Radiological Category Neuroradiology Principal Modality 1 CT Principal

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Radiological Category: Neuroradiology Principal Modality (1): CT Principal Modality (2): MRI Case Report #0139

Radiological Category: Neuroradiology Principal Modality (1): CT Principal Modality (2): MRI Case Report #0139 Submitted by: Walid K. Adham , M. D. Faculty reviewer: Leo Hochhauser, M. D. Date accepted: January 2005 This case report was presented at the Annual Kay Vydareny Film Interpretation Competition at the Association of University Radiologists (AUR) Meeting, 20 April 2003.

Case History 19 year old woman presents with fever, mild left facial swelling, and

Case History 19 year old woman presents with fever, mild left facial swelling, and massive bilateral cervical lymphadenopathy.

Radiological Presentations

Radiological Presentations

Radiological Presentations

Radiological Presentations

Test Your Diagnosis Which one of the following is your choice for the appropriate

Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis? After your selection, go to next page. • Chronic sinusitis • Rosai Dorfman Disease • Sarcoidosis • Lymphoma • Langerhans histiocytosis

Findings and Differentials Findings: Expansile multilobular soft tissue mass within the left maxillary sinus

Findings and Differentials Findings: Expansile multilobular soft tissue mass within the left maxillary sinus that is eroding into the maxillary walls and extending into the left nasal cavity. Differentials: • Chronic sinusitis • Rosai Dorfman Disease • Sarcoidosis • Lymphoma • Langerhans histiocytosis

Discussion Rosai Dorfman Disease is also known as Sinus Histiocytosis with Massive Lymphadenopathy. This

Discussion Rosai Dorfman Disease is also known as Sinus Histiocytosis with Massive Lymphadenopathy. This is a non-neoplastic, self-limiting disease of unknown etiology that most commonly presents in the first or second decade of life. It is characterized by proliferation and accumulation of histiocytes within the lymphatics or extranodal sites. Patients typically present with painless lymphadenopathy, fever, hypergammaglobulinemia, and an elevated erythrocyte sedimentation rate. Bilateral nontender cervical lymphadenopathy occurs in 87% of patients at the time of presentation. Extranodal sites including the central nervous system, skin, kidneys, bones, and gastrointestinal tract are involved in 43% of patients. Most cases of CNS Rosai Dorfman involve the leptomeninges and appear as a duralbased mass that is associated with surrounding vasogenic edema. The associated edema helps to differentiate Rosai Dorfman from a meningioma but biopsy is needed for definitive diagnosis. Differential considerations for other sites of disease within the head and neck include Wegener’s granulomatosis, sarcoidosis, Langerhan’s histiocytosis, eosinophilic granuloma, and lymphoma. Treatment is not typically necessary since spontaneous regression occurs in most cases. Patients with progressive symptoms have been treated with chemotherapy or radiation therapy and have shown limited response. Surgery is reserved for patients who develop life-threatening obstructive symptoms.

Discussion References Rosai J, Dorfman RF: Sinus histiocytosis with massive lymphadenopathy: a newly recognized

Discussion References Rosai J, Dorfman RF: Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathologic entity. Arch Pathol: 1969; 87: 63 -70. Goodnight JW, Wang MB, et al. Extranodal Rosai-Dorfman Disease of the Head and Neck. Laryngoscope. 1996; 106: 253 -256.

Diagnosis Rosai Dorfman Disease.

Diagnosis Rosai Dorfman Disease.