A case of pancreatic mucinous cystadenocarcinoma with malignant ascites without recurrence for more than 8 years after surgery.

Mucinous cystadenocarcinoma (MCAC) with malignant ascites is rare. We report a case of a 28-year-old woman who presented with epigastric pain. The ascites in the Douglas fossa was identified at a nearby gynecology clinic. Computed tomography showed a multiloculated cystic lesion (9.5 × 6.4 cm) in the tail of the pancreas, which was diagnosed as mucinous cystic neoplasm on imaging. Staging laparoscopy was performed, and rapid cytology of ascites revealed adenocarcinoma, leading to a diagnosis of unresectable MCAC. Subsequently, combination chemotherapy with gemcitabine plus S-1 was initiated. Although there were no remarkable changes in the imaging findings, the peritoneal dissemination node was not consistently recognized in any of the imaging findings, and distal pancreatectomy was performed. A peritoneal dissemination node was not observed in the laparotomy findings, but the peritoneal lavage cytology was positive. The postoperative pathological result was non-invasive MCAC, and the ascites was suspected to be caused by cyst rupture. The patient has been recurrence-free, including the reappearance of ascites, for > 8 years after adjuvant therapy with S-1. Although careful follow-up will be required in the future, the very good prognosis in this case suggests that MCAC with malignant ascites without obvious peritoneal dissemination should be considered for surgical resection.

Metastatic mucinous cystadenocarcinoma of the pancreas presenting as intractable back pain.

An 85-year-old woman presented to the emergency department for evaluation of gradually worsening upper back pain of 3 weeks' duration. Her pain radiated down her right arm and was associated with right arm weakness. She reported visiting pain medicine specialists who administered steroid and lidocaine injections, which failed to improve her symptoms. Initial CT of her chest revealed a large lytic lesion within her posterior right fourth rib extending into her T4 vertebral body. The primary malignancy, a mucinous cystadenocarcinoma of the pancreas, was later found on a CT of the abdomen. The patient was also found to have multiple pulmonary, hepatic and lymphatic metastases from the pancreatic primary tumour.

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.

Pseudomyxoma Peritonei as a First Manifestation of KRAS-Mutated Urachal Mucinous Cystadenocarcinoma of the Bladder: A Case Report.

Only 28 cases of pseudomyxoma peritonei (PMP) arising from urachal neoplasms have been reported. We report one example of this extremely rare disease with KRAS mutational status in its spectrum of pathology. A 45-year-old woman presented with urachal frankly invasive mucinous cystadenocarcinoma confined to the dome of the bladder, which clinically manifested as PMP and was not detected at the first surgery. The primary tumour was revealed 6 months later because of its recurrence as PMP. Microscopic investigation revealed tubular adenoma and cystadenocarcinoma communicating with the bladder lumen and transitioning from the urachal urothelium to the mucinous epithelium. A urachal remnant was identified near the neoplasm. On immunohistochemistry, the tumour proved positive for CK7, CK20, CEA, and CDX2. Staining for ß-catenin revealed expression in both the cytoplasm and cell membrane. Mismatch repair protein expression was normal. Somatic KRAS-mutation (G12V) was revealed in tubular adenoma, cystadenocarcinoma, and mucinous carcinoma peritonei and may play an oncogenic role in the malignant transformation of urachal mucosa and the development of PMP.

Bleeding from isolated gastric varices as complication of a mucinous cystic neoplasm of the pancreas: A case report.

INTRODUCTION: Bleeding from isolated gastric varices, though uncommon, may be life threatening and may occur as a consequence of splenic vein thrombosis by tumoral compression and subsequent left-sided portal hypertension. CASE OVERVIEW: We report the case of a 37-year old woman, previously diagnosed with a benign pancreatic cyst, who presented with severe gastric variceal bleeding. DIAGNOSIS, THERAPEUTICS INTERVENTIONS, AND OUTCOMES: Abdominal ultrasound (US) and computed-tomography (CT) revealed enlargement and changed morphology of the cystic tumor located on the body of the pancreas. Left-sided portal hypertension was disclosed resulting from splenic vein occlusion. Salvage left spleno-pancreatectomy with lymphadenectomy was undertaken with an excellent postoperative outcome. Histological analysis established the diagnosis of mucinous cystic neoplasm with foci of adenocarcinoma. CONCLUSION: Surgical treatment proved to be the election one, leading to a steady hemostasis, removal of the lesion, positive diagnosis, and resolution of the varices. Imaging follow-up of pancreatic cyst should also assess vascular patency to identify the patient at risk for gastric bleeding and to select patients who benefits from surgical resection.

[Usefulness of Transumbilical Laparoscopic-Assisted Appendectomy for a Suspected Case of Hydrops Processus Vermiformis].

A-77-year-old man presented to our hospital with high fever and lower abdominal pain. Enhanced CT of the abdomen revealed swelling of the appendix with wall thickening and fluid collection. We diagnosed appendicitis with abscess formation and performed transumbilical laparoscopic-assisted appendectomy after the inflammation improved in response to antibiotics. Operative findings revealed a cystic lesion ofthe appendix and strong adhesion ofthe appendix to the terminal ileum. Based on these operative findings, we changed the operative procedure to a single-incision laparoscopic assisted ileocecal resection because ofthe possibility ofhydrops processus vermiformis. Histopathological findings revealed hyperplasia ofthe glandular epithelium with nuclear enlargement. Mucinous cystadenocarcinoma ofthe appendix was diagnosed. Additional surgery was not performed due to the patient's request. The patient has been free from recurrent disease for approximately 6 months after the surgery. Transumbilical laparoscopic-assisted appendectomy is useful for preventing pseudomyxoma peritonei and easing changes in extended operations for suspected cases of hydrops processus vermiformis.

Mucinous cystadenocarcinoma of renal pelvis presenting as pyonephrosis.

Mucinous cystadenocarcinoma of renal pelvis is a rare epithelial tumor with poor prognosis. It is postulated to arise from metaplastic glandular mucosa in response to chronic irritation, and comprises less than 0.3% of total renal pelvic tumors. We present this case of a tumor noted in a 45-year-old lady that was diagnosed as mucinous cystadenocarcinoma on histological examination after radical nephrectomy. The patient is remaining well over a follow-up of three months.

Mucinous cystic neoplasm in heterotopic pancreas presenting as colonic polyp.

CONTEXT: Pancreatic heterotopia in itself is rare in the colon and to the best of our knowledge a neoplasm arising in a heterotopic tissue in the colon has not been reported. We herein report a pancreatic cystic neoplasm arising from heterotopic pancreatic tissue in colon. CASE REPORT: A 44-year-old lady presented with a history of lower abdominal colic, associated with mucoid loose stools 10-15 times/day. Fecal occult blood was positive on two occasions. On examination, a vague mass was palpable in the left upper quadrant of abdomen. Colonoscopy showed a polypoid growth at the splenic flexure of colon, which on biopsy was reported to be an inflammatory polyp. She underwent a laparoscopic converted to open left hemicolectomy. Post-operatively she developed a intra-abdominal collection which formed a controlled pancreatic fistula after percutaneous drainage. Histopathology revealed pancreatic heterotopia with pancreatic mucinous cystic neoplasm. CONCLUSION: Despite advances in patient care, preoperative diagnosis of heterotopic pancreas is difficult.

[Appendiceal mucocele as differential diagnostic problem of palpable mass in the right lower abdominal region]. / Az appendix mucoceléje, mint a jobb oldali alhasi terime differenciáldiagnosztikai problémája.

Appendiceal mucocele is a rare disease (0.2-0.3% of all appendectomies) and it is defined as abnormal accumulation of mucoid material in the appendiceal lumen. Almost half of the patients are asymptomatic. The most common clinical manifestation is pain and palpable mass in the right iliac fossa, which is difficult to differentiate from the malignant or benign adnexal masses. By presenting our three cases, we would like to draw attention to the diagnostic difficulties of the pain and palpable mass in the right lower abdominal region.

[A woman with invagination of the colon]. / Een vrouw met een colocolische invaginatie.

A 78-year-old woman presented at the emergency room with abdominal pain and diarrhea. At physical examination an abdominal tumour was palpated. The CT-scan showed an invagination of the colon, whereupon an explorative laparatomy was performed. A right-hemicolectomy was conducted and pathology showed an invasive mucineus cystadenocarcinoma in the caecum.

Cistadenoma mucinoso del apéndice cecal como causa de gangrena intestinal / Appendiceal mucocele causing large bowel gangrene

Introduction: Appendiceal mucocele are lesions located in the cecum, which are found in 0.2 to 0.3 percent of appendectomies and correspond to 8-10 percent of all appendiceal tumors. Clinical case: We report a 83 years old female consulting for abdominal pain lasting 24 hours. On physical examination, signs of peritoneal irritation were found. The patient was operated, finding an appendiceal tumor that was coiled up in the mesentery with large bowel gangrene. An intestinal resection, terminal ileostomy, appendectomy and surgical lavage were performed. The pathological study of the surgical piece reported a mucinous cystadenoma.

Introducción: Los tumores del Apéndice Cecal corresponden al 0,5 por ciento de las neoplasias gastrointestinales y se encuentran entre el 0,8 por ciento y el 1 por ciento de las apendicectomías. El término Mucocele Apendicular (AM), se refiere a un grupo de lesiones localizados a nivel del ciego cuyo rango de incidencia está entre el 0,2 por ciento y el 0,3 por ciento de todas las apendicectomías, y entre el 8-10 por ciento de todos los tumores apendiculares. Las presentaciones clínicas más frecuentes son: cuadro clínico de apendicitis aguda, como hallazgo incidental como en el caso de esta paciente, o como masa abdominal en fosa ilíaca derecha. Caso clínico: Se presenta un caso de Cistade-noma Mucinoso Apendicular (CAM) encontrado como hallazgo incidental en un abdomen agudo quirúrgico con la particularidad de presentarse con una necrosis intestinal.

[A case of a patient with giant mucinous cystadenocarcinoma who presented with abdominal pain].

We report a case of a patient in whom a giant mucinous cystadenocarcinoma was treated with distal pancreatectomy. A 37-year-old woman was admitted to the hospital complaining of intermittent epigastric pain. The laboratory data revealed a marked increase in serum levels of carcinoembryonic antigen( CEA 22 ng/mL), cancer antigen( CA) 19-9( 258,129 U/ mL), and CA125 (53 U/mL). A computed tomography (CT) scan revealed a cystic tumor, 15 cm in diameter, in the body of the pancreas. The tumor presented as a multilocular cyst with enhanced nodules. On positron emission tomography (PET)-CT,[ 18F] fluorodeoxyglucose uptake by the nodules of the cyst was noted. Under the diagnosis of malignant mucinous cystic neoplasm, we performed distal pancreatectomy, splenectomy, partial gastrectomy, and left adrenalectomy because the tumor was suspected to be invading the stomach and left adrenal gland. The tumor was histologically diagnosed as invasive mucinous cystadenocarcinoma with ovarian-like stroma. The patient survived for 14 months after surgery without tumor recurrence. Invasive mucinous cystadenocarcinoma of the pancreas has high rates of lymph node metastasis and early recurrence after surgery. We believe that we would have had to perform complete tumor resection equivalent to that of invasive ductal carcinoma of the pancreas if the mucinous cystic neoplasm was found to be malignant preoperatively.

Radiologic findings of urachal mucinous cystadenocarcinoma causing pseudomyxoma peritonei.

Urachal mucinous cystadenocarcinoma causing pseudomyxoma peritonei is very rare. We report a case of a 59-year-old man with urachal mucinous adenocarcinoma associated with pseudomyxoma peritonei, and our radiologic findings. Ultrasonography revealed a well delineated, large cystic tumor adjacent to the anterior wall of the abdomen. Computed tomography and magnetic resonance imaging revealed a tumor of which the left posterior wall was defective. A large amount of ascites was present in the peritoneal cavity. The ascites caused displacement of the intestinal structures toward the dorsal region. The tumor wall and septa in the ascites were well enhanced on contrast-enhanced images. Radiologically, pseudomyxoma peritonei due to rupture of urachal cystic tumor was considered. The pathologic diagnosis was mucinous adenocarcinoma and pseudomyxoma peritonei.

[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.

Ruptured mucinous cystic neoplasm with an associated invasive carcinoma of pancreatic head in a pregnant woman: report of a case and review of literature.

Mucinous cystic neoplasm (MCN) of the pancreas is characterized by mucin-producing columnar epithelium and an ovarian-type stroma. It occurs almost exclusively in women and is almost always located in the pancreatic body or tail. Here, we report a case of large MCN located in the pancreatic head but not in the body nor tail in a 32-year-old pregnant woman, which was thought to have grown rapidly during pregnancy. It was ruptured at 34 weeks of gestation and the patient was admitted to the emergency department of the University of Fukui Hospital with an acute abdomen. Emergency cesarean section followed by pancreaticoduodenectomy was performed. The tumor consisted of many small cysts lined by a single-layer of mucinous epithelium with papillary growth and partial solid parts showing invasive growth and sarcomatoid changes, indicating mucinous cystic neoplasm with an associated invasive carcinoma (previously referred as mucinous cystadenocarcinoma). Thickened septa revealed ovarian-type stroma strongly positive for α-inhibin and partly positive for progesterone receptor immunohistochemically. We also review and discuss previous reports of MCNs including those with an associated invasive carcinoma in pregnant patients.

Tumor spectrum of adult intussusception.

INTRODUCTION: Adult intussusception is rare. Most general and colorectal surgeons are unfamiliar with its etiology and optimal management. PATIENTS AND METHODS: Patients older than 16 years and diagnosed with intestinal intussusception between January 1990 and June 2006 were retrospectively reviewed. Data related to presentation, diagnosis, treatment, and pathology were analyzed. RESULTS: Seventy-two patients underwent surgery for intestinal intussusception. Neoplasm was identified as the cause of intussusception in 66 (92%) cases, and 6 (8%) were idiopathic. The incidence of malignant colonic intussusception (63%) was significantly higher than that of enteric intussusception (20%), P = 0.001. Primary colon adenocarcinoma (8 of 10 patients, 80%) and malignant lymphoma (2 of 10 patients, 20%) were the two most common underlying malignant lesions in the colon. Lipoma (15 of 40 patients, 38%) and Peutz-Jegher adenoma (10 of 40 patients, 25%) were the two most common lesions of benign small bowel neoplasms while 27% (3 of 11) of malignant enteric intussusception cases were malignant lymphoma and metastatic respectively. CONCLUSION: Lipoma is the most common benign tumor in both small and large bowel intussusception. Whereas 80% of tumors associated with small bowel intussusception were benign, two-thirds of colonic intussusceptions had resulted from primary adenocarcinoma.