Primary Retroperitoneal Cystoadenocarcinoma: A Systematic Review.

BACKGROUND/AIMS: The aim of this study was to perform a systematic review on primary retroperitoneal cystoadenocarcinoma (PRC), which is an extremely rare disease. METHODS: According to PRISMA guidelines, all the literature about PRC from 1977 to 2015 was reviewed. Thirty articles were selected; characteristics of the patients were collected and described; time to recurrence and overall survival (OS) were investigated when available. RESULTS: Thirty seven patients were included of whom 33 were females; the median age at presentation was 43. PRC was more common in postmenopausal women. Surgery was the standard therapy; the role of chemotherapy and/or radiotherapy was uncertain. Thirty percent of the patients relapsed after 58 months from the surgery; the rupture of the cyst occurred in 13% of the cases and it was associated with poor prognosis as well as premenopausal status. At 125 months from the diagnosis, 72% of the patients were alive and the median OS was not reached. CONCLUSIONS: The present systematic review about PRC is the first performed until the date of drafting this paper. We described some clinical features of PRC and their possible prognostic value. No conclusive data can be presented due to the small population analyzed and to publication bias.

Recto-vaginal septum cystadenocarcinoma: a case report and review of the literature.

BACKGROUND: Carcinoma of the recto-vaginal septum is a quite rare location and related to peritoneal and primary ovarian carcinomas. There are only few reports in the literature with a very poor prognosis. CASE PRESENTATION: Here we report the case of a 63 years old woman with past medical history of left oophorectomy presenting with a pelvic pain. The magnetic resonance imaging (MRI) demonstrated a 10 cm mass located in the recto-vaginal septum. A block resection was performed allowing the retrieval of a 10 cm solid tumor of the recto-vaginal septum. Peritoneal biopsies and the right ovary were normal the final diagnosis was cystadenocarcinoma of the recto-vaginal septum. The patient received adjuvant chemotherapy and displays no sign of recurrence 36 months after diagnosis. CONCLUSION: The management of recto-vaginal septum carcinoma with en bloc resection should be performed to avoid peritoneal spread and improve prognosis.

Diagnosis and Management of Pancreatic Cystic Neoplasms.

Incidentally discovered pancreatic cystic lesions are increasingly common, affecting up to 10% to 15% of patients undergoing cross-sectional imaging. Although some pancreatic cystic neoplasms harbor invasive malignancy or the potential to progress over time, a majority are benign and can be observed safely. Accurate diagnosis is key to appropriate management. Diagnosis requires a multidisciplinary and multimodal approach. This review discusses each type of pancreatic cystic neoplasm and the current data on diagnosis and treatment.

The risk of carcinogenesis in congenital choledochal cyst patients: an analysis of 214 cases.

BACKGROUND: Choledochal cysts are most common in Asian populations. In addition, the incidence of biliary tract malignancies from choledochal cysts is increasing, but the risk of carcinogenesis is still unclear. MATERIAL AND METHODS: Clinical data from 214 congenital choledochal cyst cases from 1968 to 2013 were retrospectively analyzed. RESULTS: Todani type I was more common (139, 65.0%) than type IVa (53, 24.8%) or type V (17, 7.9%) in these choledochal cyst patients. Biliary tract malignant tumors occurred in the gallbladder (2, 13.3%), common bile duct (10, 66.7%), and intrahepatic bile duct (3, 20%) in 15 patients (7.0%), including one patient in whom malignant transformation occurred in the intrahepatic bile duct in a type IVa patient 15 years after extrahepatic cyst resection. An age at symptom onset ≥ 60 years was a risk factor (p < 0.001), while an initial complete surgery was a protective factor for carcinogenesis (p = 0.001). CONCLUSIONS: Choledochal cysts should be removed once diagnosed because of an increased risk of malignant transformation with increasing age. Complete cyst removal is necessary for the first surgical treatment. Additional hepatectomy should be considered for type IVa choledochal cysts because cholangiocarcinoma can arise from the intrahepatic bile duct years after the extrahepatic cyst excision.

Ovarian tumors associated with pregnancy: a 20-year experience in a teaching hospital.

OBJECTIVE: Pregnancy associated with ovarian tumors was reviewed over a 20-year period to determine the maternal and fetal outcome in patients undergoing surgery during pregnancy. METHOD: A retrospective study of 94 cases of ovarian tumors treated surgically during pregnancy was investigated for incidence, clinico-pathological features and outcome in a teaching hospital between June 1987 and May 2007. RESULTS: The overall incidence of ovarian tumor in pregnant women was 1 in 505 (0.2%) deliveries. Diagnosis of 69.2% tumors resulted in the first and second trimesters of pregnancy. Twenty-two (23.4%) patients presented as an emergency at different periods of gestation and 16 (17.1%) tumors were incidentally discovered at cesarean section which underlines the significance of examining the ovaries routinely at cesarean section. Benign teratoma (39.4%) and serous cystadenoma (24.5%) were the most common types of ovarian tumors found in the study. The incidence of malignant tumors was 5.3%. Tumors with low malignant potential comprised 40% of malignancy. The miscarriage rate after surgery was 44.4% in the first trimester compared with 16.6% in the second trimester. The preterm birth rate was 4.3% in the series. CONCLUSION: The value of clinical and ultrasound examinations in early pregnancy as a diagnostic aid is highlighted. Whenever an ovarian tumor is detected in pregnancy, malignancy should always be suspected. Treatment of an ovarian tumor in pregnancy should be tailored according to the age, parity, clinical presentation, gestational age and histopathology of the tumor. Removal of persisting or enlarging ovarian masses as soon as possible is important to obtain a final histologic diagnosis and rule out malignancy. Early diagnosis and appropriate treatment of malignant tumors offers the best prognosis for the patient.

Risk factors for epithelial ovarian cancer by histologic subtype.

Previous epidemiologic studies suggest that the major histologic subtypes of epithelial ovarian cancer may have different risk factor profiles; however, no known prospective study has systematically examined differences in risk by subtype. The authors used Cox proportional hazards regression, stratified by histologic subtype and time period, to examine the association between ovarian cancer risk factors and incidence of serous invasive, endometrioid, and mucinous ovarian cancers in the US Nurses' Health Study (1976-2006) and Nurses' Health Study II (1989-2005). For each exposure, they calculated P-heterogeneity using a likelihood ratio test comparing models with separate estimates for the 3 subtypes versus a single estimate across subtypes. Analysis included 221,866 women and 721 cases with the histologies of interest (496 serous invasive, 139 endometrioid, 86 mucinous). In analyses of reproductive/hormonal exposures, the associations with age, duration of breastfeeding, age at natural menopause, and duration of estrogen use differed significantly by subtype (all P-heterogeneity < or =0.05). The associations with several nonreproductive exposures also appeared to vary by subtype, but only the association with smoking differed significantly (P-heterogeneity = 0.03). Results suggest that associations with several ovarian cancer risk factors vary by subtype, and these differences are consistent with known similarities between each major histologic subtype and its normal tissue counterpart.

Carcinoma of the nasal cavity and paranasal sinuses in Denmark 1995-2004.

OBJECTIVE: To evaluate the treatment outcome for sino-nasal carcinomas in Denmark from 1995-2004 and compare the results to the previous Danish survey covering 1982-1991. DESIGN: Retrospective follow-up. MATERIALS AND METHODS: In the five Danish head and neck oncology centres, charts of all consecutive patients with sino-nasal carcinomas were reviewed and data extracted to a common database. Altogether 242 patients from the period 1995-2004 were identified. Of these 162 (67%) were male and 80 (33%) female. Histologies included squamous cell carcinoma (55%), adenocarcinoma (28.5%), adenoid-cystic carcinoma (5.0%), undifferentiated carcinoma (4.5%), transitiocellular carcinoma (1.7%), mucoepidermoid carcinoma (0.8%), neuroendocrine carcinoma (2.5%), small cell carcinomas (1.2%) and carcinomas not otherwise specified (0.8%). Treatments included radiotherapy alone 79 (33%), surgery alone 29 (12%), combined surgery and radiotherapy 96 (40%), palliative/no treatment 38 (16%). A total of 204 (86%) patients were treated with curative intent. RESULTS: Of the 204 patients treated with curative intent, 94 (46%) relapsed. Most failures were in T-site (63, 30%). N-site failures were 10 (5%) and M-site failures six (3%). Failure occurring in T+N-site, T+M-site, N+M-site and T+N+M-site were seven (3%), two (1%), one (0.5%) and five (3%) respectively. The 5-year actuarial local, nodal and loco-regional control rates were 55 + or - 4%, 86 + or - 3%, 49 + or - 4%, respectively. The overall 5-year actuarial survival rate for the entire cohort was 47 + or - 3%, and the corresponding cancer-specific 5-year actuarial survival rate was 57 + or - 3%. Female gender, nasal cavity tumour, adenocarcinoma and low clinical stage were significant positive prognostic factors in univariate analysis. A Cox multivariate analysis showed that only tumour site and clinical stage were independent significant prognostic factors. CONCLUSION: The current series has confirmed stage and tumour site as independent prognostic factors. Compared to the previous Danish survey covering the period 1982-1991, the overall survival and cancer-specific survival rates have improved significantly.

A population-based analysis of 1037 malignant ovarian tumors in the pediatric population.

BACKGROUND: Concerns of malignant potential have impacted the utilization of ovarian salvage for treatment of ovarian masses in children. METHODS: The Surveillance, Epidemiology, and End Results (SEER) registry was analyzed for all females < or =19 y diagnosed with an ovarian tumor between 1973 and 2005. RESULTS: Overall, 1037 pediatric patients with ovarian tumors were identified. Approximately 61.7% of tumors occurred in patients 15 to 19 y old. The age-adjusted incidence of all malignant pediatric ovarian tumors in those < or =9 y was 0.102 versus 1.072 per 100,000 in those aged 10 to 19 y. The majority of cases (57.4%) present at an early localized stage. The predominant pathology was germ cell tumors in all age groups (77.4%). Overall 5- and 10-y survival rates are 91.7% and 91.4%, respectively. By multivariate analysis, advanced disease stage (HR 3.17, P<0.001), lack of surgery (HR 4.49, P =0.039), and poorly differentiated tumors (HR 3.40, P=0.011) were associated with worse outcomes. CONCLUSIONS: Malignant ovarian tumors are rare, particularly in patients under 5 y of age. Furthermore, the most common histologies are of low metastatic potential and carry high cure rates. Thus, the surgeon should implement ovarian-sparing strategies on the affected ovary unless a malignancy is clearly suspected and conserve the contralateral ovary in all children.

Cystic neoplasms of the exocrine pancreas: an update of a nationwide survey in Korea.

OBJECTIVE: The purpose of this study was to update a previous study of cystic neoplasms of the pancreas (PCNs) conducted in Korea by the authors. METHODS: Clinicopathologic data and factors associated with malignancy were evaluated from PCNs originating from the exocrine pancreas diagnosed between January 1993 and June 2005 in 30 university hospitals throughout Korea. RESULTS: A total of 1064 pathologically confirmed PCNs, which consisted of the following diagnoses, were collected: intraductal papillary mucinous neoplasm (IPMN), 436; mucinous cystic neoplasm (MCN), 268; solid pseudopapillary neoplasm (SPN), 195; serous cystic neoplasm (SCN), 162; acinar cell cystic neoplasm 2; and mature teratoma, 1. No malignant SCNs were diagnosed. In IPMN, advanced age, pancreatic head involvement, and hyperbilirubinemia were associated with malignancy based on multivariate analysis. In MCN, pancreatic head involvement was associated with malignancy based on multivariate analysis. CONCLUSIONS: Intraductal papillary mucinous neoplasms were the most common PCN observed in Korea. Solid pseudopapillary neoplasms were observed more frequently than those in studies from western countries. In IPMNs, advanced age was associated with malignancy, suggesting an adenoma-carcinoma sequence. Involvement of the pancreatic head was associated with malignancy in both IPMNs and MCNs, possibly warranting prompt surgical interventions.

Epithelial ovarian cancer and low malignant potential (LMP) tumors associated with a lower incidence of second primary breast cancer.

OBJECTIVES: Differences in the clinical characteristics of ovarian tumors of low malignant potential (LMP) and ovarian cancer have suggested divergences in tumor biology. The aim of this population-based study was to compare the risk of a second primary breast cancer after a history of either an LMP tumor or an epithelial ovarian cancer. METHODS: Breast cancers were evaluated among 3297 women with a history of LMP tumors, and 45,986 women with a history of epithelial ovarian cancer, within the Surveillance, Epidemiology, and End Results (SEER) Program. The expected incidence of breast cancer was then determined as a function of year, age, race, and geographic location, and combined with the observed incidence to derive the standardized incidence ratio (SIR). RESULTS: Forty-one (1.2%) patients with an LMP history were diagnosed with breast cancer, where 56.8 cases were expected, for an SIR of 0.72 [95% confidence interval (CI) 0.52-0.98]. Similarly, 734 patients (1.6%) with a history of ovarian cancer were diagnosed with breast cancer, where 809 were expected, for an SIR of 0.91 (95% CI 0.84-0.98). Overall, LMP patients were younger and had a shorter time between diagnoses. LMP patients also had a significantly lower relative risk of developing second primary breast cancers. CONCLUSION: Patients with a history of having either an LMP tumor or an epithelial ovarian cancer have a less than expected risk of subsequent breast cancer. Patients with LMP tumors are at lower risk than patients with a history of ovarian cancer for the development of these second malignancies.

Initial chemotherapy followed by surgical cytoreduction for the treatment of stage III/IV epithelial ovarian cancer.

OBJECTIVE: The purpose of this study was to evaluate differences in morbidity, progression-free interval, and survival in women with advanced epithelial ovarian cancer treated with initial chemotherapy versus initial surgery. STUDY DESIGN: All women with epithelial ovarian cancer who were treated surgically at our hospital between January 1, 1995, and January 1, 2003, were eligible; the cases of 200 patients met the criteria and underwent retrospective chart review. RESULTS: Ninety-eight patients (49%) had initial chemotherapy, and 102 patients (51%) had initial surgery. Patients who received initial chemotherapy were more likely to have stage IV disease (initial chemotherapy, 27%, vs initial surgery, 8%; P = .042) and grade 3 disease (initial chemotherapy, 73%, vs initial surgery, 61%; P = .025). Optimal cytoreduction was achieved more often in patients who received initial chemotherapy (initial chemotherapy, 86%, vs initial surgery, 54%; P < .001). Only optimal cytoreduction (P = .022), and not treatment choice (P = .089), had an impact on median survival. CONCLUSION: Initial chemotherapy is a reasonable alternative to initial surgery for the treatment of selected patients with advanced epithelial ovarian cancer.

Cystic neoplasms of the pancreas; what a clinician should know.

Primary cystic neoplasms of the pancreas (serous cystic neoplasms, mucinous serous neoplasms, and intraductal papillary mucinous neoplasms) are lesions of emerging importance. With the wide availability of modern imaging methods, these neoplasms are being recognized with increasing frequency. Due to the improvement of these sophisticated imaging techniques, it is often possible to differentiate preoperatively these primary pancreatic cystic neoplasms not only from other cystic pancreatic disorders (such as pancreatic pseudocysts) but also from one another. This differentiation is very important for the clinician, since these neoplasms have radically different biologic behavior. Serous cystic neoplasms are uniformly benign and usually do not mandate resection unless the lesion is symptomatic. In contrast, mucinous cystic neoplasms and intraductal papillary mucinous neoplasms have a premalignant or overtly malignant tendency, and therefore should be managed aggressively by pancreatic resection. In these mucinous cystic neoplasms, recognition of an underlying malignancy is often not possible without a detailed histopathologic examination of the entire resected specimen. In the absence of invasive disease, prognosis is excellent after appropriate surgery. The presence of invasive malignancy signifies a poor prognosis.

Improved survival with clinical guidelines? Evaluation of a quality register linked to clinical guidelines for ovarian cancer in the western health care region in Sweden between 1 September 1993 and 1 June 1998.

BACKGROUND: New clinical guidelines (CGs) for ovarian cancer in the western health care region in Sweden were established, beginning in September 1993 and still in effect. METHODS: A retrospective evaluation of 5 years of quality registration linked to CGs for ovarian cancer in this region was undertaken. The study material comprised 718 patients. Relative survival rates for the studied patients were compared with National Cancer Register data for the western health care region during the same period. The National Cancer Register data were also used to compare survival rate during the studied period and the preceding 5-year period. RESULTS: Relative 5-year survival rate in our material was 46.1%. Relative survival in western Sweden during the studied period was found to be improved compared with that during the preceding period (P<0.02). CONCLUSIONS: The CGs have led to an improved, tighter organization, with fewer clinicians in special 'tumor teams' performing more aggressive tumor reduction surgery. Chemotherapy prescription is centralized, while the actual administration is decentralized. This has probably been important for the good 5-year survival results.

Synchronous primary neoplasms of the uterine corpus and the ovary: a case report.

OBJECTIVE: To determine the frequency of synchronous primary neoplasia of the ovaries in patients with primary malignant neoplasia of the uterus, and to analyze the clinical and histological characteristics of these cases. MATERIALS AND METHODS: Clinicopathological data from a series of patients treated for primary malignant neoplasia of the uterus between 1985 and November 2003 have been studied retrospectively. RESULTS: Synchronous primary neoplasia of the ovaries was found in 13 out of 173 patients (7.5%) treated for primary malignant neoplasia of the uterus. In four patients (2.3%) the histological findings suggested ovarian metastases from primary endometrial adenocarcinoma. In four other cases (2.3%) there was extension of the primary uterine sarcoma to the ovaries. In the remaining five cases (2.9%) primary endometrial adenocarcinoma coexisted with: a) ovarian cystadenocarcinoma in two cases, b) ovarian fibromathecoma in two cases, and c) ovarian tumor of borderline malignancy in one case. CONCLUSIONS: Coexistence of distinct primary neoplasias in the uterus and ovaries is rare. Diagnosis of two primary malignancies in the uterus and ovaries should be based on histological examination. Treatment should be appropriate for both tumors, taking into consideration that treatment of one tumor will not lead to subtreatment of the other.

Familial clustering of ovarian and endometrial cancers.

Data on the association of ovarian cancer with other cancers in families are limited, and no data are available on the involvement of specific morphological types. The nationwide Swedish Family-Cancer Database on 10.2 million individuals and 19175 invasive ovarian cancers was used to calculate standardised incidence ratios (SIRs) and 95% confidence intervals (CIs) for familial ovarian cancer in 0-66-year-old daughters when mothers or sisters were affected. The SIR for concordant ovarian cancers was increased. When the mother or sister had breast cancer, the SIRs were 1.21 and 1.48, respectively; when they had endometrial cancer, the SIRs were 1.45 and 2.53. Multiple myeloma in the mother was associated with a risk of ovarian cancer in the daughter. The risk of endometrioid ovarian cancer was 3.40 in the daughter when the mother presented with endometrial cancer. Our data show a strong familial coupling of ovarian and endometrial cancers, which appears to be specific to the endometrioid morphology.

[Clinical characteristics of clear cell carcinoma of the ovary].

OBJECTIVE: To study the clinical characteristics of clear cell carcinoma of the ovary. METHODS: Forty three patients with clear cell carcinoma of the ovary and 51 patients with serous adenocarcinoma of the ovary who were admitted in Peking Union Medical College Hospital between 1984 to 2000 were analyzed retrospectively, and their chemosensitivities and the survival rates were compared. RESULTS: The percentage of early stage patients in the clear cell carcinoma of the ovary and the serous adenocarcinoma of the ovary was 14.4% and 3.8% respectively, the difference was significant (P < 0.005). In the late stage patients who underwent satisfactory cytoreductive surgery, the chemo-resistant rate (88.9%) in the clear cell carcinoma of the ovary was significantly higher than that (57.1%) of the serous adenocarcinoma of the ovary (P < 0.02), the 1-year survival rate (79.0%) in the clear cell carcinoma of the ovary was significantly lower than that (96.2%) of the serous adenocarcinoma of the ovary (P < 0.01). In the late stage patients who underwent unsatisfactory cytoreductive surgery, the chemo-resistant rate and the survival rate had no significant difference between the clear cell carcinoma of the ovary and the serous adenocarcinoma of the ovary (P > 0.05). CONCLUSIONS: There are more early stage patients with clear cell carcinoma of the ovary. We should conduct auxiliary therapy and close follow up to them after surgery. Clear cell carcinoma of the ovary is chemo-resistant to platinum-based chemotherapy and has poor prognosis.

Hereditary multifocal renal cystadenocarcinomas and nodular dermatofibrosis in 51 German shepherd dogs.

The clinical findings in German shepherd dogs with hereditary multifocal renal cystadenocarcinomas and nodular dermatofibrosis are presented. Between 1978 and 1996, 51 cases were examined. Eight cases were detected after being offered a clinical examination because the disease was present in a parent. The remaining 43 dogs were diagnosed after an unsolicited visit to a clinic because of a specific problem. Skin lesions were the main reason (37 per cent) the owners presented their dog for examination. The mean age at diagnosis of renal cystadenocarcinomas and nodular dermatofibrosis was 8.2 years. The male-to-female ratio was 1.1, while the corresponding figure for a reference population was 1.25. Enlarged and abnormally shaped kidneys were palpated in 60 per cent of the dogs and were detected by radiography in 86 per cent of cases. The renal lesions, including metastases, were the main reason for euthanasia and death. The mean age at death was 9.3 years, and the mean age at the first detection of nodular dermatofibrosis was 6.4 years.

Cystic neoplasm mistaken for pancreatic pseudocyst.

The authors report a case of cystic neoplasm of the pancreas treated by cystojejunostomy two years and half earlier. On that occasion, a false diagnosis of pancreatic pseudocyst had been suggested. Cystic neoplasm of the pancreas has a far better prognosis than adenocarcinoma. However, early detection is difficult and erroneous initial diagnosis may lead to delayed treatment and poor long-term results. In our case, though surgical treatment was aggressive and apparently radical, early recurrence of neoplasm was observed. Only early accurate diagnosis of pancreatic cystadenocarcinoma might improve the results of surgical treatment.

Ovarian cancer incidence and mortality in American Indian, Hispanic, and non-Hispanic white women in New Mexico.

Although ethnic and racial differences in ovarian cancer incidence and mortality have been reported worldwide, few published data have addressed the epidemiology of ovarian cancer among U.S. American Indians and Hispanics. We reviewed ovarian cancer incidence and survival data from New Mexico's population-based cancer registry collected from 1969 to 1992, and examined state vital records data for ovarian cancer deaths collected from 1958 to 1992, focusing on ethnic differences in occurrence and outcomes of ovarian malignancies. Non-Hispanic white women had age-adjusted incidence rates that were slightly higher (13.3/100,000) than rates for American Indians (11.4) and Hispanics (10.7) over the 24-year period. Ovarian cancer mortality rates were also higher for non-Hispanic whites than for minority women. Neither incidence rates nor mortality rates for ovarian cancer improved over the span of the study period. In addition, the stage at diagnosis did not shift substantially over time for any of the ethnic groups studied, nor did the distribution of various histopathological types shift proportionately. Only slight improvement was observed in 5-year survival over the time period of the study, with greater gains among younger (50 years old or less) versus older women. Ethnic differences in ovarian cancer incidence and mortality were apparent in our population-based data. However, our analysis indicated no reduction in ovarian cancer incidence or mortality in our state over the past quarter century and only slight improvement in 5-year survival.