Thoracic SMARCA4-Deficient Undifferentiated Tumor With ALK Fusion Treated With Alectinib Achieved Remarkable Tumor Regression: Case Report

Sheng, Jin; Han, Weidong; Pan, Hongming

doi:10.1016/j.jtocrr.2023.100476

Cited by 1 publication

(1 citation statement)

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“…Targetable co-mutations such as epidermal growth factor EGFR, ALK, and ROS proto-oncogene 1 are infrequent in SMARCA4-UT patients. Sheng et al reported a case of SMARCA4-UT with EML4-ALK fusion, which was successfully treated with alectinib [ 66 ]. More recently, an observational study including SMARCA-UT patients and SMARCA4-deficient NSCLC reported three cases of patients with ALK mutation, including one EML4-ALK rearrangement [ 4 ].…”

Section: Smarca4-ut and Co-occurring Mutationsmentioning

confidence: 99%

Treatment of Thoracic SMARCA4-Deficient Undifferentiated Tumors: Where We Are and Where We Will Go

Longo,

Catino,

Montrone

et al. 2024

IJMS

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Recently, the fifth edition of the WHO classification recognized the thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT) as a separate entity from conventional non-small cell lung cancer with SMARCA4 deficiency because of the different clinicopathological characteristics of these two diseases. SMARCA4-UT mainly occurs in young to middle-aged adults and involves a large mass compressing the tissues surrounding the mediastinum and lung parenchyma. Unfortunately, SMARCA4-UT shows a high probability of recurrence after upfront surgery as well as radiotherapy resistance; moreover, chemotherapy has low efficacy. Moreover, given the recent classification of SMARCA4-UT, no data concerning specific clinical trials are currently available. However, several case reports show immunotherapy efficacy in patients with this disease not only in a metastatic setting but also in a neoadjuvant manner, supporting the development of clinical trials. In addition, preclinical data and initial clinical experiences suggest that inhibiting pathways such as CDK4/6, AURKA, ATR, and EZH2 may be a promising therapeutic approach to SMARCA4-UT.

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Section: Smarca4-ut and Co-occurring Mutationsmentioning

confidence: 99%