Yao WQ, Ma XY, Wang GH. Clinicopathologic features of SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma: A case report and review of literature. World J Gastrointest Oncol 2026; 18(1): 114021 [PMID: 41607752 DOI: 10.4251/wjgo.v18.i1.114021]
Corresponding Author of This Article
Gui-Hua Wang, MD, Professor, Department of Pathology, The First Affiliated Hospital of Zhejiang University School of Medicine, No. 1260 Kangliang Street, Liangzhu Street, Hangzhou 311112, Zhejiang Province, China. mamaninghao@163.com
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Oncology
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Case Report
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Jan 15, 2026 (publication date) through May 2, 2026
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World Journal of Gastrointestinal Oncology
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1948-5204
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Yao WQ, Ma XY, Wang GH. Clinicopathologic features of SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma: A case report and review of literature. World J Gastrointest Oncol 2026; 18(1): 114021 [PMID: 41607752 DOI: 10.4251/wjgo.v18.i1.114021]
World J Gastrointest Oncol. Jan 15, 2026; 18(1): 114021 Published online Jan 15, 2026. doi: 10.4251/wjgo.v18.i1.114021
Clinicopathologic features of SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma: A case report and review of literature
Wan-Qi Yao, Xin-Yi Ma, Gui-Hua Wang
Wan-Qi Yao, Gui-Hua Wang, Department of Pathology, The First Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou 311112, Zhejiang Province, China
Xin-Yi Ma, Department of Pathology, Pathological Diagnosis Center of Dian Diagnostic Technology Group Co., Hangzhou 311112, Zhejiang Province, China
Author contributions: Yao WQ obtained all the required data and drafted the article after literature review; Ma XY responsible for performing immunohistochemical staining; Wang GH critically revised the article and approved the final version of the article.
Informed consent statement: Informed written consent was obtained from the patient for the publication of this article and any accompanying data.
Conflict-of-interest statement: There is no conflict of interest associated with any of the senior author or other coauthors contributed their efforts in this manuscript.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Gui-Hua Wang, MD, Professor, Department of Pathology, The First Affiliated Hospital of Zhejiang University School of Medicine, No. 1260 Kangliang Street, Liangzhu Street, Hangzhou 311112, Zhejiang Province, China. mamaninghao@163.com
Received: September 11, 2025 Revised: November 2, 2025 Accepted: December 1, 2025 Published online: January 15, 2026 Processing time: 124 Days and 0.9 Hours
Abstract
BACKGROUND
SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a highly aggressive tumor, and spontaneous splenic rupture (SSR) as its presenting manifestation is rarely reported among pancreatic malignancies.
CASE SUMMARY
We herein report a rare case of a 59-year-old female who presented with acute left upper quadrant abdominal pain without any history of trauma. Abdominal imaging demonstrated a heterogeneous splenic lesion with hemoperitoneum, raising clinical suspicion of SSR. Emergency laparotomy revealed a pancreatic tumor invading the spleen and left kidney, with associated splenic rupture and dense adhesions, necessitating en bloc resection of the distal pancreas, spleen, and left kidney. Histopathology revealed a biphasic malignancy composed of moderately differentiated pancreatic ductal adenocarcinoma and an undifferentiated carcinoma with rhabdoid morphology and loss of SMARCB1 expression. Immunohistochemical analysis confirmed complete loss of SMARCB1/INI1 in the undifferentiated component, along with a high Ki-67 index (approximately 80%) and CD10 positivity. The ductal adenocarcinoma component retained SMARCB1/INI1 expression and was positive for CK7 and CK-pan. Transitional zones between the two tumor components suggested progressive dedifferentiation and underlying genomic instability. The patient received adjuvant chemotherapy with gemcitabine and nab-paclitaxel and maintained a satisfactory quality of life at the 6-month follow-up.
CONCLUSION
This study reports a rare case of SMARCB1/INI1-deficient undifferentiated rhabdoid carcinoma of the pancreas combined with ductal adenocarcinoma, presenting as SSR - an exceptionally uncommon initial manifestation of pancreatic malignancy.
Core Tip: SMARCB1-deficient undifferentiated rhabdoid carcinoma of the pancreas represents a highly aggressive and rare neoplasm. This study suggests that aggressive multimodal therapy, including surgical resection and chemotherapy, may prolong survival in selected patients; however, the overall prognosis remains poor. Given its distinct molecular characteristics and potential resistance to standard therapies, further research into targeted treatment approaches and prospective clinical studies is urgently warranted.
