Primary Retroperitoneal Mucinous Cystadenocarcinoma during Long-term Administration of Infliximab for the Treatment of Crohn's Disease.

We herein report a rare case of primary retroperitoneal mucinous cystadenocarcinoma (PRMC) in a 60-year-old man. The patient, who had been treated with infliximab for Crohn's disease of the colon for 13 years, was referred to our hospital for lower back pain. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging revealed multiple cystic lesions in the right retroperitoneum, the calcification of the cyst, and bone lesions. Bone and CT-assisted biopsies of the retroperitoneal lesions revealed poorly differentiated adenocarcinoma. The patient was diagnosed with PRMC with bone metastases using immunohistochemical staining and positron emission tomography/CT.

[A Case of Giant Pancreatic Mucinous Cystic Neoplasm That Was Difficult to Preoperative Diagnosis].

A 43-year-old woman with about abdominal distension was referred to our hospital for a more detailed examination. Abdominal CT showed 27 cm-sized cystic lesion with the calcification along the partition wall and a nodular hyperplasia. We suspected pancreatic pseudocyst, primary retroperitoneal tumor and we performed tumorectomy. The resected specimen had a maximum diameter of 27 cm. The histopathological diagnosis was mucinous cystadenocarcinoma of the pancreas with ovarian-type stroma. The adjuvant chemotherapy treated with gemcitabine was selected for 3 courses. She continues to do well without any recurrences 7 months later.

Dramatic Response to Carboplatin Plus Paclitaxel in Pancreatic Mucinous Cystadenocarcinoma with Liver Metastasis.

Mucinous cystic neoplasm (MCN) of the pancreas is a rare cystic tumor occurring in the pancreatic body and tail in young to middle-aged women that is pathologically characterized by an ovarian-like stroma. Chemotherapy for recurrent/advanced pancreatic MCN has been based on chemotherapy regimens for pancreatic ductal adenocarcinoma, but the prognosis is poor. We herein report a 37-year-old woman with pancreatic mucinous cystadenocarcinoma with liver metastasis that responded dramatically to carboplatin plus paclitaxel therapy (CBDCA+PTX). CBDCA+PTX may be a treatment option for recurrent/advanced pancreatic MCN with an ovarian-like stroma.

A 35 Year Old Bangladeshi Lady with Hereditary Mucinous Ovarian Cancer, Complicated with Omental Metastasis.

Approximately 80% ovarian tumors are benign, and these arise mostly in young adult females. Malignant tumors are more prevalent in ageing women, between the ages of 45-65 years. Mucinous ovarian cancer represents about 5% of epithelial ovarian cancers (EOC). We have reported a case of mucinous cystadenocarcinoma in 35-year-old lady with metastasis to momentum. Imaging (Radiograph & CT scan) studies showed a large right sided pelvic mass with probable origin in the right ovary. Cancer antigen-125 was elevated, while carcinoembrionic antigen and alpha-fetoprotein were normal. Mutational profiles shown distinct finding, as KRAS mutations positive nevertheless p53 and BRCA mutations are absent. She had undergone total abdominal hysterectomy with bilateral salphingo-oopherectomy along with pelvic dissection for removal of lymph nodes at the age of 35. She was given 3 cycles of chemotherapy with cisplatin and paclitaxel. To the best of our knowledge, this is the one of the little cases of ovarian mucinous cystadenocarcinoma being reported at a relatively young age and the first case being reported from Bangladesh.

[A Case of Mucinous Cystoadenocarcinoma with Metastatic Liver Tumor in a Young Woman Lacking Specific Findings].

The patient was a 17-year-old woman. An abnormal urinalysis was observed, and abdominal echography showed a pancreatic cystic mass. At the first examination, computed tomography(CT)and endoscopic ultrasound(EUS)showed a unilocular cystic mass of 60mm in size in the pancreatic body and tail, and no malignant findings were observed. After 14 months, CT and positron emission tomography-computed tomography(PET-CT)showed that the cystic mass had a mural nodule with FDG accumulation, and presence of a tumor in hepatic S8 with FDG accumulation. Laparoscopic distal pancreatectomy and hepatic subsegmentectomy of S8 were performed for diagnostic and therapeutic purposes. The pathological diagnosis was mucinous cystadenocarcinoma(MCC)and metastatic liver cancer in the form of MCC. S-1 was administered after surgery. Nine months after resection, multiple metastatic liver tumors were found, and GEM plus nab-PTX was administered. After 2 courses of treatment, the patient is still alive without new lesions.

Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors: A Retrospective Multicenter Analysis.

OBJECTIVES: Two issues were put forth by clinicians in the management of the advanced stages of rare variants of pancreatic ductal adenocarcinoma and other exocrine histotypes with peculiar clinical and pathological features: Do chemotherapy regimens recommended in pancreatic ductal adenocarcinoma patients have a clinical activity in rare pancreatic tumors? Or should other chemotherapy combinations be considered in this subset of patients? METHODS: We conducted a multicenter retrospective study that collected data from 2005 to 2016 at 14 Italian cancer centers with the aim to evaluate tumor response and time to progression for first- and second-line and overall survival. RESULTS: Of approximately 4300 exocrine pancreatic cancer patients, 79 advanced cases affected by rare histological types were identified, with pancreatic acinar cell cancer (n = 23), pancreatic adenosquamous cancer (n = 16), and mucinous cystic neoplasm with an associated invasive mucinous cystadenocarcinoma (n = 15) most represented. Survival analyses for each subgroup in relation with the different chemotherapy regimens showed the lack of statistical significance correlations. CONCLUSIONS: Because of the lack of clinical trials in patients affected by these rare pancreatic histotypes, only their molecular classification would help clinicians in future therapeutic choice.

[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.

Management of a ruptured mucinous mesenteric cyst with hyperthermic intraperitoneal chemotherapy.

Mesenteric cysts are rare intra-abdominal cysts that are generally regarded as benign, and the incidence of malignancy is often cited to be 3%. The typical recommendation for treatment is complete excision to minimize recurrence. Excision can be performed laparoscopically, but this can lead to intra-abdominal dissemination of the cyst contents. There has been one case report describing the development of pseudomyxoma peritonei following rupture of a mesenteric cyst. We describe the treatment and outcome of a patient who underwent cytoreductive surgery with hyperthermic intraperitoneal chemotherapy for the treatment of an incompletely resected mucinous cystadenocarcinoma originating from the colonic mesentery.

Molecular abnormalities in ovarian carcinoma: clinical, morphological and therapeutic correlates.

The histopathological classification of ovarian surface epithelial carcinomas (referred to hereafter as 'ovarian carcinoma') has shifted over the past 10 years to reflect more clearly our understanding of molecular events during carcinogenesis. Ovarian carcinoma is no longer viewed as a single entity but as multiple disease processes, with each having different molecular pathways altered during oncogenesis, resulting in differences in clinical and pathological features, such as biomarker expression, pattern of spread and response to chemotherapy. There are five subtypes of ovarian carcinoma that are sufficiently distinct and well-characterized that they should be considered to be different diseases, i.e. high-grade serous, clear cell, endometrioid, mucinous and low-grade serous, from most to least common, respectively. This review summarizes the molecular abnormalities of these five ovarian carcinoma subtypes, relating them to clinical and pathological features.

Metastatic mucinous ovarian cancer and treatment decisions based on histology and molecular markers rather than the primary location.

Approximately 22,000 cases of ovarian cancer occur each year in the United States, and likely fewer than 2000 cases of mucinous ovarian cancers. Although 90% of patients with mucinous ovarian cancer present with stage I disease and have curative surgeries, advanced-stage disease is known to have a poor response to standard platinum- and taxane-based chemotherapy. Despite limited enthusiasm, standard chemotherapy is still recommended for most patients with advanced-stage mucinous malignancies of the ovary. This report presents an unusual case of a woman with HER2-positive metastatic mucinous carcinoma of the ovary treated with chemotherapy regimens typically used for colorectal malignancies, followed by epidermal growth factor receptor-targeted therapies.

Peritoneal pseudomyxoma arising from the urachus.

Pseudomyxoma peritonei (PMP) arising from urachal tumors is extremely rare. To our knowledge, natural history, tumor biological behaviour, morbidity, treatment, and prognosis of PMP arising from the urachus are determined by the associated PMP. Management of urachal tumors with associated PMP should be based on aggressive locorregional therapy with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy, similar to PMP arising from other origins.

[A case of mucinous cystadenocarcinoma of the appendix with abdominal wall abscess successfully treated by percutaneous drainage and systemic chemotherapy-a case report].

A 76-year-old woman was diagnosed of mucinous cystadenocarcinoma of the appendix. Since there was wide direct invasion into the right psoas muscle, she was judged as inoperable although she had no bowel obstruction. She was received FOLFIRI and bevacizumab treatment. After the first cycle, the tumor progressed rapidly and formed the abdominal wall abscess at the right groin. Since she had a fever and pain at the right groin and the abscess reached the hypodermic, we put a drainage tube into the abscess. The tube was placed, which made her symptoms improved markedly. We have been continuing with FOLFIRI treatment and drainage for 10 months without progressive disease. We report a rare case of the conservative therapy of mucinous cystadenocarcinoma of appendix with abdominal wall abscess.

Primary retroperitoneal mucinous cystadenocarcinoma: a case report and review of the literature.

A 50-year-old female complained of a painless abdominal distension. Histopathologic examination after cystectomy showed a primary poorly differentiated retroperitoneal mucinous cystadenocarcinoma with a sarcoma-like mural nodule. The patient subsequently underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy, appendectomy, omentectomy and lymphadenectomy. Adjuvant chemotherapy consisted of 6 times carboplatin (AUC 7) in monotherapy (every 4 weeks). Based on 49 cases of primary retroperitoneal mucinous cystadenocarcinoma, we discuss the histogenesis and we define the appropriate treatment.

Mucinous advanced epithelial ovarian carcinoma: clinical presentation and sensitivity to platinum-paclitaxel-based chemotherapy, the GINECO experience.

BACKGROUND: Advanced mucinous epithelial ovarian carcinoma (mEOC) has been associated with a worse prognosis than the more common serous epithelial ovarian carcinomas (sEOC), but it remains unclear whether this observation reflects a more aggressive clinical presentation and/or chemoresistance. PATIENTS AND METHODS: Data from four randomized phase III and one phase II advanced epithelial ovarian carcinoma (EOC) first-line clinical trials were retrospectively collected, yielding 1118 patients with advanced EOC (International Federation of Gynecology and Obstetrics stages IIB-IV), 85% of whom were treated with paclitaxel (Taxol)-carboplatin-based chemotherapy. RESULTS: Based on 786 patients with sEOC and 54 (5%) with mEOC, peritoneal carcinomatosis were more limited in mEOC, which was more frequently stages IIB-IIIB (32% versus 19%, P = 0.001) and had more frequently macroscopic complete resection after initial surgery (50% of stages II-III versus 30%, P = 0.02). In contrast, visceral metastases (stage IV) were more frequent in mEOC (30% versus 15%, P = 0.004). mEOC had a lower response rate to carboplatin-paclitaxel, and shorter progression-free and overall survival rates, for both stage IV and optimally debulked stages II-III patients. CONCLUSIONS: Advanced mEOC appears to be highly chemoresistant and complete resection of peritoneal metastases is unable to reverse its poor prognosis. New therapeutic options are needed.

[Expression of B7-H1 protein in human pancreatic carcinoma tissues and its clinical significance].

BACKGROUND AND OBJECTIVE: B7-H1, a member of B7 family, is expressed in tumor cells and has emerged as an important immune modulator capable of suppressing host immunity by inhibiting T cells function. This study was to probe into the correlation between the expression level of B7-H1 protein in pancreatic carcinoma tissues and clinicopathological characteristics and prognosis. METHODS: The expression of B7-H1 was measured in 40 cases of pancreatic carcinoma tissues and 10 cases of normal corresponding paracarcinoma tissues by immunohistochemistry. The relationship between the expression level of B7-H1 and clinicopathological characteristics and survival was analyzed. RESULTS: The positive rate of B7-H1 was significantly higher in the tumor tissues [45.00% (18/40)] than in the normal corresponding paracarcinoma tissues [0(0/10)] (P<0.05); moreover, B7-H1 expression was significantly associated with the staging of tumor and preoperative serum CA19-9 level (P<0.05). The multivariate cox proportional hazards regression analysis of prognostic factors for overall survival and relapse-free survival showed that the expression of B7-H1 was an independent factor for poor prognosis. CONCLUSION: B7-H1 protein was expressed in human pancreatic carcinoma tissues, and was associated with the prognosis.

[Analysis of treatment and prognosis of recurrent epithelial ovarian carcinoma].

OBJECTIVE: To discuss the prognostic factors of recurrent ovarian epithelial carcinoma and to analyze the curative effect of post-relapse treatment. METHODS: The clinical records of 293 patients with ovarian epithelial carcinoma were reviewed retrospectively. There were 199 recurrent cases during the following up. RESULTS: All the 199 patients received chemotherapy. And 173 patients only received chemotherapy. 16 patients received surgery and chemotherapy and the other 10 patients received radiotherapy and chemotherapy. 158 patients received platinum-based chemotherapy again and 41 patients received chemotherapy without platinum. The response rate of all the patients was 43.7% (87/199), the response rate of only chemotherapy was 39.9% (69/173), the response rate of surgery and chemotherapy was 75.0% (12/16), and the response rate of radiotherapy and chemotherapy was 60.0% (6/10). The patients were divided into four groups according to the progression free interval (PFI). The response rates in groups that PFI < or = 6 months, 7 - 12 months, 13 - 24 months and > 24 months were 5.1%, 47.2%, 82.1% and 96.0%, respectively. The median survival time in the 16 patients received second cyto-reductive surgery was 41 months. Multivariate analysis revealed that PFI was significantly correlated with prognosis of recurrent ovarian epithelial carcinoma (OR = 0.589, P = 0.021). CONCLUSION: PFI is an individual prognostic factor for survival of recurrent ovarian epithelial carcinoma. PFI is significantly associated with the response rate of chemotherapy. Optimal secondary cytoreductive surgery may improve the overall survival of recurrent patients. The response rate of paclitaxel plus platinum chemotherapy in platinum-sensitive patients is higher than that of other platinum-based chemotherapy.

[Profiling of serum tumor markers in patients with epithelial ovarian carcinoma].

OBJECTIVE: To investigate whether the change of serum tumor markers profile after chemotherapy in epithelial ovarian carcinoma and evaluate the clinical significance. METHODS: The levels of CA(125), CA(19-9) and CP2 before and after initial surgery, during primary chemotherapy and follow-up were serially measured and analyzed retrospectively in 28 cases of recurrent epithelial ovarian carcinoma patients and 20 cases of primary chemo-resistant ovarian carcinoma patients from Jan 1999 to July 2007. According to whether the change of serum tumor markers profile, all the patients were divided into two groups: marker changed-group and marker un-changed group. The average follow up period was 25 months. RESULTS: (1) The changes of tumor marker profile were included the number and(or) types of markers, which included 13 cases (46%, 13/28) of the recurred cases and 9 cases (45%, 9/20) of the primary chemo-resistant cases. (2) For recurrent ovarian carcinoma changed tumor marker profile, the highest pathology type was serous histological type (77%, 10/13), while was mucinous histological type (4/9) for primary chemo-resistant patients. (3) For recurred patients, the median progression-free survival (PFS) and median overall survival (OS) in marker changed-group (22.2 and 60.0 months) were significantly longer than that in marker un-changed group (17.4, 46.0 months; P < 0.05). For primary chemo-resistant ovarian carcinoma patients, median OS in marker changed-group (15.9 months) was significantly shorter than that in marker un-changed group (25.0 months; P < 0.05). CONCLUSION: The profile of serum tumor makers in epithelial ovarian carcinoma may be changed after chemotherapy, which should be concomitantly determinate different serum tumor markers for monitoring the response to chemotherapy and follow-up of patients.