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BMC Cancer

, 16:895

Medical and radiation oncology

Abstract

BackgroundNUT midline carcinoma NMC is a rare and highly aggressive malignancy. Although more information on NMC has been recently accumulating in the literature, most oncologists and pathologists remain unfamiliar with the clinical and pathologic features of this disease. The clinical features of NMC sometimes mimic those of other malignancies, and NMC can therefore be overlooked if the diagnosis is not suspected. We present the case of a young male with NMC arising in the mediastinum with elevated serum alpha-fetoprotein levels suggestive of an extragonadal nonseminomatous germ-cell tumor.

Case presentationA 28-year-old Japanese male presented with cough and left-sided chest pain for 6 weeks. The patient had a mediastinal tumor with metastases to the right lung, lymph nodes, and bones at initial presentation. Nonseminomatous germ cell tumor was suspected due to the young age, location of the tumors, and elevated serum alpha-fetoprotein. However, biopsy confirmed the diagnosis of NMC with immunohistochemistry. The tumor briefly responded to cytotoxic chemotherapy but subsequently progressed and became refractory to the chemotherapy regimen. External beam radiotherapy was administered with dramatic shrinkage of the tumor and a metabolic response on 18-fluoro-2-deoxyglucose positron emission tomography-computed tomography F-FDG PET-CT scan. However, the patient died 4.5 months after the diagnosis of NMC.

ConclusionsSerum levels of alpha-fetoprotein may be elevated in patients with NMC. Regardless of the level of tumor markers, immunohistochemistry for NUT should be performed in cases of poorly differentiated carcinomas without glandular differentiation arising in the midline structures. F-FDG PET-CT is useful for staging and assessing responses to therapy.

KeywordsNUT midline carcinoma Tumor markers Alpha-fetoprotein Radiotherapy PET-CT Case report AbbreviationsAFPAlpha-fetoprotein

BETiBET inhibitor

EBRTExternal beam radiotherapy

EBUS-TBNAEndobronchial ultrasound-guided transbronchial needle aspiration

FISHFluorescence in situ hybridization

frfractions

GCTGerm cell tumor

HDACiHDAC inhibitor

LDHLactate dehydrogenase

NMCNUT midline carcinoma

NSGCTNonseminomatous germ cell tumor

NUTNuclear protein in testis

β-hCGβ-human chorionic gonadotropin

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Autor: Yohei Harada - Takafumi Koyama - Kengo Takeuchi - Kazufusa Shoji - Kazuei Hoshi - Yu Oyama

Fuente: https://link.springer.com/







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