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Mesothelioma, peritoneal [keywords]
- Malignant Peritoneal Mesothelioma in an Adolescent Male With BAP1 Deletion. [JOURNAL ARTICLE]
- J Pediatr Hematol Oncol 2014 Sep 12.
Diffuse malignant peritoneal mesotheliomas in children are uncommon, aggressive tumors with a grave prognosis. We herein report the clinical, radiologic, and pathologic findings of a 16-year-old male. The adolescent presented with a history of abdominal pain, nausea and daily, nonbilious, nonbloody emesis for 3 weeks. Radiographic imaging suggested small bowel obstruction. The diagnostic work-up and differential diagnoses are discussed. Histologically, the tumor was composed of epithelioid cells with a papillary and glandular architectural pattern. A few glands appeared to produce mucinous material. Histochemistry revealed PAS diastase resistant mucin, an inconspicuous finding in diffuse malignant peritoneal mesothelioma. An extensive immunohistochemistry panel (calretinin, WT-1, D2-40, CK 7, CAM 5.2, CK 5/6, CEA, B72.3, CK 20, CD10, CD30, CD15, CD117, PLAP, S100, TFE3, and EMA) confirmed the diagnosis. Of special interest, BAP1 staining was cytoplasmic and consistent with 3p deletion detected by conventional cytogenetics. The ultrastructural analysis demonstrated long microvilli, desmosomes, and intercellular junctions which further supported the diagnosis.
- Comparison of genomic abnormality in malignant mesothelioma by the site of origin. [JOURNAL ARTICLE]
- J Clin Pathol 2014 Sep 12.
Malignant mesothelioma (MM) results from the accumulation of a number of acquired genetic events at the onset. In MM, the most frequent changes are losses in 9p21, 1p36, 22q12 and 14q32, and gains in 5p, 7p and 8q24 by comparative genomic hybridisation analysis. We have examined various genomic losses and gains in MM and benign mesothelial proliferation by fluorescence in situ hybridisation (FISH) analysis. 9p21 deletion was reported to be less frequent in peritoneal than in pleural MMs. This study analysed various genomic losses and gains in MM by the site of origin using FISH analysis.We performed FISH analysis using paraffin-embedded tissues from 54 cases (40 pleural and 14 peritoneal) of MMs and compared the frequency of genomic abnormality by the site of origin.9p21 deletion was shown in 34 of 40 cases (85%) of pleural MMs, and was less frequent in five of 14 cases (36%) of peritoneal MMs (p<0.001) by FISH analysis. By contrast, 5p15 and 7p12 amplification was more significantly frequent in peritoneal than in pleural MMs. No difference between the two sites of MM in other genes was found.9p21 homozygous deletion assessed by FISH has been reported to be useful for differentiating MM from reactive mesothelial proliferation, but it should be noted that 9p21 deletion was less frequent in peritoneal MM. Our study suggests that the pathway of the genetic abnormality might vary between pleural and peritoneal MM.
- [Sarcomatoid malignant mesothelioma: a clinicopathologic and immunohistochemical analysis of 22 cases]. [English Abstract, Journal Article]
- Zhonghua Bing Li Xue Za Zhi 2014 Jun; 43(6):364-9.
To elaborate on the clinical and pathologic features of sarcomatoid malignant mesothelioma (SMM), its diagnostic criteria and differential diagnoses.Twenty-two cases of SMM retrieved from in-house and consultation files (between January 2009 to September 2013) were reviewed with emphasis on the clinicopathologic characteristics, immunophenotypes and the prognostic impact.The mean age of the patients was 54 years (ranged from 24-73 years). There was no sexual predilection and the majority of the patients did not have history of asbestos exposure. Overall, 14 tumors developed in the pleura and 8 cases arose from the peritoneal cavity. Clinically, patients presented signs and symptoms in accord with the location of the tumors, notably coughing, shortness of breath, and chest pain for patients with pleural origin, and nausea, abdominal distention and abdominal pain for those with peritoneal primary. In most cases, CT and MRI scan demonstrated lobulated masses (8/11). However, diffuse infiltrative growth patterns were observed exclusively in a minority of pleural cases (3/11). No visceral lesion was observed in any case. Histologically, 19 cases had either fibrosarcomatous or undifferentiated pleomorphic sarcoma-like appearance. Two cases were consistent with desmoplastic mesothelioma. One case contained osteosarcomatous element. All cases expressed pan-cytokeratin (AE1/AE3), and most cases were also positive for D2-40 (15/20). The staining of calretinin (9/21) and WT1 (10/14) was generally weak and focal. They were all negative for TTF-1, napsin A, SP-A, p63 and CD34. Follow-up information (range from 1 to 36 months) was available in 11 cases, 6 of which were alive with unresectable tumor, 1 patient with recurrent disease and 4 patients succumbed to disease. The overall survival was 5 months (mean 8 months).The diagnosis of SMM is achieved by comprehensive evaluation of medical history, imageological and pathological findings. Since calretinin immunoreactivity is infrequently observed in SMM, application of pan-cytokeratin and D2-40 immunostains offers a reasonable alternative for diagnosis. Diagnosis of SMM can be made by excluding a variety of spindle cell neoplasms with overlapping features, such as sarcomatoid carcinoma, synovial sarcoma, solitary fibrous tumor and fibrous pleuritis.
- Benign cystic mesothelioma associated with ipsilateral renal agenesis: a case report and review of literature. [JOURNAL ARTICLE]
- Pediatr Dev Pathol 2014 Sep 10.
Abstract Benign Cystic Mesothelioma (BCM) is an uncommon peritoneal lesion that usually occurs in reproductive age females with a history of abdominal surgery. Occasional expression of estrogen and progesterone receptor in these cells may explain female predilection. Reports of BCM in males are rare. We describe a case of BCM associated with ipsilateral renal agenesis in a young male without any surgical history. The cyst lining stained positive for cytokeratin, Wilms Tumor-1, epithelial membrane antigen, CD10, estrogen receptor and progesterone receptor and negative for PAX-8. Only three cases of BCM associated with congenital renal anomalies have been reported . To the best of our knowledge, this is the first case of BCM associated with ipsilateral renal agenesis in an adult male and the first male case of BCM displaying estrogen and progesterone receptor positivity. Such a case reveals the presence of congenital anomalies should be considered in patients with BCM.
- Advances in the management of peritoneal mesothelioma. [REVIEW]
- World J Gastroenterol 2014 Sep 7; 20(33):11700-11712.
Malignant peritoneal mesothelioma (PM) is an infrequent disease which has historically been associated with a poor prognosis. Given its long latency period and non-specific symptomatology, a diagnosis of PM can be suggested by occupational exposure history, but ultimately relies heavily on imaging and diagnostic biopsy. Early treatment options including palliative operative debulking, intraperitoneal chemotherapy, and systemic chemotherapy have marginally improved the natural course of the disease with median survival being approximately one year. The advent of cytoreduction (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has dramatically improved survival outcomes with wide median survival estimates between 2.5 to 9 years; these studies however remain largely heterogeneous, with differing study populations, tumor biology, and specific treatment regimens. More recent investigations have explored extent of cytoreduction, repeated operative intervention, and choice of chemotherapy but have been unable to offer definitive conclusions. CRS and HIPEC remain morbid procedures with complication rates ranging between 30% to 46% in larger series. Accordingly, an increasing interest in identifying molecular targets and developing targeted therapies is emerging. Among such novel targets is sphingosine kinase 1 (SphK1) which regulates the production of sphingosine-1-phosphate, a biologically active lipid implicated in various cancers including malignant mesothelioma. The known action of specific SphK inhibitors may warrant further exploration in peritoneal disease.
- Myxoid variant of peritoneal epithelioid malignant mesothelioma. A case report. [JOURNAL ARTICLE]
- Cesk Patol 2014; 50(3):149-151.
The myxoid variant of a diffuse malignant epithelioid mesothelioma is a rare tumor. To the best of our knowledge, only three cases of this type of mesothelioma involving the peritoneum have been reported in the literature to date. Although it is rare in the peritoneal cavity, it should be included in the differential diagnosis of the more common myxoid/mucinous abdominal lesions (e.g. mucinous carcinomas or pseudomyxoma peritonei), which can myxoid MM mimic. We report the case of a 60-year-old female with a myxoid variant of malignant peritoneal mesothelioma. Histologically, the tumor consisted of medium-sized to large epithelioid cells with a moderate to abundant amount of eosinophilic cytoplasm. Some of the tumor cells contained intracytoplasmic, optically clear vacuoles. The nuclei were irregular with coarse chromatin and some exhibited prominent nucleoli. Some of the cells were multinucleated. Mitotic figures were rare. Most of the tumor cells were located within an ample myxoid background. Immunohistochemically, the tumor cells showed a diffuse positivity for cytokeratin cocktail AE1/AE3, calretinin, D2-40, and cytokeratin 7. Vimentin, HBME-1 and WT-1 were only focally positive. Progesterone receptors showed positivity in rare tumor cells (up to 5%). Other markers examined, including cytokeratin 20, estrogen receptors, BerEP4, CEA, TTF-1, GCDFP-15, and CD15 were negative. Keywords: malignant mesothelioma - myxoid variant - peritoneum.
- Effect of NSAIDS and COX-2 inhibitors on the incidence and severity of asbestos-induced malignant mesothelioma: Evidence from an animal model and a human cohort. [JOURNAL ARTICLE]
- Lung Cancer 2014 Aug 18.
Non-steroidal anti-inflammatory drugs (NSAIDs) and COX-2 inhibitors have been associated with lower incidence rates of some cancers. Because asbestos can cause chronic inflammation at the pleural and peritoneal surfaces we hypothesised that NSAID and COX-2 inhibitors would inhibit the development of asbestos-induced mesothelioma.A murine model of asbestos-induced mesothelioma was used to test this hypothesis by providing the NSAID, aspirin, daily in the feed at 50mg/kg or 250mg/kg. In a parallel study, the relationship between the use of NSAID and COX-2 inhibitors and mesothelioma was investigated in a human cohort of 1738 asbestos exposed people living or working in Wittenoom, Western Australia (a crocidolite mine site).Aspirin did not alter the rate of disease development or increase the length of time that mice survived. Aspirin had a small but significant effect on disease latency (the time between asbestos exposure and first evidence of disease; p<0.05) but disease progression was not affected by the continued presence of the drug. In the Wittenoom cohort, individuals who reported use of NSAIDs, COX-2 inhibitors or both did not have a lower incidence of mesothelioma (HR=0.85; 95% CI=0.53-1.37, p=0.50), (HR=0.69; 95% CI=0.21-2.30, p=0.55) and (HR=0.43; 95% CI=0.16-1.13, p=0.087) respectively.We conclude that NSAIDs and COX-2 inhibitors do not moderate mesothelioma development or progression in a human cohort exposed to asbestos and this result is confirmed in an autochthonous mouse model.
- [Diagnosis and analysis of asbestos induced peritoneal mesothelioma]. [Journal Article]
- Zhonghua Lao Dong Wei Sheng Zhi Ye Bing Za Zhi 2014 May; 32(5):347.
- Malignant pleural and peritoneal mesothelioma consequential to brief indirect asbestos exposure. [Journal Article]
- J Clin Imaging Sci 2014.:35.
This report highlights that pleural and peritoneal mesothelioma can occur without direct asbestos exposure as was seen in our young patient. The patient had indirect exposure for as short as 3 months as a child, 15 years earlier, when she was residing with her miner father in the district of Jharia, Jharkhand, which is an asbestos-rich mining area in eastern India. The patient presented with chest pain and breathlessness. Chest X-ray showed opaque right hemithorax. Typical contrast- computed tomography (CECT) enhanced radiological features included nodular, soft-tissue attenuation and homogenously enhancing rind-like mass causing scalloping of the underlying lung and liver. Similar lesions were also found involving the pelvis. Diagnosis of malignant mesothelioma was confirmed on lung biopsy. Under-reporting of exposure is usual because it is unrecognized by both patients and investigators.
- Multimodality imaging of common and uncommon peritoneal diseases: a review for radiologists. [JOURNAL ARTICLE]
- Abdom Imaging 2014 Aug 20.
Peritoneal disease can be caused by a wide spectrum of pathologies. While peritoneal disease is usually caused by primary or secondary malignancies, benign diseases can occur and mimic malignancies. This article begins with an overview of peritoneal embryology and anatomy followed by a detailed description of the multimodality imaging appearance of peritoneal diseases. Common diseases include peritoneal carcinomatosis, pseudomyxoma peritonei, lymphomatosis, sarcomatosis, and tuberculous peritonitis. The uncommon diseases which cause peritoneal disease include desmoid fibromatosis, desmoplastic small round cell tumor, malignant mesothelioma, well-differentiated mesothelioma, multicystic mesothelioma, papillary serous carcinoma, leiomyomatosis, extramedullary hematopoiesis, inflammatory pseudotumor and amyloidosis. This manuscript will help the radiologist become familiar with the different peritoneal spaces, pathways of spread, multimodality imaging appearance and differential diagnoses of peritoneal diseases in order to report the essential information for surgeons and oncologists to plan treatment.