Inflammatory Myofibroblastic Tumor of the Kidney in a Child: Report of a Case

Inflammatory myofibroblastic tumor (IMT), also known as inflammatory pseudotumor, is an uncommon benign tumor that has been reported in various locations throughout the body, but rarely in the kidney. To our knowledge, only 22 cases of renal IMT have been described in the literature. Renal IMT in children is difficult to differentiate from a malignancy such as Wilms' tumor. We report a case of a 9-year old girl with a left renal mass mimicking malignancy on preoperative diagnostic images, which was pathologically revealed to be an IMT. We review the literature and discuss the pathophysiology and clinical features of this unusual tumor.     

An Endobronchial Inflammatory Myofibroblastic Tumor Treated by Modified Left One-stoma-type Carinoplasty

Endobronchial inflammatory myofibroblastic tumor is a rare primary lung disease. A 39-year-old woman with dyspnea and a productive cough underwent complete surgical resection of a small-sized inflammatory myofibroblastic tumor that invaded the left main bronchus and the carina with lung-saving modified left one-stoma-type carinoplasty. We report this case with a review of literature.     

Inflammatory myofibroblastic tumor of the lung: a benign lesion with aggressive behavior

Inflammatory myofibroblastic tumor is a rare solid tumor that most often affects children and young adults. They present as myofibroblastic cell proliferations accompanied by inflammatory cells made up mostly of plasma cells. Although benign, the tumor may be very aggressive locally. In this report we describe a 22-year-old woman with primary invasive myofibroblastic tumor of the left lower lobe leading to a left pneumonectomy.     

Inflammatory myofibroblastic tumor of renal pelvis presenting with prolonged fever and abdominal pain in children: report of 1 case and review of literature

Inflammatory myofibroblastic tumor is a reactive proliferative lesion that occurs extremely rarely in the renal pelvis, especially during childhood. We report 1 such case that occurred in a child who presented with prolonged fever and abdominal pain. Ultrasonography and abdominal computed tomography revealed a left renal pelvic mass. She received conservative surgical treatment. The diagnosis was confirmed by pathological and immunohistochemical studies. Distinguishing inflammatory myofibroblastic tumor from other malignant renal pelvic tumors in children is essential to preventing unnecessary nephrectomy.     

Inflammatory myofibroblastic tumors of the kidney: a clinicopathologic and immunohistochemical study of 12 cases

Inflammatory myofibroblastic tumor is a rare entity composed of spindle cells admixed with variable amounts of extracellular collagen, lymphocytes, and plasma cells. In the genitourinary tract, inflammatory myofibroblastic tumor most commonly occurs in the bladder. Isolated case studies of inflammatory myofibroblastic tumor of the kidney, renal pelvis, and ureter have been previously reported. Our series includes 12 cases of inflammatory myofibroblastic tumor occurring in the renal pelvis (six cases), renal parenchyma (four cases), and immediate perirenal soft tissue (two cases). Clinical presentation included flank pain (two patients), painless gross hematuria (one patient), and ureteropelvic junction stenosis with hydronephrosis (one patient). The remaining eight patients were asymptomatic. All patients underwent nephrectomy. The tumors were characterized by firm white tissue or had a myxoid "gelatinous" appearance. Three histologic patterns were identified in the tumors, including a myxoid vascular pattern, a compact spindle cell pattern, and a hypocellular fibrous pattern. Immunohistochemical and electron microscopic studies supported a myofibroblastic proliferation. All cases were negative for anaplastic lymphoma kinase. Follow-up was available in eight cases and ranged from 1 to 17 years with no evidence of recurrence. Based on this series, renal inflammatory myofibroblastic tumor is a proliferative lesion of myofibroblasts of uncertain pathogenesis with no identified potential for recurrence or metastases.     

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。     

Inflammatory pseudotumor of the kidney: report of a case

We report a case of inflammatory pseudotumor of the kidney. A 73-year-old man presenting with general malaise and minimal grade fever visited a medical department in our hospital. Computerized tomography incidentally revealed a tumor, 3.5 cm in diameter, in the lower pole of the left kidney, and he was referred to our outpatient department. Selective left renal arteriography disclosed an avascular mass on the affected site. Left radical nephrectomy was performed under the diagnosis of avascular renal cell carcinoma. Histological examination demonstrated a tumor composed of spindle-shaped fibroblastic cells infiltrated by variable numbers of plasma cells, small lymphocytes and histiocytes. The pathologic diagnosis was renal inflammatory pseudotumor. Fourteen previously reported cases of this tumor have reviewed in the literature.     

Complete surgical resection of inflammatory myofibroblastic tumor with carinal reconstruction in a 4-year-old boy

Inflammatory myofibroblastic tumor of the carina and the main bronchus is a rare tumor. The authors report here on a case of a 4-year-old boy with an inflammatory myofibroblastic tumor at the carina and extending to the left main bronchus. He presented with fever and a cough of 2 months' duration. Preoperative assessment of the tumor revealed an intraluminal round mass arising from the carina and extending into the left main bronchus, and this caused near-total obstruction of the left main bronchus and the subsequent total collapse of the entire left lung. The complete resection of the mass with carinal reconstruction was successful. The tumor was a round mass measuring 1.5 × 1 cm. It had characteristic features of an inflammatory myofibroblastic tumor, namely, the proliferation of spindle-shaped fibroblasts and myofibroblasts.     

Pulmonary inflammatory myofibroblastic tumor invading the left atrium

Inflammatory myofibroblastic tumor is a rare solid tumor that most often affects children and young adults. Although benign, the tumor may be very aggressive locally. We describe a 9-year-old boy with primary inflammatory myofibroblastic tumor of the left upper lobe involving the left atrium.     

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.     

Myofibroblastic tumor causing severe neonatal distress. Successful surgical resection after embolization

This report describes a case of a term male 3.1 kg, normal delivery, 38 weeks of gestation with a record of hydramnios by prenatal sonography. He had fetal acute suffering and respiratory distress. The first radiographic study showed a mass filling the whole left thorax cage causing erosion of the inferior edge of the third rib. The mediastinum was displaced to the right. Computed tomography scan confirmed a homogeneous tumor that filled the left thorax and displaced the mediastinum to the right without invasion. Surgical biopsy informed of a highly vascularized mesenchymal tumor. The tumor was embolized with Ivalon microparticles obtaining a nearly avascular mass. Complete surgical excision was made, including the whole mass and costal segments. Microscopically, it was an inflammatory myofibroblastic tumor. It was composed mainly of spindle-shaped cells without malignant features. On immunohistochemistry, the tumor showed positive staining for vimentin, whereas antidesmin antibodies and S-100 protein were negative. The aim of this article is to present an extremely uncommon case of neonatal distress caused by an intrathoracic, extrapulmonary myofibroblastic tumor. Complete surgical resection was possible after embolization.     

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.     

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.     

Primary carcinoma in situ of the upper urinary tract: a case report]

A case of primary carcinoma in situ of the upper urinary tract in a 72-year-old woman is reported. The patient who complained of left lower abdominal pain was referred for a suspicion of left ureteral stone. An excretory pyelogram showed mild left ureteral stricuture at the level of L3, but a stone was not detected in the ureter at the same level. Cytology of voided urine was positive for malignant cells several times. Cystoscopic examination revealed no abnormality in the bladder. Retrograde left pyelogram demonstrated the ureteral stricture and no lesions either of stone or tumor in the ureter. However malignant cells were detected cytologically in the left ureteral catheteral urine. Left total nephroureterectomy with the bladder cuff was carried out under the preoperative diagnosis of carcinoma in situ of the upper urinary tract. Macroscopically, the wall of the ureter at the stenotic level had induration without apparent tumor mass. The pathological diagnosis was transitional cell carcinoma in situ from the renal pelvis to the mid-ureter. The primary carcinoma in situ of the upper urinary tract is rare. To our knowledge, this case is the 26th case reported in the Japanese literature.     

Inflammatory myofibroblastic tumor of the ulnar nerve. Case report and review of the literature

Inflammatory myofibroblastic tumors with involvement of cranial and peripheral nerves are exceedingly rare. The authors present the case of a 67-year-old man with an inflammatory myofibroblastic tumor of the left ulnar nerve, which was identified intraoperatively and mimicked a malignant neoplastic lesion. Histopathological examination revealed loosely structured fibrous tissue and collagen deposits intermingled with patchy infiltrates of lymphocytes, plasma cells, and histiocytes penetrating the endo- and epineurium of the affected nerve fascicles. There was strong expression of vimentin and actin in spindle cells throughout the lesion. The histiocytes were CD68- and major histocompatibility complex class II-positive, but lacked CD1a expression. A review of the literature revealed nine histopathologically confirmed cases of inflammatory myofibroblastic tumors involving peripheral or cranial nerves in which slight differences in histopathological features and surgical management were found, which are discussed here.     

Inflammatory Myofibroblastic Tumor of the Ileocecal Mesentery Mimicking Abdominal Lymphoma in Childhood: Report of Two Cases

An inflammatory myofibroblastic tumor is an uncommon benign tumor located in various organs that can be misdiagnosed as a malignant neoplasm. We herein present two patients with ileocecal inflammatory myofibroblastic tumors. An abdominal mass was detected in a 13-year-old girl and a 15-year-old boy who presented with paleness, fatigue, intermittent fever, and night sweating. The radiological findings confirmed a mass originating from the ileocecal region. The presumptive diagnosis was Burkitt’s lymphoma. The histopathological diagnosis was inflammatory myofibroblastic tumor. After a surgical resection, all systemic symptoms rapidly resolved. Inflammatory myofibroblastic tumor is a rare pseudosarcomatous clinical and pathological entity. Although this tumor is more commonly reported in the lung, it can be detected in extrapulmonary sites, including the mesentery. Because the choice of treatment for this tumor is conservative surgery, an accurate preoperative analysis is important to avoid any unnecessary aggressive surgical intervention or other therapeutic approaches.     

直肠及肛周炎性肌纤维母细胞瘤三例

目的 探讨直肠及肛周炎性肌纤维母细胞瘤的临床特点及其疗效.方法 回顾性分析2005年1月至2011年6月术后病理证实为直肠或肛周炎性肌纤维母细胞瘤3例患者的临床资料.结果 炎性肌纤维母细胞瘤局部表现为浸润性生长,MRI或CT常表现为富血管实性肿块,影像学难以同直肠癌或肉瘤、血管瘤鉴别,术前肠镜活检或局部针吸活检常难以明确肿瘤性质,需手术切除后整体送检,并行免疫组化确诊.3例患者均行保肛手术,1例患者术后16个月出现复发,再次行根治性手术,随访至今.3例患者已分别存活67、55和35个月.结论 直肠及肛周炎性肌纤维母细胞瘤术前不易确诊,病理诊断主要依赖免疫组化.局部完整切除可以在保留肛门的同时获得肿瘤的根治,局部复发也常有再次手术机会.     

Is calcifying fibrous pseudotumor a late sclerosing stage of inflammatory myofibroblastic tumor?

Calcifying fibrous pseudotumor is a recently described distinctive lesion, characterized by the presence of abundant hyalinized collagen with psammomatous or dystrophic calcifications and a lymphoplasmacytic infiltrate. The cause and pathogenesis are unclear, but a possible relationship with other pseudotumors, like nodular fasciitis or inflammatory myofibroblastic tumor, has been proposed by some authors. However, cases with overlapping histologic features have not been reported. A 17-year-old girl with multiple peritoneal calcifying fibrous pseudotumors and inflammatory myofibroblastic tumors (inflammatory pseudotumors) is described. Some multinodular lesions showed calcifying fibrous pseudotumors next to inflammatory myofibroblastic tumors. Transitional stages between calcifying fibrous pseudotumor and inflammatory myofibroblastic tumor were also present. This case clearly illustrates a histogenetic relationship between calcifying fibrous pseudotumor and inflammatory myofibroblastic tumor, and it suggests that calcifying fibrous pseudotumor is a late sclerosing stage of inflammatory myofibroblastic tumor, at least in some cases.     

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.