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Indian Journal of Cancer 2021The primary retroperitoneal serous adenocarcinoma (PRSAC) is a rare malignant tumor of the retroperitoneum. It shares the same pathological and biological behavior with... (Review)
Review
The primary retroperitoneal serous adenocarcinoma (PRSAC) is a rare malignant tumor of the retroperitoneum. It shares the same pathological and biological behavior with ovarian serous carcinoma. Most of the cases develop as peritoneal adenocarcinoma and rarely occur in the retroperitoneum. It is reported as serous surface papillary carcinoma of the peritoneum and extraovarian peritoneal serous papillary carcinoma. We present a case of PRSAC in a 60-year-old woman. Only 11 cases of PRSAC have been reported from 1983 to 2019. Histopathological features with immunohistochemical expressions are important to diagnose PRSAC. The outcome and survival mainly depend on the possibility of surgical resection. Molecular genetics of PRSAC should also be studied in relation with its ovarian counterpart.
Topics: Cystadenocarcinoma, Serous; Female; Humans; Middle Aged; Ovarian Neoplasms; Prognosis; Retroperitoneal Neoplasms
PubMed: 33402586
DOI: 10.4103/ijc.IJC_528_19 -
International Journal of Gynaecology... Jul 2018To compare the outcome of patients with uterine papillary serous cancer (UPSC) carrying a BRCA mutation with that of patients with UPSC who are BRCA wild-type.
OBJECTIVE
To compare the outcome of patients with uterine papillary serous cancer (UPSC) carrying a BRCA mutation with that of patients with UPSC who are BRCA wild-type.
METHODS
The present retrospective, multicenter cohort study included women with UPSC who were diagnosed between January 1, 1993, and December 31, 2014, and were tested for the BRCA mutation at three Israeli medical centers. Data were collected from the medical records, and patient and tumor characteristics and disease outcomes were compared between BRCA mutation carriers and noncarriers. The primary outcome was overall survival.
RESULTS
In total, 14 BRCA mutation carriers and 50 noncarriers were included. Both groups had similar treatment modalities (P=0.530). A non-significant trend toward BRCA mutation carriers being diagnosed more frequently at an advanced stage compared with noncarriers was observed (P=0.090). Median overall survival (25 vs 37 months; P=0.442), progression-free survival (37 vs 29 months; P=0.536), and disease-specific survival (60 vs 39 months; P=0.316) were similar between the carrier and noncarrier groups.
CONCLUSIONS
Although not significant, BRCA mutation carriers tended to have more advanced disease at diagnosis. However, the survival was similar irrespective of the BRCA status in this small group. Further research is needed to confirm these findings in a larger cohort.
Topics: Aged; Aged, 80 and over; BRCA1 Protein; BRCA2 Protein; Cystadenocarcinoma, Serous; Disease-Free Survival; Endometrial Neoplasms; Female; Humans; Middle Aged; Mutation; Retrospective Studies; Uterine Neoplasms
PubMed: 29572834
DOI: 10.1002/ijgo.12486 -
Journal of Gynecologic Oncology May 2019To compare the survival outcomes of adjuvant radiotherapy and chemotherapy in women with uterine-confined endometrial cancer with uterine papillary serous carcinoma...
Survival outcomes of adjuvant radiotherapy and chemotherapy in women with stage I serous papillary and clear cell carcinoma of the endometrium: a Korean multicenter study.
OBJECTIVE
To compare the survival outcomes of adjuvant radiotherapy and chemotherapy in women with uterine-confined endometrial cancer with uterine papillary serous carcinoma (UPSC) or clear cell carcinoma (CCC).
METHODS
Medical records of 80 women who underwent surgical staging for endometrial cancer were retrospectively reviewed. Stage I UPSC and CCC were pathologically confirmed after surgery. Survival outcomes were compared between the adjuvant radiotherapy and chemotherapy groups.
RESULTS
Fifty-four (67.5%) and 26 (32.5%) women had UPSC and CCC, respectively. Adjuvant therapy was administered to 59/80 (73.8%) women (25 radiotherapy and 34 chemotherapy). High preoperative serum cancer antigen-125 level (25.1±20.2 vs. 11.5±6.5 IU/mL, p<0.001), open surgery (71.2% vs. 28.6%, p=0.001), myometrial invasion (MI) ≥1/2 (33.9% vs. 0, p=0.002), and lymphovascular space invasion (LVSI; 28.8% vs. 4.8%, p=0.023) were frequent in women who received adjuvant therapy compared to those who did not. However, the histologic type, MI ≥1/2, and LVSI did not differ between women who received adjuvant radiotherapy and those who received chemotherapy. The 5-year progression-free survival (78.9% vs. 80.1%, p>0.999) and overall survival (77.5% vs. 87.8%, p=0.373) rates were similar between the groups. Neither radiotherapy (hazard ratio [HR]=1.810; 95% confidence interval [CI]=0.297-11.027; p=0.520) nor chemotherapy (HR=1.638; 95% CI=0.288-9.321; p=0.578) after surgery was independently associated with disease recurrence.
CONCLUSION
Our findings showed similar survival outcomes for adjuvant radiotherapy and chemotherapy in stage I UPSC and CCC of the endometrium. Further large study with analysis stratified by MI or LVSI is required.
Topics: Adenocarcinoma, Clear Cell; Adult; Aged; Aged, 80 and over; Chemotherapy, Adjuvant; Combined Modality Therapy; Cystadenocarcinoma; Cystadenocarcinoma, Papillary; Cystadenocarcinoma, Serous; Endometrial Neoplasms; Female; Humans; Hysterectomy; Middle Aged; Neoplasm Staging; Radiotherapy, Adjuvant; Republic of Korea; Retrospective Studies; Survival Analysis; Treatment Outcome
PubMed: 30887761
DOI: 10.3802/jgo.2019.30.e44 -
Surgery Mar 2015Although surveillance guidelines for resected invasive mucinous neoplastic cysts are well-established, those for noninvasive cysts are not defined. We used our...
BACKGROUND
Although surveillance guidelines for resected invasive mucinous neoplastic cysts are well-established, those for noninvasive cysts are not defined. We used our experience with resected noninvasive mucinous neoplastic cysts to define recurrence rates and the optimal frequency of postoperative imaging follow-up.
METHODS
We reviewed the medical records of 134 patients with resected, pathologically confirmed noninvasive mucinous neoplasms between 2002 and 2012. Demographics, comorbidities, cyst characteristics, and recurrence were evaluated. Survival analysis was used to estimate the distribution of time to recurrence and regression models were used to investigate factors associated with recurrence.
RESULTS
Eighty-seven patients with intraductal papillary mucinous neoplasms (IPMNs) were compared with 47 patients with mucinous cystic neoplasms (MCNs). Those with MCNs were more often females (P = .001), significantly younger (P = .001), more symptomatic (P = .009), and had cysts more often located in the tail (P < .001). Median follow-up was 42 months. Recurrence rates for IPMNs were 0%, 5%, and 10% versus 0% for MCNs respectively at postoperative years 1, 2, and 3 (P = .014). On multivariable analysis, size >3 cm (P = .027), higher grade dysplasia (P = .043), and positive resection margins (P < .001) were significantly associated with recurrence.
CONCLUSION
Resected noninvasive IPMNs with moderate- or high-grade dysplasia and negative resection margins require imaging follow-up every 2 years, given the 16% overall recurrence rate. Although the follow-up interval for noninvasive, low-grade, dysplastic IPMNs with negative margins could be lengthened, all noninvasive IPMNs having positive margins require yearly follow-up at the minimum. Resected noninvasive MCNs--irrespective of grade and margin status--do not require surveillance, although the development of branch duct-IPMNs in the remnant pancreas can be investigated in the long term at the discretion of the provider.
Topics: Adenocarcinoma, Mucinous; Adult; Aged; Aged, 80 and over; Carcinoma, Pancreatic Ductal; Carcinoma, Papillary; Cystadenocarcinoma, Mucinous; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasm Recurrence, Local; Pancreatic Neoplasms; Tomography, X-Ray Computed
PubMed: 25596773
DOI: 10.1016/j.surg.2014.09.028 -
Oral Oncology Jul 2021A 72-year-old female was referred for diagnosis of a lesion located in the right buccal mucosa, with duration unknown. At intraoral examination, the lesion appeared as a...
A 72-year-old female was referred for diagnosis of a lesion located in the right buccal mucosa, with duration unknown. At intraoral examination, the lesion appeared as a well-delimited, mobile, and submucosal nodule. A benign mesenchymal neoplasm was the main hypothesis of diagnosis. Histopathological analysis revealed salivary gland neoplasm formed by atypical cells often arranged in microcystic structures, with frequent intraluminal papillary projections. The tumor cells presented positivity for CK7 and negativity for CK20. Based on these features, the diagnosis of cystadenocarcinoma was established. The patient was submitted to wide surgical resection. No recurrence was observed after 48 months. Although rare, cystadenocarcinoma should be considered in the differential diagnosis of oral submucosal nodules.
Topics: Aged; Cystadenocarcinoma; Diagnosis, Differential; Female; Humans; Mouth Mucosa; Salivary Gland Neoplasms; Salivary Glands
PubMed: 33958288
DOI: 10.1016/j.oraloncology.2021.105314 -
Indian Journal of Pathology &... 2016Papillary cystadenocarcinoma (PCAC) is a rare salivary gland tumor characterized by a predominantly cystic growth that often exhibits intraluminal papillary growth...
Papillary cystadenocarcinoma (PCAC) is a rare salivary gland tumor characterized by a predominantly cystic growth that often exhibits intraluminal papillary growth without specific histologic features of other cystic salivary gland tumors. The preoperative cytological diagnosis can pose a diagnostic challenge as it has to be differentiated from other cystic papillary tumors such as mucoepidermoid carcinoma, papillary cystic variant of acinic cell carcinoma, and low-grade cribriform CAC. It is considered to be a low-grade malignant salivary gland tumor with an indolent biological behavior. We report a case of PCAC of the parotid in a 55-year-old male diagnosed on fine needle aspiration cytology. Although it showed mild atypia cytologically, on excision tumor showed vascular and perineural invasion with regional node metastasis indicating a wider morphologic spectrum than what is described. This prompted us to write a case report describing the cytological and histological features of this rare tumor and also discuss the diagnostic challenges.
Topics: Biomarkers, Tumor; Biopsy, Fine-Needle; Cystadenocarcinoma, Papillary; Cytological Techniques; Histocytochemistry; Humans; Immunohistochemistry; Keratin-7; Male; Microscopy; Middle Aged; Mucins; Parotid Neoplasms
PubMed: 27510680
DOI: 10.4103/0377-4929.188114 -
International Journal of Surgery... Aug 2016The purpose of this study was to investigate the clinicopathological characteristics, treatment methods, and prognostic factors in women with uterine papillary serous...
Optimal cytoreduction, depth of myometrial invasion, and age are independent prognostic factors for survival in women with uterine papillary serous and clear cell carcinomas.
OBJECTIVE
The purpose of this study was to investigate the clinicopathological characteristics, treatment methods, and prognostic factors in women with uterine papillary serous carcinoma (UPSC) and uterine clear-cell carcinoma (UCCC).
STUDY DESIGN
All patients who had undergone surgery for UPCS and UCCC between January 1995 and December 2012 were retrospectively reviewed. Patients with missing data, who did not undergo surgical staging and patients with mixed tumor histology were excluded. Multivariate regression models were used to identify the risk factors for overall survival (OS) and progression-free survival (PFS).
RESULTS
A total of 49 UPSC and 22 UCCC women were included. The majority of the patients were at stage I [IA, 22 (31%) and IB, 18 (25.4%)]. Stages II, III, and IV were identified in 9 (12.7%), 13 (18.3%), and 9 (12.7%) of cases, respectively. Optimal cytoreduction was achieved in 71.8% of cases. Recurrences occurred in 16 patients (22.5%). The 5-year OS rates were 67% for UPSC; 76% for UCCC; 68% for both histology, respectively. Multivariate analysis pointed out that age>67 years (odds ratio (OR): 3.85, p = 0.009 and OR: 3.35, p = 0.014), >50% myometrial invasion (MI) (OR: 2.87, p = 0.037 and OR: 2.46, p = 0.046) and optimal cytoreduction (OR: 3.26, p = 0.006 and OR: 2.77, p = 0.015) were the independent prognostic factors for both PFS and OS.
CONCLUSIONS
Our study demonstrated that optimal cytoreduction, >50% MI, and age >67 years are the most significant factors affecting survival in women with UPSC and UCCC.
Topics: Adenocarcinoma, Clear Cell; Age Factors; Aged; Cystadenocarcinoma, Serous; Cytoreduction Surgical Procedures; Disease-Free Survival; Female; Humans; Middle Aged; Neoplasm Recurrence, Local; Neoplasm Staging; Prognosis; Retrospective Studies; Turkey; Uterine Neoplasms
PubMed: 27365052
DOI: 10.1016/j.ijsu.2016.06.041 -
International Journal of Gynecological... Jan 2016"Invasive micropapillary serous carcinoma" has been proposed as a synonym for low-grade serous carcinoma by some expert pathologists. In contrast, Singer and colleagues...
"Invasive micropapillary serous carcinoma" has been proposed as a synonym for low-grade serous carcinoma by some expert pathologists. In contrast, Singer and colleagues reported that some serous carcinomas with conspicuous invasive micropapillary pattern (SC-IMPs) can show high-grade nuclear atypia. However, the molecular features of such tumors have not been well documented. The aim of this study was to demonstrate and emphasize the fact that high-grade serous carcinoma confirmed by immunohistochemistry and molecular analysis can show conspicuous invasive micropapillary pattern. We selected 24 "SC-IMPs" and investigated: (1) their morphologic features; (2) the immunostaining pattern of p53 protein; and (3) KRAS/BRAF/TP53 gene mutations. The 24 SC-IMPs were subdivided into low-grade and high-grade tumors based primarily on the nuclear atypia, with the mitotic rate used as a secondary feature: low grade (n=5) and high grade (n=19). Low-grade SC-IMPs were characterized by low-mitotic activity, absence of abnormal mitosis, presence of serous borderline tumor, occasional BRAF mutation, and infrequent TP53 mutation. High-grade SC-IMPs were characterized by high-mitotic activity, presence of abnormal mitosis, conventional high-grade serous carcinoma, frequent TP53 mutation, and lack of KRAS/BRAF mutation. We demonstrated that high-grade serous carcinoma confirmed by aberrant p53 immunostaining and molecular analysis can show conspicuous invasive micropapillary pattern, validating Singer and colleague's report. Serous carcinoma with conspicuous invasive micropapillary pattern should not be readily regarded as low-grade serous carcinoma. Nuclear grade is the most important diagnostic feature in the SC-IMPs.
Topics: Adult; Aged; Carcinoma, Papillary; Cystadenocarcinoma, Serous; DNA Mutational Analysis; Female; Humans; Immunohistochemistry; Middle Aged; Mutation; Neoplasm Grading; Neoplasm Invasiveness; Ovarian Neoplasms; Peritoneal Neoplasms; Proto-Oncogene Proteins B-raf; Proto-Oncogene Proteins p21(ras); Tumor Suppressor Protein p53; Young Adult
PubMed: 26166721
DOI: 10.1097/PGP.0000000000000211 -
Oncogene Aug 2021Endometrial carcinoma (EC) is the fourth-most common cancer in women in the United States, and generally carries a favorable prognosis. However, about 10% of EC patients...
Endometrial carcinoma (EC) is the fourth-most common cancer in women in the United States, and generally carries a favorable prognosis. However, about 10% of EC patients have a rare and aggressive form, uterine serous papillary carcinoma (USPC), which carries a much higher mortality rate. The developmental transcription factor PAX8 is expressed in nearly 100% of USPCs. We show that PAX8 plays a critical antiapoptotic role in USPC and this role is established via transcriptional activation of two aberrant signaling pathways. First, PAX8 positively regulates mutated p53, and missense p53 mutations have an oncogenic gain of function effect. Second, PAX8 directly transcriptionally regulates p21, in a p53-independent manner, and p21 acquires a growth promoting role that is mediated via cytoplasmic localization of the protein. We propose that mutated p53 and cytoplasmic p21 can independently mediate the pro-proliferative role of PAX8 in USPC. In addition, we performed a genome-wide transcriptome analysis to detect pathways that are regulated by PAX8, and propose that metabolism and HIF-1alpha -related pathways are potential candidates for mediating the role of PAX8 in USPC. Taken together our findings demonstrate for the first time that PAX8 is an essential lineage marker in USPC, and suggest its mechanism of action.
Topics: Apoptosis; Cystadenocarcinoma, Serous; Endometrial Neoplasms; Female; Gene Expression Profiling; Humans; Oncogenes
PubMed: 34244607
DOI: 10.1038/s41388-021-01925-z -
Clinical calculator predictive of chemotherapy benefit in stage 1A uterine papillary serous cancers.Gynecologic Oncology Jan 2020Determine the utility of a clinical calculator to predict the benefit of chemotherapy in stage IA uterine papillary serous cancer (UPSC).
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.
Topics: Aged; Algorithms; Cystadenocarcinoma, Papillary; Cystadenocarcinoma, Serous; Female; Humans; Machine Learning; Neoplasm Staging; Nomograms; Predictive Value of Tests; Prognosis; Proportional Hazards Models; Reproducibility of Results; Uterine Neoplasms
PubMed: 31796203
DOI: 10.1016/j.ygyno.2019.10.017