Papillary cystadenocarcinoma of the parotid gland: a rare sub-variant of salivary gland adenocarcinoma

Papillary cystadenocarcinoma of the salivary gland is a very rare malignant neoplasm accounting for only 2% of all salivary gland lesions. In 1991 it was first included as a separate entity in the World Health Organization (WHO) classification of salivary gland tumors and in 2017 WHO Classification, the tumor was clubbed as a sub-variant of adenocarcinoma, not otherwise specified. It most commonly occurs in the major salivary glands. Herein we report a case of salivary papillary cystadenocarcinoma in a 54-year-old female, who presented with rapid enlargement of the right parotid swelling. Based on radiology and fine-needle aspiration cytology, a working diagnosis of the malignant tumor involving the superficial lobe of the right parotid gland was made. In view of the malignant nature of the swelling, superficial parotidectomy was done. The histopathology and immunohistochemistry of the mass confirmed the diagnosis of papillary cystadenocarcinoma of the right parotid. With the revised 2017 WHO classification of salivary gland tumors, it is important to report all rare subtypes in order to understand their biology and behavior.

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

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 (p60 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.

An analysis of current treatment practice in uterine papillary serous and clear cell carcinoma at two high volume cancer centers / 부인종양

OBJECTIVE: Despite the rarity of uterine papillary serous carcinoma (UPSC) and uterine clear cell carcinoma (UCCC), they contribute disproportionately to endometrial cancer deaths. Sufficient clinical information regarding treatment and prognosis is lacking. The aim of this study is to evaluate treatment outcomes in a rare cancer cohort based on the experience at two tertiary care cancer centers. METHODS: Clinicopathologic data were retrospectively collected on 279 patients with UPSC and UCCC treated between 1995 to 2011. Mode of surgery, use of adjuvant treatment, and dissection of paraaoritc lymph nodes were evaluated for their association with overall survival (OS) and progression-free survival (PFS). RESULTS: 40.9% of patients presented with stage I disease, 6.8% of patients presented with stage II disease and 52.3% of patients presented with stages III and IV. Median follow-up was 31 months (range, 1 to 194 months). OS and PFS at 5 years were 63.0% and 51.9%, respectively. OS and PFS were not affected by mode of surgery (open vs. robotic approach; OS: hazard ratio [HR], 0.68; 95% confidence interval [CI], 0.28 to 1.62; PFS: HR, 0.78; 95% CI, 0.40 to 1.56). Adjuvant treatment was associated with improved OS in stages IB-II (HR, 0.14; 95% CI, 0.02 to 0.78; p=0.026) but not in stage IA disease. There was no difference in OS or PFS based on the performance of a paraaoritc lymph node dissection. CONCLUSION: Minimally invasive surgical staging appears a reasonable strategy for patients with non-bulky UPSC and UCCC and was not associated with diminished survival. Adjuvant treatment improved 5-year survival in stages IB-II disease.

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

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.

A Case of Cystadenocarcinoma Misdiagnosed as a Benign Tumor

Cystadenocarcinoma of the salivary gland is a rare malignant tumor. It was first defined as papillary cystadenocarcinoma in the 1991 World Health Organization (WHO) classification, and it was reclassified as cystadenocarcinoma in the 2005 WHO classification. It is a low-grade neoplasm that features slow growing and predominantly cystic growth. We report a case of cystadenocarcinoma occurring on the parotid gland of a 61-year-old female patient presenting palpable mass on her left cheek. Preoperative examination may not reveal typical malignant characteristics. Such as in our case, the differential diagnosis between cystadenocarcinoma and benign lesion is difficult occasionally. We discuss the clinical and histopathological features of cystadenocarcinoma with the review of the literature.

Papillary cystadenocarcinoma of ovary presenting as obstructive jaundice: a rare presentation

Obstructive jaundice resulting from malignancy of gastrointestinal tract and hepatobiliary tract has been reported in various studies. Ovarian malignancy leading to obstructive jaundice due to portal lymphadenopathy is of rare occurrence. We report a case presented with obstructive jaundice and on further evaluation, found to have ovarian papillary cyst adenocarcinoma with secondaries at porta hepatis which was managed successfully by neoadjuvant chemotherapy followed by cytoreductive surgery

Collision of Three Histologically Distinct Endometrial Cancers of the Uterus

A collision tumor is defined by the presence of two separate masses in one organ, which are pathologically distinct. We described a 70-yr-old patient who complained of abnormal vaginal bleeding with a collision tumor of the uterine corpus. The patient received total hysterectomy, bilateral salphingo-oophorectomy, bilateral pelvic-paraaortic lymphadenectomy, omentectomy, and intraperitoneal chemotherapy. The uterine corpus revealed three separate masses, which were located at the fundus, anterior and posterior wall. Each tumor revealed three pathologically different components, which were malignant mixed mullerian tumor, papillary serous carcinoma, and endometrioid adenocarcinoma. Among these components, only the papillary serous carcinoma component invaded the underlying myometrium and metastasized to the regional lymph node. Adjuvant chemotherapy and radiation therapy were performed. The patient is still alive and has been healthy for the last 8 yr. We have reviewed previously reported cases of collision tumors which have occurred in the uterine corpus.

Clinical Approach to Incidental Pancreatic Cystic Lesions / 대한소화기학회지

Cystic lesions of the pancreas are being incidentally recognized with increasing frequency and become a common finding in clinical practice. Despite of recent remarkable advances of radiological and endoscopic assessment and a better understanding of natural history of certain subgroups of cystic lesions, differentiating among lesions and making an optimal management plan is still challenging. A multimodal approach should be performed to evaluate incidentally detected cystic lesions. Emerging evidence supports selective nonoperative management for the majority of patients with cystic lesions, but, for those in whom a suspicion of malignancy remains, surgery is indicated. Concerning long-term follow-up, there is limited data to support the ideal modality, intensity, and duration. Therefore, evidence-based guidelines for the diagnosis, management, and follow-up of cystic lesions of the pancreas should be established.

Advanced primary peritoneal carcinoma: clinicopathological and prognostic factor analyses / 浙江大学学报（英文版）（B辑：生物医学和生物技术）

<p><b>OBJECTIVE</b>To investigate the factors favoring a positive prognosis for advanced primary peritoneal carcinoma (PPC).</p><p><b>METHODS</b>Twenty-four cases meeting the criteria for PPC were analyzed retrospectively for the clinicopathologic profiles. Immunohistochemistry was used to determine the expressions of p53, Top2alpha, Ki-67 and Her-2/neu. Then all these clinicopathological factors and molecular markers were correlated with the prognosis.</p><p><b>RESULTS</b>There were 15 cases of primary peritoneal serous papillary carcinoma (PPSPC), 6 cases of mixed epithelial carcinoma (MEC) and 3 cases of malignant mixed Mullerian tumor (MMMT). All patients underwent cytoreductive surgery with optimal debulking achieved in 3 cases. Among those receiving first-line chemotherapy, 13 patients received the TP regimen (paclitaxel-cisplatin or carboplatin) and 7 patients received the PAC regimen (cisplatin-doxorubicin-cyclophosphamide). The median overall survival of all patients was 42 months, while the breakdown for survival time for patients with PPSPC, MMT and MEC was 44, 13 and 19 months, respectively. The expressions of p53, Top2alpha and Ki-67 were all demonstrated in 11 cases respectively. None showed the expression of Her-2/neu. There were significant differences in the median survival between patients with PPSPC and those with MMMT (44 months vs 13 months, P<0.05), also between patients receiving TP combination and those receiving the PAC regimen (75 months vs 28 months, P<0.05). Another significant difference in the median progression-free survival (PFS) was identified between patients with positive p53 immunostaining and those with negative p53 immunostaining (15 months vs 47 months, P<0.05), whereas age, menopausal status, residual tumor size and the other molecular factors did not significantly impact survival.</p><p><b>CONCLUSION</b>Patients with PPC should be treated with a comprehensive management plan including appropriate cytoreductive surgery and responsive chemotherapy. Overestimating an optimal debulking surgery may not benefit survival. The pathologic subtype, chemotherapy regimen and p53 overexpression were significant prognostic factors.</p>

Impact of neoadjuvant chemotherapy on the survival of patients with stage IIIc and IV epithelial ovarian cancer / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To compare the survival of patients with stage IIc or IV epithelial ovarian cancer treated either with neoadjuvant chemotherapy (NAC) followed by cytoreductive surgery or primary cytoreductive surgery (PCS) followed by adjuvant chemotherapy.</p><p><b>METHODS</b>The clinical and pathological data of 160 patients with stage IIIc or IV epithelial ovarian cancer diagnosed pathologically between 1997 and 2005 were retrospectively reviewed. Forty-two patients were treated with NAC followed by cytoreductive surgery (NAC group) and 118 patients with PCS followed by adjuvant chemotherapy (PCA group).</p><p><b>RESULTS</b>The overall response rate of NAC group was 69.1%. No significant difference was observed between the NAC group and PCS group in operating time, intra-operative blood loss and units of blood-transfusion (P > 0.05). Optimal cytoreductive surgery was performed in 88.1% of NAC group versus in 71.2% of PCS group (P < 0.05). In those who had optimal cytoreductive surgery, the recurrent rate was 43.2% in NAC group versus 56.0% in PCS group without significant difference between two groups (P > 0.05). The disease-free survival and progression-free survival was 7 and 8 months in NAC group, which were significantly shorter than 13 and 18 months in PCS group (P < 0.05), however, the median overall survival (OS) was 34 months in NAC group versus 43 months in PCS group without significant difference (P > 0.05). In the patients with optimal cytoreductive surgery, it was 34 months in NAC group versus 48 months in PCS group without significant difference either between two groups (P > 0.05).</p><p><b>CONCLUSION</b>Neoadjuvant chemotherapy followed by cytoreductive surgery can improve the rate of optimal cytoreductive surgery for the patients with stage IIIc or IVepithelial ovarian cancer, but this regimen may neither reduce the recurrent rate nor prolong the survival when compared with the patients treated with primary cytoreductive surgery followed by adjuvant chemotherapy.</p>

Tumor ovárico de bajo potencial maligno (borderline): patrón seroso micropapilar / Ovarian serous borderline tumors with micropapillary patterns

Se describen los tumores de bajo potencial maligno (borderline), su importancia clínica y la conducta terapéutica recomendada a partir de los conocimientos actuales. Se destaca la existencia de la variedad micropapilar la que según algunos autores presenta una mayor frecuencia de implantes invasivos y de recurrencias a corto y mediano plazo, lo que requeriría una conducta más activa. Se describe la importancia de los implantes invasivos y la microinvasión. Se presentan dos casos clínicos con su respectiva histología.

The tumours of low malignant potential or borderline, their clinical importance and the recommended therapeutic approach from the present knowledge are described. The existence of a micropapilar variety according to some authors, presents a greater frequency of invasive implant and recurrences to short and medium term, which would require a more active conduct. The importance of invasive implant and the microinvasión is described. Two clinical cases with their respective histology are presented.

Report of a case of Papillary cystic solid tumor of pancreas

Papillary cystic tumor of pancreas is a rare neoplasm which principally affects young women. It has a good prognosis and has a low malignant potential. Because of its similarity to other cystic pancreatic lesions, preoperative diagnosis is difficult to make. Our patient was a 19 years old girl who presented with an abdominal mass. Definitive diagnosis was not made preoperatively. The diagnosis of papillary cystic neoplasm was made through histopathological examination of the specimen after surgery. The patient has had no evidence of recurrence during the last six months. This rare tumor may be symptomless or may present with vague symptoms such as feeling of abdominal heaviness. Complete removal of the tumor will culminate in a low recurrence rate

Analysis of therapeutic result and prognostic factor in primary fallopian tube carcinoma / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To investigate the impact of treatment modality and clinicopathologic profile on prognosis in primary fallopian tube carcinoma.</p><p><b>METHODS</b>The data of 64 cases with primary fallopian tube carcinoma treated between January 1991 and June 2006 were analyzed. The clinicopathological data were retrospectively analyzed.</p><p><b>RESULTS</b>The overall 5-year survival rate of this series was 56.3%. The overall 3- and 5-year survival rate was 84.6% and 65.4% in surgical staging group versus 58.3% and 33.3% in no surgical staging group with a significant difference between two groups (P = 0.0429; P = 0.043), which was 89.5% and 68.4% in optimal cytoreduction group versus 66.7% and 41.7% in suboptimal cytoreduction group (P = 0.0466; P = 0.0444). However, there was no significant difference in 3-year and 5-year survival rate between the group with pelvic lymphadenectomy and the group without (84.2% vs. 69.2%, P = 0.4667; 63.1% vs. 53.8%, P = 0.459), and also between the group treated using CAP/CP regimen and the group by TP regimen for chemotherapy (81.8% vs. 80.0%, P = 0.8946; 59.1% vs. 60.0% P = 0.9582). It was found that the 5-year survival was correlated with FIGO stage (III-IV vs. I - II, P = 0.0197), differentiation grade (G3 vs. G1 + G2, P = 0.003), pathologic type (other type vs. serous, P = 0.0494), lymph nodes status (positive vs. negative, P = 0.0295).</p><p><b>CONCLUSION</b>Surgical staging, optimal cytoreduction, differentiation grade, pathologic type, lymph node status are important factors influencing the 5-year survival in primary fallopian tube carcinoma. Pelvic lymphadenectomy is necessary and feasible to perform during the procedure of surgical staging and cytoreduction. CAP/CP and TP regiment are similarly effective in adjuvant chemotherapy for primary fallopian tube carcinoma.</p>

Syringocystadenocarcinoma Papilliferum: A Case Report

Syringocystadenocarcinoma papilliferum (SCACP) is a rare form of adenocarcinoma of the skin. This is the malignant counterpart of syringocystadenoma papilliferum (SCAP) and usually develops on the scalp in a long-standing lesion identified clinically as SCAP. We describe a 65-yr-old Korean man with a nodule on the right supra-pubic area with a 2-yr duration. Histologically this tumor had a similar overall configuration as in SCAP, but the tumor was asymmetric and poorly circumscribed, extending into the deep dermis and showed cytologic atypia. The tumor cells showed positive reaction to GCDFP-15, but negative reaction to CEA and HMFG-1. We established the diagnosis of SCACP in the patient, and a wide excision was performed to remove the tumor. The patient has been well without relapse or metastasis for 2 yr.

A Case of Papillary Cystadenocarcinoma Arising from Submandibular Gland / 대한이비인후과학회지

Papillary cystadenocarcinoma originated from salivary gland is a lower grade neoplasm that rises most commonly in major salivary glands, although involvement of lip, palate, buccal mucosa and tongue has also been described. Papillary cystadenocarcinoma was first defined in 1991 by WHO. Until then, it was classified as an atypical type of adenocarcinoma. We experienced a case of papillary cystadenocarcinoma of the submandibular gland, so we report this case with a review of the literature.

Papillary cystadenocarcinoma in a Müllerian duct cyst: report of a case with literature review / 中华男科学杂志

<p><b>OBJECTIVE</b>To study the diagnosis and treatment of Müllerian duct cysts and their involvement with malignancy.</p><p><b>METHODS</b>A 44-year-old male patient with papillary cystadenocarcinoma involving a Müllerian duct cyst was presented. The presentation treatment, and pathological and radiological appearances were retrospectively analysed and discussed with literature review. The main manifestation was intermittent episode of hemospermia accompanying terminal hematuria and infertility for 15 years. Final diagnosis was determined by the findings of transrectal ultrasound scan, CT scan, MRI imaging, cystoscopic examination and biopsy.</p><p><b>RESULTS</b>Exploratory laparotomy was performed through a suprapubic retrovesical approach. The finding that a duct-like wedge of tumor tissue passed through the prostate near cyst neck to the posterior urethra without affecting the adjacent prostatic tissue during tylectomy confirmed that it arises from Müllerian duct system. Pathohistologic examination disclosed a papillary cystadenocarcinoma and it infiltrated the wall of the cyst. Both seminal vesicles and ejaculatory duct had no carcinoma invasion.</p><p><b>CONCLUSION</b>Müllerian duct cyst involving with malignancy is exceedingly rare, the diagnosis is based on the findings of transrectal ultrasound scan, CT scan, MRI imaging, cystoscopic examination. The final diagnosis depends on the pathohistologic examination. Lumpectomy is effective and have a good outcome.</p>

Synchronous primary cancer of the endometrium and ovary / 中华肿瘤杂志

<p><b>UNLABELLED</b>To investigate the clinical and pathological characteristics, treatment, and The data of 12 patients prognosis of synchronous primary cancer of the endometrium and ovary. Methods with synchronous primary cancer of the endometrium and ovary were retrospectively reviewed . Results Eight patients had the same histological type of endometrioid carcinoma in both uterus and ovary, 4 patients had different histological types in uterus and ovary. Synchronous primary cancer of the endometrium and ovary was difficult to be dignosed preoperatively. All ovarian tumors were small with an average diameter of 7 cm. Infertility was common among these patients(40.7%). Most of them had early stage I lesion (66.7%). endometrioid carcinomas was the main pathologic type (66.7%). All patients were treated surgically followed by chemotherapy with a 3-year survival rate of 66.7% (8/12).</p><p><b>CONCLUSION</b>Synchronous primary endometrium and ovary cancer is a specific kind of tumor different from either the primary endometrium carcinoma or ovary carcinoma, and usually can be detected in early stage with a good prognosis.</p>