Kaposiform hemangioendothelioma (KHE) is a vascular tumor of very low incidence, which occurs mostly in children and infants. The tumor is recognized for its locally aggressive, yet rarely metastatic behavior. It may cause consumptive coagulopathy known as Kasabach-Merritt phenomenon. We report a distinctive case, where an 11-year-old boy is presented with progressive thoracolumbar scoliosis without any symptom or neurological sign. The patient underwent spinal deformity correction via posterior pedicle screw instrumentation and fusion, along with tumor biopsy. The pathology report confirmed KHE. The patient did not show a prominent progression of scoliosis after the surgery without any further treatments. . Many of scoliotic patients do not have any apparent cause, thereby regarded as idiopathic scoliosis. The presented case is where kaposiform hemangioendothelioma is likely to be linked to the patient's scoliosis. We demonstrate the possibility of secondary scoliosis should always be kept in mind of orthopaedic doctors. We also conclude that secondary scoliosis does not show exacerbation after growth completion.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7029270 | PMC |
| http://dx.doi.org/10.1155/2020/1839053 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Neonatal Cutaneous Vascular Anomalies.
Neoreviews
January 2025
Vascular Anomalies Center, Division of Pediatric Surgery, Children's Hospital Colorado, Aurora, Colorado.
Vascular anomalies are broadly classified into 2 categories: vascular tumors and vascular malformations. Vascular anomalies frequently present as cutaneous lesions in infants. This review summarizes vascular anomalies that most commonly present as dermatologic lesions in the neonatal period, with a focus on the clinical findings, pathophysiology and histology, relevant radiographic findings, and management of common vascular anomalies such as infantile hemangiomas, congenital hemangiomas, and Kaposiform hemangioendothelioma, along with vascular malformations, including capillary, lymphatic, venous, and arteriovenous malformations.
View Article and Find Full Text PDFAbdominal kaposiform hemangioendothelioma presenting as volvulus and recurring intestinal obstruction in a neonate: a case report.
J Med Case Rep
December 2024
Department of Surgery, Aga Khan University, Karachi, Pakistan.
Background: Kaposiform hemangioendothelioma is a rare vascular tumor primarily occurring in infants and children. The most common sites for kaposiform hemangioendothelioma are extremities, with very few cases of abdominal kaposiform hemangioendothelioma reported in neonates. Making a diagnosis of Kaposiform hemangioendothelioma can be challenging when the patient presents with generalized symptoms such as bilious vomiting and constipation that can be attributed to other more common causes of intestinal obstruction.
View Article and Find Full Text PDFHemostasis and thrombosis risks and management in vascular anomalies.
Hematology Am Soc Hematol Educ Program
December 2024
Pediatric Hematology-Oncology, University of Arkansas for Medical Sciences, Little Rock, AR.
The role of the hematologist in the management of vascular anomalies is evolving. Several vascular tumors and malformations are associated with complex coagulation derangements. Kaposiform hemangioendothelioma or tufted angiomas may present with a consumptive coagulopathy known as the Kasabach-Merritt phenomenon (KMP).
View Article and Find Full Text PDFAdvances in vascular anomalies: refining classification in the molecular era.
Histopathology
December 2024
Department of Pathology, Boston Children's Hospital, and Harvard Medical School, Boston, Massachusetts, USA.
Article Synopsis
- The understanding of vascular anomalies has progressed since the original classification by Mulliken and Glowacki, with current research focusing on distinguishing vascular tumors from malformations.
- Recent advancements in molecular diagnostics have improved the identification and management of complex vascular lesions, including Kaposiform hemangioendothelioma and intramuscular fast-flow anomalies.
- The review discusses the genetic mutations involved, particularly in the RAS/PI3K/mTOR pathways, and highlights new targeted treatments like PI3K and MEK inhibitors that can enhance patient outcomes.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!