Radiotherapy and Rosai-Dorfman disease

ABSTRACT

Rosai-Dorfman disease (RDD) also known as histiocytosis with lymphadenopathy is an uncommon, often self-limiting benign, non-Langerhans-cell histiocytic proliferative disorder. It usually affects young adults and children, with no predilection by sex. More than 80% of patients present with painless cervical lymphadenopathy, with or without fever. Other presentation sites include upper aero-digestive tract, orbits and the paranasal sinuses. Extra nodal involvement occurs in up to 40% of cases and common sites of involvement include the skin, upper and lower respiratory tract, soft tissue, bones, kidney, liver, orbit and central nervous system. Treatment is not necessary in most instances, but some patients, when symptomatic, may require medical intervention. There is no established guideline for the management of this condition and various therapeutic modalities are used, including surgery, radiotherapy and or chemotherapy.

RESUMO

A doença de Rosai–Dorfman, também conhecida como histiocitose com adenopatia, é uma desordem linfo-proliferativa de histiócitos não Langerhans, rara e geralmente auto limitada. Ela usualmente afeta adultos jovens e crianças, sem predileção por sexo, com ou sem a presença de febre associada. Outros sítios de acometimento incluem o trato aéreo e digestivo altos, orbitas e seios paranasais. O envolvimento extra-nodal pode ocorrer em até 40% e os sítios comumente acometidos são a pele, trato aéreo alto e baixo, partes moles, ossos, rim, fígado, orbita e sistema nervoso central. O tratamento não é necessário na maioria dos casos, porem, em alguns pacientes pode ser necessário algum tipo de in-tervenção. Não existe um protocolo de tratamento estabelecido para essa doença, e são varias as opções terapêuticas que podem ser empregadas, dentre elas cirurgia, radiotera-pia e quimioterapia.

Keywords:

Histiocytosis, sinus - Proliferative disorder - Radiotherapy - Surgery

Descritores:

Histiocitose sinusal - Transtorno histiocítico proliferativo - Radioterapia - Cirurgia

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