Report of a case: Retroperitoneal mucinous cystadenocarcinoma with rapid progression

IntroductionRetroperitoneal mucinous cystic neoplasms are uncommon, and little is known about the etiology of the disease. Malignant forms of these are extremely rare. Here, we report a case of primary retroperitoneal mucinous cystadenocarcinoma (PRMC), which demonstrated unexpectedly aggressive progression despite finding only a limited area of adenocarcinoma.Presentation of caseA 62-year-old woman with a complaint of abdominal discomfort was admitted to the hospital. Abdominal CT and MRI showed multiple large retroperitoneal cysts dislocating the right kidney nearly to the center of the abdomen. Transabdominal resection of the cysts was performed. Those cysts contained 1100 ml of mucinous fluids in total. Cytological examination of those fluids revealed no malignant cells. The cyst wall was lined with mucinous epithelial cells, and contained some ovarian-type stroma. Also, there was a focal area of adenocarcinoma in the cyst wall, and the lesion was diagnosed as primary retroperitoneal mucinous cystadenocarcinoma. Eight months later, the patient developed lumbar bone metastasis. Chemotherapy with S-1, an oral fluoropyrimidine, and docetaxel had been begun immediately; however, the disease had rapidly spread in the retroperitoneum. Eventually, the patient died of the disease 15 months after surgery.DiscussionRetroperitoneal mucinous cystic neoplasms are considered to be metaplasia of embryonal coelomic epithelium. Complete excision without rupture is essential. However, variance of biological aggressiveness might exist in PRMCs.ConclusionRetroperitoneal mucinous cystadenocarcinoma is a rare tumor, and it is urgently necessary to elucidate the etiology of an effective therapy for the disease.     

Duplex vermiform appendix: case report of a ruptured second appendix

Although the finding of appendiceal duplication is uncommon, its misdiagnosis and mismanagement may yield poor clinical outcomes and serious medicolegal consequences. Laparoscopic surgical exploration was performed on a 17-year-old male patient with right lower quadrant pain and a history of a previous appendectomy. Inspection of the cecum revealed a second appendix, which was retrocecal, ruptured, and gangrenous. Appendiceal duplication should be considered in all cases of lower abdominal pain, and careful inspection of the cecum for appendiceal anomalies should be performed. The Cave-Wallbridge classification system will be presented to assist surgeons in diagnosing appendiceal duplications.     

Clinicopathological features and the outcome of surgical management for adenocarcinoma of the appendix

AIM:To present a comprehensive analysis of incidence,clinicopathological features,appropriateness of surgical procedures,and survival for adenocarcinoma of the appendix.METHODS:A retrospective case analysis was conducted for the 10-year period 1998-2008.All patients diagnosed with adenocarcinoma of the appendix were analyzed for their demographics details,clinical features,tumor incidence and characteristics,tumor stage,surgical procedures performed,and their survival.RESULTS:Nine thousand three hundred and twentythree patients underwent appendectomies during the study period,and of these,10 (0.1%:8 men and 2 women with a mean age of 53.1 years,age range 21-83 years) were found to have primary adenocarcinoma of the appendix.Appendicular neoplasia was not suspected pre-operatively in any of the patients.Six (60%) patients underwent secondary right hemicolectomy.Four (40%) cases had appendectomy alone,and two of them died,whereas all those who underwent right hemicolectomy are alive and disease free.Five (50%) were reported to have grade 1 disease,three (30%) grade 2,and two (20%) grade 3 with mean survival of 34,48,and 22 mo,respectively.Six (60%) patients presented with advanced disease (Duke’s C and D).At the end of follow up (mean period:37.9 mo),eight patients are alive and disease free at the end of follow up.Overall mean survival was 36.3 mo (conf idence interval;16%-56%) with 41.3 and 16 mo for men and women,respectively.Mean survival for those with and without lymph node involvement was 33.6 and 40.2 mo,respectively.Right hemicolectomy gave better results than appendectomy alone,although the difference was not statistically signif icant due to the small number of cases.CONCLUSION:Adenocarcinoma of the appendix is extremely rare neoplasm with varied presentations,and is usually advanced when diagnosed.Right hemicolectomy is the treatment of choice for such tumors.     

A case of mucinous cystadenocarcinoma of the appendix penetrating the urinary bladder

The patient, an 81-year-old woman, had the chief complaint of macroscopic hematuria. Cystoscopy revealed a bladder tumor, which was determined by biopsy to be mucin-producing adenocarcinoma. Appendiceal carcinoma that invaded the bladder were diagnosed preoperatively by air-contract barium enema and magnetic resonance. She was treated with partial cystectomy and resection of the cecum. Sixteen cases of appendiceal carcinoma with invasion into the bladder have been reported in Japan.     

Appendical fistulae formation as a complication of primary Crohn's disease prior to surgical management: Report of a case

We report herein the unusual case of a 55-year-old man who presented with primary Crohn's disease of the appendix with fistulae. In this patient there was no history of previous appendectomy. Although fistula formation around the orifice of the appendix after appendectomy for primary Crohn's disease of the appendix has been observed in a number of long-term follow-up studies, preoperative fistula formation is extremely rare.     

Intussusception of a mucocele of the appendix secondary to an obstruction by endometriosis: Report of a case

(Received for publication on May 29, 1998; accepted on Nov. 6, 1998)     

Pseudomyxoma peritonei concomitant with early gastric cancer: Report of a case

The preoperative diagnosis of both appendiceal carcinoma and pseudomyxoma peritonei is difficult because of the nonspecific nature of the associated symptoms. More than 50% of all patients with carcinoma of the appendix are diagnosed with and treated for acute appendicitis. In addition, there have been few reports on patients with an appendiceal carcinoma or pseudomyxoma peritonei in association with other synchronous or metachronous malignancies of the alimentary tract. We herein report the first known patient with synchronous early gastric cancer and pseudomyxoma peritonei, in which the origin was correctly identified preoperatively.     

Intussusception of the appendix with a calcified fecalith

We treated a patient with a complete invagination of the appendix which contained one large laminated calcified fecalithy. Colonofiberscopy showed a dimpling submucosal tumor, which was palpated as a bony hard tumor at laparotomy. This finding suggested that the fecalith caused an intussusception of the appendix and that such an intussusception should be suspected when there is a bony hard dimpling submucosal tumor in the cecum.     

Acute epiploic appendagitis at the tip of the appendix mimicking acute appendicitis: A rare case report with literature review

BACKGROUND Acute epiploic appendagitis of the appendix(AEAA) is a rare self-limiting inflammatory disorder of the epiploic appendages(EA) close to the vermiform appendix, which often times mimicking the presentation of acute appendicitis(AA). To date, very few cases of AEAA have been reported. We report a case of a52-year old man with the clinical suspicion of AA, but post-operative specimen examination confirmed AEAA as the final diagnosis.CASE SUMMARY A 52-year-old morbidly obese man presented to the emergency department with a 1-d history of the right lower quadrant(RLQ) abdominal pain. Physical examination revealed localized RLQ tenderness mimicking AA. The computed tomography abdomen was inconclusive, and a decision was made to perform laparoscopic appendectomy(LA). During the LA, an infarcted epiploic appendage at the tip of appendix and adherent to the abdominal wall was found,which was entirely excised. Final pathology showed congested and hemorrhagic epiploic appendage without any accompanied acute inflammatory changes in the wall of the appendix. Postoperative course was uneventful and he was doing well at seven months follow-up.CONCLUSION The possibility of AEAA should be considered in patients clinically suspected of having AA. Surgery is considered for those refractory to conservative management, with inconclusive diagnosis or develop complications at presentation.     

Ureteral Metastasis from Appendiceal Cancer: Report of a Case

We report a case of primary appendiceal cancer that metastasized to the ureter. The patient was a 51-year-old woman who complained of persistent left back pain and right lower abdominal pain. After a detailed examination she was diagnosed to have cancer of the appendix, multiple liver metastases, and left hydronephrosis. Since an obstruction of the ileocecum was obvious, she underwent an ileocecal resection and a resection of the spindle-shaped tumor invading the left ureter. Lymph node metastases were found at the root of the superior mesenteric artery. The spindle-shaped tumor was considered to be due to invasion of the appendiceal cancer to the left ureter. The patient died 5 months postoperatively.     

Intussusception of the appendix by mucinous cystadenoma. Report of a case with an unusual clinical presentation

Appendiceal mucocele is a very rare clinical condition. Associated ascites and an ovarian mass could suggest synchronous ovarian cystadenocarcinoma with pseudomyxoma peritonei. We describe the case of a 36-year-old female with a mucinous cystadenoma of the appendix causing intussusception, diagnosed by CT but not by US scan, since the associated anomalous fixation of the caecum was misleading in defining the precise anatomical site. Although the CT findings were accurate, the synchronous presence of an ovarian cyst and ascites did not allow us to rule out preoperatively a concurrent cystadenocarcinoma of the ovary with pseudomyxoma peritonei. The appropriate surgical treatment was performed on the basis of intraoperative frozen section examination. Surgical treatment depends on the nature of the mucocele: retention forms are effectively treated by appendectomy, while neoplastic conditions require a more extended resection. Treatment of associated ovarian cystadenocarcinoma and pseudomyxoma peritonei includes right colectomy, bilateral ovariectomy and omentectomy. Although a precise preoperative diagnosis of mucocele associated with intussusception of the appendix has been reported as possible, concomitant ascites and ovarian masses, as in the present case, could mimic pseudomyxoma peritonei from concurrent ovarian cystadenocarcinoma. Intraoperative histopathology is required in order to perform the most appropriate treatment.     

Mucinous cystadenocarcinoma of the appendix. Apropos of a case]

A case of mucinous cystadenocarcinoma discovered into a voluminous benign mucocele of the appendix, is presented. The clinical feature is a tumor of the right iliac fossa. The identification of a limited area of epithelial degenerescence in the cyst wall--by peroperative microscopic examination--permits immediate enlargement of the cystectomy by a right hemicolectomy. The prognosis of the tumor is generally excellent as soon as the diagnosis is early and the removal large enough.     

Laparoscopic resection of a primary retroperitoneal mucinous cystadenoma: Report of a case

(Received for publication on July 4, 1996; accepted on Mar. 4, 1997)     

Pulmonary mucinous cystadenocarcinoma: report of a case and review of the literature

A case of primary mucinous cystadenocarcinoma of the lung is presented. The patient was a 42-year-old woman with a 5-cm left lung mass. Left lower lobectomy was performed and analysis of a frozen section revealed mucinous cystadenocarcinoma. The tumor was a fibrous, walled cyst containing abundant mucinous material. Sparse groups of malignant cells were microscopically observed in pools of mucin; thus, the tumor resembled mucinous cystadenocarcinoma that occurs in the ovary, appendix, or pancreas. The tumor we found is a very rare intrapulmonary neoplasm that is differentiated from a metastatic lesion and mucinous bronchoalveolar carcinoma by its very different clinical course and prognosis.     

Adenocarcinoid of the appendix: report of two cases

Adenocarcinoid of the appendix is a rare tumor with the histological features of both adenocarcinoma and carcinoid tumor. However, its biological behavior and malignant potential are still unclear. We treated two patients with this unusual tumor; a 60-year-old man and a 79-year-old woman. Both patients were initially diagnosed with acute appendicitis followed by an appendectomy. At surgery, the appendix was seen to be acutely inflamed without any macroscopic signs of tumor. Postoperative histological analysis revealed an adenocarcinoid tumor in the appendix, which had spread diffusely into its wall without forming a mass. Immunohistochemical staining with p53, MIB-1, bcl-2, and carcinoembryonic antigen suggested that neither of these tumors were particularly aggressive. Adenocarcinoid of the appendix is a rare tumor, which is very difficult to diagnose preoperatively and even macroscopically, making histological examination essential. Received: January 25, 2002 / Accepted: September 3, 2002 Reprint requests to: O. Watanabe     

Prolonged survival in a patient with recurrent pulmonary metastases secondary to mucinous cystadenocarcinoma of the appendix with pseudomyxomatous peritonei

We report a 65-year-old man presenting with recurrent pulmonary metastases 20 years after an appendectomy for mucinous cystadenocarcinoma with pseudomyxomatous peritonei. He underwent bilateral staged metastatectomies for metastases 7 years after the diagnosis and further metastasectomy after a recent recurrence. This is a rare case of recurrent pulmonary metastatic mucinous cystadenocarcinoma, and despite poor prognosis and nondefinitive initial treatment, this patient remains alive and well 20 years later.     

A goblet cell carcinoma of the appendix presenting as an acute appendicitis: a case report

Abstract

Background: A goblet cell carcinoma of the appendix is a rare neoplasm with histological features of both adenocarcinomas and carcinoid tumours. It has a more aggressive behaviour in comparison to the more common classic neuroendocrine appendiceal tumours. Clinical presentation is variable and a significant proportion of patients present with an acute appendicitis. The aggressive evolution of these tumours makes the management more challenging.

Case report: We report the case of a 43-year-old man with acute right iliac fossa pain who was initially diagnosed with an acute appendicitis. Histological analysis of the resected appendix demonstrated the presence of a goblet cell carcinoma with perineural and lymphovascular invasion. No distant metastasis was present. A right hemicolectomy was performed and adjuvant chemotherapy was administered.

Conclusion: Goblet cell carcinomas is a distinct entity which should be delineated from the classical appendiceal carcinoids. Its clinical evolution obliges a more aggressive therapeutic strategy.     

Curatively resected primary malignant melanoma of the esophagus: Report of a case

We report herein a rare case of primary esophageal malignant melanoma in a 49-year-old Japanese woman who presented with a 3-week history of dysphagia. Esophagogram and esophagoscopy demonstrated a polypoid tumor in the upper to mid-thoracic portion of the esophagus, and a radical subtotal esophagectomy was performed through a right thoracotomy. Histological and immunohistochemical studies proved this tumor to be a primary esophageal malignant melanoma. She received no postoperative adjuvant therapy and no signs of recurrence have been clinically observed for the 22 months since her operation.     

Retroperitoneal mucinous cystadenocarcinoma in a man: case report and review of the literature

In the English medical literature, 27 cases of primary retroperitoneal mucinous cystadenocarcinoma have been published. We report the second case of a primary retroperitoneal mucinous cystadenocarcinoma in a man. The patient was an 83-year-old man, with a large 26 x 20 x 16-cm retroperitoneal cystic mass causing abdominal discomfort and cachexia, who underwent excision of the mass. Prior reports suggest that this type of tumor has an aggressive clinical course, and surgical excision is the treatment of choice. These rare tumors need to be included in the differential of retroperitoneal cystic tumors.