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atypical gastrointestinal stromal tumor

Massimiliano Salati, Giulia Orsi, Luca Reggiani Bonetti, Fabrizio Di Benedetto, Giuseppe Longo, Stefano Cascinu
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract, and are characterized by a broad spectrum of clinical, histological and molecular features at presentation. Although focal and scattered calcifications are not uncommon within the primary tumor mass, heavy calcification within a GIST is rarely described in the literature and the clinical-biological meaning of this feature remains unclear. Cases with such an atypical presentation are challenging and may be associated with diagnostic pitfalls...
March 15, 2017: World Journal of Gastrointestinal Oncology
Andrew C Berry, Rahman Nakshabendi, Ozdemir Kanar, Sean Hamer
BACKGROUND: Tumors within a Meckel diverticulum are a rare complication observed in only 0.5%-3.2% of symptomatic cases. The majority of tumors are benign, but some malignant tumors, such as gastrointestinal stromal tumors (GISTs) can occur. CASE REPORT: We report the case of a 48-year-old female who presented with severe abdominal pain and nausea and was found to have a GIST arising from a Meckel diverticulum. CONCLUSION: The differential diagnosis of a pelvic mass in a middle-aged female presenting with gastrointestinal symptoms must remain broad...
2017: Ochsner Journal
Atsuo Imagawa, Mitsuru Tomizawa, Satoshi Okumura, Sho Toyoda, Hiroshi Kawashima, Kansuke Yamamoto, Aya Ito, Naoto Mizumura, Ken Yoo, Hiromitsu Maehira, Masao Ogawa, Masayasu Kawasaki, Masao Kameyama, Michiko Yoshimura, Seiichi Hirota
A 49-year-old man visited our hospital with a chief complaint of abdominal pain that began 1 day before his visit.An approximately 30 cm tumor that was extensively in contact with the gastric wall in the abdominal cavity was detected on computed tomography(CT).An elevated lesion covered with normal mucosa on the posterior wall of the greater curvature was detected on upper endoscopy.He was diagnosed with a submucosal tumor of the stomach, and he underwent surgery. Surgical findings revealed an elastic soft tumor with a maximal dimension of 38 cm that projected from the posterior wall of the stomach beyond the gastric wall...
November 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
Larissa Merten, Abbas Agaimy, Evgeny A Moskalev, Johannes Giedl, Claudia Kayser, Helene Geddert, Inga-Marie Schaefer, Silke Cameron, Martin Werner, Philip Ströbel, Arndt Hartmann, Florian Haller
OBJECTIVES: Loss-of-function mutations in TP53 and CDKN2A have been found at varying frequencies in gastrointestinal stromal tumors (GISTs), while no mutations of RB1 have been reported to date. The aim of the current study was to determine the mutation frequency of TP53, RB1, and CDKN2A in GISTs. METHODS: A cohort of 83 primary untreated GISTs was analyzed for mutations in TP53, RB1, and CDKN2A by massive parallel sequencing. Tumors with mutations in TP53 and RB1 were analyzed by fluorescence in situ hybridization for the corresponding gene loci...
December 2016: American Journal of Clinical Pathology
S F Lax
The 2014 World Health Organization (WHO) classification of uterine tumors revealed simplification of the classification by fusion of several entities and the introduction of novel entities. Among the multitude of alterations, the following are named: a simplified classification for precursor lesions of endometrial carcinoma now distinguishes between hyperplasia without atypia and atypical hyperplasia, the latter also known as endometrioid intraepithelial neoplasia (EIN). For endometrial carcinoma a differentiation is made between type 1 (endometrioid carcinoma with variants and mucinous carcinoma) and type 2 (serous and clear cell carcinoma)...
November 2016: Der Pathologe
Ke-Kang Sun, Song Xu, Jinzhen Chen, Gang Liu, Xiaojun Shen, Xiaoyang Wu
Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms that arise in the gastrointestinal tract, accounting for ~1% of gastric malignancies. The present study reports the case of a GIST of the stomach in a 75-year-old man who presented with abdominal distension and anorexia for 1 month. Gastroscopy was unremarkable. Ultrasound and computed tomography (CT) scans showed a giant intraabdominal cystic lesion of unknown origin. The lesion was initially believed to be a duplication cyst, a pancreatic pseudocyst or a liver cyst in the pre-operative diagnosis...
October 2016: Oncology Letters
Vlado Janevski, Redžep Selmani, Vesna Janevska, Liljana Spasevska, Julija Zhivadinovik
INTRODUCTION: Gastrointestinal stromal tumors are the most common mesenchymal tumors of the digestive tract. Leiomyosarcomas of the gastrointestinal tract are rare mesenchymal neoplasms which grossly and histologically resemble gastrointestinal stromal tumors. They may be differentiated from gastrointestinal stromal tumors by using immunohistochemistry and they are typically positive for a smooth muscle actin and desmin and negative for c-kit, CD34 and DOG1.1. They often express calponin and h-caldesmon...
November 2015: Medicinski Pregled
Jae Min Chun, Kyoung Hoon Lim
INTRODUCTION: Gastrointestinal stromal tumors (GISTs) of the appendix are extremely rare. To date, only 15 cases have been reported in the English literature. Here, we present a new case of an appendiceal GIST with appendicitis. PRESENTATION OF CASE: A 68-year-old man who complained of right lower abdominal tenderness underwent surgery for a cystic mass mimicking Meckel's diverticulum. Laparoscopy revealed a mass protruding from the proximal appendix with distal appendicitis...
2016: International Journal of Surgery Case Reports
Ho Seok Seo, Ji Yeon Hyeon, Ok-Ran Shin, Han Hong Lee
C-kit-negative gastrointestinal stromal tumors (GISTs) are uncommon, and there have been few reports about the diagnosis and treatment of c-kit-negative GISTs in the stomach. We report the case of a patient who was diagnosed with a huge and atypical GIST in the stomach. The GIST was completely resected and finally diagnosed as c-kit-negative GIST based on immunohistochemical staining of tumor cells, which were negative for CD117 and CD34 and positive for Discovered on GIST-1 (DOG1). C-kit-negative GISTs could be treated by complete resection and/or imatinib, which is the same treatment for c-kit-positive GISTs...
December 2015: Journal of Gastric Cancer
Hwan-Jung Yun, Hyewon Ryu, Yoon Seok Choi, Ik-Chan Song, Deog-Yeon Jo, Samyong Kim, Hyo Jin Lee
Chemokine receptors are key mediators of normal physiology and numerous pathological conditions, including inflammation and cancer. This receptor family is an emerging target for anticancer drug development. C-X-C motif receptor 7 (CXCR7) is an atypical chemokine receptor that was first cloned from a canine cDNA library as an orphan receptor and was initially named receptor dog cDNA 1 (RDC1). Shortly after demonstrating that RDC1 binds with its ligand, stromal cell-derived factor-1α and interferon-inducible T-cell α chemoattractant, RDC1 was officially deorphanized and renamed CXCR7, as the seventh receptor in the CXC class of the chemokine receptor family...
September 2015: Oncology Letters
Inga-Marie Schaefer, Christopher Dm Fletcher, Jason L Hornick
The diagnosis of malignant peripheral nerve sheath tumor is challenging, particularly in the sporadic setting. Inactivation of the polycomb repressive complex 2 (PRC2), resulting from inactivating mutations of its constituents SUZ12 or EED1, has recently been identified in 70-90% of malignant peripheral nerve sheath tumors. Homozygous PRC2 inactivation results in loss of histone H3K27 trimethylation (H3K27me3). PRC2 inactivation promotes tumor progression and may render patients sensitive to epigenetic-based targeted therapies...
January 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Tsuguka Shiwa, Kenji Oki, Masayasu Yoneda, Koji Arihiro, Haruya Ohno, Rui Kishimoto, Nobuoki Kohno
The gene succinate dehydrogenase subunit B (SDHB) encodes a protein comprising part of the mitochondrial complex II, which links the Krebs cycle and the electron-transport chain. Heterozygous germ-line SDHB mutations causes familial pheochromocytoma-paraganglioma syndrome and has also been linked to gastrointestinal stromal tumors, as well as renal cell carcinomas. We herein report a patient with a germ-line SDHB mutation who presented with an atypical meningioma that was identified as originating from a somatic SDHB mutation...
2015: Internal Medicine
Claudia Trombatore, Stefano Palmucci, Giuseppe Angelico, Enrico Vasquez, Giuseppe Petrillo, Stefano Puleo, Antonio Di Cataldo
Extragastrointestinal stromal tumors (EGISTs) are a rare subgroup of gastrointestinal stromal tumors (GISTs), arising from outside the walls of gastrointestinal tubular organs. We report a case of an EGIST of the lesser omentum that represented a diagnostic challenge. Due to its atypical radiologic findings, it was preoperatively mistaken for pedunculated hepatic hemangioma. Histopathologically, it showed epithelioid structure and c-kit negative, very uncommon for GIST. Only a few cases of EGISTs have been previously reported...
November 2015: Clinical Imaging
Kürşat Rahmi Serin, Metin Keskin, Mine Güllüoğlu, Ali Emre
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. They can arise from any part of the tract as well as the omentum, mesentery or retroperitoneum. In this study, we present a case of a GIST originating from an extraordinary site, the pancreas. Evaluation of 30-year-old man with complaints of abdominal distension revealed a cystic, distal pancreatic lesion 13 cm in diameter. There were no intra-abdominal or distant organ metastases. The patient was operated with a diagnosis of cystic pancreas tumor, distal pancreatectomy and splenectomy was performed...
2013: Ulusal Cerrahi Dergisi
Gustaw Lech, Wojciech Korcz, Emilia Kowalczyk, Tomasz Guzel, Marcin Radoch, Ireneusz Wojciech Krasnodębski
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...
March 21, 2015: World Journal of Gastroenterology: WJG
Ameet Kumar, C K Jakhmola, Shivraj Singh Chauhan, Apoorv Singh
INTRODUCTION: Gastrointestinal stromal tumors (GIST) are solid tumors. A duodenal GIST masquerading as a cystic lesion has never been reported. We report a large duodenal cyst that finally turned out to be a GIST and was managed without a pancreaticoduodenectomy (PD). PRESENTATION OF CASE: A 55 year old lady presented with painful lump in epigastrium. A CT scan revealed a large exophytic cystic lesion from the duodenum with a small solid component. An endoscopy showed a polypoid lesion in the second part of the duodenum adjacent to what looked like a diverticulum...
2015: International Journal of Surgery Case Reports
Yujie Yuan, Jinning Ye, Chuangqi Chen, Wu Song, Jianbo Xu, Jianhui Chen, Yaping Xing, Yulong He
The current study reports the case of a 60-year-old male that presented with a four-month history of recurrent chest pain. Chest X-ray examination revealed a left-sided pleural effusion. A closed thoracic drainage procedure was performed, but the chest pain relapsed shortly afterwards. The pleural fluid was exudative, with no tumor cells detected. A computed tomography scan subsequently revealed a large mass located in the splenic curve of the colon, with involvement of the greater curvature of the stomach...
March 2015: Oncology Letters
Cristiano de Assis Pereira Hansen, Fábio Freire José, Naiara Pedrassi Engracia Garcia Caluz
A 74-year-old female patient underwent a Roux-en-Y cystjejunostomy for pancreatic pseudocyst developed several melena episodes and she was surgically reappraised. The main diagnostic concern was a pancreatic cystic neoplasm. A 12 × 8.0 × 5.0 cm retro-gastric lesion was resected and pathology report indicated an unsuspected gastrointestinal stromal tumor (GIST). The report aimed to describe an atypical presentation of GIST.
October 2014: Clinical Case Reports
Mario Ojeda-Uribe, Sylvain Merieau, Marie Guillon, Olivier Aujoulat, Olivier Hinschberger, Jean-Claude Eisenmann, David Kenizou, Agathe Debliquis, Agnès Veyradier, François Chantrel
Drug-mediated thrombotic microangiopathy may cause life-threatening medical emergencies. Novel targeted therapies have dramatically changed the prognosis of a number of oncological diseases. Tyrosine kinase inhibitors of the Breakpoint Cluster Region-Abelson (BCR-ABL) oncoprotein are used in patients with chronic myeloid leukemia or Philadelphia chromosome-positive acute lymphoblastic leukemia. Imatinib mesylate, which was the first anti-BCR-ABL tyrosine kinase inhibitor, has demonstrated a high tolerance profile and efficacy in these patients for many years...
April 2016: Journal of Oncology Pharmacy Practice
Yijun Qi, Wendi Zhao, Zhengguang Wang, Tuanjie Li, Xiangling Meng
Gastrointestinal stromal tumors (GISTs) are the most common among gastrointestinal mesenchymal tumors, but its prognosis has not been accurately predicted by the current risk stratification guidelines, National Institutes of Health classification. In this study, we evaluated the predictive factors for GIST prognosis in a retrospective analysis of 332 patients. The data collected included tumor sites, including the esophagus, stomach, duodenum, small intestine, and extragastrointestinal sites; tumor size; microscopic indicators for malignant tumor behavior, such as the number of dividing cells, cell necrosis, atypical morphology, and invasion into the muscular or mucous layer; and previously established immunohistochemical indicators, CD117, CD34, and discovered on GIST-1 (DOG-1)...
2014: Tohoku Journal of Experimental Medicine
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