Neurofibromatosis type 1 (NF1) is a common inherited disorder with an autosomal dominant trait. We encountered an NF1 patient who showed adrenal pheochromocytoma, and analyzed expression of neurofibromin in an excised...Neurofibromatosis type 1 (NF1) is a common inherited disorder with an autosomal dominant trait. We encountered an NF1 patient who showed adrenal pheochromocytoma, and analyzed expression of neurofibromin in an excised specimen. A 54-year-old man showing multiple neurofibromas and café-au-lait spots in the skin was pointed out to have a right adrenal tumor by ultrasonography. Abdominal CT also revealed a right adrenal tumor. He was diagnosed with neurofibromatosis type 1 with no family history. Urine catecholamines, metanephrine, and normetanephrine levels were elevated. MIBG scintigraphy showed positive right adrenal uptake, and so pheochromocytoma was also diagnosed. The patient underwent laparoscopic right adrenalectomy. The excised adrenal specimen of this patient was stained with anti-neurofibromin polyclonal antibody. The NF1 pheochromocytoma was completely negative for neurofibromin protein expression, while the NF1 non-pheochromocytomatous adrenal medulla was neurofibromin-positive in the cytoplasm and nucleus. The clear discrepancy in neurofibromin expression between pheochromocytoma cells and “normal” adrenal medullary cells of the patient may well be explained by Knudson’s two-hit hypothesis.展开更多
A 69-year-old woman was diagnosed with primary aldosteronism. An enhanced computed tomography (CT) scan before surgery indicated a right adrenal tumor outside the liver. Venous sampling tests revealed unilateral overp...A 69-year-old woman was diagnosed with primary aldosteronism. An enhanced computed tomography (CT) scan before surgery indicated a right adrenal tumor outside the liver. Venous sampling tests revealed unilateral overproduction of aldosterone by the right adrenal gland. Separation of the right adrenal cortex from the liver parenchyma was impractical during a laparoscopic right adrenalectomy because of the solid attachment between the two. Therefore, the existence of adrenohepatic fusion was determined. An incision was made within the right adrenal gland, leaving completely the intrahepatic adrenal tissue on the inner side of the liver, because a partial hepatectomy was not preoperatively planned, and the patient was not informed of the consent before the surgery. Pathological examination did not reveal macro- or micro-adenomas in the resected right adrenal tissue. Aldosterone to renin ratio was as high as 1380 at 22 days following the surgery. Therefore, aldosteronoma originated from the adrenohepatic fusion that remained on the inner side of the liver was highly suspected. The patient’s blood pressure was well controlled, and she did not prefer hepatectomy to be further performed, and therefore, medical therapy was continued. When planning the type of surgery (laparoscopic or open) in these potentially confusing cases, it might be necessary to consider a possibility of the unexpected intraoperative diagnosis and the immediate measures to be performed based on the diagnosis.展开更多
High mobility group box (HMGB) proteins are nuclear nonhistone chromosomal proteins that bend DNA, bind preferentially to distorted DNA structures, and promote the assembly of site-specific DNA binding proteins. Recen...High mobility group box (HMGB) proteins are nuclear nonhistone chromosomal proteins that bend DNA, bind preferentially to distorted DNA structures, and promote the assembly of site-specific DNA binding proteins. Recent reports indicate that HMGB1 has a dual function, a cytokine in addition to a nuclear protein. The increased expression of HMGB1 has been reported for several different tumors. Here, we assessed HMGB1 and HMGB2 expressions in two cases of papillary renal cell carcinoma. One case with pT1a, Grade 2 showed HMGB1 expression in the nucleus and cytosol and HMGB2 expression in the nucleus, but not in the cytosol. In the other case, there were three renal tumors, one of which was clear cell renal cell carcinoma with pT1a, Grade 3 and two were papillary renal cell carcinomas, Grade 2 (5 mmand2 mmin the diameter). Both HMGB1 and HMGB2 were expressed in the nucleus and cytosol of papillary carcinoma. In the clear cell carcinoma of this case, HMGB1 expression was stained both in the nucleus and cytosol, while HMGB2 was observed in the nucleus, but not in the cytosol. More samples need to be further investigated in order to draw conclusions concerning HMGB expressions in papillary renal cell carcinomas.展开更多
A 59-year-old male was admitted to our hospital because of incidentally found right retroperitoneal tumor. He had undergone removal of a hemangioma in the left oral cavity four years before. An abdominal CT scan perfo...A 59-year-old male was admitted to our hospital because of incidentally found right retroperitoneal tumor. He had undergone removal of a hemangioma in the left oral cavity four years before. An abdominal CT scan performed in our hospital revealed poorly enhanced bilateral retroperitoneal tumors adjacent to kidneys. Those tumors were of low signal intensity on T1-weighted images and high on T2-weighted images by magnetic resonance imaging. The right retroperitoneal tumor of 2.5 cm in size was surgically removed and histopathological examination indicated cavernous hemangioma. The smaller left retroperitoneal tumor of 1.1 cm in size was left untouched to be followed up, as we supposed that it has the same benign pathology. There have been no previous cases of retroperitoneal cavernous hemangioma as a presentation of multiple hemangiomas.展开更多
文摘Neurofibromatosis type 1 (NF1) is a common inherited disorder with an autosomal dominant trait. We encountered an NF1 patient who showed adrenal pheochromocytoma, and analyzed expression of neurofibromin in an excised specimen. A 54-year-old man showing multiple neurofibromas and café-au-lait spots in the skin was pointed out to have a right adrenal tumor by ultrasonography. Abdominal CT also revealed a right adrenal tumor. He was diagnosed with neurofibromatosis type 1 with no family history. Urine catecholamines, metanephrine, and normetanephrine levels were elevated. MIBG scintigraphy showed positive right adrenal uptake, and so pheochromocytoma was also diagnosed. The patient underwent laparoscopic right adrenalectomy. The excised adrenal specimen of this patient was stained with anti-neurofibromin polyclonal antibody. The NF1 pheochromocytoma was completely negative for neurofibromin protein expression, while the NF1 non-pheochromocytomatous adrenal medulla was neurofibromin-positive in the cytoplasm and nucleus. The clear discrepancy in neurofibromin expression between pheochromocytoma cells and “normal” adrenal medullary cells of the patient may well be explained by Knudson’s two-hit hypothesis.
文摘A 69-year-old woman was diagnosed with primary aldosteronism. An enhanced computed tomography (CT) scan before surgery indicated a right adrenal tumor outside the liver. Venous sampling tests revealed unilateral overproduction of aldosterone by the right adrenal gland. Separation of the right adrenal cortex from the liver parenchyma was impractical during a laparoscopic right adrenalectomy because of the solid attachment between the two. Therefore, the existence of adrenohepatic fusion was determined. An incision was made within the right adrenal gland, leaving completely the intrahepatic adrenal tissue on the inner side of the liver, because a partial hepatectomy was not preoperatively planned, and the patient was not informed of the consent before the surgery. Pathological examination did not reveal macro- or micro-adenomas in the resected right adrenal tissue. Aldosterone to renin ratio was as high as 1380 at 22 days following the surgery. Therefore, aldosteronoma originated from the adrenohepatic fusion that remained on the inner side of the liver was highly suspected. The patient’s blood pressure was well controlled, and she did not prefer hepatectomy to be further performed, and therefore, medical therapy was continued. When planning the type of surgery (laparoscopic or open) in these potentially confusing cases, it might be necessary to consider a possibility of the unexpected intraoperative diagnosis and the immediate measures to be performed based on the diagnosis.
文摘High mobility group box (HMGB) proteins are nuclear nonhistone chromosomal proteins that bend DNA, bind preferentially to distorted DNA structures, and promote the assembly of site-specific DNA binding proteins. Recent reports indicate that HMGB1 has a dual function, a cytokine in addition to a nuclear protein. The increased expression of HMGB1 has been reported for several different tumors. Here, we assessed HMGB1 and HMGB2 expressions in two cases of papillary renal cell carcinoma. One case with pT1a, Grade 2 showed HMGB1 expression in the nucleus and cytosol and HMGB2 expression in the nucleus, but not in the cytosol. In the other case, there were three renal tumors, one of which was clear cell renal cell carcinoma with pT1a, Grade 3 and two were papillary renal cell carcinomas, Grade 2 (5 mmand2 mmin the diameter). Both HMGB1 and HMGB2 were expressed in the nucleus and cytosol of papillary carcinoma. In the clear cell carcinoma of this case, HMGB1 expression was stained both in the nucleus and cytosol, while HMGB2 was observed in the nucleus, but not in the cytosol. More samples need to be further investigated in order to draw conclusions concerning HMGB expressions in papillary renal cell carcinomas.
文摘A 59-year-old male was admitted to our hospital because of incidentally found right retroperitoneal tumor. He had undergone removal of a hemangioma in the left oral cavity four years before. An abdominal CT scan performed in our hospital revealed poorly enhanced bilateral retroperitoneal tumors adjacent to kidneys. Those tumors were of low signal intensity on T1-weighted images and high on T2-weighted images by magnetic resonance imaging. The right retroperitoneal tumor of 2.5 cm in size was surgically removed and histopathological examination indicated cavernous hemangioma. The smaller left retroperitoneal tumor of 1.1 cm in size was left untouched to be followed up, as we supposed that it has the same benign pathology. There have been no previous cases of retroperitoneal cavernous hemangioma as a presentation of multiple hemangiomas.
