Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease
We present a challenging diagnostic and management case of Rosai-Dorfman-Destombes disease in a 48-year-old woman with a relapsing, partially steroid-responsive syndrome comprising patchy, non-length-dependent radiculoneuropathy with diffuse pachymeningitis and widespread systemic disease, and recent dramatic response to novel mitogen-activated kinase pathway inhibition. We discuss the clinical characteristics, diagnosis, recent breakthroughs in pathogenesis and emerging treatment options for Rosai-Dorfman disease and for the histiocytoses with neurological sequelae, including Langerhans cell histiocytosis and Erdheim-Ches...
Source: Practical Neurology - May 9, 2022 Category: Neurology Authors: Antonia S Carroll Carolynne M Doherty Julian Blake Stephen J Hunt Chandrashekar Hoskote Feargal McNicholl Rahul Phadke Oonagh Sheehy Fion D Bremner Shirley D'Sa Christopher McNamara Mary M Reilly Source Type: research

Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease
We present a challenging diagnostic and management case of Rosai-Dorfman-Destombes disease in a 48-year-old woman with a relapsing, partially steroid-responsive syndrome comprising patchy, non-length-dependent radiculoneuropathy with diffuse pachymeningitis and widespread systemic disease, and recent dramatic response to novel mitogen-activated kinase pathway inhibition. We discuss the clinical characteristics, diagnosis, recent breakthroughs in pathogenesis and emerging treatment options for Rosai-Dorfman disease and for the histiocytoses with neurological sequelae, including Langerhans cell histiocytosis and Erdheim-Ches...
Source: Practical Neurology - May 9, 2022 Category: Neurology Authors: Antonia S Carroll Carolynne M Doherty Julian Blake Stephen J Hunt Chandrashekar Hoskote Feargal McNicholl Rahul Phadke Oonagh Sheehy Fion D Bremner Shirley D'Sa Christopher McNamara Mary M Reilly Source Type: research

Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease
We present a challenging diagnostic and management case of Rosai-Dorfman-Destombes disease in a 48-year-old woman with a relapsing, partially steroid-responsive syndrome comprising patchy, non-length-dependent radiculoneuropathy with diffuse pachymeningitis and widespread systemic disease, and recent dramatic response to novel mitogen-activated kinase pathway inhibition. We discuss the clinical characteristics, diagnosis, recent breakthroughs in pathogenesis and emerging treatment options for Rosai-Dorfman disease and for the histiocytoses with neurological sequelae, including Langerhans cell histiocytosis and Erdheim-Ches...
Source: Practical Neurology - May 9, 2022 Category: Neurology Authors: Antonia S Carroll Carolynne M Doherty Julian Blake Stephen J Hunt Chandrashekar Hoskote Feargal McNicholl Rahul Phadke Oonagh Sheehy Fion D Bremner Shirley D'Sa Christopher McNamara Mary M Reilly Source Type: research

Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease
We present a challenging diagnostic and management case of Rosai-Dorfman-Destombes disease in a 48-year-old woman with a relapsing, partially steroid-responsive syndrome comprising patchy, non-length-dependent radiculoneuropathy with diffuse pachymeningitis and widespread systemic disease, and recent dramatic response to novel mitogen-activated kinase pathway inhibition. We discuss the clinical characteristics, diagnosis, recent breakthroughs in pathogenesis and emerging treatment options for Rosai-Dorfman disease and for the histiocytoses with neurological sequelae, including Langerhans cell histiocytosis and Erdheim-Ches...
Source: Practical Neurology - May 9, 2022 Category: Neurology Authors: Antonia S Carroll Carolynne M Doherty Julian Blake Stephen J Hunt Chandrashekar Hoskote Feargal McNicholl Rahul Phadke Oonagh Sheehy Fion D Bremner Shirley D'Sa Christopher McNamara Mary M Reilly Source Type: research

Rosai-Dorfman Disease of the Chest Wall Following Mastectomy for Breast Cancer
We present a unique case of multifocal soft tissue RDD originating from previous mastectomy site and retroperitoneum. The patient is a 62-year-old African American female with a prior history of bilateral breast invasive ductal adenocarcinoma. 2 years following completion of therapy, our patient re-presented with an abdominal wall mass. The core biopsy was discordant, and the mass had rapid growth prompting excision. After the excision of the abdominal wall mass pathology confirmed RDD. A PET confirmed a solitary mass behind the left kidney this mass was biopsied and confirmed that it was RDD in the retroperitoneum. Due to...
Source: The American Surgeon - May 5, 2022 Category: Surgery Authors: Mollie A Merrell Emily E Grenn W Shannon Orr Source Type: research