First Report of Retroperitoneal Mucinous Cystadenoma in a Patient with Hirsutism.

Primary retroperitoneal mucinous cystadenomas (PRMC) are rare benign neoplasms with only 55 documented cases in the English literature so far. A 19-year-old female exhibited hirsutism and was found to have a cystic mass measuring 5.8 cm × 3.9 cm × 5.8 cm in the left retroperitoneum. During subsequent work up, a high pre-operative value of dehydroepiandrosterone sulfate (DHEA-S) was noted. The patient was referred to surgical oncology and underwent an uneventful laparoscopic cystectomy. Pathology classified the cyst as PRMC. Post-operatively, the patient's DHEA-S levels normalized, though there was no appreciable decrease in the patient's hirsutism in the short-term follow-up. The origin of PRMC is uncertain. Due to their unknown biological potential, surgical resection is usually recommended. To the best of our knowledge, this is the first report documenting a PRMC and elevated levels of androgens in conjunction with hirsutism.

[Mucinous cystic liver tumors: diagnosis and surgical treatment]. / Mutsinoznye kistoznye opukholi pecheni: diagnostika i khirurgicheskoe lechenie.

AIM: To optimize diagnostics and treatment of cystic liver tumors. MATERIAL AND METHODS: The analysis included outcomes of 46 patients with liver cystic tumors. RESULTS AND DISCUSSION: The use of abdominal Doppler-sonography (37 patients), abdominal contrast-enhanced CT (44 patients) and MRI of abdominal cavity with MR-cholangiography (24 patients) defined radiological semiotics of cystic liver diseases. The most important features of cystic tumors are intraluminal septums with blood flow (82% of patients), solid component (6.8%), daughterly cysts (11.3%), as well as biliary hypertension (39.2% of patients). Research of oncomarkers (CEA, SA 19-9, AFP) in 40 patients showed increased level of SA 19-9 only in case of cystadenocarcinoma and intraductal papillary mucinous neoplasm of biliary type. Benign and malignant cystic tumors had increased contents of oncomarkers in all cases. Surgical treatment was used in 42 patients. Extended liver resections were performed in 10 (23.8%) patients, atypical and anatomical resections (removal of less than 3 segments) - in 31 (73.8%) patients. In one case we applied cryoablation of CA in segment I of the liver in view of invasion into the wall of inferior vena cava and hepatoduodenal ligament. In 2 cases surgery was carried out laparoscopically. Also robot-assisted technique was used in 3 patients. Immunohistochemical study was performed in 22 (44.8%) patients. The diagnosis of CAC and biliary type of IPMN was confirmed in case of high expression of CK7, SK19, MUC1, S100p, SDH2, p53 antibodies. Cystadenomas were associated with moderate expression of ER, PR and p53 antibodies by stroma and CK7, SK19, CDX2, MUC1, S100p antibodies by epithelium. CONCLUSION: There are considerable difficulties of differential diagnosis of liver cystic tumors. Therefore, the use of single algorithm of diagnostics and treatment is necessary to confirm accurately the diagnosis at the perioperative stage. Cystic tumor is more likely to be assumed in women with solitary cyst in segment IV of liver. If the diagnosis is suspected or confirmed anatomical liver resection with complete tumor removal is necessary to prevent the recurrence.

Pancreatic mucinous cystadenoma with serum CA 19-9 over 1,000,000 U/mL: a case report and review of the literature.

BACKGROUND: The diagnosis of pancreatic cystic neoplasms has become more accurate recently. In some cases, however, doubt remains regarding the lesion's malignant potential. CA 19-9 has long been identified as a reliable biomarker in differentiating pancreatic benign and malignant lesions, especially in non-jaundiced patients. CASE REPORT AND DISCUSSION: We report a case of a young female who presented with a mucinous lesion in the tail of the pancreas and a serum CA 19-9 over 1,000,000 U/mL. She was taken to surgery and had a distal pancreatectomy and splenectomy. Pathology reports showed only a mucinous cystadenoma. After 1 year of follow-up, her serum CA 19-9 was normal. Following that, the work-up in these lesions, the role of the biomarker in pancreatic adenocarcinoma and in the differentiation between benign and malignant lesions is discussed.

Comparison of pelvic masses score (PMS) and Risk of Malignancy Index (RMI 3) in the evaluation of pelvic masses.

PURPOSE: Ovarian cancer is the fourth cause of death from cancer in women worldwide and the majority of its diagnoses is made in an advanced stage of the disease. Several sonographic scoring systems have been created for a better preoperative discrimination between benign and malignant pelvic masses. The aim of this study was to evaluate the performances of the Risk of the Malignancy Index 3 (RMI 3) and the Pelvic Masses Score (PMS). MATERIALS AND METHODS: This retrospective study was performed in 55 women admitted to the department of Obstetrics and Gynecology of University of Udine for surgical exploration of pelvic masses between 2009 and 2012. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated for both the scores. RESULTS: PMS showed a sensitivity of 100%, a specificity of 93.8%, a PPV of 70%, and a NPV of 100%, while RMI 3 yielded a sensitivity of 85%, a specificity of 91%, a PPV of 60%, and a NPV of 97.8%. CONCLUSION: The authors found that, in discriminating between benign and malignant pelvic disease, the PMS method was more reliable than RMI3. PMS is a simple scoring system which can be used in clinical practice.

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 x 23 x 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.

Enhancement of ovarian tumor classification by improved reproducibility in matrix-assisted laser desorption/ionization time-of-flight mass spectrometry of serum glycans.

The serum N-glycome is a promising source of biomarker discovery. Matrix-assisted laser desorption/ionization time-of-flight (MALDI-TOF) mass spectrometry (MS) profiling of serum N-glycans was attempted for differentiating borderline ovarian tumor from benign cases, for which a low data spread is essential. An experimental protocol using matrix-prespotted MALDI plates and fast vacuum drying of the loaded N-glycan samples was developed, thereby minimizing the intensity variations in the replicates to an average relative standard deviation (RSD) of 3.96% for the highest N-glycan peak (m/z 1485.53) of the Sigma-Aldrich serum standard. When applied to sera of ovarian tumors, this procedure exhibited an average RSD of 5.74% for m/z 1485.53 and of 7.28% for all MS peaks. This improved reproducibility combined with the OVA-Beyond(®) screening software resulted in 75.1% and 79.4% correct classification for benign and borderline tumor samples, respectively, while the classification rates by the conventional ovarian tumor marker CA-125 were 54.4% and 53.1%, respectively. Both true positive rate and true negative rate fluctuated with small numbers of markers and converged as the number of markers increased. Cross-validations were performed in comparison with CA-125. These results suggest that our optimized process for MALDI-TOF MS of the serum glycome has a great potential for the screening of early stage ovarian cancer.

Cystic "feminine" pancreatic neoplasms in men. Do any clinical alterations correlate with these uncommon entities?

INTRODUCTION: Mucinous cystic neoplasm (MCN) and solid pseudopapillary neoplasm (SPN) of the pancreas are uncommon hormone-related pancreatic tumors (HRPTs) with a clear predominance in young women. This trial aims to investigate the possible association between HRPTs development in males and phenotypic and sex hormone alterations. METHODS: We performed a retrospective analysis of our database between February 1990 and February 2012. Risk factors for sexual dysfunction were considered exclusion criteria. We investigated secondary sexual characteristics development, sex hormone level and overall sexual dysfunction degree according with the International Index of Erectile Function Questionnaire (IIEF). RESULTS: We initially identified 25 patients [(MCN: n = 16 (64%); SPN: n = 9 (36%)]. At follow-up, 5 patients were lost, 8 resulted dead and 3 were excluded according to exclusion criteria. We finally enrolled 9 patients (MCN: n = 5; SPN: n = 4). Puberty occurred within physiological age for 7 patients, whereas it was delayed in 2 cases. Three patients revealed mild to moderate sexual dysfunction, along with low testosterone level in two cases. One patient presented hormonal alteration with a normal IIEF score. DISCUSSION: In this study, the first in literature with similar aim, hormonal and/or sexual dysfunction was present in 4 out of 9 patients affected by HRPT. The rarity of these lesions makes further trials to be needed for reliable conclusions.

Carcino Embryonic Antigen and long-term follow-up of mucinous pancreatic cysts including intraductal papillary mucinous neoplasm.

BACKGROUND AND AIMS: The utility of Carcino Embryonic Antigen (CEA) in differentiating malignant from benign pancreatic cysts is controversial. We sought to examine the role of CEA in differentiating benign from malignant cysts and its utility in progression of cyst size in follow-up. METHODS: Retrospective chart review of patients who underwent Endoscopic Ultrasound with Fine Needle Aspiration for mucinous cysts between 1998 and 2010. CEA was measured in benign and malignant mucinous cysts. Coefficient of determination (R(2)) was used to measure the association between change in cyst size and CEA. Mann-Whitney test was used to compare the median values of CEA. RESULTS: 143 patients (38.4% males) were included (mean age 68.9 ± 0.8 years). 105 patients had intra-cystic CEA measured. 63 patients underwent surgery while 80 patients were in the follow-up group. In the surgical group, median CEA value for benign and malignant mucinous neoplasms was 796 and 438 ng/ml, respectively (p=0.79). The median follow-up was 21 months. There was no correlation between CEA level and progression in cyst size in patients who had >6 months of follow-up, R(2)=0.0002. Malignant transformation was observed in 5 (5.9%) patients. CONCLUSION: CEA level was not predictive of malignant cyst nor cyst size progression over follow-up.

Discovery of diagnostic biomarkers for pancreatic cancer in immunodepleted serum by SELDI-TOF MS.

MOTIVATION: Reports of serum pancreatic cancer (PC) biomarkers using SELDI-TOF MS have been inconsistent because different chip surfaces and interference with high-abundant proteins. This study examines the influence of these factors on the detection of discriminating diagnostic biomarkers. METHODS: Serum from fourteen from patients with PC, disease controls (DC, n = 14) and healthy volunteers (HV, n = 14) were evaluated by SELDI using H50, IMAC, Q10 and CM10 chips. A further evaluation was undertaken after depletion of seven high-abundant proteins using spin cartridges. RESULTS: More protein peaks were detected in whole serum than in depleted serum for IMAC, H50 and Q10 chips: 60 vs 39, 56 vs 48 and 69 vs 65, respectively, while the CM10 found less peaks in serum (27 vs 47 peaks). However, there were more differentially expressed peaks in the depleted serum samples for PC vs DC and PC vs HV samples using the H50, Q10 and CM10 ProteinChip arrays, whereas for IMAC arrays, more discriminating peaks were seen in non-depleted serum. The highly significant peaks observed on Q10, CM10 and H50 are consistent with the previous finding of ApoA-I (m/z 27,910-28000) and ApoA-II (m/z 8758 and 17,240). In addition, a number of new discriminating protein peaks were found on different ProteinChip arrays, notably peaks at m/z 4280 and 7763 on IMAC arrays. CONCLUSION: This study confirms the diagnostic value of ApoA-I&II and identifies further potential diagnostic biomarkers for pancreatic cancer when multiple chip surfaces are used with depletion of the most highly-abundant proteins.

Serum C-reactive protein in the differential diagnosis of ovarian masses.

OBJECTIVE: A number of serum tumor markers have been investigated to aid clinicians in the differential diagnosis of ovarian masses. Serum C-reactive protein (CRP) is a widely used biomarker of inflammation and has been previously shown to be a promising biomarker in patients with ovarian cancer. STUDY DESIGN: In a retrospective single-center study, we evaluated serum CRP in 576 patients with benign and in 242 patients with malignant (ovarian tumors of low malignant potential [LMP]: n=44, epithelial ovarian cancer [EOC]: n=198) ovarian masses. Results were correlated to clinical data. RESULTS: Median (25th, 75th percentiles) serum CRP in patients with benign ovarian tumors, with ovarian tumors of LMP, and with EOC were 0.5 (0.5, 0.6)mg/dL, 0.5 (0.5, 0.9)mg/dL, and 1.36 (0.5, 4.9)mg/dL, respectively (p<0.001). In the subgroup of patients with EOC, serum CRP significantly correlated with FIGO stage (p<0.001), residual tumor mass (p<0.001), and patients' age (p=0.04), but not with tumor grade (p=0.2) and histologic type (p=0.4). In univariable and multivariable models including serum CRP, serum CA 125, and patients' age, serum CRP independently predicted the presence of malignant ovarian masses (p<0.0001; Odds Ratio [OR] 5.3, 95% Confidence Interval [CI] 3.8-7.4). Serum CRP had a sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) for identifying malignant ovarian masses of 49.8%, 84.1%, 57.1%, and 79.8%, respectively. CONCLUSION: Serum CRP is associated with the presence of malignant ovarian tumors independent of serum CA 125 and patients' age and can therefore be used as additional diagnostic marker in the differential diagnosis of ovarian masses.

[Clinical value of serum CA19-9, CA125 and CP2 in mucinous ovarian tumor: a retrospective study of 273 patients].

OBJECTIVE: To evaluate the diagnostic and prognostic value of serum CA19-9, CA125 and CP2 in mucinous ovarian tumors. METHODS: In this retrospective study, the serum CA19-9, CA125 and CP2 levels of 273 hospitalized patients with ovarian tumors of either mucinous or non-mucinous type were analyzed. RESULTS: (1) CA19-9 had the biggest area under curve (AUC) in mucinous tumors followed with CA125 while CA125 and CP2 had bigger AUC in non-mucinous tumor. (2) For the diagnosis of mucinous tumors, CA19-9 and CA125 combination showed a greatly increased sensitivity compared with CA19-9 or CA125 alone (93.8% versus 75.0% and 66.7%, P<0.05) with no significant improvement of the specificity (P>0.05). For the diagnosis of non-mucinous tumors, CA125 and CP2 combination showed an increased sensitivity compared with CA125 or CP2 alone (85.0% versus 80.7%, P>0.05, 85.0% versus 70.6%, P<0.05) with no significant improvement of the specificity (P>0.05). (3) Seventy percent of tumor marker-positive patients could undergo cytoreductive surgery. Compared with those who could not undergo cytoreductive surgery, they were more likely to have normal tumor marker two months after surgery (P<0.05) and longer interval to re-elevation of tumor markers (P>0.05), with lower recurrence and death rate (P<0.05). All of the 20 tumor marker-negative patients could have cytoreductive surgery with only 10% recurrence. (4) CA19-9 increased mainly in recurrent mucinous tumor, while CA125 increased dominantly in recurrent non-mucinous tumor. (5) The survival rate of CA125 and CP2 positive patients was much lower than CA125 and CP2 negative patients (P<0.05), while the survival rate was similar between CA19-9 positive and CA19-9 negative patients. CONCLUSIONS: CA19-9 is a sensitive index for diagnosis of mucinous ovarian tumors. Combination of CA19-9 with CA125 can improve the sensitivity of diagnosis and postoperative monitoring of mucinous ovarian tumors. Combination of CA125 with CP2 is more valuable in the diagnosis of non-mucinous ovarian tumors.

Peritoneal fluids from patients with certain gynecologic tumor contain elevated levels of bioactive lysophospholipase D activity.

Levels of lysophosphatidic acid (LPA), an important phospholipid mediator, in serum and ascitic fluid from ovarian cancer patients were shown to be higher than those from healthy women and from patients with other type of cancer, respectively. Although LPA in human serum seems mainly to be generated by lysophospholipase D (lysoPLD), the source and pathway for LPA in the ascitic fluid remain still obscure. In this study, we examined whether lysoPLD activity producing bioactive LPA in human peritoneal fluid was significantly elevated under pathological statuses. Lysophospholipase D activity in human peritoneal fluids was measured by quantifying choline released from exogenous lysophosphatidylcholine on their incubation at 37 degrees C. We also compared the activity of lysoPLD in sera from patients with different gynecologic diseases. We found relatively high lysoPLD activity in peritoneal fluids from patients with ovarian cancer, dermoid cyst or mucinous cystadenoma, whereas there were no significant differences in the serum lysoPLD activity among clinical groups and healthy subjects. The lysoPLD in the peritoneal fluid was found to have similar substrate specificity and metal ion requirement to those of serum lysoPLD, that has been identified as autotaxin, a tumor cell-motility stimulating protein. Our results suggest that increased lysoPLD activity in peritoneal fluid from patients with certain gynecologic tumors might be relevant to its potential of tumor progression.

The value of standard serum tumor markers in differentiating mucinous from serous cystic tumors of the pancreas: CEA, Ca 19-9, Ca 125, Ca 15-3.

BACKGROUND: Differentiating between mucinous cystic tumors (MCTs) and serous cystic tumors (SCTs) can be a troubling diagnostic dilemma in pancreatology: when SCTs present in their macro-oligocystic form they must be resected because MCT cannot be ruled out, and some tumors considered benign are actually MCTs, which delays diagnosis and places patients at increased risk. Examination of preoperative serum tumor markers may help improve preoperative diagnosis. MATERIALS AND METHODS: The tumor markers CEA, Ca 19-9, Ca 125, and Ca 15-3 were examined in 157 patients with SCTs or MCTs. RESULTS: Positive CEA marker status is an indicator of an MCT, although sensitivity is low at 17%. Using three serum tumor markers (CEA, Ca 19-9, and Ca 125), 27% of MCTs were found to have two or more markers positive, compared to none for the SCTs. Sensitivity decreases to 13% for differentiating benign MCTs from benign SCTs but specificity remains 100%. CONCLUSIONS: In the differential diagnosis of SCTs vs. MCTs no reliable serum tumor marker exists which can diagnose SCTs and spare some patients unnecessary operations. Nonetheless, positive CEA serum marker status and or the presence of more than two positive serum markers (CEA, Ca 19-9, or Ca 125) indicates the presence of an MCT and can prevent delay in diagnosis.

Role of tumor markers in the diagnosis of cystic and intraductal neoplasms.

Despite recent advances in imaging procedures, the correct diagnosis of cystic lesions of the pancreas is lacking in about one third of cases. Cyst fluid analysis can help in the differential diagnosis, particularly in patients with unilocular or paucilocular lesions, thus precluding unjustified resection in patients with benign cystic lesions of the pancreas. Although use of cystic fluid marker analysis is helpful in several situations, it is crucial to carefully evaluate the clinical context with appraisal of patient's demographics, clinical symptoms, and morphologic data. A multidisciplinary approach is advised and should improve the overall diagnostic performance and lead to better management strategies in patients presenting with such tumors of the pancreas.

Mucinous cystadenoma of the appendix with raised serum carcinoembryonic antigen concentration: clinical and pathological features.

A case of mucinous cystadenoma mimicking ovarian cancer is reported. Serum carcinoembryonic antigen (CEA) concentration was raised, and computed tomography of the abdomen and pelvis demonstrated a long oval shaped cystic mass measuring 9 cm in length on the right anterior side of the uterus. Because of possible right ovarian cancer, laparotomy was performed and the mass was found to be a mucinous cystadenoma of the appendix. This case indicates that mucinous cystadenoma of the appendix may show an unusual presentation including its location as well as the high serum CEA, mimicking ovarian cancer. Therefore, gynaecologists as well as gastroenterologists should consider its possibility as a differential diagnosis of the right adnexal mass in a patient without previous appendectomy.

Serum tumor markers and cyst fluid analysis are useful for the diagnosis of pancreatic cystic tumors.

BACKGROUND: This study was performed to evaluate the utility of serum and cyst fluid analysis for enzymes (amylase and lipase) and tumor markers (carcinoembryonic antigen, CA 19-9, CA 125, and CA 72-4) in the differential diagnosis of cystic pancreatic lesions. METHODS: Serum and cyst fluid were obtained from 48 patients with pancreatic cysts (21 pseudocysts, 14 mucinous cystic neoplasms, 6 ductal carcinomas, and 7 serous cystadenomas), observed between 1989 and 1994. RESULTS: Serum CA 19-9 levels were significantly higher in ductal carcinomas (all > 100 U/mL) and mucinous cystic neoplasms (P < 0.05). CA 72-4 cyst fluid levels were significantly higher in mucinous cystic tumors (P < 0.005), with 95% specificity and 80% sensitivity in detecting mucinous or malignant cysts. A combined assay of serum CA 19-9 and cyst fluid CA 72-4 correctly identified 19 of 20 (pre-) malignant lesions (95%), with only 1 false-positive result (3.6%). Cytology showed a sensitivity of 48% and specificity of 100%. CONCLUSIONS: Any pancreatic cyst with high serum CA 19-9 values, positive cytology, or high CA 72-4 in the fluid should be considered for resection.