Diagnosis Of Inflammatory Myofibroblastic Tumor Research Articles

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.

Diagnosis of inflammatory myofibroblastic tumor in a pediatric patient initially suspected of tuberculosis

BackgroundSymptoms of inflammatory myofibroblastic tumor (IMT) are atypical, and histopathological misdiagnosis of IMT is still inevitable. Here we present a pediatric case that an eight-year-old boy with recurrent fever for fifteen months, received anti-tuberculosis therapy for five months and was ultimately confirmed to be IMT.Case presentationAn eight-year-old boy experienced a recurrent fever for fifteen months, accompanied by cough, vomiting, meteorism, night sweating, and emaciation. Thoracoabdominal computer tomography revealed multiple enlarged lymph nodes in the thorax, abdomen, and axilla, as well as minimal bilateral pleural effusion. Histopathological examinations of the intestines and greater omentum implied fibrous tissue hyperplasia along with eosinophil and lymphocyte infiltration. The patient was initially misdiagnosed with tuberculosis, and symptoms were relieved partially following anti-tuberculosis treatment. However, after four months, the symptoms aggravated again and a subsequent histopathological analysis of a second sample from the greater omentum revealed the presence of IMT. Eventually, after surgical resection of the lesions and chemotherapy, the clinical symptoms in the child gradually alleviated.ConclusionsThe clinical course of IMT is variable, and pediatricians should pay attention to differentiating IMT from tuberculosis.

Spindle cell carcinoma of the larynx: a rare entity

Squamous cell carcinoma (SCC) is the commonest malignancy encountered in the larynx in the Indian population. Spindle cell carcinoma is an extremely rare malignant tumor and is a variant of SCC with a mesenchymal like malignant cell component. This is a case report of a 58 years old patient who presented with hoarseness of voice of 7 months duration. He was diagnosed to have a right vocal cord polypoidal growth with mobile vocal cords. He underwent direct laryngoscopy and biopsy of the lesion which was reported as spindle cell neoplasm/inflammatory myofibroblastic tumor (IMT). Immunohistochemistry (IHC) was carried out and confirmed the diagnosis of inflammatory myofibroblastic tumor. He was doing well and the follow up videolaryngoscopic examinations and PET CT were normal. At subsequent follow-up 19 months later, he was found to have a recurrent right vocal cord lesion. Direct laryngoscopy and biopsy was repeated along with laserization of the right vocal cord. Histopathology was reported as spindle cell malignant neoplasm. Immunohistochemistry was reported as spindle cell carcinoma. He was then sent for radiation therapy. The patient responded well to radiation and is tumor free at last follow-up. He has been advised regular follow-ups. Spindle cell carcinoma is an extremely rare laryngeal malignancy. It typically originates in the glottis. The treatment for spindle cell carcinoma includes surgery, radiation or a combination of these modalities. Its prognosis is worse than squamous cell carcinoma and patients require close follow-ups.

Inflammatory Myofibroblastic Tumor of the Distal Bile Duct Mimicking Cholangiocarcinoma: A Case Report

Inflammatory myofibroblastic tumor has been described in various locations. However, its appearance in the periampullary region is uncommon and has rarely been reported in the literature. We report a case of a 37-year-old Moroccan male with painless jaundice and pruritus. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography showed a 2-cm-long stricture in the distal bile duct that suggests a cholangiocarcinoma. There was no involvement of adjacent structures at computed tomography of abdomen. The patient underwent a pancreaticoduodenectomy. The tumor histology showed regular spindle cells arrayed in fascicles, admixed with lymphocytes, plasma cells and eosinophils. Immunohistochemical analysis showed positive staining for smooth muscle actin, H-caldesmon. In contrast, the tumor cells did not express cytokeratin, PS100, CD68, CD23, CD117 and ALK. Based on these data, the diagnosis of inflammatory myofibroblastic tumor of distal bile duct was made. We report this new case in the aim of shedding more light on this entity and the challenges in making the diagnosis in such location.

Hypertrophic pachymeningitis due to IgG4-related disease (RD-IgG4). A case report

IntroductionHypertrophic pachymeningitis (HP) is a clinico-radiological entity characterized by a thickening of the dura mater that may be focal or diffuse and manifested by a variety of neurological syndromes. Aetiologically, it is classified as infectious, neoplastic, autoimmune, and idiopathic. Many of these formerly idiopathic cases have been shown to fall into the spectrum of IgG4-related disease. ObjectiveTo describe the case of a patient attended for neurological involvement due to hypertrophic pachymeningitis with initial diagnosis of inflammatory myofibroblastic tumour and final diagnosis of IgG4-related disease. CaseA 25-year-old woman with neurological symptoms of 3 years’ evolution characterized initially by right hypoacusis, evolving with headache and diplopia. Magnetic resonance imaging (MRI) of the encephalon showed pachymeningeal thickening with involvement of vasculo-nervous structures in the tip of the cerebellum, cavernous sinus, ragged foramen, and optic chiasm. The patient presented for consultation with the result of an incisional biopsy that reported a proliferative lesion combining fibrous elements of fascicular or swirling arrangement with collagenized streaks with dense, lymphoplasmacytic infiltrate and some macrophages, with negative staining for ALK 1, with a diagnosis of inflammatory myofibroblastic tumour.Due to suspicion of IgG4-related disease (IgG4-RD) the biopsy was sent for review and pertinent complementary studies were requested. Biopsy reviewNon storiform fibrosis, predominantly lymphoplasmacytic infiltrate, histiocytes, and polymorphonuclear infiltrate in sectors, without granulomas or atypia. Staining for germs negative. Immunohistochemistry with 50–60 IgG4+/HPF cells and range of 15%–20%, CD68+ in histiocytes, CD1a−, S100−.The patient presented deterioration of visual acuity due to ophthalmic nerve involvement, so glucocorticoid treatment was started in pulses and rituximab with regression of symptoms and imaging improvement of the lesions. ConclusionHP is a clinical imaging syndrome with variable symptoms and aetiologies that poses a diagnostic challenge.In this case the initial diagnosis was inflammatory myofibroblastic tumour, which is a neoplasm of variable behaviour, locally aggressive, and can metastasize; it is one of the main differential diagnoses of IgG4-related disease because they share anatomopathological features, including storiform fibrosis.IgG4-RD is an immune-mediated condition that can have single or multiple involvement. Its diagnosis is complex when it presents with single organ involvement or in non-typical organs (CNS, meninges) in which data are scarce, as in the case of our patient with single organ involvement of the CNS.Although there are classification criteria to guide non-specialists in the diagnosis, the sum of the clinical picture, imaging, laboratory, pathological anatomy, and immunohistochemistry will always be evaluated together for a definitive diagnosis.

Abstract 14723: A Rare Case of Constrictive Pericarditis: Inflammatory Myofibroblastic Tumor

Case Presentation: A 14-year-old female presented with a 3-week history of lower extremity edema, abdominal distension, fatigue, and dyspnea on exertion. Her examination was notable for an elevated jugular venous pulse, abdominal distension with hepatomegaly, and 2+ pitting edema in the bilateral lower extremities. Laboratory findings were within normal limits. EKG showed low-amplitude QRS and echocardiogram confirmed bi-atrial enlargement, a dilated and non-compressible IVC, dyskinetic interventricular septum, and elevated right atrial pressures. Chest computed tomography demonstrated mediastinal mass with findings of a 6.2 x 4-centimeter mass in the pre-vascular space, diffuse necrotic mediastinal lymphadenopathy, and constrictive pericarditis with associated congestive hepatopathy. Cardiac magnetic resonance imaging revealed significant enhancement in T2 STIR with severe circumferential delayed enhancement, diastolic bounce, and respirophasic shift. A right heart catheterization was pursued which revealed elevated filling pressures but preserved cardiac index. The patient underwent video-assisted thoracoscopic biopsy of the mass with an initial histologic diagnosis of inflammatory myofibroblastic tumor (IMT). She underwent radical pericardiectomy for symptom resolution. Discussion: IMT is composed of spindle cells mixed with infiltrates of varying numbers of inflammatory cells. Although IMT can present in any age group, most patients who develop IMT are in adolescence. IMT is incredibly rare and its etiology still remains unclear. It remains difficult to distinguish IMT from a malignant tumor on the basis of imaging. As a result, in most cases, a definitive diagnosis is made based on the histopathological findings from either a resected tumor or a needle biopsy. Management of IMTs presents a challenge as treatment protocols have not clearly been established; radical local excision of the tumor is the mainstay of treatment.

Inflamatorní myofibroblastický tumor v otorinolaryngologii

Introduction: Inflammatory myofibroblastic tumour (IMT) is a rare tumour with borderline biological behaviour composed of fibroblasts and myofibroblasts and accompanied by non-neoplastic inflammatory cells. It occurs mostly in children and young adults. The disease is most commonly seen in abdominal soft tissues (omentum, mesentery, etc.), in the lungs and also has been reported in other parts of gastrointestinal tract and other organs. In the head and neck region IMT is not so common, it may occur in larynx, trachea or nasal sinuses. The primary therapeutic approach is a complete surgical excision. Radiation, chemotherapy or biological treatment modalities are used in aggressive, non-resectable, and recurrent tumours or in case of metastases. Aims: Through a case report on a small set of patients and up-to-date information from the literature, the authors want to share knowledge about the rare diagnosis of IMT in ENT, its possible clinical manifestations, differential diagnosis and appropriate treatment. The group of patients, the case report: The paper presents patients with IMT treated at the Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Hradec Kralove in the years 2013–2020. The case of a 11-year-old boy with progressive dyspnoea caused by polypoid tracheal tumour is discussed in more detail. The tumour was removed by external surgical approach with bronchoscopy assistance. Histological examination proved an IMT. Conclusion: IMT is a rare neoplasm which must be included in the differential diagnosis of tumours of the respiratory tract, especially in children and young adults. The treatment consists of complete surgical removal and regular follow-up due to the risk of local recurrence. Keywords: inflammatory myofibroblastic tumour – dyspnoea in a child – tracheal tumour

Short-Time Development of Esophageal Inflammatory Myofibroblastic Tumor Which Resected by Endoscopic Submucosal Dissection.

A 53-year-old man was pointed out an elevated lesion in the esophagus by screening upper gastrointestinal series. A esophagogastroduodenoscopy showed a submucosal tumor 10 mm in diameter in the lower thoracic esophagus (Figure A). A biopsy specimen taken from the tumor showed no malignancy. One year later, the tumor had more than doubled in size (Figure B). So, we decided to perform endoscopic ultrasonography–guided fine-needle aspiration for this tumor. Endoscopic ultrasonography showed a hypoechoic mass 21 mm in size which come from the second layer. The biopsy specimen showed spindle cell proliferation and actin and anaplastic lymphoma kinase positivity (Figure C), which lead to the diagnosis of inflammatory myofibroblastic tumor (IMT). Because of the strong accumulation on positron emission tomography/computed tomography and the growing trend of submucosal tumor, we decided to perform endoscopic submucosal dissection (ESD). The pathological results were consistent with IMT which complete resected by ESD (Figure D). No recurrence has been observed for a year. Although IMT is a tumor of mesenchymal origin which occurs systemically, there are few reports of its occurrence in the esophagus. This is the first case in which short-time growth can be observed and resected by ESD.

Giant inflammatory myofibroblastic tumor of oral cavity: a case report and literature review

Background: Inflammatory myofibroblastic tumor is an infrequent lesion that is seen in the lungs, abdomen, skin, soft tissue, genital system, and mediastinum. It is rare in the oral cavity. Case presentation: A 58-year-old woman presented with a mass in the left buccal mucosa. An extensive surgical intervention was performed. On gross examination, the mass was 9.5 x 8 x 3.5 cm in size. Microscopically, the tumor included spindle-shaped myofibroblast-like cells intermingling chronic inflammatory cells. Differential diagnosis of inflammatory myofibroblastic tumor is extensive and includes benign and malignant spindle cell tumors, such as cranial fasciitis, solitary fibrous tumor, fibrosarcoma, and rhabdomyosarcoma. Conclusion: Inflammatory myofibroblastic tumors are classified as tumors of intermediate biological potential due to a tendency of local recurrence and low risk of distance metastasis. We found it appropriate to submit this case because of its rarity and rapid growth; also, it is the largest size oral cavity location inflammatory myofibroblastic tumor that has been reported so far.

Inflammatory myofibroblastic tumors of the urinary bladder: a clinicopathological study of ten cases

0bjective To investigate the clinicopathological features,immunohistochemical phenotypes, molecular genetic alterations,diagnosis and differential diagnosis of inflammatory myofibroblastic tumor (IMT) of the urinary bladder. Methods: Ten cases of IMT of the urinary bladder (three cases at Ningbo Diagnostic Pathology Center from September 2011 to December 2020, five in-house diagnosed cases and two consultation cases at Shanghai Rui Jin Hospital from June 2011 to December 2020) were collected retrospectively. The clinicopathologic features and immunophenotypic profiles were studied by light microscopy and immunohistochemistry (EnVision method). The translocation of ALK gene was detected by fluorescence in situ hybridization (FISH). Results: Of the 10 patients, eight were male and two were female. The patients' age range was 16 to 62 years (median 36 years).The main clinical presentation was hematuria and urinary irritation. Three cases were located at the dome of the urinary bladder, four cases were in the left lateral wall and the remaining three cases were in the right lateral wall. The tumor size ranged from 1.5 cm to 8.5 cm. In eight cases, the tumors were mainly submucosal, and in some cases extending to the muscular layer of the urinary bladder. In two cases, the tumors were mainly located in the muscular layer and focally extended to the submucosa and adventitia. Histologically, four cases had the nodular fasciitis-like pattern, three cases had fibrohistiocytoma-like pattern, two cases had mixed histologic patterns and the remaining case showed leiomyosarcoma-like histologic features. Immunohistochemically, the tumor cells expressed SMA (10/10),calponin (9/10),desmin (6/10) and CKpan (9/10). Cytoplasmic staining for ALK1 and ALK (5A4) was detected in 7 of 10 cases and 8 of 10 cases, respectively. Nuclear and cytoplasmic staining for ALK (D5F3) was detected in 7 of 10 cases. Among eight cases with material available for FISH analysis, ALK rearrangement was present in five cases. Follow-up data were available in eight patients and none had local recurrence nor distant metastasis. Conclusion: IMT of the urinary bladder is an uncommon mesenchymal neoplasm with intermediate malignant potential.It has special clinicopathologic features, and a minority of cases have local tumor recurrence.

Rare presentation of inflammatory myofibroblastic tumor in a failed renal allograft

Inflammatory myofibroblastic tumors (IMT) are rare, mesenchymal tumors that can occur in any anatomic location. IMTs have a variable clinical course but usually require wide surgical excision to prevent local recurrence. There have been limited case reports of IMT occurring in solid organ transplant recipients. Herein we report on a case of IMT presenting in a failed renal allograft. A 53-year-old male awaiting re-transplant presented with pain and a palpable mass in his allograft. Imaging demonstrated an infiltrative soft tissue mass encasing the renal hilum. Percutaneous biopsy demonstrated a myofibroblastic proliferation with myxoid background and no high-grade features. The tumor cells were diffusely positive for anaplastic lymphoma kinase-1 (ALK-1) and had a Ki-67 proliferation index of 10%. These findings were consistent with a diagnosis of IMT. A transplant nephrectomy was performed with wide margins to achieve an R0 resection. Pathology on the resection specimen confirmed an IMT that measured 6.5 cm x 6.3 cm. The patient has no evidence of local recurrence at 6-months follow-up and has been relisted for a second kidney transplant.

Clinicopathological features of inflammatory myofibroblastic tumor

Objective: To investigate the clinicopathological diagnosis and differential diagnosis of inflammatory myofibroblastic tumor (IMT). Methods: Thirty-two cases of IMT collected at the People's Hospital of Jiangsu Province from May 2010 to May 2020 were evaluated for their clinical, histologic, immunohistochemical and genomic features, and relevant literature was reviewed. Results: There were 19 male and 13 female patients, with age ranging from 5 to 65 years (mean, 37 years). The tumors were located in the lung and mediastinum (10 cases), gastrointestinal tract and mesentery/omentum (12 cases), urinary bladder (5 cases), head and neck (3 cases), somatic soft tissue (1 case), and retroperitoneum (1 case). Four cases of epithelioid inflammatory myofibroblastic sarcoma (EIMS) were all located intra-abdominally. Histologically, the tumor cells were myofibroblasts and fibroblasts arranged in predominantly fusiform pattern, with variably edematous to myxoid background or sclerotic collagenized stroma, and variably mixed chronic or acute inflammatory cells infiltration. EIMS were composed mainly of epithelioid tumor cells, with myxoid stroma and numerous neutrophils. Immunohistochemically, the tumor cells expressed cytoplasmic ALK (25/32, 78%), whereas the four EIMS showed nuclear membrane ALK staining pattern. The tumor cells also expressed CKpan (8/19), SMA (24/32, 75%) and desmin (12/32, 38%); all four EIMS also showed strong positivity for desmin. Fluorescence in situ hybridization (FISH) for ALK gene rearrangement showed split apart signals in 12 of 15 cases, most commonly with atypical signals. Next-generation sequencing (NGS) was performed in three tumors and showed that one case of lower leg IMT harbored a novel CLIP2-ALK fusion, and two cases of EIMS harbored RANBP2-ALK fusion. Follow-up data were available in 29 patients. Twenty-two patients were alive with no evidence of tumor, four patients had tumor recurrences (three patients were treated with crizotinib and were alive with tumor), and three patients died of the disease (including two patients with EIMS). Conclusions: IMTs show a wide morphologic spectrum, and should be differentiated form a variety of benign or malignant tumors. Immunohistochemistry (ALKp80, ALKD5F3) and FISH (ALK break-apart probe) could assist the diagnosis of IMT, with NGS recommended for the atypical cases.

Anaplastic Lymphoma Kinase‐Positive Inflammatory Myofibroblastic Tumor Mimicking Meningioma: A Case Report

A 15-year-old girl presented with intermittent nausea and vomiting, headache, and vision changes. MR imaging of the brain revealed an avidly enhancing infratemporal dural-based mass arising from the tentorium, with hyperintensity on T2WI and transdural extension into the posterior cranial fossa. The well-encapsulated fibrous tumor was resected en bloc after cauterization of its rich tentorial arterial supply. Histologic examination demonstrated pleomorphic myofibroblastic cells admixed with an inflammatory infiltrate. Spindle cells showed strong, diffusely positive immunostaining for anaplastic lymphoma kinase, and genomic sequencing uncovered a tropomyosin 3 gene and anaplastic lymphoma kinase fusion and an activating mutation in the Kirsten rat sarcoma oncogene. A diagnosis of inflammatory myofibroblastic tumor was made. Primary intracranial involvement of inflammatory myofibroblastic tumor is exceptionally rare, and few cases that feature an anaplastic lymphoma kinase translocation have been described. Inflammatory myofibroblastic tumor‐CNS is an important differential diagnosis for dural-based lesions in children and young adults due to its propensity for recurrence and malignant degeneration.

A rare case of inflammatory myofibroblastic tumour in the maxillary sinus: masquerading a malignancy

<p>Aim establishing the diagnosis of inflammatory myofibroblastic tumour is very challenging due to its varied clinical and radiological manifestations. We report a rare presentation of a 5 years old female child with inflammatory myofibroblastic tumour, which was endoscopically resected with postop steroid therapy. Case report, a 5 years old female child presented to ENT OPD with complaints of right nasal obstruction since, 4 months along with recurrent minor episodes of spontaneous bleeds from the right nasal cavity. On examination a huge fleshy reddish polypoidal and pulsatile mass was seen filling the entire right nasal cavity associated with right eye proptosis. MRI with contrast showed expansion of right maxillary sinus bony walls with internal soft tissue leision measuring 41 mm (TRANS) × 36 mm (AP)×31 mm (CC) seen enhancing with contrast. Vascular supply is from maxillary artery. Under anaesthesia right external carotid artery ligation was done followed by removal of mass from right nasal cavity. Histopathological examination was consistent with inflammatory myofibroblastic tumour. On immunohistochemistry spindle cells showed cytoplasmic positivity for SMA. Inflammatory myofibroblastic tumour though a very unusual tumour, should be considered in the differential diagnoses of tumours in the nasal cavity. A vigilant clinical evaluation and extensive imaging along with histopathological examination can guide us to an early diagnosis and proper treatment resulting in better patient satisfaction.<strong></strong></p>

Inflammatory Myofibroblastic Tumour.

Inflammatory myofibroblastic tumour (IMT) is a rare entity.Since IMT can occur in different locations, its clinical presentation is varied, with non-specific symptoms.Prognosis is usually benign, but relapse and metastasis have been reported.

Inflammatory myofibroblastic tumor of the small intestine with intussusception in a child: Case report and literature review

Abstract Inflammatory myofibroblastic tumor (IMT) is a rare but benign entity masquerading as a malignant tumor. We herein report an 18-month-old boy with small bowel wall IMT who presented with intussusception. He was transferred to our hospital with a complaint of abdominal pain and diagnosed with intussusception. On laparotomy, the small bowel wall mass consisted of solid tissue measuring 2.5 × 3.0 cm. The postoperative pathology examination revealed numerous spindle cells arranged in dense bundles with diffuse infiltration of inflammatory cells and tumor involvement of the mucosa and serosa. As the postoperative pathology examination resulted in a diagnosis of IMT and suspected partial resection, small bowel resection and mesenteric lymph node resection were performed two months after the first operation. A pathology examination after the second surgery revealed no evidence of residual IMT and negative findings for metastasis in the regional lymph nodes. IMT is a locally aggressive lesion. The immunophenotypic feature of ALK-1 in particular is associated with a high recurrence rate. Long-term follow-up is absolutely needed.

A Novel ALK–THBS1 Fusion in a Laryngeal Inflammatory Myofibroblastic Tumour: A Case Report and Literature Review

Inflammatory myofibroblastic tumor (IMT) is an uncommon neoplasm most frequently seen in the abdomino-pelvic region and lungs of children and young adults. Laryngeal tumors are rare. We present a case of a 23-year-old patient with a 5month history of laryngitis and aphonia unresolved by corticotherapy. Laryngoscopy revealed a small, non-ulcerated, subepithelial, polypoid mass arising from the right vocal cord. The diagnosis of IMT with ALK expression was supported by histopathologic and molecular analysis. The THBS1 fusion partner was identified by RNA-sequencing analysis for the first time in a laryngeal IMT. This fusion partner has only been identified in six uterine IMTs thus far. Conservative excision of the lesion yielded excellent functional results for the patient. The voice was preserved and no recurrences were seen after 6months of follow-up.

Activity of chemotherapy in inflammatory myofibroblastic tumor (IMT): A retrospective analysis within the Italian Rare Tumours Network (RTR).

e22545 Background: IMT is a rare soft tissue tumour often curable with surgical resection. Nonetheless, in advanced cases a medical therapy is needed. Few data are available on the role of conventional chemotherapy. We report on a retrospective study on the activity of cytotoxic chemotherapy in pts with IMT treated within the RTR. Methods: Patients (pts) of any age with a centrally confirmed diagnosis of IMT treated with anthracycline-based or methotrexate (MTX) and vinorelbine/vinblastine-based regimens between 2008 and 2018 were reviewed. ALK status was evaluated by immunohistochemistry and FISH. Response was assessed by RECIST 1.1. Results: Twenty pts were retrospectively identified (ALK positive = 12, under assessment = 8); median age at the time of treatment start = 38, range = 5-77; disease extent: local disease = 2, locally advanced = 9, metastatic disease = 9. Thirteen pts (65%) received an anthracycline-based regimen, 7 (35%) MTX and vinorelbine/vinblastine. In the anthracycline-based group, 6 (46%) and 7 (54%) pts were treated for localized and advanced disease, respectively. Twelve pts were evaluable for response. Best responses were: 1 CR (8%), 6 PR (50%), 2 SD (17%), 3 PD (25%), for an ORR = 58%. 8 pts received a complete surgical resection at the end of the medical treatment and 6 are disease free. At 39 mos median follow-up, mPFS for 7 pts treated for advanced disease was 5.4 mos (IQR: 1.9-18.8). In the MTX and vinorelbine/vinblastine group, 2 (28%) and 5 (72%) pts were treated for localized disease and advanced disease, respectively. All pts were evaluable for response. Best responses were: 5 PR (50%), 3 SD (30%), 2 PD (20%), for an ORR = 50%. Two pts received a complete surgical resection after the end of chemotherapy and both are disease free. At 56.4 mos median follow-up, mPFS for pts with advanced disease was not reached, with disease progression reported only in 1 pt. Conclusions: Chemotherapy is an option in localised IMT when tumour shrinkage is needed as well as for tumour control in advanced disease. Clearly, its efficacy should be compared with ALK inhibitors, although of course both groups of agents may be resorted to stepwisely in advanced cases in need for medical therapy.

Pediatric orbital inflammatory myofibroblastic tumor: A rare case

This case report highlights, a 4 year old female child with gradually increasing exophytic mass involving eye ball in right orbit. Enucleation with mass excision was done and histopathological examination reported the diagnosis of inflammatory myofibroblastic tumor (IMT). These are benign tumors of unknown etiology. We are reporting this case to enlighten on considering IMT as differential diagnosis of solid tumors in orbit and also early diagnosis and treatment can save vision and eye. Keywords: Pseudotumor, Plasma cells, Orbital mass, Pseudoneoplasia, IMT.

A Rare Tumor of the Mediastinum: Inflammatory Myofibroblastic Tumor

Introduction: Inflammatory myofibroblastic tumors (IMTs) arising in the mediastinum is rare. Their etiology remains unknown and their diagnosis is often overlooked before the use of surgery which allows the proper diagnosis and adequate treatment.Case report: We report a case of the 56-year-old woman that had a mediastinal mass discovered after a long complaint of chest discomfort. Chest contrast-enhanced computed tomography (CT) showed a heterogeneously enhanced mass in the middle mediastinum The diagnosis was confirmed by histopathology and immunohistochemical study after surgical resection through a thoracotomy The patient was well and had no recurrence 6 months after surgery.Conclusion: The diagnosis of IMT should be kept in mind and included in the differential diagnosis of mediastinal masses.