A case of ruptured splenic cyst with elevated serum levels of CEA treated by laparoscopic unroofing.

Splenic cysts are rare and tend to have elevated tumor markers, of which carbohydrate antigen (CA) 19-9 is the most frequently elevated. Therefore, splenic cysts with elevated serum carcinoembryonic antigen (CEA) levels and without CA19-9 elevation are extremely rare. A 26-year-old woman presented with sudden upper abdominal pain while sleeping. Contrast-enhanced computed tomography (CT) showed an 85-mm simple splenic cyst in the upper pole and a moderate amount of ascites around the spleen. The serum levels of CEA, but not CA19-9, were elevated. Spontaneous rupture of a splenic cyst was diagnosed. We performed elective laparoscopic unroofing. The histological findings revealed a stratified squamous epithelium on the inner surface of the cystic wall. On immunohistochemical examination, the squamous epithelium was found to be positive for cytokeratin (CK)7, negative for calretinin, and positive for CEA. The histological diagnosis was an epidermoid cyst. Three months after the operation, the elevated serum tumor marker levels of CEA were normalized. Splenic cysts with high levels of CEA and low levels of CA19-9 are extremely rare. Laparoscopic unroofing is a useful operative procedure for ruptured splenic cysts with elevated levels of serum tumor markers.

Testis Sparing Surgery for Benign Testicular Masses: Diagnostics and Therapeutic Approaches.

PURPOSE: Small benign testicular masses are often misinterpreted as germ cell tumors and immediate inguinal orchiectomy is performed. We analyzed the diagnostic and therapeutic workup of testicular masses to improve preoperative stratification algorithms. MATERIALS AND METHODS: We performed a retrospective, single center analysis of the records of 522 patients diagnosed with primary testicular masses of unknown malignant potential. RESULTS: A total of 28 patients (5%) showed a primary benign tumor after resection, including Leydig cell tumors in 9 (32%), epidermoid cysts in 9 (32%), adenomatoid tumors in 8 (29%) and Sertoli cell tumors in 2 (7%). The median volume of benign tumors was significantly less than that of malignant tumors (0.75 cm3, range 0.1 to 2.1 vs 15, range 4.5-39.9, p ≤0.001). At a cutoff of 2.8 cm3 tumor volume most accurately differentiated between benign and malignant disease, and it was a predictor of malignancy with 83% sensitivity and 89% specificity (OR 1.389, 95% CI 1.035-1.864, p = 0.029). Symptom duration in patients with benign tumors was significantly longer (365 days, range 25.5 to 365 vs 20, range 7 to 42, p ≤0.001). Also, tumor markers were unaltered in benign lesions. In patients with benign tumors significantly more fertility disorders or cryptorchidism were found (p ≤0.001) as well as a tendency toward lower testosterone (3.9 µg/l, range 0.9 to 4.9 vs 5.3, range 3.5 to 6.8, p = 0.084). Testis sparing surgery was performed in 22 of all patients (79%) with benign tumors. There was no case of relapse during followup. CONCLUSIONS: Nongerm cell tumors should be considered when small testicular masses have a volume of less than 2.8 cm3 and there are hormone disorders or normal tumor markers. Immediate orchiectomy should be avoided, favoring testis sparing surgery.

Primary Epidermoid Cyst of Biliary Duct Presenting as Choledochal Cyst.

Choledochal cyst is a cystic dilation of the biliary tree that can increase the risk of malignancy in bile ducts and the gallbladder. These are usually lined by bile duct epithelium, which may undergo intestinal and squamous metaplasia. This is the first report of clinically diagnosed type II choledochal cyst that is entirely lined by metaplastic stratified squamous epithelium, unlike most other cysts, which are histologically lined by bile duct epithelium. This observation can potentially explain the underlying pathogenic mechanism of rare reports of squamous cell carcinomas arising in bile duct systems.

The implication of tumor biomarker CA19-9 in the diagnosis of intracranial epidermoid cyst.

OBJECT: The diagnosis of intracranial epidermoid cyst (IEC) relies solely on MRI, which is time and money consuming. The application of tumor biomarkers in IEC has never been systematically studied. Here we screened a group of commonly used tumor biomarkers to assess their diagnostic value in IEC. RESULTS: Serum tumor biomarkers were assessed in 42 IECs and 42 paired healthy controls. Only serum CA19-9 level was significantly higher in the IEC group (median 20.3U/ml vs. 6.5U/ml, p < 0.001). Area under curve for CA19-9 was 0.806 (95% CI 0.700-0.912), with cutoff value of 13.15 U/ml (sensitivity 71.4%, specificity 97.6%). Tumor size was significantly different between CA19-9 positive and CA19-9 negative groups(64.14 ± 67.91cm3 vs. 19.43 ± 13.76 cm3, p = 0.04) and linear regression analysis revealed a positive correlation. Neither the extent of resection nor recurrence rate showed any significant difference between the two groups. METHODS: This is a retrospective study of IEC patients treated between 2009 and 2014. We analyzed the expression of common serum tumor biomarkers, including carbohydrate antigen 19-9 (CA19-9), carcinoembryonic antigen, carbohydrate antigen125 and squamous cell carcinoma in both IEC and healthy control group. Receiver operating characterisitics curves were constructed to evaluate the diagnostic accuracy. CONCLUSIONS: Our data indicated that for serum CA19-9 level higher than 13.15U/ml, after excluding the possibility of gastrointestinal system tumor, lung cancer, inflammation and other related diseases, the existence of IEC should be considered. Further prospective study is needed to gain more understanding of the value of CA19- 9 in postoperative evaluation and surveillance.

A Splenic Epithelial Cyst: Increased Size, Exacerbation of Symptoms, and Elevated Levels of Serum Carcinogenic Antigen 19-9 after 6-year Follow-up.

A 58-year-old man, who had presented with a large cyst between the pancreatic tail and splenic hilum 6 years previously, was referred to our hospital with exacerbation of abdominal distention. Computed tomography revealed a well-demarcated, unilocular cyst, with a beak sign for the pancreas, without wall thickening or nodules suggestive of a non-neoplastic cyst. Compared with 6 years previously, the cyst had increased in size from 14.7 cm to 19.5 cm, and the serum carcinogenic antigen 19-9 level had increased from 635 U/mL to 1,918 U/mL. To prevent spontaneous rupture, laparotomy was performed, and the cyst was pathologically diagnosed as a splenic epithelial cyst.

A case report of spontaneous rupture of intracranial epidermoid cyst with dramatic increase of serum carbohydrate antigen 199: a three-year follow-up study.

BACKGROUND: Tumor markers are widely applied in clinical practice, however, few serum markers have been found for intracranial tumors. Herein, we firstly report an intracranial epidermoid cyst case with extremely high level of serum CA 199. Furthermore, the relationship between CA 199 level and intracranial epidermoid cyst was closely followed for a long period. CASE PRESENTATION: We report a case of 41-year-old man with a history of 2 months' headache and sudden exacerbation for 3 days. Radiology examination suggested multiple lesions spreading along ventricular system. Laboratory tests showed exceeding increase of serum CA 199. The patient underwent craniotomy and continuous lumber drainage. Post-operative pathology proved a ruptured intracranial epidermoid cyst. MRI scans and serum CA 199 were closely followed up for three years. CONCLUSION: This case suggests an important role of serum CA 199 in the diagnosis and follow-up of intracranial epidermoid cyst. Ruptured intracranial epidermoid cyst should be considered for a sudden onset case with multiple intracranial lesions and dramatically increased serum CA 199.

Elevated serum CA 19-9 level associated with a splenic cyst: which is the actual clinical management? Review of the literature.

Splenic cysts are relatively rare entities. The differential diagnosis for these lesions includes parasite infections, results of previous trauma or infarction, congenital forms, primitive splenic neoplasm or cystic metastasis. They can be either symptomatic, causing mainly abdominal pain, or asymptomatic, thus being diagnosed as in incidental finding during radiological examination for other clinical reasons: among these a raised serum level of CA 19-9 can be a case. It has been demonstrated that epidermoid and mesothelial congenital cyst can be associated with a pathological level of this tumor marker which is usually correlated to biliopancreatic and colonic carcinomas. The aim of the present study is to present the case of an asymptomatic epidermoid splenic cyst associated with a continuous increase of CA 19-9 and to describe the applied clinical workup and surgical management by laparoscopic total splenectomy. Moreover, to analyze the demographics, clinical and pathological features of these infrequent lesions and to confront our therapeutic management with that of the other reported cases, we conducted a systematic review of the literature.

Huge splenic epidermoid cyst with elevation of serum CA19-9 level.

A 30-year-old female was referred to our hospital for further examination of liver dysfunction. A huge, soft mass was noted in her left upper quadrant on physical examination. Abdominal ultrasonography and computed tomography revealed a huge cystic tumor of 20 cm in the hilus of the spleen. Serum CA19-9 was 491 U/ml, and splenectomy was performed under suspicion of a malignant cystic tumor. The inner surface of the cyst was lined by squamous epithelial cells that were immunohistochemically positive for CA19-9. Serum CA19-9 level was normalized after the surgery. Our case of a very rare, huge epidermoid cyst of the spleen suggests that measurement of the serum CA19-9 level is useful for evaluating therapeutic efficacy of a splenic epidermoid cyst.

Giant epidermoid cyst of the spleen with elevated CA 19-9 production managed laparoscopically: report of a case.

True splenic cysts are uncommon and are associated with elevated serum and intracystic tumor marker CA 19-9 levels. A 33-year-old woman presented to our hospital with a chief complaint of epigastralgia. Computed tomography of the abdomen showed a 10-cm cystic lesion in the spleen. The serum carbohydrate antigen (CA) 19-9 level was 3,347 U/mL (normal, <37 U/mL). Total laparoscopic splenectomy was performed, and the serum level of CA 19-9 had normalized 2 weeks later. Pathological examination showed a benign true epidermal cyst of the spleen with strong immunohistological staining for CA 19-9. Splenic epidermoid cysts most often occur in young women, and laparoscopic surgery to remove cysts of this type is minimally invasive. Thus, laparoscopic surgery should be the method of first choice for most cases of splenic benign true cyst.

Epidermoid cyst in an intrapancreatic accessory spleen: three case reports and review of the literatures.

The development of an epidermoid cyst in an intrapancreatic accessory spleen is an extremely rare lesion, with only 17 cases being reported in the English literature. All such cases were located in the pancreatic tail, some of which showed carbohydrate antigen 19-9 (CA19-9) immunoreactivity in the lining of the epithelium. A few of them indicated an elevation of the serum CA19-9 level. Here we report three cases of an epidermoid cyst in an intrapancreatic accessory spleen. Cases 1 and 2 were 57-year-old and 70-year-old women, while case 3 was a 37-year-old man. All three cases were asymptomatic. Serum CA19-9 levels showed within normal limits (case 1), slightly elevated (case 2), and clearly elevated (case 3). They underwent a distal pancreatectomy with splenectomy (cases 1 and 2) and without splenectomy (case 3). Grossly, the surgical specimen was a well-demarcated, multiple (case 1) or solitary (cases 2 and 3) cystic mass in the pancreatic tail. A high level of fluid CA 19-9 was detected in case 1. Microscopically, the cystic walls were lined with squamous and cuboidal epithelium, which were surrounded by normal splenic tissue and hyalinized fibrous tissue. The lining squamous epithelium was revealed as nonkeratinizing (Cases 1 and 2) or keratinizing (Case 3). Immunohistochemically, CA19-9 was positive in the monolayer and surface layer of the cuboidal epithelium, but negative for the keratinizing squamous epithelium. As for the histogenesis, it is suggested that the cystic lining of the epithelium may derive from the pancreatic duct which protrudes into the accessory spleen.

Retroperitoneal masses with associated human chorionic gonadotropin production: Report of two cases.

BACKGROUND: The production of human chorionic gonadotropin (hCG) can be stimulated by both pregnancy and malignancy. METHODS: Two patients with increased serum hCG and a retroperitoneal mass were monitored throughout the course of disease to evaluate fluctuations in hCG concentrations. Pathologic examination was performed on the resection specimens. RESULTS: The retroperitoneal masses were identified as a benign epidermoid cyst and pancreatic ductal adenocarcinoma. The serum hCG concentration of the patient with the splenic epidermiod cyst decreased upon resection of the mass, and immunohistochemical staining demonstrated that the cyst lining expressed hCG. This is the first report of hCG secretion by an epidermoid cyst. The serum hCG concentrations of the patient with pancreatic adenocarcinoma paralleled the secretion of cancer antigen 19-9 and reflected the course of disease progression. Additional testing suggested that a different form of hCG was produced by each neoplasm. CONCLUSIONS: hCG secretion may be associated with both benign and malignant neoplasms. Laboratory testing can be utilized to further characterize increased serum hCG, and may help to determine the etiology of the increased hCG.

Benign splenic epithelial cyst accompanied by elevated Ca 19-9 level: a case report.

We report the case of a 30-year-old woman with a benign epidermoid splenic cyst and a high CA 19-9 serum level (268 U/mL). The patient underwent resection of the cyst and splenectomy. After removal of the cyst, the serum CA 19-9 level decreased to a normal level within 6 weeks. True non-parasitic splenic cysts are rare. Approximately 30 cases of benign true splenic cysts with a high CA 19-9 serum level have been published.

Laparoscopic splenic total cystectomy in a patient with elevated CA 19-9.

True nonparasitic splenic cysts are rare. Reports of approximately 30 cases of benign true splenic cysts producing elevated CA 19-9 serum levels have been published. The traditional treatment of splenic epidermoid cysts is splenectomy. However, with all the advances in laparoscopic surgery, conservative laparoscopic approaches are accepted as the most preferred techniques for this benign disease. Laparoscopic cystectomy with its minimal invasiveness and low morbidity is the ideal technique for properly selected patients. So far, only one case report of laparoscopic cystectomy for splenic epidermoid cyst with elevated CA 19-9 levels has been published. This is the second known report of this procedure being performed to remove a splenic epidermoid cyst in a patient with elevated CA 19-9 levels.

Hormone profiles in Klinefelter's syndrome with and without testicular epidermoid cyst.

Hormonal studies were performed in patients of Klinefelter's syndrome with and without testicular epidermoid cyst. Mean levels of plasma gonadotropins were elevated above normal ranges. The mean testosterone level in plasma was abnormally low. The mean prolactin level in plasma was within the normal limit. However, mean plasma levels of these hormones were not significantly different between the two groups. The administration of LH-RH resulted in low response of plasma gonadotropins. The response of plasma testosterone to the administration of hCG was also abnormally low. However, in the LH-RH and hCG tests there were no significant differences between the two groups. These data suggested that the hormonal disturbance in Klinefelter's syndrome is unrelated to the occurrence of testicular epidermoid cyst.