Diffuse Peritoneal Disease Research Articles

Clinical and Radiological Parameters to Discriminate Tuberculous Peritonitis and Peritoneal Carcinomatosis.

It is challenging to differentiate between tuberculous peritonitis and peritoneal carcinomatosis due to their insidious nature and intersecting symptoms. Computed tomography (CT) is the modality of choice in evaluating diffuse peritoneal disease. We conducted an ambispective analysis of patients suspected as having tuberculous peritonitis or peritoneal tuberculosis between Jan 2020 to Dec 2021. The study aimed to identify the clinical and radiological features differentiating the two entities. We included 44 cases of tuberculous peritonitis and 45 cases of peritoneal carcinomatosis, with a median age of 31.5 (23.5-40) and 52 (46-61) years, respectively (p ≤ 0.001). Fever, past history of tuberculosis, and loss of weight were significantly associated with tuberculous peritonitis (p ≤ 0.001, p = 0.038 and p = 0.001). Pain in the abdomen and history of malignancy were significantly associated with peritoneal carcinomatosis (p = 0.038 and p ≤ 0.001). Ascites was the most common radiological finding. Loculated ascites, splenomegaly and conglomeration of lymph nodes predicted tuberculous peritonitis significantly (p ≤ 0.001, p = 0.010, p = 0.038). Focal liver lesion(s) and nodular omental involvement were significantly associated with peritoneal carcinomatosis (p = 0.011, p = 0.029). The use of clinical features in conjunction with radiological findings provide better diagnostic yields because of overlapping imaging findings.

Is MDCT an accurate tool to differentiate between benign and malignant etiology in diffuse peritoneal disease?

There is often considerable overlap of imaging findings in benign and malignant peritoneal diseases. We evaluated patients with diffuse peritoneal disease, to assess the diagnostic value of MDCT in predicting benign or malignant etiology in patients with unknown etiology, by analyzing the various patterns of involvement, particularly tuberculosis (TB) vs malignancy. One hundred and thirty-six patients with diffuse peritoneal disease who had abdominal CT and subsequently underwent omental biopsies were included in the study. Peritoneal, mesenteric and omental involvement by disease was evaluated on CT using specific parameters. The presence of lymphadenopathy, ascites, scalloping of organs, involvement of liver and spleen, were also compared between benign and malignant conditions using histopathology as the gold standard. In 136 patients, 72 benign and 64 malignant pathologies were classified as per histopathology. Higher age (p < 0.001), increasing omental thickness (mean 25.2mm, p = 0.004), omental caking (p < 0.001), > 10mm mesenteric/peritoneal nodules (p < 0.03), visceral scalloping (p = 0.001), free ascites (p = 0.003), serosal involvement (p = 0.004) and bilateral pleural effusion (p = 0.02) were associated with malignant etiology. Mesenteric thickening/stranding (p = 0.02), mesenteric adenopathy (p < 0.001), necrotic nodes (p = 0.02), splenomegaly (p = < 0.001) and higher attenuation (> 20HU) of ascitic fluid (p < 0.001) were associated with benign etiology. The presence of mesenteric thickening or stranding (p = 0.01), splenomegaly (p = 0.02), higher ascitic fluid attenuation > 20HU (p = < 0.01), mesenteric adenopathy (p < 0.01), necrotic nodes (p = 0.03) favored tuberculosis. CT had diagnostic accuracy (79.3, 86.7%), sensitivity (79.2, 74.6%) and specificity (79.4, 97%) for observers 1 and 2, respectively (Kappa 0.713). Contrast-enhanced MDCT has good sensitivity, specificity and accuracy in differentiating benign and malignant etiologies of diffuse peritoneal disease. Multiple common parameters can be used to differentiate between tuberculous peritonitis and peritoneal carcinomatosis.

Diagnostic performance of positron emission tomography in the staging of pseudomyxoma peritonei

BackgroundPseudomyxoma peritonei (PMP) is a rare clinical entity, commonly derived from a mucin-producing tumour of the appendix. International consensus is unclear on the role of positron emission tomography (PET) in preoperative staging. This study aimed to assess the ability of preoperative PET in predicting the histological grade of PMP. MethodsAll patients scheduled for cytoreductive surgery (CRS) +/− hyperthermic intraperitoneal chemotherapy (HIPEC) for PMP who underwent preoperative PET at a single centre between June 2007 and June 2020 were included. A nuclear medicine physician, blinded to patient outcomes, retrospectively reviewed imaging studies to assess for maximum tumour standardised uptake value (SUV) to mean liver SUV ratio (SUVTLR) and maximum porta hepatis SUV to mean liver SUV ratio (SUVPLR). ResultsBetween April 2007 and December 2020, a total of 204 patients underwent surgical intervention for PMP. Of these, 124 (60.8%) met the inclusion criteria. Median peritoneal carcinomatosis index for the entire cohort was 9 and complete cytoreduction (CC0/1) was achieved in 109 (88%) patients. Patients with high-grade PMP were more likely to have diffuse peritoneal disease (p < 0.001) and higher SUVTLR (p<0.001). The area under the ROC curve (AUC) of SUVTLR in predicting high-grade pathology was 71% (p = 0.003). Patients with a SUVTLR ≤ 0.78 had improved disease-free survival (p = 0.042). ConclusionPreoperative PET showed positive correlation with high-grade PMP and acceptable sensitivity and specificity as a diagnostic tool. PET should be considered a useful adjunct to standard imaging for predicting histological grade in the staging of patients with PMP.

A Rare and Aggressive Lymphoma; Burkitt Lymphoma in the Terminal Ileum

Burkitt Lymphoma (BL) is an aggressive form of high-grade B-cell neoplasm that is rarely identified in the adult population. The World Health Organization (WHO) describes three clinical variants; endemic, sporadic, and immunodeficiency associated.The following case is a rare presentation of Primary small intestine Burkitt lymphoma in an adult male presenting with fatigue, dyspnea on exertion, and unexplained weight loss. We present a case of an 81-year-old male with a past medical history of stage I Hodgkin's lymphoma treated with chemotherapy(may be state chemo regime) approximately 8 years ago, non-insulin-dependent diabetes, hypertension, hyperlipidemia, chronic kidney disease who presented to our a tertiary center with the complaints of increasing weakness, fatigue, and unexplained weight loss. In the emergency department the patient was found to have acute kidney injury and hypercalcemia 14.1mg/dL (Ca 8.4 - 10.3 mg/dL). Due to concern for malignancy with hypercalcemia and the patient's history of Hodgkin's lymphoma a computed tomography (CT) chest, abdomen and pelvis was obtained. Imaging revealed concerning findings of peritoneal carcinomatosis with gastrointestinal malignancy as possible source. Gastroenterology was consulted and an EGD as well as colonoscopy were performed.Colonoscopy revealed diverticulosis in the sigmoid colon and in the descending colon, a malignant-appearing tumor in the distal ileum that was biopsied. Pathology revealed an aggressive B-cell lymphoma with Burkitt like morphology and a germinal center phenotype. The infiltrate was positive for CD 20, CD 10, BCL 2, BCL 6, and MUM1. The Ki 67 proliferation rate was greater than 90%. Overall, the morphology and high proliferation rate were consistent with Burkitt lymphoma. The patient chose to follow up at an alternative facility for his ongoing oncology care. Burkitt lymphoma is a very aggressive form of B-cell lymphoma the sporadic form occurring worldwide with a predilection for children. Clinical symptoms depend on the site of involvement. There is frequently extranodal involvement commonly occurring in the gastrointestinal tract. CT findings can include peritoneal thickening, as well as ascites, and omental involvement. Often times when there is diffuse peritoneal disease, it is usually associated with a focal gastrointestinal mass. Although rare, Burkitt lymphoma should be kept on the differential when investigating CT findings of what appears to be peritoneal carcinomatosis.2530_A Figure 1. peritoneal carcinomatosis2530_B Figure 2. Malignant appearing tumor in the distal ileum

Perplexing Malignant Peritoneal Mesothelioma in a Young Woman Without Exposure

Introduction: Malignant mesothelioma is a rare disease that most often occurs in the pleura as a result of environmental exposure. Malignant peritoneal mesothelioma (MPM) is an even less common disease, accounting for approximately 10-15% of all cases of mesothelioma. Interestingly, MPM occurs more often in females than pleural mesotheliomas do for reasons that are not entirely understood. This higher proportion of females is thought to reflect their higher incidence of non-asbestos related disease. We report the case of a young woman with no known environmental exposure history who was diagnosed with MPM. Case Report: A healthy 20-year-old woman presented with dehydration and malnutrition. She reported a 2 month history of nausea, vomiting, diarrhea and abdominal pain. She also endorsed anorexia and an associated 20-30 pound weight loss over the same period. She had presented to her local emergency department 3 times with these complaints and had been found to have small pockets of ascites as well as hepatic sinusoidal dilatation on ultrasound. Additional workup including CT imaging, liver biopsy, paracentesis, colonoscopy and upper endoscopy had failed to identify any pathology. An MRI showed extensive soft tissue implants and omental caking in the abdomen and pelvis with abnormal peritoneal thickening and enhancement. The patient underwent a CT-guided biopsy of the omental soft tissue, which demonstrated a mesothelioma of epithelioid type. She was treated with peritonectomy, omentectomy, small bowel resection, total abdominal hysterectomy and installation and circulation of hyperthermic intraperitoneal chemotherapy. Biopsies of all sampled tissue showed extensive involvement by MPM. Of note, extensive imaging and evaluation did not capture any pleural involvement. Discussion: MPM is an exceedingly rare disease. Although previous studies have demonstrated an association between MPM and asbestos exposure in men, the same association has not been deduced in female populations. Other contributing exposures that have been described include radiation, chronic peritonitis, mica exposure and the simian virus-40. Both this patient's young age and lack of any such exposures reinforce the prevailing current wisdom that the risk factors for this rare, yet highly deadly disease have yet to be properly defined.Figure: A) Coronal MRI revealing broad sheet of omental caking along the left abdomen extending into the pelvis and diffuse peritoneal thickening and enhancement both along the abdominal wall and the bowel surfaces. Overall findings are suspicious for peritoneal malignancy. B) Coronal MRI revealing overall reduction in diffuse peritoneal disease burden with resolution of omental caking after therapy.

Radiological predictors of complete cytoreduction in 59 patients with peritoneal mesothelioma treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy at a UK referral centre.

To assess the imaging features of peritoneal mesothelioma and identify key anatomical sites that aid patient selection for complete cytoreduction. Pre-operative imaging of 59 (32 males, 27 females) patients who underwent cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) for histologically proven peritoneal mesothelioma [36 malignant peritoneal mesothelioma, 23 cystic mesothelioma were reviewed. Imaging findings were correlated with surgical outcome. Best imaging predictors of complete cytoreduction, n = 22 and major tumour debulking, n = 12 were assessed. Most patients (88.9%) had diffuse peritoneal disease with mean radiological peritoneal cancer index of 18 ± 12 (range 2-39). Disease in the lesser omentum (n = 10), porta hepatis (n = 8), perigastric area (n = 5), mesentery (n = 25), small bowel (n = 17), hydronephrosis (n = 1), concurrent pleural disease (n = 2), lymph nodes (n = 1) and abdominal wall disease (n = 4) was considered unfavourable. While 78.9% of patients who underwent complete cytoreduction had no disease at unfavourable sites, 75% of those who underwent MTD did have disease at these sites. There was significant difference in the radiological peritoneal cancer index, severity of upper abdominal disease, small bowel and mesenteric involvement between patients who underwent complete cytoreduction and MTD for malignant peritoneal mesothelioma. Complete cytoreduction was not achieved in the presence of a rind of soft tissue around the small bowel (p = 0.016) and was unlikely in the presence of large volume upper abdominal disease (p = 0.06). Involvement of key anatomical sites such as small bowel serosa and large volume upper abdominal disease reduced the likelihood of achieving complete cytoreduction in patients with malignant peritoneal mesothelioma. Advances in knowledge: Demonstration of small bowel disease and large volume upper abdominal disease on imaging in patients with malignant peritoneal mesothelioma can be used to identify patients who may not benefit from cytoreductive surgery.

Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in pediatric ovarian tumors: a novel treatment approach.

Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have been used in adults with ovarian carcinoma proving overall survival benefit in randomized trials, but measured in months. Diffuse peritoneal disease from pediatric type ovarian tumors is rare. We applied CRS and HIPEC to pediatric girls with diffuse peritoneal disease as part of a clinical trial. In all patients complete cytoreduction was followed by HIPEC using 100 mg/m2 of cisplatin for 90 min in a closed technique. All received neoadjuvant chemotherapy. Patients with disease outside of the abdominal cavity were excluded. Of 101 pediatric CRS and HIPEC operations, 8 had ovarian primary tumors and multifocal peritoneal disease. There were three yolk sac tumors (germ cell, mixed teratoma), one Sertoli–Leydig, one PNET of the ovary, one choriocarcinoma, one juvenile granulosa cell tumor and one adenocarcinoma. Age ranged 4–18 years. Three of the 8 (37 %) recurred and died. The remaining 63 % are disease free 2–6 years post HIPEC. Overall survival and relapse-free survival in this cohort was 64 and 62 %, respectively [CI 0.64 (0.34, 1); 0.62 (0.37, 1)]. This is the first report of CRS and HIPEC in pediatric ovarian tumors. HIPEC may be effective in pediatric-type ovarian tumors. More study is needed in a larger cohort.

Carcinoid of the Duodenal Bulb: A Rare Occurrence

Introduction: The incidence of neuroendocrine tumors has increased significantly in the last few decades. Carcinoid tumor, a subtype of neuroendocrine tumors, arises from the enterochromaffin cells of the epithelial lining of the gastrointestinal tract, retroperitoneum and large bronchi. Duodenal carcinoids are extremely rare and can present with a variety of symptoms including epigastric pain, vomiting, anemia, and weight loss. We report a rare case of a patient who presented with iron deficiency anemia and was found to have multiple carcinoids of the duodenal bulb. Case: 60 year-old male presented to his primary care physician's office to establish care and reported a history of GERD for which he was taking Omeprazole 20 mg daily in the morning. He underwent routine laboratory investigations revealing iron deficiency anemia which prompted the need for an endoscopy. His initial EGD showed a 5 mm duodenal bulb polyp with biopsies showing changes suggestive of a hyperplastic polyp. Given his persistent anemia and an inconclusive workup otherwise including a colonoscopy, he underwent a second-look EGD after six months which showed two submucosal duodenal bulb lesions, 15 mm and 5 mm, both of which were biopsied with cold biopsy forceps and showed a neoplasm formed by nests of cells with moderately clear-to-pale eosinophilic cytoplasm and nuclei with finely stippled chromatin. A chromogranin stain was performed and was positive, thereby rendering a diagnosis of a carcinoid tumor. He underwent an octreoscan and a CT scan which showed no metastatic disease. The patient underwent a Billroth procedure. Intra-operatively, no evidence of diffuse peritoneal disease or metastases to the intra-abdominal viscera was observed. The liver did not have any palpable masses. He tolerated the procedure well and was discharged home with a surveillance EGD showing no evidence of recurrence. Discussion: Duodenal carcinoids, although rare, should be kept in the differential diagnosis of the polypoid lesions of the duodenum and may occasionally cause anemia. Two duodenal carcinoids occurring concomitantly and presenting as iron deficiency anemia is extremely rare. These lesions typically have a low metastatic potential and have a good curative rate if localized and diagnosed early.

Solid tumors of the peritoneum, omentum, and mesentery in children: radiologic-pathologic correlation: from the radiologic pathology archives.

Intraperitoneal solid tumors are far less common in children than in adults, and the histologic spectrum of neoplasms of the peritoneum and its specialized folds in young patients differs from that in older patients. Localized masses may be caused by inflammatory myofibroblastic tumor, Castleman disease, mesenteric fibromatosis, or other mesenchymal masses. Inflammatory myofibroblastic tumor is a mesenchymal tumor of borderline biologic potential that appears as a solitary circumscribed mass, possibly with central calcification. Castleman disease is an idiopathic lymphoproliferative disorder that appears as a circumscribed, intensely enhancing mass in the mesentery. Mesenteric fibromatosis, or intra-abdominal desmoid tumor, is a benign tumor of mesenchymal origin associated with familial adenomatous polyposis. Mesenteric fibromatosis appears as a mildly enhancing, circumscribed solitary mass without metastases. Diffuse peritoneal disease may be due to desmoplastic small round cell tumor (DSRCT), non-Hodgkin lymphoma, or rhabdomyosarcoma. DSRCT is a rare member of the small round blue cell tumor family that causes diffuse peritoneal masses without a visible primary tumor. A dominant mass is typically found in the retrovesical space. Burkitt lymphoma is a pediatric tumor that manifests with extensive disease because of its short doubling time. The bowel and adjacent mesentery are commonly involved. Rhabdomyosarcoma may arise as a primary tumor of the omentum or may spread from a primary tumor in the bladder, prostate, or scrotum. Knowledge of this spectrum of disease allows the radiologist to provide an appropriate differential diagnosis and suggest proper patient management.

Overexpression of the Six1 Homeobox Gene Is Associated with Diffuse Peritoneal Spread and Larger Residual Disease after Maximal Cytoreductive Effort in Advanced Ovarian Cancer

Study Design: Between January 2003 and June 2009, we collected fresh tumor and extracted highquality RNA from the omental/peritoneal metastases of 47 patients with stage IIB-IV ovarian cancer. Clinical data were abstracted from the patients’ medical records. Expression of Six1 level by quantitative RT-PCR was compared with preoperative factors and intraoperative findings using the χ2 test and the Fisher exact test. The effect of Six1 elevation on survival was assessed with the Kaplan/Meier method. Results: The mean age of patients enrolled was 60 (range 33 - 84). The histological subtypes were 77% serous (36/47), 11% endometrioid (5/47), 4% mucinous (2/47), and 4% clear cell (2/47). Eighty-one percent were optimally cytoreduced. Median Six1 expression for the samples was 114 fg/ng 18S rRNA and Six1 overexpression, defined as >300 fg/ng 18S rRNA, was observed in 19% of tumors. Six1 expression above sample median was associated with peritoneal disease (p = 0.049) and inability to optimally cytoreduce (p = 0.02). Six1 overexpression was associated with worsened survival in the high grade serous subgroup (43 months versus 71 months, p = 0.039 Log Rank test). Conclusions: Elevated levels of Six1 predict peritoneal disease * Corresponding author.

Laparoscopic resection of paraaortic or paracaval lesions: feasibility and outcome

Laparoscopic retroperitoneal lymph node dissection of paraaortic and paracaval lymph nodes is used to stage nonseminomatous germ cell tumors. Primary tumors can arise from the retroperitoneum, and tumors from nonurologic malignancy also may metastasize to retroperitoneal lymph nodes. This study aimed to describe the authors' experience with laparoscopic resection of these lesions. A consecutive series of patients between January 2007 and June 2011 with paraaortic, aortocaval, or paracaval tumors with a maximum diameter smaller than 10 cm and confined to the abdomen were considered for laparoscopic resection. Data were collected on size and pathology of the lesions, anesthesia time, postoperative stay, and postoperative morbidity and mortality. In this study, 25 patients with a median age of 49 years were assessed for laparoscopic resection. Eight patients were considered unsuitable for a laparoscopic approach because of tumor location (n = 5), previous retroperitoneal surgery (n = 1), stoma (n = 1), or lesion not clearly visible on computed tomography (n = 1). Of the 17 patients undergoing laparoscopic resection, 1 was found to have diffuse peritoneal disease at laparoscopy, whereas another was converted to an open procedure due to bleeding. All the laparoscopic patients had an R0 resection. The median hospital stay was significantly shorter in the laparoscopic group (2 days) than in the open group (6 days) (P = 0.009). One patient in the laparoscopic group with a functioning paraganglioma and advanced cardiac disease died on postoperative day 7. Laparoscopic paraaortic and paracaval surgery for primary and recurrent tumors of the retroperitoneum is feasible, with clear resection margin rates similar to that observed for open surgery.

Ovarian Carcinomatosis: How the Radiologist Can Help Plan the Surgical Approach

Ovarian carcinoma is the most common cause of death due to gynecologic malignancy. Peritoneal involvement is present in approximately 70% of patients at the time of initial diagnosis. The disease spreads abdominally by direct extension, exfoliation of tumor cells into the peritoneal space, and dissemination of tumor cells along lymphatic pathways. Carcinomatosis characterizes an advanced stage of disease in which peritoneal disease has spread throughout the upper abdomen (stage IIIC) or in which diffuse peritoneal disease is accompanied by malignant pleural infiltration or visceral metastases (stage IV). Common sites of intraperitoneal seeding of ovarian carcinoma include the pelvis, omentum, paracolic gutters, liver capsule, and diaphragm. Soft-tissue thickening, nodularity, and enhancement are all signs of peritoneal involvement. Advanced-stage disease is treated either with initial cytoreductive surgery (debulking) followed by adjuvant chemotherapy, or with initial neoadjuvant chemotherapy followed by debulking. Radiologic imaging plays an important role in the selection of patients who may benefit from neoadjuvant chemotherapy before debulking. However, accurate interpretation of the imaging findings is challenging and requires a detailed knowledge of the complex peritoneal anatomy, directionality of flow of peritoneal fluid, and specific disease sites that are likely to present particular difficulties with regard to surgical access and technique. Although there is as yet no clear consensus on the criteria for resectability of peritoneal lesions, extensive involvement of the small bowel or mesenteric root, involved lymph nodes superior to the celiac axis, pleural infiltration, pelvic sidewall invasion, bladder trigone involvement, and hepatic parenchymal metastases or implants near the right hepatic vein are considered indicative of potential nonresectability. Implants larger than 2 cm in diameter in the diaphragm, lesser sac, porta hepatis, intersegmental fissure, gallbladder fossa, or gastrosplenic or gastrohepatic ligament also may represent nonresectable disease.

Morbidity, mortality and overall survival in elderly women undergoing primary surgical debulking for ovarian cancer: A delicate balance requiring individualization

ObjectiveTo assess outcomes and identify underlying predictors of outcomes in a cohort of women over the age of 65 treated for primary ovarian cancer (OC). MethodsConsecutive patients ≥65 with stage IIIC or IV OC treated with primary surgery and adjuvant chemotherapy at Mayo Clinic between January 1, 1994 and December 31, 2004 were retrospectively assessed. We analyzed the impact of perioperative factors (age, albumin, CA125, American Society of Anesthesiologist (ASA) score, amount of ascites, presence of carcinomatosis, creatinine, need for urgent surgery, stage of disease, surgical complexity score and amount of residual disease) on surgical outcomes (morbidity, mortality, overall survival (OS) and ability to receive chemotherapy). ResultsTwo hundred eighty patients met inclusion criteria. Age was associated with higher ASA score, lower albumin, and higher creatinine; stage, diffuse peritoneal disease, and surgical complexity were not associated with age. Median OS decreased with increasing age and residual disease (RD), and the impact of RD was greater on older patients. All patients benefited similarly when RD=0 [median OS 5.9years for age 65–69 vs. 5.0years in those ≥80 (p=0.5516)], for RD<1cm, and OS was 3.4 vs. 2.1years respectively for youngest vs. oldest patients (p=0.068). Perioperative morbidity was observed in 37.5% of patients ≥75. Independent predictors of poor perioperative outcome included preoperative albumin ≤3g/dL, urgent surgery, age, and stage (p<0.05). Independent predictors of overall survival included creatinine, albumin, surgical complexity score, amount of residual disease, stage and age. ConclusionAge is an independent predictor of OS in OC. A significant number of elderly women are able to undergo a complete cytoreduction and experience OS similar to that of younger patients. However, the benefits to incomplete cytoreduction are less clear in women ≥75. These observations highlight the need to use emerging predictors of outcomes in decision making and to focus care in centers able to render patients with no visible residual disease.

Intraperitoneal Treatment for Peritoneal Mucinous Carcinomatosis of Appendiceal Origin After Operative Management

To determine prognostic factors and the impact of intraperitoneal (IP) treatment after surgical resection of peritoneal mucinous carcinomatosis (PMC) of appendiceal origin. PMC is a rare, malignant, intra-abdominal neoplasm that produces large amounts of mucin. Patients typically present with diffuse peritoneal disease. After surgical treatment, multiple locoregional recurrences are common; recurrences outside the abdomen are infrequent. Treatment regimens include debulking, radiotherapy with IP radioisotopes, and chemotherapies (IP, systemic, or both). Because reported data are variable and heterogeneous, treatment evaluations are challenging. We retrospectively reviewed 115 consecutive patients with PMC who underwent maximal surgical resection with or without postoperative therapy between 1985 and 2000 at Mayo Clinic Rochester. After maximal resection, 37 patients received IP 5-fluorouracil, 35 of whom also received IP chromic phosphate P 32. The Kaplan-Meier method was used to estimate overall survival (OS) and disease-free survival. All gross disease was removed in 61% of patients. With a median follow-up of 6.1 years, the median OS was 8.1 years. Median OS for patients receiving versus not receiving IP therapy was 23.5 years versus 7.5 years, respectively. The 5-, 10-, and 15-year OS for those receiving and not receiving IP therapy was 82%, 65%, and 52% versus 60%, 27%, and 15%, respectively. Adverse prognostic factors for OS identified by univariate analysis included partial mucin debulking, adenocarcinoma histology, systemic chemotherapy, diffuse IP disease at presentation, and no IP therapy. On multivariate analysis, diffuse IP disease at presentation and no IP therapy remained significant. A separate analysis was performed for the 70 patients who underwent gross total resection, 51% of whom received IP therapy. Adverse prognostic factors for OS included adenocarcinoma histology, systemic chemotherapy, and no IP therapy. This large, single-institution, retrospective series with long-term follow-up suggests that IP chromic phosphate P 32 and 5-fluorouracil after maximal surgical resection of PMC of appendiceal origin is associated with improved OS and disease-free survival.

Prognostic Significance of Localized Extra-appendiceal Mucin Deposition in Appendiceal Mucinous Neoplasms

Appendiceal mucinous neoplasms confined to the mucosa are benign, whereas those with disseminated peritoneal mucin deposits often follow an indolent, but malignant, course. Not infrequently, appendiceal mucinous neoplasms are associated with localized periappendiceal mucin deposits, but lack diffuse peritoneal involvement. Mucin deposits in these cases may be acellular or contain neoplastic epithelium (cellular mucin). Although some investigators consider both acellular and cellular periappendiceal mucin to pose no, or minimal, risk for recurrent disease, the biologic importance of localized extra-appendiceal mucin has never been evaluated. We identified 65 patients with appendiceal mucinous neoplasms, all of whom had localized periappendiceal mucin deposits without diffuse peritoneal involvement, and assessed them for the presence of extra-appendiceal epithelium and clinical outcome. Forty-nine (75%) appendices were submitted in total for histologic evaluation. Most (77%) cases showed acellular periappendiceal mucin, but 15 (23%) had scant extra-appendiceal epithelium (range: 1 to 12 cell clusters). Upon follow-up (mean: 48 mo), 2 (4%) patients with acellular periappendiceal mucin developed diffuse peritoneal disease, but neither of these appendices was submitted in total for histologic evaluation. In contrast, 5 of 15 (33%) patients with cellular periappendiceal mucin developed mucinous ascites, including 1 who eventually died of disease (P=0.03). Thus, patients with appendiceal mucinous neoplasms and acellular periappendiceal mucin are unlikely to develop recurrent disease. However, microscopic examination of the entire appendix is necessary, as lesions with extra-appendiceal tumor cells are more likely to progress to disseminated disease and result in death of the patient, even if the mucin is paucicellular and confined to the periappendiceal region.

Primary Peritoneal Tumors: Imaging Features with Pathologic Correlation

Primary peritoneal tumors are uncommon lesions that arise from the mesothelial or submesothelial layers of the peritoneum. Primary malignant mesothelioma, multicystic mesothelioma, primary peritoneal serous carcinoma, leiomyomatosis peritonealis disseminata, and desmoplastic small round cell tumor are the most prominent of these rare lesions. Primary malignant mesothelioma is a highly aggressive malignancy that occurs most commonly in older men and that has a strong association with high levels of asbestos exposure. It manifests most often as diffuse sheetlike or nodular thickening of the peritoneal surfaces, but it may occasionally be a localized mass. Multicystic mesothelioma occurs most frequently in women and has benign or indolent biologic behavior in the majority of patients. It is a multilocular cystic mass that arises from the pelvic peritoneal surfaces. Primary peritoneal serous carcinoma occurs almost exclusively in women. It is histologically identical to ovarian serous carcinoma and may be indistinguishable from metastatic ovarian carcinoma at imaging studies. Leiomyomatosis peritonealis disseminata is a rare, benign proliferative process that also occurs exclusively in women and is characterized by multiple smooth muscle nodules throughout the peritoneum. Desmoplastic small round cell tumor is a highly aggressive malignancy of unknown origin that occurs most often in the peritoneal cavity of young men. This unusual group of tumors is linked together by a common site of origin and imaging manifestations that mimic those of peritoneal carcinomatosis. Knowledge of the spectrum of imaging findings in this group of primary peritoneal tumors, along with their clinical and pathologic characteristics, is important in the evaluation of patients with diffuse peritoneal disease.

Peritoneal mesothelioma presenting as an acute surgical abdomen due to jejunal perforation

Peritoneal mesothelioma is a rare disease associated with poor prognosis. Acute abdomen as the first presentation is an extremely rare occurrence. We report an exceptional case of a patient who was found to have a jejunal perforation due to infiltration of peritoneal mesothelioma. A 62-year-old man was admitted with clinical signs of peritonitis. Computerized tomographic scans showed a mass distal to the ligament of Treitz, thickening of the mesentery and a small amount of ascites. Emergency laparotomy revealed a perforated tumor 15 cm distal to the ligament of Treitz and diffuse peritoneal disease. Segmental small bowel resection and suboptimal cytoreduction were performed. Histopathology and immunohistochemistry showed infiltration of malignant mesothelioma. During the postoperative period pleural mesothelioma was also diagnosed. Despite adjuvant chemotherapy, the patient died of disseminated progressive disease 7 months after surgery. Peritoneal mesothelioma is a rare malignancy with grim prognosis. Small bowel involvement is a poor prognostic indicator. Our case of a small bowel perforation due to direct infiltration by peritoneal mesothelioma appears to be the first reported in the English literature.

Carcinoma papilar seroso peritoneal primario. Presentación de 12 casos

Objective The purpose of this study was to investigate the clinical findings, treatment and outcome of Primary Peritoneal papillary serous carcinoma in 12 patients admitted to Miguel Servet hospital between June 30, 1999, and June 30, 2004. Material and methods This is an uncommon disease characterized by peritoneal carcinomatosis without other identifiable primary tumor; it typically presents resembling ovarian cancer, with abdominal pain and distention and in an advanced stage. Results They were managed by surgical exploration, tumor debulking where possible and postoperative chemotherapy. Prognosis is poor. Conclusions the presence of diffuse peritoneal disease and the absence of an ovarian mass on CT and an elevation of serum CA 125 level are suggestive of Primary Peritoneal papillary serous carcinoma.

Radiotherapy Following Extrapleural Pneumonectomy(EPP) for Mesothelioma: The Brigham and Women’s Hospital Experience

Purpose/Objective: We have previously published the world’s largest series of patients undergoing EPP for malignant pleural mesothelioma (MPM). (Sugarbaker et al. JCTS 99’) Building on those results we have published the patterns of failure of patients treated with low dose hemithoracic radiation of 30-40Gy (Baldini et al. Ann Thor Surg 97’) demonstrating a benefit to post-operative RT but with a high local failure rate of 65%. Following that publication, two attempts at the BWH/DFCI have been made to improve local control. The first was moderate dose RT 40Gy with concurrent platinum based chemotherapy and the second was high dose hemithoracic RT alone to 54Gy as per the technique of Yajnik et al.(IJROBP 03′) from MSKCC. We report the treatment details and outcome of those patients. Materials/Methods: Between 1999 and 2004, 68 patients were referred to our department following EPP for adjuvant therapy. We reviewed their treatment as part of an IRB approved study. Forty-three patients were treated closer to home and twenty-five patients received adjuvant RT @ BWH/DFCI. Of the 25 patients Median age was 57 years (44–75). 19 patients were male and six were female. 16 patients had epithelial histology with seven patients having mixed and two patients with sarcomatoid histology. Brigham stage 1 in 11 patients, ten had stage 2 and four stage 3. Ten patients were treated with APPA fields to 30Gy to the entire hemithorax with a limited chest wall boost to 40Gy and any grossly positive nodes were boosted to 54Gy. All of these patients received concurrent cisplatin chemotherapy at 50mg/m2 weekly. A second cohort of patients was treated with high dose RT to 54 Gy as per the study of Yajnik et al. The high dose cohort were treated with either sequential adjuvant,(5 patients with pemetrexed based), or neoadjuvant, (9 patients with gemcitabine and platinum)chemotherapy. Results: With a median potential follow-up of 30 months, the median survival of the entire group is 17.5 (95% CI 12.3–22.8) months. Eight patients (32%) remain alive and disease free, seven of these patients were treated with high dose hemithoracic RT and sequential chemotherapy. All had epithelial histology, and 3/8 were node positive. Of the remaining 17 patients who have experienced disease failure, 6 patients have experience death from distant failure alone, 5/6 with diffuse peritoneal disease. 11/25 (44%) patients have had documented local failure. Three patients have experience local only failure 2/3 died of local recurrence in the ipsilateral mediastinum alone. All three failures were in the radiation field. Eight patients experienced local and distant failure with in-field local failure occurring as the first site of failure in 6/8 patients. Of the eleven local in-field failures all were in an areas of the chest that received 30Gy or less because of normal tissue constraints. Conclusions: High dose hemithoracic radiation appears to benefit patients with favorable histology following EPP for MPM. In-field local failure remains a significant obstacle to improvement in outcome for these patients. Alternatives such as IMRT, intraoperative chemotherapy and concurrent RT and pemetrexed regimens are being pursued.

From the archives of the AFIP: radiologic staging of ovarian carcinoma with pathologic correlation.

Ovarian cancer is the deadliest gynecologic malignancy, with approximately 70% of patients having peritoneal involvement at the time of diagnosis. It spreads predominantly by direct invasion and intraperitoneal dissemination. The staging system is surgically based, with stage I disease being limited to one or both ovaries. In stage II disease, there is extraovarian spread of tumor, but it does not extend beyond the pelvis. Stages III and IV disease are considered advanced, with stage III ovarian cancer including diffuse peritoneal disease involving the upper abdomen and stage IV disease having distant metastases including hepatic lesions. Common sites of intraperitoneal seeding include the omentum, paracolic gutters, liver capsule, and diaphragm. Thickening, nodularity, and enhancement are all signs of peritoneal involvement. Although computed tomography is the most common imaging modality used to stage ovarian cancer, magnetic resonance imaging has been shown to be equally accurate. Currently, however, no imaging modality allows microscopic spread of disease to be ruled out, and a full staging laparotomy is always required. Early ovarian cancer is treated with comprehensive staging laparotomy, whereas advanced but operable disease is treated with primary cytoreductive surgery (debulking) followed by adjuvant chemotherapy. Patients with unresectable disease may benefit from neoadjuvant (preoperative) chemotherapy before debulking.