Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I |

Rosai-Dorfman Disease (RDD) FREE TO VIEW

Theo Trandafirescu, MD; Dennis Genin, MD
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Queens Hospital Center, Jamaica, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1087A. doi:10.1016/j.chest.2016.08.1194
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: The differential diagnosis of neck masses can be divided into congenital, inflammatory and neoplastic processes.

CASE PRESENTATION: A 56 y.o.f. with past medical history of HTN,CHF NYHA class 1,initially presented to the ED in early march of 2014 with SOB secondary to rapidly enlarging neck mass. Patient reports she first noted a midline neck swelling in 2013 but as not causing her discomfort.Patient underwent CT of the neck showing large aggressive appearing soft tissue mass arising from the thyroid gland with substernal extension into the mediastinum. The mass invaded the trachea and esophagus and encased the L common carotid artery proximally, R innominate, R subclavian and R common carotid arteries. Scattered clustered prominent in size cervical lymph nodes were present and suspicious for metastatic disease. Thyroid US showed a lobulated heterogenous thyroid gland, isthmus measuring 3 cm, R lobe 10x7x5cm and L lobe 8x6x3cm. FNA of the thyroid gland was performed and showed atypical histiocytic proliferation. Shortly after diagnosis, the patient received chemotherapy followed by XRT therapy with good response. She was discharged home for follow up.

DISCUSSION: RDD is a rare, non-neoplastic disorder characterized by the proliferation and accumulation of histiocytes most commonly in the lymph nodes of the body, but can also occur in extranodal sites. Although the onset is idiopathic, there has been some literature describing possible links to immune dysfunction, viral infections, such as HHV, parvovirus B19, EBV, and IgG4-related diseases . Signs and symptoms of RDD can vary widely among patients. In most cases of RDD, patient has nonspecific symptoms such as fever, pallor, weight loss, malaise, rhinitis, but it can also present with painless, massive lymphadenopathy, neutrophilia, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia [2]. Extranodal manifestations can involve the skin (most common), liver, spleen, CNS involvement, nasal cavity, various bones, and the eyes and eye sockets. The differential diagnosis for RDD is broad including undifferentiated carcinoma, tuberculosis, sarcoidosis, granulomatosis ,melanoma, leukemia, Langherhans histiocytosis, thyroiditis and lymphoma.

CONCLUSIONS: RDD is a male predominant disease, affecting children, adolescents or young adults, but of the previously reported cases involving the thyroid gland it has occurred in elderly females in their mid-50s and one young female age 15. It has unique cytological features, e.g. the abundant pale cytoplasm and emperipolesis.

Reference #1: 1. Vujhini SK, Kolte SS, Satarkar RN, Srikanth S. Fine needle aspiration diagnosis of Rosai-Dorfman Disease involving thyroid. J Cytol.

Reference #2: 2. Ha H, Kim KH, Ahn YJ, Kim JH, Kim JE, Yoon SS. A rare case of Rosai-Dorfman disease without lymphadenopathy. Korean J Int Med.

Reference #3: 3. Boissière L, Patey M, Toubas O, Vella-Boucaud J, Perotin-Collard JM, Deslée G, Lebargy F, Dury S. Tracheobronchial Involvement of RDD

DISCLOSURE: The following authors have nothing to disclose: Theo Trandafirescu, Dennis Genin

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