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.
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Authors | Paola Zagami, Alessandro Comandone, Marco Fiore, Giacomo Giulio Baldi, Giovanni Grignani, Bruno Vincenzi, Alessandro Gronchi, Gabriele Antonarelli, Antonella Boglione, Elisabetta Pennacchioli, Giuseppe Curigliano, Fabio Conforti, Tommaso Martino De Pas |
Journal | Cancer medicine
(Cancer Med)
Vol. 12
Issue 16
Pg. 17047-17055
(08 2023)
ISSN: 2045-7634 [Electronic] United States |
PMID | 37455549
(Publication Type: Observational Study, Multicenter Study, Journal Article)
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Copyright | © 2023 The Authors. Cancer Medicine published by John Wiley & Sons Ltd. |
Topics |
- Humans
- Leiomyosarcoma
(diagnosis, therapy, pathology)
- Retrospective Studies
- Neoplasm Recurrence, Local
(therapy)
- Sarcoma
- Gastrointestinal Stromal Tumors
(therapy, drug therapy)
- Italy
(epidemiology)
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