A case report of an angiomyxoma in the liver.

Angiomyxomas are uncommon myxoid tumours arising most commonly from the pelvis. A 46-year-old woman with a history of polycystic kidney disease presented asymptomatically for surveillance ultrasonography; changes were noted in the size and morphology of her liver cysts. Subsequent radiological assessment displayed features suspicious of malignancy and a right hemihepatectomy was performed with curative intent. Pathological examination of the resected specimen found histology consistent with an angiomyxoma arising primarily from the liver parenchyma. Follow-up review of the patient has been uneventful with annual imaging showing no evidence of recurrence. Angiomyxomas do not characteristically invade other tissues. However, any liver lesion displaying suspicious features of malignancy should be resected in the absence of disseminated disease.

Preoperative differential diagnosis between intrahepatic biliary cystadenoma and cystadenocarcinoma: a single-center experience.

AIM: To investigate preoperative differential diagnoses made between intrahepatic biliary cystadenoma and intrahepatic biliary cystadenocarcinoma. METHODS: A retrospective analysis of patient data was performed, which included 21 cases of intrahepatic biliary cystadenoma and 25 cases of intrahepatic biliary cystadenocarcinoma diagnosed between April 2003 and April 2013 at the General Hospital of PLA. Potential patients were excluded whose diagnoses were not confirmed pathologically. Basic information (including patient age and gender), clinical manifestation, duration of symptoms, serum assay results (including tumor markers and the results of liver function tests), radiological features and pathological results were collected. All patients were followed up. RESULTS: Preoperative levels of cancer antigen 125 (12.51 ± 9.31 vs 23.20 ± 21.86, P < 0.05) and carbohydrate antigen 19-9 (22.56 ± 26.30 vs 72.55 ± 115.99, P < 0.05) were higher in the cystadenocarcinoma subgroup than in the cystadenoma subgroup. There were no statistically significant differences in age or gender between the two groups, or in pre- or post-operative levels of alanine aminotransferase, aspartate aminotransferase, total bilirubin (TBIL), and direct bilirubin (DBIL) between the two groups. However, eight of the 21 patients with cystadenoma and six of the 25 patients with cystadenocarcinoma had elevated levels of TBIL and DBIL. There were three cases in the cystadenoma subgroup and six cases in the cystadenocarcinoma subgroup with postoperative complications. CONCLUSION: Preoperative differential diagnosis relies on the integration of information, including clinical symptoms, laboratory findings and imaging results.

Evaluation of hepatic cystic lesions.

Hepatic cysts are increasingly found as a mere coincidence on abdominal imaging techniques, such as ultrasonography (USG), computed tomography (CT) and magnetic resonance imaging (MRI). These cysts often present a diagnostic challenge. Therefore, we performed a review of the recent literature and developed an evidence-based diagnostic algorithm to guide clinicians in characterising these lesions. Simple cysts are the most common cystic liver disease, and diagnosis is based on typical USG characteristics. Serodiagnostic tests and microbubble contrast-enhanced ultrasound (CEUS) are invaluable in differentiating complicated cysts, echinococcosis and cystadenoma/cystadenocarcinoma when USG, CT and MRI show ambiguous findings. Therefore, serodiagnostic tests and CEUS reduce the need for invasive procedures. Polycystic liver disease (PLD) is arbitrarily defined as the presence of > 20 liver cysts and can present as two distinct genetic disorders: autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (PCLD). Although genetic testing for ADPKD and PCLD is possible, it is rarely performed because it does not affect the therapeutic management of PLD. USG screening of the liver and both kidneys combined with extensive family history taking are the cornerstone of diagnostic decision making in PLD. In conclusion, an amalgamation of these recent advances results in a diagnostic algorithm that facilitates evidence-based clinical decision making.

Hepatobiliary cystadenoma and cystadenocarcinoma: a single center experience.

AIMS AND BACKGROUND: Hepatobiliary cystadenoma and cystadenocarcinoma are rare cystic lesions of the liver. The aim of the study was to discuss the clinical features, diagnostic methods and surgical treatment of hepatobiliary cystadenoma and cystadenocarcinoma in our hospital. METHODS: Six patients with hepatobiliary cystadenomas and four with hepatobiliary cystadenocarcinomas were evaluated. We collected detailed clinical data, and all patients were followed. RESULTS: Three patients of the 6 with cystadenomas and 2 patients of the 4 with cystadenocarcinomas had marked elevation of CA19-9 (average, 707.0 U/ml and 1078.5 U/ml, respectively). CT scan with contrast revealed typical lesions in all 10 cases, i.e., cyst-occupying lesions with separations in the liver. All patients with hepatobiliary cystadenoma were treated by partial hepatectomy. None of them recurred at a mean follow-up of 40 months. Three patients with hepatobiliary cystadenocarcinoma underwent hepatectomy, without recurrence or metastasis at a mean follow-up of 32 months. CONCLUSIONS: Tumor markers (CA19-9) and imaging findings may be helpful for an early diagnosis. Complete resection is still the best choice. Even for hepatobiliary cystadenocarcinoma, considering the low malignant grade, we suggest that for the best prognosis radical excision should be attempted.

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.

Clinical significance of serum DKK-1 in patients with gynecological cancer.

Dickkopf-1 (DKK-1) is a secreted protein involved in embryonic development. Dickkopf-1 is also implicated in osteoporosis, arthritis, and cancer and represents a potential therapeutic target for the treatment of these diseases. Because DKK-1 encodes a secreted protein, we investigated whether the DKK-1 protein is secreted into the sera of patients with gynecological cancer. The levels of DKK-1 protein were assessed by enzyme-linked immunosorbent assay in the sera of 104 patients with gynecological cancer including 36 with ovarian, 40 with cervical, and 28 with endometrial cancers. The serum levels of DKK-1 protein were higher in patients with cervical (314.13 [385.02] pg/mL, P = 0.000) and endometrial (46.95 [21.62] pg/mL, P = 0.000) cancers than in healthy individuals (29.45 [11.86] pg/mL). The serum levels of DKK-1 protein were associated with clinical stage in all patients with gynecological cancer. In patients with cervical cancer, the serum levels of DKK-1 protein were also associated with histological type and lymphatic metastasis. Dickkopf-1 protein detection using enzyme-linked immunosorbent assay as a molecular marker can contribute to the detection and the diagnosis of cervical and endometrial cancers, especially for cervical squamous cell cancer.

Serum phenylalanine concentrations in patients with ovarian carcinoma correlate with concentrations of immune activation markers and of isoprostane-8.

Increased blood concentrations of essential amino acid phenylalanine are common in patients with HIV infection, in trauma and sepsis and in patients with cancer. The reason for this phenomenon is still unclear. However, all these clinical conditions are known to be linked with inflammation and immune activation. Oxidative stress resulting from chronic immune activation and inflammation could impair activity of phenylalanine (4)-hydroxylase (PAH) and thus give rise to increased phenylalanine concentrations. We therefore examined in 20 patients with ovarian cancer a possible association of serum concentrations of phenylalanine and tyrosine with immune activation markers 75 kDa soluble tumor necrosis factor-alpha receptor (sTNF-R75) and neopterin, and of oxidative stress marker isoprostane-8. Phenylalanine concentrations were higher in patients with higher FIGO stage and correlated with concentrations of sTNF-R75 (rs=0.441) and neopterin (rs=0.346; both p<0.05). No such correlations existed for tyrosine levels. The phenylalanine to tyrosine ratio (phe/tyr), an estimate of PAH activity, correlated somewhat stronger with sTNF-R75 (rs=0.549; p<0.01) and neopterin (rs=0.497; p=0.01). Finally, phenylalanine concentrations correlated with isoprostane-8 concentrations (rs=0.450, p=0.02). Correlations of phenylalanine and phe/tyr with immune activation markers point to a potential role of inflammation and immune activation in the accumulation of phenylalanine. The relationship between oxidative stress marker isoprostane-8 and phenylalanine as well as sTNF-R75 concentrations suggests a link between reactive oxygen species formed during chronic immune activation and inflammation and the decline of PAH activity, which might underlie the increase of phe/tyr (248 words).

[Diagnosis and treatment of intrahepatic biliary cystadenocarcinoma-a report of 11 cases].

BACKGROUND & OBJECTIVE: Intrahepatic biliary cystadenocarcinoma (IBC) is a rare intrahepatic malignant tumor which is scarcely reported, and there is relatively little experience in the diagnosis and treatment. This study was to analyze the clinicopathologic features, diagnosis, and treatment of IBC. METHODS: Clinical data of 11 patients with pathologically confirmed papillary IBC, treated between Mar. 1999 and Oct. 2006 with surgical operation in West China Hospital, were analyzed retrospectively. Of the 11 patients, 2 were men and 9 were women, with a median age of 54 (range 45-68). RESULTS: The chief complaints of the IBC patients were pain and distention in the epigastrium. Four cases were determined by immunohistochemistry, and showed cytokeratin 7 (CK7) expression. Four patients showed infiltration of carcinoma cells in the surrounding liver tissues; 3 of them received palliative hepatectomy and 1 received radical excision; they survived for 12-23 months. The rest 7 showed carcinoma cells confined to the cyst wall, and received radical excision; 3 of them survived for over 3 years; of the rest 4 patients, 1 received operation again 10 months later because of tumor recurrence and was still alive 14 months after the second operation, 1 suffered from intrahepatic multi-metastasis 12 months after operation and received expectant treatment, 1 suffered from ascites 15 month after operation and died without further treatment, 1 was lost during follow-up. CONCLUSIONS: IBC occurs mainly in elder women, and its malignant degree is lower than that of solid carcinoma. The prognosis of the patients with IBC confined to the cyst wall after complete tumor removal is better than that of the patients with IBC infiltrated into the liver.

[The plasma levels and diagnostic utility of granulocyte colony stimulating factor (G-CSF) and macrophage - colony stimulating factor (M-CSF) in ovarian cancer patients]. / Stezenie i przydatnoscdiagnostyczna czynnika stymulujacego kolonie granulocytarne (G-cSF) i makrofagalne (M-cSF) w osoczu chorych na raka jajnika.

UNLABELLED: G-CSF and M-CSF are of the glycoproteins called colony-stimulating factors (CSFs). Some clinical investigations have shown an autologous production of G-CSF and M-CSF in various human cell lines in vitro and by tumors in vivo. AIM OF STUDY: We have investigated the plasma levels and diagnostic utility of G-CSF M-CSF and commonly accepted tumor marker, such as CA 125 in ovarian cancer patients before surgery and healthy subjects. MATERIAL AND METHODS: We tested 30 patients with ovarian cancer and 30 healthy women (control group). G-CSF and M-CSF were determined using enzyme-linked immunosorbent assay (ELISA), CA 125 was measured by microparticle enzyme immunoassay (MEIA). RESULTS: G-CSF, M-CSF and CA 125 plasma levels were significantly higher in ovarian cancer patients compared to the control group. The diagnostic sensitivity of M-CSF was higher than for G-CSF and CA 125 (57%, 50% i 53%, respectively). The diagnostic specificity was high and equal for all tested parameters (93%). Positive predictive value was higher for M-CSF and CA 125 (equal 89%). Negative predictive value was the highest for M-CSF in comparison to G-C.SF and CA 125 (68%, 65%, 66,7%, respectively). CONCLUSIONS: Our study suggests that especially M-CSF can be clinically useful in diagnostic ovarian cancer, but further investigation and confirmation by a prospective study is necessary.

Is there a role for chemoradiation in the management of cystadenocarcinoma of the pancreas?

Despite a paucity of data, cystadenocarcinoma of the pancreas has been considered to be resistant to chemoradiation, with a limited effect similar to that of the more common pancreatic adenocarcinoma. We describe a case of a partially excised cystadenocarcinoma with a positive surgical margin that was treated by neoadjuvant chemoradiation. No epithelial elements were found on histologic examination after reresection. Three previous cases of dramatic response of pancreatic cystadenocarcinoma to chemoradiation have been described in the literature. The current dogma alleging poor response of pancreatic cystadenocarcinoma to chemoradiation may be in error.

Potential clinical utility of CA-125 in responsive but persistent large-volume ovarian cancer following platinum-based chemotherapy.

BACKGROUND: Despite the demonstrated clinical utility of the serum CA-125 antigen level in ovarian cancer, controversy exists regarding interpretation of "discordant" results between changes in this tumor marker and measurable disease masses. CASE: A patient with ovarian cancer cared for in the Gynecologic Cancer Program of the Cleveland Clinic Foundation receiving second-line single-agent carboplatin for recurrent disease demonstrated a major response in serum CA-125, but minimal shrinkage of a large painful abdominal mass. A laparotomy was performed both to define the nature of this mass and to attempt to relieve symptoms. The mass was found to be a large "inflamed pseudotumor with central necrosis." No viable tumors cells were found. CONCLUSION: This case represents an excellent example of the remarkably complex biology of malignant disease and suggests how evaluation of changes in CA-125 in women with ovarian cancer may be utilized in individual patients to develop optimal management plans.

Biliary cystadenocarcinoma: sonographic and cytologic findings.

Biliary cystadenocarcinoma is a rare hepatic cystic tumor. We report sonographic, CT, and MRI findings in an unusual case in an 87-year-old man. The diagnosis was aided by sonographically guided needle aspiration of the lesion, which revealed elevated levels of carcinoembryonic antigen and CA 19-9 in the cystic fluid.

Levels of serum neopterin are increased in pancreatic cancer patients and correlate with the prognosis.

UNLABELLED: Neopterin has been recognized as a valid marker for cellular immune activation. Raised neopterin levels indicate the stimulation of macrophages and indirectly T-cell activation. So far no data is available on serum neopterin in pancreatic cancer patients. PATIENTS: In this prospective clinical study serum neopterin values were evaluated in 83 patients with adenocarcinoma of the pancreas (PC), 47 patients with chronic pancreatitis (CP), 8 patients with cystadenocarcinoma (CA) of the pancreas and 24 healthy controls (HC). RESULTS: Serum neopterin was significantly elevated in PC (p < 0. 05) and CA (p < 0.04) as compared to HC. There was no difference found between CP and HC. Pancreatic cancer patients with neopterin levels above 2 pmol/ml had a significantly better survival (p < 0. 05) regardless of stage. In stage III and IV (UICC) this difference was highly significant (p < 0.001). Serum levels of neopterin in resectable patients were also significantly correlated with increased survival and in multivariate analysis proved to be an independent prognostic factor. Neopterin in PC was neither correlated with sex, resectability nor with CA 19/9 and CEA. CONCLUSIONS: Patients suffering from PC who did show activated cellular immune response reflected in elevated neopterin levels above 2 pmol/ml had a significantly better prognosis regardless of tumor stage. In advanced stages elevated neopterin concentrations were significantly associated with increased survival. Cellular immune response seems to influence survival in these advanced stages to a higher degree as expected. These findings underline the possibility for supportive immunotherapy in this patient group.

Soluble adhesion molecules in serum and cyst fluid from patients with cystic tumours of the ovary.

The potential of the soluble forms of the adhesion molecules ICAM-1 (sICAM-1), CD44std (sCD44std) and E-cadherin (sE-cadherin) was tested for the diagnosis of benign and malignant cystic epithelial tumours of the ovary. Concentrations of sICAM-1, sCD44 std and sE-cadherin were measured by enzyme-linked immunosorbent assay (ELISA) in the serum and cyst fluid obtained from 23 patients with luteal cysts, 29 with cystadenomas, nine with dermoid cysts, five with borderline tumours and 11 with carcinomas. Serum concentrations of sICAM-1, but not of sCD44std and sE-cadherin, were constantly elevated compared with normal controls. Cyst fluid concentrations of sICAM-1, sCD44std and sE-cadherin were elevated in borderline and malignant tumours compared with cystadenomas (P = 0.034, 0.006 and 0.001, respectively). In conclusion, our results suggest that serum concentrations of adhesion molecules have no diagnostic value in ovarian tumours, whereas cyst fluid concentrations may facilitate distinction between benign lesions and borderline or malignant tumours.

Prognostic significance of soluble interleukin-2 receptor-alpha in adenocarcinoma of the pancreas.

Soluble interleukin-2-receptor-alpha (sIL-2Ralpha) serum concentrations were examined in chronic pancreatitis patients, patients with cystadenocarcinoma of the pancreas, patients with adenocarcinoma of the pancreas and healthy blood donors. sIL-2Ralpha serum concentrations in pancreatic cancer patients were significantly higher than those of normal control subjects or chronic pancreatitis patients. In patients with adenocarcinoma of the pancreas no significant differences were found between sIL-2Ralpha and tumor size, grading, resectability and lymph node involvement. In Kaplan-Meier regression analysis patients with adenocarcinoma of the pancreas with low sIL-2Ralpha levels (<500 U/ml) lived significantly shorter than patients with sIL-2Ralpha concentrations above 500 U/ml (P < 0.01), suggesting that determination of sIL-2Ralpha serum concentrations could provide additional important information about prognosis.

Solitary splenic metastasis of an adenocarcinoma of the ovaries.

Metastatic involvement of the spleen by carcinoma occurs in a setting of widespread malignant disease. Solitary splenic metastasis in the absence of disseminated disease is rare. We report a case of a 55-year-old woman in whom the sole clinical manifestation of recurrent papillary serous cystadenocarcinoma of the ovary was an elevated serum CA125 level due to a solitary splenic metastasis.

[Value of the TAG-72 (CA 72-4) tumor marker in primary diagnosis of ovarian carcinoma. A comparison with the established CA-125 marker]. / Stellenwert des Tumormarkers TAG-72 (CA 72-4) bei der Primärdiagnostik des Ovarialkarzinoms. Ein Vergleich mit dem etablierten Marker CA-125.

The value of the TAG-72 (CA 72-4) serum marker in primary diagnosis was investigated in 110 patients with histologically diagnosed ovarian cancer. A reference group consisted of 103 patients with benign pelvic masses. Compared to the well-established CA-125, TAG-72 showed a low sensitivity of 42% in the detection of ovarian cancer. By contrast, when the cut-off level for TAG-72 was set at 6 U/ml, it showed a very high specificity of 99%. When the measurement of TAG-72 was combined to that of CA-125, improvements in both the specificity (as compared to a single CA-125 determination) and the sensitivity (as compared to a single TAG-72 assay) were observed. In such a combined assay, our results suggest that the best predictive value (positive and negative) was obtained if CA-125 is assigned a relatively high cut-off value (65 U/ml) in conjunction with a low cut-off level (3.2 U/ml or 4 U/ml) for TAG-72. In the present study, at threshold values of 65 U/ml respectively, a sensitivity of 86%, a specificity of 83% and a positive and negative predictive value of 85% were obtained. In mucinous carcinomas of the ovary, however, the additional TAG-72 determination did not lead to a better predictive power than did CA-125 measurement alone.

CA 50 compared with CA 19-9 as a serum tumour marker for pancreatic carcinoma.

This study compared the sensitivity and specificity of CA 50 and CA 19-9 as serum tumour markers for pancreatic cancer. One hundred and seventy one subjects were evaluated: 50 healthy controls, 50 patients with pancreatic carcinoma and 71 patients with chronic pancreatitis. Eighty per cent of the pancreatic cancer patients had raised CA 19-9 serum levels and 82% had raised CA 50 serum levels. In the group of patients ith chronic pancreatitis, false positive tests occurred in 8.4% for CA 19-9 and 11.3% for CA 50. For both markers the serum level showed a severe elevation in the advanced stage of cancer disease. Despite the good sensitivity and specificity of CA 50 as a serum tumour marker for pancreatic carcinoma, no major advantage was found compared to CA 19-9. Moreover, if the CA 50 cut-off, level is raised to 85 U/ml to exclude overlap with chronic pancreatitis, sensitivity falls sharply to 46% compared with 72% for CA 19-9 when a 100 U/ml cut-off level is used.

[Effect of rG-CSF on the morphology and function of neutrophils in ovarian cancer chemotherapy].

The combined use of rG-CSF in ovarian cancer chemotherapy prevents a decrease in the number of neutrophils and promotes their recovery. rG-CSF also protects against infections, enhancing chemotherapeutic effectiveness. It is reported that neutrophils produced by rG-CSF not only increase in number but also in function. Some clinicians, however, doubt whether neutrophils mobilized or produced by rG-CSF have sufficient ability to function practically in clinical cases. We therefore examined, by means of flow cytometry, the neutrophils' phagocytosis and bactericidal ability and we found both normal. No morphological abnormalities were seen in these neutrophils. In our observations, moreover, no effect was exerted on the leukocyte-membrane antigen. It was concluded that (a) rG-CSF was very effective in protecting against the diminution of neutrophils by chemotherapy and (b) in promoting their recovery, and (c) also the function, morphology and leukocyte-membrane antigen of these neutrophils were normal.

Organization of type III collagen in benign and malignant ovarian tumors. An immunohistochemical study.

BACKGROUND: Abnormal interaction with the extracellular matrix is a basic property of malignant cells. Type III collagen is a major constituent of the extracellular matrix of soft tissues. METHODS: Deposition of the aminoterminal propeptide of type III procollagen (PIIINP) was studied in benign (n = 41), borderline (n = 4), and malignant (n = 32) human ovarian tumors using the avidin-biotin-immunoperoxidase technique and affinity-purified antibodies to human PIIINP: It was then compared with the serum PIIINP concentrations of the patients at the time of operation. RESULTS: In malignant tumors, the distribution of PIIINP was irregular both close to the epithelial cancer cells and further away, in the stroma. Another feature typical of malignant tumors was the varying staining intensity of the PIIINP-positive fibers. The benign tumors were characterized by a regular organization and an intensive staining of PIIINP: Borderline tumors showed a slightly decreased staining intensity and altered PIIINP distribution. A significant positive correlation was found between the PIIINP concentration in serum and the degree of irregularity in the distribution of PIIINP: CONCLUSIONS: These preliminary results indicate that malignant transformation in ovarian tumors is associated with disintegration of adjacent collagenous structures and with alterations in type III procollagen metabolism, which also leads to increased serum PIIINP levels. They suggest that biochemical or immunohistochemical detection of the PIIINP antigen could be clinically useful in ovarian tumors.