Clinical calculator predictive of chemotherapy benefit in stage 1A uterine papillary serous cancers.

OBJECTIVE: Determine the utility of a clinical calculator to predict the benefit of chemotherapy in stage IA uterine papillary serous cancer (UPSC). PATIENTS AND METHODS: Data were collected from NCDB from years 2010-2014. Based on demographic and surgical characteristics, a clinical score was developed using the random survival forest machine learning algorithm. RESULTS: Of 1,751 patients with stage IA UPSC, 1,012 (58%) received chemotherapy and 739 (42%) did not. Older age (HR 1.06), comorbidities (HR 1.31), larger tumor size (HR 1.27), lymphovascular invasion (HR 1.86), positive peritoneal cytology (HR 2.62), no pelvic lymph node dissection (HR 1.51), and no chemotherapy (HR 2.16) were associated with poorer prognosis. Compared to no chemotherapy, patients who underwent chemotherapy had a 5-year overall survival of 80% vs. 67%. To better delineate those who may derive more benefit from chemotherapy, we designed a clinical calculator capable of dividing patients into low, moderate, and high-risk groups with associated 5-year OS of 86%, 73%, and 53%, respectively. Using the calculator to assess the relative benefit of chemotherapy in each risk group, chemotherapy improved the 5-year OS in the high (42% to 64%; p < 0.001) and moderate risk group (66% to 79%; p < 0.001) but did not benefit the low risk group (84% to 87%; p = 0.29). CONCLUSION: Our results suggest a clinical calculator is useful for counseling and personalizing chemotherapy for stage IA UPSC.

Difficult Diagnosis of Peritoneal Serous Papillary Carcinoma in a 63-year-old Woman: A Case Report.

BACKGROUND: Peritoneal serous papillary carcinoma (PSPC) is a rare disease. It is clinically and histologically similar to progressive ovarian serous adenocarcinoma and involves normal-sized ovaries, making it challenging to diagnose. In this report, we describe a case of peritoneal serous papillary carcinoma that was difficult to identify and how we made a correct diagnosis in order to begin a timely course of treatment. CASE PRESENTATION: A 63-year-old woman with chief complaints of dizziness and abdominal pain was examined, but showed no particular abnormality. Class III cytology of the endometrium was detected through magnetic resonance imaging and a laparotomy was performed on suspicion of endometrial cancer. The patient was finally diagnosed with peritoneal serous papillary carcinoma and was treated with surgical resection and the standard indicated course of chemotherapy. CONCLUSIONS: The diagnosis and treatment of peritoneal serous papillary carcinoma may be delayed or may not be performed unless Class III findings are detected through uterine mucosal cytology before surgery. Surgeons should not hesitate to perform laparotomy when necessary to identify and appropriately treat patients, even if abnormalities are not detected in the preoperative examination.

A case of a mucin-producing bile duct tumor diagnosed over the course of 6 years.

We report a case of a mucin-producing intraductal papillary neoplasm of the intrahepatic bile duct (M-IPNB) diagnosed over a period of 6 years. A 64-year-old man underwent follow-up evaluations for an abdominal aortic aneurysm at our hospital. In 2009, a computed tomography (CT) scan revealed a simple hepatic cyst in segment 3 of the liver. Annual CT scans initially showed almost no change in the size or shape of the cyst. The cystic lesion, which measured 5 cm in 2014, had increased to 11 cm by 2015, and a solid component was detected within the cyst. A biliary cystic tumor was suspected and we performed a left lateral hepatectomy. Pathological examination showed that the papillary lesion in the cyst included adenocarcinoma and adenoma components. We diagnosed M-IPNB in 2015. Identification of the solid component of the cyst, as well as an increase in cyst diameter in the image analyses, was critical for diagnosis of M-IPNB.

Extrauterine Spread, Adjuvant Treatment, and Prognosis in Noninvasive Uterine Papillary Serous Carcinoma of the Endometrium: A Retrospective Multicenter Study.

OBJECTIVES: The aim of this retrospective multicenter study was to investigate the frequency of extrauterine metastasis and to evaluate the importance of surgical staging and adjuvant treatment among patients with noninvasive uterine papillary serous carcinoma (UPSC) of the endometrium. MATERIALS AND METHODS: A multicenter, retrospective department database review was performed to identify patients with UPSC of the endometrium who underwent surgical staging between 2000 and 2015 at 4 Gynecologic Oncology Centers in Turkey. Demographic, clinicopathological, and survival data were collected. RESULTS: A total of 182 patients with primary UPSC of the endometrium were identified. Of these, 33 (18.1%) had tumors limited to the endometrium with no myometrial invasion. Twenty (60.6%) of these 33 patients had no extrauterine involvement and International Federation of Gynecology and Obstetrics 2009 stage 1A disease was diagnosed after complete staging. The remaining 13 (39.4%) patients had disease beyond the uterine corpus including 5 with omental, 3 with adnexal, 1 with cervical stromal involvement, 1 with disease in the pelvic lymph nodes, and 1 with isolated para-aortic lymph node metastasis. Two patients had metastases in more than one location including omentum/adnexa/pelvic-para-aortic lymph nodes and omentum/pelvic-para-aortic lymph nodes, respectively. Of the 20 patients with disease confined to the endometrium, 6 (30%) patients received adjuvant treatment. CONCLUSIONS: Noninvasive UPSC has a high tendency for extrauterine spread and omentum is the most commonly involved location. Therefore, comprehensive surgical staging including omentectomy and pelvic-para-aortic lymph node dissection is mandatory in this group of patients. Risk of extrauterine spread is significantly associated with the presence of lymphovascular space invasion, elevated preoperative CA 125 levels, and positive peritoneal cytology. Adjuvant therapy for women with endometrium-confined disease improves neither progression-free survival nor overall survival.

Syringocystadenocarcinoma Papilliferum In Situ, a Variant of Cutaneous Adenocarcinoma In Situ: A Case Report With Literature Review.

Syringocystadenocarcinoma papilliferum in situ, a variant of cutaneous adenocarcinoma in situ, is extremely rare. Only 9 cases have been published to date with 2 cases demonstrating pagetoid epidermal involvement. In this study, we report a case of syringocystadenocarcinoma papilliferum in situ with pagetoid epidermal involvement arising from a long-standing nevus sebaceus on the scalp of a 60-year-old woman.

Primary Peritoneal Papillary Serous Cystadenocarcinoma - A Rare Malignancy.

Primary peritoneal papillary serous carcinoma is a rare primary malignancy diffusively involving the peritoneum of abdomen and pelvis. Epithelial ovarian cancers and primary peritoneal cancers arise from the common germinal epithelium which develops from the coelomic epithelium. Due to a common embryonic origin of the ovary and the peritoneum, carcinoma of the ovary and primary peritoneal carcinoma have much histological similarity. However the incidence of Primary peritoneal serous carcinoma is considerably lower than that of epithelial ovarian cancer and is mostly seen in elderly women. Early stages of this disease may be asymptomatic; symptoms of the advance stages of the disease include abdominal distention, abdominal lump, non-specific abdominal pain, vomiting and dyspnoea all as a result of massive ascites. Patients diagnosed with primary peritoneal papillary serous carcinoma are treated using the same staging, surgical and chemotherapeutic approach as epithelial ovarian cancer because of the similarities in biological behavior.

Ovarian serous borderline tumors with noninvasive and invasive peritoneal implants: A case report each.

Serous borderline tumors (SBT) are defined by the World Health Organization (WHO) as serous neoplasms that show epithelial proliferation greater than that seen in serous cystadenomas, as evidenced by cellular stratification, cytologic atypicality, and epithelial tufting, but which exhibit no evidence of "destructive stromal" invasion and can show extra-ovarian implants. Characterization of invasive peritoneal implants from patients with noninvasive serous ovarian tumors has important prognostic and treatment implications. Peritoneal implants have been classified as either noninvasive or invasive based on their histopathologic appearance. Three criteria were applied for the diagnosis of "invasive" implants: Invasion of underlying normal tissue, micropapillary architecture, and solid epithelial nests surrounded by clefts. We encountered two cases of unilateral ovarian serous borderline tumors with non-invasive peritoneal implants in a 43-year-old female, and invasive peritoneal implants in 76-year-old female.

Uterine papillary serous carcinoma pre-operatively diagnosed as endometrioid carcinoma: Is omentectomy necessary?

BACKGROUND: Uterine papillary serous carcinoma (UPSC) is a relatively rare but aggressive uterine malignancy comprising approximately 10% of endometrial cancers. Many women pre-operatively misdiagnosed as having endometrioid carcinoma have ultimately UPSC on final pathology. These women receive inadequate surgical staging without omentectomy. AIM: To assess the value of omentectomy on disease-free interval and overall survival in women with UPSC who had an initial diagnosis of endometrioid carcinoma. METHODS: This retrospective study included all women treated for the final diagnosis of UPSC in our centre from January 2007 to December 2012. Data regarding patient demographics, staging procedures, histology results, adjuvant therapy and follow-up outcomes were recorded. RESULTS: Of the 52 women with a final diagnosis of UPSC, more than 45% had an initial diagnosis of endometrioid carcinoma. All women underwent hysterectomy and removal of the adnexa. Lymph node evaluation was performed in 75% of women. Omentectomy was performed in 30/52 women (58%). Of those, three women (10%) had omental involvement. Mean disease-free interval with omentectomy was 24.5 months versus 30.5 months without (P = 0.29). Mean overall survival was 33 months with an omentectomy and 29 months without (P = 0.32). Recurrence patterns did not differ between groups. CONCLUSION: Women diagnosed pre-operatively with endometrioid carcinoma and eventually found to have UPSC can expect no change in prognosis despite not having undertaken a full staging procedure. Repeat surgery for omentectomy is probably of no benefit.

The incidence of pelvic and para-aortic lymph node metastasis in uterine papillary serous and clear cell carcinoma according to the SEER registry.

OBJECTIVE: In this study we utilized the Surveillance, Epidemiology and End-Results (SEER) registry to identify risk factors for lymphatic spread and determine the incidence of pelvic and para-aortic lymph node metastases in patients with uterine papillary serous carcinoma (UPSC) and uterine clear cell carcinoma (UCCC) who underwent complete surgical staging and lymph node dissection. METHODS: Nine hundred seventy-two eligible patients diagnosed between 1998 to 2009 with International Federation of Gynecology and Obstetrics (FIGO) 1988 stage IA-IVA UPSC (n=685) or UCCC (n=287) were identified for analysis. Binomial logistic regression was used to determine risk factors for lymph node metastasis, with the incidence of pelvic and para-aortic lymph node metastases reported for each FIGO primary tumor stage. The Cox proportional hazards regression model was used to determine factors associated with overall survival. RESULTS: FIGO primary tumor stage was the only independent risk factor for lymph node metastasis (p<0.01). The incidence of pelvis-only and para-aortic lymph node involvement according to the FIGO primary tumor stage were as follows: IA (2.3%/3.8%), IB (7.5%/5.2%), IC (22.5%/16.9%), IIA (20.8%/13.2%), IIB (25.7%/14.9%), and III/IV (25.7%/24.3%). Prognostic factors for overall survival included lymph node involvement (hazard ratio [HR], 1.42; 95% confidence interval [CI], 1.09 to 1.85; p<0.01), patient age >60 years (HR, 1.70; 95% CI, 1.21 to 2.41; p<0.01), and advanced FIGO primary tumor stage (p<0.01). Tumor grade, histologic subtype, and patient race did not predict for either lymph node metastasis or overall survival. CONCLUSION: There is a high incidence of both pelvic and para-aortic lymph node metastases for FIGO stages IC and above uterine papillary serous and clear cell carcinomas, suggesting a potential role for lymph node-directed therapy for these patients.

Identification of a preoperative predictive factor for lymph node metastasis in uterine papillary serous carcinoma: long-term results from a single institution.

OBJECTIVE: The purpose of this study was to identify preoperative clinicopathological predictive factors for lymph node (LN) metastasis in women diagnosed with uterine papillary serous carcinoma (UPSC). METHODS: Women diagnosed with UPSC in our institution from 1997 to 2012 were identified. All patients underwent hysterectomy and bilateral salpingo-oophorectomy plus pelvic and/or para-aortic lymphadenectomy. The predictive values of the risk factors for LN metastasis were analyzed using χ and multivariate logistic regression analyses. RESULTS: A total of 94 patients met our study criteria. A CA-125 cutoff of 47.5 IU/mL on the receiver operating characteristic curve provided the best sensitivity and specificity (56.5% vs 90.1%, respectively) for LN metastasis prediction. The sensitivities and specificities of old age (≥60 years), body mass index of 25 kg/m or greater, deep myometrial invasion, tumor size greater than 2 cm, tumor size greater than 4 cm, preoperative CA-125 greater than 47.5 IU/mL, LN metastasis on imaging, and extrauterine spread on imaging for the presence of a positive LN were 39.1%, 34.8%, 30.4%, 34.8%, 21.7%, 56.5%, 43.5%, and 52.2%, and 52.1%, 45.1%, 78.9%, 57.7%, 83.1%, 90.1%, 93.0%, and 90.1%, respectively. Preoperative CA-125 (P < 0.001), LN metastasis on preoperative imaging (P < 0.001), and extrauterine spread on preoperative imaging (P = 0.009) were risk factors for LN metastasis on univariate analysis. Multivariate analysis revealed that preoperative CA-125 (P = 0.001) was the only independent risk factor for LN metastasis. CONCLUSIONS: Preoperative CA-125 is a preoperative predictive factor for LN metastasis in UPSC.

[ULTRASOUND CRITERIA OR THE OPERABILITY AND EFFICACY OF THE TREATMENT OF THE MALIGNANT EPITHELIAL OVARIAN TUMOURS].

In order to isolate the main sonographic criteria of ovarian cancer operability the dynamical U.S. examination was performed on 65 women with epithelial tumors of II-III stages before and during 5 years after treatment beginning, which included (in different combinations) cytoreductive surgery and neoadjuvant chemotherapy. Only total 14 (21.5%) relapses were revealed. The U.S. prognostic criteria of the ovarian cancer treatment efficacy with and without neoadjuvant chemotherapy were defined.

Paratesticular papillary serous cystadenocarcinoma: a rare entity in Indian population.

Ovarian-type surface epithelial carcinomas of testis and paratestis are very rare. These tumors develop from mesothelial inclusions or abnormalities in the development of coelomic epithelium. The diagnosis of these lesions can be difficult, both clinically and radiologically, as morphological, immunohistochemical and ultrastructural features overlap with serous tumors arising in the female genital tract. The pathologist should be aware of the histopathological features and immunostains which help in reaching definite diagnosis. To the best of our knowledge only one such case has been reported in Indian literature. We hereby report a case of young male with this unusual lesion diagnosed on histopathology.

[Predictors of recurrence and prognosis in patients with stage I and II endometrial carcinoma].

OBJECTIVE: To explore the high-risk clinicopathological features for the recurrence and prognosis of endometrial carcinoma diagnosed as International Federation of Gynecology and Obstetrics (FIGO) stagesIand II. METHODS: Three hundreds ninety-eight consecutive patients with clinical stage I, II endometrial adenocarcinoma underwent primary surgical therapy between Oct. 1990 to Oct. 2010 were studied retrospectively to analyze the correlation between clinicopathological risk factors and the disease recurrence or prognosis. RESULTS: Thirty-six patients (9.0%, 36/398) developed recurrence, 21 of them (58.3%, 21/36) relapsed within 5 years of treatment. The results shown that the disease recurrence were significantly associated with clinicopathological factors including: age [≤ 50 years versus > 50 years, 1.9% (2/103) versus 11.5% (34/295); P = 0.004];histologic types [adenocarcinoma versus serous papillary adenocarcinoma, 8.0% (27/338) versus 6/14; P = 0.000]; tumor grade [grade 1, 2 (7.4%, 25/338) versus grade 3 (17.9%, 10/56); P = 0.022]; depth of myometrial invasion [none (3.4%, 2/59) versus < 1/2 (7.5%, 18/240) versus ≥ 1/2 (16.2%, 16/99); P = 0.011];tumor size [ ≤ 2 cm (5.9%, 17/287) versus > 2 cm (17.1%, 19/111);P = 0.000]. peritoneal cytology [negative (6.9%, 22/317) versus positive (23.8%, 5/21); P = 0.019]; ER status [negative (20.8%, 11/53) versus positive (5.5%, 16/293); P = 0.000]; PR status [negative (30.0%, 15/50) versus positive ( 4.1%, 12/295); P = 0.031]. But lympho vascular space invasion (LVSI), p53 or PTEN status were not significant effect on recurrence and prognosis.Univariate analysis of survival indicated that age, histology, tumor grade, depth of myometrial invasion, tumor size, peritoneal cytology and PR status were correlated with overall survival (OS) and disease free survival (DFS; all P < 0.05), while ER status only impact on DFS (P < 0.05). CONCLUSIONS: The results showed that age, histologic type of carcinoma, myometrial invasion, tumor grade, peritoneal cytology, tumor size, ER and PR status are significant predictors for recurrence and prognosis of FIGO I, II stage endometrial carcinoma. Regular follow-up after treatment is essential for patients within 5 years.

The survival outcome and patterns of failure in node positive endometrial cancer patients treated with surgery and adjuvant radiotherapy with curative intent.

OBJECTIVE: The purpose of this study was to evaluate the patterns of failure, overall survival (OS), disease-free survival (DFS) and factors influencing outcome in endometrial cancer patients who presented with metastatic lymph nodes and were treated with curative intent. METHODS: One hundred and twenty-six patients treated between January 1996 to December 2008 with surgery and adjuvant radiotherapy were identified from our service's prospective database. Radiotherapy consisted of 45 Gy in 1.8 Gy fractions to the whole pelvis. The involved nodal sites were boosted to a total dose of 50.4 to 54 Gy. RESULTS: The 5-year OS rate was 61% and the 5-year DFS rate was 59%. Grade 3 endometrioid, serous, and clear cell histologies and involvement of upper para-aortic nodes had lower OS and DFS. The number of positive nodes did not influence survival. Among the histological groups, serous histology had the worst survival. Among the 54 patients relapsed, only three (6%) failed exclusively in the pelvis and the rest of the 94% failed in extrapelvic nodal or distant sites. Patients with grade 3 endometrioid, serous and clear cell histologies did not influence pelvic failure but had significant extrapelvic failures (p<0.001). CONCLUSION: Majority of node positive endometrial cancer patients fail at extrapelvic sites. The most important factors influencing survival and extrapelvic failure are grade 3 endometrioid, clear cell and serous histologies and involvement of upper para-aortic nodes.

Surgical resection and outcome of pancreatic cystic neoplasms in China: analysis of a 16-year experience from a single high-volume academic institution.

BACKGROUND: To investigate the clinicopathological features of surgically resected pancreatic cystic neoplasms (PCNs) at a single institution in China. METHODS: The medical charts of patients who operated in the Second Affiliated Hospital, Zhejiang University School of Medicine between 1 January 1997 and 30 June 2013, were pathologically shown to have PCNs. RESULTS: There was a reliable increase trend not just in the overall number of patients (3 to 75) but additionally in the number of incidentally diagnosed patients across the periods (33.3% to 48.0%). In 83 of 111 cases, preoperative diagnoses matched with pathology, whereas the remaining cases (16/28) were misdiagnosed as pancreatic cancer. The proportion of malignancy in mucin producing neoplasms was 24.3% (9 out of 37). Elevated serum carbohydrate antigen (CA19-9) or carcinoembryonic antigen (CEA) was independently associated with malignancy. The overall survival rate was 96.4%. CONCLUSIONS: The proportion of PCNs within this series differs with that revealed in Western countries. Appropriate preoperative differential diagnosing of PCNs remains challenging. It is strongly recommended that patients with elevated CA19-9 or CEA levels undergo surgical resection.

Papillary cystadenocarcinoma of the lacrimal gland after radiation for bilateral retinoblastoma.

The authors report a case of papillary cystadenocarcinoma of the lacrimal gland after irradiation for bilateral retinoblastoma. A 32-year-old man with a history of bilateral retinoblastoma, diagnosed shortly after birth, was treated with enucleation of the OS and a single session of radiation to the OD. Over 30 years later, he presented with an orbital mass of the right lacrimal gland that on biopsy demonstrated papillary cystadenocarcinoma.

Syringocystadenocarcinoma papilliferum with locoregional metastases.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare malignant counterpart of syringocystadenoma papilliferum (SCAP). To date, less than 30 cases have been reported, and of those, only three had locoregional metastases [1,2]. Herein, we report a case of a 42-year-old man with a rapidly growing nodule on his right parietal scalp ultimately diagnosed as SCACP arising in association with a preexisting SCAP and nevus sebaceus. This case differs from prior reports in the tumor's rapid growth and aggressive course with the development of locoregional metastases within 5-weeks from initial presentation.