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.     

A splenic cyst and a high serum CA 19-9: a case report

A 22-year-old man was admitted to our hospital with a palpable mass in the left upper quadrant. He had a history of blunt abdominal trauma. Computer tomography showed a large splenic cyst. Another finding was a markedly elevated concentration of the tumor marker CA 19-9 in his serum and cystic fluid. After surgical removal of the cyst this concentration decreased to a nearly normal level. Because no cellular lining of the cyst was found at histological examination, the cyst could have been regarded as 'false' or a 'pseudocyst'. However, since several cases of epithelial splenic cysts in combination with a high serum CA 19-9 have been described recently, our diagnosis was of an epithelial 'true' cyst. We assume that, for some reason, in primary splenic cysts, the thin cellular lining can be released before histological examination is performed.     

Determination of tumor marker levels in cystic fluid of benign liver cysts

The levels of tumor markers in cystic fluid and serum were measured in six patients with benign biliary cyst of the liver. AFP in the cystic fluid was lower than the upper normal limit for serum in all cases, and CEA in the cystic fluid was higher than the upper normal limit for serum in one of the six cases. CA19-9, DU-PAN 2, and SPAN 1 in cystic fluid were much higher than the upper normal limit for serum in all cases (more than 100-fold for CA19-9, twofold for DU-PAN 2, and ninefold for SPAN 1). CA19-9, DU-PAN 2, and SPAN 1 in cystic fluid were significantly higher than the levels in the corresponding serum. Positive immunohistochemical staining against CA19-9, DU-PAN 2, and SPAN 1 was observed in the cytoplasm of the epithelial cells of the cyst wall. These results suggested that the high concentrations of CA19-9, DU-PAN 2, and SPAN 1 in the cystic fluid were due to secretion from the epithelial cells in the benign biliary cysts.     

Giant benign true cyst of the spleen with high serum level of CA 19-9.

We report the case of a 21-year-old woman with a giant (20 cm) benign mesothelial splenic cyst and a high CA 19-9 serum level (1240 U/ml). The patient underwent resection of the cyst and splenectomy. True non-parasitic splenic cysts are very rare. Only 10 cases of benign true splenic cysts, with a high CA 19-9 serum level, have ever been published in the medical literature written in English. These are reviewed in this paper. We believe that the inner cellular lining of the cyst wall produces CA 19-9, which causes the high CA 19-9 serum level. This is supported by the facts that (1) the inner cellular lining is strongly positive for immunohistochemistry with CA 19-9, and (2) the CA 19-9 serum level returned to normal after resection of the cyst.     

Mucinous biliary cystadenoma with mesenchymal stroma: Expressions of CA 19-9 and carcinoembryonic antigen in serum and cystic fluid

a case of mucinous biliary cystadenoma with mesenchymal stroma (CMS tumor) in a 64-year-old woman is reported. The patient presented with acute abdominal pain and a palpable mass in the upper abdomen. Computed tomography and abdominal sonography showed characteristic multilocular cysts in the left lobe of the liver. Serum CA 19-9 was elevated to 108 U/ml with normal carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP) levels. The levels of CA 19-9 and CEA in the cystic fluid were high at 7430 U/ml and 576ng/ml, respectively. The serum CA 19-9 returned to 35 U/ml 4 weeks after tumor resection. These corresponding findings of both tumor markers in the serum and cystic fluid imply that (1) CA 19-9 and CEA both exist in the epithelial component of CMS tumors as evidenced by immunohistochemical stain, (2) serum CA 19-9 is a valuable marker in the diagnosis and monitoring of CMS, and (3) in cystic fluid, there are more significantly high levels of CA 19-9 in CMS compared with levels in simple cyst and polycystic liver disease. Therefore, measurement of CA 19-9 in cystic fluid and serum may be helpful in the differential diagnosis of hepatic cystic lesions.     

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.     

Two cases of pancreatic cystadenocarcinoma with elevated CA 19-9 levels in the cystic fluid in comparison with two cases of pancreatic cystadenoma

A 39-year-old woman was hospitalized with left hypochondralgia. Ultrasonography and abdominal computed tomography showed a cystic mass in the tail of the pancreas. Echo-guided aspiration of the cyst was performed, and a markedly elevated CA 19-9 level in the cystic fluid was found. A surgical operation was performed, and the tumor was radically resected. The pathologic diagnosis was papillary adenocarcinoma of the pancreas. Another case of pancreatic cystadenocarcinoma also had an extremely high cystic CA 19-9 level, whereas two cases with benign pancreatic cysts had very low cystic CA 19-9 levels. Moreover, positive localization of CA 19-9 in the cancerous tissue was clearly demonstrated by an immunohistochemical technique, indicating active secretion of CA 19-9 into the cyst, while CA 19-9 in the tissue of pancreatic cystadenoma was almost non-existent. Measurement of the CA 19-9 level in the cystic fluid might therefore be a valuable additional aid in the diagnosis of cystadenocarcinoma of the pancreas.     

Giant biliary mucinous cystadenoma of the liver

Biliary mucinous cystadenomas (BMC) of the liver are rare benign cystic tumors, however an estimated 20% undergo malignant transformation. They have recently been redefined as mucinous cystic neoplasms in the 2010 WHO classification. The preferred treatment is through radical resection, as there are high recurrence rates with other treatment modalities; however this is often not possible in patients with bilobar or giant cysts, and liver transplantation may be indicated. We present a patient with a giant biliary mucinous cystadenoma of the liver and discuss the management with reference to the literature. A 47 year-old woman presented with a 6-week history of moderate epigastric discomfort on a background of 12 months of symptom-free abdominal distension. A giant cystic bilobar tumor of the liver measuring 22 × 23 × 17 cm was diagnosed and characterised by ultrasound scan and magnetic resonance imaging. Serum bilirubin, alkaline phosphatase and gamma-glutamyl transpeptidase were elevated, though other laboratory data including tumor markers (CEA, aFP, CA19-9) were within normal limits. Total excision of the cyst was not possible due to its size and position, and the patient underwent cyst drainage, a sub-total cyst excision and omentoplasty. Histology confirmed a benign biliary mucinous cystadenoma with an ovarian stroma. Though the patient remained clinically well, routine post-operative computed tomography (CT) surveillance showed an 11 cm recurrent cyst at 6 months. A partial cyst resection with close follow-up, regular CA19-9 serology and ultrasound/CT imaging, may be a reasonable alternative for bilobar or giant cysts. However should any features pathognomonic of malignancy develop, then a liver transplantation is indicated.     

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.     

Carbohydrate antigen 19‐9 is extremely elevated in polycystic liver disease

Background/Aims: Carbohydrate antigen 19‐9 (CA19‐9) is used as a biomarker to differentiate benign from malignant gastrointestinal disorders. We examined the value of CA19‐9 measurement in polycystic livers after observing high CA19‐9 cyst fluid levels in a benign polycystic liver case. Methods: We determined CA19‐9 levels in serum (n=120) and hepatic cyst fluid (n=81), from patients with polycystic livers (n=109) and simple hepatic cysts (n=24). Further, we analysed CA19‐9 expression in normal and polycystic liver tissue (n=17). Results: Cyst fluid CA19‐9 levels from both polycystic livers and simple hepatic cysts were extremely high (median 91 000 U/ml, range 14–15 870 000 U/ml; median 85 000 U/ml, range 332–1 744 000 U/ml respectively). Serum CA19‐9 levels were significantly higher in polycystic liver patients (median 30 U/ml, range 0–1200 U/ml) compared with patients with simple hepatic cysts (median 10 U/ml, range 3–200 U/ml, P=0.0011). Serum CA19‐9 levels correlated with those in cyst fluid (r=0.3979, P=0.0399), polycystic liver volume (r=0.3870, P=0.0025) and the size of the largest cyst (simple cysts group; r=0.5319, P=0.0280). Cyst epithelia showed strong CA19‐9 expression. Evacuation of cyst fluid in four patients resulted in a dramatic decrease in the serum CA19‐9 levels (60–95%). Conclusions: CA19‐9 levels are high in the cyst fluid and serum of polycystic liver disease patients due to production and secretion by cyst epithelia. It does not reflect malignancy in these patients and may be of value as a biomarker for intervention efficiency assessment.     

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.     

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.     

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.     

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.     

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     

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…     

Ruptured epidermoid cyst and haematoma of spleen: a diagnostic clue of high levels of serum carcinoembryonic antigen, carbohydrate antigen 19-9 and Sialyl Lewis x

Splenic epidermoid cyst is a rare disease and that with haematoma is even more rare. The case of epidermoid cyst of the spleen is described, in a 36-year-old Japanese female, manifesting as left hypochondralgia and rupture of the cyst. Clinical features were splenic lesion 14 cm in diameter and consisting of round-hypovascular and crescent-hypervascular sublesions. Extravasation of cystic fluid was detected in abdominal cavity Preoperative diagnosis was difficult due to such uncommon features, however high levels of serum tumour markers (carcinoembryonic antigen, carbohydrate antigen 19-9, Sialyl Lewis x) strongly suggested epidermoid cyst. Laparotomic splenectomy and cholecystectomy were performed for splenic lesion and gallstones, and serum tumour markers decreased following surgery. Pathological diagnosis of the round-hypovascular lesion was epidermoid cyst and crescent-hypervascular lesion was haemorrhage (haematoma).     

Infected hepatic cyst

We describe an unusual case involving an infected hepatic cyst. An 88-year-old woman presented with acute onset of right upper quadrant abdominal pain, mild left lower abdominal pain, diarrhea, and fever. On admission, computed tomography revealed multiple hepatic cysts including an 8-cm cyst located in the left medial segment of the liver, which demonstrated a thickened wall enhanced with contrast media. Ultrasonography showed an 8-cm hypoechoic lesion which differed in appearance from the other, anechoic hepatic cysts. The serum concentration of C-reactive protein was 29.8 mg/dL; white blood cell count, 12,800/microL; CA19-9, 96 U/mL; and CEA, 2.2 ng/mL. Diagnosis of infected hepatic cyst was made by percutaneous transhepatic drainage of the cyst. Milky fluid was obtained and the patient's right upper quadrant abdominal pain resolved after drainage. The cyst fluid CA19-9 concentration was 18,000 U/mL. Cytology of the cyst fluid was negative. Serum CA19-9 (41 U/mL) and CEA (1.8 ng/mL) concentrations were improved 1 week after drainage. Escherichia coli was cultured from the drainage fluid. The patient was discharged 27 days after admission. Percutaneous transhepatic drainage is effective in the treatment of infected hepatic cysts.     

Combination of cyst fluid CEA and CA 125 is an accurate diagnostic tool for differentiating mucinous cystic neoplasms from intraductal papillary mucinous neoplasms

Background/objectivesDespite advances in imaging techniques, diagnosis and management of pancreatic cystic lesions still remains challenging. The objective of this study was to determine the utility of cyst fluid analysis (CEA, CA 19-9, CA 125, amylase, and cytology) in categorizing pancreatic cystic lesions, and in differentiating malignant from benign cystic lesions.MethodsA retrospective analysis of 68 patients with histologically and clinically confirmed cystic lesions was performed. Cyst fluid was obtained by surgical resection (n = 45) or endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) (n = 23). Cyst fluid tumor markers and amylase were measured and compared between the cyst types.ResultsReceiver operating characteristic (ROC) curve analysis of the tumor markers demonstrated that cyst fluid CEA provided the greatest area under ROC curve (AUC) (0.884) for differentiating mucinous versus non-mucinous cystic lesions. When a CEA cutoff value was set at 67.3 ng/ml, the sensitivity, specificity and accuracy for diagnosing mucinous cysts were 89.2%, 77.8%, and 84.4%, respectively. The combination of cyst fluid CEA content >67.3 ng/ml and cyst fluid CA 125 content >10.0 U/ml segregated 77.8% (14/18) of mucinous cystic neoplasms (MCNs) from other cyst subtypes. On the other hand, no fluid marker was useful for differentiating malignant versus benign cystic lesions. Although cytology (accuracy 83.3%) more accurately diagnosed malignant cysts than CEA (accuracy 65.6%), it lacked sensitivity (35.3%).ConclusionsOur results demonstrate that cyst fluid CEA can be a helpful marker in differentiating mucinous from non-mucinous, but not malignant from benign cystic lesions. A combined CEA and CA 125 approach may help segregate MCNs from IPMNs.     

Epithelial cyst arising in an intrapancreatic accessory spleen: a diagnostic dilemma

An epithelial cyst in an intrapancreatic accessory spleen (ECIAS) is a rare disease that is commonly misdiagnosed preoperatively. To identify the clinical and imaging features of ECIAS, we reviewed the relevant medical literature. Twenty-one cases of ECIAS were identified, including our own. The cases were mainly diagnosed as mucinous cystic neoplasm (MCN) preoperatively based on clinical and imaging features, such as, a woman in middle age; elevation of serum CA19-9 levels; location in the tail of the pancreas; and a solid component resembling a mural nodule. ECIAS is another lesion to be considered in the differential diagnosis of MCN.