SESSION TYPE: Miscellaneous Student/Resident Cases I
PRESENTED ON: Tuesday, October 23, 2012 at 11:15 AM - 12:30 PM
INTRODUCTION: Maintaining a broad differential is important when evaluating patients who present with soft tissue masses with lymphadenopathy. We describe a less common variant of a rare disorder presenting as a right axillary wall mass.
CASE PRESENTATION: An otherwise healthy 28 year old male presented to the primary care clinic for evaluation of a progressively growing painless right axillary wall mass which had progressed to the size of a grapefruit since the patient first noted the mass 2 years ago. The patient’s review of symptoms was positive for night sweats and weight loss. Within the last month patient had negative PPD and HIV Elisa as part of a routine health screening for his employment. Exam revealed a 20 cm x 30cm mass non-tender, nonpulsatile mass soft tissue mass of the anterior axilla. Additionally, the patient was found to extensive palpable supraclavicular and axillary lymphadenopathy. On labs, the patient had an elevated eryrthrocyte sedimentation rate of 65mm/hr and lactate dehydrogenase of 300 IU/L. Otherwise other labs including a complete blood count and comprehensive metabolic panel were unremarkable. Given concern for malignancy, a CT of the chest was performed which revealed a right infraxillary peduculated mass with evidence of extensive lymphadenopathy concerning for malignancy. The patient then underwent an core biopsy which revealed that the patient had sinus histiocytosis with frequent emperipolesis. Flow cytometry was negative for T and B cell lymphoma. The mass was later surgically excised without further complication.
DISCUSSION: SHML, also known as Rosai-Dorfman Syndrome, is a rare benign lymphoproliferative disorder resulting from the overproduction of non Langherhans histiocytes. Primarily, lymph nodes are involved with this disorder; however, less commonly other extranodal sites of the skin, soft tissue, thyroid, CNS, bone, breast, and adnexa can be involved as well. The typical presentation of this disorder includes bilateral cervical lymphadenopathy with anemia, leukocytosis, and elevated sedimentation rates. The diagnosis is made primarily on histological appearance of histiocytes with lymphophagocytosis and immunochemical staining positive for S-100 protein. The etiology of this disorder is unknown and it thought to be a reactive proliferative disorder which self-regresses. Currently, there appears to be no uniform treatment modality. In certain rarer instance, as in our case, surgical resection is necessary to reduce tumor burden.
CONCLUSIONS: SHML is very rare disorder which should remain on differential when approaching patients with soft tissue masses and lymphadenopathy even if malignancy seems likely.
1) Foucar E, Rosai J, Dorfman RF. Sinus histiocytosis withmassive lymphadenopathy (Rosai-Dorfman Disease):review of the entity. Semin Diagn Pathol 1990;7:19-73
DISCLOSURE: The following authors have nothing to disclose: Aalok Patel, Sameer Chopra, Milan Patel, Bhavin Adhyaru
No Product/Research Disclosure InformationEmory, Atlanta, GA