Malignant Peritoneal Mesothelioma

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

LATS1, BAP1, LATS2, EGFR, PAX8, ALK, CD274, TWIST1, MET, WT1, PTEN, CEACAM5, CDKN2A, IMP3, CALB2, KRT20, UVRAG, RPE65, SDC1, MIR34A, CXCL12, SPP1, TLR3, TP53, C17orf97, RAB7B, VTN, RAB7A, TP63, PTGS2

LATS1, BAP1, LATS2, EGFR, PAX8, ALK, CD274, TWIST1, MET, WT1, PTEN, CEACAM5, CDKN2A, IMP3, CALB2, KRT20, UVRAG, RPE65, SDC1, MIR34A, CXCL12, SPP1, TLR3, TP53, C17orf97, RAB7B, VTN, RAB7A, TP63, PTGS2, CKAP4, SMUG1, CLDN15, TMEM30B, KAZALD1, ALB, MAPK1, PSG2, MAPK13, BIRC2, ATM, CDH11, CEACAM3, CEACAM7, CLDN4, DES, ECM1, EGF, EPHB2, ESR1, ESR2, EZH2, FLI1, FLII, FUT4, GNA11, HAS1, JAK3, KDR, MT1E, COX2, NF2, PGR, PIK3CA, MTCO2P12

Drugs

5-[8-methyl-9-(1-methylethyl)-2-(4-morpholinyl)-9H-purin-6-yl]-2-pyrimidinamine, Amatuximab, Autologous dendritic cells pulsed with allogeneic tumour cell lysate

5-[8-methyl-9-(1-methylethyl)-2-(4-morpholinyl)-9H-purin-6-yl]-2-pyrimidinamine, Amatuximab, Autologous dendritic cells pulsed with allogeneic tumour cell lysate, Genetically modified serotype 5/3 adenovirus coding for granulocyte-macrophage colony-stimulating factor, Pegylated recombinant arginine deiminase, Tazemetostat ( TAZVERIK ), Wilms' tumour 1 (WT1) vaccine, doxorubicin hydrochloride in a lipid-based pegylated nanoparticle modified with a 31-aminoacid peptide targeting nucleolin

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Malignant peritoneal mesothelioma is a primary peritoneal malignancy occurring in the lining cells (mesothelium) of the peritoneal cavity.

Epidemiology

Peritoneal mesothelioma accounts for 10 to 30% of all malignant mesotheliomas. The annual incidence is approximately 1/500,000 in France but reaches 1/200,000 in some parts of Europe (Italy). Men are predominantly affected.

Clinical description

The tumors are usually diagnosed in late adulthood (median age: 55 years). Typical presenting features are abdominal distention, abdominal pain, presence of an abdominal mass, impaired general state, weight loss, and ascites. Dyspnea, coagulation disorders, edema of the lower limbs and intestinal occlusion may be observed.

Etiology

The relationship between peritoneal mesothelioma and asbestos exposure is unclear, especially in women, and has not been established unlike in malignant pleural mesothelioma. Other causes have been reported such as exposure to erionite, viral infection and vaccine products and/or genetic factors.

Diagnostic methods

Diagnosis is based on imaging techniques, such as ultrasound and chest-abdominal-pelvic computed tomography (CAP-CT). Diagnosis is confirmed histologically on tissue biopsy and by relevant immunostaining results (positive for calretinin and negative for carcinoembryonic antigen (CEA)), and should be performed by two experts.

Differential diagnosis

Differential diagnosis includes peritoneal carcinomatosis secondary to colorectal or gastric cancer and primary peritoneal carcinoma (see this term).

Management and treatment

Treatment strategies require a multidisciplinary approach and must be discussed by a panel of physicians in a specialized center. There are currently no validated recommendations on clinical management and no cytotoxic agents have been granted a European Marketing Authorization (MA) in this indication. Currently proposed treatment with curative intent involves a combination of cytoreductive surgery (visceral resections and peritonectomy procedures) with hyperthermic intraperitoneal chemotherapy (HIPEC) (off-label use) in specific patients (young, good general status, low tumor volume). Systemic chemotherapy (off-label use) is sometimes used in palliative treatment.

Prognosis

With palliative treatment (systemic chemotherapy), median survival does not reach 1 to 2 years. Following cytoreductive surgery and HIPEC, median survival of more than 50 months, and 5-year survival of more than 50% may be obtained.