Case Report: Unusual Clinical Presentation of a Rare Cardiac Inflammatory Myofibroblastic Tumor in Children: The Differential Diagnosis With Pediatric Emergencies

Abstract

Introduction: There are still no guidelines about pediatric cardiac cancers. The purpose of this work is to provide new scientific data facilitating the differential diagnosis of a rare cardiac tumor with an unusual presentation, such as the cardiac inflammatory myofibroblastic tumor (IMT).Case Presentation: A 3-year-old male child presented with several symptoms including unconsciousness, vomiting, and drowsiness. A clinical and neurological examination revealed a unilateral (right) motor delay and positive unilateral Babinski sign. Electrocardiogram (ECG) was normal.Diagnostic Assessment: The total body computed tomography (CT) scans showed hypodensity in the left temporal–parietal lobe, a large hypodense area in the right frontal lobe, and a second area in the left frontal lobe were found with head CT. A magnetic resonance (MR) also noted cerebral areas of hypointensity. The echocardiographic images revealed an ovoid mass, adherent to the anterolateral papillary muscle. The histological exams, performed with hematoxylin–eosin, Masson's trichrome, Alcian blue PAS, Weigert and Van-Gieson stain, allowed observing the microscopic structure of the neoplastic mass. The immunohistochemical analysis was performed through subsequent antibodies: anti-vimentin, anti-actina, anti-ALK, anti-CD8, anti-CD3, anti-CD20, anti-kappa and lambda chains, and anti CD68 antibodies. The healthcare professionals diagnosed a cardiac IMT with brain embolism.Differential Diagnosis: The ventricular localization, observed through radiological exams, required a differential diagnosis with fibroma and rhabdomyoma, the presence of brain embolism with sarcoma, and its morphology with fibroma. Neurological symptoms might be attributed to encephalitis, primitive cerebral cancer, such as astrocytoma or neuroblastoma, cerebral metastases due to any malignancy, or embolic stroke.Conclusion: New studies are encouraged to better define IMT behavior and draw up guidelines confirming the crucial role of multidisciplinary approach and treatment protocol selected on the basis of the characteristics of the tumors, in the case of this rare type of cancer.

A fatal case of paediatric post-traumatic pyomyositis

“…Further, a multidisciplinary approach is needed when dealing with unexplained infant deaths. [17][18][19][20][21]…”

Out of the blue: inflammatory myofibroblastic tumour identified during repair of tetralogy of Fallot with absent pulmonary valve

“…Immunohistochemical analysis demonstrates that tumour cells express actin and vimentin, inconsistently express epithelial membrane antigen, CD31 and CD163, and do not express ALK1, S100, or CD34. [1][2][3][4][5][6] Whenever possible, complete resection of cardiac inflammatory myofibroblastic tumour is the definitive treatment. 6 When complete resection is not an option, partial resection while preserving cardiac function is the goal.…”

“…3 Failure to treat these benign neoplasms can be catastrophic due to direct coronary invasion, venous thromboembolism, or sudden death. 3,4 When managed surgically, cardiac inflammatory myofibroblastic tumours have a very favourable outcome.…”

Inflammatory myofibroblastic tumour of the mitral valve

“…The presentation of this disease with cerebral infarct is rare 5 . However, it will affect the prognosis of the children and leave them with sequelae.…”