Pseudomyxoma peritonei of appendiceal cancer with metastasis to the stomach: report of a case

Appendiceal cancer associated with pseudomyxoma peritonei is a relatively low-grade malignancy rarely associated with extraperitoneal metastasis. We report herein the case of a 71-year-old man in whom a metastasis was found in the stomach 2 years after he underwent surgery for pseudomyxoma peritonei of appendiceal cancer. He was referred to our hospital after presenting with anorexia and vomiting. Gastrofiberscopy, abdominal computed tomography, and ultrasound examination all revealed a mass 4 × 4 cm in size, containing a small ulcer, in the antrum of the greater curvature of the stomach. The histopathological diagnosis made from a biopsy of the tumor was mucinous cystadenocarcinoma. A distal partial gastrectomy was performed and the resected specimens from the appendiceal cancer resected 2 years earlier showed the same histological pattern as that of the gastric lesion. To the best of our knowledge, this is only the second report of pseudomyxoma peritonei secondary to mucinous cystadenocarcinoma of the appendix that metastasized to the stomach. Received: September 12, 2001 / Accepted: January 8, 2002     

Pseudomyxoma peritonei, origin from appendix: report of cases with images

This report demonstrates the cases of a 47-year-old housewife, a 54-year-old female, and an 80-year-old woman in whom pseudomyxoma peritonei was found accidentally during surgery. Pseudomyxoma peritonei is a rare disease. No effective treatment is known. Modern treatments include radical surgical excision with appendicectomy and oophorectomy in women and adjuvant hyperthermic intraperitoneal and systemic chemotherapy. Major causes of morbidity and mortality are bowel obstruction and biliary obstruction. Five-year survival rate, depending on whether the disease is benign or malignant, is about 53-75%. Median survival is about 2 years under surgical management.     

Fracture of the acetabulum: a retrospective review of ninety-one patients treated at a single institution

Acetabular fracture result in fairly good outcome after the anatomic reduction in the displaced fracture fragments and damaged joint structure, but some patients will inevitably suffer from hip joint problems during their courses after the insult. We retrospectively reviewed 91 patients with acetabular fractures to investigate the causes of clinical failure and relationship among the fracture types, selected treatment options and their courses. Ninety-one patients (73 men and 18 women) with an average age of 49 years (range 18–80) at the time of injury were followed up for an average of 8.6 years (range 2–18). Judet–Letournel classification of fracture type and Matta’s rating regimen of functional and radiographic patient’ assessment were conducted. Conservative treatment was provided in 20 patients, in which 19 attained excellent/good, and one fair clinical results. All achieved excellent/good radiographic outcome. Surgically treated patients (n = 71) with critical dislodgement of the fracture fragment showed that 64 (90%) attained excellent/good and 7 (10%) fair/poor clinical outcomes. Sixty-three (89%) attained excellent/good and 8 (11%) fair/poor postoperative radiographic outcome. Five patients with poor radiographic outcome after surgery subsequently required total hip arthroplasty, due to the development of hip joint osteoarthritis in 3 and femoral head avascular necrosis in 2. We conclude that displacement of the joint surface should be reduced to less than 3 mm in accordance with the selection of the most appropriate surgical approach for open reduction/fixation in each fracture type; however, comminuted fracture and avascular necrosis of the femoral head may be the cause of poor clinical results.     

Fracture of the pelvic ring: a retrospective review of 224 patients treated at a single institution

Pelvic ring fracture presents with a wide spectrum of clinical status and fracture type that requires multimodal treatment strategy. We report our experience in the treatment of 224 pelvic ring fractures in terms of clinical and radiological findings, mode of treatment, surgical data, and functional outcome at final follow-up. The study subjects were 140 men and 84 women (mean age 58 years, range 8–94). Surgery was conducted in 63 patients, while 161 were treated conservatively. The average follow-up period was 7.1 years (range 1–15). AO-Orthopaedic Trauma Association classification of fracture type, Injury Severity score rating, Rommens and Hessmann’s ambulatory and pain assessment, and radiographic studies were conducted. Conservative treatment was provided in 161 (73%) patients, including simple bed rest with definitive external fixation, skeletal traction, and/or pelvic sling. Of these, 148 patients achieved excellent/good results, but the remaining 13 cases complained of severe pain at the sacroiliac joints caused by malunion or fibrous union of the joint. All 63 (27%) patients who were treated surgically, using most frequently a combination of anterior extraperitoneal and Pfannenstiel approaches, showed excellent/good clinical and radiographic results. Treatment of unstable pelvic ring fracture should be urgent and based on biomechanical and anatomic reconstructive strategy, paying utmost care to associated injuries. A combination of open stabilization of posterior sacroiliac area and anterior fixation of pubic rami and symphysis pubis is recommended for unstable anteroposterior compression, lateral compression, vertical instability, and unilateral or bilateral posterior injuries to the pelvic ring.     

Pseudomyxoma peritonei: review on a cluster of peritoneal mucinous diseases

Pseudomyxoma peritonei is an uncommon condition characterized by abundant extracellular mucinous material and peritoneal tumoural implants. A ruptured appendiceal mucinous adenoma seems to be the most common origin, but low grade ovarian malignancies are often associated. Peritoneal gelatinous diseases must be subdivided into disseminated peritoneal adenomucinosis, peritoneal mucinous carcinomatosis and intermediate cases. These entities have significantly different prognosis. Debulking surgery is the most common treatment but some are in favour of an ultra radical surgery with hyperthermic intra-peritoneal chemotherapy, with some encouraging results.     

Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution.

OBJECTIVE: To analyze treatment and survival of a large cohort of patients with retroperitoneal soft-tissue sarcomas (STS) treated and prospectively followed at a single institution. SUMMARY BACKGROUND DATA: Retroperitoneal STS are relatively uncommon and constitute a difficult management problem. Although surgical resection is often difficult or impossible, current chemotherapy is not effective and radiation is limited by toxicity to adjacent structures. Thus, complete surgical resection remains the most effective modality for selected primary and recurrent disease. METHODS: Five hundred patients with retroperitoneal STS were admitted and treated between July 1, 1982, and September 30, 1997, and prospectively followed. Patient, tumor, and treatment variables were analyzed for disease-specific and disease-free survival. Survival was determined with the Kaplan-Meier method. Statistical significance was evaluated using the logrank test for univariate influence and Cox model stepwise regression for multivariate influence. RESULTS: Two hundred seventy-eight patients (56%) had primary disease and 222 (44%) recurrent disease. Median follow-up was 28 months (range 1 to 172 months), 40 months for survivors. Median survival was 72 months for patients with primary disease, 28 months for those with local recurrence, and 10 months for those with metastasis. For patients with primary or locally recurrent tumors, unresectable disease, incomplete resection, and high-grade tumors significantly reduced survival time. CONCLUSIONS: In this study of patients with retroperitoneal STS, stage at presentation, high histologic grade, unresectable primary tumor, and positive gross margin are strongly associated with the tumor mortality rate. Patients approached with curative intent should undergo aggressive attempts at complete surgical resection. Incomplete resection should be undertaken only for symptom relief.     

Liposarcoma: Clinico-pathological analysis,prognostic factors and survival in a series of 307 patients treated at a single institution

Background and objectives

Liposarcoma (LPS) is a malignant mesenchymal tumor and the most common soft tissue sarcoma. Four different subtypes are described: well differentiated (WD) LPS or atypical lipomatous tumor (ALT), dedifferentiated (DD) LPS, myxoid LPS, and pleomorphic LPS (PLS). The objective of the study was to investigate prognostic factors and clinical outcome of liposarcoma.

Retrospective long-term follow-up analysis in 21 patients with chordomas of various sites treated at a single institution

Twenty-one patients with chordoma were treated at the Radiation Oncology Center, Mallinckrodt Institute of Radiology, between 1949 and 1986. Thirteen patients had sacrococcygeal tumors, five had clival tumors, two had nasopharyngeal tumors, and one had a lumbar spine tumor. Nine patients were treated with surgery alone, eight patients with subtotal resection and postoperative irradiation, and four patients with radiotherapy alone after biopsy. The 5- and 10-year actuarial survival rates were 74% and 46%, respectively. The 10-year actuarial survival rate was significantly better in patients treated with surgery alone or surgery and irradiation than in those treated with radiotherapy alone (52%, 32%, and 0%, respectively, p = 0.02). Although all patients ultimately suffered a recurrence, those with lumbosacral tumors treated with surgery and irradiation had a longer mean disease-free survival period (6.6 years) than those treated with surgery alone (4.1 years) (p = 0.08). Disease-free survival times of patients with base of the skull tumors was not significantly different between the treatment groups. Irradiation after resection of chordomas appears to increase the time to first relapse in lumbosacral tumors and should be considered after subtotal resection.     

Long-term followup of 150 patients with testicular cancer treated at a single institution.

Between 1977 and 1988, 150 patients with disseminated primary testicular germ cell tumors were treated with cisplatin, vinblastine and bleomycin. Of the 150 patients 90 (60%) achieved a complete response to chemotherapy. An additional 33 patients achieved a complete response after removal of residual masses following chemotherapy. Thus, 123 of 150 patients (82%) achieved a disease-free status following chemotherapy with or without an operation. After a median followup of 49 months the estimated long-term probability of remaining without failure and of surviving is 77%. With this data base a multivariate analysis of prognostic factors determined the Indiana University staging system to be highly predictive. Other staging systems proved to be less useful. The subset of patients with minimal and moderate disease by Indiana staging containing mature teratoma in the orchiectomy specimen has a particularly excellent prognosis (99% actuarial survival) with chemotherapy.     

Chromophobe renal cell carcinoma: a clinicopathologic study of 203 tumors in 200 patients with primary resection at a single institution

Despite multiple studies, many clinicopathologic issues about chromophobe renal cell carcinoma (RCC) remain contentious; for example, its biological behavior-whether better or similar to papillary RCC, the incidence of sarcomatoid features, and whether pathologic features such as necrosis, nuclear grade, and tumor stage predict worse outcome. We studied 203 consecutive primary chromophobe RCCs resected at our institution in an attempt to answer these and other questions. The tumors showed significant progressive decrease in size and stage (P=0.047 and 0.001) from 1980 to 2000. Five patients had metastasis at presentation, and further disease-specific events (recurrence/metastasis/death due to disease) occurred in 8 more. Only 4 of 203 tumors had sarcomatoid features. Over median follow-up of 6.1 years (range, 0.1 to 18 y), 5-year and 10-year disease-specific events occurred in 3.7% (95% CI, 1.5%, 7.4%) and 6.4% (95% CI, 2.7%, 12.2%) patients. Outcomes showed significant association with tumor size, small-vessel invasion, sarcomatoid features, and microscopic necrosis (P≤0.05 each). pT stage or nodal metastasis tended to show some association, without reaching statistical significance (P=0.05 and 0.06, respectively). A modified tumor grading scheme, somewhat similar to that proposed recently, mitotic index, cytologic eosinophilia, and architecture, were not significantly associated with outcome. In conclusion, sarcomatoid differentiation is quite uncommon in chromophobe RCC. Tumor size, small-vessel invasion, sarcomatoid differentiation, and microscopic necrosis are the only features that are significantly associated with adverse outcome. On the basis of this long follow-up on a large number of cases, chromophobes seem to have better clinical outcomes than those reported for clear cell and papillary RCCs.     

Cholangiocarcinoma: thirty-one-year experience with 564 patients at a single institution

OBJECTIVE: To assess long-term survival and prognostic factors in a large series of patients with bile duct cancer. SUMMARY BACKGROUND DATA: The incidence of bile duct cancer is low but increasing. Determinants of survival vary in the literature, due to a lack of sufficient numbers of patients in most series. METHODS: We studied 564 consecutive patients with bile duct cancer operated upon between 1973 and 2004. Patients were divided into intrahepatic, perihilar, and distal groups. Principle outcome measures were complications, 30-day mortality, and survival. RESULTS: Of the 564 patients, 44 (8%) had intrahepatic, 281 (50%) had perihilar, and 239 (42%) had distal tumors. Approximately half (294, 52%) were treated before 1995, while 270 (48%) were treated thereafter. The perioperative mortality rate was 4%. In log-rank analyses, survival was higher in the later time period (P = 0.002), in patients with intrahepatic disease (P = 0.001), with negative resection margins (P < 0.001), with well/moderately differentiated tumors (P < 0.001), and those with negative lymph nodal status (P < 0.001). In multivariate analysis, negative margins (P < 0.001), tumor differentiation (P < 0.001), and negative nodal status (P < 0.001), but not tumor diameter, were significant independent prognostic factors. In R0-resected patients, lymph node status (P < 0.001), but not tumor diameter, histology, or differentiation, further predicted survival. The median survivals for R0-resected intrahepatic, perihilar, and distal tumors were 80, 30, and 25 months, respectively, and the 5-year survivals were 63%, 30%, and 27%, respectively. CONCLUSION: R0 resection remains the best chance for long-term survival, and lymph node status is the most important prognostic factor following R0 resection.     

Infectious and catheter-related complications in pediatric patients treated with peritoneal dialysis at a single institution

Continuous ambulatory peritoneal dialysis (CAPD) and continuous cycling peritoneal dialysis (CCPD) are the predominant dialytic modalities for the majority of children while awaiting transplantation. Wide acceptability of peritoneal dialysis is hindered by infectious complications. A retrospective review of 367 pediatric patients treated with CAPD/CCPD for at least 3 months from September 1980 through December 1994 revealed that the peritonitis incidence ranged from 1.7 to 0.78 episodes per patient-year. No differences in peritonitis rates were observed between patients treated with CAPD or CCPD. Gram-positive organisms were responsible for the majority of peritonitis episodes. Age, sex, race, primary renal disease, presence of nephrotic syndrome, and serum albumin level were not associated risk factors. Longer time on treatment and diminished serum IgG level were associated with increased peritonitis incidence. Treatment was successfully completed at home in most cases. Almost half of the catheter losses were caused byStaphylococcus, Pseudomonas, and fungal peritonitis and tunnel/exit-site infections. Infectious complications are still the major causes of morbidity and treatment failure in patients treated with CAPD/CCPD. Thus, controlled studies are needed to assess methods for prevention or improvement of peritonitis rates in this patient population.     

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.     

Glioblastoma multiforme-report of 267 cases treated at a single institution

OBJECTIVE: Glioblastoma multiforme (GBM) is the most common and most malignant primary brain tumor in adults. We present 267 cases treated at a single institution and discuss clinical characteristics and prognostic factors with regard to the neurosurgical literature. METHODS: Included in this study were 267 patients who underwent craniotomy for newly diagnosed GBM between 1990 and 2001 at our department. Clinical charts and radiographic images were reviewed. Association to patient survival was estimated using log-rank test. RESULTS: Median patient age was 61 years (mean, 59.5; range, 22-86 years), the male-female ratio was 1.2:1. In 35 cases (13.1%) the tumor was multicentric. Most of the tumors were classified as primary GBM (87.6%). During follow-up,72 patients (26.4%) underwent recraniotomy for GBM recurrence and 3 patients (1.1%) developed spinal drop metastases. Overall median survival was 47 weeks (range, 5-305 weeks). The following parameters were significantly associated with prolonged survival: (1) age 61 years or younger, P < .001; (2) Karnofsky performance scale score of 70 or more, P < .001; (3) radiotherapy with a total dose of at least 54 Gy, P < .001; (4) chemotherapy, P < .001; (5) total tumor resection, P = .014; (6) recraniotomy for GBM recurrence, P = .012. CONCLUSIONS: Glioblastoma multiforme remains an important cause of morbidity and mortality from intracranial tumors. The overall prognosis is dismal, although interdisciplinary therapy can significantly prolong survival and allows a small subgroup of patients to survive 3 years or more.     

Supratentorial calcified pseudotumour: experience of a single institution and review of the literature

Background

Calcified pseudotumours (CPTs) are rarely found in the central nervous system (CNS). Information on supratentorial CPT is limited. We add our experience treating three supratentorial CPTs. Clinical presentation, pathological findings and clinical course are discussed, and a literature review is provided.

Method

Between September 2011 and December 2012, three patients (age range 33–60 years) were diagnosed with symptomatic supratentorial CPTs, for which they underwent resection. Their clinical, radiological, and surgical data were retrospectively retrieved from their medical records and reported.

Results

Symptoms at presentation included headache (n?=?1), blurred vision (n?=?1), and cognitive decline (n?=?1). One tumour was in the left frontal lobe very close to the convexity surface, and the other two were in the midline adjacent to the corpus callosum. Radiological features included a calcified mass on computerised tomography (n?=?3), a hypointense (n?=?2) or hyperintense (n?=?1) signal on T2 and a rim of enhancement with some non-homogenous lesional enhancement on T1-gadolinium (Gd) sequences on magnetic resonance imaging (n?=?2). Two patients underwent gross total resection and had an uneventful postoperative course. The third underwent partial removal due to the tumour’s adherence to adjacent vascular structures and suffered postoperative complications. On pathology, all lesions had low cellularity fibrovascular tissue mixed with lobules of a haematoxylinophilic, chondromyxoid-like matrix surrounded at its periphery by a variable layer of palisading spindle, epithelioid or multinucleated giant cells. All three CPTs had focal inflammatory infiltrates and variable degrees of metaplastic calcification and ossification.

Conclusions

Surgical removal is recommended for benign intracranial CPTs that are symptomatic, are associated with brain oedema, or show tumour growth. Stable and asymptomatic CPTs can be followed.     

Infants with medulloblastoma: a single institution review of survival

Although the prognoses of children with medulloblastoma have improved dramatically over the last several decades, the reported survival for very young children with this tumor remains poor. We undertook a retrospective review of patients less than 36 months of age at the time of the diagnosis of a medulloblastoma who were treated at our institution during a 36-year period. Of 28 such patients, 10 patients are presently surviving without disease at a median of 150 months from diagnosis. The presence of a metastatic tumor at the time of diagnosis was identified as a significant indicator of a poor prognosis, and all completely staged patients without metastasis are surviving without a recurrence of disease. This report suggests that the survival of very young children with a medulloblastoma may not be as poor as has been previously reported, particularly if a disseminated tumor is not present at the time of diagnosis.     

Parotidectomy: ten-year review of 237 cases at a single institution.

OBJECTIVE: To review a single surgeon's experience with parotidectomy with an emphasis on examining the appropriate use of partial superficial parotidectomy and the differences in early outcomes observed with the various types and extent of parotidectomy used. STUDY DESIGN AND SETTING: A series of 237 patients who underwent parotidectomy over a 10-year period was reviewed. RESULTS: Postoperative complications included facial nerve weakness (18%), sialocele (6.3%), wound infection (3.8%), hematoma (3.8%), and symptomatic Frey's syndrome (1.7%). More extensive surgical procedures, including complete superficial or total parotidectomy, were associated with a 2.7 times greater incidence of immediate postoperative facial nerve weakness compared with partial superficial parotidectomy. CONCLUSION: Partial superficial parotidectomy is associated with a decreased incidence of transient postoperative facial nerve weakness compared with more extensive procedures such as complete superficial or total parotidectomy. Intraoperative frozen section was an accurate means of selecting patients for the partial superficial parotidectomy procedure. SIGNIFICANCE: Partial superficial parotidectomy is an effective method for treating benign tumors confined to the superficial lobe.