Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P10721 (c-kit)
 6,575 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In the pancreas, a variety of non-neoplastic conditions may form solid masses that may mimic cancer. Up to 5% of pancreatectomies performed with the preoperative clinical diagnosis of carcinoma will prove to be non-neoplastic by pathologic examination, although this figure is decreasing with improved diagnostic modalities. Chronic inflammatory lesions are the leading cause of this phenomenon ("pseudotumoral pancreatitis"), and among these, autoimmune and paraduodenal pancreatitides (discussed separately in this issue) are most important. In this article, we will focus on the noninflammatory lesions that may form tumor-like lesions of the pancreas. Adenomyomatous hyperplasia of ampulla of Vater is a subtle lesion that is difficult to define; larger examples (>5 mm) have been found to be the cause of obstructive jaundice. Accessory (heterotopic) spleen may form a well-defined nodule within the tail of the pancreas and is typically mistaken for endocrine neoplasm. Lipomatous hypertrophy is the replacement of pancreatic tissue with mature adipose tissue that occasionally leads to moderate to marked enlargement of the pancreas. Hamartomas are very rare if the entity is defined strictly. They are characterized by irregularly arranged mature pancreatic elements admixed with stromal tissue. A cellular, spindle-cell variant with c-kit (CD117) expression is recognized. Pseudolymphoma forms well-defined nodules composed of hyperplastic lymphoid tissue. Rarely, foreign-body deposits, granulomatous inflammations (such as sarcoidosis or tuberculosis), and congenital lesions may form tumoral lesions. In conclusion, it is important to recognize the types of conditions that form pseudotumors in the pancreas so that they can be distinguished from ductal adenocarcinomas, especially clinically, but also pathologically. Nonspecific terms such as "inflammatory pseudotumor" ought to be avoided, and every attempt should be made to classify a "pseudotumor" into a more specific diagnostic category discussed above.
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PMID:Pancreatic pseudotumors: non-neoplastic solid lesions of the pancreas that clinically mimic pancreas cancer. 1627 45

Malignant gastrointestinal stromal tumor (GIST) consists a rare neoplasm, developing in small intestine and stomach. The presenting manifastations include weakness, weight loss, nausea, melena and anaemia. The present case refers to a 65 years old female patient with a GIST of the ampulla of Vater presenting with obstructive jaundice. Diagnosis was achieved pre-operatively by biopsies collected through diagnostic ERCP. The tumour was locally excised, with preservation of the ampulla. The histological analysis suggested low grade GIST positive for both CD 117 (c-kit) and CD34. Two years after the surgery the patient remains free of disease. Malignant GIST of the ampulla of the Vater is extremely rare as only few similar cases have been described in the literature. This is the first time a GIST being presented as obstructive jaundice ever reported. Despite the unavailability of EUS-FNA, the diagnosis was set preoperatively and the tumor was resected.
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PMID:Malignant gastrointestinal stromal tumor of the ampulla of Vater presenting with obstructive jaundice. 1685 23

We report a rare case of myeloid sarcoma (MS) of the extrahepatic bile ducts presenting as obstructive jaundice in a patient without leukemia at time of diagnosis. A 75-year-old female presented with a one-month history of abdominal pain and jaundice. Computerized tomography scan of the abdomen showed stenosis of the extrahepatic bile ducts. Endoscopic retrograde cholangiography disclosed an irregular narrowing of the common biliary duct, suggestive of a cholangiocarcinoma, and resection was performed. Histologic examination showed diffuse transmural infiltration of malignant cells. These cells exhibited medium-sized round nuclei with central nucleoli and eosinophilic cytoplasm, and were strongly positive for myeloperoxidase, CD68, lysozyme, CD45, CD117 (c-kit protein) and CD43. Eight months following surgery the patient presented with multiple cutaneous nodules and bone marrow trephine biopsy showed acute myelomonocytic leukemia. A literature search identified two previously reported cases of MS of the extrahepatic biliary duct. MS should be taken into consideration in the differential diagnosis of a patient with obstructive jaundice. Immunohistochemistry is essential for a correct diagnosis.
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PMID:Myeloid sarcoma of the extrahepatic bile ducts presenting as obstructive jaundice. 1694 23

A 36-year-old male with neurofibromatosis type 1 (NF-1) presented with symptoms of obstructive jaundice. Imaging showed a periampullary mass, which on endoscopic retrograde cholangiopancreatography biopsy proved to be a somatostatinoma. A Whipple's procedure was performed and a somatostatinoma of the duodenum was confirmed. In addition, the patient had a gastrointestinal stromal tumor (GIST) of the jejunum with accompanying hyperplasia of interstitial cells of Cajal. The somatostatinoma was histologically characteristic with pseudoglandular and solid patterns together with psammoma bodies and lymphovascular invasion. The GIST did not display mutations in c-kit or platelet-derived growth factor receptor genes. The novel finding in this case was the presence of several vessels in the submucosa and muscularis propria of the duodenum displaying prominent intimal hyperplasia and in keeping with so-called neurofibromatosis-associated vasculopathy. These abnormal vessels were within and close to the somatostatinoma only and were not found away from the tumor. It is thought that the vasculopathy is related to NF-1 with abnormal neurofibromin possibly playing a role.
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PMID:Vasculopathic changes, a somatostatin-producing neuroendocrine carcinoma and a jejunal gastrointestinal stromal tumor in a patient with type 1 neurofibromatosis. 1948 62

Pancreatic schwannomas are uncommon. About 60% of pancreatic schwannomas develop cystic lesions, and the differential diagnosis from other cystic pancreatic tumors is difficult. A 43-year-old man presented for evaluation of liver dysfunction detected during a medical checkup. Blood testing detected obstructive jaundice. A computed tomography scan revealed a well-defined polycystic tumor of about 5 cm at the pancreatic head. We performed surgical resection to treat the patient's symptoms and facilitate long-term management. Histopathological examination revealed spindle-shaped cells. Immunohistochemical studies showed S100 protein expression and the absence of CD34 and c-kit protein expression. Finally, we diagnosed a schwannoma. Pancreatic schwannoma is usually asymptomatic. The present case presented with obstructive jaundice, which is reportedly a rare symptom. Pancreatic schwannomas should be considered as a differential diagnosis of pancreatic cystic tumors. Dilatation of the pancreatic duct and the 18-fluorodeoxyglucose positron emission tomography findings are important for the differential diagnosis.
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PMID:A Surgically Resected Pancreatic Schwannoma with Obstructive Jaundice with Special Reference to Differential Diagnosis from Other Cystic Lesions in the Pancreas. 2960 41