Treatment and outcomes in pediatric inflammatory myofibroblastic tumors - A systematic review of published studies.

Inflammatory myofibroblastic tumor (IMT) is a soft tissue neoplasm which can be locally invasive, recur, or in rare cases metastasize. Often originating from the abdomen or thorax, IMT most commonly affects children and young adults. Due to its rarity comprehensive reports detailing clinical management and outcome(s) are sparse and often based on limited index case numbers. This study systematically analyzes outcome metrics of pediatric IMT and identifies risk factors for mortality. Medline/Embase databases were searched in accordance with PRISMA guidelines. Final analysis included 57 studies with 673 IMT patients (355 males, 53 %). Individual patient data was available for 405 cases with a median follow-up period of 36 months. Tumor sites included abdomen/pelvis (n = 233, 58 %), thorax (n = 125, 31 %), head/neck (n = 34, 8 %), and extremities (n = 13, 3 %). Surgical tumor resection was the mainstay of treatment, while only 20 patients (5 %) were treated non-operatively. Recurrence(s) were reported in 80 patients (20 %) with 34 (12 %) requiring reoperation. Positive tumor margins were a significant risk factor for tumor recurrence (p < 0.0001). Chemo/radiotherapy was reported in 98 patients (25 %). Most patients (94 %) survived; 81 % (n = 237) with no evidence of recurrent disease, 14 % (n = 41) were alive with disease, and 25 (6 %) died of disease. Positive margins at primary operation, and metastatic disease were associated with mortality (p < 0.0001 for both). IMT is a rare tumor with favorable outcome for the majority of patients. Whilst most patients will present with benign tumors, complete surgical resection (R0) is crucial, as positive surgical margins are a significant risk factor for tumor recurrence and mortality.

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

ABSTRACT: 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.

Inflammatory myofibroblastic tumors: A short series with an emphasis on the diagnostic and therapeutic challenges.

BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) are a distinct entity of mesenchymal tumors. We present the challenges in their diagnosis and management. MATERIALS AND METHODS: A retrospective study with detailed clinical, radiological, and histopathological (HPE) features along with management and outcome of 10 biopsy-proven patients with IMT, between 2001 and 2020. RESULTS: The location included intracranial (5), orbital (4), and spinal (1) with M : F = 7 : 3. The mean age of onset was in the third decade. The commonest symptom was headache, while proptosis and blurred vision occurred in orbital IMTs. HPE revealed diffuse infiltration of mixed inflammatory cells over proliferating myofibroblasts. Smooth muscle antigen immunoreactivity was noted in fibroblastic spindle cells of all IMTs. However, we did not find anaplastic lymphoma kinase expression in any of our cases, as this is only found in ~ 50% of all IMTs. Tumor infiltration into adjacent tissue was noted in 4 patients. Surgical excision was limited to orbital IMTs, as most central nervous system (CNS) tumors were not amenable for resection. Steroid administration showed moderate improvement in the IMT-CNS patients but also required additional immunomodulation. Four patients had a median long-term follow-up of 7 years. Two patients had recurrent lesions demonstrated by imaging after 2 years of initial presentation. CONCLUSION: IMTs are rare and ambiguous tumors of unknown etiology that can occur anywhere in the body. Clinical and radiological features may not be specific to determine the diagnosis, but it should be considered as a differential diagnosis. Extensive thorough workup with histopathology along with the help of immunohistochemistry is conducive to better clinical outcomes. Surgical biopsy with extensive and total resection of these tumors along with steroid and radiotherapy may enhance the survival outcomes.

Systemic Brucellosis with Arrhythmogenic Cardiac Inflammatory Pseudotumor.

BACKGROUND Cardiac inflammatory pseudotumors are rarely observed. Their etiology might include immunologic abnormalities, fibrogenetic disorders, specific reactions to infections or abnormalities related to trauma, necrosis, or neoplasm. Life-threatening ventricular tachycardia and cases of sudden death related to cardiac tumors have been reported. The present report describes and discusses diagnostic and therapeutic solutions for the treatment of nonsarcoid multiorgan pseudotumors with cardiac involvement. CASE REPORT A 38-year-old woman presented to the clinic with symptomatic ventricular tachycardia. As coronary artery disease, cardiomyopathy, and channelopathy were ruled out, and electrocardiograms were not typical of idiopathic arrhythmia, the patient underwent detailed diagnostics which included targeted endomyocardial biopsy, which revealed a cardiac inflammatory pseudotumor. Laborious testing (and eventually, antibiotic therapy) led to ex juvantibus diagnosis of multiorgan disseminated brucellosis with cardiac involvement. Treatment with ceftriaxone, doxycycline, and rifampicin resulted in a complete resolution of all lesions after 3 months, and sustained recovery was observed during a 5-year follow-up. As the risk of ventricular tachycardia could not be reliably predicted, the patient had a subcutaneous implantable cardioverter-defibrillator implanted. CONCLUSIONS A vast diagnostic armamentarium of modern medicine allowed us to diagnose an unsuspected and rare cardiac inflammatory pseudotumor. In the case of travelers, the possibility of regionally specific illnesses, especially infections, must be taken into consideration as possible causes of arrhythmias. Cardiac magnetic resonance imaging may be useful in patients with 'idiopathic ventricular tachycardias' to detect non-apparent myocardial lesions which may result from the underlying cause of the arrhythmia.

Imaging features, therapies, and outcomes of fibrosing inflammatory pseudotumor of the nasopharynx: A systematic review.

BACKGROUND AND PURPOSE: Fibrosing inflammatory pseudotumor (FIP) of the nasopharynx is a rare nonneoplastic inflammatory lesion that is frequently mistaken for malignancy or infection. It is often misdiagnosed by radiologists as nasopharyngeal carcinoma or lymphoma, resulting in multiple biopsies and delays in diagnosis. The purpose is to understand trends in clinical presentation, imaging findings, treatment modalities, and patient outcomes of nasopharyngeal FIP. METHODS: MEDLINE and EMBASE databases were queried for articles related to FIP of the nasopharynx. Articles that described cases of FIP involving the nasopharynx were selected. Studies that described inflammatory pseudotumor of the nasopharynx associated with known distinct pathologic entities were excluded. RESULTS: A total of 19 articles describing 37 patients were included. MRI findings were reported in 32 patients. All cases demonstrated avid gadolinium enhancement. T2-weighted imaging characteristics were described or displayed for 21 patients: 95% demonstrated hypointensity compared to surrounding mucosa. Fluorodeoxyglucose (FDG) PET/CT findings were reported for 6 patients, all demonstrating mild FDG avidity. CONCLUSIONS: Nasopharyngeal FIP is a histological diagnosis of exclusion that presents with facial pain and, often, cranial neuropathies. Relatively low T2-weighted MRI signal should inform the interpreting neuroradiologist to include FIP as a differential consideration for both the ordering provider and pathologist. Corticosteroid therapy is the most frequently employed therapy and results in partial or complete response in most patients. Radiation therapy and anti-inflammatory medications have also been reported to have potential therapeutic roles.

Paediatric Gastrointestinal, Hepatic and Pancreatic Inflammatory Myofibroblastic Tumours, A Single Centre Experience.

ABSTRACT: Inflammatory myofibroblastic tumours (IMTs) are rare soft tissue tumours. Reports of gastrointestinal tract, liver and pancreas tumours are limited. The objective of this study is to identify presenting features, contributing prognostic / etiological factors and any variability in outcomes in the context of different historical treatments. We retrospectively reviewed the records of seven children treated at our hospital between 2006 and 2019 and assessed the demographic, presentation, treatment, immunohistochemistry, and outcomes of their tumours. Age range at presentation was 4 months-15 years with a male predominance. Presentations were typically due to local mass effect or incidental discovery. Systemic symptoms were rare. Outcomes were good with six out of seven stable or in remission irrespective of treatment. Surgical resection where possible is the treatment of choice. Medical therapy had good outcomes with chemotherapy acting as first line treatment when required. The only negative prognostic factor identified was local spread at the time of presentation.

Inflammatory myofibroblastic tumor of maxillary sinus successfully treated with radiotherapy and corticosteroid: report of a rare case.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a mesenchymal tumor mainly found in lung or retroperitoneum and rarely affects head and neck region. Extensive English literature search reveals that less than fifty cases of head and neck IMT have been reported so far, maxillary sinus being fewer. CASE PRESENTATION: We present a case of IMT involving maxillary sinus in a 48-year-old gentleman who attained complete clinico-radiologic response after treatment with radiotherapy (RT) and concurrent oral prednisolone. CONCLUSIONS: This is the first report where such magnificent response was attained in primary setting treated with RT and steroids as opposed to surgery which used to be considered as standard of care till now.

Recurrent endobronchial inflammatory myofibroblastic tumors: Novel treatment options.

Endobronchial inflammatory myofibroblastic tumors (IMTs) rarely occur in children younger than 10 years of age and have intermediate malignant potential. A 7-year-old girl initially presented with pneumonia. After failing outpatient treatment, she re-presented in status asthmaticus. Computed tomography showed a left mainstem endobronchial mass which was resected bronchoscopically. Pathology was consistent with IMT. Surveillance bronchoscopy identified a recurrence. Despite a left upper lobectomy, recurrence led to further treatment with celecoxib and argon plasma coagulation. Follow-up bronchoscopy revealed complete resolution. She remains disease and symptom-free at her six-year follow-up.

IgG4-related disease with pseudotumor formation in the larynx.

Mikulicz's disease, an immunoglobulin G4-related disease (IgG4-RD) occurs frequently in the head and neck region but rarely in the larynx. We report a case of IgG4-RD with pseudotumor formation in the larynx. A 50-year-old man presented at our facility for a complete physical examination and diagnostic testing of a left arytenoid tumor. On examination, a large supraglottic mass was noted with signs of dyspnea. Movement disorder of the vocal fold was absent. The patient underwent surgery with general anesthesia to improve respiratory symptoms and a resected specimen was submitted for diagnosis. The pathology findings revealed lymph follicle formation, interstitial follicular fibrosis, angiogenesis, and inflammatory cell infiltration with plasmacytosis. Immunohistologic staining at high magnification revealed one hundred or more IgG4-positive plasma cells and fifty percent IgG4/IgG. In addition, obliterative phlebitis was observed. Medical history was positive for retroperitoneal fibrosis with serum IgG4 levels below the diagnostic criteria of IgG4-RD, but histological characteristics of IgG4-RD were met. The diagnosis of IgG4-RD was established, oral treatment with prednisolone (0.6 mg/kg/day) was initiated, and the tumor reduced in size. IgG4-RD may cause irreversible functional impairment. Early diagnosis and treatment are important in reducing the risk of permanent impairment of vocal fold mobility.

Selective arterial embolization as neoadjuvant treatment in hip pseudotumors.

OBJECTIVES: This study aims to investigate the significance of selective arterial embolization (SAE) as neoadjuvant for the treatment of pseudotumor (PT) associated with hip arthroplasty and present our case series including all the known bearing techniques. PATIENTS AND METHODS: This retrospective study included 16 patients (9 males, 7 females; mean age 75.5 years; range, 55 to 87 years) affected by hip PT. Seven patients were treated only surgically without any adjuvant treatment (group A), while nine patients were performed preoperative SAE (group B). Pseudotumors were grouped according to magnetic resonance imaging and computed tomography (CT) classifications. Durations of all operations and number of blood units given intra- and postoperatively were recorded. RESULTS: Although embolization did not change the amount of intraoperative bleeding (p=0.619), a common vascular network leading to PT was observed in all patients in angiography. Group A's mean duration of operation was shorter than group B (p=0.03); however, this condition was attributed to larger and more complex lesions of patients who underwent embolization. According to CT classification, blood loss was more and duration of operation was longer in severe stages (p=0.046 and p=0.035, respectively). CONCLUSION: Successful demonstration of vascular network in patients with PT strengthens the idea that SAE technique may be used particularly in severe cases according to CT classification. Our findings suggest that PT may develop in all commonly used bearings.

Multifocal Inflammatory Pseudotumor of the Temporal Bone, Maxillary Sinus, and Orbit.

OBJECTIVE: This is the first report of multifocal inflammatory pseudotumor (IPT) involving the temporal bone, orbit and paranasal sinus, and the use of rituximab as adjunctive therapy in multifocal temporal bone IPT. PATIENT: We describe a 46-year-old man with orbital and maxillary sinus IPT, whose disease progressed despite radiation and steroid burst. He then developed contralateral mastoid disease, otalgia, aural fullness, and hearing loss. INTERVENTION: He was initiated on rituximab and prednisone therapy. Mastoidectomy with near-total tumor removal was accomplished and histopathology confirmed IPT. A literature review was also performed. MAIN OUTCOME MEASURE: Tumor regression or recurrence. RESULT: Despite disease progression after radiation and steroids, his orbital, sinus, and mastoid disease improved after surgery, steroids, and rituximab. A review of four other previously reported cases of multifocal disease involving the temporal bone suggest that multifocal disease may be a more aggressive entity with higher recurrence rate compared with solitary disease. Although surgery and steroids are typically recommended, there is currently no consensus treatment recommendation. CONCLUSIONS: Multifocal IPT of the temporal bone is a rare but aggressive entity for which surgery and steroid combination therapy should be first line treatment. We suggest rituximab may be an effective adjunctive treatment particularly for recurrent disease or where systemic therapy may be favored.

Inflammatory myofibroblastic tumor of the pleura with adjacent chest wall invasion and metastasis to the kidney: a case report.

BACKGROUND: Inflammatory myofibroblastic tumor is a rare benign neoplasm that frequently involves the lung and abdominopelvic region, and is found mainly in children and young adults. Inflammatory myofibroblastic tumor tends to be locally invasive or recurrent, and rarely metastasizes. CASE PRESENTATION: A 76-year-old Korean man presented with a history of upper back pain for 2 months and motor weakness in both lower extremities for 2 days. Contrast-enhanced computed tomography of his chest and abdomen showed a large heterogeneous pleural mass involving the right fifth rib and vertebral body and a mass infiltrating the right renal hilum. Computed tomography-guided percutaneous needle biopsy of the pleural mass was performed. The histological findings on hematoxylin and eosin staining showed proliferation of spindle cells with infiltration of lymphocytes and plasma cells. Immunohistochemistry showed neoplastic cells positive for CD68, focally positive for smooth muscle actin, and negative for cytokeratin and desmin. Inflammatory myofibroblastic tumor was diagnosed based on the histological examination. Treatment with glucocorticoids (methylprednisolone 1 mg/kg) and radiotherapy (5 days/week for 3 weeks at 3 Gy/fraction, 45 Gy/15 days) was started. After 1 month, chest computed tomography showed a reduction in the size of the pleural mass, and abdominopelvic computed tomography showed decreased infiltration around the right renal pelvis. CONCLUSIONS: Inflammatory myofibroblastic tumor is a rare neoplasm of intermediate malignant potential due to a tendency for local recurrence and it rarely develops distant metastases. Complete surgical resection is the primary treatment. However, unresectable and metastatic inflammatory myofibroblastic tumor can be treated with systemic therapy, including glucocorticoids, radiotherapy, and/or chemotherapy.

Treatment-refractory ALK-positive inflammatory myofibroblastic tumour of the oral cavity.

We present a challenging case of a previously healthy 23-year-old man who developed an inflammatory myofibroblastic tumour of the hard palate, harbouring a rearrangement of the anaplastic lymphoma kinase (ALK) locus. Despite surgical intervention, radiotherapy and ALK-inhibition therapy, the tumour recurred locally and metastasised to regional lymph nodes, and the patient passed away roughly 9 months after diagnosis from local progression. The rapid progression of this patient's disease and its resistance to treatment demonstrate the potentially aggressive clinical course of inflammatory myofibroblastic tumours. ALK-inhibition therapy was unsuccessful in this ALK-positive tumour, highlighting the need for further investigation of markers predictive of disease progression and treatment response.

Mycobacterial spindle cell pseudotumour: epidemiology and clinical outcomes.

INTRODUCTION: Mycobacterial spindle cell pseudotumour (MSP) is a rare disease characterised by tumour-like local proliferation of spindle-shaped histiocytes containing acid-fast positive mycobacteria. The aim of this literature review is to describe the clinical parameters and treatment outcomes of patients with MSP. METHODS: A literature search was conducted using the search terms related to mycobacteria and spindle cell tumours. A previously unreported stem cell transplant recipient from our institution diagnosed with MSP was also included. Demographics, comorbidities, site of infection, treatment and clinical outcomes were analysed. RESULTS: Fifty-one patients were analysed. Twenty-six (51%) had HIV infection. Mycobacterium avium complex was the most frequent organism isolated in 24 (47.1%) followed by Mycobacterium tuberculosis complex in eight (16%) cases. Lymph nodes were the most common site of infection (45.1%). Twenty (39.2%) patients received antimycobacterial agents, 12 (23.5%) underwent surgical resection and six (11.8%) received antimycobacterial agents plus surgery. Treatment was successful in 24 (47.1%) patients and failed in 15 (29.4%); 13 of these 15 patients died. Antimycobacterial therapy was significantly associated with successful outcome compared with surgical resection or no treatment (P<0.001). CONCLUSION: MSP is a rare condition associated primarily with immunodeficiencies. Antimycobacterial therapy is significantly associated with successful outcome.

[Inflammatory myofibroblastic tumor of the hepatobiliary pancreatic system: report of three cases and literature review].

Objective: To improve the understanding, diagnosis and treatment of inflammatory myofibroblastic tumor (IMT) of the hepatobiliary pancreatic system through the cases review. Methods: The clinical data of three cases of pathologically-proved IMT of the hepatobiliary pancreatic system was reported and relevant literatures were reviewed. Results: Among three cases of IMT discussed, there consisted of a case of liver, a case of gallbladder and a case of pancreas. The clinical manifestations were nonspecific, and the preoperative diagnosis was difficult. All the patients with suspected malignant tumors underwent surgical treatment. The prognosis was good, with no recurrence. Conclusion: IMT is a benign tumor and is rare in hepatobiliary pancreatic system. The clinical manifestations are not typical and the imaging examinations are not specific. Its diagnosis depends on pathology. Surgery is an effective treatment. IMT has a tendency of recurrence, and its postoperative follow-up is of great significance.

Primary Hepatic Lymphoma Complicated by a Hepatic Inflammatory Pseudotumor and Tumor-Forming Pancreatitis.

BACKGROUND: Hepatic inflammatory pseudotumor (IPT) is considered to be benign in biological behavior, and its malignant transformation is extremely rare. There has only been one published case of primary hepatic lymphoma complicated by hepatic IPT. CASE PRESENTATION: A 73-year-old man presented with obstructive jaundice and a pancreatic head mass. Histology of the mass revealed chronic pancreatitis with lymphoid follicle formation, leading to a diagnosis of a suspicion of follicular pancreatitis. After a choledochojejunostomy, a hepatic tumor was detected, and a biopsy revealed lymphoplasmacytic infiltration. Immunohistochemistry confirmed the polyclonal nature of lymphoplasma cells, indicative of an IPT. The hepatic tumor disappeared during follow-up, but the patient exhibited a high fever related to tumor recurrence. A biopsy revealed the co-existence of a diffuse large B-cell lymphoma and an IPT. IgG4-related disease was excluded because storiform fibrosis, obliterative phlebitis, and a significant increase in IgG4-immunoreactive cells were absent in all investigated tissues. The tumor completely disappeared after chemotherapy. CONCLUSION: Careful observation is necessary in this kind of situation because the presence of a hepatic IPT may represent an increased risk of malignant transformation.