Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal tumours of the gastrointestinal tract, but they represent less than 3% of all gastrointestinal tract malignancies. This is a detailed case study of a 52-year-old male patient treated for very uncommon histological subtype of gastric GIST with atypical clinical presentation, asymptomatic progress and late diagnosis. The resected tumour, giant in diameters, was confirmed to represent the most rare histopathologic subtype of GISTs - sarcomatoid epithelioid GIST. We report this case and review the literature with a special focus on pathomorphological evaluation, biological aggressiveness and prognostic factors. To our knowledge this is the first report of giant GIST of very uncommon sarcomatoid epithelioid subtype. It is concluded that clinicians should pay attention to the fact that initial diagnosis may be delayed due to mildly asymptomatic and non-specific clinical presentation. Asymptomatic tumours diagnosed at a late stage, which is often the case, can be large on presentation. Prognosis for patients diagnosed with GIST depend on tumour size, mitotic rate, histopathologic subtype and tumour location. That is why early diagnosis and R0 resection, which is usually feasible and safe even in giant gastric sarcomatoid epithelioid subtype of GISTs, are the key factors for further treatment and good prognosis.
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http://dx.doi.org/10.3748/wjg.v21.i11.3388 | DOI Listing |
ESMO Open
January 2025
Department of Thoracic Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands; Department of Pulmonary Medicine, Leiden University Medical Center, Leiden, The Netherlands. Electronic address:
Background: Diffuse pleural mesothelioma (dPM) is an aggressive malignancy, primarily treated with palliative systemic therapy. Since 2022, nivolumab-ipilimumab (nivo/ipi) has replaced chemotherapy as the standard first-line treatment for dPM in the Netherlands. Chemotherapy remains a rational second-line treatment.
View Article and Find Full Text PDFRespir Investig
January 2025
Department of Respiratory Medicine, International Medical Center, Saitama Medical University, 1397-1 Yamane, Hidaka-City, Saitama, 350-1298, Japan. Electronic address:
Pleural mesothelioma (PM) is a rare and highly aggressive malignancy originating from the pleural lining, with a median overall survival of merely 1 year. This cancer primarily arises from mesothelial cells following exposure to carcinogenic, biopersistent mineral fibers, particularly asbestos. The histological subtypes of mesothelioma are epithelioid (approximately 60%), sarcomatoid (20%), and biphasic (20%), exhibiting epithelioid and sarcomatoid characteristics.
View Article and Find Full Text PDFAnn Surg
January 2025
The Thoracic Surgery Oncology laboratory and the International Mesothelioma Program (www.impmeso.org), Division of Thoracic Surgery and the Lung Center, Brigham, and Women's Hospital, and Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA.
Objective: We hypothesize that recurrence following pleurectomy decortication (PD) is primarily local. We explored factors associated with tumor recurrence patterns, disease-free interval (DFI), and post-recurrence survival (PRS).
Summary Background Data: Tumor recurrence is a major barrier for long-term survival after pleural mesothelioma (PM) surgery.
Mod Pathol
January 2025
Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, 10065, USA. Electronic address:
Histologic features, including architectural patterns, cytologic features, and 2021 World Health Organization nuclear grade have been shown to have prognostic significance in epithelioid diffuse pleural mesothelioma (DPM). Biphasic and sarcomatoid DPM, regardless of morphology, have worse outcomes. These prognostic findings are well-established but correlation of architectural patterns, cytologic features, and nuclear grade with genetic alterations has not been well studied.
View Article and Find Full Text PDFIntroduction: In August 2018, the Japanese PMDA approved nivolumab, an immune checkpoint inhibitor (ICI), for previously treated, unresectable, advanced, or recurrent pleural mesothelioma (PM) based on the MERIT trial, a phase II study of 34 cases. However, concerns regarding limited evidence persist.
Methods: We retrospectively analyzed 83 patients with previously treated, unresectable, advanced, or recurrent malignant pleural mesothelioma (MPM) treated with nivolumab from August 2018 to May 2022.
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