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Gardner's syndrome: a case report and review of the literature
C Fotiadis1, D-K Tsekouras, P Antonakis
18 Tripoleos Street, Melissia, Athens 15721, Greece. costfot@yahoo.gr
Insights
Gardner's syndrome, a genetic disorder, can manifest with colon polyps, bone tumors, and soft tissue growths. This case highlights a successful treatment approach for an 11-year-old girl with a desmoid tumor and Gardner's syndrome.
Area of Science:
- Genetics and Oncology
- Gastroenterology
- Surgical Pathology
Background:
- Gardner's syndrome is an autosomal dominant disorder.
- Characterized by colonic polyposis, osteomas, and soft tissue tumors.
- Presents with diverse colonic and extracolonic symptoms across a wide age range.
Observation:
- An 11-year-old female presented with a lumbar desmoid tumor.
- The patient was diagnosed with Gardner's syndrome.
- The case involved both colonic and extracolonic manifestations.
Findings:
- The patient underwent surgical management.
- Treatment included desmoid tumor resection.
- Restorative proctocolectomy and ileal pouch anal anastomosis were performed.
Implications:
- This case demonstrates a comprehensive surgical strategy for Gardner's syndrome.
- Highlights the importance of multidisciplinary management for complex cases.
- Contributes to understanding treatment outcomes for desmoid tumors in this syndrome.
Abstract:
Gardner's syndrome is an autosomal dominant disease characterized by the presence of colonic polyposis, osteomas and a multitude of soft tissue tumors. The syndrome may present at any age from 2 mo to 70 years with a variety of symptoms, either colonic or extracolonic. We present a case of a 11-year-old female patient with Gardner's syndrome who presented with a lumbar area desmoid tumor and treated with resection of the desmoid, restorative proctocolectomy and ileal pouch anal anastomosis, A review of the current literature has been performed.
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