Synchronous occurrence of gastrointestinal stromal tumors and other primary gastrointestinal neoplasms

Gastrointestinal stromal tumors (GISTs) are the most common types of malignant mesenchymal tumors in the gastrointestinal tract, with an estimated incidence of 1.5/100.000 per year and 1–2% of gastrointestinal neoplasms. About 75–80% of patients have mutations in the KIT gene in exons 9, 11, 13, 14, 17, and 5–10% of patients have mutations in the platelet-derived growth factor receptor a (PDGFRA) gene in exons 12, 14, 18. Moreover, 10–15% of patients have no mutations and are classified as wild type GIST. The treatment for metastatic or unresectable GISTs includes imatinib, sunitinib, and regorafenib. So far, GIST therapies have raised great expectations and offered patients a better quality of life, but increased pharmacological resistance to tyrosine kinase inhibitors is often observed. New treatment options have emerged, with ripretinib, avapritinib, and cabozantinib getting approvals for these tumors. Nowadays, immune checkpoint inhibitors form a new landscape in cancer therapeutics and have already shown remarkable responses in various tumors. Studies in melanoma, non-small-cell lung cancer, and renal cell carcinoma are very encouraging as these inhibitors have increased survival rates. The purpose of this review is to present alternative approaches for the treatment of the GIST patients, such as combinations of immunotherapy and novel inhibitors with traditional therapies (tyrosine kinase inhibitors).

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Extraordinarily Large Malignant Extra Gastro Intestinal Stromal Tumor Presenting as an Abdominal Mass

Journal of BP Koirala Institute of Health Sciences ◽

10.3126/jbpkihs.v2i2.27875 ◽

2019 ◽

Vol 2 (2) ◽

pp. 84-87

Author(s):

P Paudyal ◽

TN Subba ◽

A Pradhan ◽

S Dhakal ◽

S Karki ◽

...

Keyword(s):

Gastrointestinal Stromal Tumors ◽

Histopathological Examination ◽

Abdominal Mass ◽

Correct Diagnosis ◽

Standard Technique ◽

Gastrointestinal Neoplasms ◽

Epithelial Tumor ◽

Stromal Tumors ◽

Cut Surface ◽

Histology And Immunohistochemistry

Background: Gastrointestinal stromal tumors (GIST) are rare of all gastrointestinal neoplasms with histology and immunohistochemistry similar to gastrointestinal stromal tumors. They occur outside the gastrointestinal tract rarely and are, hence, called the extra gastrointestinal stromal tumors (EGIST). Herein, we report a rare case of malignant extra intestinal gastrointestinal tumor. Case report: A 39 yr old female presented to the surgical OPD with a lump in the abdomen since 4 months. We received a lobulated tumor which measured 40x25x20 cm3 and showed capsule. Cut surface was predominantly solid, grey white with areas of hemorrhage and necrosis along with cystic areas. Histopathology and immunohisto chemistry revealed it to be a malignant EGIST of mesentery because of no continuity of the tumor with the small intestine microscopically. Conclusion: EGIST is a rare no epithelial tumor of mesentery. Omentum histopathological examination is a standard technique to diagnose the tumor and to differentiate it immunohistochemically from other tumors having similar morphology. Correct diagnosis plays valuable role in the treatment.

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Gastrointestinal Stromal Tumors in Equids

Veterinary Pathology ◽

10.1354/vp.38-6-689 ◽

2001 ◽

Vol 38 (6) ◽

pp. 689-697 ◽

Cited By ~ 43

Author(s):

F. Del Piero ◽

B. A. Summers ◽

J. F. Cummings ◽

G. Mandelli ◽

E. A. Blomme

Keyword(s):

Gastrointestinal Stromal Tumors ◽

Clinical Signs ◽

Neuron Specific Enolase ◽

Mesenchymal Cells ◽

Gastrointestinal Neoplasms ◽

Glial Fibrillary Acid Protein ◽

Stromal Tumors ◽

Acid Protein ◽

S 100 ◽

Homogeneous Matrix

Eleven gastrointestinal neoplasms from 10 aged horses and 1 pony were examined grossly, histologically, immunohistochemically, and (in two cases) ultrastructurally. Clinical signs were associated with two neoplasms, and the other nine tumors were incidental findings at laparotomy or necropsy. The neoplasms were solitary (9/11) or multifocal (2/11), well demarcated, serosal or mural masses of stomach (1), jejunum (1), ileum (3), cecum (5), and/or colon (2). Microscopic examination revealed discrete spindle cells arranged in compact patterns with fascicles and whorls or cribriform pattern with fascicles and rare palisades, often with a myxoid interstitial matrix. Three tumors infiltrated between the muscularis interna and the muscularis externa at the myenteric plexi. All neoplasms were vimentin positive, 3/11 were S-100 positive, 2/11 were muscle actin positive, and no neoplasm was positive for glial fibrillary acid protein, desmin, factor VIII, chromogranin, or neuron-specific enolase. Of the two tumors studied ultrastructurally, one contained an admixture of smooth muscle cells and cells resembling Schwann cells, and the second was populated by homogeneous fusiform mesenchymal cells separated by homogeneous matrix. Gastrointestinal stromal tumors (GIST) have been recognized in humans, more recently in dogs and nonhuman primates, and now in equids. Most of these tumors are comprised of a loosely arranged network of spindled cells separated by myxoid matrix. GIST may be composed of myogenic, neurogenic, combined myogenic and neurogenic, and undifferentiated mesenchymal cells.

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Gigantic GIST: A Case of the Largest Gastrointestinal Stromal Tumor Found to Date

Case Reports in Surgery ◽

10.1155/2018/6170861 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

Abdalla Mohamed ◽

Youssef Botros ◽

Paul Hanna ◽

Sang Lee ◽

Walid Baddoura ◽

...

Keyword(s):

Tyrosine Kinase ◽

Gastrointestinal Stromal Tumor ◽

Gastrointestinal Stromal Tumors ◽

Kinase Inhibitors ◽

Kinase Inhibitor ◽

Stromal Tumor ◽

Definitive Treatment ◽

Gastrointestinal Neoplasms ◽

Mesenchymal Tumors ◽

Stromal Tumors

Gastrointestinal stromal tumors are uncommon when compared to all gastrointestinal neoplasms but are the most common mesenchymal tumors of the gastrointestinal tract. The largest gastrointestinal stromal tumor ever recorded in literature weighed approximately 6.1 kg and measured 39 cm × 27 cm × 14 cm. About two-thirds of GISTs are malignant. The tumor size, mitotic rate, cellularity, and nuclear pleomorphism are the most important parameters when considering prognosis and recurrence. The definitive treatment for these tumors is resection. In the year 2000, the first patient was treated with the tyrosine kinase inhibitor imatinib and since then, gastrointestinal stromal tumors with high-risk features have been treated successfully with tyrosine kinase inhibitors. We present the largest gastrointestinal stromal tumor recorded in medical literature measuring 42.0 cm × 31.0 cm × 23.0 cm in maximum dimensions and weighing in at approximately 18.5 kg in a 65-year-old African-American male who presented with increased abdominal distention. The mass was successfully excised, and the patient was treated with imatinib without local or distant recurrence 1.5 years postoperatively.

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