Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rosai-Dorfman disease (RDD) is a systemic disease and its etiology is not well understood. It is a very rare but established disease. We report a case of RDD diagnosed in the upper airway that became a life-threatening condition because of the stricture of the subglottic space. A 49-year-old female complained of nasal obstruction, hoarseness and lacrimation. After evaluation following by fiberscopic examination and CT scanning of the head and neck, sinusitis and laryngeal masses were detected. Endoscopic sinus surgery and endolaryngeal microsurgery were performed simultaneously. The masses in the nasal cavity and larynx showed the same histological findings. Proliferative large histiocytes with voluminous clear cytoplasm and rounded nuclei, and lymphophagocytosis known as emperipolesis are the characteristic features of RDD. In this case, extranodal manifestations in the subglottic space gradually emerged postoperatively. Tracheotomy was performed to ensure an airway and prevent suffocation. An extensive treatment is not required in most patients with RDD, however, some patients who have extranodal manifestations involving the airway may detoriorate into a life-threatening condition.
Auris Nasus Larynx 2004 Sep
PMID:Rosai-Dorfman disease revealed in the upper airway: a case report and review of the literature. 1536 64

Rosai-Dorfman disease (RDD) is a rare benign systemic histiocytic proliferation, characterized by massive lymph node enlargement and sometimes associated with extranodal involvement. Even though it is considered as a benign disease, fatalities can occur due to its unusually large size and its location. Our case highlighting a primary extranodal site of vallecula, which is extremely rare and not reported in literature before. It presented with almost complete obstructing the oropharyngeal airway creating a life threatening situation, needed emergency tracheostomy.
Auris Nasus Larynx 2016 Apr
PMID:An unusual presentation of extranodal Rosai-Dorfman disease threatening the airway. 2618 62

Rosai-Dorfman disease (RDD) is a rare proliferative histiocytic disorder that is characterized by persistent massive lymphadenopathy mimicking malignant tumors. Extranodal RDD is uncommon, and more severe fibrosis and fewer histiocytes in lesions make the diagnosis of RDD more difficult than that of nodal RDD. We herein described a 31-year-old male patient with isolated soft tissue RDD of the head and neck. The patient was referred to our hospital with a right neck mass. Computed tomography (CT) scans showed a diffuse enhanced tumor with an unclear border in the right side of the neck. 18F-fluorodeoxyglucose positron emission tomography (PET)/CT revealed high uptake in the corresponding lesion. Second wide local excisional biopsy led to a diagnosis of RDD, and immunohistochemistry was useful for diagnosing RDD. A systemic treatment with steroids improved his symptoms, including the neck mass. Physicians need to consider the diagnostic difficulty associated with extranodal soft tissue RDD as well as its rarity.
Auris Nasus Larynx 2016 Jun
PMID:Extranodal soft tissue Rosai-Dorfman disease of the head and neck and its diagnostic difficulty. 2652 42