An inflammatory myofibroblastic tumor (IMT) is an uncommon, benign tumor of myofibroblastic spindle cells. An IMT can occur in any part of the body. However, involving kidney is exceedingly rare. When this rare entity occurs in children, it becomes incredibly challenging to distinguish this rare entity from other malignancies such as Wilms tumor. Although imaging studies of the abdomen and pelvis add to the diagnosis, however, histological examination and immunohistochemical staining remain the gold standard for the precise diagnosis of this rare entity. To the best of our knowledge, only 48 cases of renal IMT have been published in the medical literature so far. We report the case of a nine-month-old girl who was brought with complaints of hematuria, and later, imaging and histological confirmation revealed an anaplastic lymphoma kinase (ALK)-negative IMT of the kidney.
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http://dx.doi.org/10.7759/cureus.23289 | DOI Listing |
Nat Rev Urol
January 2025
Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA.
Approximately 20% of paediatric and adolescent/young adult patients with renal tumours are diagnosed with non-Wilms tumour, a broad heterogeneous group of tumours that includes clear-cell sarcoma of the kidney, congenital mesoblastic nephroma, malignant rhabdoid tumour of the kidney, renal-cell carcinoma, renal medullary carcinoma and other rare histologies. The differential diagnosis of these tumours dates back many decades, when these pathologies were identified initially through clinicopathological observation of entities with outcomes that diverged from Wilms tumour, corroborated with immunohistochemistry and molecular cytogenetics and, subsequently, through next-generation sequencing. These advances enabled near-definitive recognition of different tumours and risk stratification of patients.
View Article and Find Full Text PDFR I Med J (2013)
February 2025
Department of Medicine, Division of Cardiology, Alpert Medical School of Brown University, Providence RI.
Cardiac amyloidosis (CA) is an infiltrative disease that results from the deposition of amyloid fibrils in the myocardium, resulting in restrictive cardiomyopathy. The amyloid fibrils are predominantly derived from two parent proteins, immunoglobulin light chain (AL) and transthyretin (ATTR), and ATTR is further classified into hereditary (ATTRv) and wild-type (ATTRwt) based on the presence or absence, respectively, of a mutation in the transthyretin gene. Once thought to be a rare entity, CA is increasingly recognized as a significant cause of heart failure due to improved clinical awareness and better diagnostic imaging.
View Article and Find Full Text PDFArch Argent Pediatr
January 2025
Hospital Italiano de Buenos Aires, City of Buenos Aires, Argentina.
Hyperparathyroidism is a rare entity in pediatrics. It is defined as the increased production of parathyroid hormone. It may be due to a primary defect of the parathyroid glands (primary hyperparathyroidism) or to a compensatory parathyroid hormone production to correct hypocalcemia states of various origins (secondary hyperparathyroidism).
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Immunodermatology, National Medical Institute of the Ministry of the Interior and Administration, Warsaw, Masovian, Poland.
Linear IgA bullous dermatosis (LABD) is a rare subepidermal blistering disorder characterized by the presence of linear IgA deposits at the basement membrane zone (BMZ) by direct immunofluorescence (DIF). This entity was first described by Chorzelski and Jablonska from Warsaw Center of Bullous Diseases, Poland. The disease affects children and adults, whereby they differ in terms of clinical picture and course.
View Article and Find Full Text PDFCase Rep Pediatr
January 2025
Department of Internal Medicine, Lebanese Hospital Geitaoui University Medical Center, Beirut, Lebanon.
Red ear syndrome (RES) is a rare clinical entity presenting with paroxysmal erythema of one or both ears associated with a burning sensation or earache. The onset of symptoms could be either spontaneous or triggered by touch, stress, coughing, sneezing, neck movements, chewing, and combing hair. While most cases are usually described in adults, it can rarely present in children.
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