A rare inflammatory myofibroblastic tumor appearing both inside and outside the heart.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is an uncommon cardiac tumor that primarily affects infants, children, and young adults. While complete surgical resection generally leads to a favorable prognosis, accurate diagnostic tests remain limited. CASE PRESENTATION: We describe the case of a 26-year-old female who had a dual tumor inside and outside the heart and was misdiagnosed by echocardiography and MRI. We also review 71 cases of cardiac IMTs from the literature regarding their epidemiology, clinical presentation, and outcome. CONCLUSION: Early detection of this rare disorder is essential for optimal surgical management.

The Potential Utility of 18 F-FDG PET/CT in Monitoring Corticosteroid Therapy in Inflammatory Pseudotumor.

ABSTRACT: A 69-year-old man with suspected gastrointestinal stromal tumor was referred to 18 F-FDG PET/CT. Images showed increased metabolism in a jejunal wall thickening, 2 liver lesions, and hepatic lymph nodes. The patient underwent wedge biopsy of the liver, which revealed inflammatory pseudotumor. The patient was treated with 20 mg/d prednisone, with a gradual dose reduction. A partial metabolic response was achieved after 2 months of therapy, and a final PET/CT showed complete metabolic response after 9 months. This clinical case shows the potential role of PET/CT in the assessment of the response of the inflammatory pseudotumor to corticosteroid therapy.

A surgical case of inflammatory pseudotumor by hepatic anisakiasis.

Anisakiasis is a parasitic infection caused by the ingestion of raw or undercooked seafood infected with Anisakis larvae. It generally affects the gastrointestinal tract, particularly the stomach, but very rare cases have been reported in which infection of the liver leads to the formation of inflammatory pseudotumors. We herein report an extremely rare case of an inflammatory pseudotumor induced by hepatic anisakiasis that was laparoscopically resected for the purpose of both diagnosis and treatment. A 51-year-old woman underwent a routine medical checkup by ultrasound examination, which incidentally detected a 15-mm mass on the surface of S6 of the liver. Because a malignant tumor could not be ruled out on several preoperative imaging studies, laparoscopic partial resection of the liver was performed. Histopathological examination revealed Anisakis larva in the inflammatory pseudotumor, suggesting hepatic anisakiasis. This report describes an extremely rare case of an inflammatory pseudotumor induced by hepatic anisakiasis. Because the preoperative diagnosis could not be obtained by several imaging modalities, laparoscopic liver resection with a sufficient margin might be suitable for diagnosis and treatment of this disease.

Inflammatory Pseudotumour Of The Urachus: Case Report And Literature Review.

A 52 year old woman presented to the emergency department of Affiliated Hospital of Zunyi Medical University, Zunyi, China in May 2022, complaining of a palpable lower abdominal mass since two days. She denied haematuria, umbilical drainage, or any other urinary symptoms. Previous health record indicated that the patient was diagnosed with urachal inflammatory pseudotumour. Inflammatory pseudotumourous masses of the urachal canal are rare chronic inflammatory disorders with only a few case reports. Ultrasonography is the preferred method for diagnosing urachal lesions. Contrast- enhanced ultrasonography (CEUS) allows real-time visualization of the microvascular blood flow within the solid lesion, reducing the probability of misdiagnosis of the disease. We have reported a case of urachal inflammatory pseudotumour and analyzed its ultrasonographic findings from two-dimensional conventional ultrasonography and CEUS to provide support for the diagnosis of urachal inflammatory pseudotumour in the clinic and to assist clinical selection of effective treatment modalities.

IgG4-related hepatic inflammatory pseudotumor in a patient with serum IgG4-negative type 1 autoimmune pancreatitis.

IgG4-related disease (IgG4-RD) can cause heterogeneous lesion in various organs. Serum IgG4 levels are useful in monitoring patients with IgG4-RD; however, when it is negative, more careful observation is required. A 58-year-old woman who had been diagnosed with serum IgG4-negative type 1 autoimmune pancreatitis (AIP) 3 years prior visited our hospital for the evaluation of a liver tumor. She had visited a nearby hospital 1 month prior with complaints of a swelling in her right neck, and histological examinations were suggestive of IgG4-related sialadenitis. A positron emission tomography scan showed fluoro-deoxy-glucose accumulation in her right liver lobe; therefore, she was referred to our hospital. Liver tumor biopsy showed inflammatory cell infiltration and storiform fibrosis, without histological findings indicative of a malignancy. Many IgG4-positive cells were detected in immunostaining; thus, an IgG4-related hepatic inflammatory pseudo-tumor was diagnosed. After increasing in steroid dosage, the patient remained recurrence-free with 2 years. To our knowledge, this is the first report of mass-forming IPT for serum IgG4-negative type 1 AIP. Occasionally, IgG4-related IPT may appear in the periphery of the liver, and serum IgG4-negative cases should be more carefully observed because serum IgG4 is not an indicator.

Infantile inflammatory myofibroblastic tumour of the sigmoid colon: a diagnostic dilemma.

An inflammatory myofibroblastic tumour (IMT) is an uncommon neoplasm composed of inflammatory cells and myofibroblasts in a fibrous stroma. They are mostly seen in the lungs and rarely involve the gastrointestinal tract. An 8-month-old infant presented with a history of lower abdominal lump for 2 months. Her CT scan confirmed a large, lobulated mass in the retroperitoneum arising from the pelvis. The mass was found to be arising from the sigmoid colon on laparotomy which was excised. Histopathology showed a cellular tumour composed of spindle cells and inflammatory lymphocytic infiltrate. Immunohistochemistry revealed positive staining for anaplastic lymphoma kinase and smooth muscle actin, confirming the diagnosis of IMT. The patient is doing well at her 6-month follow-up. Ours is the youngest case of sigmoid IMT among the only other series of eight cases reported in the literature indicating its rarity.

Successful treatment with laparoscopic extended posterior sectionectomy for inflammatory pseudotumor in the liver with para-aortic lymphadenitis.

Inflammatory pseudotumor (IPT) is a rare disease that requires a differential diagnosis from malignancies. We describe a case of hepatic IPT with para-aortic lymphadenopathy, treated with a stepwise strategy of laparoscopic surgery. A 61-year-old woman was referred with a liver lesion. Computed tomography revealed a 13 cm well-defined lesion in segments VII-VI. The patient also had bead-like enlarged lymph nodes from the perihilar to the para-aortic regions. Although percutaneous lymph node biopsy showed no evidence of malignancy, 18 F-fluorodeoxyglucose positron emission tomography revealed accumulation in the lesion and lymph nodes. Lymph nodes were harvested laparoscopically for intraoperative pathological examination. With no evidence of malignancy, laparoscopic liver resection was continuously performed as a diagnostic treatment. The patient was given a pathological diagnosis of IPT and was discharged on the 16th day and is well 2 years after surgery. The minimally invasive laparoscopic approach to diagnostic treatment could be useful with secure advantages.

Giant inflammatory pseudotumor of the lung: a case report.

The inflammatory myofibroblastic tumor, which was first described in 1939, is a rare neoplasm that accounts for 0.04%-0.7% of all lung neoplasms. These neoplasms occur most often in children, as they are the most common primary lung tumors in children. Preoperative diagnosis of such patients using bronchoscopy with endoluminal biopsy and transthoracic biopsy is not always informative and often the diagnosis can only be established during surgery. The presented case shows that on rare occasions, a giant myofibroblastic tumor of the lung may be encountered in adults, and radical intervention with subsequent rehabilitation can lead to full recovery.

Large Inflammatory Myofibroblastic Tumor of the Esophagus: A Case Report and Literature Review.

Inflammatory myofibroblastic tumor (IMT) is a rare tumor composed of myofibroblasts with inflammatory blood cell infiltration. It commonly occurs in the lungs and rarely in the esophagus. We herein report a valuable case of IMT originating in the esophagus. A 60-year-old Japanese woman with dysphagia had a large subepithelial lesion in the cervical esophagus, which was 15 cm in length. Surgical resection was performed to confirm the pathological diagnosis and improve the symptoms. The postoperative diagnosis was IMT composed of multiple nodules. There was no recurrence or metastasis within one year after surgery.

Inflammatory myofibroblastic tumor of the lung involving the central nervous system in pediatric patients.

INTRODUCTION: The inflammatory myofibroblastic tumor (IMT) is a very rare lesion with an incidence of less than 0.1% of total neoplasms and with main affection in the lungs. Involvement in the central nervous system is extremely rare, but with a much more aggressive course than IMT diagnosed in the rest of the body. We report the 2 cases presented in our neurosurgery department to date; both were treated satisfactorily without intercurrences in 10 years of follow-up. HISTORICAL BACKGROUND: The World Health Organization described the IMT as a distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. CLINICAL PRESENTATION: Clinical manifestations of patients with CNS IMT vary and may consist of headache, vomiting, seizures, and blindness. Seizures are the most common symptom in patients with focal lesions. DIAGNOSIS: The true origin of this entity remains to be elucidated, but to date, etiologies ranging from chromosomal alterations to autoimmune or postinfectious mechanisms have been described. Due to its rarity and non-specificity in imaging, the final diagnosis of IMT in the brain parenchyma relies on pathological examination. MANAGEMENT: Treatment options are controversial and include total or subtotal removal, high-dose steroids, and radiation therapy. In the last decade, the development of ALK Tyrosine Kinase Inhibitors allows the possibility of chemotherapy in those patients harboring ALK mutations. CONCLUSION: IMT is a rare tumor that can exceptionally be found in the CNS. The cause is still unknown although the different studies focus on a neoplastic origin. The diagnosis is based in the use of different modalities of imaging and with histological confirmation. Optimal management is gross total resection whenever possible, is the only established curative treatment. Further research with longer follow-up is needed to clarify the natural history of this rare tumor.

The MRI features of renal inflammatory pseudotumor: A case report and literature review.

RATIONALE: Inflammatory pseudotumor is rare observed in renal immunoglobulin G4-related disease. PATIENT CONCERNS: A 65-year-old female presented with a mass in the right kidney which was found in physical examination. DIAGNOSES: Based on the imaging findings and clinical manifestations, we preliminarily judged that the mass of the right kidney was renal cell carcinoma. INTERVENTIONS: The patient finally underwent total nephrectomy. OUTCOMES: The final result of microscopic pathological examination is renal inflammatory pseudotumor. LESSONS: There are some characteristics on magnetic resonance imaging of renal inflammatory pseudotumor, which can improve diagnosis rate by combining with medical history and clinical manifestations.

IgG4-negative pituitary inflammatory pseudotumor with sphenoidal involvement resembling a macroadenoma.

BACKGROUND: Inflammatory pseudotumors (IPTs) are rare benign conditions of unknown etiology that can affect any part of the body. IPTs are most commonly associated with Immunoglobulin G4 (IgG4)-related disease. Central nervous system IPTs, especially with pituitary involvement, are even rarer entities. The presence of an IgG4-negative pituitary IPT with simultaneous extracranial involvement has not been reported. CASE REPORT: We present the case of a 41-year-old female with past medical history of rheumatoid arthritis and a diagnosis of pituitary IPT with coexisting sphenoidal (extracranial) involvement mimicking a pituitary macroadenoma at presentation. The patient underwent multiple consecutive biopsies, and an extensive workup prior to establishing the diagnosis. Laboratory work-up showed normal serum IgG4 and unremarkable liver function tests. CONCLUSION: Pituitary lesions with simultaneous sphenoidal involvement in patients with IgG4-negative systemic inflammatory disease should raise the clinical suspicion for intracranial IPTs, as these tumors can mimic aggressive counterparts causing adjacent bony erosion, and local invasion.

IgG4-related Inflammatory Pseudotumor with Imaging Findings Similar to Meningioma.

IgG4-related inflammatory pseudotumor is a feature of IgG4-related disease and develops in various organs. Intracranial IgG4-related inflammatory pseudotumor is rare, and data on the clinical course and response to treatment are insufficient in the literature. We herein report a patient with IgG4-related inflammatory pseudotumor who had magnetic resonance imaging findings similar to meningioma. Tumorectomy was discontinued because of the intraoperative rapid diagnosis, which revealed the infiltration of lymphocytes and plasma cells. She received oral prednisolone therapy for IgG4-related inflammatory pseudotumor, and the tumor size had significantly decreased after six months of treatment.

Pseudotumor inflamatorio hepático, una entidad de difícil diagnóstico / Inflammatory pseudotumor of the liver: a condition hard to diagnose

RESUMEN El pseudotumor inflamatorio hepático es una lesión muy infrecuente, sin una etiología ni patogenia claras. Su diagnóstico preoperatorio no es habitual pero, en caso de realizarse, puede evitar la cirugía. Presentamos el caso de un paciente joven, con antecedente de lupus cutáneo que, tras debutar con una pancreatitis aguda, presenta episodios de colangitis y cuyos hallazgos radiológicos no permiten descartar la presencia de un colangiocarcinoma, por lo que se realiza hepatectomía izquierda, siendo el diagnóstico histológico final de pseudotumor inflamatorio hepático.

ABSTRACT Inflammatory pseudotumors of the liver are rare and lack clear etiology and pathogenesis. The preoperative diagnosis is seldom made but it avoids unnecessary surgery. We report the case of a young male patient with a history of cutaneous lupus and episodes cholangitis after an acute pancreatitis. As the imaging tests could not rule out cholangiocarcinoma, a left liver resection was performed, and the final histologic diagnosis was inflammatory pseudotumor of the liver.