Extrapulmonary inflammatory pseudotumor in children

Inflammatory pseudotumor (IPT), an unusual etiology of an intraabdominal mass, is typically heralded by fever, anemia, thrombocytosis, and systemic symptoms that bear a resemblance to an inflammatory diathesis. Two cases of IPT seen within a 1-year period are presented: an adrenal IPT simulating a neuroblastoma was seen in an 8-year-old girl, and IPT in the jejunal mesentery mimicked a sarcoma in a 9-year-old boy. Although IPTs are histologically benign, they can be locally aggressive and often mimic a malignancy both radiographically and grossly. This diagnosis is rarely considered in the differential diagnosis of an abdominal mass.     

Gastric inflammatory myofibroblastic proliferation in children

Gastric inflammatory myofibroblastic proliferation (IMP) is an extremely rare entity in children, which to our knowledge has only been mentioned in case reports. We describe the ninth pediatric case and review the literature concerning the etiology, clinical and laboratory features, pathology, treatment, and outcome. There has been a predominance in preschool females. Abdominal pain, upper gastrointestinal hemorrhage, and an abdominal mass, either isolated or associated, have been the main clinical features. Iron-deficiency anemia has been a constant finding. Lesions are elevated and involve the full thickness of the gastric wall, usually with ulceration of the luminal surface; extragastric extension suggesting malignancy is frequent. Diagnosis is made by histology after surgical excision. There was no mortality directly related to gastric IMP, and only one case recurred after surgical excision. The pathogenesis is controversial, but the finding of Helicobacter pylori in our case may indicate an inflammatory origin. Awareness of this benign lesion and its mimicry of malignancy is important so that inappropriately aggressive therapy can be avoided. Accepted: 16 May 1997     

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.     

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.     

Aggressive manifestations of inflammatory pulmonary pseudotumor in children

We present three cases that illustrate the locally invasive radiographic appearance that inflammatory pulmonary pseudotumor can assume. Awareness and inclusion of inflammatory pseudotumor in the differential diagnosis of aggressive pleuropulmonary and mediastinal processes may have critical treatment implications. Received: 5 February 1998 Accepted: 29 June 1998     

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。     

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。     

Acute orbital myositis and idiopathic inflammatory pseudotumor in children: three cases]

Orbital pseudo-tumors account for approximately 8% of the orbital tumors. They are scarcely described in children and raise diagnostic dilemas, especially when they present as an isolated extra-ocular muscle swelling called idiopathic orbital myositis (IOM). CASE REPORTS: A diagnosis of inflammatory orbital pseudotumors (IOPT) was made in three children aged 7, 13 and 14 years from clinical and CT scan and/or MRI findings associated with histological data in two of them. Two of these IOPT presented as IOM. Analysis was made in a effort to eliminate intra-orbital tumors, especially rhabdomyosarcoma. CONCLUSION: The abrupt onset of the orbital signs, often related in the literature, was not a specific diagnostic criterion regarding to embryonic rhabdomyosarcoma. The inconstant presence of inflammatory signs and the absence of local osseous defect could be more discriminating. Although diagnostic procedure, including histological documentation, was difficult, even dangerous, surgical biopsy should be recommended. Eventually, considering the mandatory multidisciplinary care, steroid therapy response should be evaluated as a diagnostic test.     

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.     

Paraneoplastic opsoclonus myoclonus syndrome associated with inflammatory myofibroblastic tumor in a pediatric patient

Opsoclonus myoclonus syndrome (OMS) is a rare neurological syndrome caused by a paraneoplastic autoimmune process that affects children with neuroblastic tumors. Treatment includes corticosteroids, intravenous gamma globulin (IVIG), rituximab, and other immunosuppressive therapies. Here, we describe a patient diagnosed with OMS associated with a localized inflammatory myofibroblastic tumor. The patient has no evidence of tumor recurrence following surgical resection with 8‐month follow‐up. The neurologic symptoms resolved with corticosteroids and IVIG. This case demonstrates that in children, neoplasms other than neuroblastoma may be associated with this paraneoplastic syndrome, and highlights the importance of evaluating patients with OMS for underlying malignancies.     

ALK inhibition in two emblematic cases of pediatric inflammatory myofibroblastic tumor: Efficacy and side effects

There is an increasing interest for anaplastic lymphoma kinase (ALK) inhibitors in pediatric oncology for specific entities such as ALK‐driven inflammatory myofibroblastic tumor (IMT). IMT treatment can be challenging due to localization of the tumor and in rare cases of metastasis. When standard surgical treatment is not feasible, ALK inhibitors may play an important role, as recently reported for the first‐generation ALK inhibitors (crizotinib). However, data on the second‐generation ALK inhibitors are limited. We report two emblematic cases of IMT in pediatric patients, treated with the second‐generation ALK inhibitor ceritinib in the context of a clinical trial (NCT01742286).     

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.     

Complete resection of a huge hypervascular inflammatory myofibroblastic tumor in right hemithorax after embolization

Background

Inflammatory myofibroblastic tumor (IMT) is a rare and mostly benign tumor that has the possibility of malignant change.

Methods

Radiological findings revealed a huge mass that filled most of the right hemithorax of a 17-monthold female infant. Tumor extirpation was stopped due to massive bleeding and limited exposure of the tumor. Embolization was conducted to obstruct the arteries feeding the mass. Complete resection was performed.

Results

Histopathologic examination led to the diagnosis of IMT. Postoperative recovery was uneventful.

Conclusion

Hypervascularity of IMT should be considered. Preoperative embolization can be effective to reduce intraoperative blood loss and facilitate the surgical procedure.

     

Invasive inflammatory myofibroblastic tumor of the spleen treated with partial splenectomy in a child

Inflammatory myofibroblastic tumor (IMT) is a rare benign tumor that can be found in intra-abdominal organs such as the liver, intestine, extrahepatic bile ducts, and mesentery. The spleen is an extremely unusual location for an IMT. The authors report the case of a 14-year-old boy with invasive splenic IMT, present a review on the current literature about childhood splenic IMT, and emphasize the necessity of total excision of the tumor together with tumor-invaded surrounding tissues.     

Clinical and pathologic considerations in a case of inflammatory myofibroblastic tumor of the spleen

Inflammatory myofibroblastic tumors, also called inflammatory pseudotumors, are rare pseudosarcomatous proliferations. Their behavior is generally not aggressive, but in most instances they can simulate malignant neoplasms, from which they are impossible to distinguish before excision. One case of myofibroblastic tumor of the spleen in a 5-year-old boy is described: the patient was treated with a partial splenectomy, which was found to be the best approach for diagnosis and treatment. The purpose of this report is to call attention to this entity with peculiar histologic and immunohistochemical characteristics, which has to be included in the differential diagnosis of splenic lesions and may be excised by a nonaggressive procedure.