Massive Lymphadenopathy Rosai-Dorfman Disease Research Articles

Sinus histiocytosis and massive lymphadenopathy (Rosai-Dorfman disease) in an 8 year old female child: a rare case report

Introduction: Sinus histiocytosis with massive lymphadenopathy is an infrequent, self-resolving histoproliferative disorder of benign nature, also known as Rosai–Dorfman Disease. The typical manifestation of this disease includes bilateral, progressive cervical lymphadenopathy along with pyrexia. Laboratory manifestations consist of raised erythrocyte sedimentation rate, leukocytosis and hypergammaglobulinemia. Case report: An 8 year old female child presented with progressive, painful cervical and submandibular lymphadenopathy of three months duration. There was polymorphic leukocytosis and anemia along with raised Erythrocyte sedimentation rate and hypergammaglobulinemia. Fine Needle Aspiration cytology revealed sinus histiocytosis with massive lymphadenopathy, which was confirmed by the cervical lymph node biopsy. The histopathology revealed dilated sinuses filled with lympho plasma cells, large histiocytes and engulfed neutrophils (Emperipolesis). Conclusion: Massive, progressive bilateral cervical and sub mandibular lymphadenopathy, suggesting Sinus histiocytosis and massive lymphadenopathy-Rosai–Dorfman Disease. This was confirmed by Cytology, histopathology and immuno- histochemistry.

Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman disease: a unique case presentation

Rosai and Dorfman first described sinus histiocytosis with massive lymphadenopathy (SHML) in 1969 with an article detailing 4 cases in which they differentiated this disease entity from the grouping of diseases categorized as histiocytosis X, where it was previously classified. Also known as Rosai-Dorfman disease (RDD), it is clinically characterized as massive, painless, bilateral, symmetric cervical lymphadenopathy, with fever and leukocytosis. An 11-year-old African American boy was referred to our clinic for extraction of a severely decayed tooth #30 and evaluation of a large right-sided neck mass. Initially, the patient had been seen by his general dentist who had diagnosed the mass as an odontogenic abscess. After 2 courses of different antibiotics, no changes in the mass were noted. Subsequently, the patient was sent to the emergency department where CT revealed multiple right-sided neck masses with the largest measuring 4 × 2 cm. The patient underwent an incisional biopsy by otolaryngology and a diagnosis of necrotic lymph tissue was made. Upon our examination, the carious tooth #30 was felt to be an incidental finding and fine-needle aspiration cytology of the largest mass was performed in 2 places. This also provided a diagnosis of necrotic lymph tissue. In concert with the patient and his mother, the decision was made to excise the mass because of psychosocial concerns. A massive right-sided lymph node attached to the submandibular gland was found and excised without complication. Histologic examination with S-100 stain confirmed a diagnosis of RDD. The patient healed well following surgery and has experienced no further lymphadenopathy. This case presentation and review of the literature is unique, as the patient presented with unilateral cervical lymphadenopathy only. Open biopsy and 2 fine-needle aspirations all returned as necrotic lymph tissue. Obtaining the correct diagnosis was additionally hampered by coincidental dental pathology on the affected side and final diagnosis was made only by excisional biopsy, which is not necessarily indicated in cases of RDD.