Calcifying fibrous tumor is a benign mass lesion classically described as a soft tissue tumor. Ho... more Calcifying fibrous tumor is a benign mass lesion classically described as a soft tissue tumor. However, a thorough review of the literature reveals that it can occur virtually anywhere, including the tubular gastrointestinal (GI) tract. Its clinical manifestations are variable in the GI tract, and its imaging findings are nonspecific. However, it has unique histologic and immunophenotypical features that must be recognized by GI pathologists to differentiate it from an assortment of other rare mesenchymal lesions of the abdomen and GI tract. Calcifying fibrous tumor is composed of a paucicellular collagen matrix, interspersed calcified bodies, and a sparse inflammatory infiltrate. Although calcifying fibrous tumor is benign, pathologists must be aware that it may occur in the GI tract to differentiate it from other potentially more aggressive, rare mesenchymal lesions.
Mild idiopathic adulthood ductopenia (IAD) is a rare cholestatic disease of unknown cause and cha... more Mild idiopathic adulthood ductopenia (IAD) is a rare cholestatic disease of unknown cause and characterized by interlobular bile duct loss in less than 50% of the portal tracts. We describe the case of a middLe-aged male who presented with persistent elevation of transaminases and alkaline phosphatase. He had a normal biliary tree on endoscopic retrograde cholangiopathy and negative anti-mitochondrial antibody. His liver biopsy specimen showed chronic biliary disease, duct loss in 4 out of 15 portal tracts and prominent cholestasis. Based on the work-up, he likely had mild IAD. Liver transplantation would be necessary if his disease becomes progressive.
Ki-67 proliferative index (Ki-67 index) is suggested to be an important prognostic variable and i... more Ki-67 proliferative index (Ki-67 index) is suggested to be an important prognostic variable and is included as one of the grading parameters for neuroendocrine tumors. The present study was undertaken to determine the usefulness of the Ki-67 index and the corresponding tumor grade in predicting progression-free survival (PFS) of patients with ileal well-differentiated neuroendocrine tumors (wNETs). Tumors from 57 patients with ileal wNETs were studied. Immunohistochemical staining for Ki-67 was performed on the primary as well as selected metastatic tumors and quantitated by computer-assisted image analysis using the Ariol system. The tumors were graded based on mitotic activity and Ki-67 index. Clinical and pathological variables affecting the PFS were analyzed. There were 29 women and 28 men, with a mean age of 59 years. At the time of initial presentation, 8 patients (14%) had localized disease (stages I and II), 29 patients (51%) had regional (nodal/mesenteric) spread (stage III...
We report a rare case of nesidioblastosis and hyperplasia of alpha cells, microglucagonoma, and n... more We report a rare case of nesidioblastosis and hyperplasia of alpha cells, microglucagonoma, and nonfunctioning islet cell tumor of the pancreas. The patient's clinical presentation, diagnosis, treatment, pancreas pathology, and follow-up are reviewed. A 60-year-old patient was incidentally found to harbor a pancreatic mass with markedly elevated glucagon levels but without glucagonoma syndrome. She was initially diagnosed with glucagonoma, and the tumor was resected. Pathological examination demonstrated that the tumor was a nonfunctioning islet cell tumor and revealed nesidioblastosis and hyperplasia of alpha cells and microglucagonoma in the apparently normal surgical margin. The patient still had high postoperative glucagons levels which were suppressed by somatostatin analog treatment. No pancreatic tumors recurred 36 months after surgery. This is the third case of alpha-cell nesidioblastosis reported in the English literature. Nesidioblastosis and hyperplasia of alpha cells should be considered in the differential diagnosis of hyperglucagonemia. Somatostatin analog may be used to suppress glucagon secretion in alpha-cell hyperplasia.
Calcifying fibrous tumor is a benign mass lesion classically described as a soft tissue tumor. Ho... more Calcifying fibrous tumor is a benign mass lesion classically described as a soft tissue tumor. However, a thorough review of the literature reveals that it can occur virtually anywhere, including the tubular gastrointestinal (GI) tract. Its clinical manifestations are variable in the GI tract, and its imaging findings are nonspecific. However, it has unique histologic and immunophenotypical features that must be recognized by GI pathologists to differentiate it from an assortment of other rare mesenchymal lesions of the abdomen and GI tract. Calcifying fibrous tumor is composed of a paucicellular collagen matrix, interspersed calcified bodies, and a sparse inflammatory infiltrate. Although calcifying fibrous tumor is benign, pathologists must be aware that it may occur in the GI tract to differentiate it from other potentially more aggressive, rare mesenchymal lesions.
Mild idiopathic adulthood ductopenia (IAD) is a rare cholestatic disease of unknown cause and cha... more Mild idiopathic adulthood ductopenia (IAD) is a rare cholestatic disease of unknown cause and characterized by interlobular bile duct loss in less than 50% of the portal tracts. We describe the case of a middLe-aged male who presented with persistent elevation of transaminases and alkaline phosphatase. He had a normal biliary tree on endoscopic retrograde cholangiopathy and negative anti-mitochondrial antibody. His liver biopsy specimen showed chronic biliary disease, duct loss in 4 out of 15 portal tracts and prominent cholestasis. Based on the work-up, he likely had mild IAD. Liver transplantation would be necessary if his disease becomes progressive.
Ki-67 proliferative index (Ki-67 index) is suggested to be an important prognostic variable and i... more Ki-67 proliferative index (Ki-67 index) is suggested to be an important prognostic variable and is included as one of the grading parameters for neuroendocrine tumors. The present study was undertaken to determine the usefulness of the Ki-67 index and the corresponding tumor grade in predicting progression-free survival (PFS) of patients with ileal well-differentiated neuroendocrine tumors (wNETs). Tumors from 57 patients with ileal wNETs were studied. Immunohistochemical staining for Ki-67 was performed on the primary as well as selected metastatic tumors and quantitated by computer-assisted image analysis using the Ariol system. The tumors were graded based on mitotic activity and Ki-67 index. Clinical and pathological variables affecting the PFS were analyzed. There were 29 women and 28 men, with a mean age of 59 years. At the time of initial presentation, 8 patients (14%) had localized disease (stages I and II), 29 patients (51%) had regional (nodal/mesenteric) spread (stage III...
We report a rare case of nesidioblastosis and hyperplasia of alpha cells, microglucagonoma, and n... more We report a rare case of nesidioblastosis and hyperplasia of alpha cells, microglucagonoma, and nonfunctioning islet cell tumor of the pancreas. The patient's clinical presentation, diagnosis, treatment, pancreas pathology, and follow-up are reviewed. A 60-year-old patient was incidentally found to harbor a pancreatic mass with markedly elevated glucagon levels but without glucagonoma syndrome. She was initially diagnosed with glucagonoma, and the tumor was resected. Pathological examination demonstrated that the tumor was a nonfunctioning islet cell tumor and revealed nesidioblastosis and hyperplasia of alpha cells and microglucagonoma in the apparently normal surgical margin. The patient still had high postoperative glucagons levels which were suppressed by somatostatin analog treatment. No pancreatic tumors recurred 36 months after surgery. This is the third case of alpha-cell nesidioblastosis reported in the English literature. Nesidioblastosis and hyperplasia of alpha cells should be considered in the differential diagnosis of hyperglucagonemia. Somatostatin analog may be used to suppress glucagon secretion in alpha-cell hyperplasia.
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