Abstract:We report a case of a child with a right ventricular inflammatory myofibroblastic tumor (IMT) who presented with fever, viral symptoms, and abdominal discomfort. Including this case, 49 intracardiac tumors have been previously reported in all age groups. The majority of intracardiac IMTs occur in pediatric patients, with approximately half presenting in children aged <12 months. Intracardiac IMTs are generally described as benign tumors; however, depending on their location, the initial presentation may… Show more
“…56 In addition to myocardial infarction and tumour site derived symptoms, one-third of patients present with constitutional symptoms, such as fever, weight loss, malaise, and unspecific viral respiratory or gastrointestinal viral symptoms. 55,57 Echocardiographically, inflammatory myofibroblastic tumours appear as a heterogeneous mass arising from the endocardium. At cardiac magnetic resonance, the mass appears as isointense on T1, hypointense on T2 weight images and demonstrates intense delayed contrast enhancement.…”
Cardiac tumours are extremely rare in children. Although more than 90% are benign, children can develop obstructive or embolisation derived symptoms, arrhythmias, constitutional symptoms, cardiac tamponade, or even sudden death. Although the majority are asymptomatic, and some spontaneously regress, appropriate follow-up is needed on a case-by-case basis, as patients may develop late symptoms. Definitive diagnosis is only possible through histological analysis; however, it is possible to infer tumour type with a high grade of certainty based on imaging features, particularly cardiac magnetic resonance. Surgical resection is advocated for those causing symptoms, obstruction, cardiac dysfunction, and high risk of embolisation. The aim of this review is to present the state of the art related to cardiac tumours in the paediatric population, in the context of our own experience.
“…56 In addition to myocardial infarction and tumour site derived symptoms, one-third of patients present with constitutional symptoms, such as fever, weight loss, malaise, and unspecific viral respiratory or gastrointestinal viral symptoms. 55,57 Echocardiographically, inflammatory myofibroblastic tumours appear as a heterogeneous mass arising from the endocardium. At cardiac magnetic resonance, the mass appears as isointense on T1, hypointense on T2 weight images and demonstrates intense delayed contrast enhancement.…”
Cardiac tumours are extremely rare in children. Although more than 90% are benign, children can develop obstructive or embolisation derived symptoms, arrhythmias, constitutional symptoms, cardiac tamponade, or even sudden death. Although the majority are asymptomatic, and some spontaneously regress, appropriate follow-up is needed on a case-by-case basis, as patients may develop late symptoms. Definitive diagnosis is only possible through histological analysis; however, it is possible to infer tumour type with a high grade of certainty based on imaging features, particularly cardiac magnetic resonance. Surgical resection is advocated for those causing symptoms, obstruction, cardiac dysfunction, and high risk of embolisation. The aim of this review is to present the state of the art related to cardiac tumours in the paediatric population, in the context of our own experience.
“…Therefore, the variety of signs and symptoms can be presented by this tumour. Pang et al 11 reported a case of the right ventricular IMT in an 11-month-old infant presented with febrile illness. Oh et al 20 described an IMT in the appendix of an 85-year-old man with the history of gastric cancer.…”
Inflammatory myofibroblastic tumour (IMT) is a rare neoplasm, occurring most often in children and young adults. IMTs have intermediate biological behaviour with the chance of local invasion, recurrence and even distant metastasis. Wide range of clinical presentations makes the precise diagnosis of IMT more challenging. The best method for definitive diagnosis is tissue biopsy and newer imaging modalities including fleurodeoxyglucose (FDG) positron emission tomography (PET)/CT are useful tools in detection of disease recurrence or distant metastasis. Complete surgical resection is the best-known treatment for this tumour. Here we are presenting an IMT case in a 12-year-old girl in which her recurrent pulmonary IMT was diagnosed based on FDG PET/CT findings and referred for further salvage treatment. Overall imaging modalities are not specific, but PET/CT scan can be useful tool for evaluation of IMT regarding initial staging and restaging to assess treatment response and recurrence.
“…Scientists believe that cytogenetic abnormalities play an important role. Apart from its biological characteristics, the discovery that IMT may harbor chromosomal rearrangements at the 2p23 Locus, where the gene site for anaplastic lymphoma kinase (ALK) is, also firmed the borderline neoplasm property of IMT[ 6 - 8 ]. According to several case reports, approximately 30 kinds of genetic rearrangement have been found in IMT, most of which are ALK fusion genes[ 9 - 12 ].…”
BACKGROUND
A biliary inflammatory myofibroblastic tumor (IMT) is a rare type of mesenchymoma that, although it has a broad age spectrum, usually occurs in adults. Diagnosis is difficult because biliary IMTs often exhibit nonspecific clinical symptoms and imaging features, resulting in delayed or inappropriate treatment. Although most IMTs are benign, some show malignant properties such as infiltration, recurrence, and metastasis.
CASE SUMMARY
Here, we retrospectively describe a 10-month-old infant who was admitted to our hospital due to stubborn jaundice. The patient responded poorly to routine medical treatment and his clinical manifestations and laboratory tests lacked specificity, so we turned to repeated ultrasound scans and other imaging examinations. As both hepatosplenic ultrasonography and diffusion-weighted magnetic resonance imaging demonstrated a space-occupying lesion, an exploratory laparotomy was performed. The final diagnosis made over two mo after the disease onset was infant biliary cirrhosis caused by a biliary IMT, which partially infiltrated into the liver. This infant is the youngest case of biliary IMTs that has been reported till now. The patient underwent an incomplete resection of the mass and Kasai Portoenterostomy. However, because of cirrhosis, he also received a paternal liver transplant. Since some IMTs show malignant properties, we proceeded with a three-year of follow-up; however, no recurrence or metastasis has been noted.
CONCLUSION
Neoplastic disease such as IMTs should be considered when routine medical treatment of obstructive jaundice is not successful. Observation of dynamic imaging changes is helpful for diagnosis. Periodic follow-up is necessary for IMTs.
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