Gliomatosis peritonei

Gliomatosis peritonei, the occurrence of mature glial peritoneal implants, is a rare complication that is observed almost exclusively in the setting of ovarian teratoma. The clinicopathologic features of four such cases associated with ovarian teratoma of all grades are described herein. Gliomatosis peritonei is a benign condition. Although its presence is believed to suggest a more favorable prognosis in cases of high-grade ovarian teratoma, this was not found in one of our cases. Extensive histopathologic sampling of peritoneal implants is essential to exclude the presence of other teratomatous or germ cell elements, a negative factor in prognosis.     

Gliomatosis peritonei

Gliomatosis peritonei, the miliary implants of mature glial tissues on the peritoneum or omentum, is a rare complication of solid ovarian teratoma. Our case is reported and 38 previously reported cases are reviewed. The grade of the primary tumors varied from grade 0 to grade 3. Only five cases were composed entirely of mature tissues. Five of the 39 patients died. Despite of varied therapy, the rest of the patients were alive from 3 months to 38 years later. Inspite of intraperitoneal implants, the prognosis in patients with these tumors is good, irrespective of the mode of therapy. On the basis of this study, we recommended a conservative therapy for the primary tumor and therapy for the implants is not required.     

Ovarian teratoma with peritoneal and lymph node metastases of mature glial tissue: a benign condition

The presence of widespread intraperitoneal metastases in patients with ovarian tumors usually indicates advanced disease. However, this is not always the case. A rare type of ovarian tumor, ie, mature solid teratoma, may be associated with intraabdominal metastases of mature glial tissue (gliomatosis peritonei). In spite of such metastases, the prognosis in patients with this tumor is excellent, regardless of the mode of therapy. In these patients radical surgical treatment, eg, bilateral salpingo-oophorectomy, is unnecessarily destructive, and therapy for the metastases is not required.     

Pseudomyxoma peritonei--diagnosis and treatment

Pseudomyxoma peritonei is an unusual condition in which diffuse collections of gelatinous fluid are associated with mucinous implants on the peritoneal surfaces and omentum. It is now generally accepted that pseudomyxoma peritonei is due to neoplastic mucus-secreting cells within the peritoneal cavity. The diagnosis is not difficult before surgery, owing to the development of radiologic imaging studies. This disease is a malignancy that, even after excision of the metastatic areas, has a poor prognosis, since no effective therapy has yet been established besides debulking surgery.     

Growing Teratoma Syndrome with Synchronous Gliomatosis Peritonei during Chemotherapy in Ovarian Immature Teratoma: A Case Report and Literature Review

Coexistent growing teratoma syndrome (GTS) and gliomatosis peritonei (GP) arising during chemotherapy of ovarian immature teratoma (IMT) is extremely rare and can be misdiagnosed as recurrent or progressive disease. We present a 33-year-old woman diagnosed with GTS with synchronous GP during chemotherapy of IMT. She underwent ovarian cystectomy due to ovarian immature teratoma and chemotherapy were administered. The α-fetoprotein (AFP) concentration decreased from 28.7 ng/mL to normal after the second cycle. Four days after the third cycle of chemotherapy, ultrasound and CT revealed an 8-cm mass with negative tumor markers in the pouch of Douglas. An exploratory laparotomy was conducted, and a smooth round cystic-solid 8-cm mass was noted in the pouch of Douglas. Extensive peritoneal seeding glial nodules were also observed on the surface of the uterus, peritoneum, and omentum. The patient underwent a partial omentectomy, intact resection of the tumor, and resection of most of the glial nodules. Postoperative pathology demonstrated a pure mature cystic teratoma component in the mass, as well as diffuse GP involving the uterine serosa, peritoneum, and omentum; this diagnosis of GTS with synchorous GP should be considered in IMT patients with mass newly identified during chemotherapy while tumor markers are normal after treatment.     

Molecular analysis of an immature ovarian teratoma with gliomatosis peritonei and recurrence suggests genetic independence of multiple tumors

Immature ovarian teratoma is a common germ cell tumor of young women. Patients with immature teratoma often exhibit multiple neoplasms, including tumors outside the ovaries, and occasionally a rare benign condition termed gliomatosis peritonei (GP). These multiple neoplasms are generally believed genetically-linked progeny of the ovarian tumor resulting from local recurrence/spread. In this study, we performed a molecular analysis of a single patient clinically diagnosed with immature ovarian teratoma, GP, and recurrent pelvic mucinous teratoma. Microsatellite PCR and amplicon analysis was performed to genetically characterize tissue samples from omental glial implants and multiple peritoneal tumors. PCR-based amplification of microsatellite markers identifies unique genetic differences (allelic variation) between tumors resulting from divergent natural histories among multiple tumor nodules in a single patient. A total of 21 different microsatellite markers were employed, and seven provided informative results (D3S1744, D6S1056, D7S2846, D14S306, D16S764, D18S858, D22S420). These markers demonstrated mutually exclusive genetic differences among the tumors from this patient, establishing the neoplasms as genetically distinct from each other (non-identical), and that no lineage relationship exists among them. This observation suggests that the multiple tumors arising in this patient with immature ovarian teratoma, GP, and recurrent pelvic mucinous neoplasm represent multiple independent tumors rather than true tumor recurrence/spread. The results of this study suggest strongly that patients with recurrent teratoma may be afflicted with a tumor-prone syndrome where one or more peritoneal cell types or populations are predisposed to neoplastic conversion and formation of tumors as a result of an endogenous or exogenous neoplastic stimuli.     

Unusual origins of Pseudomyxoma peritonei

Pseudomyxoma peritonei is a rare neoplastic condition in that gelatinous intraperitoneal fluid collections and mucinous implants on the peritoneal surfaces and omentum are found. The pathological origin is usually an adenoma or well-differentiated adenocarcinoma of the appendix. A smaller number of cases arises from ovarian tumors. We report two unusual cases of pseudomyxoma peritonei. As a child, the first patient underwent several surgical procedures of the large bowel to relieve the consequences of Hirschsprung disease that were complicated by recurrent enteric fistulae. Colonic epithelial cells, with neoplastic changes due to chronic inflammation in the presence of enteric fistulae, were probably dislocated during these episodes, causing pseudomyxoma peritonei. In the second patient, pseudomyxoma was caused by intraperitoneal seeding of a mucinous urachal adenocarcinoma. We hypothesize that seeding of mucus producing epithelial cells into the abdominal cavity may result in this rare entity called pseudomyxoma peritonei, regardless of the source.     

Pseudomyxoma peritonei

Pseudomyxoma peritonei is a rare form of mucinous ascites associated with peritoneal and omental implants. The origin is controversial, and recent immunohistochemical and molecular genetic evidence suggests the appendix to be the likely site. The condition often presents as an incidental finding at laparotomy. Ultrasonography, computed tomography and magnetic resonance imaging aid in preoperative diagnosis. Treatment remains controversial, surgery being the main stay. The role of intraperitoneal and systemic chemotherapy is poorly defined. We review the literature on the pathology, clinical features and treatment options in pseudomyxoma peritonei.     

大脑胶质瘤病研究进展

大脑胶质瘤病是中枢神经系统一种少见的恶性疾病,以广泛浸润大脑半球多个脑叶为特点,有多种组织病理学分型,各类型之间各有特点。该病尚未被深入认识,在发病机制和治疗等方面仍未研究清楚。本文就大脑胶质瘤病的相关最新研究进展展开综述,以供广大医护人员在临床实践中参考和借鉴。     

Mature ovarian teratoma with gliomatosis peritonei--a case report

Gliomatosis peritonei (GP), a rare condition related to ovarian teratomas, is characterized by miliary implants of mature glial tissues on the peritoneum or omentum. We report herein a case of mature teratoma of the ovary with GP with imaging features and pathological correlation.     

Extraperitoneal presentation of pseudomyxoma peritonei as retroperitoneal invasion with skin fistula-a case report

Pseudomyxoma peritonei (PMP) is characterized by mucinous ascites and implants throughout the peritoneal cavity. The tumor rarely involves retroperitoneum. We present a patient of PMP with retroperitoneal presentation and spontaneous development of a skin fistula. There had been only 7 such cases reported.     

Borderline epithelial tumours of the ovary--a retrospective analysis of 31 cases

Thirty one cases of epithelial borderline tumours of the ovary recorded over a period of six years were reviewed. The incidence of borderline tumours was 6% in relation to ovarian epithelial malignancies, with serous and mucinous types comprising three fourth of the lesions. The serous tumours were bilateral in 39%, revealed surface growth in 17% and had peritoneal implants in 11% of cases. The mucinous tumours were bilateral in 11% and had associated pseudomyxoma peritonei in 22% of cases. Nuclear grade appeared to correlate with extraovarian spread and surface growth in the serous borderline tumours, but not in the mucinous borderline tumours. The endometrioid borderline tumours and mixed epithelial borderline tumours were rare lesions. Twenty one patients (68%) presented in Stage-la. Surface growth correlated with recurrences. The prognosis remained good in serous borderline tumours even in the presence of implants as these were non-invasive. The mean disease free survival was 43.03 months. There was no statistical difference in disease free survival of patients with and without implants.     

Pseudomyxoma peritonei of appendiceal origin. The Memorial Sloan-Kettering Cancer Center experience.

BACKGROUND. Pseudomyxoma peritonei is a rare clinical entity in which the peritoneal surfaces and omentum are involved with diffuse gelatinous mucinous implants. It originates from ruptured mucinous tumors of the appendix or ovary. METHODS. The authors examined the experience with 34 patients with pseudomyxoma peritonei seen at Memorial Sloan-Kettering Cancer Center from 1952-1989. Of these, 17 cases were identified to be of appendiceal origin. RESULTS. All patients underwent celiotomy and cytoreduction. The median survival time from diagnosis was 75 months. It was found that long-term survival can be achieved by operation alone. When conditions do recur, chemotherapy may be valuable. CONCLUSIONS. Pseudomyxoma peritonei of appendiceal origin is a rare low-grade malignancy. Initial treatment consists of cytoreduction in an attempt to render the patient locally disease-free. Long-term survival can be obtained by operation alone, even if gross disease is present at the end of the procedure. Systemic chemotherapy should be reserved for patients with proven recurrence.     

Pseudomyxoma peritonei

Pseudomyxoma peritonei (PMP) is an uncommon "borderline malignancy" generally arising from a perforated appendiceal epithelial tumour. Optimal treatment involves a combination of cytoreductive surgery (CRS) with heated intraperitoneal chemotherapy (HIPEC). Controversy persists regarding the pathological classification and its prognostic value. Computed tomography scanning is the optimal preoperative staging technique. Tumour marker elevations correlate with worse prognosis and increased recurrence rates. Following CRS with HIPEC, 5-year survival ranges from 62.5% to 100% for low grade, and 0%-65% for high grade disease. Treatment related morbidity and mortality ranges from 12 to 67.6%, and 0 to 9%, respectively. Surgery and HIPEC are the optimal treatment for PMP which is at best a "borderline" peritoneal malignancy.     

Indikationen für das HIPEC-Verfahren

For decades, there was therapeutic nihilism regarding patients with peritoneal carcinomatosis or primary peritoneal tumors. Development of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemoperfusion (HIPEC) completely changed this attitude. Nowadays, CRS and HIPEC are standards of care for pseudomyxoma peritonei and peritoneal mesothelioma, and are frequently used in secondary peritoneal metastasis. This review article summarizes the currently available publications and results for the different tumor entities, and critically discusses the indications: patients with pseudomyxoma peritonei and peritoneal mesothelioma can be treated with curative intent in cases of complete CRS; they also benefit from incomplete resection and HIPEC. In colorectal cancer, CRS and HIPEC are recommended in patients with a peritoneal cancer index (PCI) < 20. Proactive second-look surgery and HIPEC enable curative control of peritoneal carcinomatosis in patients at a high risk of peritoneal recurrence. In gastric cancer, limited peritoneal metastasis might be treated with CRS and HIPEC in individual patients. Staging laparoscopy is mandatory and has a decisive role in therapy planning.     

Congenital pleuroperitoneal communication in a patient with pseudomyxoma peritonei

BACKGROUND AND OBJECTIVES: Pseudomyxoma peritonei syndrome is a rare disease arising from a perforated appendiceal adenoma. The syndrome is characterized by progressive accumulation of mucinous ascites and tumor within the peritoneal cavity. Direct extension of pseudomyxoma peritonei to the pleural cavity is uncommon and has been associated with surgical penetration of the diaphragm at the time of cytoreduction. METHODS: We review the case of a patient who presented with mucoid peritoneal and pleural fluid consistent with spontaneous pleural spread of pseudomyxoma peritonei. RESULTS: Surgical exploration confirmed direct pleuroperitoneal communication by macroscopic diaphragmatic fenestration. CONCLUSIONS: This is a rare phenomenon. We outline a therapeutic approach to be applied when pleural involvement is suspected in patients with pseudomyxoma peritonei syndrome.     

Comparative analysis of perioperative intraperitoneal chemotherapy regimen in appendiceal and colorectal peritoneal carcinomatosis

Background

Perioperative intraperitoneal chemotherapy (PIC) is delivered by intraoperative hyperthermic intraperitoneal chemotherapy (HIPEC) and early postoperative intraperitoneal chemotherapy (EPIC). The relative survival benefits of each or both regimens are explored in this large series of patients undergoing cytoreduction at a single institution.

Methods

Patients with a complete (CCR0) or optimal (CCR1) cytoreduction who received intraperitoneal chemotherapy for appendiceal and colorectal peritoneal carcinomatosis were included for analysis. PIC regimens were delivered according to the treatment protocol. Standardized statistical analyses were performed.

Results

Of 262 patients, 98 patients (37%) had colorectal peritoneal carcinomatosis, 108 patients (41%) had low-grade pseudomyxoma peritonei and 56 patients (21%) had appendiceal peritoneal carcinomatosis. For pseudomyxoma peritonei, recurrence-free survival (RFS) did not vary with PIC regimen, 5-year survival was 86% in the HIPEC and EPIC group and 64% in the HIPEC or EPIC group (P = 0.070). For appendiceal peritoneal carcinomatosis, RFS and overall survival (OS) did not vary with PIC regimen. For colorectal peritoneal carcinomatosis, the median RFS was 33 months in the HIPEC and EPIC group, 19 months in the HIPEC alone group and 20 months in the EPIC alone group (P = 0.046). OS did not vary with PIC regimen.

Conclusion

From our experience, without compromising the perioperative morbidity and mortality, PIC consisting of HIPEC and EPIC appears to be associated with potential survival benefits of improved OS in pseudomyxoma peritonei and RFS in colorectal peritoneal carcinomatosis.     

Intraperitoneal administration of a combined CDDP, 5-FU and MMC therapy for pseudomyxoma peritonei

Pseudomyxoma peritonei, which is the seeding of the peritoneum by mucin-secreting metastatic deposits and the filling of the peritoneal cavity by these secretions, is a malignancy that even after excision of the metastatic areas has a poor prognosis, since no effective therapy has yet been established. Herein, we report five cases of pseudomyxoma peritonei that responded to a combined postsurgical therapy consisting of CDDP, 5-FU and MMC. A 59-year-old women had a complete response who underwent an appendectomy, a right ovariectomy, and an omentectomy due to pseudomyxoma peritonei, and was postoperatively given a 50 mg intraperitoneal administration of CDDP. Further, a subcutaneous implant-type reservoir was positioned intraperitoneally for postoperative chemotherapy. At 14 days after surgery, therapy was begun which consisted of CDDP (70 mg/m2/day 1), 5-FU (350 mg/m2/day 1) and MMC (6 mg/m2/day 1) administered intraperitoneally, and 5-FU (350 mg/m2/day 1-3) that was continuously infused by an intravenous drip. She received 4 courses of postoperative chemotherapy. As a result of this therapy, the serum CEA values, which had elevated postoperatively, fell to within their respective normal ranges, and at 44 months postoperatively, this patient remains alive. Given the results above, intraperitoneal administrations of this combined CDDP, 5-FU, MMC therapy may be effective for patients with pseudomyxoma peritonei.     

MRI evaluation of bulky tumor masses in the mesentery and bladder involvement in peritoneal carcinomatosis

AIM: Peritonectomy procedures with intraperitoneal chemohyperthermia are an effective but costly treatment for peritoneal carcinomatosis (PC). Consequently a proper selection of patients is necessary. We evaluated the benefit of MRI prior to surgery, in the detection of two of the main surgery contraindications: bulky mesenteric tumors and bladder implants. METHODS: Three experts retrospectively reviewed abdominal and pelvic MRI from 19 cases of surgically proved PC (ovary: 7; colorectal: 7; gastric: 2; pseudomyxoma peritonei: 2; appendix: 1). RESULTS: Mesenteric tumors were always identified as hypersignal masses on axial and coronal fat suppression gadolinium-enhanced T1 images (n=3). Three out of five bladder implants were detected. The two cases of bladder implants that were not detected on MRI were missed because the bladder was not filled. The best sequence for the detection of bladder involvement was axial T2-weighted images with bladder filling. CONCLUSIONS: Evaluating the preoperative resectability of PC is crucial for patient management. MRI seems to reliably detect bulky mesenteric tumors and bladder implants on condition the bladder is filled and appropriate sequences are used.