The rare entity of gastrointestinal leiomyosarcomas: An Italian multicenter retrospective study in high-volume referral centers.
Cancer Med
; 12(16): 17047-17055, 2023 08.
Article
em En
| MEDLINE
| ID: mdl-37455549
ABSTRACT
BACKGROUND:
After a huge efficacy of imatinib in treating patients with gastrointestinal stromal tumors (GISTs) was proven, a maximum effort was made to make a differential diagnosis between GISTs and gastrointestinal leiomyosarcomas (GI-LMS), showing the latter to be an extremely rare tumor entity. Limited data on GI-LMS biology, clinical behavior and drug-sensibility are available, and the clinical decision-making in this subgroup of patients is usually challenging.METHODS:
We conducted a multicenter, retrospective observational study on patients with diagnosed GI-LMS from 2004 to 2020 within six high-volume referral centers in Italy.RESULTS:
Thirty-three patients had diagnosis of KIT-negative GI-LMS confirmed by sarcoma-expert pathologist. The most common site of origin was the intestine. Twenty-two patients had localized disease and underwent surgery with a median follow-up of 72 months, median disease-free survival was 42 months. Overall survival (OS)-rate at 5 years was 73% and median OS was 193 months. Five out of 10 patients with local relapse received a salvage surgery, and 2/5 remained with no evidence of disease. Thirteen patients received neoadjuvant (6) or adjuvant (7) chemotherapy, and 2/13 patients remained free from relapse. The median OS for patients with metastatic LMS was 16.4 months.CONCLUSION:
GI-LMS is very rare and extremely aggressive subgroup of sarcomas with a high tendency to systemic spread. Localized GI-LMS at diagnosis may be cured if treated with adequate surgery with or without (neo) adjuvant chemotherapy, while de-novo metastatic disease appeared to have a poor prognosis. Clinical effort to understand GI-LMS biology and clinical behavior and to develop active treatment strategy, especially for metastatic-disease, is warranted.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Sarcoma
/
Tumores do Estroma Gastrointestinal
/
Leiomiossarcoma
Tipo de estudo:
Clinical_trials
/
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Humans
País/Região como assunto:
Europa
Idioma:
En
Revista:
Cancer Med
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Itália