Soft tissue Rosai-Dorfman disease of the posterior mediastinum

Abstract

Rosai-Dorfman disease (RDD) consists of sinus histiocytosis with massive lymphadenopathy. Extranodal involvement occurs in up to 43% of cases. However, isolated soft tissue RDD is rare. Isolated mediastinal RDD is exceedingly rare, and there have been only three previous reports. Involvement of the posterior mediastinum in RDD has been reported only in the context of disseminated RDD. Here, we report the case of a 49-year-old female patient with a two-year history of cervical pain and lymphadenomegaly, which resolved spontaneously. A CT scan revealed a left paravertebral mass with a diameter of 6 cm. The patient was submitted to surgical excision of the mass. Microscopic examination and immunophenotyping of the surgical specimen led to a diagnosis of RDD. During a 12-month follow-up period, the patient complained of mild cough and chest pain. Periodic imaging tests showed no sign of recurrence, and no postoperative cervical lymphadenomegaly was detected.

Rosai-Dorfman disease mimicking mediastinal lymphoma

“…8 Although the number of cases is insuffi cient to elucidate the characteristics of the disease, accumulation of more mediastinal RDD cases in the future may generate new fi ndings.…”

Extranodal Rosai–Dorfman disease: a rare soft tissue neoplasm masquerading as a sarcoma

“…The largest report of RDD (1969) involved 423 cases, with 182 patients having extranodal disease [3]. Only 13 patients in this series presented with soft-tissue RDD without detectable lymphadenopathy [3]. Here we describe an unusual case of RDD in a middle-aged African American female presenting as a painful right medial thigh mass.…”

“…A rare disease, RDD is distributed worldwide, predominantly affecting young people and with a slight male predominance [2]. The disease typically presents with bilateral painless lymphadenopathy of the head and neck as well as fever, leukocytosis, elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia [3,4]. Extranodal presentations have also been described, with the most common sites including skin and nasal sinuses [4].…”

“…RDD histiocytes are unique because they phagocytose intact lymphocytes and other immune cells, a histological hallmark of the disease termed emperipolesis [5]. The largest report of RDD (1969) involved 423 cases, with 182 patients having extranodal disease [3]. Only 13 patients in this series presented with soft-tissue RDD without detectable lymphadenopathy [3].…”

Soft Tissue Rosai-Dorfman Disease: Case report

“…5 Nodalbased RDD usually regresses spontaneously, whereas isolated STRDD is indolent in nature and may recur years or even decades later. 6 This report describes a case of isolated STRDD which presented initially as a painless nodular cheek mass. The importance of including this rare entity in the differential diagnosis of histiocytic soft tissue lesions is highlighted.…”