Case Of Inflammatory Myofibroblastic Tumor Research Articles

Locally Aggressive and Recurrent Facial Inflammatory Myofibroblastic Tumor in a Pediatric Patient

Inflammatory myofibroblastic tumor (IMT) is a rare tumor type with a prognosis ranging from benign to locally aggressive. Initially described as a reactive lesion most commonly of the lungs, cases of IMT have now been reported in rare instances in the head and neck, which may be more aggressive than other tumor locations. IMT frequently afflicts children and adolescents, but pediatric cases of IMT in the head and neck region are rare. This report serves to describe a rare presentation of a recurrent and locally aggressive facial IMT in a pediatric patient that required multiple surgical resections alongside medical management.

A case of inflammatory myofibroblastic tumor of small intestine, presented as intussusception

A case of inflammatory myofibroblastic tumor of small intestine, presented as intussusception

Inflammatory Myofibroblastic Tumor Masquerading as Congenital Pelvic-ureteric Junction Obstruction

ABSTRACT Inflammatory myofibroblastic tumor (IMT) is a very rare tumor with still rare occurrence in the genitourinary system, presenting as a pelvic-ureteric junction (PUJ) obstruction (PUJO). We report a case of IMT at the PUJ mimicking congenital PUJO and review the relevant literature.

Inflammatory myofibroblastic tumor of urinary bladder in young male: A case report and literature review

The objective of this study is to present a rare case of inflammatory myofibroblastic tumor (IMT) of the urinary bladder in young male, detailing its clinical presentation, diagnostic approach, treatment strategy, and outcome. Additionally, the objective includes conducting a literature review to provide insights into the characteristics, management, and prognosis of this uncommon tumor entity.A 20-year-old male presenting with dysuria and dribbling of urine underwent diagnostic workup including imaging studies, cystoscopy. On cystoscopy there was a presence of 5x4 cm sized pedunculated mass in the urinary bladder. Partial cystectomy is done and specimen is examined for histopathology. Histopathological examination and Immunohistochemistry study of the tumor specimen was performed to confirm the diagnosis of inflammatory myofibroblastic tumor. A thorough literature review was conducted to gather relevant studies on IMT of the urinary bladder, focusing on clinical presentations, diagnostic modalities, treatment approaches, and outcomes. Diagnostic evaluation revealed a mass lesion in the urinary bladder, and histopathological examination following partial cystectomy confirmed the diagnosis of inflammatory myofibroblastic tumor. The patient underwent surgical excision of the tumor followed by adjuvant therapy. Regular follow-up examinations showed no evidence of tumor recurrence. The literature review identified a limited number of reported cases of IMT of the urinary bladder in young male, emphasizing its rarity and diverse clinical presentations. Various diagnostic modalities and treatment options, including surgery, chemotherapy, and targeted therapy, were discussed in the reviewed literature. Inflammatory myofibroblastic tumor of the urinary bladder is a rare neoplasm at younger age that can present with nonspecific symptoms, making its diagnosis challenging. However, timely recognition and appropriate management, including surgical resection and adjuvant therapy, can lead to favorable outcomes with low recurrence rates. This case report highlights the importance of considering IMT in the differential diagnosis of bladder masses in young patient and underscores the need for further research to better understand the pathogenesis and optimal treatment strategies for this rare tumor entity.

Podcast on Emerging Treatment Options for Pediatric Patients with ALK-Positive Anaplastic Large Cell Lymphoma and Inflammatory Myofibroblastic Tumors.

Anaplastic large cell lymphoma (ALCL) and inflammatory myofibroblastic tumor (IMT) are rare cancers observed predominantly in children and young adults. ALCL accounts for 10-15% of all pediatric non-Hodgkin lymphomas and is commonly diagnosed at an advanced stage of disease. In children, 84-91% of cases of ALCL harbor an anaplastic lymphoma kinase (ALK) gene translocation. IMT is a rare mesenchymal neoplasm that also tends to occur in children and adolescents. Approximately 50-70% of IMT cases involve rearrangements in the ALK gene. A combination of chemotherapeutic drugs is typically used for children with ALK-positive ALCL, and the only known curative therapy for ALK-positive IMT is complete surgical resection. Crizotinib, a first-generation ALK inhibitor, was approved in the USA in 2021 for pediatric patients and young adults with relapsed or refractory ALK-positive ALCL; however, its safety and efficacy have not been established in older adults. In 2022, crizotinib was approved for adult and pediatric patients with unresectable, recurrent, or refractory ALK-positive IMT. This podcast provides an overview of ALK-positive ALCL and IMT. We discuss the current treatment landscape, the role of ALK tyrosine kinase inhibitors, and areas of future research.

Successful Conversion Surgery after Ceritinib Monotherapy in a Patient with Advanced Inflammatory Myofibroblastic Tumor Harboring SQSTM1-ALK fusion

Abstract Inflammatory myofibroblastic tumor (IMT) is an intermediate malignant neoplasm, and approximately 50% of patients are anaplastic lymphoma kinase positive (ALK+). Given this unique trait, ALK tyrosine kinase inhibitors (TKIs), initially developed for ALK+ nonsmall cell lung carcinoma, were expected to be effective. Subsequently, crizotinib, a first-generation ALK-TKI, was approved by the U. S. Food and Drug Administration, and other generations of ALK-TKIs have since been tested for their efficacy. In this study, we report a case of unresectable IMT who showed a partial response to ceritinib, a second-generation ALK-TKI, allowing conversion surgery to be performed. Furthermore, the patient was found to have a rare ALK translocation, sequestosome 1 (5)-ALK (20), detected by next-generation sequencing. In conclusion, this case presents real-world evidence to establish the role of ceritinib as a first-line treatment for ALK+ IMT, which can contribute to further studies on ALK+ IMT.

Unravelling the Enigma of Inflammatory Myofibroblastic Tumours: A Cross-sectional Study from a Regional Cancer Centre, Karnataka, India

Introduction: Inflammatory Myofibroblastic Tumour (IMT) is an unusual neoplasm with intermediate malignant potential, which rarely metastasises. IMT occurs in various anatomical sites, and the initial descriptions were primarily centered on its manifestation in the lungs. Although the age range is broad, IMT is most common in the first three decades of life, with a slight female predominance. Recent data and Anaplastic Lymphoma Kinase (ALK) gene aberrations confirm a neoplastic process for these lesions. Aim: To study the clinicopathological features of IMTs in a regional cancer centre in South India. Materials and Methods: A cross-sectional study included 17 cases of IMT over six years, from May 2014 to May 2020, at Memorial Kidwai Memorial Institute of Oncology (KMIO), Bengaluru, Karnataka, India. The clinicopathologic and immunophenotypic features were analysed on needle biopsies and resected specimens. Correlation between the expression of ALK-1 and histological patterns, mimickers, metastasis, and prognosis were described. Results were analysed using Microsoft Excel 2019 and Medcalc calculator. Results: Seventeen cases of IMTs were included in this study, out of which two were in the lung, and the rest were extrapulmonary. Sixteen cases were unifocal on presentation; however, one IMT of extremity showed evidence of secondaries on bone scan. The mean age at presentation was 33.47 years, and the maleto-female ratio was 1:1.12. Among the seventeen cases, sites are as follows: one each in the retroperitoneum, liver, soft-tissue (extremity), two each in the breast, lung, female genital tract, urinary bladder, and three each in the head and neck region (maxilla, trachea, and alveolus) and mesentery. Microscopically, IMTs showed various histological patterns. Immunostaining for ALK-1 was positive in seven out of seventeen cases, and the rest were diagnosed by excluding closer mimickers. Conclusion: The present study provides crucial insights into the clinicopathological features of IMTs. Immunohistochemistry (IHC) is an essential diagnostic tool that helps accurately differentiate IMT from its mimickers by identifying ALK protein expression. This distinction is essential for guiding targeted therapy in recurrent or metastatic cases.

Unraveling the spectrum of inflammatory myofibroblastic tumors in the lung: A comprehensive case series highlighting endobronchial, pleural, and lung parenchymal tumors

ObjectivesDiverse cases of inflammatory myofibroblastic tumors (IMTs) in the lung (pleural, endobronchial, and parenchymal) are presented while discussing the (preoperative) diagnostic challenges and treatment modalities. Other objectives include emphasizing the significance of gene rearrangements and highlighting the multidisciplinary approach in addressing IMTs. MethodsFour cases of IMT in the lung are presented, including a young adolescent girl with an ETV6-neurotrophic tyrosine receptor kinase 3 (NTRK3) gene rearrangement, a 5-year-old boy with challenging preoperative diagnosis, and 2 middle-aged women with respectively pleural and endobronchial tumors with one peribronchial relapse. ResultsThe cases demonstrate the diverse clinical presentations and diagnostic complexities associated with IMT in the lung. Surgical resection remains the primary treatment modality, with complete resection leading to a cure in most patients. Unfortunately, aggressive relapse can occur, as in our last case of an endobronchial tumor. Frozen section may confirm the presence of malignant cells perioperatively and impact further treatment. The presence of gene rearrangements, such as ETV6-NTRK3, suggests potential therapeutic implications. ConclusionsEarly detection and complete surgical removal of IMT are crucial for effective treatment. Identifying gene rearrangements such as ETV6-NTRK3 holds promise for targeted therapies. Diagnostic challenges, including the controversy of biopsies and preoperative evaluations, underscore the importance of a multidisciplinary approach. Anatomopathological recognition of IMT stays demanding. Close surveillance is necessary due to potential relapse, whereas frozen section perioperatively can help further treatment. This case series emphasizes the diagnostic challenges and therapeutic considerations for IMT in the lung.

A rare case of inflammatory myofibroblast tumor of the stomach successfully treated by inverted laparoscopic and endoscopic cooperative surgery

BackgroundAn Inflammatory myofibroblastic tumor (IMT) is a rare intermediate malignancy characterized by myofibroblast proliferation and inflammatory cell infiltration. Various organs are the primary sites of origin. However, primary tumors originating in the stomach tend to be extremely rare, making the diagnosis difficult. Herein, we present a case of IMT originating in the stomach that was effectively managed using inverted laparoscopic endoscopic cooperative surgery (LECS).Case presentationA 47-year-old male who was admitted to the hospital because of a submucosal tumor that was discovered during upper gastrointestinal endoscopy. The diameter of the tumor was approximately 20 mm. A KIT-negative gastrointestinal stromal tumor was suspected based on the biopsy findings. Therefore, partial resection of the stomach was performed using inverted laparoscopic and endoscopic cooperative surgery. Histopathological examination revealed collagen fiber proliferation from the submucosal layer to the muscular layer, accompanied by infiltration of spindle-shaped cells, lymphocytes, and numerous inflammatory cells. Immunohistochemistry results were positive for SMA and negative for CD34, desmin, and c-kit. IgG4-positive cells were observed with an IgG4/IgG ratio > 50%, and specific nuclei were positive for ALK. Therefore, IMT was diagnosed. This condition may be difficult to diagnose both before and after surgery because of its rarity and submucosal tumor-like morphology.ConclusionWhen a submucosal tumor originating in the stomach is observed, IMT should be considered. Partial resection of the stomach with LECS and immunohistochemical diagnosis may be useful.

PET/CT Imaging of Inflammatory Myofibroblastic Tumor of the Thigh.

A 13-year-old male patient presented with right leg pain and walking difficulty. Contrast-enhanced magnetic resonance imaging showed a softtissue lesion between muscle groups in the anterior half of the right thigh. The excisional biopsy result ended in an inflammatory myofibroblastic tumor (IMT). The 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) scan showed hypermetabolism in the multifocal soft tissue lesion and also confirmed that no other distant foci were present. A three-phase Tc-99m-methylene diphosphonate study of the region showed heterogeneously increased vascularity within the region. We described an unusual case of IMT in a pediatric patient and the importance of PET/CT scanning to delineate the lesion.

Inflammatory Myofibroblastic Tumor of Gallbladder Mimicking Gallbladder Cancer: A Case Report

A primary inflammatory myofibroblastic tumor (IMT) of gallbladder is an extremely rare. Clinical and radiologic features of IMTs are nonspecific and can mimic malignant neoplasm. Herein, we present a case of gallbladder IMT, which was initially suspected to be gallbladder cancer preoperatively.

A case of inflammatory myofibroblastic tumor of the orbit

A case of inflammatory myofibroblastic tumor of the orbit

Inflammatory Myofibroblastic Tumor of the Gallbladder: A Review of a Potential Pitfall

Inflammatory myofibroblastic tumor (IMT) is a unique neoplasm that rarely spreads to other parts of the body. IMT has a wide anatomical distribution, with the mesentery, omentum, retroperitoneum, and pelvis being the most commonly affected. A careful search in the English literature resulted in finding seven cases of IMT arising from the gallbladder. Gallbladder IMT presents a clinical challenge and is preoperatively diagnosed as a malignant gallbladder tumor in most cases. Histopathological assessment is essential for correct diagnosis and management. The prognosis is favorable, with most cases showing no signs of recurrence or metastasis. Even in patients who have had surgical resection, long-term follow-up is required.

A case of inflammatory myofibroblastic tumor of the lung which caused hemothorax and hemoptysis metachronously

A case of inflammatory myofibroblastic tumor of the lung which caused hemothorax and hemoptysis metachronously

Pediatric pancreatic lesions: a clinicopathological analysis of 42 cases

Objective: To investigate the clinicopathological characteristics of pancreatic lesions in children. Methods: The clinicopathological data of pancreatic lesions in children were analyzed including 42 cases of pancreatic tumors diagnosed from January 2000 to May 2021 in Guangzhou Women's and Children's Medical Center, Guangzhou, China. Histological and immunohistochemical assessments were performed. Related literature was reviewed. Results: The 42 pediatric patients with pancreatic lesions aged 1 day to 12 years (mean, 4.25 years). There were 23 males and 19 females. Clinical presentations included abdominal masses, abdominal pain, vomiting and persistent hypoglycemia after birth. Ultrasound and computerized tomography examination showed space-occupying pancreatic lesions in 31 cases, but no detectable pancreatic lesions in 11 cases. Histologically, among the 42 cases, 22 cases (52.4%) were neoplastic, including 18 cases of epithelial origin. Nine cases of pancreatoblastoma showed that the epithelial tumor cells were arranged in a trabecular pattern, with squamous nests. Six cases of solid-pseudopapillary tumors revealed hemorrhagic and necrotic cysts and monomorphic epithelioid cells arranged in solid sheets, nests or pseudopapillae. Two cases of neuroendocrine tumors showed tumor cells arranged in cords or nests; one case had a mitotic count of about 3/10 high power field, and a Ki-67 index of about 5%, which was consistent with G2 neuroendocrine tumor; the other case showed tumor cells with cytological atypia, brisk mitoses, about 25/10 HPF and a Ki-67 index of about 80%, consistent with small-cell type neuroendocrine carcinoma. The case of acinar cell carcinoma showed high cellularity, tumor cells in solid, cord-like or acinar-like arrangement with little stroma, and monotonous tumor cells with single distinct nucleolus. There were 4 cases of mesenchymal tumors, including 3 cases of Kaposi's hemangioendothelioma and 1 case of inflammatory myofibroblastic tumor. Among the 20 cases (47.6%) of non-neoplastic lesions, there were 11 cases of hyperinsulinism with ATP-sensitive potassium channel abnormality (HAPCA). Severn cases of diffuse type HAPCA in which the islets scattered between the pancreatic acinar tissue, enlarged, and prominent nuclei. Three cases of focal type HAPCA showed pancreatic islet hyperplasia in the form of nested nodules (0.6-1.5 cm). One case of atypical type HAPCA had extensive islet hyperplasia in pancreatic tissue, and scattered proliferation of nest-like nodules was noted. There were also 7 cases of pseudocyst and 2 cases of congenital cyst. Immunohistochemically, pancreatoblastomas were diffusely positive for CKpan, CK8/18, and β-catenin (nuclear staining of squamous nests only). Solid-pseudopapillary tumors expressed CD10, cyclin D1, CD99, vimentin, CD56, and β-catenin (nuclear staining). Neuroendocrine tumors were positive for CK, Syn, NSE, CgA, CD56, and β-catenin (membranous staining). The acinar cell carcinoma was positive for CK8/18, trypsin, and β-catenin (membranous staining). Conclusions: Pancreatic lesions in children have a wide range of histopathological types. HAPCA is the most common lesion of newborns. Pediatric pancreatic tumors are rare and mostly malignant. It is important to recognize them and make correct pathological diagnoses.

Inflammatory myofibroblastic tumor of the ureter in a paediatric patient

Inflammatory myofibroblastic tumor (IMT) is a rare soft-tissue neoplasm which has been described in a variety of locations. In the urogenital system, it occurs mainly in the bladder and the kidney. IMT arising from the ureter is exceedingly rare. We report an exceptional case of IMT arising from the ureteric submucosa in a three-year-old female child. The patient presented with pan-hematuria and no other symptoms. Urinalysis revealed numerous red blood cells, culture was negative for infection. A CT Urogram showed the lesion was involving most of the ureter. A left radical nephroureterectomy was performed, and she has remained well to date. Ureteral IMT is extremely rare and often asymptomatic, which can result in a delayed diagnosis. Radical excision is the best therapeutic approach with excellent survival outcomes.

Inflammatory Myofibroblastic Tumor A Rare Case in Kidney

Inflammatory Myofibroblastic Tumour (IMT) or ‘pseudotumour’ of the kidney is a uncommon benign tumour, common in young adults with unknown etiology. Clinical feature and radiological investigations are usually inconclusive. Often, biopsy is inconclusive required a management similar to that of Renal Cell Cancer (RCC). Diagnosis is based on immunohistochemistry. We are reporting a case of IMT in a 32-year-old female patient who presented with left flank pain and haematuria which on evaluation was suggestive of left renal cell carcinoma. IMT was confermed by Surgery, histopathological examination and Immunohistochemistry. Bangladesh J. Urol. 2021; 24(1): 112-115

Inflammatory Myofibroblastic Tumour of Lung, A Rare Entity

Inflammatory fibroblastic tumour of lung is a rare mesenchymal tumour usually affecting the children and young adults. It is usually considered as a benign entity however it has high potential for recurrence and local invasion. Exact pathogenesis of tumour is not known. Complete surgical excision is the treatment of choice. Due to high chance of recurrence, patients are usually kept on close follow up. Here we present a case of inflammatory myofibroblastic tumour of lung , who presented with history of pain and shortness of breath.She was diagnosed a case of inflammatory myofibroblastic tumour with the help of CT scan chest and CT Guided biopsy.She underwent right sided pneumonectomy and remained symptom free on follow up.
 Key Words. Inflammatory, Myofibroblasts, Pneumonectomy, Tumour.

A unique case of inflammatory myofibroblastic tumor of the liver manifesting with biloma: A case report.

Anaplastic lymphoma kinase (ALK)-negative hepatic inflammatory myofibroblastic tumors (IMTs) harboring the ETS variant transcription factor 6-neurotrophic receptor tyrosine kinase 3 (ETV6-NTRK3) fusion gene and manifesting with biloma are extremely rare, and their biological behavior is unclear. The present study reports the case of a 45-year-old female with ALK-negative IMT of the liver harboring the ETV6-NTRK3 fusion gene and manifesting with biloma. Computed tomography of the abdomen confirmed the lesions to be a low-density mass, measuring 11.2×8.5×10.5 cm, located in the left lobe of the liver, and a lower-density mass, measuring 8.5×6.1×5.9 cm, located in the interior of the tumor. As the suspicion of a malignancy remained high, surgical resection of the left hepatic lobe, including the tumor, was undertaken. Intraoperatively, a tumor (12×10×9 cm), with an unclear boundary, incomplete capsule and fish-like texture, was found in the left lateral lobe of the liver, and a biloma, measuring 8×6 cm, was identified inside the tumor. Pathological examination revealed spindle cell proliferation with infiltration of chronic inflammatory cells and mucinous degeneration. Immunohistochemical studies showed negativity for ALK, CD117, CD34, discovered on GIST-1, desmin, smooth muscle actin, S-100, CD21, pan-cytokeratin, epithelial membrane antigen, CD23 and CD35, but positivity for vimentin staining, and 5% Ki-67-positive cells. Fluorescence in situ hybridization studies assessing characteristic genetic rearrangements using ALK, RET, ROS1, MDM2, MGEA5 and ETV6 break-apart assays, revealed the presence of the ETV6-NTRK3 fusion oncogene and negativity for ALK, RET, ROS1, MDM2 and MGEA5. The patient was discharged 7 days post-operatively, without any adjuvant treatment. No recurrence of symptoms was noted at the 3-year follow-up. To the best of our knowledge, this is the first report of biloma in an ALK-negative IMT of the liver, which may increase our understanding of hepatic IMT.

Inflammatory Myofibroblastic Tumor of the Subglottis: A Rare Case Report.

Inflammatory myofibroblastic tumor (IMT) is a very rare mesenchymal tumor that can behave as a locally benign or aggressive lesion. Here, we present an extremely rare case of IMT involving the subglottic of a middle-aged male. Steroid therapy treatment alleviated dyspnea, but the recurrence of dyspnea was reported two months later. Both sides of the subglottic tumor were excised following treatment, and the surgical specimens were subjected to histopathological evaluation and diagnosis for IMT. At 4years after excision, follow-up laryngoscopy revealed symmetric vocal cords without evidence of any subglottic mass. Subglottic mass is often asymptomatic until it presents with hoarseness or dyspnea, and subglottic IMT is rare. Based on the successful treatment of our case, complete surgical excision is highly recommended for this rare tumor. However, further research is needed to discover a more effective and cost-effective treatment approach.