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Multicentric extra-abdominal desmoid tumors arising in bilateral lower limbs
Tokiko Shimoyama1, Koji Hiraoka, Takanori Shoda
1Department of Orthopaedic Surgery, Kurume University School of Medicine, Kurume, Japan.
Rare Tumors
|December 9, 2010
Summary
Extra-abdominal desmoid tumors, rare in young adults, typically affect limbs and trunk. This case highlights a rare multicentric occurrence in both lower limbs, expanding understanding of tumor distribution.
Area of Science:
- Oncology
- Pathology
- Surgical Oncology
Background:
- Desmoid tumors are fibroblastic neoplasms characterized by local invasion and high recurrence rates.
- Extra-abdominal desmoid tumors (EADTs) are uncommon, predominantly affecting the trunk and proximal extremities of young adults.
- Multicentricity in EADTs is a rare phenomenon, suggesting distinct biological behavior or predisposition.
Observation:
- This report details an exceptionally rare case of multicentric extra-abdominal desmoid tumors.
- The tumors manifested bilaterally within the lower limbs, an unusual location for multicentric EADTs.
- The patient was a young adult, aligning with the typical demographic for desmoid tumors.
Findings:
- The occurrence of multiple, distinct desmoid tumors in both lower extremities simultaneously is highly unusual.
- This presentation challenges the commonly observed distribution patterns of EADTs.
- The findings underscore the potential for EADTs to arise in diverse and unexpected anatomical sites.
Implications:
- This case expands the known spectrum of clinical presentations for extra-abdominal desmoid tumors.
- It suggests that clinicians should maintain a high index of suspicion for multicentric EADTs, even in atypical locations.
- Further research into the genetic and molecular underpinnings of multicentric EADTs may elucidate specific risk factors or pathogenic mechanisms.