Well-Differentiated Papillary Mesothelioma with Omental Calcifications: A Case Report and Review of the Literature.

BACKGROUND Omental calcifications of the peritoneum are typically small and asymptomatic. However, larger psammomatous bodies that cause symptoms such as abdominal pain and bloating are often associated with tumors such as primary serous papillary carcinoma, mesothelioma, or metastatic ovarian cancer. CASE REPORT We describe omental calcifications in a 68-year-old woman who had been asymptomatic for the last 10 years. The case details the histomorphologic features and immunohistochemical signature of a 4.0×3.5×1.0 cm mass consisting of mature adipose tissue that was surgically removed together with an 8.5×6.5×1.8 cm irregular intra-abdominal/mesenteric mass composed of yellow-red fatty tissue. Microscopic sections contained fat with variable clustered classic/psammomatous calcifications, some with a thin epithelioid periphery, in association with a very focal and subtle papillary surface epithelial/mesothelial proliferation. Tumor cell invasion was not observed during examination. Immunohistochemical staining showed that mesothelial cells in the mass were strongly positive for calretinin and focally positive for EMA, CK903, and vimentin. Strong nuclear positivity for PAX8 was also reported. Additional stains were added in response to this pattern, showing strong positivity for CK8, moderate positivity for BAP1, focal positivity for ER, minimal positivity for CD56, and negativity for CK5/6 and D2-40. Three possible explanations are suggested for the phenomenon observed in the pathology slides: reactive mesothelial hyperplasia, well-differentiated papillary mesothelioma, or serous papillary carcinoma of the peritoneum. CONCLUSIONS Findings suggest that these calcifications are a benign, reactive phenomenon, and that the abundance of psammoma bodies may be related to ongoing crops of papillary mesothelial hyperplasia or benign well-differentiated papillary mesothelioma.

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.

Prior breast cancer and tamoxifen exposure does not influence outcomes in women with uterine papillary serous carcinoma.

OBJECTIVES: To evaluate progression-free survival (PFS) and overall survival (OS) outcomes in women diagnosed with uterine papillary serous carcinoma (UPSC) who have had (UPSCBR+) or have not had (UPSCBR-) an antecedent history of breast cancer and to correlate their outcomes to prior tamoxifen exposure. METHODS: Data were collected for women diagnosed with UPSC at two academic institutions between January 1997 and July 2012. Patient demographics, tumor histology, stage, and treatments were recorded. Patients were divided into two groups: those with and without a personal history of breast cancer. Within the UPSCBR+ cohort, we identified those with a history of tamoxifen use. Cox regression modeling was used to explore associations between selected covariates of interest and the time-to-event outcomes of PFS and OS. RESULTS: Of 323 patients with UPSC, 46 (14%) were UPSCBR+. Of these, 15 (33%) had a history of tamoxifen use. UPSCBR+ patients were older than UPSCBR- (median years, 72 vs. 68, p=0.004). UPSCBR+ women showed no significant difference in PFS or OS compared to UPSCBR- (p=0.64 and p=0.73 respectively), even after controlling for age (p=0.15 and p=0.48 respectively). Within the UPSCBR+ cohort, there was no difference in PFS or OS with or without tamoxifen exposure (p=0.98 and p=0.94 respectively). CONCLUSIONS: There was no difference in PFS or OS between the UPSCBR+ and UPSCBR- cohorts. We did not demonstrate significant OS or PFS differences in women who took tamoxifen prior to their endometrial cancer diagnosis. These findings have implications for counseling, and should be encouraging to women who are facing their second cancer diagnosis.

"Simple Mucinous Cyst" of the Pancreas: A Clinicopathologic Analysis of 39 Examples of a Diagnostically Challenging Entity Distinct From Intraductal Papillary Mucinous Neoplasms and Mucinous Cystic Neoplasms.

Pancreatic cysts >1 cm lined by nonpapillary mucinous epithelium without ovarian-type stroma pose diagnostic challenges. The term "simple mucinous cyst" was recently proposed for this entity. Our goal was to determine the clinicopathologic characteristics of these cysts, as they have not been previously described. Of the 39 patients with pancreatic resections included in this study, the mean age was 65 years and the female-to-male ratio was 4:1. The characteristics of the cysts are as follows: 82% had elevated cyst fluid carcinoembryonic antigen levels, 67% were unilocular, 69% occurred in the body/tail, 92% did not communicate with pancreatic ducts, the mean size was 2.4 cm (range, 1.0 to 5.5 cm), the cyst contents tended to be serous (48%) or viscous (28%), all had a smooth lining (only 1 had focal excrescences) composed of bland columnar mucinous epithelium (low-grade dysplasia) in 92% with focal high-grade dysplasia in 8%, and 65% had degenerative changes (granulation-like tissue, hemorrhage, and myxoid stroma). The cyst lining was CK7+ and 97% had a MUC5AC+ and/or MUC6+ gastric phenotype; overt intestinal features were absent. In total, 55% of cysts tested (fluid and/or resections) harbored KRAS mutations. The term "simple mucinous cyst" is useful to apply to >1 cm mucinous cysts that do not have characteristic features of intraductal papillary mucinous neoplasms or mucinous cystic neoplasms. KRAS mutations can be detected in these typically bland cysts, and in rare instances, focal high-grade dysplasia may be present. Hence, these cysts should be viewed as neoplastic and treated similarly to other mucinous pancreatic cysts.

Cytomorphological findings and histological correlation of papillary cystadenocarcinoma of the parotid: Not always a low-grade tumor.

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.

Analysis of clinical and pathological characteristics, treatment methods, survival, and prognosis of uterine papillary serous carcinoma.

PURPOSE: Uterine papillary serous carcinoma (UPSC) is an atypical variant of endometrial carcinoma with a poor prognosis. It is commonly associated with an increased risk of extrauterine disease. The aim of this study was to investigate clinical and pathological characteristics, therapeutic methods, and prognostic factors in women with UPSC. METHODS: All patients who underwent surgery for UPSC at a single high-volume cancer center between January 1995 and December 2010 were retrospectively reviewed. Patients who did not undergo surgical staging and those with mixed tumor histology were excluded. Univariate and multivariate regression models were used to identify the risk factors for overall survival (OS) and progression-free survival (PFS). RESULTS: A total of 46 patients were included, the majority of whom having stage I disease (IA, 13 [28.2%] and IB, 12 [26.7%]). Stages II, III, and IV were identified in 5 (10.9%), 8 (17.4%), and 8 (17.4%) women, respectively. Optimal cytoreduction was obtained in 67.3% of patients. Recurrences developed in 8 (17.4%) patients. Multivariate analysis confirmed that lymphovascular space invasion (LVSI) (odds ratio [OR] 26.83, p = 0.003) was the only independent prognostic factor for OS, whereas LVSI and optimal cytoreduction were found to be independent prognostic factors for PFS (OR 6.91, p = 0.013 and OR 2.69, p = 0.037, respectively). The 5-year overall survival rate was 63%. CONCLUSIONS: Our study demonstrated that LVSI is the only independent prognostic factor for OS, whereas LVSI and optimal cytoreduction are independent prognostic factors for PFS in patients with UPSC.

The prognostic significance of hematological parameters in women with uterine serous papillary carcinoma (USPC).

OBJECTIVES: Preoperative hematologic parameters: thrombocytosis, leukocytosis and anemia have been demonstrated to be independent poor prognostic factors in ovarian and endometrial cancers. However, little is known about their relation to uterine serous papillary carcinoma (USPC). We evaluated several preoperative hematologic parameters and their association with clinicopathologic features, disease progression and overall survival in USPC patients. STUDY DESIGN: This was a retrospective cohort study reviewing charts of all patients with a histologic pure USPC at two gynecologic oncology centers from January 2000 through July 2012. All patients had comprehensive hematologic tests prior to primary surgical treatment and were exposed to the same adjuvant treatment protocol. RESULTS: The study included 56 patients, mean age at diagnosis 69.4±15. Six (11%) had platelet count above 400000 10(6)/L, of them four (66%) were dead at the end of follow up (HR=1.4, p=0.48; CI 95% 0.5-4.3). The mean hemoglobin level was 12.3g/dl, fibrinogen 437.5mg/dL and lymphocytes 2013/µL. None of these parameters was significantly associated with 5 year survival. Leukocyte and neutrophil levels were adversely associated with survival. Of 15 patients with leukocytosis >10000/µL, 67% were dead at the end of follow up (HR=3.98, p=0.003; CI 95% 1.6-9.8). Of the 27 with neutrophils above 65%, 14 (52%) were dead at the end of follow up (HR=3.1; p=0.015; CI 95% 1.2-7.8). CONCLUSIONS: In patients with USPC, leukocytosis and neutrophilia are associated with aggressive tumor biology, and may predict a lower 5 year survival.

Clinical Significance of Positive Pelvic Washings in Uterine Papillary Serous Carcinoma Confined to an Endometrial Polyp.

Uterine papillary serous carcinoma (UPSC) represents 10% of endometrial carcinomas. Significant number of patients initially present with extrauterine disease. The role of adjuvant treatment in low stage, especially polyp-confined UPSC is controversial. This multi-institutional study evaluated the significance of positive pelvic washing (PW) and adjuvant treatment on disease recurrence in a setting of endometrial polyp-confined UPSC. Surgical pathology files from 3 institutions were searched for cases of endometrial polyp-confined UPSC. Following histologic review, cases were clinically staged as Stage I, without myoinvasion or lymphovascular invasion. Clinicopathologic characteristics, results of PW, and type of adjuvant therapy were recorded. Statistical analysis using the Kaplan-Meier method for survival and Fisher exact test were performed. Thirty-three patients were included in the study. All patients were diagnosed with polyp-confined UPSC. The size of the polyp ranged from 0.3 to 4.3 cm. PW was positive for tumor cells in 8/33 (24%) patients. Twenty-two patients (66.6%) received some type of adjuvant treatment. Six patients (18%) developed recurrent disease. There was no significant difference in disease-free survival in the patients receiving adjuvant treatment versus not (P=0.375). However, there was significant association (P=0.0013) between positive PW and disease recurrence. Data are conflicting whether positive PW affects prognosis in low-stage endometrial carcinomas. Our study showed that in UPSC, malignant cells can be present in PW without lymphovascular invasion or myoinvasion and may have negative prognostic implication. Our data also reflect the controversies in the role of adjuvant treatment in endometrium-confined UPSC.

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.

Levatorplasty for Symptomatic Posterior Prolapse due to Recurrent Malignant Ascites.

BACKGROUND: Although infrequently described, massive ascites due to malignancy contributes to symptomatic pelvic organ prolapse. CASE: A 73-year-old woman with recurrent ovarian cancer and massive ascites underwent a levatorplasty for repair of posterior prolapse after failing conservative management. CONCLUSIONS: Management of patient with cancer with prolapse is complex. Patients with cancer with ascites also have pelvic organ prolapse, in addition to other, better described sequelae of increased intra-abdominal pressure. These patients should be treated specifically for prolapse, with therapy, including type of surgery, chosen with special consideration of their underlying disease.

[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.

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.

Case of uterine papillary serous carcinoma following tamoxifen treatment that could not be diagnosed during screening.

This case involved a 69-year-old woman who had been taking tamoxifen for 5 years after breast cancer surgery. She was referred to our clinic for endometrial cancer screening when tamoxifen was first prescribed. Subsequently, transvaginal ultrasonography and endometrial cytology were performed every 6 months. Despite these regular examinations, stage IVb papillary serous carcinoma was detected 8 months after the end of tamoxifen administration. Total abdominal hysterectomy was performed, but only a small polyp was seen upon macroscopic examination of the uterus. However, papillary serous carcinoma was found microscopically in almost all lymphovascular spaces in the uterus from the endometrium to the serosa. On the surface of the polyp, only endometrial intraepithelial carcinoma with positive immunostaining for p53 was detected. Chemotherapy, including a platinum compound, was administrated, but unfortunately it was ineffective and the patient died of her disease 14 months after the operation.

Syringocystadenocarcinoma papilliferum in situ: report of a case with late recurrence.

Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare cutaneous neoplasm with apocrine differentiation. Only 27 cases have been reported up-to-date, 8 of them described as carcinomas in situ. Two cases with local recurrence and 3 cases with regional lymph node metastases have been documented. The authors present the case of a 32-year-old female with a SCACP in situ on the scalp that recurred 8 years after the excision of the primary tumor. No SCACP with late recurrence have been previously reported. This case highlights the need for a long-term follow-up in patients with this type of carcinoma.

Photodynamic detection and management of intraperitoneal spreading of primary peritoneal papillary serous carcinoma in a man: report of a case.

As a peritoneal surface malignancy, primary peritoneal papillary serous carcinoma (PPPSC) almost always occurs in women. Our search of the literature found only two previous case reports of men with PPPSC, both with very short survival. We report the case of a 63-year-old man with PPPSC, treated effectively with cytoreductive surgery and docetaxel-based hyperthermic intraperitoneal chemotherapy following six cycles of docetaxel-based laparoscopic neoadjuvant intraperitoneal and cisplatin-based systemic chemotherapy. Furthermore, we detected intraoperative intraperitoneal spreading of the tumor after the oral administration of 5-amino levulinic acid (5-ALA). The patient remains in good health without ascites 18 months after his diagnosis. Thus, primary peritoneal papillary serous carcinoma should be managed by intraperitoneal chemotherapy combined with peritonectomy procedures. Moreover, the intraoperative detection of the intraperitoneal spreading of the tumor after administering oral 5-ALA shows that this is an exciting and promising diagnostic technique, which needs to be confirmed by further studies.

Solid and cystic papillary neoplasm of the pancreas in a 18-year-old female: a case report.

CONTEXT: Solid and cystic papillary neoplasm of the pancreas is an extremely rare neoplasm that mostly affects young females in the mean age of 25 years and accounts for about 0.2-2.7% of all pancreatic tumors. CASE REPORT: A 18-year-old female presented with progressively increasing mass in the left hypochondrium and epigastric regions and vague abdominal pain. There was no history of jaundice and vomiting. The mean diameter of the tumors was 17x24 cm. Preoperative core needle revealed solid and cystic papillary neoplasm. Distal pancreatectomy and splenectomy were performed. The patient did not receive adjuvant therapy and no tumor recurrence was detected in follow up. CONCLUSION: Solid and cystic papillary neoplasm may reach large dimensions with a benign behavior and is curable by surgical excision. Differential diagnosis from other tumors with aggressive behavior is therefore important.

Clear cell papillary cystadenocarcinoma of the epididymis: a case report and immunohistochemistry of markers for renal cell carcinoma.

Neoplasms of the epididymis are uncommon, and malignant tumors are extremely rare. We report a case of clear cell papillary cystadenocarcinoma of the epididymis presenting with a long history of painless scrotal mass on the left side. Immunohistochemical markers for clear cell renal cell carcinoma (RCC) were examined to distinguish between clear cell papillary cystadenocarcinoma of the epididymis and metastatic clear cell renal cell carcinoma. The present case was positive for cytokeratin-7, PAX2, vinculin, vimentin and carbonic anhydrase IX. Expression of CD10 was focally observed. In contrast, no immunoreactivities for α-methylacyl-CoA racemase, RCC marker, glutathione S-transferase α or C-KIT were detected. The immunophenotypic profile of clear cell papillary cystadenocarcinoma of the epididymis closely resembles that of clear cell papillary RCC, although the immunohistochemical markers tested in this study are useful to make a differential diagnosis between clear cell papillary cystadenocarcinoma of the epididymis and metastatic clear cell RCC.

The clinical relevance of rising CA-125 levels within the normal range in patients with uterine papillary serous cancer.

The utility of cancer antigen 125 (CA-125) levels as an adjunct method of monitoring patients with uterine papillary serous carcinoma (UPSC) or endometrial serous carcinoma after surgery and adjuvant treatment has been reported. Our goal was to determine the significance of rising CA-125 levels within the normal range in these patients in the posttreatment surveillance setting. All patients with UPSC who underwent surgical staging and had preoperative CA-125 measurement from 1999 to 2008 were included in this analysis. Information was extracted from records to assess the changes in CA-125 values with clinical and/or radiographic detection of recurrence. Of the 56 evaluable patients, 23 (41%) recurred. Of the 23 patients that recurred, 11 had serial CA-125 levels measured in remission. Elevated CA-125 levels at diagnosis were significantly associated with disease recurrence and advanced stage (P = .01, P = .001, respectively). The rise in CA-125 by 10 U/mL in the normal range and ≥ 15 U/mL were associated with disease recurrence (P < .001, P < .001, respectively). In multivariate analysis, only CA-125 level ≥ 15 U/mL was significantly associated with worse progression-free survival. In this small cohort of patients with recurrent UPSC after remission, surveillance of CA-125 levels may have a role in disease surveillance and management.