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

Mahathi Komaragiri,Michael Dardik,Maria Laureana Santos-Zabala,Lauren S Sparber,Ronald S Chamberlain

doi:10.1186/1477-7819-11-63

Abstract

IntroductionRosai–Dorfman disease (RDD) is a rare proliferative histiocytic disorder of unknown etiology. RDD typically presents with generalized lymphadenopathy and polymorphic histiocytic infiltration of the lymph node sinuses; however, occurrences of extranodal soft tissue RDD may rarely occur when masquerading as a soft tissue sarcoma.Materials and methodsA comprehensive search of all published cases of soft tissue RDD without associated lymphadenopathy was conducted using PubMed and Google Scholar for the years 1988 to 2011. Ophthalmic RDD was excluded.ResultsThirty-six cases of extranodal soft tissue RDD, including the current one, have been reported since 1988. Anatomical distribution varied among patients. Four (11.1%) patients presented with bilateral lesions in the same anatomic region. Pain was the most common symptom in six (16.8%) patients. Sixteen (41.6%) patients were managed surgically, of which one (2.8%) case experienced recurrence of disease.ConclusionRDD is a rare inflammatory non-neoplastic process that should be considered in the differential diagnosis of a soft tissue tumor. Thus, differentiation of extranodal RDD from more common soft tissue tumors such as soft tissue sarcoma or inflammatory myofibroblastic tumor is often difficult and typically requires definitive surgical excision with histopathological examination. While the optimal treatment for extranodal RDD remains ill-defined and controversial, surgical excision is typically curative.

Highlights

Rosai–Dorfman disease (RDD) is a rare proliferative histiocytic disorder of unknown etiology
Sinus histiocytosis with massive lymphadenopathy (SHML) is a class II histiocytosis first described as a unique clinicopathologic entity by Rosai and Dorfman in 1969 [1]
Lymph nodes are more commonly involved, any organ may be affected – the term RDD has been adopted in place of SHML [1]

Summary

Introduction

Rosai–Dorfman disease (RDD) is a rare proliferative histiocytic disorder of unknown etiology. RDD typically presents with generalized lymphadenopathy and polymorphic histiocytic infiltration of the lymph node sinuses; occurrences of extranodal soft tissue RDD may rarely occur when masquerading as a soft tissue sarcoma. Case presentation A 56-year-old African American female presented to the Saint Barnabas Medical Center (Livingston, NJ, USA) with a 1-year history of an enlarging painful right medial thigh mass. Her medical history was significant for hypertension, diabetes, hypercholesterolemia, degenerative disk disease and asthma. The patient reported a pulling, painful sensation in the knee joint as well as discomfort upon standing for long periods of time. The patient’s symptoms progressed and a wide local excision of the mass was completed

Results

Discussion

Conclusion