自身免疫性胰腺炎14例临床分析

目的 探讨自身免疫性胰腺炎(AIP)的特征性表现,以提高对其的认识程度和诊治水平.方法 对2002年2月至2008年4月间收治的14例AIP患者的临床表现以及影像学、实验室、病理学资料和治疗效果进行回顾性分析,其中男性13例,女性1例,年龄35～71岁,平均53岁.临床表现为进行性皮肤巩膜黄染11例,持续性或间歇性上腹胀痛不适3例.结果 CT检查表现为胰腺弥漫性肿大伴胰管弥漫性不规则狭窄11例,胰头局灶性肿大伴胰头处胰管狭窄3例,全部病例均有胆总管胰腺段狭窄.共有12例患者接受免疫球蛋白G(IgG)和自身抗体检测,其中7例IgG浓度升高,6例自身抗体阳性.14例AIP患者中,9例就诊时存在或随访中出现胰腺外其他自身免疫性疾病.病理检查显示3例胰腺导管周围有致密的淋巴细胞和浆细胞浸润伴间质纤维化.因怀疑胰腺恶性肿瘤而行手术治疗7例,给予皮质类固醇激素治疗7例,治疗结果满意.随访中有4例复发.结论 MP应该作为胰腺癌的一种鉴别诊断,避免因误诊而进行不必要的手术治疗.     

Multifocal lesions with pancreatic atrophy in IgG4-related autoimmune pancreatitis: report of a case

We herein report a case of IgG4-related autoimmune pancreatitis (AIP). A 72-year-old male with jaundice visited our hospital complaining of epigastralgia. A blood chemistry analysis revealed elevated serum levels of total bilirubin and DUPAN-II. Computed tomography (CT) revealed irregularly shaped pancreatic masses with a stricture of the main pancreatic duct (MPD) in the head and tail that were interposed by marked atrophy with MPD dilation in the body. F-18 fluorodeoxyglucose (FDG)-positron emission tomography/CT revealed abnormally intense FDG uptake only at the masses. During surgery, another small tumor was also found in the atrophied body; therefore, a total pancreatectomy was performed under the diagnosis of multiple pancreatic cancers. The histological analysis revealed fibrosis with dense and diffuse infiltrations of lymphocytes and IgG4-positive plasma cells. The pancreatic parenchyma of the body was firmly replaced by fibrosis. AIP can lead to the formation of multiple pancreatic lesions, and thus the correct diagnosis is occasionally difficult to establish in atypical cases.     

Minute pancreatic carcinoma with initial symptom of acute pancreatitis

We experienced a case of minute pancreatic carcinoma in a 59-year-old man who complained of upper abdominal pain after drinking alcohol. Abdominal ultrasonography (US) revealed dilatation of the main pancreatic duct (MPD). Abdominal computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) showed slight dilatation of the MPD and its obstruction near the portal vein. Endoscopic retrograde cholangiopancreatography (ERCP) demonstrated occlusion of the MPD, and cytology of aspirated pancreatic juice was negative for malignancy. With the diagnosis of benign localized obstruction of the MPD, the patient underwent surgery. There was a clear demarcation of hardness and color of the pancreas on the left margin of the superior mesenteric vein, and the caudal pancreas was hard and fibrotic. Intraoperative US revealed slight dilatation of the MPD, and the aspiration cytology result was class IV. First, segmental resection of the pancreas was performed, but pathological examination of frozen section showed neither malignancy nor stenotic lesion. An additional small portion of the proximal pancreas was resected. The specimen included a ductal carcinoma, 5 mm in diameter. Accordingly, a pylorus-preserving pancreatoduodenectomy was performed. Microscopically, the minute carcinoma had already penetrated the duct wall and infiltrated lymph vessels and veins. The patient has been under close observation at our outpatient clinic, and so far there have been no signs of recurrence. To improve the poor prognosis of pancreatic cancer, we should be alert to the occurrence of acute pancreatitis as an initial symptom. Received: December 31, 2001 / Accepted: June 10, 2002 Offprint requests to: M. Imamura     

Multiple ectopic lesions of focal islet adenomatosis identified by positron emission tomography scan in an infant with congenital hyperinsulinism

Congenital hyperinsulinism (HI) exists in 2 histologic forms, focal and diffuse, and rarely has been attributed to lesions in ectopic pancreatic tissue. The ability to distinguish focal from diffuse HI and locate focal lesions has been difficult, thus limiting the optimal management of HI. We present a case of HI resulting from focal pancreatic and ectopic pancreatic lesions. After a near-total pancreatectomy failed to improve the patient's condition, a positron emission tomography (PET) scan performed with ¹⁸F-fluoro-l-dihydroxyphenylalanine demonstrated a focal lesion remaining in the head of the pancreas as well as 4 hot spots inferior to the remaining pancreas. Surgical exploration found pancreatic rests in the jejunum responsible for the hot spots seen on PET. Resection of the remainder of the pancreas as well as the small intestinal lesions resulted in correction of the patient's HI. Pathology confirmed the presence of focal HI lesions in the pancreatic head and small intestinal specimens. This case supports the ability of ectopic pancreatic tissue to contribute to the pathology of HI. It highlights the ability of PET to successfully identify focal lesions, including ectopic tissue, responsible for hyperinsulinemic hypoglycemia.     

Diagnosis of autoimmune pancreatitis with cholesterol granuloma mimicking intraductal papillary-mucinous carcinoma: A case report

IntroductionPancreatic cysts are often observed incidentally on abdominal computed tomography (CT). For cysts involving intracystic nodules, malignant neoplasms such as intraductal papillary-mucinous carcinoma (IPMC) should be suspected. In contrast, cholesterol granuloma (CG) rarely occurs in the pancreas, and CG-associated autoimmune pancreatitis (AIP) has not yet been reported. To our knowledge, this is the first reported case of AIP with CG mimicking IPMC.Presentation of caseA 56-year-old woman underwent abdominal CT for preoperative breast cancer screening. Asymptomatic polycystic lesions were detected in the pancreatic tail (maximum diameter, 5 cm). Magnetic resonance cholangiopancreatography and endoscopic ultrasonography revealed main pancreatic duct obstruction and a lesion with intracystic nodules (maximum diameter, 10 mm). Serum levels of pancreatic cancer tumor markers and IgG4 were within normal ranges. Because IPMC was suspected, distal pancreatectomy and splenectomy with regional lymphadenectomy were performed after surgery for breast cancer. Pathological examination of the specimen revealed no epithelial neoplasm; however, cholesterol crystals with foreign body giant cells were observed. Moreover, IgG4-positive plasma cells, diffuse lymphocyte infiltration, storiform fibrosis, and obliterative phlebitis were identified in the non-cystic pancreatic parenchyma. The final diagnosis was AIP with CG.DiscussionCG in the pancreas is rare and its pathogenesis remains unclear. The findings of the present case suggest that chronic inflammation due to AIP may cause local bleeding, and that a reaction to the leaked blood cells causes CG.ConclusionsAlthough preoperative diagnosis may be difficult, AIP with CG should be considered as a differential diagnosis in pancreatic cysts involving nodular lesions.     

自身免疫性胰腺炎的CT表现特征(附4例分析)

目的探讨自身免疫性胰腺炎(AIP)的CT表现特征。方法收集4例经临床、实验室检查和类固醇激素治疗有效的AIP患者的CT资料。CT检查包括平扫、双期(动脉期和门静脉期)增强扫描,在CT图像上观察胰腺的形态、大小、质地、强化程度、胰管、胰腺边缘、胰周及腹膜后各间隙等情况并分析AIP的CT表现特征。结果3例AIP表现为胰腺弥漫性肿大,1例为胰头的局限性肿大;病变区域胰腺实质在动脉期强化减弱但出现明显的延时强化;2例在胰腺病变区周围环绕包膜样结构;2例伴胆总管胰头段狭窄,1例ERCP显示胰管不规则狭窄;经类固醇激素治疗后,CT复查显示胰腺情况明显好转。结论AIP的CT表现具有一定的特征性。     

Endoscopic ultrasound guided fine needle aspiration biopsy of autoimmune pancreatitis: diagnostic criteria and pitfalls

Autoimmune pancreatitis (AIP) is a benign inflammatory disease of the pancreas that mimics pancreatic malignancy both clinically and radiologically. The fine needle aspiration biopsy (FNAB) features of AIP have not previously been documented. We report our experience with AIP, highlight pitfalls, and perform a comprehensive analysis of the cytomorphologic features of this condition. We identified 16 patients with AIP, initially evaluated by endoscopic ultrasound (EUS)-guided FNAB, 11 of whom subsequently underwent a pancreatoduodenectomy. We compared these to a cohort of EUS-guided aspirates from ductal carcinoma of the pancreas (n = 16) and chronic pancreatitis, not otherwise specified (NOS) (n = 19). On all 51 cases, we semiquantitatively evaluated presence and atypia of ductal cells, presence and cellularity of stromal fragments, and inflammatory cells, type and distribution. Fifty percent (8 of 16) of the AIP cases presented as obstructive jaundice. EUS and CT scan showed mass lesions in 10 and 6 cases, respectively. There were three false-positive cytologic diagnoses, an adenocarcinoma, a solid-pseudopapillary tumor and a mucinous neoplasm. Ductal epithelium was inconspicuous and was seen in 6 cases. The FNAB samples showed background lymphocytes in three AIP cases, a feature absent in the control cohort. Stromal fragments with embedded lymphocytes (greater than 30 per 60x) were seen in 37.5% of AIP cases and only rarely with adenocarcinoma (12.5%) and pancreatitis, NOS (0%). The cellularity of stromal fragments was significantly higher in AIP than in the control group. The presence of stromal fragments of high cellularity with a lymphoid infiltrate in conjunction with clinical and radiology findings could potentially both establish a diagnosis of AIP and exclude carcinoma, thus preventing pancreatic resection.     

The value of fluorodeoxyglucose positron emission tomography (FDG-PET) in differentiation of pancreatic lesions

Fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET) is a functional imaging modality that is based on the metabolic activity which is higher in most malignant tumors than benign tissues. This short review describes the basics of FDG-PET and gives a discussion of its role in differentiation of focal pancreatic lesions. The diagnostic accuracy in patients with active inflammation or cancer of the pancreas can be improved by dynamic acquisition of focal pancreatic lesions.     

Noninvasive diagnosis of focal hyperinsulinism of infancy with [18F]-DOPA positron emission tomography

Congenital hyperinsulinism of infancy (CHI) is characterized by severe hypoglycemia due to dysregulated insulin secretion, associated with either focal or diffuse pathology of the endocrine pancreas. The focal condition is caused by a paternally inherited mutation in one of the genes encoding the subunits of the beta-cell ATP-sensitive potassium channel (SUR1/ABCC8 or Kir6.2/KCNJ11) and somatic loss of maternal 11p15 alleles within the affected area. Until now, preoperative diagnostics have relied on technically demanding and invasive catheterization techniques. We evaluated the utility of fluorine-18 l-3,4-dihydroxyphenylalanine ([(18)F]-DOPA) positron emission tomography (PET) to identify focal pancreatic lesions in 14 CHI patients, 11 of which carried mutations in the ABCC8 gene (age 1-42 months). To reduce bias in PET image interpretation, quantitative means for evaluation of pancreatic [(18)F]-DOPA uptake were established. Five patients had a visually apparent focal accumulation of [(18)F]-DOPA and standardized uptake value (SUV) >50% higher (mean 1.8-fold) than the maximum SUV of the unaffected part of the pancreas. When these patients were operated on, a focus of 4-5 x 5-8 mm matching with the PET scan was found, and all were normoglycemic after resection of the focus. The remaining nine patients had diffuse accumulation of [(18)F]-DOPA in the pancreas (SUV ratio <1.5). Diffuse histology was verified in four of these, and pancreatic catheterization was consistent with diffuse pathology in four cases. In conclusion, [(18)F]-DOPA PET is a promising noninvasive method for the identification and localization of the focal form of CHI.     

The occult insulinoma operative localization by quick insulin radioimmunoassay

Hypoglycemia secondary to organic hyperinsulinism in children can be caused by diffuse or localized pancreatic lesions. Differentiation between these two types of lesions is of utmost importance since the surgical approach will be different. Some tumors escape detection by all preoperative investigations including ultrasound, scintiscan, arteriography, and computerized tomography. We are reporting on a 12-year-old boy with organic hyperinsulinism in whom we were unable to localize a tumor preoperatively. Peroperative determination of insulin levels from a number of sites on the pancreas enabled us to localize an insulin-producing pancreatic adenoma. This technique can be done easily by catheterizing the splenic and portal vein through a branch of the splenic vein and by serial sampling at 2 cm intervals along the portal and splenic veins. Insulin levels at these sites were determined by quick double-antibody radioimmunoassay, which allows the determination of the insulin levels within 50 minutes after sampling. There was a perfect biochemic and anatomic correlation allowing us to perform a precise distal pancreatectomy. The technique can be used to localize pancreatic adenomas and to decide how much pancreas to remove in diffuse lesions avoiding "blind" pancreatectomies.     

Histological classification of nesidioblastosis: efficacy of immunohistochemical study of neuron-specific enolase

Pancreatic specimens of six patients with nesidioblastosis were studied by light microscopy and immunohistochemistry in order to classify the changes of the endocrine pancreas and relate them to the clinical outcome. Neuron-specific enolase immunostaining was useful in demonstrating the distribution of whole pancreatic endocrine cells. The six cases with nesidioblastosis were classified into two types; four cases of focal type (head 1, body 1, tail 2), and two cases of diffuse type. The histological classification show a correlation to the clinical outcome. The patients with a focal body or tail type showed normal blood sugar levels immediately after operation. On the other hand the patients with both diffuse type and a focal head type needed medical treatment within a few months after operation. Therefore, these cases should be followed with great care postoperatively. A partial 80% to 90% pancreatectomy might be sufficient for each type except for the focal head type. When the intraoperative examination (both macroscopic and microscopic) is suggestive of focal head type, a pancreatectomy of 95% or more is called for.     

Value of 18-Fluorodeoxyglucose Positron Emission Tomography in the Management of Patients With Cystic Tumors of the Pancreas

OBJECTIVE: To assess the reliability of 18-fluorodeoxyglucose positron emission tomography (18-FDG PET) in distinguishing benign from malignant cystic lesions of the pancreas. SUMMARY BACKGROUND DATA: The preoperative differential diagnosis of cystic lesions of the pancreas remains difficult: the most important point is to identify malignant or premalignant cysts that require resection. 18-FDG PET is a new imaging procedure based on the increased glucose metabolism by tumor cells and has been proposed for the diagnosis and staging of pancreatic cancer. METHODS: During a 4-year period, 56 patients with a suspected cystic tumor of the pancreas underwent 18-FDG PET in addition to computed tomography scanning, serum CA 19-9 assay, and in some instances magnetic resonance imaging or endoscopic retrograde cholangiopancreatography. The 18-FDG PET was analyzed visually and semiquantitatively using the standard uptake value. The accuracy of 18-FDG PET and computed tomography was determined for preoperative diagnosis of a malignant cyst. RESULTS: Seventeen patients had malignant tumors. Sixteen patients (94%) showed 18-FDG uptake with a standard uptake value of 2.6 to 12.0. Twelve patients (70%) were correctly identified as having malignancy by computed tomography, CA 19-9 assay, or both. Thirty-nine patients had benign tumors: only one mucinous cystadenoma showed increased 18-FDG uptake (standard uptake value 2.6). Five patients with benign cysts showed computed tomography findings of malignancy. Sensitivity, specificity, and positive and negative predictive values for 18-FDG PET and computed tomography scanning in detecting malignant tumors were 94%, 97%, 94%, and 97% and 65%, 87%, 69%, and 85%, respectively. CONCLUSIONS: 18-FDG PET is more accurate than computed tomography in identifying malignant pancreatic cystic lesions and should be used, in combination with computed tomography and tumor markers assay, in the preoperative evaluation of patients with pancreatic cystic lesions. A positive result on 18-FDG PET strongly suggests malignancy and, therefore, a need for resection; a negative result shows a benign tumor that may be treated with limited resection or, in selected high-risk patients, with biopsy, follow-up, or both.     

Intraoperative ultrasonography by high-resolution annular array transducer for intraductal papillary mucinous tumors of the pancreas

BACKGROUND: Intraductal papillary mucinous tumors of the pancreas (IPMT) spread along the main pancreatic duct (MPD) or branch duct, or both. It is important to intraoperatively determine the extent of IPMT lesions in the MPD to perform a successful operation. METHODS: This study included 27 consecutive patients with IPMT who were referred for operation and who had undergone endoscopic ultrasonography, endoscopic retrograde pancreatography, and computed tomography as a diagnostic examination. Intraoperative ultrasonography with a 7.5-MHz annular array transducer and a 7. 5-MHz conventional transducer was performed. All patients underwent operation, and the pathology was confirmed. Endoscopic ultrasonography, endoscopic retrograde pancreatography, intraoperative conventional ultrasonography, and intraoperative annular array ultrasonography results were correlated with those from a pathologic examination of the resected specimens. The diagnostic accuracy of these 4 modalities in the detection of IPMT lesions in the MPD and the branch duct was confirmed. RESULTS: The diagnostic criterion of IPMT lesions by intraoperative annular array ultrasonography was the presence of echogenic masses with irregular margins in the MPD or branch duct, or both. An IPMT lesion in the MPD was found in 22 of the 27 patients. Intraoperative annular array ultrasonography detected 5 cases of IPMT lesions in the MPD that could not be detected by endoscopic ultrasonography or endoscopic retrograde pancreatography. In 3 of 5 patients, pancreatic resection was extended to remove IPMT lesions. Intraoperative annular array ultrasonography detected 3 multifocal lesions that could not be detected by preoperative examinations. For the diagnosis of IPMT lesions in the MPD, the respective sensitivity, specificity, and overall accuracy of intraoperative annular array ultrasonography were 86%, 100%, and 89%; the respective values were 50%, 100%, and 59% for endoscopic retrograde pancreatography; 59%, 100%, and 67% for endoscopic ultrasonography, and 32%, 100%, and 44% for intraoperative conventional ultrasonography. For the diagnosis of IPMT lesions in the branch duct, the respective sensitivity, specificity, and overall accuracy of intraoperative annular array ultrasonography were 95%, 100%, and 96%. CONCLUSIONS: Intraoperative annular array ultrasonography is suitable for longitudinal scanning of the MPD, useful for accurate diagnosis of the extent of IPMT in the pancreas, and valuable for planning surgical strategy and successful operations for IPMT.     

胰腺实性局灶性病变超声造影增强模式初探

目的 探讨各种胰腺实性局灶性病变(s-FLPs)超声造影增强模式.方法 采用SonoVue 结合低机械指数厌阶超声造影技术回顾性分析56例确诊的胰腺实性局灶性病变,观察不同胰腺实性局灶性病变在不同时相的增强特征.结果 胰腺癌85.7%(36/42)实质灌注早期和实质灌注晚期均保持低增强水平呈Ⅰ型增强模式,90.5%(38/42)增强晚于胰腺实质,血管期肿瘤内町见不规则分布的扭曲走行微血管缓慢由周边向中央灌注;肿瘤样胰腺炎75%(6/8)实质灌注早期和实质灌注晚期均保持等增强水平呈Ⅱ型增强模式,75%(6/8)与胰腺实质同时增强,血管期肿块内可见均匀分布的微血管快速全瘤灌注;胰腺内分泌肿瘤2/3呈实质灌注早期为高增强和实质灌注晚期为低增强呈Ⅳ型增强模式,2/3增强早于胰腺实质,血管期肿瘤内微血管快速伞瘤灌注;转移瘤增强模式与原发肿瘤类型有关,血管期肿瘤内微血管快速全瘤灌注.结论 不同胰腺实性局灶性病变的超声造影有其典型的增强模式,从而克服了BUS对s-FLPs缺乏组织特性的不足,有望成为s-FLPs鉴别诊断的一种重要影像学方法.     

Intraductal papillary mucinous tumors of the pancreas confined to secondary ducts show less aggressive pathologic features as compared with those involving the main pancreatic duct

Intraductal papillary mucinous tumors (IPMTs) of the pancreas are rare tumors characterized by a malignant potential. Because of the progress of imaging procedures, smaller cystic pancreatic lesions are now detected and some of them correspond to IPMTs that involve ectatic pancreatic branch ducts but spare the main pancreatic duct. To investigate differences in morphology and clinical behavior of branch and main duct types of IPMT, a surgical series of 43 cases was studied. All pathologic specimens of IPMT, surgically resected in our institution between October 1987 and July 1998, were analyzed. In all cases, the entire pancreatic specimen was systematically examined. IPMT of the branch type was found in 13 (30%) patients, whereas IPMT of main pancreatic duct type that involved the main pancreatic duct and branch ducts was observed in 30 (70%) patients. Patients with IPMT of the branch type were younger (median age, 55 yrs vs 64 yrs), and all but one of the lesions were located in the head and neck of the pancreas (vs 17 of 30 patients with the main duct type). The size of the cysts ranged from 4 to 55 mm, and the major duct showed a mild dilation in most cases. In contrast to the main pancreatic duct type, which showed invasive carcinoma and in situ carcinoma in 11 (37%) of 30 patients and 6 (20%) of 30 patients, respectively, IPMT of the branch type showed significantly less aggressive histologic lesions with five (39%) patients with simple hyperplasia, six (46%) patients with atypical hyperplasia, and two (15%) patients with in situ carcinoma. No invasive carcinoma was observed in this group. IPMT of the branch type occurs in younger patients and is associated with less aggressive histologic features than is the main pancreatic duct type. Our findings raise the difficult issue of clinical management of IPMT of the branch type as a distinctive group.     

Abdominal Visceral Lesions in von Hippel-Lindau Disease: Incidence and Clinical Behavior of Pancreatic and Adrenal Lesions at a Single Center

Introduction Von Hippel-Lindau disease (VHL) is a dominantly inherited multi-system syndrome. Although pheochromocytoma is the hallmark endocrine neoplasm, pancreatic lesions occur frequently, and their management can be complex. This report describes 26 patients from a single institution with pancreatic or adrenal lesions (or both) in the background of VHL. Methods We reviewed records for all patients treated for adrenal (type 2 VHL) or pancreatic manifestations of VHL at our institution from 1990 to 2004. Results Forty patients with VHL were identified from 24 pedigrees; 26 (65%) had adrenal or pancreatic involvement (or both). Seven patients had isolated pheochromocytomas, 16 had isolated pancreatic lesions, and 3 had both. Of the 10 patients with pheochromocytomas, 4 had bilateral adrenal involvement. All but three pheochromocytomas were diagnosed in patients aged 40 years or younger. Of the 19 patients with pancreatic lesions, 8 had cystic lesions, 8 had neuroendocrine tumors, and 3 had both. Of 11 patients with neuroendocrine tumors, 4 had metastases (3 hepatic, 1 nodal). No patient with cystic pancreatic lesions developed carcinoma. Conclusions VHL should be familiar to the endocrine surgeon because of the high incidence (65% in our series) of visceral endocrine lesions (pancreas 40%, adrenal gland 18%, both 7%). As seen in other hereditary endocrinopathy syndromes, pheochromocytomas are frequently bilateral; therefore,close follow-up of the contralateral gland in young patients with a unilateral lesion is critical. Cystic lesions of the pancreas may be closely monitored, whereas neuroendocrine tumors should be removed because of the risk of metastasis. Pancreatic pathology is not uncommon in VHL patients with pheochromocytoma and should be sought during the preoperative assessment.     

Mucinous carcinoma of Vater's ampulla with a unique extension along the main pancreatic duct

We report a case of mucinous carcinoma of Vater's ampulla with a unique extension along only the main pancreatic duct (MPD) and microinvasion to the pancreas. A 52-year-old man was referred to our hospital for the evaluation and treatment of acute pancreatitis. Abdominal computed tomography (CT) demonstrated swelling in the head of the pancreas with a mass in the duodenum. Hypotonic duodenography and endoscopic examination revealed a well-defined mass, measuring about 25 mm in size, in Vater's ampulla. A biopsy specimen of the tumor showed moderately differentiated adenocarcinoma. A pylorus-preserving pancreaticoduodenectomy with a regional lymphadenectomy was performed, under a preoperative diagnosis of adenocarcinoma of Vater's ampulla with direct invasion into the head of the pancreas. The resected specimen of the duodenum confirmed the presence of the mass, which measured 22 × 15 mm in size, in Vater's ampulla. Microscopically, the tumor consisted of two components: moderately differentiated adenocarcinoma in the peripheral region of the tumor Vater's papilla and mucinous carcinoma in the central region of the tumor. The mucinous carcinoma component uniquely extended along only the MPD with microinvasion to the pancreas. Immunohistochemically, both the moderately differentiated adenocarcinoma and the mucinous carcinoma were positive for cytokeratin 20 (CK20) and negative for cytokeratin 7 (CK7) which is the pattern of intestinal-type carcinoma of Vater's ampulla. We concluded that the original site of this tumor may have been the duodenal epithelium of Vater's ampulla originally moderately differentiated adenocarcinoma—which subsequently changed to mucinous carcinoma that extended along only the MPD with microinvasion to the pancreas.     

Cervical metastases of glucagonoma in a patient with multiple endocrine neoplasia type 1: Report of a case

Multiple endocrine neoplasia type 1 (MEN 1) is a syndrome characterized by tumors of the parathyroid glands, pancreatic islet cells, duodenum, and pituitary gland. We report a case of cervical metastases of glucagonoma with MEN 1. The patient was a 34-year-old woman admitted to our hospital with epigastric pain. Her medical history included two resections of prolactinoma and two upper GI hemorrhages secondary to duodenal ulcers. Computed tomography (CT) showed two hypervascular lesions in the tail of the pancreas and cervical ultrasound showed multiple hypoechogenic ovoid images in the neck. A cervical CT scan confirmed two 15-mm lymph nodes in the left cervical region and 111In-DOTATOC imaging showed focal abnormal somatostatin expression in the pancreatic tail and the cervical nodes. The patient had asymptomatic hypoglycemic episodes, with blood sugar levels as low as 30 mg/dl, which raised our suspicion of MEN 1 associated with pancreatic insulinoma. Thus, we performed a distal pancreatectomy with bilateral cervical dissection and parathyroid gland resection. Histopathological examination revealed 12 pancreatic tumors as well as metastases in four cervical lymph nodes. The resected parathyroid glands had normal structure, suggesting parathyroid hyperplasia. A follow-up CT scan, 18 months after surgery, showed new tumors in the head of the pancreas and in the duodenal wall. A pancreatoduodenectomy was performed and histopathological examination revealed nine nonfunctioning endocrine tumors in the pancreas, one tumor in the duodenal wall, and metastases in two peripancreatic lymph nodes. The patient recovered well and remains asymptomatic.     

Autoimmune pancreatitis diagnosed after pancreatoduodenectomy and successfully treated with low-dose steroid

A 69-year-old woman presented with obstructive jaundice and a 30-mm hypoechoic mass in the pancreatic head on ultrasonography. Magnetic resonance imaging (MRI) revealed enlargement of the pancreatic head with dilatation of the upstream main pancreatic duct and no dilatation of the proximal biliary tree. Endoscopic retrograde pancreatography showed a localized irregular narrowing of the main pancreatic duct in the head of the pancreas. Pylorus-preserving pancreatoduodenectomy (PPPD) was performed under the diagnosis of pancreatic head cancer. Histopathological examination showed fibrosis with lymphoplasmacytic infiltration, suggesting the diagnosis of autoimmune pancreatitis (AIP). Serum IgG concentration was within normal limits immediately after the operation but was elevated 4 months later, when MRI showed enlargement of the remnant pancreas, with a peripheral rim of low intensity. Oral administration of prednisolone was initiated, at a dose of 5 mg/day. The serum IgG concentration declined and MRI showed improvement of the pancreatic enlargement and the disappearance of the peripheral rim. AIP has not relapsed for 1 year so far, with the patient being kept on 5 mg/day prednisolone. This communication reports a patient with AIP showing an interesting clinical course.     

Malignant islet cell tumor projecting into the main pancreatic duct

We report herein a rare case of islet cell tumor showing a unique growth pattern in a patient who developed repeated acute pancreatitis as the tumor's initial symptom. Preoperative imaging examinations showed dilatation of the main pancreatic duct (MPD) and cysts around the pancreatic tail. A distal pancreatectomy with splenectomy was performed because the pancreatitis was localized in the distal pancreas and was not controlled by various drug therapies. Grossly, the tumor consisted of two component parts: a markedly infiltrative part in the pancreatic parenchyma, and a papillary elevated part in the MPD. The MPD was obstructed by the tumor spreading widely along the distal MPD. Microscopically, the tumor was composed entirely of islet cell tumors (nonfunctioning), with several foci of venous and lymphatic involvement. Based on its growth behavior, we assumed that the tumor may have arisen from the MPD or from islet cells closely adjacent to the MPD. The patient's postoperative course was uneventful and he is doing well 2 years after the operation. We discuss the growth pattern of the tumor and the cause of the pancreatitis. Received: March 30, 2000 / Accepted: July 12, 2000