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Карцинома серединної лінії NUT

ORPHA:443167· ICD-10 C80.9· NUT midline carcinoma

Визначення(English summary)

A rare tumor characterized by a rapidly growing mass usually arising along the midline, defined by the presence of NUTM1 rearrangements. Histopathological examination shows a poorly differentiated carcinoma, often with evidence of squamous differentiation. Patients present with unspecific signs and symptoms due to mass effect, depending on the location. Extensive local invasion of adjacent structures, lymph node involvement, and distant metastatic disease are often present at the time of diagnosis. Prognosis is generally poor.

Поширеність
<1 / 1 000 000
Успадкування
Not applicable
Вік початку
Adolescent, Adult, Childhood, Elderly