Primary Mucinous Cystadenocarcinoma of the Breast: A Rare Case Report With Review of Literature.

Primary mucinous cystadenocarcinoma (MCA) of the breast is a rare variant of breast carcinoma known to have a favorable prognosis despite showing a basal-like phenotype. We describe a case of MCA breast in a 45-year-old female with a palpable mass in the breast. On the basis of the histopathological and immunohistochemical evaluation of a lumpectomy specimen with the absence of mass anywhere else on whole-body imaging, a diagnosis of primary MCA was rendered. Mismatch repair protein evaluation showed this tumor to be microsatellite stable. Molecular testing revealed the absence of Kirsten rat sarcoma viral oncogene homolog (KRAS), neuroblastoma RAS viral oncogene homolog (NRAS), and v-raf murine sarcoma viral oncogene homolog B1 (BRAF) mutations. To date only 27 cases of MCA breast have been reported. To the best of our knowledge, ours is the first case documenting diffuse Cytokeratin 20 (CK20) positivity, microsatellite stability, and the absence of KRAS, NRAS, and BRAF mutations in these tumors. The rarity of this tumor further evokes an interest in this case. A better understanding of the disease warrants a review of more cases with longer follow-ups.

Intraperitoneal mitomycin C improves survival compared to cytoreductive surgery alone in an experimental model of high-grade pseudomyxoma peritonei.

Pseudomyxoma peritonei (PMP) is a rare cancer commonly originating from appendiceal neoplasms that presents with mucinous tumor spread in the peritoneal cavity. Patients with PMP are treated with curative intent by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). The value of adding HIPEC to CRS has not been proven in randomized trials, and the objective of this study was to investigate the efficacy of intraperitoneal mitomycin C (MMC) and regional hyperthermia as components of this complex treatment. Xenograft tissue established from a patient with histologically high-grade PMP with signet ring cell differentiation was implanted intraperitoneally in 65 athymic nude male rats and the animals were stratified into three treatment groups; the cytoreductive surgery group (CRSG, CRS only), the normothermic group (NG, CRS and intraperitoneal chemotherapy perfusion (IPEC) with MMC at 35 ºC), and the hyperthermic group (HG, CRS and IPEC at 41 ºC). The main endpoints were survival and tumor weight at autopsy. Adequate imitation of the clinical setting and treatment approach was achieved. The median survival was 31 days in the CRSG, 60 days in NG and 67 days in HG. The median tumor weights at autopsy were 34 g in CRSG, 23 g NG and 20 g in HG. In conclusion, the addition of IPEC with MMC after CRS doubled the survival time and reduced tumor growth compared to CRS alone. Adding regional hyperthermia resulted in a modest improvement of treatment outcome.

[Appendix Cancer with Long-Term Survival after Multimodality Therapy-A Case Report].

We report a case of long-term survival in a 65-year-old woman with recurrent appendix cancer. In March 2002, she was diagnosed with appendix cancer and underwent ileocecal resection. The pathological diagnosis was mucinous cystadenocarcinoma, pT2N0M0, Stage Ⅰ. In April 2006, ovariohysterectomy was performed for right ovarian metastases. In February 2011, tumor resection was performed for disseminated recurrence after 4 courses of systemic chemotherapy(bevacizumab plus mFOLFOX6). Although no recurrent lesions had been detected on imaging, stepwise elevation of serum CEA level was observed from June 2016. In November 2017, computed tomography scan revealed a slow-growing tumor on the liver. We performed partial resection of the right hemidiaphragm for the disseminated tumor, and the pathological diagnosis was mucinous adenocarcinoma. The patient has been on continuous postoperative follow-up without recurrence until June 2019. Appendix cancer is relatively rare and has a worse prognosis compared to colorectal cancer because of higher frequency of disseminated metastases. With the multimodality therapy, our patient showed long-term survival over 17 years despite a disseminated recurrence. In cases of mucinous cystadenocarcinoma of the appendix, persistent follow-up and aggressive treatment are recommended.

Successful pregnancy after mucinous cystic neoplasm with invasive carcinoma of the pancreas in a patient with polycystic ovarian syndrome: a case report.

BACKGROUND: The incidence of invasive cancer within a mucinous cystic neoplasm of the pancreas varies between 6 and 36%. Polycystic ovarian syndrome is a disorder characterized by hyperandrogenism and anovulatory infertility. One surgical treatment that can restore endocrine balance and ovulation in polycystic ovarian syndrome is partial ovarian destruction. Successful pregnancies following preconception pancreaticoduodenectomies (Whipple procedures) and chemoradiation to treat pancreatic neoplasms have been reported rarely but none were diagnosed with pre-cancer polycystic ovarian syndrome-associated infertility. Gemcitabine is an antimetabolite drug used for the treatment of pancreatic cancer that can have profound detrimental effects on oogenesis and ovarian function. Whether the ovarian destructive property of gemcitabine could act as a method to restore ovulation potential in polycystic ovarian syndrome is unknown. CASE PRESENTATION: A 40-year-old white American woman with a history of pancreatic cancer treatment with a Whipple procedure and chemoradiation with gemcitabine had a successful pregnancy after years of pre-cancerous anovulatory infertility and polycystic ovarian syndrome. She received no fertility agents and delivered full term via a spontaneous vaginal delivery with no pregnancy complications. CONCLUSION: Gemcitabine treatment for pancreatic cancer may result in resumption of ovulation in women with polycystic ovarian syndrome and these women should be counseled accordingly.

Management of patients with pancreatic cystic lesions: A case-based survey.

BACKGROUND: Pancreatic cystic lesions (PCL), including intraductal papillary mucinous neoplasia (IPMN), harbor different malignant potential and the optimal management is often challenging. The present study aims to depict the compliance of experts with current consensus guidelines and the accuracy of treatment recommendations stratified by the medical specialty and hospital volume. METHODS: An international survey was conducted using a set of 10 selected cases of PCL that were presented to a cohort of international experts on pancreatology. All presented cases were surgically resected between 2004 and 2015 and histopathological examination was available. Accuracy of the treatment recommendations was based on the European and international consensus guideline algorithms, and the histopathological result. RESULTS: The response rate of the survey was 26% (46 of 177 contacted experts), consisting of 70% surgeons and 30% gastroenterologists/oncologists (GI/Onc). In the case of main-duct IPMN (MD-IPMN), surgeons preferred more often the surgical approach in comparison with the GI/Onc (55 versus 44%). The mean accuracy rate based on the European and international consensus guidelines, and the histopathological result, were 71/76/38% (surgeons), and 70/73/34% (GI/Onc), respectively. High-volume centers achieved insignificantly higher accuracy scores with regard to the histopathology. Small branch-duct IPMN with cysts <2 cm and malignant potential were not identified by the guideline algorithms. CONCLUSION: The survey underlines the complexity of treatment decisions for patients with PCL; less than 40% of the recommendations were in line with the final histopathology in this selected case panel. Experts and consensus guidelines may fail to predict malignant potential in small PCL.

[Diagnosis and treatment of pancreatic cysts]. / Diagnostik og behandling af pancreascyster.

Incidental cystic lesions of the pancreas are often detected due to the increased use of cross-sectional imaging. Since mucinous cysts have a malignant potential, whereas pseudocysts and serous cystadenomas are benign, the distinction is of key clinical importance. Current recommendations advocate the use of multiple imaging modalities (CT/MRI/endoscopic US/endoscopic US & fine-needle aspiration) during evaluation and follow-up. This review describes the most frequent cystic lesions of the pancreas and suggests a simple investigation and treatment algorithm.

[Diagnosis and treatment of pancreatic cysts].

Incidental cystic lesions of the pancreas are often detected due to the increased use of cross-sectional imaging. Since mucinous cysts have a malignant potential, whereas pseudocysts and serous cystadenomas are benign, the distinction is of key clinical importance. Current recommendations advocate the use of multiple imaging modalities (CT/MRI/endoscopic US/endoscopic US & fine-needle aspiration) during evaluation and follow-up. This review describes the most frequent cystic lesions of the pancreas and suggests a simple investigation and treatment algorithm.

[Morphology of secondary ovarian tumors and metastases]. / Morphologie sekundärer Ovarialtumoren/Ovarmetastasen.

The distinction between primary and secondary (metastatic) ovarian tumors is essential for the selection of appropriate surgical interventions, chemotherapeutic treatment and prognostic evaluation for the patient. Metastatic tumors of the ovary range between 5 % and 30 %. The majority of ovarian metastases in Europe and North America derive from colorectal (25-50 %) and breast cancers (8-25 %). A major issue is the differential diagnosis of mucinous tumors. Major features favoring metastasis include bilaterality, size < 10 cm, ovarian surface involvement, extensive intra-abdominal spread, and infiltrative growth within the ovary involving the corpus albicans and corpora lutea. An algorithm using bilaterality and tumor size (cut-off 10 cm) allows correct categorization in approximately 85 % of the cases. Although immunohistochemistry (especially CK7 and CK20 in mucinous tumors) using a panel of antibodies plays a valuable role and is paramount in the diagnosis, the results must be interpreted with caution and within the relevant clinical and histopathological context. It is necessary to note that the correct diagnosis of ovarian metastases always needs interdisciplinary and multidisciplinary approaches.

[Cystic tumors of the pancreas - - our experience with diagnostics]. / Cystické nádory pankreatu -  nase zkusenosti.

Number of newly diagnosed cystic pancreatic tumors is permanently increasing. This fact is primarily related to the development of new diagnostic methods. The main representative ones are: serous cystadenoma, mucinous cystic neoplasm, intraductal papillary mucinous neoplasm and solid pseudopapillar tumor. Because of the malignant potential of these lesions, proper indication of surgical treatment is extremely important. The article highlights and describes our experience in diagnostics and therapy of cystic pancreatic tumors diagnosed in the General Teaching Hospital Prague in the period: 1/ 2008- 12/ 2012. All patients were investigated by computerised tomography and endoscopic ultrasound with fine -  needle aspiration biopsy. Thirty seven patients in total were diagnosed with cystic pancreatic tumors: 19 with serous cystadenoma, 5 with mucinous cystic neoplasm, 5 with mucinous cystadenocarcinoma, 5 with intraductal papillary mucinous neoplasm and 3 with solid pseudopapillar tumor. In 14 cases patients were indicated for surgery, in 1 case signs of malignant transformation were found. Determination of the optimal strategy for diagnostic and therapeutic procedures in patients with cystic pancreatic tumors requires the dia-gnosis, treatment and followup observation in adequately equiped specialized centers.

[Diagnosis and treatment of the epithelial ovarian cancer at the West African Cancer Center of Dakar]. / Prise en charge diagnostique et thérapeutique des tumeurs épithéliales malignes de l'ovaire dans le Centre Ouest-Africain de lutte Contre le Cancer de Dakar.

INTRODUCTION: Epithelial ovarian cancer are the most frequent of ovarian cancer, their prognosis is very bad. The aim of this study is to describe the diagnosis, the treatment and to assess the survival rate of the patients. METHODS: It was a retrospective study realized at the Cancer Institute of Dakar from December 2000 to January 2007. We have collected 117 patients with epithelial ovarian cancer. The mean age was 49 years. Patients were comprised: 22 stage I, 32 stage II, 35 stage III and 26 stage IV. Primary surgery was performed to 34 patients and the other patients were treated with chemotherapy and surgery. The survival rate was assessed by Kaplan-Meier method and the Logrank test had allowed to compare the survival among age and optimal surgery. RESULTS: Optimal surgery R0 was done in 20 cases and surgical resection R2 was performed in 45 cases. Pathological exam had found 65 serous cystadenocarcinoma, 28 mucinous cystadenocarcinoma and 21 endometrioid cystadenocarcinoma, one malignant tumor of Brenner. Overall survival at five years was 13.3%. The survival among optimal surgery was 16.3 and 2.3% for suboptimal surgery. There was no significant difference of the survival among patients who were less than 40 years old (P = 0.334). CONCLUSION: Prognosis of epithelial ovarian cancer is worse in Senegal as like as in the world. To improve the survival of our patients, we must detect the early diagnosis of these tumors and to introduce the neoadjuvant chemotherapy before optimal surgery.

Type-specific response to neoadjuvant chemotherapy: ovarian high-grade serous carcinoma versus colorectal mucinous carcinoma.

BACKGROUND: Ovarian carcinomas are currently managed as a single entity with no stratification for histological type. The foundation of treatment is a combination of surgery and chemotherapy. Women who are not candidates for up-front debulking surgery, either because of performance status or widespread disease, are often offered neoadjuvant chemotherapy in an effort to shrink their tumour and make resection possible. CASE: A 76-year-old woman was treated with neoadjuvant platinum-based chemotherapy for advanced ovarian cancer. At interval debulking surgery, she was found to have a concurrent mucinous colorectal carcinoma that was essentially unaffected by her treatment. CONCLUSION: This case serves as an in vivo demonstration of the greater resistance to platinum-based treatments of mucinous carcinomas than of the "typical" high-grade serous ovarian cancer.

Appendiceal mucinous cystadenocarcinoma with implantation metastasis to the incision scar and cutaneous fistula.

INTRODUCTION: Mucinous cystadenocarcinoma of the appendix with invasion of the anterior abdominal wall and cutaneous fistula development is a rare condition. To the best of the authors' knowledge, five such cases have been reported in the medical literature. METHODS AND RESULTS: The patient was a 68-year-old man who presented with a mass growing through the incisional scar and a skin fistula with mucinous discharge 1 year after appendectomy via McBurney's incision. Carcinoembroyonic antigen and carbohydrate antigen 19-9 concentration was elevated. Abdominal computed tomography scan revealed a mass in the cecum with involvement of the abdominal wall. Right hemicolectomy with en bloc abdominal wall implant and skin fistula resection with clear margins was performed. DISCUSSION: The patient received intraperitoneal intra- and postoperative chemotherapy. Gross examination of the specimen showed a mass emerging from the remnant stump of the appendix. The patient showed no relapse 1 year after surgery. Clinical, paraclinical features, and treatment options are discussed with review of the literature.

[A long-term survival case in advanced mucinous cystadenocarcinoma of the appendix using cytoreductive surgery and S-1/CDDP chemotherapy].

Prognosis of patients with advanced mucinous cystadenocarcinoma of the appendix (AMCA) is extremely poor. However, there has been no established treatment strategy. We preliminary report here a successfully treated case with AMCA using intensive cytoreductive surgery and chemotherapy. A 61-year-old woman had a right lower abdominal pain and was diagnosed as acute appendicitis. At surgery, about 5 cm tumor with mucosal fluid was detected at the distal part of the appendix. The tumor was invading the ileum and bladder. We performed appendectomy with tumor, partial resection of the small intestine and debridement of mucosal fluid. Histopathology revealed AMCA invading the ileum and bladder. After non curative surgery, we started S-1 plus cisplatin chemotherapy, which S-1 was given orally, twice daily for 3 consecutive weeks, and cisplatin was given intravenously on day 1, 8 and 15 followed by a 3-week rest period. After 6 courses starting with chemotherapy, a complete response was obtained. We followed by S-1 until two years after the initial surgery. At 36 months after the initial surgery, CT scan demonstrated a peritoneal recurrence. Then, she underwent intensive peritonectomy with intraperitoneal hyperthermic chemotherapy. Currently, she had no apparent recurrence for 59 months after initial surgery.

Prognostic impact of prechemotherapy serum levels of HER2, CA125, and HE4 in ovarian cancer patients.

OBJECTIVE: Human epididymis protein 4 (HE4) has attracted a lot of interest as a relatively novel biomarker for ovarian carcinoma. Research focus has been directed at HE4 as a diagnostic tool with potential for better triage of women with adnexal masses but the prognostic aspect of HE4 in ovarian cancer patients remains to be elucidated. The aim of the present study was to investigate the prognostic value of prechemotherapy serum HER2, cancer antigen 125 (CA125), and HE4 levels in ovarian cancer patients receiving standard combination chemotherapy. METHODS: Serum from 139 patients with newly diagnosed ovarian cancer was analyzed for HER2, CA125, and HE4 using enzyme-linked immunosorbent assay assays. Samples were collected just before first-line chemotherapy, and all patients were treated with carboplatin-paclitaxel combination chemotherapy. RESULTS: Increasing levels of serum HE4 (grouped into quartiles) was significantly associated with worse progression-free survival (PFS) (P < 10) and overall survival (P < 10). After adjustment in the Cox model, HE4 serum levels remained an independent prognostic parameter for PFS, with a hazard ratio of 1.77 (95% confidence interval, 1.03-3.04; P = 0.040) for patients with HE4 levels above the median compared with patients with HE4 levels below the median. The shorter PFS for patients with high levels of HE4 also translated into an independent significant difference in overall survival (hazard ratio, 3.17 [95% confidence interval, 1.41-7.10]; P = 0.005).Serum HER2 and CA125 levels did not demonstrate an independent prognostic value. CONCLUSIONS: High levels of serum HE4 is a strong and independent indicator of worse prognosis in epithelial ovarian cancer patients.