Mediastinal Rosai-Dorfman Disease With Widespread Lesions: When Surgical Biopsy Is Needed

Publication date: January 2020Source: The Annals of Thoracic Surgery, Volume 109, Issue 1Author(s): Simone Furia, Nazarena Nannini, Anna Pascarella, Cristiano BredaRosai-Dorfman disease (RDD) is a rare benign disorder of the histiocytes, affecting lymph nodes in its classic form. Extranodal RDD is considered the uncommon subtype and potentially impairs all tissues and intrathoracic organs. In our report, a 18F-fluorodeoxyglucose positron emission tomography scan of a mediastinal mass infiltrating the lungs and of widespread lesions suggested the presence of a metastatic disease. Open thoracic biopsy was required for immunochemistry and histopathology. Mediastinal mass with lesions disseminated throughout the body is an unusual presentation of extranodal RDD that calls for a challenging differential diagnosis to rule out the suspicion of metastatic malignancy.
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research

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CONCLUSIONS: Pulmonary involvement in RDD is rare, proteiform, and sometimes severe. The MEK inhibitor cobimetinib can lead to dramatic responses. PMID: 31669429 [PubMed - as supplied by publisher]
Source: Chest - Category: Respiratory Medicine Authors: Tags: Chest Source Type: research
A 47-year-old Afro-Caribbean lady presented with a 6-month history of multiple asymptomatic indurated, erythematous papules, nodules and plaques on thighs bilaterally. A deep incisional skin biopsy showed features in keeping with Rosai-Dorfman syndrome (RDD). Screening CT scan and MRI scans revealed retroperitoneal and sacral fibrosis associated with retroperitoneal and para-aortic lymphadenopathy. A retroperitoneal lymph node biopsy excluded an underlying lymphoma. Despite treatment with super potent topical steroids, protopic ointment, dapsone, azathioprine and mycophenolate mofetil, the patient ’s cutaneous lesion...
Source: Journal of the American Academy of Dermatology - Category: Dermatology Source Type: research
Publication date: Available online 14 June 2019Source: The Annals of Thoracic SurgeryAuthor(s): Simone Furia, Nazarena Nannini, Anna Pascarella, Cristiano BredaAbstractRosai-Dorfman disease (RDD) is a rare benign disorder of the histiocytes, affecting lymph-nodes in its classical form. Extranodal RDD is considered the uncommon subtype and potentially impairs all tissues as well as intrathoracic organs. In our report, a FDG-PET scan of a mediastinal mass infiltrating the lungs and of widespread lesions suggested the presence of a metastatic disease. Open thoracic biopsy was required for immunochemistry and histopathology. M...
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
Abstract Rosai-Dorfman disease (RDD) is a rare benign disorder of the histiocytes, affecting lymph-nodes in its classical form. Extranodal RDD is considered the uncommon subtype and potentially impairs all tissues as well as intrathoracic organs. In our report, a FDG-PET scan of a mediastinal mass infiltrating the lungs and of widespread lesions suggested the presence of a metastatic disease. Open thoracic biopsy was required for immunochemistry and histopathology. Mediastinal mass with lesions disseminated throughout the body is an unusual presentation of extranodal RDD that calls for a challenging differential d...
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Ann Thorac Surg Source Type: research
Conclusion: We describe a rare presentation of RDD as a solitary choroidal mass in an elderly patient with overlapping features of uveal melanoma. Definitive diagnosis could only be made on histology. RDD should be considered in the differential diagnosis of a choroidal lesion in the elderly.Ocul Oncol Pathol
Source: Ocular Oncology and Pathology - Category: Opthalmology Source Type: research
Rationale: Rosai-Dorfman disease (RDD) is a rare benign histiocytic disease that is commonly characterized by massive painless cervical lymphadenopathy and systemic manifestations. Isolated extranodal involvement, especially spinal involvement, is extremely rare. Patient concerns: A 28-year-old man presented with intermittent dorsodynia and bilateral lower-limb weakness and numbness. A magnetic resonance scan (MRI) showed an extradural lesion of the T6-T9 thoracic spine that lead to cord compression. Diagnoses: Histopathological findings showed distinctive emperipolesis and immunohistochemistry results that were po...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Conclusion: RDD is an important diagnosis that must be considered in the differential diagnosis of an orbital mass.Ocul Oncol Pathol
Source: Ocular Oncology and Pathology - Category: Opthalmology Source Type: research
This report will focus on the diagnostic imaging, treatment, and outcomes for 3 cases of Rosai-Dorfman disease. Imaging has typically utilized computed tomography (CT)/magnetic resonance imaging to detect extranodal involvement. However, the addition of fluorodeoxyglucose positron emission tomography/CT scans has shown value in identifying lesions unidentified or ambiguous on other modalities. Fluorodeoxyglucose positron emission tomography/CT detected disease involvement in 2 instances either not reported or not felt to be significant on correlative CT imaging. Areas of involvement included the stomach/liver in case 1, an...
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Online Articles: Original Articles Source Type: research
Conclusions: Magnetic resonance imaging, particularly postcontrast MRI, is the optimal modality for assessment of hypothalamic lesions. Peripheral enhancement with polygon sign and optic tract or chiasm edema without visual impairment are highly suggestive of hypothalamitis.
Source: Journal of Computer Assisted Tomography - Category: Radiology Tags: Neuroradiology Source Type: research
We report a history of Moroccan female 57 years old who was thought to have recurrent hemoptysis with endobronchial obstructive tumor and atelectasis of the lung. The diagnosis of Rosai-Dorfman disease was eventually made following a range of investigations including CT scans and histological analysis of specimens after bronchial d ébulking through rigid bronchoscopy. She has an upper right destroyed lobe and lobectomy was performed with histologically a definitive diagnosis of RDD. She remains well with no further progression of the disease. We think that Rosai-Dorfman disease can mimic endobronchialcarcinoma, so h...
Source: Indian Journal of Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
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