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Primary mesenteric angiosarcoma in a child with associated lymphangiectasia: a case report
E C C Castro1, C Galambos, Peter H Shaw
1Department of Pediatric Pathology and Division of Pediatric Hematology/Oncology, Children's Hospital of Pittsburgh, Pittsburgh, PA 15213, USA.
Summary
Mesenteric angiosarcoma is a rare childhood cancer, often a secondary malignancy. This case highlights a rare presentation in a child with acute abdomen and associated lymphangiectasia, emphasizing the need for awareness of this rare tumor.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Vascular Tumors
Background:
- Angiosarcomas are rare vascular neoplasms with distinct epidemiological profiles in pediatric versus adult populations.
- Pediatric angiosarcomas typically manifest in soft tissues or the liver, differing from adult presentations often linked to extremities and lymphedema.
- Mesenteric angiosarcoma is exceptionally rare in children, with adult cases frequently associated with prior Hodgkin's lymphoma.
Observation:
- A pediatric patient presented with acute abdominal pain, necessitating emergency surgical intervention.
- Intraoperative findings revealed a mesenteric angiosarcoma.
- The tumor was associated with lymphangiectasia of the bowel and mesentery, a rare co-occurrence.
Findings:
- The case details a rare instance of primary mesenteric angiosarcoma in a child.
- Histopathological examination confirmed angiosarcoma with concurrent lymphangiectasia.
- Literature review and discussion on nomenclature for these unusual tumors are included.
Implications:
- This report expands the understanding of rare pediatric vascular tumors.
- Highlights the importance of considering rare diagnoses in pediatric acute abdomen.
- Contributes to the literature on mesenteric angiosarcoma and associated lymphatic abnormalities.