Median Line Cancer Involving The NUT Gene

Median Line Cancer Involving The NUT Gene

Summarize

Molecular features

        NUT midline carcinoma is a malignant tumor characterized by the rearrangement of the NUT gene. It is extremely rare and highly aggressive. In about 75% of cases, the NUT gene on chromosome 15q14 fuses with the BRD4 gene on chromosome 19p13, resulting in the production of the BRD-NUT fusion protein. In the remaining cases, the NUT gene fuses with either BRD3 on chromosome 9q34 or NSD3 on chromosome 8p11, leading to a variant form known as NUT variant.

Epidemiological

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Etiology & Risk Factors

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Classification & Staging

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Clinical Manifestations

        Childhood NUT midline carcinoma occurs in the midline epithelial structures, typically in the mediastinum and upper respiratory and digestive tracts. Clinically, it is characterized by squamous differentiation or very aggressive undifferentiated cancer, with or without squamous differentiation. Although this tumor was first identified in children and young adults, individuals of all ages can be affected.

Clinical Department

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Examination & Diagnosis

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Clinical Management

Treatment of midline cancer in children

        The treatment of pediatric NUT midline carcinoma involving the NUT gene is a multimodal regimen that includes systemic chemotherapy, surgery, and radiotherapy.

        Cisplatin, taxanes, and alkylating agents have been used in the treatment of this cancer with some success. Although these drugs often show quick results, the disease can progress rapidly in its early stages. Two cases of children with refractory diseases have responded to vorinotinib, suggesting that such drugs may be effective in treating this type of malignant tumor. Another promising new class of drugs is BET bromodomain inhibitors, which are being studied for their effectiveness against NUT midline carcinoma in adult patients.

Prognosis

The prognosis for this type of cancer is poor, with a median survival of less than one year. Preliminary data suggest that tumors with NUT variants may have a longer course.

Follow-up & Review

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Daily Care

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Cutting-edge therapeutic and clinical Trials

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References

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Audit specialists

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