Work

Functional shoulder girdle reconstruction in paediatric desmoid tumour

Year: 2025

Type: article

Abstract: Desmoid tumours are rare fibroblastic neoplasms that, although benign, may be locally aggressive, invasive, and disabling. In children with Gardner syndrome, they typically arise in the abdominal wall, whereas scapular involvement is uncommon and surgically challenging because of the risk of scapular winging after resection of stabilizing muscles. We report the case of a 4-year-old boy with Gardner syndrome and multifocal desmoid disease, including a large tumour of the left shoulder girdle. Radical resection required sacrifice of several stabilizers, and scapular stability was preserved using a dynamic reconstructive technique: a trapezius flap elongated with lumbosacral fascia, passed through a bone tunnel in the scapula and anchored to adjacent muscles. Despite recurrence requiring re-excision and chemotherapy, the patient remained disease-free at 4-year follow-up, with preserved scapular stability. This case underscores the importance of dynamic reconstruction techniques, avoidance of mutilating resections, and multidisciplinary management in paediatric multifocal desmoid disease.

Source: Journal of Surgical Case Reports

DOI: 10.1093/jscr/rjaf830

Authors: Maria Albuquerque, Cláudia Constantino, Pedro Martins, Diogo Casal

Institutions: Unidade Local de Saúde de São José, Instituto Português de Oncologia Francisco Gentil, University of Lisbon, Universidade Nova de Lisboa

Language: English

Topic: Soft tissue tumor case studies

Subfield: Rheumatology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $1,000