Congenital infantile fibrosarcoma (CIF) is a rare tumor in children that occurs in the first years of life. It usually arises in the extremities but some cases affect the trunk, neck, abdomen, or retroperitoneum. Surgical resection has been traditionally the treatment of choice but the development of genomic analysis and targeted therapies has shed light on new therapeutic options.
View Article and Find Full Text PDFEur J Vasc Endovasc Surg
September 2021
Infantile fibrosarcoma (IFS) is a rare pediatric tumor which often presents the gene fusion. encodes the neurotrophin-3 growth factor receptor tyrosine kinase, a druggable therapeutic target. Selective tropomyosin receptor kinase (TRK) inhibitors, such as larotrectinib, have shown efficacy and safety in the treatment of IFS.
View Article and Find Full Text PDFJ Vasc Surg Venous Lymphat Disord
November 2020
Background: Venous malformations (VMs) are the most frequent congenital vascular malformations. Pain and deformity are the main symptoms and usually progressive in untreated patients. Current therapeutic options are surgery (frequently partial resections with an uncomfortable postoperative period), sclerotherapy (often limited because of the need for high doses and the potential subsequent toxicity), and percutaneous intralesional laser treatment.
View Article and Find Full Text PDFParkes Weber syndrome (PWS) is characterized by the association of high flow vascular malformation and overgrowth of a part of the body, usually a limb. In a previous review of 10 patients with PWS from our hospital we described a case of congenital short femur and four cases of severe lymphedema. We present a case of PWS associated with a nodular proliferative form not previously described.
View Article and Find Full Text PDFWe present the case of a 9-year-old boy diagnosed with blue rubber bleb nevus syndrome, who showed a very large left cervical cystic lymphangioma. He was previously subjected to various treatments for lesions in the intestinal tract including blood transfusions for anemia, sclerosis, enterotomies or resections. The tumor was resected without any complications and the anatomopathologic report confirmed this diagnosis.
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