ROSAI DORFMAN DISEASE MANAGEMENT, ACTIVE INTERVENTION OR WAIT AND WATCH: A CASE SERIES

ABSTRACT Rosai-Dorfman disease (RDD) is a rare histiocytic disorder commonly manifested as histiocytic proliferation of lymph nodes and extra nodal tissues initially described as a separate entity by Rosai and Dorfman under the term sinus histiocytosis with massive lymphadenopathy (SHML) in 1969. The treatment strategies can be variable and based on the severity of disease, v ital organ invol vement and pos s ible complications. The first case was a 47 years old female presenting with nasal obstruction and submandibular lymphadenopathy since few days. The second case was a 8 year old girl presented with stridor and lymphadenopathy in the submandibular region. In both the cases spontaneous resolution was expected and intervention was done only if there werecomplications. Patients were followed up every three months for two years and both the cases have resolved completely without any sequelae of the disease.Here we present two cases of Rosai-Dorfman disease presented to our hospital, this case series adds to the growing knowledge of Rosai-Dorfman di sease, along with under s tanding of pathophysiology, clinical diagnosis, treatment strategies and recovery options in cases where complications occurred.

Systemic and Craniospinal Rosai Dorfman Disease with Intraparenchymal, Intramedullary and Leptomeningeal Disease

Rosai Dorfman disease is a rare histiocytic disorder of over-production of non-Langerhans histiocytes, which typically manifests with massive lymphadenopathy and sinonasal involvement. We report a rare case of systemic and disseminated craniospinal Rosai-Dorfman disease with intraparenchymal and leptomeningeal involvement, but no sinus or dural-based disease. The diagnosis was established by biopsy of a hypothalamic mass. Additionally, UCSF500 Next Generation Sequencing demonstrated a solitary pathogenic alteration affecting the BRAF oncogene, which supports the morphologic and immunohistochemical diagnosis of Rosai-Dorfman disease.

Histiocytosis in Nigerian children: A report of two variants

Histiocytoses are a rare group of proliferative disorders with very similar clinical and histological pictures. We present a case report of two variants seen in an eight-month-old female and five-month-old male in a tertiary hospital in southern Nigeria. They both presented with painless neck swellings and fever, leucocytosis, neutrophilia and lymphopenia. Initial histologic examinations of the cervical lymph nodes biopsy posed a diagnostic conundrum. However, Immuno-histochemical analysis done on both sample showed CD1a, positive S100 in keeping with Langerhans cell histiocytosis in the former. While, that of the latter showed strongly positive CD68, positive S-100 in 30% cells in keeping with Sinus histiocytosis with massive lymphadenopathy (SLMH) in the latter. Clinicians should have a high index of suspicion for histiocytosis in children presenting with generalised lymphadenopathy. Also, apart from the routine histology, immunohistochemistry analysis is recommended for all cases

Primary Rosai-Dorfman Disease in 39-Year-Old Woman With Osseous Tibial Lesion Manifestion: A Case Report and Literature Review

Rosai-Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy (SHML), is a very rare and typically benign disorder of unknown etiology with <10% bone involvement. The report is of a case seen at the authors’ hospital of a patient presenting with several months’ onset unspecified nontraumatic ankle pain. There was no physical mass or lymphadenopathy appreciated on examination. Plain radiographs and magnetic resonance images demonstrated an osteolytic lesion at the medial malleolus. Biopsy revealed the diagnosis of intraosseous manifestation of Rosai-Dorfman disease.

Secondary biphenotypic leukemia following Rosai-Dorfman-Disease A coincidence?

Rosai-Dorman-Disease is one subgroup of Non-Langerhans cell histiocytosis with massive lymphadenopathy. It is a generally a benign and self-limiting disease, but an association to hemato-lymphoid malignancies or immune diseases is suspected. We present the case of a 6-year old male patient with multifocal extranodal RDD developing a secondary biphenotypic, treatment-resistant leukemia during treatment. Hypothysing that RDD and the secondary leukemia could be based on the same malign clone or on a cancer predispostion syndrome, whole-exome-sequencing of a saliva sample and leukemic cells were performed. A common marker of cell lines of histiocytosis and leukemic blasts has not been found.

Diagnosis of Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease) by Fine Needle Aspiration Cytology in a Paediatric patient

Rosai-Dorfman Disease (RDD) is also known as Sinus Histiocytosis of Massive Lymphadenopathy(SHML). Clinically Rosai-Dorfman Disease is characterized by massive, painless, bilateral cervicallymph node enlargement, often mimics lymphoma. Microscopically, it shows dilatation of lymphaticsinuses occupied by numerous lymphocytes and proliferation of histiocytes with abundant paleeosinophilic cytoplasm containing engulfed lymphocytes or plasma cells- emperipolesis.

An extremely rare case of Rosai–Dorfman disease in the thymus

Background There were very few reports of Rosai–Dorfman disease in the thymus, which known as sinus histiocytosis with massive lymphadenopathy. It usually accompanied with other systemic symptoms such as fever, malaise, night sweats, or weight loss in the short term. Case presentation We herein report a rare case of RDD of thymic origin and a review of the literature concerning the clinical and pathological features of this disease, which is often misdiagnosed as thymoma. The patient was underwent thymectomy to extirpate the lesion completely by video-assisted thoracic surgery. Conclusions To the best of our knowledge, this is the fourth thymus occurring RDD case with proof via histology and IHC. Our findings suggest the difficulty of making a diagnosis before surgery and more cases will need to be reported in order to facilitate the preoperative diagnosis of such a rare tumor.

HISTOCYTOPATHOLOGICAL DIAGNOSIS OF ROSAI-DORFMAN DISEASE: CASE REPORT AND LITERATURE REVIEW

Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) being a rare benign proliferative self-limiting disease of the cells of macrophage-histiocyte family is of unknown etiology and presents with massive lymphadenopathy. We are hereby reporting a case of RDD presenting with massive bilateral cervical and submandibular lymphadenopathy, diagnosed by histocytopathology.

Rosai Dorfman Disease

Rosai-Dorfman disease (RDD) is referred to as Sinus Histiocytosis Massive Lymphadenopathy (SHML) of unknown origin. We are reporting a rare case of Rosai Dorfman disease, a case of 6 years old male with a history of multiple painless bilateral submandibular and cervical lymphadenopathy.

ROSE in Rosai–Dorfman–Destombes (RDD) disease: a cytological diagnosis

Background Rosai–Dorfman–Destombes (RDD) is also known as sinus histiocytosis with massive lymphadenopathy (SHML). It is a benign proliferative disorder of histiocytes, affecting lymph nodes, rarely with extra-nodal involvement. Rapid on-site evaluation (ROSE) with fine-needle aspiration cytology (FNAC) can be utilized as a minimally invasive investigation to avoid unnecessary surgery of this self-limiting disease. Case presentation A 65-year-old female presented with complaints of bilateral cervical lymphadenopathy since 1 year. Rapid on-site stain with FNAC from bilateral cervical lymph nodes revealed features of Rosai–Dorfman–Destombes (RDD) disease. Conclusion FNAC with rapid on-site evaluation can provide a simple and cost-effective method for looking at the unique cytological features of the disease and act as a first-line investigation.