ورم ديسمويد في جدار البطن: ماذا تفعل عندما تفشل المراقبة الفعّالة

References

DOI: 10.1001/jamaoncol.2024.1805

• Superficial sites of disease represent the best indications for surgery in cases of continuous progression, particularly in abdominal wall DT.
• If complete resection is feasible, it can be well tolerated if the reconstruction is performed optimally, and future pregnancies are possible even with a prosthetic mesh repair.
• Cryotherapy may also be considered before surgery.
• Given its safety profile, nirogacestat may well become the first-line treatment for DTs when an active medical treatment is indicated.
• Until recently, systemic treatment options for DT consisted of only tyrosine kinase inhibitors, such as sorafenib (high quality of evidence according to GRADE) and pazopanib (very low quality of evidence according to GRADE); low-dose (very low quality of evidence according to GRADE) or conventional chemotherapeutic regimens (very low quality of evidence according to GRADE), including low-dose chemotherapy with methotrexate and/or vinorelbine administered intravenously or orally; and liposomal doxorubicin, as outlined in the 2020 consensus guideline.
• The consensus of the Desmoid Tumor Working Group was that for most of the available treatments, a course of at least 6 months is required (provided there is absence of frank progression) prior to assessing effectiveness.