High-risk population screening is essential to improve diagnosis rate.
Contrast-enhanced chest/abdominal CT is preferred; MRI/PET-CT are optional when available (strongly recommended).
Biopsy & Pathology 🧬
CT-guided biopsy as standard; ultrasound-guided biopsy/thoracoscopy as supplements (strongly recommended).
Histopathology is gold standard; report subtype (epithelioid/sarcomatoid/biphasic), Ki-67, nuclear grade, and growth pattern (epithelioid).
IHC panel (≥3 markers: Calretinin, CK5/6, WT-1, D2-40, BAP1, MTAP) and FISH (CDKN2A homozygous deletion) for differential diagnosis and in situ mesothelioma (strongly recommended).
Molecular Testing 🧪
Common mutations: TP53, BAP1, CDKN2A, LATS1/2, NF2, SETD2.
BAP1 testing is recommended for young patients without asbestos exposure or with family tumor history (strongly recommended).
Surgery ⚕️
Operable epithelioid MPM (stages I–IIIA) is eligible for surgery; pleurectomy/decortication (P/D) is preferred (strongly recommended).
Extrapleural pneumonectomy (EPP) is reserved for selected cases due to higher morbidity/mortality.
R0 resection is challenging; MDT discussion is mandatory.
Radiotherapy & TTFields 📡
Adjuvant hemithoracic RT (IMRT preferred) may reduce local recurrence in postoperative patients with good PS, lung/kidney function, and no distant disease (strongly recommended).
Tumor treating fields (TTFields) is a promising novel modality for comprehensive management when available (recommended).
Systemic Therapy 💊
First-line: Pemetrexed plus cisplatin is standard for advanced MPM.
Immunotherapy (e.g., immune checkpoint inhibitors) has become a key component of first-line and salvage therapy; combination regimens are preferred in eligible patients.
Targeted therapy is evolving, with ongoing trials for actionable targets.
Palliative Care & Follow-Up 🛡️
Symptom management (chest pain, dyspnea, pleural effusion) is integral.
Regular follow-up with CT and clinical assessment to monitor recurrence and adjust treatment.