Elbow flexion reconstruction after arm-sparing excision for high-grade triton sarcoma: a case report.
Malignant peripheral nerve sheath tumor
Malignant triton tumor
Neurofibrosarcoma
Rhabdomyoblastic differentiation
Journal
Journal of medical case reports
ISSN: 1752-1947
Titre abrégé: J Med Case Rep
Pays: England
ID NLM: 101293382
Informations de publication
Date de publication:
04 Jul 2020
04 Jul 2020
Historique:
received:
09
01
2020
accepted:
02
04
2020
entrez:
5
7
2020
pubmed:
6
7
2020
medline:
30
3
2021
Statut:
epublish
Résumé
Soft tissue sarcomas affecting the root of an upper extremity raise the question of limb amputation depending on their location, size, and malignancy. Malignant triton tumors are a rare subtype of neurofibrosarcomas that have been poorly reported in the literature. We report the case of a challenging reconstruction of the upper extremity using a pedicled latissimus dorsal flap. A 25-year-old Occidental man was referred to our sarcoma unit for the management of a large, high-grade malignant peripheral nerve sheath tumor with no regional or distant extension and very fast progression. He was treated first by concomitant neoadjuvant radiotherapy and chemotherapy. Carcinologic excision was performed "en bloc" including the skin, the tumor, and the flexor muscles of our patient's elbow. Coverage of the skin defect and elbow flexion restoration were achieved by using a homolateral pedicled musculocutaneous latissimus dorsi flap. Histological analysis showed an R0 resection. The reconstruction process recovered a complete bending of his elbow. He is still in remission at 26 months follow-up. A malignant triton tumor is a rare, aggressive, and high-grade sarcoma. It was successfully treated and this case report describes an effective treatment modality. Reconstructive surgery, allowing large, complete tumor removal, is indispensable after neoadjuvant chemotherapy and radiotherapy.
Sections du résumé
BACKGROUND
BACKGROUND
Soft tissue sarcomas affecting the root of an upper extremity raise the question of limb amputation depending on their location, size, and malignancy. Malignant triton tumors are a rare subtype of neurofibrosarcomas that have been poorly reported in the literature. We report the case of a challenging reconstruction of the upper extremity using a pedicled latissimus dorsal flap.
CASE PRESENTATION
METHODS
A 25-year-old Occidental man was referred to our sarcoma unit for the management of a large, high-grade malignant peripheral nerve sheath tumor with no regional or distant extension and very fast progression. He was treated first by concomitant neoadjuvant radiotherapy and chemotherapy. Carcinologic excision was performed "en bloc" including the skin, the tumor, and the flexor muscles of our patient's elbow. Coverage of the skin defect and elbow flexion restoration were achieved by using a homolateral pedicled musculocutaneous latissimus dorsi flap. Histological analysis showed an R0 resection. The reconstruction process recovered a complete bending of his elbow. He is still in remission at 26 months follow-up.
CONCLUSIONS
CONCLUSIONS
A malignant triton tumor is a rare, aggressive, and high-grade sarcoma. It was successfully treated and this case report describes an effective treatment modality. Reconstructive surgery, allowing large, complete tumor removal, is indispensable after neoadjuvant chemotherapy and radiotherapy.
Identifiants
pubmed: 32620140
doi: 10.1186/s13256-020-02384-y
pii: 10.1186/s13256-020-02384-y
pmc: PMC7334847
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
103Commentaires et corrections
Type : ErratumIn
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