Pancreatic Gastrointestinal Stromal Tumors Research Articles

Rare Double Primary Malignancies: A Pancreatic Gastrointestinal Stromal Tumor Mimicking as a Metastatic Lesion of Myoepithelial Carcinoma of Parotid Gland

Pancreatic gastrointestinal stromal tumors (PGISTs) are exceptionally rare, accounting for <5% of extra-gastrointestinal stromal tumors (EGISTs) and <1% of malignant pancreatic neoplasms. We present a unique case of concurrent double primary malignancies in a 46-year-old female with a history of recurrent myoepithelial carcinoma of the parotid gland, managed through surgical resection and adjuvant therapy. She presented with an enlarging abdominal mass, initially suggestive of pancreatic metastasis. Immunohistochemical analysis revealed positive staining for smooth-muscle actin (SMA) and CD34 in both parotid and pancreatic tissues. Importantly, CD117 expression was confined to the pancreatic tissue, confirming the diagnosis of PGIST rather than metastasis. Subsequently, a splenic-sparing distal pancreatectomy was performed, followed by immediate imatinib therapy. This case underscores the potential for the coexistence of rare primary malignancies with unique histopathological characteristics and organ involvement. When encountering a newly developed lesion in a distant organ, surgeons must consider the possibility of metastasis to guide therapeutic decision-making. Early diagnosis and appropriate intervention are paramount, particularly in the case of PGIST, given its infrequent presentation and clinical complexities.

Liver metastasis from pancreatic gastrointestinal stromal tumor presenting 5 years after resection: a rare case with management challenges

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors arising from the gastrointestinal tract. Stromal tumors arising outside the gastrointestinal tract are called extra-gastrointestinal stromal tumors, usually found in omentum, mesentery, retroperitoneum and gallbladder. We report a case of 31 years old male with 3-month history of painless jaundice and undocumented weight loss. Computerized tomography scan showed a well-defined, rounded enhancing lesion in the uncinate process of pancreas measuring 53x49mm compressing the distal common bile duct. Percutaneous biopsy confirmed gastrointestinal stromal tumor. He underwent Whipple’s procedure followed by adjuvant imatinib due to high-risk features. He remained well for a long time and developed oligo-metastasis in liver 5 years after resection while he was still on imatinib. The patient underwent hepatic resection and remains well one year after the second resection on adjuvant treatment. Pancreatic extragastrointestinal stromal tumor involving uncinate process is a rare disease entity. First line of management is negative margin resection followed by adjuvant therapy with imatinib for high-risk tumors. In case of resectable liver metastasis, resection of metastasis followed by multimodal therapy seems to prolong the disease free and overall survival.

Pancreatic GIST in a Patient with Limited Stage Small Cell Lung Cancer: A Case Report and Review of Published Cases

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract and usually occur in the stomach and the small intestine. The pancreas is an extremely rare primary site for GISTs and there are 25 reported cases of pancreatic GIST with most being treated with surgical resection. We describe a 52-year-old African-American female who was diagnosed with limited stage small cell carcinoma in November 2009 and treated with concurrent cisplatin/etoposide chemotherapy and radiation. She subsequently achieved complete remission. Two years later she was diagnosed with localized pancreatic GIST by endoscopic ultrasonography guided fine needle aspiration. We treated her with a tyrosine kinase inhibitor (TKI) imatinib 400 mg oral dose daily as she declined surgery. Her disease is stable based on computed tomography imaging scans 40 months after diagnosis without any metastasis. To the best of our knowledge, our case is the second case of localized pancreatic GIST treated with TKI monotherapy.

Pancreatic Gastrointestinal Stromal Tumor after Upper Gastrointestinal Hemorrhage and Performance of Whipple Procedure: A Case Report and Literature Review

Patient: Male, 56Final Diagnosis: Pancreatic GISTSymptoms: Abdominal painMedication: NoneClinical Procedure: Whipple procedureSpecialty: SurgeryObjective:Rare diseaseBackground:Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors in the gastrointestinal system. These types of tumors originate from any part of the tract as well as from the intestine, colon, omentum, mesentery or retroperitoneum. GIST is a rare tumor compared to other types of tumors, accounting for less than 1% of all gastrointestinal tumors.Case Report:A 56-year-old male patient was hospitalized due to an upper gastrointestinal hemorrhage and the start of abdominal pain on the same day. In the upper gastrointestinal endoscopy that was performed, a solitary mass was found in the second section of the duodenum and a blood vessel (Forrest type 2a) was seen. The extent and location of the mass was detected by abdominal tomography. After hemodynamic recovery, a Whipple procedure was performed without any complications. A subsequent histopathological examination detected a c-kit-positive (CD117) pancreatic GIST with high mitotic index.Conclusions:The most effective treatment method for GISTs is surgical resection. In patients with a head of pancreatic GIST, the Whipple procedure can be used more safely and effectively.

Malignant extra-gastrointestinal stromal tumor of the pancreas: Report of two cases and review of the literature

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that arise from the gastrointestinal tract. In rare cases, these tumors are found in intra-abdominal sites unrelated to the gastrointestinal tract, such as the mesentery, omentum and retroperitoneum. However, pancreatic extra-gastrointestinal stromal tumors are extremely rare, with only 14 previous cases reported. A 61-year-old man with no clinical symptoms had a routine check-up, during which an abdominal mass located in the pancreas tail was detected. Abdominal surgery was performed with resection of the pancreas tail and the spleen, and he was diagnosed with low-risk GISTs. Another 60-year-old man with no clinical symptoms underwent Computed tomography which revealed a well-demarcated tumor, 6 cm in diameter, in the head of the pancreas. He was diagnosed with pancreatic GISTs. Here, we describe two rare cases of pancreatic GISTs and review the cases previously reported in the literature.

Diagnosis, therapy and prognosis of pancreatic gastrointestinal stromal tumor

Objective To investigate the clinical characteristics, diagnosis, treatment and prognosis of pancreatic gastrointestinal stromal tumor (GIST). Methods We reported a case and reviewed the medical literature on pancreatic malignant GIST. We searched the Pubmed and main domestic database. The clinical data of the reported cases were studied, and their predictive factors for postoperative recurrence and metastasis were analyzed. Results Between January 1980 and July 2010, 16 cases of pancreatic GIST were reported. There were 7 males and 9 females, with a median age pf 56.5 (31-72)years. The clinical symptoms were nonspecific. The main presentation was upper abdominal pain or discomfort. A preoperative diagnosis was suspected on radiological examination. The tumor mainly appeared as a well-defined solid-cystic mass. Irregular enhancement appeared in the circumferential and solid portion of the tumor on enhanced CT scan sequences. The pancreatic and biliary ducts were rarely dilated. Endoscopic ultrasound guided fine needle aspiration cytology (EUS-FNA) was helpful in preoperative diagnosis. Of the 15 surgical patients, 14 underwent complete resection, while the remaining received cyst-jejunostomy. A correct diagnosis was made on histopathology and immunohisto-chemistry. On a mean follow up of 21 months (range, 1-60) in 14 patients, all patients were alive.Recurrence or metastasis occurred in 4 patients with tumors of high malignant potential. On univariate analysis, the only significant predictor for adverse outcome was mitoses≥10/50 HPF. Conclusions Pancreatic GIST is a rare tumor of relatively low malignant potential. It has a better prognosis than ductal adenocarcinoma. It is important to arrive at a correct diagnosis and treat the tumor with radical resection. Aggressive surgical resection is potentially curative. Imatinib is recommended in the treatment of patients with tumors with high malignant potential. Key words: Pancreatic neoplasm; Gastrointestinal stromal tumor; Diagnosis; Therapy

転移性膵平滑筋肉腫の1例―EUS-FNAの有用性と問題点―

A woman in her 60's presented with a tumor of the pancreatic body. Pan-hysterectomy had been performed under a diagnosis of uterine leiomyoma 11 years previously. A sample obtained by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) revealed the histopathological proliferation of spindle-shaped bundles of atypical cells, and immunohistochemical staining demonstrated that these cells were positive for KIT. Therefore, distal pancreatectomy was performed under a diagnosis of pancreatic gastrointestinal stromal tumor (GIST). Immunohistochemical staining of surgical specimens demonstrated that the tumor cells were positive for desmin and negative for KIT and CD34. The low-grade leiomyosarcoma in pathological specimens of the uterine myoma obtained 11 years previously histologically resembled the pathological findings of the pancreatic specimens except for atypical nuclei and mitotic cells. Therefore, the final diagnosis was extremely rare metastatic leiomyosarcoma of the pancreas. Herein, we report metastasis of uterine leiomyosarcoma to the pancreas and discuss the usefulness and limitations of EUS-FNA.