Painful left shoulder

Abstract

Sinus histiocytosis with massive lymphadenopathy (SHML), characterized by histiocyte-laden massive lymph node enlargement, was initially described by Rosai and Dorfman in 1969 as a rare, non-neoplastic and self-limiting disease [1]. The classical presentation of massive symmetrical lymph node enlargement has a predilection for cervical lymph nodes. Extranodal disease, with or without lymphadenopathy, is termed “Rosai–Dorfman disease” (RDD) [2] and most commonly involves the skin, upper respiratory tract, and bone, occurring in approximately 43% of patients [3]. Osseous lesions are seen in approximately 8% of patients [3]. Isolated osseous involvement without lymphadenopathy or other extranodal disease as in this case is exceedingly rare [4]. RDD can present at any age but has typically been found in the first or second decade of life, with a mean age of 20.6 years [3]. 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. This case demonstrates the typical clinical and osseous imaging characteristics of the uncommon isolated primary osseous form of RDD. The patient had no lymphadenopathy clinically, nor upon CT imaging of the body. Although the patient complained of fatigue and was anemic with a hemoglobin of 8.7 g/dl, the other often-seen clinical manifestations of fever and weight loss were absent. Other frequently associated lab findings including an elevated erythrocyte sedimentation rate, leukocytosis, reversal of the T4/T8 lymphocyte ratio, and hypergammaglobulinemia [3, 7], were normal or not studied. The typical radiographic lytic bone lesion of RDDmay have a poorly or sharply defined margin, most often in a medullary location. Bone lesions may be single monostotic, multiple monostotic, or polyostotic. As in this case, bone lesions usually do not have periosteal reaction or sclerosis, but these appearances may occur. Intralesional calcification has not been reported. Serial imaging generally reveals a decrease in lesion size leading to complete resolution; sometimes a sclerotic focus remains. [2, 7, 8] In general, and in this case, the radiographic appearance is that of a semi-aggressive or an aggressive condition that suggests a differential diagnosis including osteomyelitis, multiple myeloma, lymphoma, sarcoma, Langerhans cell histiocytosis, metastatic disease, and rare entities such as lipid storage disorders and sarcoid [8, 9]. MRI commonly demonstrates elevated signal intensity on T2-weighted sequences, low signal intensity on T1-weighted sequences, soft tissue extension, and adjacent soft tissue edema which The case presentation can be found at doi:10.1007/s00256-011-1325-x.