scholarly journals Presentation of External Ear Rosai-Dorfman Disease With Laryngeal Involvement

2021 ◽  
pp. 014556132110167
Author(s):  
Kenji R. Kobayashi ◽  
Yusif Hajiyev ◽  
Maren Y. Fuller ◽  
Gunes Orman ◽  
Elton M. Lambert

Rosai-Dorfman disease (RDD) is a rare benign systemic histiocytic proliferation characterized by massive lymph node enlargement and sometimes associated with extranodal involvement. Even though it is considered to be benign, death can occur depending on the extent and location. Our case highlights a primary extranodal site of the right pinna with extension through the Eustachian tube to the subglottis. A previously healthy 15-year-old female presented with 1-year right pinna swelling, slowly enlarging and becoming more bothersome. An incisional biopsy was performed on the ear along with S100 staining yielding a diagnosis. After multidisciplinary case discussion, clofarabine monotherapy and systemic therapy for Langerhans cell histiocytosis has started. Rosai-Dorfman disease can be a general disorder, often affecting the lymph nodes. Unlike a nodal disease, extranodal disease could involve any site on the patient’s anatomy. Head and neck lesions are the most common extranodal lesions. Rosai-Dorfman disease is self-limited in more than 20% of the cases with spontaneous regression without intervention; 70% of the patients have noticeable symptoms and vital organ involvement requiring treatments such as surgery, steroids, radiation, and chemotherapy. In our case, the patient had wide involvement and presented without any serious breathing difficulties; we decided to start with monotherapy with chemotherapy and systematic glucocorticoid treatment.

2020 ◽  
Vol 11 (4) ◽  
pp. 5187-5191
Author(s):  
Sivapriya G Nair ◽  
Jina Raj ◽  
Sajesh K Menon ◽  
Suhas Udayakumaran ◽  
Roshni P R

Rosai Dorfman disease is a rare histiocytic disorder. It is also known as Sinus Histiocytosis. It is with massive lymphadenopathy involves an overproduction of a type of white blood cell. The disease is rarely associated with intracranial and intraorbital involvement. Intracranial Rosai-Dorfman can mimic meningioma. Other pathologies also underline its pathologies. Here, we report a nine-year-old boy with a history of proptosis of the right eye and presenting with multiple skull lesions. Histopathological study revealed Sphenopetroclival lesion, which features that of Rosai Dorfman Disease. His MRI scan of the brain was taken, which showed evidence of right optic nerve meningioma with sella and suprasellar extension, causing severe proptosis. The child underwent right frontotemporal craniotomy with petrosectomy and Transylvanian, subtemporal approach to multicompartmental Rosai-Dorfmans lesion. After four months, the patient had a recurrence of the disease on which chemotherapy and steroids were started, which also did not show much response while taking an MRI scan. A corticosteroid is a useful option in the Central Nervous System Rosai Dorfman disease treatment. But this patient showed a negative outcome to the treatment.


2021 ◽  
Vol 4 (1) ◽  
pp. 33-37
Author(s):  
John Ogunkoya ◽  
Oluwatosin Yetunde Adesuyi

Background: The diaphragm is one of the most important muscles of respiration in the body separating the abdomen from the thorax. Abnormalities of the diaphragm could be congenital or acquired, morphological or functional while pulmonary infection e.g. pulmonary tuberculosis, is implicated in its etiology. Case presentation: A 63-year- old man with six weeks history of cough productive of yellowish sputum. Chest X-ray showed a uniform well-circumscribed opacity in the right lower lobe abutting on or in continuum with the right diaphragm consistent with a diaphragmatic hump. Sputum Gene Xpert was positive for Mycobacterium tuberculosis. Chest CT scan revealed bilateral lymph node enlargement with hyperdense lesions in the anterior basal segment of the right lower lobe and medial bronchopulmonary segments of the right middle lobe. He was treated for 6 months with first-line anti-tuberculosis drugs. Discussion: The incidence of the diaphragmatic hump on chest radiograph worldwide and among Nigerians is unknown. The association of diaphragmatic hump with chest infection has been well document. The association of diaphragmatic hump with pulmonary tuberculosis is uncommon. Conclusion: A high index of suspicion is needed to diagnose pulmonary tuberculosis with atypical clinical and radiological presentations. Such prompt diagnosis will aid the treatment of the disease.


2019 ◽  
Vol 2019 ◽  
pp. 1-6
Author(s):  
Andrew B. Ross ◽  
Kirkland W. Davis ◽  
Darya Buehler ◽  
Brian Y. Chan

Rosai-Dorfman disease (RDD), sometimes known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder that most commonly presents as painless, massive cervical lymphadenopathy in young adults. Extranodal disease can occur in up to 40% of patients but primary involvement of bone is rare. We present two cases of primary RDD of bone: one case of multifocal osseous RDD presenting as a painful lesion in the elbow, and one case of a solitary osseous lesion presenting as a painful lesion in the wrist.


2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Abdulvahap Akyigit ◽  
Hadice Akyol ◽  
Oner Sakallioglu ◽  
Cahit Polat ◽  
Erol Keles ◽  
...  

Rosai-Dorfman disease is a rarely seen disease with unknown etiology. Extranodal involvement is most commonly seen in the head and neck region. Histopathologically, it is characterized by histiocytic cell proliferation. This paper presents a case of a 15-year-old male patient who presented with nasal obstruction and was surgically treated for a mass filling in the left nasal meatus that was diagnosed to be Rosai-Dorfman disease by histopathological examination.


2009 ◽  
Vol 124 (3) ◽  
pp. 345-348 ◽  
Author(s):  
M Ilie ◽  
N Guevara ◽  
L Castillo ◽  
P Hofman

AbstractBackground:Rosai–Dorfman disease is a rare, idiopathic, histiocytic proliferative disorder with a distinctive microscopic appearance, which was formerly thought to be a disease process limited to lymph nodes. However, extranodal involvement has been documented in less than half of the reported patients, but rarely without associated lymphadenopathy.Case report:We report the case of a 43-year-old Senegalese woman who presented with a polypoid, intranasal mass caused by Rosai–Dorfman disease. A diagnosis of a granulomatous process, including rhinoscleroma, was initially discussed. The correct diagnosis was made histologically by demonstrating aggregates of histiocytes with large amounts of cytoplasm, emperipolesis and protein S100 antigen expression. Despite using ancillary methods (molecular biology and electron microscopy), we failed to demonstrate any associated pathogen.Conclusion:Diagnosis of Rosai–Dorfman disease can be very difficult, in particular in adults from Africa with pure, isolated, intranasal localisation, in whom clinical and radiological features may mimic other infectious or neoplastic disorders. The diagnosis is made based on the histological presence of large histiocytes with lymphophagocytosis. Moreover, immunohistochemical analysis of these histiocytes using anti-protein S100 antibody shows strong positivity.


1992 ◽  
Vol 106 (3) ◽  
pp. 249-251 ◽  
Author(s):  
W. Y. W. Tsang ◽  
J. K. C. Chan ◽  
W. K. Ho ◽  
H. C. Yu ◽  
L. T. C. Chow

AbstractA 40-year-old woman presented with a nodule over the tragus of the right ear. A biopsy was initially reported as showing non-specific inflammation. In view of the persistence of the lesion, the histological material was reviewed, leading to revision of the diagnosis to Rosai-Dorfman disease, a diagnosis further confirmed by immunoreactivity of the histiocytes for s-100 protein. This case represented the extranodal form of Rosai-Dorfman disease in the absence of lymph node involvement.


2012 ◽  
Vol 67 (5) ◽  
pp. e225-e226 ◽  
Author(s):  
Christopher G. Bunick ◽  
David Leffell ◽  
Marcus Bosenberg ◽  
Joachim Yahalom ◽  
Jennifer N. Choi

2012 ◽  
Vol 126 (8) ◽  
pp. 837-839 ◽  
Author(s):  
J Ajduk ◽  
M Ries ◽  
D Vagic ◽  
A Batos-Tripalo

AbstractBackground:Temporomandibular joint fistula into the external auditory canal is a very rare condition. We report a case of spontaneous fistula which presented with repeated episodes of clear fluid otorrhoea and ear infection.Case report:A 53-year-old woman complained of occasional episodes of clear fluid otorrhoea from the right ear followed by infection. Otoscopic examination showed a normal tympanic membrane and normal skin in the external auditory meatus. Otomicroscopy showed a small punctum on the anteroinferior part of the meatus. Computed tomography identified a foramen of Huschke. Surgery revealed a fistula between the meatus and the temporomandibular joint, which was successfully resolved.Conclusion:This report presents a rare case of a fistula between the temporomandibular joint and the external auditory meatus, associated with Huschke's foramen. Such a defect can be quite difficult to detect. It should be suspected in cases of chronic otorrhoea when neither middle nor external ear disease is apparent. Surgery is usually successful, and is advised in cases with repeated complications.


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