Role of cystic fluid in diagnosis of the pancreatic cystadenoma and cystadenocarcinoma

BACKGROUND/AIMS: Early and accurate diagnosis of cystic neoplasm of the pancreas is difficult especially for the differentiation of benign or malignancy. In this study, we try to compare EUS-guided fine needle aspiration biopsy combined with measurement of the cyst fluid and serum levels of CEA, and CA19-9 for the preoperative diagnosis of pancreatic cystadenoma or cystadenocarcinoma. METHODOLOGY: Retrospective analysis was made on the clinical data of 37 patients with pancreatic cystadenoma and 48 patients with cystadenocarcinoma from 1998 to 2005. RESULTS: Carcinoembryonic antigen (CEA), and CA19-9 of the cyst fluid and serum combined with EUS-guided fine needle aspiration biopsy was made. Examination of serum CEA, and CA19-9 resulted in 21.0+/-18.0, 2.7+/-1.7 U/L and 18.7+/-17.5, 269.0+/-182.0 U/L for cystadenoma and cystadenocarcinoma respectively (P<0.05). The sensitivity of cyst fluid combined with biopsy was higher than that of a single marker. However, the sensitivity and specificity of tumor markers of cystic fluid were much higher than that of the serum (P<0.05). CONCLUSIONS: EUS-guided fine needle aspiration biopsy combined with examination of cyst fluid level of CEA and CA19-9 will be a credible means for early diagnosis of pancreatic cystadenoma and cystadenocarcinoma.     

Improved early diagnosis of cystadenocarcinoma of the pancreas

IntroductionT here are great differences between the therapy for cystadenocarcinomas and benign pancreatic lesions. Early diagnosis is propitious to selecting the appropriate therapy, and may directly influence the prognosis.[1-4] Differential diagnosis between cystadenocarcinoma and benign pancreatic lesion is difficult, because they lack typical clinical symptoms in early stage. In the commonly used examinations, the specificity of B-ultrasonography (B-US) and CT is very poor; they are di…     

Lymphoepithelial cyst of the pancreas associated with elevated CA 19-9 levels

An extremely rare case of a lymphoepithelial cyst associated with persistent elevation of serum carbohydrate antigen (CA) 19-9 levels is described. A 72-year-old man was incidentally found to have a cystic tumor in the uncus of the pancreas and to have a high serum CA 19-9 level. At 2-year follow up, the tumor size had gradually increased and the high CA 19-9 level persisted. Because the malignant potential of the tumor could not be ruled out, a laparotomy was performed. The tumor was excised from the pancreas. On pathological examination, the tumor was proven to be a lymphoepithelial cyst. After the operation, the patient’s serum CA 19-9 level decreased to the normal range. Lymphoepithelial cysts of the pancreas are a rare, benign entity. They are true pancreatic cysts, characterized by a mature, keratinizing, squamous lining surrounded by lymphoid tissue. Because the treatment options and the prognosis of these entities are quite different from those of other cystic neoplasms of the pancreas, preoperative differential diagnosis is the main issue. Thus, although they are rare, lymphoepithelial cysts should be considered in the differential diagnosis of cystic tumors of the pancreas.     

Macrocystic serous cystadenoma of the pancreas: clinicopathologic features in seven cases.

BACKGROUND: Serous cystic neoplasms of the pancreas are uncommon tumors classified as microcystic adenomas. In this article, the authors report clinico-pathologic features of seven cases of macrocystic variant of the serous cystadenoma. METHODS: Seven patients (5 females and 2 males) with a diagnosis of cystic lesion of the pancreas were observed after 1995. Clinical, radiological, and pathologic features, including immunohistochemistry, were reported. Enzymes and tumor markers CEA, CA 19-9, CA 125, CA 15-3, CA 72-4, and mucin-like carcinoma-associated antigen (MCA) were investigated in the serum and cyst fluid of the patients. Cytology was also performed. RESULTS: Six patients were symptomatic complaining abdominal pain. All cases had radiologic evidence of unilocular cyst of the pancreas. The suspected diagnosis was consistent with mucinous cystic neoplasm. Serum tumor markers were all in the normal range. After surgery, pathology showed in all cases a cyst lined with cuboidal, periodic acid-Schiff (PAS)-positive epithelium, without mucin content or atypia. Minute microcysts were found surrounding the main cavity. Immunohistochemical stains were positive for cytokeratin, CA19-9, CA15-3, CA 72-4, and MCA. CEA was unexpressed. CA 125 in the cyst fluid were found elevated in three cases and CA 19-9 in three cases. Cytology was negative in all cases. CONCLUSION: When a unilocular pancreatic cyst is found, without history of pancreatitis and gallstones, having low serum tumor markers levels and negativity of CA 72-4 and MCA in the cyst fluid, the diagnosis of the macrocystic variant of the serous cystadenoma may be suggested. At present, the diagnosis is still based on pathological examination after cyst removal.     

Endoscopic ultrasound-guided fine needle aspiration in diagnosis of cystic pancreatic lesions

Background and study aimspancreatic cysts are commonly found lesions and proper diagnosis is very important for planning further management. The study aims to evaluate the role of cyst fluid amylase and tumour markers as cancer antigen (CA 19-9) and carcinoembryonic antigen (CEA) in addition to mucin stain in diagnosing pancreatic cysts and differentiating malignant from benign lesions.Patients and methodsThis prospective study was conducted on 184 patients diagnosed to have pancreatic cystic lesions from January 2013 to January 2018. Fluid analysis for CA 19-9, CEA, amylase, mucin stain and cytopathology were done. We compared these data with the final diagnosis based on histopathology after surgical resection, positive cytopathology and long period of follow up of the patients for at least 18 months.ResultsThe highest AUC was that of cystic CEA with cut-off value of 160 ng/ml; it had a sensitivity of 60.4% and a specificity of 85%. The best cut-off value for cystic CA 19-9 was 1318 U/ml with a sensitivity of 64.1% and a specificity of 68.1%. The cut-off value of cyst amylase level was 5500 U/L, with 84.2% sensitivity and 37.1% specificity. The sensitivity of mucin stain in detecting mucinous cystic neoplasm was 85.45%, specificity was 86.05% with accuracy 85.87%.ConclusionCyst fluid analysis by investigating amylase, mucin, CA 19-9, CEA and EUS examination improves the diagnosis of different pancreatic cysts.     

Autoimmune pancreatitis associated with a large pancreatic pseudocyst

INTRODUCTIONAutoimmune pancreatitis (AIP) is a benign disease that responds well to steroid treatment. Characteristics in-clude radiological evidence of an irregular narrowing of the pancreatic main duct and a diffuse enlargement of the pancreas, together…     

Macrocystic serous cystadenoma of the pancreas

Summary Background. Serous cystic neoplasms of the pancreas are uncommon tumors classified as microcystic adenomas. In this article, the authors report clinico-pathologic features of seven cases of macrocystic variant of the serous cystadenoma. Methods. Seven patients (5 females and 2 males) with a diagnosis of cystic lesion of the pancreas were observed after 1995. Clinical, radiological, and pathologic features, including immunohistochemistry, were reported. Enzymes and tumor markers CEA, CA 19-9, CA 125, CA 15-3, CA 72-4, and mucin-like carcinoma-associated antigen (MCA) were investigated in the serum and cyst fluid of the patients. Cytology was also performed. Results. Six patients were symptomatic complaining abdominal pain. All cases had radiologic evidence of unilocular cyst of the pancreas. The suspected diagnosis was consistent with mucinous cystic neoplasm. Serum tumor markers were all in the normal range. After surgery, pathology showed in all cases a cyst lined with cuboidal, periodic acid-Schiff (PAS)-positive epithelium, without mucin content or atypia. Minute microcysts were found surrounding the main cavity. Immunohistochemical stains were positive for cytokeratin, CA19-9, CA15-3, CA 72-4, and MCA. CEA was unexpressed. CA 125 in the cyst fluid were found elevated in three cases and CA 19-9 in three cases. Cytology was negative in all cases. Conclusion. When a unilocular pancreatic cyst is found, without history of pancreatitis and gallstones, having low serum tumor markers levels and negativity of CA 72-4 and MCA in the cyst fluid, the diagnosis of the macrocystic variant of the serous cystadenoma may be suggested. At present, the diagnosis is still based on pathological examination after cyst removal.     

Cyst fluid analysis in the differential diagnosis of pancreatic cystic lesions: a pooled analysis

BACKGROUND: Pancreatic cystic tumors commonly include serous cystadenoma (SCA), mucinous cystadenoma (MCA), and mucinous cystadenocarcinoma (MCAC). A differential diagnosis with pseudocysts (PC) can be difficult. Radiologic criteria are not reliable. The objective of the study is to investigate the value of cyst fluid analysis in the differential diagnosis of benign (SCA, PC) vs. premalignant or malignant (MCA, MCAC) lesions. METHODS: A search in PubMed was performed with the search terms cyst, pancrea, and fluid. Articles about cyst fluid analysis of pancreatic lesions that contained the individual data of at least 7 patients were included in the study. Data of all individual patients were combined and were plotted in scatter grams. Cutoff levels were determined. RESULTS: Twelve studies were included, which comprised data of 450 patients. Cysts with an amylase concentration <250 U/L were SCA, MCA, or MCAC (sensitivity 44%, specificity 98%) and, thus, virtually excluded PC. A carcinoembryonic antigen (CEA) <5 ng/mL suggested a SCA or PC (sensitivity 50%, specificity 95%). A CEA >800 ng/mL strongly suggested MCA or MCAC (sensitivity 48%, specificity 98%). A carbohydrate-associated antigen (CA) 19-9 <37 U/mL strongly suggested PC or SCA (sensitivity 19%, specificity 98%). Cytologic examination revealed malignant cells in 48% of MCAC (n = 111). DISCUSSION: Most pancreatic cystic tumors should be resected without the need for cyst fluid analysis. However, in asymptomatic patients, in patients with an increased surgical risk, and, in patients in whom there is a diagnostic uncertainty about the presence of a PC, cyst fluid analysis helps to determine the optimal therapeutic strategy.     

Significances of serum level and immunohistochemical stain of CA19-9 in simple hepatic cysts and intrahepatic biliary cystic neoplasms]

BACKGROUND/AIMS: In spite of various diagnostic modalities, biliary cystic neoplasms (biliary cystadenoma and cystadenocarcinoma) remain to be difficult to diagnose preoperatively. Recently, there are some reports that elevated CA19-9 level in serum and/or cystic fluid could be a useful finding in the differential diagnosis of biliary cystic neoplasm. This study aimed to evaluate the expression of CA19-9 and to elucidate its significances in intrahepatic biliary cystic neoplasms and simple hepatic cysts. METHODS: In 8 patients with biliary cystic neoplasms and 6 simple hepatic cysts, symptoms, radiologic and laboratory findings were reviewed retrospectively. In 5 biliary cystic neoplasms (4 biliary cystadenomas, 1 biliary cystadenocarcinoma) and 5 simple hepatic cysts, immunohistochemical stainings for CA19-9 were performed with formalin-fixed, paraffin-embedded tissues. RESULTS: In 8 biliary cystic neoplasms, two cases were suspected as biliary cystadenoma preoperatively and 6 cases could not be distinguished from simple cysts or cholangiocarcinoma preoperatively. In 6 simple hepatic cysts, 3 cases were diagnosed preoperatively and 3 cases could not be distinguished from biliary cystadenoma or pancreatic pseudocyst preoperatively. Expression of CA19-9 in simple hepatic cysts and biliary cystic neoplasms were 80% in both groups. Expression of CA19-9 is not related to the elevated level of CA19-9 in serum. CONCLUSIONS: Our data suggests that the elevated level of CA19-9 in serum may not be helpful in the preoprative diagnosis of biliary cystic neoplasm.     

Diagnosis of pancreatic cystic neoplasms: a report of the cooperative pancreatic cyst study

BACKGROUND & AIMS: Cysts of the pancreas display a wide spectrum of histology, including inflammatory (pseudocysts), benign (serous), premalignant (mucinous), and malignant (mucinous) lesions. Endoscopic ultrasonography (EUS) may offer a diagnostic tool through the combination of imaging and guided, fine-needle aspiration (FNA). The purpose of this investigation was to determine the most accurate test for differentiating mucinous from nonmucinous cystic lesions. METHODS: The results of EUS imaging, cyst fluid cytology, and cyst fluid tumor markers (CEA, CA 72-4, CA 125, CA 19-9, and CA 15-3) were prospectively collected and compared in a multicenter study using histology as the final diagnostic standard. RESULTS: Three hundred forty-one (341) patients underwent EUS and FNA of a pancreatic cystic lesion; 112 of these patients underwent surgical resection, providing a histologic diagnosis of the cystic lesion (68 mucinous, 7 serous, 27 inflammatory, 5 endocrine, and 5 other). Receiver operator curve analysis of the tumor markers demonstrated that cyst fluid CEA (optimal cutoff of 192 ng/mL) demonstrated the greatest area under the curve (0.79) for differentiating mucinous vs. nonmucinous cystic lesions. The accuracy of CEA (88 of 111, 79%) was significantly greater than the accuracy of EUS morphology (57 of 112, 51%) or cytology (64 of 109, 59%) (P < 0.05). There was no combination of tests that provided greater accuracy than CEA alone (P < 0.0001). CONCLUSIONS: Of tested markers, cyst fluid CEA is the most accurate test available for the diagnosis of mucinous cystic lesions of the pancreas.     

Spontaneous rupture of a nonparasitic liver cyst complicated by intracystic hemorrhage

A case of spontaneous rupture of simple liver cyst complicated by intracystic hemorrhage is described. This rare condition was detected in a 61-year-old man who underwent left trisegmentectomy of liver under a suspected diagnosis of cystadenocarcinoma because of elevated serum levels of carbohydrate antigen (CA) 19-9 and DUPAN 2, and the presence of an intracystic structure. The resected specimen showed a benign liver cyst with intracystic hematoma and high levels of CA19-9 and DUPAN 2 in the cystic fluid. It is suggested that cyst rupture may increase serum levels of tumor markers whose levels are high in the cystic fluid, and that repeated observations of an intracystic structure may be the most reliable method to distinguish intracystic hemorrhage from cystic neoplasm. Received: November 30, 1998 / Accepted: February 26, 1999     

Macrocystic form of serous pancreatic cystadenoma

OBJECTIVES: Macrocystic serous cystadenoma of the pancreas are benign lesions with sometimes difficult diagnostic issues. We aimed to describe clinicopathological and imaging features with cyst fluid analysis in a series of patients undergoing surgery for macrocystic serous cystadenoma. METHODS: Eight patients underwent pancreatic resection for a macrocystic lesion of the pancreas diagnosed on ultrasonography or CT. Endoscopic ultrasonography and preoperative fine-needle aspiration were performed in seven patients. Immunohistochemical analysis of the surgical specimen with antibodies to carcinoembryonic-antigen (CEA), carbohydrate antigen (CA) 19-9, estrogen receptor, and progesterone receptor antibodies was performed in all cases. RESULTS: Patients included seven women and one man, with a mean age of 48 yr. Lesions were incidentally discovered on ultrasonography in six patients and had a mean size of 3 cm (range, 1.5-5 cm). Endoscopic ultrasonography revealed millimetric cysts in three cases. In the seven aspirated cysts, cytological analysis was non-contributive, but biochemical analysis showed low content of CEA (< 5 ng/ml) and CA72.4 (< 40U/ml) in all but two. At histology, cysts were lined by clear cuboidal cells. They focally expressed CA19-9 but were negative for anti-CEA, antiestrogen receptor, and antiprogesterone receptor antibodies. Microscopic cysts in the wall of the lesions were demonstrated in five cases. CONCLUSIONS: Macrocystic serous cystadenoma is a particular variant of pancreatic serous cystadenoma. Endoscopic ultrasonography may be useful in detecting peripherally located millimetric cysts in unilocular lesions, and measurement of enzymes and tumor markers in cyst fluid may also contribute to the diagnosis showing low concentrations.     

Epidermoid cyst of the intrapancreatic accessory spleen producing CA19‐9

We report a rare case of an epidermoid cyst in an accessory spleen at the pancreatic tail with producing CA19‐9. A 55‐year‐old female was admitted to our hospital, Cancer Research Institute, Kanazawa University, for close examination of a cystic lesion at the pancreatic tail and a high serum CA19‐9‐value (176 U/mL). There were almost no abdominal symptoms related to the cystic lesion. A cystic tumor approximately 3 cm in diameter and composed of multilocular cysts without a protruding portion of the inner surface was found at the pancreatic tail by ultrasound sonography, computed tomography, and magnetic resonance imaging. Endoscopic retrograde pancreatography revealed that the main pancreatic duct shifted at the pancreatic tail and there was no communication between the main pancreatic duct and cystic lesion. Based on a preoperative diagnosis of mucinous cystic tumor, distal pancreatectomy with splenectomy was performed. Histological ?ndings suggested an epidermoid cyst (3.5 × 3.0 cm) originating from an intrapancreatic accessory spleen. Immunohistochemical analysis of CA19‐9 in the epidermoid cyst showed clear staining of the inner epithelium of the cyst and amorphous or hyalinous cystic contents. The serum CA19‐9 value was con?rmed to decline to normal 2 months after resection. Physicians should not forget this disease during differential diagnosis related to pancreatic cystic lesions with elevated levels of serum tumor markers, such as CA19‐9 or carcinoembryonic antigen, although this disease is extremely rare.     

Utility of endoscopic ultrasound, cytology and fluid carcinoembryonic antigen and CA 19-9 levels in pancreatic cystic lesions

AIM： To assess the diagnostic accuracy of endoscopic ultrasound （EUS）, fluid tumor markers and cytology in distinguishing benign from （pre）malignant pancreatic cystic lesions.
METHODS： 46 consecutive patients, referred to a gastroenterologist and surgeon for a symptomatic or incidental pancreatic cyst, were reviewed. EUS, cytology, and carcinoembryonic antigen （CEA） and carbohydrate antigen （CA 19-9） levels were compared with the final diagnosis, based on surgical pathology and/or imaging follow-up of at least 12 mo. Cysts were classified as benign （pseudocyst, serous cystadenoma） or malignant/ pre-malignant （mucinous cystic neoplasm）. Receiver- operator characteristics （ROC） curve analysis was performed. RESULTS： The mean age was 56 years; 29% were male and median cyst diameter was 3 cm. Final outcome was obtained in 41 （89%） patients. Twenty-three （56%） of these 41 had surgical pathology. Twenty-three （56%） had benign lesions and 18 （44%） had malignant/premalignant lesions. Sensitivity, specificity and positive and negative predictive value of EUS alone to distinguish benign from malignant/premalignant pancreatic cystic lesions were 50%, 56%, 36% and 54% and for cytology were 71%, 96%, 92% and 85%, respectively. The corresponding values for the ROC-derived ideal cutoffs were 75%, 90%, 75%, 90% for CA 19-9 （〉 37 U/mL） and 70%, 85%, 79% and 78% for CEA （〉 3.1 ng/mL）. Subgroup analysis of those with surgical pathology yielded almost identical performance and cutoffs.
CONCLUSION： Cytology and cyst fluid tumor marker analysis is a very useful tool in distinguishing benign from （pre）malignant pancreatic cystic lesions.     

Cyst fluid analysis obtained by EUS-guided FNA in the evaluation of discrete cystic neoplasms of the pancreas: a prospective single-center experience

BACKGROUND: Accurate assessment of pancreatic cystic neoplasms is imperative before selecting available treatment options, such as surgical resection, drainage, or conservative therapy. Available modalities, CT and magnetic resonance imaging, have been inconsistent in diagnosis. Reports involving EUS and cyst fluid analysis have been encouraging, including studies of EUS features and/or cyst fluid analysis, which may differentiate pancreatic cystic neoplasms. OBJECTIVE: To retrospectively determine cyst fluid characteristics that differentiate cystic neoplasms. DESIGN: Patient evaluation included (1) EUS features (reported elsewhere) and (2) cyst fluid analysis (carcinoembryonic antigen [CEA], carbohydrate antigen 19-9 [CA 19-9], amylase and lipase, viscosity [VIS], mucin stain, and cytology). Exclusion criteria included the following: intraductal papillary mucinous tumor lesions, bloody cyst aspirate, neuroendocrine tumors, and patients without surgical histopathology. SETTING: Pancreatic Biliary Center, St Luke's Medical Center, Milwaukee, Wisconsin. PATIENTS: A total of 102 patients (60 women, 42 men; age, 23-76 years) presented for evaluation of pancreatic cystic neoplasm; 71 underwent surgical resection. RESULTS: Seventy-one of 102 patients who underwent surgery presented the following histopathologic correlates: 23 pseudocysts (PC), 13 serous cystadenoma (SCyA), 21 mucinous cystadenoma (MCyA), and 14 mucinous cystadenocarcinoma (MCyA-CA). Cyst fluid analysis of these patients showed the following: VIS was lower in PC (mean, 1.3) and SCyA (1.27) when compared with MCyA (1.84) and MCyA-CA (1.9). All mucinous neoplasms had VIS >1.6, whereas only 2 mucinous cystic neoplasms (MCN) had VIS = 1.6 (both PC). The CEA level was significantly higher in MCyA (adenoma [878 ng/mL], carcinoma [27,581 ng/mL]) vs PC (189 ng/mL), and SCyA (121 ng/mL). Amylase levels were higher in PC (7210 U/L) compared with cystic neoplasm (SCyA, 679 U/L; MCyA, 1605 U/L; MCyA-CA, 569 U/L). CONCLUSIONS: Differential diagnosis of pancreatic cystic neoplasm is significantly enhanced by cyst fluid analysis. Elevated CEA (> or =480 ng/mL) and VIS (>1.6) accurately predict MCN from SCyA and PC. Malignant from benign MCN can be differentiated by CEA levels > or =6000 ng/mL.     

Usefulness of in-phase and out-of-phase magnetic resonance imaging for the detection of pancreatic lymphoepithelial cyst

Lymphoepithelial cyst (LEC) of the pancreas is a rare benign lesion that may mimic cystic neoplasms. Diagnosis of pancreatic LEC has been depended on the elevation of serum carbohydrate antigen 19-9 (CA 19-9), biopsy of the cyst by endoscopic ultrasonography (EUS) and several abdominal imaging techniques including magnetic resonance imaging (MRI). However, it is very difficult to diagnose LEC of the pancreas preoperatively and excessive surgery has been done in spite of its benign nature. We herein report a case of pancreatic LEC which was preoperatively diagnosed by using in-phase and out-of-phase MRI and was successfully nucleated.     

Macrocystic type of serous cystadenoma with a communication between the cyst and pancreatic duct

A 42-year-old woman with a cystic lesion in the head of the pancreas was evaluated by using abdominal ultrasonography, a computed tomographic scan, magnetic resonance imaging and endoscopic retrograde pancreatography. Multiple cystic lesions, 5 cm in diameter, which had papillary protrusion inside the cyst in the head of the pancreas and had the communication between the cysts and pancreatic duct, were determined. Pylorus-preserving pancreaticoduodenectomy was performed under the diagnosis of mucinous cystic neoplasm of the pancreas. Although the cut surface of the tumor showed a macrocystic tumor of 3 cm in diameter, part of the cyst wall was cavernous. A histopathological examination showed single-layered cuboidal cells, which lead to the diagnosis as being serous cystadenoma of the pancreas. Serous cystadenoma is a rare, almost benign pancreatic tumor. The macrocystic subtype of serous cystadenoma is even more rare. We describe a patient who had this macrocystic subtype of serous cystadenoma with a communication between the cyst and pancreatic duct. This case illustrates the difficulty in the diagnosis of cystic lesions in the pancreas, and might support the single category of cystic lesions of the pancreas.     

A new case of solitary true pancreatic cyst

A true cyst of the pancreas is extremely rare, and few cases have been reported in adult patients. The authors report a new case of this rare pathological entity. A 35-year-old male patient was admitted to our unit with a cystic mass, about 6 cm in diameter, located in the pancreatic head, in proximity to the duodenum, vena cava, biliary tree and right kidney. Clinical features and imagery were suggestive for a benign neoplasm, as did surgical findings. Cystoduodenostomy was done. Histological finding revealed a cyst lined by cuboidal epithelium without morphologic alterations. Analysis of the cyst fluid showed a high level of CA 19-9 (10,000 U ml^?1). After 1-year follow-up, the patient was found to be doing well without any abdominal symptoms. Ultrasound images revealed no cyst recurrence.     

A ninety-eight-year-old woman with ruptured pancreatic anaplastic mucinous cystadenocarcinoma

A 98-year-old woman was admitted to our hospital complaining of anorexia, epigastralgia, and vomiting. An elastic hard tumor was palpable in her epigastric region. CT and US examination revealed a huge cystic lesion adjacent to the left lobe of the liver and the stomach. Her serum levels of CEA (13.6 ng/ml), CA19-9 (95 U/ml) and CA125 (99 U/ml) were high. She suffered from aspiration pneumonia on the 10th day of admission, which progressed to acute respiratory distress syndrome. On the 20th day of admission, the epigastric tumor suddenly disappeared. She passed away on the 31st day due to respiratory failure. Autopsy revealed that she had a ruptured pancreatic anaplastic mucinous cystadenocarcinoma. To the best of our knowledge, this is the oldest reported case of ruptured pancreatic cystadenocarcinoma in the world.     

Association between pancreatic cystadenocarcinoma, malignant liver cysts, and polycystic disease of the kidney

Polycystic kidney disease is an autosomal dominant disease that may be associated with cystic disease of the liver. In women, the cysts may develop early and be more troublesome than in men. Cystadenocarcinoma of the pancreas is uncommon, comprising 1% of primary pancreatic malignancies. This case report is the first to describe a familial association between polycystic kidney disease and cystadenocarcinoma of the pancreas and liver in the English medical literature. A patient with autosomal dominant polycystic kidney disease (ADPKD) and multiple hepatic cysts developed cystadenocarcinoma of the pancreas with multiple malignant liver cysts. The patient's mother, sister, and niece had ADPKD, and the patient's sister also died of pancreatic cystadenocarcinoma. We believe that the development of these two disease entities in which the primary pathology is cyst formation has a genetic association. (Gastroenterology 1997 Jun;112(6):2104-7)