Gastrointestinal leiomyosarcoma demonstrate a predilection for distant recurrence and poor response to systemic treatments.
Adult
Aged
Aged, 80 and over
Antineoplastic Agents
/ therapeutic use
Antineoplastic Combined Chemotherapy Protocols
/ therapeutic use
Chemotherapy, Adjuvant
Female
Gastrointestinal Neoplasms
/ pathology
Humans
Kaplan-Meier Estimate
Leiomyosarcoma
/ secondary
Male
Margins of Excision
Metastasectomy
Middle Aged
Neoplasm Recurrence, Local
/ therapy
Neoplasms, Radiation-Induced
/ physiopathology
Progression-Free Survival
Radiotherapy
/ adverse effects
Retrospective Studies
Survival Rate
Leiomyosarcoma
Radiation induced sarcoma
Soft tissue sarcoma
Surgery
Systemic therapy
Journal
European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
ISSN: 1532-2157
Titre abrégé: Eur J Surg Oncol
Pays: England
ID NLM: 8504356
Informations de publication
Date de publication:
10 2021
10 2021
Historique:
received:
22
02
2021
revised:
23
04
2021
accepted:
27
04
2021
pubmed:
11
5
2021
medline:
30
12
2021
entrez:
10
5
2021
Statut:
ppublish
Résumé
Primary leiomyosarcoma (LMS) of the gastrointestinal (GI) tract is rare. Limited literature exists regarding the clinical characteristics and outcome for patients with localised and metastatic disease. A retrospective chart review was performed for patients greater than 18 years of age diagnosed with GI LMS at The Royal Marsden Hospital between 1 January 2000-1 May 2020. Descriptive statistics were performed. Patients were censored at data cut-off date of 27 June 2020. Forty-six patients with a median age at diagnosis of 54 years (range 25-85) were identified. Fifteen percent (n = 7) of patients previously received abdominal radiation for an unrelated cancer. All patients with localised disease (n = 36) had resection with oncological margins. For patients who underwent potentially curative surgery, median recurrence-free survival (mRFS) was 13 months (0.4-183 months), and half of these patients (n = 18) developed recurrent disease post resection (distant n = 16, local n = 2). Median overall survival (mOS) was 27 months for patients with distant recurrence. Twenty-one percent (n = 10) of patients presented with synchronous metastatic disease and their mOS was 19 months. Median progression-free survival (mPFS) for patients treated with conventional chemotherapy ranged from 2.0 to 8.0 months. The risk of recurrence is significant, and recurrence-free survival was short even with complete oncologic resection. The relationship of prior abdominal radiotherapy to the development of GI LMS warrants further investigation. Outcomes with systemic therapy for metastatic disease were poor and there is a need for the development of more effective systemic therapies.
Sections du résumé
BACKGROUND
Primary leiomyosarcoma (LMS) of the gastrointestinal (GI) tract is rare. Limited literature exists regarding the clinical characteristics and outcome for patients with localised and metastatic disease.
METHODS
A retrospective chart review was performed for patients greater than 18 years of age diagnosed with GI LMS at The Royal Marsden Hospital between 1 January 2000-1 May 2020. Descriptive statistics were performed. Patients were censored at data cut-off date of 27 June 2020.
RESULTS
Forty-six patients with a median age at diagnosis of 54 years (range 25-85) were identified. Fifteen percent (n = 7) of patients previously received abdominal radiation for an unrelated cancer. All patients with localised disease (n = 36) had resection with oncological margins. For patients who underwent potentially curative surgery, median recurrence-free survival (mRFS) was 13 months (0.4-183 months), and half of these patients (n = 18) developed recurrent disease post resection (distant n = 16, local n = 2). Median overall survival (mOS) was 27 months for patients with distant recurrence. Twenty-one percent (n = 10) of patients presented with synchronous metastatic disease and their mOS was 19 months. Median progression-free survival (mPFS) for patients treated with conventional chemotherapy ranged from 2.0 to 8.0 months.
CONCLUSION
The risk of recurrence is significant, and recurrence-free survival was short even with complete oncologic resection. The relationship of prior abdominal radiotherapy to the development of GI LMS warrants further investigation. Outcomes with systemic therapy for metastatic disease were poor and there is a need for the development of more effective systemic therapies.
Identifiants
pubmed: 33966946
pii: S0748-7983(21)00475-3
doi: 10.1016/j.ejso.2021.04.043
pii:
doi:
Substances chimiques
Antineoplastic Agents
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
2595-2601Informations de copyright
Crown Copyright © 2021. Published by Elsevier Ltd. All rights reserved.
Déclaration de conflit d'intérêts
Declaration of competing interest RLJ is the recipient of grants/research support from MSD, GSK. AJH and MJS are the recipient of research support from Amgen. RLJ is the recipient of consultation fees from Adaptimmune, Athenex, Blueprint, Clinigen, Eisai, Epizyme, Daichii, Deciphera, Immunedesign, Lilly, Merck, Pharmamar, UptoDate. MJFS has been an advisory board member for Amgen. All other authors have no conflicts of interest to declare.