Treatment Of Inflammatory Myofibroblastic Tumor Research Articles

Inflammatory myofibroblastic disease of right petrous apex: A rare case with review of literature.

Inflammatory myofibroblastic tumor (IMFT) is a rare tumor of unknown etiology. It can involve any part of the body. The IMFT involving the base of skull is rare with only 36 cases reported in the literature. We report a rare case of IMFT of temporal bone with review of literature. A 42 year old male presented with complaints of headache and double vision and MRI brain showed lesion in the right petrous apex region suggestive of a neurogenic mass. He had excision of lesion and histopathology was suggestive of IMFT with IgG4 and ALK positive. He had complete clinical response but a month later he presented with right eyelid ptosis and decreased rotation of eye medially with recurrent lesion on MRI. Patient received radiation by SRT technique and then started on Ceretinib with partial response. The IMFT is rare tumor of unknown etiology and tumors of temporal bone are more aggressive. It is benign but locally invasive tumor. Treatment of IMFT is controversial. Extensive surgery with complete excision has about 80% response rates and with intracranial extension, adjuvant radiation is need. In head and neck IMFT response rates are lower (30 to 40%). Monoclonal antibodies and steroids are used in IMFT at recurrence. In advanced or metastatic ALK positive tumors, Crizotinib is used with a response rate of 50%. Radiotherapy (25 to 30 Gy) induces remission and helps to taper the steroids. Temporal bone IMFT is a rare tumor with multimodality approach and variable response to treatment.

Lorlatinib for the Treatment of Inflammatory Myofibroblastic Tumor after Allogeneic Hematopoietic Stem Cell Transplantation: A Case Report.

Inflammatory myofibroblastic tumors (IMTs) are rare sarcomas composed of myofibroblastic and fibroblastic cells, accompanied by inflammatory cell infiltration. Many IMTs exhibit clonal rearrangement of anaplastic lymphoma kinase (ALK). We herein report a 56-year-old woman with uterine IMT harboring a thrombospondin-1::ALK fusion that developed after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Laboratory data before systemic therapy indicated increased interleukin-6 and severe leukocytosis. The patient was treated with lorlatinib; however, the response duration was approximately two months. Similar case reports need to be compiled and evaluated to elucidate the efficacy of lorlatinib in post-allo-HSCT IMT with ALK rearrangement.

Inflammatory myofibroblastic tumors: recent progress and future of targeted therapy.

An inflammatory myofibroblastic tumor is a rare component of bone and soft-tissue sarcomas that has distinct pathological features as a lymphoplasmacytic inflammatory infiltrate. As is the case for other non-small round cell sarcomas, surgical resection remains the standard treatment strategy for inflammatory myofibroblastic tumors, but recurrence is possible. Concerning systemic therapy, the available data for conventional chemotherapy (such as those of doxorubicin-based regimens) are limited, and case reports of anti-inflammatory inflammatory myofibroblastic tumor treatments describe some degree of symptom relief and efficacy against tumor progression. However, as more information about cancer genomics accumulates, the potential for molecularly targeted therapies for inflammatory myofibroblastic tumors has become more promising. Approximately half of inflammatory myofibroblastic tumors harbor anaplastic lymphoma kinase (ALK) fusion genes, and the other half could have potentially targetable fusion genes or mutations such as ROS1, NTRK and RET; case reports demonstrating the clinical efficacy of treatments targeted to inflammatory myofibroblastic tumor have been published, as have several prospective clinical trials. Few drugs are approved for the treatment of inflammatory myofibroblastic tumor, and most of them were approved for tumor-agnostic indications. Drugs that could be used for pediatric indications and dosing in inflammatory myofibroblastic tumor have also not been established. To provide effective targeted therapy for rare diseases such as inflammatory myofibroblastic tumor, it is necessary to obtain clinical evidence by designing and performing clinical trials and to find a path toward regulatory approval.

Clinical characteristics and outcomes of 17 cases of inflammatory myofibroblastic tumor at a University Hospital in China.

The aim of the present study was to explore the clinical and pathological characteristics, diagnosis, and treatment of inflammatory myofibroblastic tumor (IMT). A total of 17 patients with IMT diagnosed between July 2010 and February 2020 were included in the present study, and the clinical characteristics, pathological features, treatment and prognosis were analyzed retrospectively. The cohort consisted of 17 participants, including 12 men and 5 women, with a mean age of 34.76 years. The most common locations of tumors were the bronchi and the lungs (9 cases, including 1 case involving the mediastinum), followed by the colon and bladder (2 cases each), and the omentum majus, mesocolon, stomach and peritoneal cavity (1 case each). Immunohistochemical staining demonstrated that the tumor cells exhibited positive staining for anaplastic lymphoma kinase p80 (13/17), smooth muscle actin (12/17), cytokeratin pan (6/17), vimentin (5/17) and desmin (4/17). The follow-up time was 18–114 months. A patient with epithelial inflammatory myofibroblast sarcoma (EIMS) succumbed to the disease, 1 case was lost to follow-up, 2 cases relapsed and the other 13 cases were considered cured. IMTs may be malignant or low-grade. EIMS is a rare and invasive variant of IMT. The clinical and imaging manifestations are often unique and vary among individuals. Once confirmed by pathology, radical surgery should be the first choice of treatment.

Inflammatory myofibroblastic tumors of the urinary tract: a clinical diagnosis and treatment review in pediatric

Objective To improve the diagnosis and treatment of inflammatory myofibroblastic tumor (IMT) of the urinary tract in pediatric. Methods The retrospective study of 12 IMT was based on information retrieved from Beijing Children′s Hospital from January 2006 to July 2015. The literatures of urinary IMT were reviewed. There were 12 cases of urinary IMT, with 8 cases in bladder, 2 in kidney, 1 in ureter and 1 in prostate. Mean age at surgery was 6.4 years old (range 2months-13 years), 6 cases males and 6 females. Tumor resection were performed in 11 patients, biopsy was performed only in 1 patient. Results HE staining revealed diffuse appearing spindle myofibroblastic cells admixed with inflammatory cells. Immunohistochemistry showed positive ration for following markers as ALK (8/12), CK18 (6/12), Desmin (7/12), SMA (8/12), Actin (1/2), Vimentin (9/12). Negative staining were seen for Myoglobin, S-100 and Ki-67 < 20%.Patients were followed up in 10 cases, lost to follow-up in 2; the mean follow-up time was 14.4 months (range 3-31 months). All patients recovered well without relapse or metastasis. Conclusions Inflammatory myofibroblastic tumors of the urinary tract in pediatric were rare, without specific characteristic in clinical features and imaging. The main treatment of IMT is complete surgical excision. Key words: Inflammatory myofibroblastic tumor; Urinary System; Anaplastic lymphoma kinase(ALK)

Treatment of Inflammatory Myofibroblastic Tumor of the Chest: The Extent of Resection

Owing to the rarity of inflammatory myofibroblastic tumor, its pathology and clinical course are poorly understood. We performed a retrospective chart review of patients diagnosed with inflammatory myofibroblastic tumor in the chest who underwent surgical resection. From 1995 to 2006, 15 patients (10 males and 5 females) underwent surgical procedures owing to inflammatory myofibroblastic tumors. The mean age of these patients was 31.3 years (range, 7 months to 61 years). Among them, 13 patients (86.7%) presented with respiratory symptoms such as cough, dyspnea, and hemoptysis. Seven patients presented with the tumor located in the lung parenchyma, 4 in the trachea, 2 in the main bronchus, 1 in the segmental bronchus, and 1 in the chest wall. The diagnosis was confirmed before surgery for only 1 patient (6.3%). The types of operations performed included lobectomies for 4 patients, wedge resections using video-assisted thoracic surgery for 4 patients, tracheal/bronchial resections with end-to-end anastomoses for 6 patients, and chest wall resection for 1 patient. Only 2 patients received adjuvant radiation therapy. We followed up with all patients postoperatively for a mean of 33.3 months (range, 1.2 months to 8.4 years). Fourteen patients were free of local recurrence or distant metastasis during the follow-up period. Inflammatory myofibroblastic tumor usually requires surgical resection for both proper diagnosis and adequate treatment. Complete resection and achieving negative margins leads to excellent outcome.

Diagnosis and treatment of inflammatory myofibroblastic tumor with malignant transformation

患者女,14岁.因腹部轻度膨隆和低热于2004年7月入院.CT和B超发现腹部有一个直径5 cm左右的肿块,实验室检查示轻度贫血外,余无异常.术中见1个边界较清楚但无包膜的肿块,约5.0 cm×4.0 cm×3.5 cm,完整切除肿块.但在腹腔、大网膜、肠管浆膜层分布几十枚直径0.5～1.5 cm的灰白色结节,无法切除。