Multimodality Imaging of a Rare Extranodal Rosai-Dorfman Disease Involving Great Vessels
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Rare disease
Multimodality
Rosai-Dorfman disease (RDD) is a rare, multisystemic histiocytic disorder, and commonly manifesting as lymphadenopathy in the young male. Abdominal manifestations of RDD are extremely rare.In August 2018, a 42-year-old man underwent an abdominal ultrasonography examination due to his weight loss of 10 kg in only three months and found a giant solid tumor was found in his spleen. Then, he was admitted to our hospital and diagnosed as a splenic mass via abdominal enhanced CT and MRI. Laparoscopic splenectomy was administrated within six days of admission due to the clear surgical indications. The pathogenesis of RDD remained poorly understood and the disease should be diagnosed based on histopathology and immunohistochemistry (IHC). The mutations in ATM and NFKBIA were observed using next generation sequencing (NGS).We reported a case of splenic involvement of RDD with NGS genetic testing, indicating the difficulty of making a diagnosis before surgery. This extremely rare case offers new references for the understanding of abdominal viscera RDD.
Rare disease
Histopathology
Abdominal ultrasonography
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Emperipolesis
Etiology
Sinus (botany)
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Multimodality
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Rosai-Dorfman disease (RDD) is a rare, benign pseudolymphomatous condition, predominantly involving lymph nodes. Rosai-Dorfman disease (RDD) (sinus histiocytes with massive lymphadenopathy) rarely affects the intracranial region without involvement of other sites. It is a rare and idiopathic histoproliferation disorder characterized by painless lymphadenopathy. We report a case of 43-year-old male who presented with unconsciousness; MRI was done and right temporofrontal mass was found. Excision was done, and on histopathology it confirmed RDD.
Unconsciousness
Emperipolesis
Histopathology
Cervical lymphadenopathy
Sinus (botany)
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Multimodality
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Rosai-Dorfman disease is an uncommon benign non-Langerhans cell histiocytosis proliferative disorder. It commonly involves the lymph nodes and is classically presented with massive cervical lymphadenopathy. It can also involve the extra-nodal sites of the body and is reported in 43% of Rosai-Dorfman disease cases. The central nervous system is rarely involved in Rosai-Dorfman disease from which intra-cranial is more common than spinal lesions. An isolated dural-based intra-cranial Rosai-Dorfman disease is extremely rare. It usually mimics meningioma on radiological imaging. It is very difficult to diagnose the Rosai-Dorfman disease on imaging and intra-operative appearances of the lesion. We present a histopathologically proven case of an isolated Rosai-Dorfman disease involving the central nervous system in 49 years old female patient which was radiologically mimicking the meningioma.
Emperipolesis
Cervical lymphadenopathy
Langerhans Cell Histiocytosis
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Multimodality
Multimodal transport
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Rosai-Dorfman disease (RDD) is a rare but usually benign non-Langerhans cell histiocytic disorder which commonly affects lymph nodes and frequently present with bilateral painless enlarged cervical lymph nodes with systemic symptoms. Although extra nodal tissue involvement is seen in considerable proportion, purely cutaneous involvement without affecting lymph nodes is very rare and forms a diverse clinical entity. Histopathology with immunohistochemical staining remains gold standard in diagnosing this rare entity. Different treatment modalities have been tried in case reports and treatment response is variable. Here we report a case of pure cutaneous RDD in a 21-yearold young female who was referred for staged excision.
Histopathology
Rare disease
Cervical lymph nodes
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Rosai-Dorfman disease (RDD) is a histiocytic disorder that has only a skin implication in a very small percentage of cases. RDD is usually painless and accompanied by disseminated lymphadenopathy. We present a rare case of a female patient that complained of grouped skin papules localized on the left leg, associated with a palpable deep nodular lesion. Initially, this was clinically mistaken for a soft tissue sarcoma, but after a total body CT and surgical excision it was identified as a non-Langerhans cell benign histiocytosis known as RDD. The patient had neither recurrence nor systemic involvement after 7 months of follow-up.
Rare disease
Langerhans Cell Histiocytosis
Histiocytic sarcoma
Skin lesion
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The purpose of this paper is to explore the uses of multimodality within early childhood education classrooms for the purposes of literacy education. Wohlwend (2008) urges educators to keep age-appropriate practices in place, even amid a shift in educational requirements and expectations. In this paper, the use of age-appropriate, multimodal practices for young learners is explored. Though there is not necessarily research specific to multimodality in early childhood, the literature shows that multimodality is present in forms of play; the use of toys, devices, and technology; drama; and social interactions. Through these modality-rich avenues, literacy development can still be achieved through engaging structures for children. Multimodality creates opportunities to position the student as the expert in their own learning and create collaborative learning environments. Potential issues with these uses of multimodality include conflicts around devices in the classroom and negative social interactions. Additional research is needed to connect the fields of multimodality and early childhood education.
Multimodality
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