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.     

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.     

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.     

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.     

Giant epithelial cyst of the accessory spleen

Splenic cysts are uncommon, and cysts derived from the accessory spleen are rare. We report a case of a huge splenic cyst derived from the accessory spleen in the omentum, concomitant with multiple epithelial cysts of the primary spleen. Both serum and cystic fluid concentrations of carbohydrate antigen (CA)19-9 were markedly elevated. A huge monolocular cyst occupied the entire abdomen. A total of 7?l of aspirated cystic fluid was turbid and yellowish-brown. The cyst wall, which consisted of CA19-9-positive squamous epithelium, contained approximately 2 × 3 × 1?cm of splenic tissue. There was a separate multicystic 16 × 8-cm spleen in the normal position. The CA19-9 serum level returned to normal postoperatively.     

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.     

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.     

Case of lymphoepithelial cyst of the pancreas]

We recently experienced a case of lymphoepithelial cyst of the pancreas, which is a rare benign pancreatic cystic tumor. A 59-year-old-man was pointed out as having a pancreatic tumor based on abdominal ultrasonography findings obtained during a medical check-up. Thereafter, abdominal ultrasonography, CT, and MRI studies revealed the presence of a multi-cystic tumor in the pancreas body to tail measuring 45 mm in size which had no infiltrating tendency. The operative findings showed a yellowish-white colored cystic tumor covered with a thin capsule, which could be easily extirpated from the pancreas. The dissected tumor, which contained a curd-like material and a condensed milky liquid, was diagnosed to be a lymphoepithelial cyst of the pancreas based on the pathological findings. Similar reports of lymphoepithelial cysts arising in the pancreas have recently been increasing.     

Lymphoepithelial cyst of pancreas. Role of endoscopic ultrasound guided fine needle aspiration

Lymphoepithelial cysts of the pancreas are extremely rare, benign, nonneoplastic cysts that may simulate pseudocysts or cystic neoplasm of the pancreas. Endosonographic and cytologic features of pancreatic lymphoepithelial cysts are poorly characterized because of their rarity. However, appropriate prospective diagnosis of pancreatic lymphoepithelial cysts using endoscopic ultrasound-guided fined needle aspiration (EUS-FNA) cytology may permit conservative management because of their benign nature. We conducted a MEDLINE search using the MeSH terms "pancreas", "cyst" and identified 16 patients who were diagnosed with endosonography or computerized tomography (CT) guided fine needle aspiration. Usefulness and pitfalls of EUS- or CT-guided cyst fluid analysis in the pre-operative diagnosis of pancreatic lymphoepithelial cysts are discussed.     

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.     

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.     

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     

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.     

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.     

Lympho-epithelial cyst of the pancreas: a difficult diagnosis. About two cases

We report two cases of lymphoepithelial cyst of the pancreas discovered incidentally on imaging in two men aged 50 and 61 years. One cyst contained hyperdense spots on CT-scan and in this case the biochemical analysis of the cyst fluid revealed high level of CEA. The unilocular cysts measured 4 and 5 cm on the surgical specimen. On microscopic examination, the cysts were lined by squamous keratinized epithelium positive for CEA, with rare mucin-secreting cells, lining on lymphoid tissue. The lymphoepithelial cyst is a rare dysembryoplastic benign lesion of the pancreas difficult to diagnose before surgery. The presence of keratin plugs on imaging is inconstant and the biochemical analysis of the cyst fluid can mislead to a diagnosis of mucinous cystic tumour. Cytological and histological analysis of fine needle aspiration material is of diagnostic interest and can avoid surgical resection when patients are symptom-free.     

Lymphoepithelial cyst of the pancreas

We experienced a rare case of lymphoepithelial cyst of pancreas. The patient was a 68-year-old man in whom a cystic lesion in the pancreas was detected when he was admitted for chronic renal failure. On ultrasonography, we detected a cystic lesion, 3 cm in diameter, with a septum, in head and body of the pancreas. On both computed tomography and magnetic resonance imaging, the cystic lesion did not show solid components. Endoscopic retrograde cholangiopancreatography showed no abnormality in the pancreatic duct and did not detect the cystic lesion. Although the lesion showed no evidence of malignancy on the images, we could not rule out malignancy, since high levels of carcinoembryonic antigen and carbohydrate antigen 19-9 were detected. We therefore performed a pancreatoduodenectomy. In the resected specimen, the lesion was a well-circumscribed and multilocular cyst that contained yellowish-white keratinous material and had no solid components. Histologically, the cyst wall was composed of mature keratinizing squamous epithelium and lymphoid tissue; it was diagnosed as a benign lymphoepithelial cyst of the pancreas.     

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…     

Lymphoepithelial cyst of the pancreas with sebaceous differentiation

We recently encountered a patient with a lymphoepithelial cyst of the pancreas with sebaceous differentiation. We sought to compare the characteristics of this patient with those previously reported in order to foster a keener understanding of this rare clinical entity. After reviewing the present patient's case in detail, we conducted a comprehensive review of the English-language literature and analyzed the clinical characteristics of reported cases of lymphoepithelial cysts. Our patient was an asymptomatic 60-year-old man who presented with an incidental finding of a cystic lesion in the tail of the pancreas documented by computed tomography. The cyst was enucleated, and was found to contain keratinized material. It was lined by squamous epithelium with small sebaceous glands, and surrounded by lymphoid tissue with germinal centers. Of 33 reported cases, only 6 (18%) contained sebaceous glands. In all patients who underwent operation, the cysts were easily resected, and the outcome was favorable. Lymphoepithelial cyst of the pancreas is rare, and may be difficult to differentiate from cystic neoplasms preoperatively. Therefore resection is indicated. The diagnosis, however, can be confirmed by careful histologic review, and the prognosis is excellent. Received: March 8, 1999 / Accepted: October 22, 1999     

Lymphoepithelial cyst of the pancreas: a new case report

We report the case of a lymphoepithelial cyst of the pancreas discovered by chance on imaging in a 54-year old man. CT-scan showed a 10 cm hypodense multilocular cystic tumor of the pancreatic isthmus. Fine-needle aspiration did not provide further information. Due to the lack of preoperative diagnosis and mostly because it was not known if the cyst was malignant or benign, the patient underwent a cephalic duodenopancreatectomy. Lymphoepithelial cyst of the pancreas is a rare benign lesion which is difficult to diagnose before surgery. Histologically, the cyst wall is lined by mature keratinizing squamous epithelium and a distinct surrounding lymphoid tissue layer. The cysts are filled with keratin plugs that are not always visualized on imaging. Cytological and histological analysis of fine-needle aspiration material if the sample material is sufficient may help avoid extensive surgery.     

Intra-abdominal sequestration of the lung and elevated serum levels of CA 19-9: a diagnostic pitfall

Background

Extralobar pulmonary sequestration is an uncommon congenital abnormality that is rarely diagnosed after the age of 40 years. We describe a 64-year-old woman with an intra-abdominal sequestration of the lung and elevated carbohydrate antigen (CA) 19-9 serum levels.

Case outline

On abdominal ultrasound a semi-solid cystic tumour was demonstrated that showed tight connection to the tail of the pancreas according to computed tomography. Cytological examination of the percutaneous biopsy did not lead to a definitive diagnosis. CA 19-9 serum levels were repeatedly elevated >250 IU/ml. With a tentative diagnosis of a tumour of the tail of pancreas the semi-solid cystic mass was resected. Frozen section histology suggested the diagnosis of pulmonary sequestration, which was confirmed by definitive histological examination. Immunohistochemical staining of the specimen with a specific monoclonal antibody against CA 19-9 showed strong immunoreactivity. Three months later the elevated CA 19-9 serum levels returned to normal.

Discussion

Elevated CA 19-9 serum levels have been described in benign pulmonary and mediastinal cystic lesions and in one case of extralobar intrathoracic lung sequestration. Although there is evidence that malignancies may arise in congenital lung cysts, CA 19-9 serum levels have not been investigated in such cases. Based on our results elevated serum values of CA 19-9 in combination with a cystic semi-solid mass in the left subphrenic space should include the differential diagnosis of extralobar pulmonary sequestration.