Treatment of sinus histiocytosis with massive lymphadenopathy (rosai‐dorfman disease): Report of a case and literature review

Abstract

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disorder of unknown etiology, usually associated with lymph node enlargement in various super®cial or deep sites. It usually shows a prolonged clinical course with occasional exacerbation and remission phases. We describe the long-term follow-up of a case of SHML that showed typical clinical features and in which various therapeutic strategies were attempted. Chemotherapy and a-interferon (IFN) were ineffective; surgery was ultimately required with satisfactory results. From an extensive literature review we found different treatment strategies in SHML in the 80 cases published between 1969 and 2000. Spontaneous resolution of adenopathies is frequently observed: 32 out of 40 cases which did not receive chemotherapy, radiotherapy, or surgery were healthy at the time of publication. Radiotherapy alone showed con¯icting results: 3 complete remissions (CR) were obtained in the 9 patients treated. Surgical debulking when required was effectiveÐ8/9 CRÐwhile chemotherapy showed generally negative results. IFN has been previously employed in only one case. In conclusion, clinical observation without treatment is advisable when possible. In the presence of vital organ compression and/or extranodal localization with important clinical signs, surgical debulking may be necessary. Radiotherapy has shown limited ef®cacy, while chemotherapy is in general ineffective. More experience is needed to evaluate the role of IFN. Am.

Overlapping immunoglobulin G4-related disease and Rosai-Dorfman disease mimicking lung cancer

“…Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare disease characterised by (mostly cervical) lymph node enlargement and fever, with a distinct cellular pattern of the lymph nodes [1]. The histopathological findings are massive sinus infiltration with histiocytes that show a large amount of cytoplasm in which lymphocytes are engulfed (emperipolesis; Rosai-Dorfman cells) that show immunohistochemical staining for S-100 protein without CD1a expression.…”

“…In the majority of cases, however, the disease is self-limiting. Most patients do not require treatment with steroids [1].…”

“…Relapsing polychondritis is a rare multi-systemic disease characterised by recurrent episodes of inflammation and destruction of cartilaginous structures [1]. Airway involvement by relapsing polychondritis, which results in tracheobronchomalacia and airway stenosis, is associated with a poor prognosis.…”

“…

Bronchial rupture related to endobronchial stenting in relapsing polychondritisTo the Editor:Relapsing polychondritis is a rare multi-systemic disease characterised by recurrent episodes of inflammation and destruction of cartilaginous structures [1]. Airway involvement by relapsing polychondritis, which results in tracheobronchomalacia and airway stenosis, is associated with a poor prognosis.

Several reports have suggested that endobronchial intervention can be beneficial in these subjects [2,3].

…”

Painful left shoulder

“…RDD is generally self-limited with spontaneous resolution; however, it may have a prolonged course with remission, exacerbation, and even death [3,5]. After reviewing treatments, Pulsoni et al suggested observation without treatment is advisable except when compression or other clinical signs indicate surgical debulking [6]. This patient was successfully treated by curettage and bone grafting.…”