Gastrointestinal Stromal Tumor

Overexpression as well as loss of MTAP and p16 expression correlated with the presence of epithelioid histology (either pure or mixed with a spindle cell component), higher grade and pT-stage, necrosis, higher NCCN risk groups, and widespread disease at presentation. Overexpression as well as loss of MTAP and p16 expression predicts shortened progression-free survival and is associated with various known poor prognostic clinicopathologic parameters.

Extraluminal cases of IFPs are extremely rare in the literature. Recognising these unusual cases is crucial to prevent misdiagnosis, guide proper surgical management and reassure patients about their prognosis.

This case report presents a highly unusual case of a primary high-grade malignant peripheral nerve sheath tumor (MPNST) of esophagus, a neoplasm of extreme rarity, with fewer than twenty histologically confirmed cases reported worldwide to date; detailing the successful management of this tumor through Orringer's transhiatal esophagectomy, complemented by comprehensive histopathologic and immunohistochemical evaluation. Our report emphasizes the crucial the role of immunohistochemistry, specifically the diagnostic value of S100, SOX10, and the exclusion of gastrointestinal stromal tumors markers (DOG1, CD117) in distinguishing MPNST, from morphologically similar esophageal submucosal tumors.

P2/3, N=255, Not yet recruiting, Ningbo Newbay Technology Development Co., Ltd

P=N/A, N=180, Not yet recruiting, ‌ People's Liberation Army General Hospital Fourth Medical Center; People's Liberation Army General Hospital Fourth Medical Center

P=N/A, N=282, Not yet recruiting, Northern Jiangsu People's Hospital; Northern Jiangsu People's Hospital

P=N/A, N=66, Not yet recruiting, Peking University International Hospital; Peking University International Hospital

We report a case of a 60-year-old male who developed intra-abdominal desmoid tumors five years after undergoing laparoscopy and endoscopy cooperative surgery (LECS) for gastric GIST, followed by adjuvant imatinib therapy...Histopathological confirmation remains crucial for accurate diagnosis. Surgical resection is the primary treatment for symptomatic desmoid tumors, but given their high recurrence rate, long-term follow-up and a multidisciplinary approach are essential for optimal management.

The present study highlights that clinicians should be aware that GIST and gastric leiomyoma can coexist in the same SEL to avoid misdiagnosis and mistreatment. In the face of the escalating drug resistance rate of GIST, researchers may derive some novel insights from the present findings for GIST treatment and management.

Extended, risk-adapted surveillance may be reasonable for small-bowel GISTs, and it may be beneficial to incorporate Ki-67 (≥5%) into site-based risk stratification. These observations remain hypothesis-generating and require validation in larger, multicenter cohorts and prospective studies.

Moderate to strong immunohistochemistry for DOG1 has been found in several other tumors, sometimes comparable to that of GISTs. This suggests that DOG1 should not be associated exclusively with GISTs and may provide a basis for further investigation into the role of DOG1 in canine malignancy.

The remaining patient had an unresectable tumor and received tyrosine kinase inhibitor therapy; however, sequential treatment with imatinib and sunitinib was clinically ineffective. However, their clinical behavior differs: D842Y-mutant GISTs consistently present as large, hypervascular tumors associated with acute complications. The therapeutic efficacy of tyrosine kinase inhibitors remains unclear, underscoring the need for further case accumulation to better define the clinical course and determine optimal treatment strategies for this rare subtype.