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.     

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.     

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.     

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.     

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.     

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.     

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.     

Serum and cystic fluid CA 19–9 determinations as a diagnostic help in liver cysts of uncertain nature

Abstract: The distinction between hepatobiliary cystadenoma or cystadenocarcinoma and simple hepatic cyst complicated by intracystic hemorrhage may prove difficult to determine on the sole basis of clinical and radiological features because of the presence of intracystic structures and septations well-demonstrated by ultrasound examination in both situations. We investigated four patients with various types of hepatic cysts, in whom diagnostic difficulties led to further investigations. In this small group, CA 19–9 serum levels were abnormal only in the two patients with cystadenoma or cystadenocarcinoma. Cystic fluid CA 19–9 values were also five times higher in cystadenoma and cystadenocarcinoma than in other benign lesions. Our data thus suggest that the determination of serum and cyst fluid CA 19–9 may be of help in distinguishing between hemorrhagic simple cyst and cystadenoma or cystadenocarcinoma.     

Silicosis characterized by increasing serum CA 19-9 in parallel with progression of lung fibrosis]

In November 1997, a 61-year-old man was admitted to our hospital complaining of dyspnea. He had worked as a miner for 10 years and had received medical treatment based on a diagnosis of idiopathic interstitial pneumonia at our hospital since 1984. In conjunction with the progression of interstitial pneumonia, the patient's serum CA 19-9 had gradually increased since 1992, reaching 9,920 U/ml in 1997. Though cancer of the pancreas or other organs was suspected, an extensive examination revealed no malignancy. In April 1998, the patient died of bacterial pneumonia. Lung autopsy specimens disclosed severe interstitial fibrosis with prominent silicotic nodules. Based on these findings, silicosis was diagnosed. In immunohistochemical staining for CA 19-9, the lumina of severely fibrotic lesions covered with epithelial cells stained positively with anti-CA 19-9 antibody. These findings suggested that serum CA 19-9 may have been produced in the epithelial cells. We speculated that increased serum CA 19-9 levels in patients with interstitial pneumonia may occasionally be more indicative of the magnitude of destruction of lung architecture than the degree of disease activity.     

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.     

Thymic cyst with elevated SLX levels in serum and cystic fluid]

A 24-year-old man was admitted to our hospital in June 1996 with complaints of anterior chest discomfort. Chest X-ray films on admission showed an abnormal mediastinal shadow with well-defined margin. Chest X-ray examinations about 6 weeks earlier had not detected any abnormalities. Laboratory tests on admission showed a high serum concentration of Siaryl Lewis X-i antigen (SLX). A computed tomographic scan of the chest showed a large (6 x 6 x 12 cm) homogeneous mass in the right anterior mediastinum. The mass was removed completely and histologically diagnosed as a thymic cyst. Biochemical analysis of fluid from the cyst revealed remarkably high levels of SLX, CA 19-9, and CEA. In immunohistochemical studies, epithelial cells from the cystic walls stained positive for SLX, CA 19-9, and CEA. After the operation, the level of serum SLX returned almost to normal.     

Cystadenoma of the liver with high levels of ACE and CA 19-9 in the cyst

The development of a cystadenocarcinoma from previously benign cystadenoma is controversial. Neither clinical nor biological factors have been described to explain this transformation. High levels of serum and cystic CEA and CA 19-9 seem to help in the diagnosis of cystadenoma but not cystadenocarcinoma. Definitive histological evaluation is the only means to discriminate malignant from benign cysts. We report a case of cystadenoma of the liver with very high cystic levels and normal serum levels of CEA and CA 19-9.     

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.     

A case of thymic cyst with thymic hyperplasia]

A 70-year-old man was admitted to our hospital for treatment of an anterior mediastinal tumor. Although he was asymptomatic, an abnormal shadow was visible on chest radiography. CT revealed a heterogeneous tumor located in the anterior mediastinum. On MRI, the tumor demonstrated low signal intensity in T1-weighted images and high signal intensity in T2-weighted images. Laboratory data, including tumor markers, were mostly within the reference ranges. Although a CT-guided needle biopsy was performed, histologic diagnosis could not be confirmed. We resected the tumor with the thymus by median sternotomy. Histopathological examination demonstrated that the tumor was a multilocular thymic cyst with thymic hyperplasia and numerous lymphocytes. The lining epithelial cells gave positive reactions to immunohistochemical staining for carbohydrate antigen 19-9. Carcinoembryonic antigen and squamous cell carcinoma antigen levels in the cystic fluid were elevated. This case appeared to be an acquired thymic cyst with thymic hyperplasia. There are few reports of such cysts.     

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.     

Functional giant parathyroid cyst with high concentration of CA19-9 in cystic fluid

A 63-year-old man was admitted to our hospital for the evaluation of hypercalcemia and anterior neck mass. Laboratory findings revealed hypercalcemia, hypophosphatemia, and hypercalciuria, as well as elevated serum levels of parathyroid hormone (PTH) and alkaline phosphatase. Computerized tomography and magnetic resonance images showed that the mass contained a cystic area. Parathyroid scintigraphy using either 99mTc-sestamibi alone or 201Tl-chloride in conjunction with 99mTc-pertechnetate for thyroid image subtraction showed uptake of the radioactivity into the cyst wall, suggesting that the mass originated from the parathyroid. Fine needle aspiration biopsy revealed that the cyst fluid was serous and bloody with extremely high concentrations of both PTH and CA19-9. The patient was diagnosed as primary hyperparathyroidism caused by parathyroid cyst and cervical exploration was performed. The cyst was dissected away along with the right lobe of the thyroid gland. After tumor removal, serum calcium and PTH levels were normalized. Histological study showed that the tumor possessed malignant potential with capsular invasion as well as moderate cellular atypia with trabecular pattern in arrangement. Parathyroid cells in the wall of the cystic tumor were immunostained positively for CA19-9, suggesting that CA19-9 in the cyst fluid was produced from the cells.     

Non-parasitic splenic cysts： A report of three cases

Primary splenic cyst is a relatively rare disease, and the majority of cases are classified as epithelial cysts. Three cases with nonparasitic splenic cysts are presented: two epithelial and one pseudocyst. All cases had an atypical symptomatology, consisted mainly of fullness in the left upper abdomen and a palpable mass. Preoperative diagnosis was established with ultrasonography and computerized tomography. Two cases with large cysts located in the splenic hilum were treated with open complete splenectomy. The most recent case, a pseudocyst, was managed laparoscopically with partial cystectomy. All cases did not have any problems or recurrence during follow-up. Laparoscopic partial cystectomy is an acceptable procedure for the treatment of splenic cysts, because it cures the disease preserving the splenic tissue. Complete splenectomy is reserved for cases in which cyst excision cannot be done otherwise.     

Ciliated hepatic foregut cyst mimicking neoplasm

We describe a ciliated hepatic foregut cyst that was clinically considered neoplastic because it was large, bilocular, and associated with a high serological level of carbohydrate antigen 19-9 (CA 19-9). Histologically, the wall of the cyst showed characteristic pseudopapillae lined by ciliated stratified columnar epithelium with interspersed goblet cells and underlying smooth muscle. The epithelium was strongly immunoreactive for CA 19-9. We therefore conclude that large size, multilocularity, and elevated CA 19-9 do not exclude ciliated hepatic foregut cysts from diagnostic consideration.     

Solitary true cyst of the pancreas in two adults: analysis of cyst fluid and review of the literature

Solitary true cyst of the pancreas in adults is extremely rare. We report two adult cases of solitary true cyst of the pancreas. In a 53-yr-old woman there was discovered, incidentally, a unilocular cyst of 7.0 × 6.5 cm in size in the tail of the pancreas that was noted on abdominal US and CT scan. A 16-yr-old boy presented with abdominal pain, and an abdominal US and CT scan revealed a 6.5 cm cystic mass located in the tail of the pancreas. Both patients underwent distal pancreatectomy. Histologically, the cyst was lined with flattened cuboidal and squamous epithelium without morphological alterations. Analysis of the cyst fluid revealed high CA 19-9 (>100,000 U/L) and Span-1 levels (>60,000 U/L) in both cases. Immunohistochemically, the lining epithelial cells of true cyst were positive for CA 19-9 staining. The clinicopathological features of solitary true cyst of the pancreas in adults are briefly reviewed.     

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.