胰岛素瘤1例报告并国内文献复习

目的：探讨胰岛素瘤（胰腺β细胞瘤）诊断和治疗方法。 方法：报告收治的1例胰岛素瘤患者并结合国内文献报道的1 362例胰岛素瘤患者资料进行分析。 结果：1 363例患者中,1 286例（94.35%）有典型Whipple三联征表现;1 099例计算胰岛素释放指数（IRI/G）患者中,1 019例（92.72%）IRI/G>0.3。术前B超、CT、MRI、选择性动脉造影（DSA）、选择性动脉内葡萄糖酸钙激惹实验（ASVS）、超声内镜检查（EUS）、经皮经肝门静脉置管分段采血测定胰岛素（PTPC）、生长抑素受体显像（SRS）、术中超声（IOUS）联合扪诊的肿瘤检出情况分别为40.90%（418/1 022）、55.20%（457/823）、52.45%（96/183）、77.42%（247/319）、89.47%（34/38）、78.68%（48/61）、86.11%（62/70）、36.36%（8/22）、93.97%（312/332）。所有患者均行手术治疗,其中1 006例（73.80%）行肿瘤剔除。病理诊断均为胰岛素瘤;42例（3.08%）为恶性变;98例（7.19%）多发;28.25%的肿瘤位于胰头部,33.90%位于胰体部,37.83%位于胰尾部。276例（20.25%）发生胰瘘。术后良性肿瘤复发16例,恶性病变复发7例。 结论：Whipple三联征和IRI/G>0.3可作为胰岛素瘤定性诊断的主要依据。可联合多种方法进行术前定位,IOUS联合扪诊是简单有效的定位诊断方法。肿瘤的局部剜除术是多数胰岛素瘤的最主要的手术治疗方式。     

胰腺脂肪替代的CT表现及临床意义(附3例报告)

目的探讨胰腺脂肪替代（fatty replacement of pancreas）的CT表现及临床意义。方法收集CT发现胰腺脂肪替代患者3例的资料。CT扫描包括平扫、动脉期及门静脉期，观察胰腺各部的形态、大小、密度、胰腺小叶及小叶间隔、胰管走行的显示情况，确定脂肪替代的部位，并结合临床资料及实验室检查结果分析其临床意义。结果①CT表现：完全性脂肪替代2例（胰头前后分、颈、体、尾），大部分脂肪替代1例（胰头前分、颈、体）；2例胰腺形态均规整，1例欠规整；2例胰腺体积稍大，1例胰腺体积正常；3例小叶间隔均增宽及胰腺小叶均萎缩；3例主胰管及分支均无狭窄梗阻。②临床情况：3例患者均有慢性腹泻，2例合并糖尿病（其中1例还合并胆总管炎伴慢性梗阻），另1例合并胆总管小结石，3例血清脂肪酶及1例血清淀粉酶降低。结论胰腺脂肪替代的CT表现有一定的特征性，常合并有胰腺内、外分泌功能不足的临床表现。     

Ruptured superior mesenteric artery aneurysm occurring in association with a heterotopic pancreas: Report of a case

Aneurysmal disease of the visceral arteries is found in only about 0.2% of the population, and the celiac trunk and superior mesenteric artery (SMA) are involved in less than 10% of all visceral aneurysms. We present herein the case of a 71-year-old woman who suffered rupture of a SMA aneurysm. Histological examination of the periarterial tissues which existed next to the aneurysm revealed a heterotopic pancreas. To the best of our knowledge, no other case of an SMA branch aneurysm presenting in association with a heterotopic pancreas has ever been described in either the Japanese or English literature. This is the first report to indicate that a heterotopic pancreas is a likely incidental factor predisposing to visceral aneurysms.     

Mixed Duct-Acinar-Islet Cell Tumor of the Pancreas: Report of a Case

A 72-year-old Japanese woman presented at our hospital complaining of altered consciousness on arising every morning. The laboratory findings showed hypoglycemia and hyperinsulinemia. Abdominal ultrasonography revealed a tumor in the body of the pancreas. With a diagnosis of insulinoma, a surgical excision of the tumor was performed. A light microscopic examination and an immunohistochemical study revealed the tumor to consist of duct, acinar, and islet cell components. Mixed tumors of the pancreas are rare, and their clinical features and pathogenesis remain unclear. A further accumulation of clinical cases as well as a large number of histopathological studies on these rare mixed tumors is needed. Received: March 2, 2000 / Accepted: July 25, 2000     

Coexistence of mucinous cystic neoplasm occurring in the head of the pancreas with annular pancreas: Report of a case

Mucinous cystic neoplasms (MCN) of the pancreas are rare tumors that are almost exclusively located in the body or the tail of the pancreas. A 60-year-old woman with no history of pancreatic disease was referred to our hospital with a chief complaint of dull pain in the upper abdomen. Abdominal computed tomography showed a multilocular cystic mass of 7.0 cm in the head of the pancreas, and endoscopic retrograde cholangiopancreatography showed no communication between the cystic mass and the main pancreatic duct. A pancreatoduodenectomy was performed for the complete resection of the tumor, and an annular pancreas was discovered by accident. The pathological examination of the tumor led to a definitive diagnosis of MCN with ovarian-type stroma. To our knowledge this is the first documented case of MCN occurring in the head of the pancreas and associated with annular pancreas.     

Pancreatic insulinoma combined with glucagon positive cell: A case report

We present a 70-year-old man who was referred for surgery with uncontrollable hypoglycemia. Ultrasonography and abdominal contrast computed tomography revealed a hypervascular tumor of 1 cm in diameter in the pancreatic tail. With a diagnosis of insulinoma, we performed a distal pancreatectomy. The patient showed a good postoperative course without any complications. The patient’s early morning fasting hypoglycemia disappeared. The respective levels of C-peptide and insulin dropped from 14.9 ng/mL and 4860 μIU/mL preoperatively to 5.3 ng/mL and 553 μIU/mL after surgery. A histopathological examination demonstrated that the tumor was a pancreatic neuroendocrine tumor, grade 1. Immunostaining was negative for insulin and positive for CD56, chromogranin A, synaptophysin and glucagon. These findings suggested that the tumor was clinically an insulinoma but histopathologically a glucagonoma. Among all insulinoma cases reported between 1985 and 2010, only 5 cases were associated with independent glucagonoma. In this report, we characterize and discuss this rare type of insulinoma by describing the case we experienced in detail.     

Isolated metastasis of uterine leiomyosarcoma to the pancreas: Report of a case and review of the literature

INTRODUCTION

Metastatic tumors of the pancreas are uncommon and rarely detectable clinically. Metastases to the pancreas are rare. We present a patient with pancreatic metastases from a leiomyosarcoma of the uterus and review the literature about the clinical features of pancreatic metastasis and its surgical management.

PRESENTATION OF CASE

A 40-year-old woman, who underwent hysterectomy, left oophorectomy, omentectomy and lymp node dissection for leiomyosarcoma of the uterus. At the follow up, the patient complained of non-specific abdominal discomfort. Preoperative diagnosis were pancreatic pseudocyst, cystadenoma or cystadenocarcinoma. At laparotomy, a cystic mass was found in the tail of the pancreas which was invased to the transverse colon mesenterium and the spleen. Distal pancreatectomy with splenectomy and transverse colon resection was performed. Histologically, the tumor was evaluated as poorly differentiated leiomyosarcoma.

DISCUSSION

Metastatic lesions of the pancreas are uncommon and less than 2% of all pancreatic malignancies. However a few cases of leiomyosarcoma with metastases to the pancreas have been reported in the literature. Before deciding that the lesion in the pancreas was metastasis, primary leiomyosarcoma of the pancreas had to be ruled out. Histologically, leiomyosarcoma of the pancreas contains interlacing spindle cells with varying degrees of atypia and pleomorphism. The surgical approach to the pancreatic metastases must be aimed complete excision of the tumor with a wide negative margin of clear tissue and maximum preservation of pancreatic remnant if possible.

CONCLUSION

In the absence of widespread metastatic disease, aggressive surgical approach with negative margins must be aimed.     

Huge cystic lymphangioma of the pancreas: report of a case

We report herein the case of a 33-year-old woman who presented with palpable abdominal swelling found to be caused by a huge lymphangioma of the pancreas. An abdominal computed tomographic (CT) scan showed a large multilocular cystic mass with water-dense contents, which was derived from the pancreatic head. A pancreaticoduodenectomy (PD) was performed because the tumor had invaded the duodenum. The resected tumor, which was 23 × 12 × 23 cm in size with 2 l of serous fluid, was pathologically diagnosed as a cystic lymphangioma. The endothelial cells lining the internal surface of the cystic spaces were immunohistochemically positive for factor VIII-R antigen and CD31. Our review of the literature revealed 45 reports of lymphangioma of the pancreas, including this one, but to the best of our knowledge this is only the fifth case that required a PD. Nevertheless, we recommend that a complete resection be performed to reduce the risk of recurrence. Received: April 3, 2000 / Accepted: March 6, 2001     

Total aortic replacement for aortitis syndrome: Report of a case

We report herein the case of a 35-year-old woman who was administered steroid therapy based on a diagnosis of aortitis syndrome. Despite this treatment, the symptoms of cardiac failure gradually progressed, and she was referred to our hospital to undergo surgery. Preoperative evaluations revealed a large aneurysm extending from the ascending aorta to the thoracoabdominal aorta. A two-staged total aortic replacement was carried out and postoperative digital subtraction angiography showed excellent reconstruction of the entire aorta.     

Mitral valve replacement in a patients with an extensively calcified mitral anulus: Report of a case

(Received for publication on Sept. 25, 1996; accepted on May 12, 1997)     

Triple valve replacement late after tetralogy of Fallot repair: Report of a case

Right ventricular failure after repair of tetralogy of Fallot (TOF) is associated with late mortality and morbidity. We report a case of tricuspid and mitral regurgitation with severe pulmonary regurgitation (PR) diagnosed 31 years after repair of TOF. The patient, a 48-year-old woman, presented with severe ventricular arrhythmia and exercise intolerance. She was treated successfully with triple valve replacement, following which her symptoms improved dramatically. She has remained well for 2 years postoperatively.     

Hydatid cyst of the pancreas:Report of an undiagnosed case of pancreatic hydatid cyst and brief literature review

AIM: To overview the literature on pancreatic hydatid cyst(PHC) disease, a disease frequently misdiagnosed during preoperative radiologic investigation.METHODS: PubMed, Medline, Google Scholar, and Google databases were searched to identify articles related to PHC using the following keywords: hydatid cyst, hydatid disease, unusual location of hydatid cyst, hydatid cyst and pancreas, pancreatic hydatid cyst, and pancreatic echinococcosis. The search included let-ters to the editor, case reports, review articles, original articles, meeting presentations and abstracts that had been published between January 2010 and April 2014 without any restrictions on language, journal, or country. All articles identified and retrieved which contained adequate information on the study population(including patient age and sex) and disease and treatment related data(such as cyst size, cyst location, and clinical man-agement) were included in the study; articles with in-sufficient demographic and clinical data were excluded. In addition, we evaluated a case of a 48-year-old fe-male patient with PHC who was treated in our clinic.RESULTS: A total of 58 patients, including our one new case,(age range: 4 to 70 years, mean ± SD: 31.4 ± 15.9 years) were included in the analysis. Twenty-nine of the patients were female, and 29 were male. The information about cyst location was available from studies involving 54 patients and indicated the follow-ing distribution of locations: pancreatic head(n = 21), pancreatic tail(n = 18), pancreatic body and tail(n = 8), pancreatic body(n = 5), pancreatic head and body(n = 1), and pancreatic neck(n = 1). Extra-pancreatic locations of hydatid cysts were reported in the studies involving 44 of the patients. Among these, no other focus than pancreas was detected in 32 of the patients(isolated cases) while 12 of the patients had hydatid cysts in extra-pancreatic sites(liver: n = 6, liver + spleen + peritoneum: n = 2, kidney: n = 1, liver + kidney: n = 1, kidney + peritoneum: n = 1 and liver + lung: n = 1). Serological information was available in the studies involving 40 patients, and 21 of those pa-tients were serologically positive and 15 were serologi-cally negative; the remaining 4 patients underwent no serological testing. Information about pancreatic cyst size was available in the studies involving 42 patients; the smallest cyst diameter reported was 26 mm and the largest cyst diameter reported was 180 mm(mean ± SD: 71.3 ± 36.1 mm). Complications were avail-able in the studies of 16 patients and showed the fol-lowing distribution: cystobiliary fistula(n = 4), cysto-pancreatic fistula(n = 4), pancreatitis(n = 6), and portal hypertension(n = 2). Postoperative follow-up data were available in the studies involving 48 patients and postoperative recurrence data in the studies of 51 patients; no cases of recurrence occurred in any patient for an average follow-up duration of 22.5 ± 23.1(range: 2-120) mo. Only two cases were reported as having died on fourth(our new case) and fifteenth days respectively. CONCLUSION: PHC is a parasitic infestation that is rare but can cause serious pancreato-biliary complica-tions. Its preoperative diagnosis is challenging, as its radiologic findings are often mistaken for other cystic lesions of the pancreas.     

Urgent total arch graft replacement in an elderly patient with acute type a dissection: Report of a case

(Received for publication on July 8, 1996; accepted on Mar. 4, 1997)     

Intraductal papillary mucinous neoplasm of the pancreas associated with neuroendocrine tumor: A case report

IntroductionIntraductal papillary mucinous neoplasm is an uncommon cystic tumor of pancreas that can be associated with ductal adenocarcinoma. Coexistence of pancreatic IPMN and neuroendocrine tumor is very rare. Here, we report the imaging features of mixed type intraductal papillary mucinous neoplasia of the pancreas with high grade dysplasia together with neuroendocrine carcinoma and perform review of the literature.PresentationA 68-year old patient has been evaluated for possible IPMN that was suspected during ultrasound. MRI revealed main and side branch duct dilatations. At the head, a contrast enhancing nodular lesion was identified. Due to the presence of high risk stigmata according to guidelines, surgery was performed. Histopathological examination revealed an unusual association, including mixed type IPMN and neuroendocrine carcinoma.DiscussionThe concomitant occurrence of pancreatic IPMN and neuroendocrine tumor has been reported in case studies and brief reviews. Yet, the imaging findings and underlying molecular mechanisms of this entity has not been fully understood. In addition to this unusual association, pancreatic intraepithelial neoplasia was also detected in the present case. Although majority of neuroendocrine tumor associated IPMNs were reported to be having low grade dysplasia, our patient had high grade dysplasia. Further studies and reviews with larger groups are needed to establish imaging features and underlying molecular mechanisms of this rare association.ConclusionAlthough the major concern during work-up of IPMN is presence of associated pancreatic ductal adenocarcinoma, the possibility of neuroendocrine tumor, in the presence of a hypervascular solid foci on imaging studies should be kept in mind.     

Leiomyosarcoma of the pancreas: Report of a case

Leiomyosarcoma of the pancreas is a rare neoplasm, with only 34 reported cases in the literature. We encountered a rare case of leiomyosarcoma of the pancreas, treated successfully by surgery. A 41-year-old woman was referred to our hospital for further examinations of a pancreatic tumor. Imaging studies demonstrated a solid and lobular mass, about 4 cm in diameter, in the body of pancreas. This mass had a nonuniform content and was encapsulated. We performed distal pancreatectomy and splenectomy for an assumed diagnosis of invasive ductal carcinoma. Macroscopically, a sagittal section of the operative specimen showed a well-circumscribed yellowish-white mass without any cystic changes. Immunohistological examination revealed that α-smooth muscle actin, desmin, and vimentin were positive, and the labeling index of MIB-1 was 50% or more. Based on these findings, we confirmed a diagnosis of leiomyosarcoma originating from the pancreas. During 14 months of follow-up to date, there has not been any evidence of local recurrence or distant metastasis.     

异位胰腺的临床特点及病理特征：附36 例报告

目的：探讨异位胰腺的临床特点、病理特征以及诊断方法。 方法：回顾近2年来收治的36例异位胰腺患者临床资料,分析其临床、病理特点及诊断方法。 结果：全组男19例,女17例,发病以30~49岁发病为多,以胃窦部为高发;36例均表现为反复上腹部不适,其中胃肠镜下诊断为异位胰腺7例,与病理诊断与的吻合率为19.4%。 结论：异位胰腺的发病原因与性别无明显关系,与年龄、浅表性胃炎及糜烂等炎性疾病有关,异位胰腺的确诊需要将临床特点与病理特点相结合。     

Repeat tricuspid valve replacement for acquired valvular heart disease: Report of a case

(Received for publication on May 30, 1997; accepted on Nov. 6, 1997)     

Giant pancreatic incidentaloma: Report of a case and literature review

INTRODUCTIONAsymptomatic lesions of the pancreas, referred to as ‘incidentalomas’, have appeared with increased frequency in recent years. Giant incidentalomas have rarely been reported in the literature.PRESENTATION OF CASEWe report herein a rare case of a giant cystic pancreatic incidentaloma measuring 12.7 cm × 8 cm, which was found in an otherwise healthy male patient during a routine genitourinary imaging work-up. The patient underwent a distal pancreatectomy and splenectomy; the pathology report demonstrated a giant serous cystadenoma of the body and tail of the pancreas.DISCUSSIONThe management of pancreatic incidentalomas is challenging. While solid lesions almost always warrant surgery, there is ongoing debate concerning the management of cystic lesions that are found incidentally in the pancreas and have no clinical manifestations.CONCLUSIONWe report herein an interesting case of a voluminous incidental cystic pancreatic lesion. The appropriate approach and the decision whether to operate or not in such cases can be puzzling to the physician.     

Aggressive angiomyxoma occurring in the scrotum: Report of a case

The authors recently treated a case of aggressive angiomyxoma occurring in the left scrotum. A 61-year-old Japanese man was diagnosed as having an inguinal irreducible hernia that descended to the bottom of the scrotum preoperatively. However, during the operation a large tumor was found between the spermatic fascia and the skin pressing the testis upward. The tumor measured 20×15×15 cm, was yellowish with a smooth surface encapsulated by a membrane similar to the pleura, and the cut surface was gelatinous. The histological findings indicated that the tumor had thick-walled vessels which had prominently increased in size, delicately waved collagen fibers, and stromal cells in a myxoid background. The patient has remained recurrence-free as of 11 months after surgery.