A case of completely isolated advanced enteric duplication cyst cancer performed partial pancreatectomy

IntroductionEnteric duplication cysts are rare and, in addition, isolated enteric duplication cysts are lower morbidity prevalence rate. These cysts lack a connection to the gastrointestinal tract or the adjacent mesenteric vasculature and have only been reported in 10 case reports. In these reports, only two reports were cases with malignant transformation. Our case was a report for the advanced cancer of the isolated enteric duplication cyst.Case presentationThe patient was a 43 year-old woman with slightly abdominal pain and mass formation. The abdominal contrast-enhanced computed tomography showed 130 × 100 × 90 mm huge cystic mass existed in right upper peritoneal cavity. The cystic mass had thickened wall and many enhanced nodules. As these imaging findings suggested a tumor originated from pancreas and the preoperative diagnose was suspect of mucinous cystic neoplasm. In operative findings, the tumor originated from pancreatic head and did not attach to gastrointestinal tract. Final pathology indicated the cyst was an isolated advanced enteric duplication cyst cancer and not originated from pancreas.ConclusionWe experienced an extremely rare case of completely isolated advanced enteric duplication cyst cancer. Unique to this case, the preoperative diagnosis was suspect of mucinous cystic neoplasm arising from pancreas head and partial pancreatectomy was performed. However, in the pathological findings, this cyst diagnosed advanced enteric duplication cyst cancer.     

Primary Hydatid Cyst of the Pancreas

Background: Hydatid cyst of the pancreas is rare, accounting for less than 1% of the various sites of hydatid disease, even in countries where echinococcosis is endemic. We report a case of pancreatic hydatid cyst to increase awareness for this important condition.

Case report: We describe a 33-year-old male with abdominal discomfort of one-year duration. On abdominal examination, there was mild tenderness with fullness in the right upper quadrant. Laboratory investigations revealed no abnormalities. Abdominal ultrasonography showed a cystic mass over the head of the pancreas. Abdominal CT scan confirmed the presence of a solitary cyst in the pancreatic head, with no enhancement on contrast CT. Indirect hemagglutination test for hydatid disease was negative. A pre-operative diagnosis of cystic neoplasm of the pancreas was made and laparo-tomy was done; on exploration there was a cystic mass arising from the head of the pancreas. A pericystectomy was carried out and the diagnosis of hydatid cyst of the pancreas was confirmed by histopathology. The postoperative period was uneventful and no recurrence was seen after 2 years of follow-up.

Conclusion: Pancreatic hydatidosis, though very rare, should be considered in the differential diagnosis of pancreatic cystic lesions in the appropriate epidaemiological setting.     

Enlarging lymphoepithelial cyst of the pancreas during 12 months of observation: report of a case

Pancreatic lymphoepithelial cysts (LECs) are rare benign pancreatic cystic lesions, the etiology of which is unknown. We report a case of a pancreatic LEC, discovered incidentally in a 63-year-old man during a follow-up clinic visit for an abdominal aneurysm. Computed tomography showed a multilocular cyst, 60-mm diameter in the body of the pancreas. This cyst increased from 6.0 to 6.5 cm during 12 months of observation. Part of the cyst was also visualized on positron emission tomography imaging. Since a pancreatic cystic neoplasm could not be ruled out, we performed distal pancreatectomy and postoperative pathological examination confirmed that the lesion was an LEC of the pancreas. Despite the conclusive postoperative findings, resection is unavoidable when a true pancreatic neoplasm cannot be excluded.     

Epithelial inclusion cyst arising within an intra-pancreatic splenunculus

INTRODUCTIONAn accessory spleen (splenunculus) may occur in up to 10% of the general population. However, an epithelial inclusion cyst originating within an intra-pancreatic splenunculus is an extremely rare finding, with only twenty-two previous cases described in medical literature.PRESENTATION OF CASEA 51-year-old male presented to our institution for investigation of altered bowel habit. Endoscopic ultrasound examination and CT scanning demonstrated an 18 mm cystic, well-demarcated lesion in the tail of the pancreas, resembling malignancy. Following laparoscopic spleen-preserving distal pancreatectomy, histological analysis confirmed epithelial inclusion cyst arising within an intra-pancreatic splenunculus.DISCUSSIONThe pre-operative radiological identification of such cystic pancreatic lesions is challenging. Surgical resection is usually performed for clinical suspicion of pancreatic malignancy.CONCLUSIONEpithelial inclusion cyst originating within an intra-pancreatic accessory spleen is an extremely rare finding, which can mimic malignancy. Nevertheless, it should be carefully considered in the differential diagnosis of cystic lesions of the tail of the pancreas.     

Pancréatectomie médiane avec anastomose pancréatogastrique pour néoformation pancréatique

Aim of the studyTo report a personal series of ten patients who underwent a medial pancreatectomy with pancreaticogastrostomy for tumor of the pancreas.Material and methodsAn equal number of five patients had solid and cystic tumors. The solid tumors were all endocrine and included two cases of insulinoma and solitary cases of VIPoma, glucagonoma, and non-secreting tumor. Five patients had a cystic tumor which was in two cases a serous cystadenoma, and in the other cases, a mucinous cystadenoma, a solid pseudopapillary tumor, and a solitary hydatid cyst of the pancreas. The operative procedure included a middle segment pancreatectomy, a suture of the proximal pancreatic stump, an anastomosis of the distal pancreatic stump with the stomach and an external transgastric drainage of the distal main pancreatic duct.ResultsIn the postoperative course, a sero-hematic collection close to the anastomosis occurred in four patients and required external drainage in three cases. All patients recovered without sequela of pancreatic dysfunction.ConclusionThis series confirms the place of medial pancreatectomy in resection of non-enucleable tumors of the middle part of the pancreas. It also shows that pancreaticogastrostomy is a technical simplification compared to Roux-en Y pancreaticojejunostomy in this particular modality of pancreatectomy.     

Pancreatic schwannoma: A rare case and a brief literature review

IntroductionPancreatic schwannoma (PS) is an extremly rare benign tumor. Less than 50 cases of pancreatic schwannoma have been described in the English literature over the past thirty years.Presentation of case reportA 63-year-old female underwent left modified radical mastectomy 2 years ago due to breast cancer. During her routine check-up, a 65 × 63 × 55 mm measured calcified, well-demarcated, cystic-mass having septations and calcifications that localized to the pancreatic head was detected by abdominal computerized tomography. She was asymptomatic and her tumor markers were in normal ranges. A standard Whipple procedure was performed, and the histo-pathological diagnosis of the resected specimen was reported as ancient schwannoma with clear surgical margins. Patient’s postoperative course was eventful. She had a biliary leakage after surgery which was managed conservatively. She is under follow-up.DiscussionPancreatic schwannoma also known as neurilemoma or neuroma is a slowly growing, encapsulated, mostly benign tumor with smooth well-delineated margins that originates from myelin producing schwann cells located on the nerve sheath of the peripheral epineurium of either the sympathetic or parasympathetic autonomic fibers. PS’s are extremly rare. The head of pancreas being involved in the vast majority of cases (40%), followed by its body (20%). Management of pancreatic schwannomas remains largely controversial. Both enucleation and radical surgical resections have revealed great therapeutic efficiency. with a well prognosis without recurrences.ConclusionAlthough rare, PS’s should be considered in the differential diagnosis of the other solid or cystic masses of the pancreas.     

��������������ǰ�������ж�

??Preoperative diagnosis of the malignancy for pancreatic cystic neoplasms FU De-liang. Department of Pancreatic Surgery of Huashan Hospital; Pancreatic Disease Institute; Shanghai Medical College, Fudan University, Shanghai 200040, China
Abstract Pancreatic cystic neoplasms (PCN) are being increasingly detected due to widespread use of abdominal imaging and improved imaging techniques. The most common types of PCN are serous cystic neoplasms (SCN), mucinous cystic neoplasms (MCN), intraductal papillary mucinous neoplasms (IPMN)and solid pseudopapillary neoplasm(SPN). Diagnosis of malignancy in PCNs cases requires radical pancreatectomy. Clinical?? radiological and laboratory characteristics of PCNs may predict underlying malignancy, including male sex, abdominal mass, weight loss, larger tumor size, local invasion, and elevated CA19-9. Recent developments of clinical, radiological, and laboratory modalities used for preoperative diagnosis and differential diagnosis of PCNs are provided this review. Analysis of DNA mutations, microRNA and proteins within pancreatic cyst fluid by EUS-FNA have identified potential biomarkers to aid with the management of patients with pancreatic cystic neoplasms.     

MSCT诊断胰腺无功能性囊性神经内分泌肿瘤

目的探讨胰腺无功能性囊性神经内分泌肿瘤(NF-CNETP)的MSCT表现。方法回顾性分析10例经手术病理证实的NF-CNETP的MSCT表现,对照病理进行分析。结果 10例NF-CNETP中,位于胰头5例,胰尾4例,同时位于胰体尾部1例;肿瘤最大径2.5~6.2cm。10例肿瘤均边界清楚,其中9例见完整包膜。平扫肿瘤密度不均匀,实性囊壁呈等或稍低密度,10例中2例可见钙化。增强扫描动脉期9例呈明显环状不均匀强化,1例含壁结节者明显强化,门静脉期均呈持续性强化。肿瘤囊壁动脉期平均CT值为(128.00±62.62)HU,门静脉期为(132.40±44.66)HU。4例胰管轻度扩张。1例胰周淋巴结转移。6例接受能谱CT双能扫描,肿瘤囊壁动脉期及门静脉期的碘浓度值与腹主动脉的碘浓度值进行标准化后分别为0.40±0.16、0.79±0.22。结论 NF-CNETP的MSCT强化方式及包膜显示具有一定特征性,对诊断与鉴别诊断有一定意义。     

Mucinous cystadenocarcinoma of pancreas mimicking gastrointestinal stromal tumor of stomach: Case report

IntroductionPancreatic mucinous cysadenocarcinoma is a rare neoplasm of pancreas which rarely presents as upper gastrointestinal bleed.Case reportA 45-year-old woman presented with history of repeated episodes of melena and abdominal pain. Upper gastrointestinal endoscopy revealed a growth in the body of the stomach likely to be a gastrointestinal stromal tumor (GIST). Endoscopic biopsy was inconclusive for GIST or malignancy. Computed tomography scan of the abdomen showed a well-defined multiloculated cystic lesion (11.8 × 11.1 × 6.4 cm) in the body and tail of the pancreas with involvement of the stomach. Preoperative diagnosis was a cystic neoplasm of the pancreas with tumor ingrowth into the stomach. The patient underwent enbloc resection of the stomach with distal pancreatectomy and splenectomy. Histopathological examination revealed low grade mucinous cystadenocarcinoma of the pancreas.DiscussionUpper gastrointestinal bleed as a manifestation of cystic neoplasms of pancreas are rarely reported. Although some cases of hemoperitoneum were reported due to rupture of tumor; luminal gastrointestinal bleed is even rarer. It may be due to involvement of porto-mesenteric axis with portal hypertension, direct invasion of the gastric wall with ulceration of the gastric mucosa, communication between the tumor and the main pancreatic duct, and from the infiltrating tumor.ConclusionOur case highlights the fact that mucinous cystadenocarcinoma of pancreas rarely can present with upper gastrointestinal hemorrhage, Moreover, if it is large enough to compress or invade the stomach, endoscopy may misdiagnose it as GIST.     

胰腺囊性肿瘤术前良恶性判断

随着影像诊断技术的广泛应用和改进,胰腺囊性肿瘤（PCN）的诊断率不断增加。常见的胰腺囊性肿瘤包括浆液性囊腺瘤（SCN）、黏液性囊腺瘤（MCN）和导管内乳头状黏液性肿瘤（IPMN）以及囊实性假乳头状肿瘤（SPN）,一旦怀疑为恶性时,均应行根治性手术切除。PCN的术前诊断和良恶性判断十分重要,目前主要依靠临床表现、影像学检查和实验室检查。当临床发现老年男性病人、血清肿瘤标记物CA19-9升高、伴有巨大胰腺肿块、体重下降、有特征性影像学表现者,应考虑恶性肿瘤的可能。主要依靠术后病理、经超声内镜下囊液抽取分析和穿刺组织活检确诊,包括肿瘤相关DNA、MicroRNA和蛋白质标记物的分析。     

A case report of simple mucinous cyst of pancreas detected by rupture

IntroductionSimple mucinous cyst (SMC) of pancreas is a disease defined by the Baltimore Consensus in 2014. Pancreatic mucus-producing neoplasms are considered to be premalignant tumors, but SMC is not considered to have a risk of malignancy or recurrence.Presentation of caseThe case was a woman in her 50s with a chief complaint of abdominal pain. A blood exam showed an increase in the inflammatory response, and a slight increase of Amylase. CT showed a cystic lesion 80 mm in size at tail of the pancreas, and disproportionate fat stranding and ascites around it. We diagnosed peritonitis associated with the rupture of a cystic lesion accompanied by pancreatitis. Abdominal pain was improving, and we decided to proceed with the detailed examination. MRI showed a uniform hyper-intensity on T2WI, and a nodular-like hypo-intensity was observed inside, which was enhanced. During the follow-up, the lesion had gradually grown and re-ruptured. As we could not deny malignancy by image findings, distal pancreatectomy was performed. The intracystic fluid was browny and turbid, and Amylase, CEA and CA19-9 of the cystic fluid were elevated. We diagnosed it SMC by histopathological findings. Currently, she had no recurrence for 1 year.DiscussionSMC is a type of true cysts, so rupture was rare. However, if the cyst wall becomes weak due to complications such as acute pancreatitis. It is probable that our case had pancreatitis and the cyst wall was weakened.ConclusionSMC detected by rupture was very rare, so we report this case.     

Solid cystic pseudopapillary tumor of pancreas with splenic metastasis: Case report and review of literature

IntroductionSolid-cystic pseudopapillary tumor of the pancreas is rare and most commonly seen in young women. We present a young women with solid-cystic pseudopapillary tumor of the pancreas and discuss the literature.Presentation of caseThirty nine years old female patient with a mass about 12 cm in the pancreas with splenic invasion seen in our clinic. After having CT and PET-CT view, patient underwent surgery. Distal pancreatectomy with mass excision and splenectomy was performed. Microscopic examination result was solid cystic pseudopapillary tumor with spleen invasion.DiscussionSolid-cystic pseudopapillary tumor of the pancreas has cystic solid pseudopapillary structures. Prognosis of tumor is better than other pancreatic tumor. Complete resection of tumor with splenic inclusion is surgical treatment.ConclusionIn case of large slow growing pancreatic tumor with splenic metastasis, solid-cystic pseudopapillary tumor of the pancreas should be considered in the diagnosis. Complete surgical resection is associated with long-term survival even in the presence of metastatic disease. Close follow-up is necessary after surgery.     

�������������ķ����벡��ѧ����

??Classification and pathology features of pancreatic cystic neoplasmas LU Zhao-hui,CHEN Jie. Department of Pathology, Peking Union Medical College Hospital , Peking Union Medical College, Chinese Academy of Medical Science,Beijing100730,China
Corresponding author: CHEN Jie,E-mail:xhblk@163.com
Abstract Serous cystadenoma consists of oligocyst, multiple microcysts or solid acinars lined by a glycogen-rich cuboidal epithelium. Based on the low risk of malignancy of SCA, surgical resection should only be considered when the cysts are causing symptoms or when differentiation from a mucinous cystic neoplasm with concerning features is not possible. Mucinous cystic neoplasm and Intraductal papillary mucinous neoplasm can all be classified into 4 catograies: low-grade dysplasia, moderate dysplasia, high-grade dysplasia and with invasive carcinoma, while the major difference between them is whether the cysts are communicated to the duct of pancreas and most MCNs develop ovarian-like stroma. Solid-pseudopapillary tumor of pancreas is a neoplasm with distinctive morphology and immunophenotype, most of them are benign although labeled with low malignancies. Most acinar cell cystadenomas are benign, just like the serous cystadenoma, only very rare cases reported to be malignant. Other miscellaneous cystic tumors consist mature teratoma, lymphoepithelial cyst, cystic lymphangioma and Multicystic adenomatoid pancreatic hamartoma, all follow a benign course. Attentions should be paid to some primary solid pancreatic tumors with cystic transformation, which might be confused with some primary cystic neoplasms.     

Epidermoid cyst originating from an intrapancreatic accessory spleen

We report a rare case of an epidermoid cyst originating from an intrapancreatic accessory spleen, in a 40-year-old Japanese man with no clinical symptoms. A cystic tumor in the pancreatic tail was detected incidentally by abdominal ultrasonography. The patient was referred to the KKR Tachikawa Hospital for further examination of the tumor. Preoperative imaging findings suggested that the tumor was an epidermoid cyst originating from an intrapancreatic accessory spleen. On both pre-and post-contrast computed tomography and magnetic resonance images, the solid compartment of the tumor had the same X-ray attenuation and intensity as the spleen. Upon surgical excision, the mass consisted of solid and cystic components that were macroscopically evident on the preoperative images. Microscopic analysis revealed that the solid component was an accessory spleen in the pancreatic tail, whereas the cystic component was lined with stratified epithelium representative of an epidermoid cyst. This is the thirteenth report (in English) of an epidermoid cyst originating from an intrapancreatic accessory spleen, and the first case to be diagnosed prior to surgery.     

胰腺囊性肿瘤的诊断与治疗

目的 探讨胰腺囊性肿瘤的诊断和治疗。方法 对本院近3年内收治的7例病人的临床特点进行回顾性分析。结果 胰腺囊性肿瘤好发于中青年女性，无急性胰腺炎．无上腹部手术及外伤史。临床表现以上腹部肿块和疼痛不适较为多见。影像学校查胰腺肿块为囊性，囊实性或不规则分叶状。6例手术治疗，l例拒绝手术。结论 因术前术中定性困难，除假性囊肿或典型的浆液性囊腺瘤外；都应按恶性肿瘤处理。     

Pancreatic endometrial cyst: report of a case

A pancreatic endometrial cyst is an extremely rare disease. Since 1984, only 7 cases have been reported, including the current case. A 35-year-old woman with a history of recurrent severe left upper abdominal pain of 3 months’ duration was found to have a cyst in the pancreatic body on the diagnostic imaging findings. With a preoperative diagnosis of mucous cystic adenoma, she underwent a distal pancreatectomy. The histopathological examination of the specimen revealed a pancreatic endometrial cyst. She complained about severe periodic abdominal pain during the convalescence, without any surgical complications. This study reviews previous reports of pancreatic endometrial cysts, and also discusses the clinicopathological features, pathogenesis, and appropriate treatment for this disease.     

A case of pancreatic carcinoma coexist with pancreatic pseudocyst

Report of Pancreatic carcinoma coexist with Pancreatic pseudocyst is rare. We have experienced a case of pancreatic carcinoma which was diagnosed and resected by coexisted pancreatic pseudocyst. The patient aged 53 years, complained of epigastric distension and visited our hospital. There was no past history of abdominal injuries and he didn't drink much. A cyst 7cm in diameter was found at pancreatic tail lesion by our examination. By blood chemistry tumor marker CA19.9 was 310 and he complicated diabetes mellitus. We suspected pancreatic carcinoma coexist with pancreatic pseudocyst, so distal pancreatectomy and splenectomy was performed. We found a solid tumor sized about 3cm in diameter at just proximal of pancreatic cyst. Since perioperative histological examination revealed adenocarcinoma, we performed lymph node dissection. The tumor was highly differentiated adenocarcinoma which invaded retroperitoneum in some lesion. The patient died 10 months later by recurrence. We concluded that we must take into account not only laboratory finding but their clinical course in such cases.     

Mucinous cystic neoplasm of the pancreas during pregnancy: the importance of proper management

We describe a case of huge mucinous cystic tumor of the pancreas in a 26-year-old woman during pregnancy. Ultrasonography demonstrated a well-delimited cystic mass in the left upper abdominal quadrant, suggestive of benignity. Magnetic resonance imaging showed a large cystic mass resembling a mucinous cystic tumor of the pancreas. After this assessment the patient underwent surgical exploration and a huge cystic tumor of the pancreas was discovered. The tumor was enucleated and distal pancreatectomy was performed. The resected margin of the specimen was free of tumor. In this case report we discuss the management of mucinous pancreatic tumors during pregnancy and we briefly review the previously reported cases of mucinous pancreatic tumors in pregnant patients. We conclude that surgical resection of these tumors should be strongly considered in pregnancy. Removal of the tumor appears to be a safe procedure without harmful effects to the fetus.     

Primary extragastrointestinal stromal tumor arising in the pancreas: report of a case

Primary extragastrointestinal stromal tumors (EGISTs) arising in the pancreas are extremely rare, with only ten cases documented to our knowledge. We report a further case of EGIST of the pancreas. The patient was a 55-year-old man who presented with postprandial abdominal discomfort. Abdominal computed tomography and magnetic resonance imaging showed a lobulated heterogenous enhancing mass, 11 cm in diameter, in the abdominal cavity. No regional lymphadenopathy, ascites, or metastasis was seen radiologically. There was no obvious lesion in the stomach or small intestine. The initial diagnosis was a solid pseudopapillary tumor or serous cystic neoplasm. The patient underwent distal pancreatectomy with splenectomy. Microscopically, the tumor consisted of spindle cells arranged in short fascicles. Mitotic figures were seen in 7/50 high-power fields. Immunohistochemical examination revealed strongly positive staining for CD117. Based on these findings, the final pathologic diagnosis was a primary EGIST of the pancreas. This case consolidates the possibility that this rare tumor can involve the pancreas as a primary site and should be included in the differential diagnosis of cystic lesions in this site.     

An isolated intestinal duplication cyst masquerading as a mucinous cystic neoplasm of the pancreas: A case report and review of the literature

IntroductionEnteric duplication cysts presenting in adulthood are rare. Isolated enteric duplication cysts, which lack a connection to the GI tract or the adjacent mesenteric vasculature, have only been cited in six previous case reports.Case presentationA 48-year-old female presented with a four-year history of intermittent nausea, vomiting and abdominal pain. Computed tomography (CT) scan of the abdomen revealed a 7 cm multi-lobular, calcified, cystic lesion intimately involved with the pancreas. Endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) was non-diagnostic; however, the cyst fluid Carcinoembryonic Antigen (CEA) level was significantly elevated leading to a presumed diagnosis of a mucinous cystic neoplasm (MCN) of the pancreas. Intraoperatively, the cystic mass was identified and notably did not have any true attachments to the neighboring pancreas, gastrointestinal tract or vasculature. Final pathology demonstrated an isolated small bowel duplication cyst.DiscussionIn this case a patient presented with a clinical picture consistent with an MCN of the pancreas. However, intraoperatively and on final pathology the mass was found to be an isolated enteric duplication cyst. This represents only the seventh such case report in an adult.ConclusionAlthough rare, isolated enteric duplication cysts can be considered in a patient presenting with chronic abdominal pain and an abdominal mass on imaging. In this case we demonstrate that an isolated enteric duplication cyst can clinically mimic an MCN of the pancreas.