year-old girl with a pseudosarcoma of the urinary bladder associated with a residual urachus, suggesting this as the originating tissue. Accepted: 23 August 1999     

Inflammatory myofibroblastic tumor of the lung in children: anaplastic lymphoma kinase (ALK) expression and clinico-pathological correlation

The inflammatory myofibroblastic tumor (IMT) is a rare neoplastic lesion with a high incidence in children and young people, and may arise in lungs, soft tissue, or viscera. It is recognized as a borderline tumor with the possibility to recur, undergo malignant transformation, and metastasize. IMT is composed of fascicles of bland myofibroblastic cells admixed with an inflammatory infiltrate consisting of lymphocytes, plasma cells, and eosinophils. We reviewed pulmonary IMT diagnosed at Garrahan Hospital in Buenos Aires, Argentina, during 12 years and examined the clinical, laboratory, and pathological features as well as molecular genetics. Eight pediatric cases were evaluated with a male-to-female ratio of 5:3 and a median age of 6 years at diagnosis. The most common lung localization was the upper lobe. All cases underwent surgical excision and no local recurrences were found. Five out of eight patients, including two cases with metastatic/multifocal lesions in the central nervous system (CNS), are alive and disease free after a median follow-up of 30 months. Anaplastic lymphoma kinase (ALK) expression was negative in all pulmonary samples by immunohistochemistry (IHC), however, rearrangement for ALK locus by fluorescence in situ hybridization was found in one lung and in two CNS samples. These findings may reflect higher sensitivity of the molecular biologic procedure compare to traditional IHC practice. In our pediatric experience, 25% of patients with lung IMT developed CNS lesions; therefore we consider that CNS screening in these patients should be considered, at diagnosis and later during follow up.     

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。     

Pediatric Inflammatory Myofibroblastic Tumor With Late Metastasis to the Lung: Case Report and Review of the Literature

Inflammatory myofibroblastic tumors (IMTs) are challenging lesions with respect to classification, differential diagnosis, and biologic potential. In children, extrapulmonary IMTs, particularly those from the abdomen or mesentery, are generally aggressive, with frequent local recurrences. There are few documented patients with distant metastasis, and most of these had metastases at presentation or developed metastases within months to a few years. We add to the short list of metastatic extrapulmonary IMTs a pediatric patient in whom the primary lesion was widespread in the abdomen at presentation and metastatic disease to the lung was diagnosed 9 years after the primary resection. We describe the clinical and pathologic features of this patient and review the characteristics of extrapulmonary IMTs with distant metastasis reported in the literature.     

小儿腹部炎性肌纤维母细胞瘤诊治探讨

目的探讨小儿腹部炎性肌纤维母细胞瘤(inflammatory myofibroblastic tumor,IMT)的诊断与治疗方法,以提高其诊治水平。方法分析首都医科大学附属北京儿童医院肿瘤外科2006—2018年收治的18例经手术病理确诊为腹部IMT患儿的临床资料,包括临床表现、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。18例患儿术前均行超声检查、CT或MRI检查。1例行穿刺及术前化疗,1例仅行开腹活检及术后化疗,其余均接受肉眼下肿瘤全切术,其中2例弥漫性腹部病变行多次手术并接受全身规律化疗。结果术后病理提示肿瘤细胞均呈梭形伴有炎症浸润,部分镜下可见玻璃样变或局灶出血。1例随访中失访,1例肿瘤位于腹膜后间隙者于术后3个月死亡,其余16例随访4~29个月,未见术后严重并发症或死亡发生,1例弥漫腹部IMT病灶患儿带瘤存活。结论IMT可来源于腹部各区域并引发不同症状。影像学检查利于定位和诊断,但缺乏特异性。大部分单发病变可以单纯经手术治愈,对患有弥漫性腹部病变的难治性病例的治疗亟待经验积累及进一步研究。     

Pulmonary inflammatory myofibroblastic tumor after Hodgkin’s lymphoma and application of PET imaging

Inflammatory myofibroblastic tumor (IMT) is a rare tumor with a particular histological pattern of myofibroblasts and mixed inflammatory infiltrate. IMT has been rarely described in association with malignancy. This case report is of a 16-year-old male who had Hodgkin's disease (stage IVA) and who after chemotherapy and radiotherapy developed IMT, 16 months post completion of therapy. The IMT was in the lung in an area which was previously involved with HD and had undergone radiotherapy. PET imaging with F(18)FDG was used in the initial diagnosis and has been used in follow-up after full surgical resection of the lesion.     

Inflammatory myofibroblastic tumor of the spleen: Report of a case in an adolescent

Inflammatory myofibroblastic tumor (IMT) is an uncommon quasineoplastic process occurring in young patients. Splenic involvement is uncommon and occurs predominantly in older, adult patients. We present the youngest patient reported to date with splenic inflammatory myofibroblastic tumor and discuss the clinical, pathological and imaging features of this lesion.     

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.     

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.     

Radiological appearance of inflammatory myofibroblastic tumour

Inflammatory myofibroblastic tumour (IMFT) is a distinct entity with variable clinical presentation and therapeutic options. We present three paediatric cases of IMFT, originated from the lung, bladder and ovary respectively. All lesions were heterogeneous, with mixed solid/cystic components and infiltrative pattern, and were interpreted as aggressive malignant neoplasms initially due to their bizarre imaging appearance. The definitive diagnosis was derived from characteristic histopathological features. Pediatr Blood Cancer 2010;54:1029–1031 © 2010 Wiley‐Liss, Inc.     

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.     

Diagnosis of neuroblastoma by electron microscopy of bone marrow aspirates.

Three patients are reported in whom the diagnosis of neuroblastoma was made following electron microscopy of a bone marrow aspirate. The ultrastructure of neuroblastoma cells is distinctive, and they can be distinguished by electron microscopy from cells of the other tumors with which neuroblastoma is often confused by light microscopy. The rapidity with which the diagnosis can be obtained through use of this procedure argues for its adoption in any patient in whom the diagnosis is suspected and who has tumor cells in bone marrow. Early initiation of appropriate therapy is made possible, and elaborate diagnostic procedures may be avoided.