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Interatrial Septal Mass Secondary to Rosai-Dorfman Disease: A Rare Cause of Complete Heart Block.

ABSTRACT: Varying degrees of atrioventricular block can be associated with old age or a manifestation of an ischemic, metabolic, or infective pathology. In patients with no clear explanation, it is important to investigate secondary causes. Our case describes the first case of an adult with Rosai-Dorfman histiocytosis presenting with complete heart block. (Level of Difficulty: Advanced.).

SUBMITTER: Butt AH

PROVIDER: S-EPMC10157082 | biostudies-literature | 2023 May

REPOSITORIES: biostudies-literature

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Interatrial Septal Mass Secondary to Rosai-Dorfman Disease: A Rare Cause of Complete Heart Block.

Butt Abdul Hadi AH Peterzan Mark M Shah Chirag C Wright Matthew M Rinaldi Christopher A CA Gill Jaswinder J Kapetanakis Stamatis S Behar Jonathan M JM

JACC. Case reports 20230421

Varying degrees of atrioventricular block can be associated with old age or a manifestation of an ischemic, metabolic, or infective pathology. In patients with no clear explanation, it is important to investigate secondary causes. Our case describes the first case of an adult with Rosai-Dorfman histiocytosis presenting with complete heart block. (<b>Level of Difficulty: Advanced.</b>). ...[more]

PMID: 37152702

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Rosai-Dorfman disease as a rare cause of cervical lymphadenopathy - case report and literature review.

Project description:Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare, benign clinical entity of unknown cause. RDD is characterised by the overproduction and accumulation of histiocytes, primarily in the lymph nodes, although it may affect every organ and system. It predominantly affects children and young adults. Typically, patients are in good general condition, with massive cervical lymphadenopathy and fever. In about 40% of cases extranodal localisation of RDD is diagnosed. In laboratory tests the most common abnormalities are increased erythrocyte sedimentation rate (ESR), leukocytosis with neutrophilia, normocytic anaemia, and hypergammaglobulinaemia. Histopathological examination remains the mainstay of diagnosis - lymph nodes have massive sinusoidal dilation, containing histiocytes positive for S-100 and CD68, and negative for CD1a. Most patients do not require treatment as spontaneous remissions are observed. We present a brief review of the literature and the case of a six-year-old boy with cervical lymphadenopathy diagnosed with RDD. So far, the patient has not required systemic treatment and has been kept under observation.

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Rosai dorfman disease of the orbit.

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Case report: Rosai-Dorfman disease with rare extranodal lesions in the pelvis, heart, liver and skin.

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Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease.

Project description:Rosai-Dorfman disease is a rare subtype of non-Langerhans cell histiocytosis. With the last major report published in 1990, there is a paucity of contemporary data on this disease. Our objective was to report the clinicopathological features, treatments and outcomes of patients seen at a tertiary referral center. Sixty-four patients with histopathological diagnosis of Rosai-Dorfman disease were identified from 1994 to 2017 (median age 50 years; range, 2-79). The median duration from symptom onset to diagnosis was seven months (range, 0-128), which was also reflected in the number of biopsies required to establish the diagnosis (median 2; range, 1-6). The most common presentation was subcutaneous masses (40%). Of the 64 patients, 8% had classical (nodal only) and 92% had extra-nodal disease (67% extra-nodal only). The most common organs involved were skin and subcutaneous tissue (52%), followed by lymph nodes (33%). Three patients had an overlap with Erdheim-Chester disease, which had not been described before. Two of these were found to have MAP2K1 mutations. Commonly utilized first line treatments were surgical excision (38%) and systemic corticosteroids (27%). Corticosteroids led to a response in 56% of the cases. Of those treated initially, 15 (30%) patients developed recurrent disease. The most commonly used systemic agent was cladribine (n=6), with 67% overall response rate. Our study demonstrates that Rosai-Dorfman disease has diverse clinical manifestations and outcomes. While this disease has been historically considered a benign entity, a subset of patients endures an aggressive course necessitating the use of systemic therapies.

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Staging surgery for intraventricular bilateral giant Rosai-Dorfman disease in children.

Project description:IntroductionRosai-Dorfman disease (RDD) is an uncommon, benign, and idiopathic histiocytic proliferative disorder. Multiple intracranial RDD is extremely rare and treatment varies.Case presentationA 9-year-old girl was admitted with 3-month history of blurred vision and facial paralysis, a 2-month history of recurrent giggle, and cognitive impairment. Computed tomography and magnetic resonance imaging scans revealed bilateral ventricular masses based on the dural membrane and the diameters of the masses were 9.1 cm and 9.2 cm, respectively. The lesions were completely removed with staging surgeries. Fifteen months after operation, blurred vision was still present but facial paralysis and giggle and cognitive impairment disappeared. Imaging examinations suggested that there were no new or recurring lesions.ConclusionFor multiple large intracranial masses, surgical treatment is necessary and staged surgery benefits perioperative safety. Active follow-up with magnetic resonance imaging is necessary.

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