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 (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.     

Inflammatory myofibroblastic tumor of the epididymis: case report and review of the literature

The inflammatory myofibroblastic tumor (IMT) is a rare benign tumor, known by several names and found in several locations throughout the body, including the lungs and genitourinary system (mostly the bladder). We report a case of a paratesticular IMT in a 17-year-old boy. Although the initial clinical diagnosis was rhabdomyosarcoma, the histopathologic examination revealed it to be an IMT. The preoperative diagnosis of a scrotal mass should differentiate between benign and malignant tumors. A definitive diagnosis of a scrotal mass should be established by surgical exploration and resection. It is important to counsel patients with scrotal masses regarding the possibility of orchiectomy, even in the case of a benign tumor such as IMT.     

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.     

膀胱炎性肌纤维母细胞瘤2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、组织生化特性和诊治方法及预后。方法：报告2例肉眼血尿伴重度贫血的膀胱炎性肌纤维母细胞瘤患者的诊治资料,并结合文献进行探讨。结果：膀胱炎性肌纤维母细胞瘤以肉眼血尿和尿痛为最常见症状,肿瘤好发于膀胱底部、顶壁、侧壁;组织学由具有平滑肌细胞和纤维母细胞特征的梭形细胞、大量慢性炎性细胞及血管样黏液构成。1例随访3年,1例随访6个月,均无瘤存活。结论：膀胱炎性肌纤维母细胞瘤为低度恶性肿瘤,罕见。确诊依据病理学检查,应与平滑肌肉瘤、横纹肌肉瘤、梭形细胞癌等鉴别;治疗原则以切除肿瘤、保留膀胱功能为主,TUPKBT、TURBT或膀胱部分切除术,术后辅以化疗药物膀胱灌注,效果佳。     

Mesenteric myofibroblastic tumor: NSAID therapy after incomplete resection

Myofibroblastic tumors are inflammatory tumors that arise in viscera and soft tissue; their etiopathology is poorly understood. They are capable of infiltration of adjacent organs, local recurrence after surgical resection, and even of distant metastasis. These characteristics result in persistent debate as to the nature of these lesions - whether they are inflammatory or neoplastic, benign or malignant lesions? Diagnosis is almost always made based on histopathological findings. Traditional management is complete surgical excision, but this may be difficult or impossible when the lesion develops in proximity to vital structures. We report the case of a 59-year-old man who was treated in our institution for mesenteric myofibroblastic tumor. Complete resection was not possible due to local infiltration of the mesentery. Recurrence was noted 3?months after surgery; treatment with steroidal and then non-steroidal anti-inflammatory medications (NSAIDs) resulted in clinical and radiologic regression of the tumor.     

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.     

Testicular tumors: wide spectrum in our short casuistics

Testicular tumors occur in 0.5 to 2 per 100,000 children. They are 1-2% of all solid tumors before puberty. The clinical history, testicular and abdominal ultrasonography, alpha-fetoprotein and human chorionic gonadotropin, estrogens and androgen levels, FSH and LH determine the diagnosis. The pathology determines the specific cell. We report seven cases, three germ cell tumors: a Yolk sac tumor in a child of 18 months and two mature teratomas in children between 2 and 11 years presenting as a painless testicular mass without other symptoms. Three tumors estrumales: one derived from Leydig cells and two of the granulosa cells, a palpable testicular mass was added precocious puberty in stage II-III of Tanner in the first, second gynecomastia in Tanner stage III and the third only with testicular mass. The seventh case, Lipoma para-testicular mass palpable. The treatment was radical orchiectomy in five cases. Testis-sparing surgery in Leydig cell tumor and resection of the paratesticular mass was performed through scrotal. The Yolk sac tumor requiring chemotherapy with good outcome. Retroperitoneal lymph node dissection is not recommended in prepubertal. Historically prepubertal testicular tumors have been treated in adults. Recent testicular preservation algorithms optimize and minimize the morbidity of adjuvant therapies. Many are benign and can be treated with preservation of the testis. Localized malignant tumors can be treated by orchiectomy.     

Nephrogenic adenoma: A rare bladder tumor in children.

OBJECTIVE: Nephrogenic adenomas of the urinary bladder are rare benign tumors in children. The purpose of our study was to obtain information about the sex distribution, presenting symptoms, intravesical locations, therapy and recurrence rates in pediatric nephrogenic adenomas. PATIENTS AND METHODS: The records of 3 children with nephrogenic adenoma of the urinary bladder diagnosed between 1990 and 1997 were reviewed to evaluate the initial symptomatology, diagnostic examinations and findings, therapeutic procedures and clinical outcome and recurrence rates. Furthermore our data are compared to the findings of all children reported in the literature. RESULTS: Including the 3 cases reported by us, the data on 18 children with nephrogenic adenoma of the bladder could be analyzed. There was a significant predominance of girls compared to boys (5:1); the medical history in all cases was remarkable for previous bladder surgery 3 months to 7 years prior to tumor diagnosis. Most children presented with unspecific symptoms of gross hematuria, dysuria and bladder instability and in all cases the final diagnosis was established after cystoscopy and histopathologic review of a tumor biopsy specimen. Therapy consisted of transurethral resection in 15 cases, partial cystectomy and open excision in 2 and 1 case, respectively. Tumor recurrence developed in 80% of the children with a latency period of 4 years. CONCLUSIONS: Nephrogenic adenomas represent an epithelial response of the urothelium to chronic inflammation or previous trauma resulting in urothelial metaplasia and the development of papillary lesions. Current treatment of choice consists of transurethral resection and fulguration of the base of the tumor and periodic cystoscopy.     

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.     

Eosinophilic cystitis associated with urethral stricture disease from pelvic trauma. Case report and literature review

We report a case of eosinophilic cystitis (EC) in a 65-year-old man with urethral stricture disease from blunt pelvic traumatic event. EC is a rare condition characterized by eosinophilic infiltration of the bladder wall, that usually presents with irritative voiding symptoms, suprapubic pain and hematuria. Etiology is still not clear although a review of the literature suggests that pathogenetic mechanisms probably engage an altered immune response in the bladder, with the inflammatory reaction caused by factors such as exogenous allergens and previous bladder injury or surgery to the bladder or the prostate. The diagnosis of EC has to be confirmed by biopsy, since in some cases it may manifest as other inflammatory and malignant bladder disorders. A conservative medical management is indicated initially, since this disease may be self-limited, with a benign course especially in children and young patients. In adults EC is more often a chronic recurrent condition that requires close follow-up since it may lead to serious progressive bladder and/or upper urinary tract disease. More invasive therapies (including transurethral resection, partial or total cystectomy) may also be required when conservative therapy fails.     

Regression of inflammatory pseudotumor of the bladder in a child with medical management

Inflammatory pseudotumor of the bladder is a benign proliferative lesion of the submucosal stroma that cannot be distinguished from malignant tumors of the bladder either endoscopically or radiographically. Although benign, the proliferative nature of the inflammatory pseudotumor histopathology has led others to recommend open surgical removal or complete transurethral resection for definitive treatment. A limited number of case reports have described inflammatory pseudotumor of the bladder in either adults or children. This is a case of biopsy-proven inflammatory pseudotumor in the bladder of a child that regressed after medical management alone.     

An intrasylvian "fibroma" in a child with cystic fibrosis: case report

OBJECTIVE AND IMPORTANCE: Intracranial fibrous tumors are uncommon during childhood. An unusual case of benign intrasylvian "fibroma" that has remained clinically and radiographically stable more than 3 years after a subtotal resection is described. CLINICAL PRESENTATION: A 9-year-old girl with cystic fibrosis presented with new-onset focal seizures referable to a large calcified left sylvian fissure mass. INTERVENTION: An open biopsy with subtotal resection of the lesion revealed a benign process characterized by exuberant fibrocollagenous tissue intermeshed with chronic inflammatory cells and foreign body giant cells, encompassing islands of gliotic brain tissue. Immunohistochemical analysis showed staining for epithelial membrane antigen and reticulin within some of the spindle cells, although the majority were nonreactive. The majority of tumor cells exhibited staining for laminin; CD34 staining was absent. Ultrastructural studies were also suggestive of a fibroblastic rather than a meningothelial origin of the lesion, with elongated cells separated by abundant extracellular collagen. Although dense adherence of the mass to the pial surface and the middle cerebral artery vessels precluded a complete resection, the patient remains seizure-free without anticonvulsant therapy more than 3 years postoperatively with no evidence of growth of the lesion. CONCLUSION: The lesion in this patient bears morphological similarity to a rare group of tumors referred to as "intracerebral fibromas," although a variety of other rare mesenchymal neoplasms were also considered within the differential diagnosis. However, the absence of any definite neoplastic features, the finding of chronic inflammatory changes, and the lack of growth of the residual tumor during an extended follow-up interval indicate that the mass may represent either an extremely indolent neoplasm or a nonneoplastic process. The differential diagnosis of intracranial fibrous tumors is contrasted with that of the reported case.     

Congenital reactive myofibroblastic tumor of the petrous bone: case report

OBJECTIVE AND IMPORTANCE: Myofibroblastic tumors are members of a diverse spectrum of neoplastic and quasineoplastic lesions that occur most commonly during childhood and typically involve soft tissues. We present a case of a congenital reactive myofibroblastic tumor of the petrous bone (i.e., cranial fasciitis) that was successfully treated with surgical excision. CLINICAL PRESENTATION: A newborn girl with congenital right facial palsy and deafness was noted during imaging evaluation to have a large enhancing mass that was destroying the right petrous bone and extending into the posterior and middle cranial fossae. INTERVENTION: After embolization, an open biopsy was performed, which revealed a moderately cellular, spindle cell neoplasm without mitosis or necrosis, with scattered lymphocytes, eosinophils, and multinucleated giant cells. The spindle cells demonstrated strong immunoreactivity for vimentin, muscle-specific actin, and alpha-smooth muscle actin, with prominent reticulin staining between individual cells. Staining for CD68, a histiocyte marker, was positive within the multinucleated giant cells and many of the spindle cells; CD34, S-100, and desmin staining was absent. On the basis of these findings, the lesion was classified as a reactive myofibroblastic tumor, consistent with a cranial variant of nodular fasciitis. Because of the large size and significant mass effect of the tumor, a resection was performed several days later, using a combined supra- and infratentorial approach. Dense adherence of the mass to the walls of the sigmoid sinus and the carotid artery precluded complete resection without sacrifice of these vessels, which was not performed because of the known potential of these tumors to remain stable or regress after extensive subtotal resection. The presumed residual tumor subsequently regressed, and the patient has exhibited no detectable residual disease in 2 years of follow-up monitoring. CONCLUSION: Reactive myofibroblastic tumors of the calvarium are uncommon lesions that superficially resemble sarcomas. Recognition of this diagnostic entity is important, to avoid unnecessary treatment with intensive adjuvant therapy. Although the management of these tumors relies predominantly on surgical resection, surgical decision-making should take into account the fact that small areas of residual disease can regress spontaneously.     

Bladder tumors in children

Our experience with bladder tumors in children is presented and the pertinent literature reviewed. Nonepithelial tumors are more common than epithelial ones, and the most common symptom is bladder outlet obstruction. Papilloma, the common epithelial tumor, usually presents with hematuria and is successfully treated by transurethral methods. Treatment of bladder neurofibroma will depend on the site and size of the tumor and on resultant symptoms and obstruction; small or moderate-size nodules may be amenable to transurethral resection, local excision, or segmental resection, but for diffuse involvement of the bladder, total cystectomy may be necessary. Hemangiomas are the most common benign connective tissue tumor, and their common location in the upper part of the bladder usually permits successful treatment by open excision or segmental resection. Rhabdomyosarcoma is the most common bladder tumor in children. On present evidence radical cystectomy is the treatment of choice. The contributions of adjunct radio- and chemotherapy are not well defined, but such treatment is recommended since it has contributed to the control of tumor in nonresectable cases. Four of 8 patients in this series are alive and well five and one-half to seventeen years, 3 having had radical cystectomy (with radiotherapy in 2 and chemotherapy in 1) and 1 segmental resection with radio- and chemotherapy. Leiomyosarcoma of the bladder has been controlled by segmental resection, but it is logical to believe that radical cystectomy would extend the possibilities of surgical control in some cases; adjunct irradiation and chemotherapy may deserve a place in treatment as well.     

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??Hepatic inflammatory myofibroblastic tumor WU Gang, CHEN Xu-chun. Department of Hepatobiliary, Faculty Working Office of General Surgery, the First Hospital of China Medical University, Shenyang 110001, China
Corresponding author: WU Gang, E-mail: WGZWL@hotmail.com
Abstract Hepatic inflammatory myofibroblastic tumor (HIMT) is a relatively rare benign liver tumors characterized by chronic infiltration of inflammatory cells and areas of fibrosis. HIMTs are difficult to diagnose because of the absence of specific symptoms or of characteristic hematological or radiological findings. Reports of HIMTs have a growing trend with recent advances in imaging technology. But there are still some difficulties in the diagnosis. Most cases can be diagnosed only through pathology after operation.     

Successful preoperative treatment of pediatric bladder inflammatory myofibroblastic tumor with anti-inflammatory therapy

Inflammatory myofibroblastic tumors are rare, locally invasive tumors that can involve the genitourinary tract. Therapy has consisted primarily of surgery, and anecdotal experience using both chemotherapy and radiotherapy, which have yielded unimpressive results. We report the first case of a bladder inflammatory myofibroblastic tumor that responded to an anti-inflammatory regimen (prednisone and Cox-2 inhibitor) before surgical extirpation.     

A case of neuropathic bladder with inflammatory pseudotumor of the bladder

A 36-year-old woman was referred with urinary incontinence and recurrent episodes of pyelonephritis. Two years prior to her visit, she underwent transurethral resection of a bladder tumor, 5 cm in diameter. Total cystectomy was suggested, as the initial diagnosis was sarcoma. Close re-evaluation of the pathological specimen lead to the final diagnosis of a benign inflammatory pseudotumor of the bladder as the tumor consisted of smooth muscle cells with white blood cell infiltration but without mitotic figures. The tumor disappeared during the follow up period. A cystography revealed bilateral vesicoureteral reflux with marked trabeculated bladder. A cystometry showed loss of bladder sensation and a low compliance bladder without detrusor contraction. Neurological examination and a magnetic resonance imaging of the spinal cord failed to prove the presence of definite neurological abnormalities. She was finally diagnosed with neuropathic bladder of unknown origin. In this case, she had been suffering from recurrent cystitis about 6 years before the resection of bladder tumor and it was suggested that the occurrence of the inflammatory pseudotumor of bladder would be related with chronic urinary tract infection due to neuropathic bladder. Urinary incontinence and urinary tract infection were controlled successfully with clean intermittent self-catheterization and adequate administration of antimicrobial drugs. Vesicoureteral reflux was treated with injection of GAX collagen into the ureteral orifices. No tumor recurrence has been found up to the present time, 5 years after the resection of bladder tumor.     

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 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.