В статье представлен клинический случай первичного изолированного злокачественного лангергансоклеточного гистиоцитоза вульвы у больной 33 лет. Показана эффективность гемивульвэктомии в сочетании с адъювантной дистанционной химиолучевой терапией. Ключевое значение в диагностике заболевания имело гистологическое и иммуногистохимическое исследование при адекватном количестве иссеченных тканей. Заболевание требует выработки единых алгоритмов обследования и лечения при мультидисциплинарном подходе.
We report a 33-year-old female patient with primary isolated malignant vulvar Langerhans cell histiocytosis (LCH). The effectiveness of hemivulvectomy with adjuvant EBRT and chemotherapy is presented. Adequate tissue excision followed by histological study with immunohistochemistry was of critical importance for the diagnosis in our case. Unified management recommendations with multidisciplinary approach are required.
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