Left-sided portal hypertension caused by serous cystadenoma of the pancreas: Report of a case

We report a case of serous cystadenoma of pancreas causing left-sided portal hypertension and gastric varices. A 68-year-old man was admitted for treatment of a pancreatic body tumor. Contrast-enhanced computed tomography (CT) showed a honeycombed cystic mass. A celiac angiogram showed a hypervascular tumor supplied mainly by a dilated splenic artery and dorsal pancreatic artery. In the venous phase, the patent splenic vein had a large hepatopetal collateral vein via the coronary gastric vein. Upper gastrointestinal endoscopy showed isolated varices of the gastric fundus. We made a preoperative diagnosis of a serous cystic tumor of the pancreas with left-sided portal hypertension and performed distal pancreatectomy with splenectomy. The resected tumor was 8 cm in diameter and had a typical honeycombed microcystic pattern with central stellate scarring. The spleen was not enlarged. Histopathological examination confirmed a diagnosis of serous cystadenoma without any sign of malignancy. Postoperative endoscopy showed disappearance of the gastric varices.     

Serous cystadenocarcinoma of the pancreas with liver metastases

Serous cystadenocarcinoma of the pancreas is a rare entity. We report a primary tumor of the pancreas in a 56-year-old woman that was histologically indistinguishable from microcystic adenoma, but which behaved in a malignant fashion. Metastatic lesions were found in the liver at the time of the initial operation. Nine years after the initial operation, new metastatic liver nodules were found, and the histologic characteristics of these lesions were quite similar to those of the pancreatic neoplasm. This is a very rare case which may support the existence of the entity, serous cystadenocarcinoma of the pancreas. Received for publication on Mar. 27, 1998; accepted on May 6, 1998     

Carcinoma ex microcystic adenoma of the pancreas: a report of a novel form of malignancy in serous neoplasms

Serous cystic neoplasms of the pancreas are generally considered benign lesions. Malignant counterparts have been occasionally described, and the diagnosis of malignancy is based solely on the presence of synchronous or metachronous metastases to the lymph nodes or liver, direct tumor invasion into adjacent organs, or vascular invasion. However, these malignant serous cystic tumors are lined by benign-appearing glycogen-rich cuboidal cells, which have been morphologically indistinguishable from benign microcystic serous cystadenoma in all the cases reported so far. We report a unique case of microcystic serous cystadenoma giving rise to carcinoma with distinctive histologic features including signet ring-like cells and solid nests. We believe that this case represents the first case of a cytologically malignant neoplasm arising from a benign serous cystadenoma (carcinoma ex microcystic serous cystadenoma).     

Clear cell endocrine pancreatic tumor mimicking renal cell carcinoma: a distinctive neoplasm of von Hippel-Lindau disease

The dominantly inherited von Hippel-Lindau disease is characterized by clear cell neoplasms in various organs including the kidney and pancreas. Determination of primary versus metastatic lesion in this setting can be a diagnostic dilemma. The authors present five cases of clear cell endocrine pancreatic tumor (EPT) closely mimicking renal cell carcinomas in five patients with a family history or histologic evidence of von Hippel-Lindau disease. In fact, two of these tumors were confused with metastatic renal cell carcinoma by fine-needle aspiration. All five tumors had a component of clear cells arranged in nests, cords, and tubules with central hemorrhage separated by thin-wall vessels resembling renal cell carcinoma. However, these tumors also exhibited cords and festoons and a gyriform pattern suggestive of an endocrine neoplasm, and expressed chromogranin and synaptophysin. Vascular invasion was identified in four tumors, one of which metastasized. The concurrent primary renal cell carcinomas and the multicentric microcystic adenomas found in three patients did not show reactivity for the neuroendocrine markers. Focal clear cell change was noted in only one of 29 endocrine pancreatic tumors arising in patients without von Hippel-Lindau disease. Eleven metastatic renal cell carcinomas in the pancreas did not show immunoreactivity with the endocrine markers. Clear cell EPTs closely mimicking renal cell carcinoma are distinctive neoplasms of von Hippel-Lindau disease. In contrast to clear cell EPT, metastatic renal cell carcinoma does not express neuroendocrine markers and lacks neurosecretory granules by electron microscopy. Von Hippel-Lindau disease should be strongly suspected in patients with renal cell carcinoma, clear cell EPT, and multifocal microcystic serous adenomas.     

胰腺囊性肿瘤的诊断与治疗

自１９６５～１９９４年，作者收治了１６例胰腺囊性肿瘤病人。男性４例，女性１２例，平均年龄５０９岁。１６例中浆液性囊腺瘤２例，良性的粘液性囊腺瘤７例，粘液性囊腺癌４例，乳头状囊腺癌１例，低分化腺癌１例，未分化癌１例。Ｂ超和ＣＴ检查对于肿物的检出、浆液性囊腺瘤内多发小囊的分辨，以及肿物周边钙化的显示具有一定价值。囊肿壁不完全的活组织检查可导致冰冻甚至石腊切片的误诊。除了个别已被确诊的无症状的浆液性囊腺瘤可进行观察，其余各种类型的病变均要行手术切除。本文对各种类型囊性肿瘤的特征、囊性肿瘤与假性囊肿的鉴别诊断以及各类手术方式进行了讨论。     

Cystic tumors of the pancreas. New clinical, radiologic, and pathologic observations in 67 patients.

Within a 12-year period we treated 67 patients (49 women, 18 men; mean age, 61 years) with cystic neoplasms of the pancreas, including 18 serous cystic adenomas, 15 benign mucinous cystic neoplasms, 27 mucinous cystadenocarcinomas, 3 papillary cystic tumors, 2 cystic islet cell tumors, and 2 cases of mucinous ductal ectasia. Mean tumor size was 6 cm (2 to 16 cm). In 39% the patients had no symptoms, and in 37% the lesions had been misdiagnosed as a pseudocyst. Computed tomography was useful for detection, for distinguishing the microcystic subgroup of serous cystadenoma, and for showing rim calcification (all 7 cases were malignant) but was not reliable for distinguishing neoplasm from pseudocyst, serous from mucinous tumors, or benign from malignant. Arteriography showed hypervascularity in 4 of 10 serous adenomas, 3 of 11 mucinous carcinomas, and 1 of 1 papillary cystic tumors. Endoscopic pancreatography showed no communication with the cyst cavity in 37 of 37 cases of cystic neoplasms but opacified the ectatic ducts in 2 of 2 cases of mucinous ductal ectasia. Stenosis or obstruction of the pancreatic duct indicated cancer. The tumor was resected by distal pancreatectomy in 25 patients, by proximal resection in 29, and by total pancreatectomy in one, with no operative deaths. Forty-four per cent of the tumors were malignant. In 10 cases the tumor was unresectable because of local extension or distant metastases, and those patients died at a mean of 4 months. Seventy-five per cent of those resected for cure are alive without evident recurrence. Because the epithelial lining of the tumor was partially (5% to 98%) absent in 40% to 72% of cases of the major tumor types, and the mucinous component comprised only about 65% of mucinous cystadenoma lining, misdiagnoses on frozen and even permanent sections were made. Mitoses and histologic solid growth correlated with malignancy. Neuroendocrine elements were seen in 87% of benign and 47% of malignant mucinous tumors. It is recommended that the terms macrocystic and microcystic be abandoned in favor of the histologic designations serous and mucinous. Incomplete examination of the cyst wall can be misleading, however. It is suggested that mucinous ductal ectasia be recognized separately from cystic tumors and that all of these lesions be resected, with the possible exception of asymptomatic confirmed serous cystadenomas.     

Unilocular macrocystic serous cystadenoma of the pancreas: a morphologic variant to be considered

Serous cystadenoma of the pancreas is a benign cystic tumor, which radiological diagnosis is easy in its typical microcystic variant. The macrocystic variant is uncommon and raises diagnostic problems with other macrocystic lesions of the pancreas such as pseudocysts and mucinous cystadenomas. We report the case of a young woman with a unilocular macrocystic serous cystadenoma of the pancreas which was identified on pathologic examination of the surgical specimen, after unconclusive abdominal ultrasound and CT-scan. This case describes an unusual clinical presentation of this cystic tumor and emphasizes that the diagnosis of such an entity is still based on pathological examination after cyst removal.     

Serous cystadenocarcinoma of the pancreas: a new entity?

Microcystic cystadenoma of the pancreas is a well-recognized although rare pathological entity. All previously reported examples of this tumor have been uniformly benign in behavior. In this case report, we present a primary tumor of the pancreas that was histologically indistinguishable from microcystic adenoma, but which behaved in a malignant fashion. Metastases were found in the stomach and liver. We believe that this case represents a new entity, which we have termed "serous cystadenocarcinoma of the pancreas."     

Macrocystic serous cystadenoma of the pancreas

We report a 47-year-old woman with macrocystic serous cystadenoma of the pancreas. She had no past history of abdominal surgery, instrumentation, or trauma. Ultrasonography and computed tomography revealed a unilocular cyst in the body of the pancreas. On magnetic resonance imaging, the cyst showed heterogeneous signal intensity on T1-weighted images, and was homogeneously hyperintense and oligolocular is on T2-weighted images. A preoperative diagnosis of mucinous cystic neoplasm of the pancreas was made, and distal pancreatectomy was performed. The resected oligolocular cyst was 5.0 × 4.5 × 3.0 cm and was lined with a single layer of cuboidal epithelium similar to that seen in microcystic serous cystadenomas. Abundant glycogen was demonstrated within the epithelial cells, as assessed by periodic acid-Schiff (PAS) staining with and without diastase digestion. The cyst exhibited a gross appearance distinct from that of typical microcystic adenomas, resulting in diagnostic difficulties for the radiologists and surgeon involved in the patient's care. Received for publication on May 20, 1999; accepted on Sept. 1, 1999     

Two cases of pancreatic serous cystadenoma

Two cases of operated pancreatic serous cystadenoma were reported with radiological findings. A 67-year-old man was admitted to our hospital complaining of pain in the left upper quadrant. Ultrasonography revealed a mass having a mixed hypoechoic and echogenic pattern at the body of the pancreas. Computed tomography showed a honeycomb appearance. Angiogram showed hypervascularity and ERCP finding showed narrowing of the main pancreatic duct at the body of the pancreas. A 73-year-old man was detected incidentally to have a low density mass with central satellite appearance by computed tomography at the tail of the pancreas during the examination for the renal tumor. The mass showed hypervascularity and A-V shunt angiographically and narrowing of the main pancreatic duct at the tail of the pancreas by ERCP. Both patients underwent distal pancreatectomy. Histopathological findings showed microcystic pattern with single lining epithelial cells which were low cuboidal or flattened and contained intracytoplasmic glycogen.     

Demonstration of microcystic disease of the pancreas by magnetic resonance cholangiopancreatography: Report of a case

A case of microcystic disease of the pancreas which was clearly demonstrated by magnetic resonance cholangiopancreatography (MRCP) is reported herein. Cystic dilatation of the pancreatic duct was recognized by computed tomography scanning and endoscopic retrograde cholangiopancreatography (ERCP). Furthermore, the existence of microcystic clusters surrounding the dilated pancreatic duct were clearly visualized by MRCP. These microcystic clusters were strongly suspected preoperatively of having caused dilatation of the major pancreatic duct. Based on these findings, a distal pancreatectomy was performed. The operative specimen showed no accumulation of mucin and no evident lesions in the dilated pancreatic duct, being inconsistent with the entity of a mucus-producing tumor. Pathological examination revealed that the inner parts of microcysts constituted columnar epithelium with mucus production and papillary growth. Thus, a final histological diagnosis of intraductal papillary adenoma with idiopathic pancreatic duct ectasia was confirmed. In conclusion, MRCP, being a less aggressive diagnostic procedure than ERCP, proved extremely useful for obtaining precise information on cystic lesions of the pancreas in this patient.     

胰腺浆液性囊腺肿瘤的临床诊断及治疗方法分析

目的研究分析胰腺浆液性囊腺肿瘤的临床诊断和治疗方法,以提高诊断率和治疗水平。方法选取我院从1997年10月至2013年1月收治的60例经病理证实的胰腺浆液性囊腺肿瘤患者作为研究对象,对其临床资料进行回顾性分析。结果 60例患者中,男性患者14例,占23.33%,女性患者46例,占76.67%;患者年龄24岁至88岁,平均年龄57.88±3.52岁。术前无明显临床症状患者20例,占33.33%。所有患者行B超检查,提示胰腺有囊性或囊实性占位者50例(83.33%),14例确诊为浆液性囊腺瘤(23.33%)。52例患者行CT检查,确诊浆液性囊腺瘤38例,诊断符合率为73.08%。所研究患者均采用手术治疗,其中行胰十二指肠切除术18例(30.00%),胰腺远端切除术40例(66.66%),肿瘤剥除术1例(1.67%),胃空肠,胆管空肠吻合术1例(1.67%)。术后有6例患者出现并发症(占10.00%),主要并发症有胰瘘,内出血,幽门梗阻,胰腺假性囊肿合并肠瘘及不同程度的胸腔积液。术后随访1至15年,除1例浆液性囊腺癌患者于术后14个月死亡外,其余预后良好,术后无复发。术后对肿瘤进行病理分型,分为微囊型浆液性囊腺瘤52例(占86.67%),寡囊型浆液性囊腺瘤8例(占13.33%),包括微囊型浆液性囊腺癌1例。寡囊型患者的术后平均住院日为(20.11±0.87)d大于微囊型(17.12±6.77)d,差异有统计学意义(P=0.030)。按肿瘤大小分为两组,直径≥4 cm组和直径4 cm组。肿瘤直径≥4 cm组患者临床症状发生率为76.56%高于直径-4 cm组临床症状发生率23.10%(P=0.021),其余临床指标经统计学处理无显著差异。结论胰腺浆液性囊腺瘤好发于中老年女性,无特异性临床表现。B超检查适用于初步诊断和筛查,CT诊断准确率较高,有重要临床诊断价值。肿瘤多发于胰体尾部,胰体尾切除术为主要手术方式。术后主要并发症为胰瘘。经手术治疗后,患者症状得到改善,远期预后较好。     

Pancreatic solid serous cystadenoma treated by laparoscopy: Presentation of a new case report and review of the literature

Solid serous cystadenoma is an uncommon benign pancreatic tumor, with only, including this case, 21 cases published so far. It is often misdiagnosis with other malignant pancreatic tumors.Below we report a new case of a solid serous cystadenoma of the pancreas treated by laparoscopic distal pancreatectomy in 53-year-old female who presented with epigastric pain. Histological and immunohistochemical examination revealed a solid serous cystadenoma of the pancreas. Preoperative diagnosis of this subtype of serous cystadenoma is difficult, and, due to its benign nature, conservative resection of the tumor is the recommended treatment.After analyzing the literature, including this case from our department, we discuss clinical presentation, imaging characteristics and histopathological findings, considering in particular difficulties in preoperative diagnosis, feasibility of laparoscopic resection.     

胰腺浆液性微囊性腺瘤临床病理学观察

目的 探讨胰腺浆液性微囊性腺瘤(serous microcystic adenoma,SMA)的临床与病理学特点.方法 对12例SMA进行临床病理、特殊染色及免疫组化观察并随访.结果 ①临床:12例SMA中,男3例、女9例,35～70岁,平均51.5岁;7例因体检发现,5例因上腹不适或疼痛就诊;肿瘤直径2.0～13 cm,平均6.0 cm,切面均呈蜂窝状,囊内含清亮液体或灰红色液体.②镜下:瘤组织由多量小囊腔构成,囊壁内衬单层立方或扁平上皮,胞浆透亮,富含糖原,部分嗜酸,核小而圆,深染,大小一致,无明显异型性,核分裂像极少或无.③特殊染色:瘤细胞PAS(+),AB-PAS(-).④免疫组化:AEl/AE3及EMA(+),CEA、Vimentin、CgA、Syn、CD34、P53及S-100均(-).10例病人随访12～71个月,均未见复发.结论 SMA为胰腺罕见的肿瘤,临床上多无明显症状,依据其特有的病理组织学形态及免疫组化染色可明确诊断.其具体生物学行为有待进一步研究.     

胰腺浆液性微囊性腺瘤临床病理学观察

目的 探讨胰腺浆液性微囊性腺瘤(serous microcystic adenoma,SMA)的临床与病理学特点.方法 对12例SMA进行临床病理、特殊染色及免疫组化观察并随访.结果 ①临床:12例SMA中,男3例、女9例,35～70岁,平均51.5岁;7例因体检发现,5例因上腹不适或疼痛就诊;肿瘤直径2.0～13 cm,平均6.0 cm,切面均呈蜂窝状,囊内含清亮液体或灰红色液体.②镜下:瘤组织由多量小囊腔构成,囊壁内衬单层立方或扁平上皮,胞浆透亮,富含糖原,部分嗜酸,核小而圆,深染,大小一致,无明显异型性,核分裂像极少或无.③特殊染色:瘤细胞PAS(+),AB-PAS(-).④免疫组化:AEl/AE3及EMA(+),CEA、Vimentin、CgA、Syn、CD34、P53及S-100均(-).10例病人随访12～71个月,均未见复发.结论 SMA为胰腺罕见的肿瘤,临床上多无明显症状,依据其特有的病理组织学形态及免疫组化染色可明确诊断.其具体生物学行为有待进一步研究.     

胰腺浆液性微囊性腺瘤临床病理学观察

目的 探讨胰腺浆液性微囊性腺瘤(serous microcystic adenoma,SMA)的临床与病理学特点.方法 对12例SMA进行临床病理、特殊染色及免疫组化观察并随访.结果 ①临床:12例SMA中,男3例、女9例,35～70岁,平均51.5岁;7例因体检发现,5例因上腹不适或疼痛就诊;肿瘤直径2.0～13 cm,平均6.0 cm,切面均呈蜂窝状,囊内含清亮液体或灰红色液体.②镜下:瘤组织由多量小囊腔构成,囊壁内衬单层立方或扁平上皮,胞浆透亮,富含糖原,部分嗜酸,核小而圆,深染,大小一致,无明显异型性,核分裂像极少或无.③特殊染色:瘤细胞PAS(+),AB-PAS(-).④免疫组化:AEl/AE3及EMA(+),CEA、Vimentin、CgA、Syn、CD34、P53及S-100均(-).10例病人随访12～71个月,均未见复发.结论 SMA为胰腺罕见的肿瘤,临床上多无明显症状,依据其特有的病理组织学形态及免疫组化染色可明确诊断.其具体生物学行为有待进一步研究.     

胰腺浆液性微囊腺瘤的不典型临床影像特点

目的:探讨胰腺浆液性微囊腺瘤的不典型临床影像表现及诊治策略。方法:回顾2008年7月至2019年10月北京协和医院收治的11例术前临床影像表现不典型的胰腺浆液性微囊腺瘤的患者资料,总结其临床表现、CT/MRI特点、术前临床诊断、手术方法、术后情况等。结果:共纳入11例不典型微囊腺瘤患者,中位年龄50(46～66)岁,女...     

Pancreatic cystadenoma

Pancreatic cystadenomas are infrequent cystic neoplasms which include 2 different lesions: microcystic cystadenomas and mucinous cystadenomas. Over a 37 year period, we observed 25 pancreatic cystadenomas: 15 microcystic cystadenomas and 10 mucinous cystadenomas. The microcystic cystadenomas corresponded either to a localized tumor (13 cases) or multiple diffuse cystic lesions of similar histology occurring within the context of Von Hippel-Lindau disease (2 cases). Localized or multiple microcystic cystadenomas are benign tumors with no risk of degeneration. Mucinous cystadenomas are potentially malignant large unilocular or multilocular cystic tumors. Preoperative differentiation between the 2 types of cystadenomas is based essentially on ultrasound and computed tomography to a lesser extent on angiography. Localized microcystic cystadenomas are low-density, hypervascularized solid or mixed tumors. Mucinous cystadenomas are anechogenic, often show septation, and are water-dense and avascular. They should be differentiated from a microcystic cystadenoma with unilocular cyst features (2 cases in our series) and especially from pancreatic pseudocyst, particularly in case of a history of trauma and associated chronic pancreatitis (2 cases). Multiple microcystic cystadenomas should be differentiated from congenital polycystic disease of the pancreas. Determination of the benign or malignant nature of a mucinous cystadenoma is difficult despite the contribution of ultrasonically-guided percutaneous puncture and microbiopsy. Such samples are either insufficient or too limited, incurring the risk of over looking an area of localized degeneration. However, intraoperative biopsy of pancreatic cyst wall can correct an erroneous diagnosis of pseudocyst (1 case) and avoid inappropriate internal drainage. Total excision of mucinous cystadenomas represents the only suitable solution in view of the malignant potential of this tumor.(ABSTRACT TRUNCATED AT 250 WORDS)     

A giant pancreatic serous microcystic adenoma with 20 years follow-up

BACKGROUND AND AIMS: There is only little information about the spontaneous course of large pancreatic serous tumours. We followed up a white woman with a giant serous microcystic adenoma over more than 20 years. CASE REPORT: At first clinical presentation, in 1986, the tumour measured 4.5 cm in diameter. Two years later, it measured 6 cm and was considered as non-resectable at laparotomy. A biopsy was obtained, and the tumour was diagnosed as lymphangioma, based on hematoxylin and eosin-staining. During the follow-up, the tumour progressively increased in size, measuring 12 cm in diameter in 1993 and 17 cm in 2000. Thus, an average growth rate of 0.83 cm per year was calculated. Unspecific abdominal discomfort and pain were the leading clinical symptoms. A colonic resection was necessary because of compression by the tumour in 1993. Portal hypertension was detected at laparotomy. Finally, the initial biopsy specimen was re-evaluated, using immunohistochemistry, and the final diagnosis of a serous microcystic adenoma was made. CONCLUSION: This unique case demonstrates that the spontaneous course of serous microcystic adenoma of the pancreas may be favourable even with huge tumour size and that immunohistochemistry may prove a valuable tool for differential diagnosis of cystic pancreatic lesions. Due to their size and progressive, space-occupying growth, these biologically benign tumours may cause injury to adjacent organs and thus clinical symptoms. For this reason, curative surgical resection is the treatment of choice for this tumour entity except for small, asymptomatic lesions, which do not require intervention. When radical resection is impossible, palliative surgery may improve the quality of life for several years. The risk of malignant transformation seems to be low even in the long-term course.     

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??Analysis of diagnosis and treatment of 20 cases of pancreatic serous cystadenoma ZHANG Lei, WANG Ke-wei,KONG Fan-min??et al. Department of Gastrointestinal Surgery,the First Affiliated Hospital,China Medical University,Shenyang 110001,China
Corresponding author:KONG Fan-min, E-mail:kong_fanmin@yahoo.com.cn
Abstract Objective To investigate the diagnosis and treatment of serous cystadenoma of the pancreas. Methods The clinical data of 20 patients with serous cystadenoma of the pancreas which were admitted into the First Hospital of China Medical University from April 2006 to April 2012 were retrospectively analyzed. Results There were 6 males (30%) and 14 females(70%).Tumors were present in the pancreatic body and tail in 15 cases,in the pancreatic head in 5 cases.The mean diameter of the tumor was 5.7cm. The clinical manifestations of upper abdominal discomfort 15 cases,abdominal pain and bloating 8 cases, including mild nausea 2 cases, jaundice 1 case. Ultrasound and CT lesion detection rate was 100%, the ultrasound localization rate 83.3%, CT 100%. All 20 patients received surgical resection. Duodenum-preserving pancreatic head resection in 1 case,pancreaticoduodenectomy was performed in 4 cases, central pancreatectomy in 2 case, distal pancreatectomy with splean in 7 cases, and spleen preserving distal pancreatectomy in 6 cases. Postoperative pancreatic fistula and intra-abdominal infections developed in 5 cases,Fistula was healed by conservative therapy in all these 5 cases. No perioperative deaths, postoperative followed up from 12 to 79 months,The median survival time 39.5 months.Found no recurrence. Conclusions Ultrasound and CT was main imaging examination for serous cystadenoma of the pancreas,and Accurate early detection and positioning lesions,Surgical resection should be adopted for serous cystadenoma of the pancreas with uncertain malignancy. Surgery should be benign surgical principles, try to retain the organs, reduce surgical trauma, serous cystadenoma prognosis is good.