Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy

1993 ◽  
Vol 79 (5) ◽  
pp. 769-773 ◽  
Author(s):  
Ellen G. Shaver ◽  
Susan L. Rebsamen ◽  
Anthony T. Yachnis ◽  
Leslie N. Sutton

✓ Sinus histiocytosis with massive lymphadenopathy was first described in 1969 by Rosai and Dorfman. The typical clinical characteristics of this disease include painless cervical lymphadenopathy, fever, and weight loss. The condition can present with an extranodal mass in about 25% of patients, and isolated masses without lymph node involvement occur rarely. The authors describe a 5-year-old boy with cavernous sinus syndrome due to an isolated extranodal form of sinus histiocytosis with massive lymphadenopathy in the temporal fossa. Several cases of this disease involving the central nervous system are reviewed. The histopathological and magnetic resonance imaging characteristics are discussed.

2018 ◽  
Vol 2 (1) ◽  
Author(s):  
Jianying Feng ◽  
Tiantian Han ◽  
Fuyong Jiao

Sinus histiocytosis with with massive lymphadenopathy is a benign lymphoproliferative disorder.In 1969, Rosai and Dorfman[1] made detailed research on it, so it was also called Rosai DorfnlRn disease (rosai.dorfmandisease, RDD).The clinical manifestations were fever, neck lymph node enlargement, leukocytosis and high gamma globulin.Histopathological findings showed that lymph node involvement was present in group RDD, and the infiltration of the cells was predominant, especially the phagocytosis of the histiocytic cells.About 43% of RDD patients have lymph node involvement in [2], in which the skin is the most common extranodal organs involved.About 10% of patients with skin damage, skin rash and morphological diversity, is easy to be misdiagnosed.In this paper, through the analysis of a case of RDD and EB virus infection, in clinical in patients with special infection can be early detection and treatment.


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.


2009 ◽  
Vol 24 (4) ◽  
pp. 760 ◽  
Author(s):  
Jinyung Ju ◽  
Yong Soo Kwon ◽  
Kae Jung Jo ◽  
Dong Ryeol Chae ◽  
Jung Hwan Lim ◽  
...  

Author(s):  
Jyotika Waghray ◽  
Pradyut Waghray

<p>Rosai-Dorfman’s disease also known as sinus histiocytosis with massive lymphadenopathy (SHML) is characterized by distorted lymph node architecture with marked dilation of lymphatic sinuses occupied by numerous lymphocytes, as well as histiocytes with vesicular nucleus and abundant clear cytoplasm with phagocytized lymphocytes or plasma cells, also known as ‘emperipolesis’. This disease of unknown etiology progresses with a benign prognosis strictly and only when an early diagnosis and treatment is made. A late diagnosis and a generalized lymph node involvement contribute to a poor prognosis. We reported a case of a 29-year-old Indian female with a 4-month history of painful unilateral cervical mass and low-grade fever with the final diagnosis of Rosai-Dorfman disease. The final diagnosis was made by fine needle aspiration (FNA) biopsy of the cervical lymph node. In conclusion, FNA biopsy can be enough to make the diagnosis in most cases due to the distinct cytological features of SHML, thereby avoiding more invasive approaches that potentially are unnecessary.</p>


2018 ◽  
Vol 4 (2) ◽  
pp. 31-35
Author(s):  
Arifullah Bangash ◽  
Syed Zafar Hassan ◽  
Ghulam Muhammad

OBJECTIVETo determine the proportion of cervical lymphadenopathy in systemic diseases, their presentation and investigative tools.METHODOLOGYThis descriptive study was carried out from 1st July 2014 to 30 June 2017 in ENT and head and neck department Naseer Teaching Hospital and Town Teaching Hospital, Peshawar. Total of 270 patients with enlarged cervical lymph nodes, of either sex and of any age were approached for inclusion into the study. Patients with suspected acute inflammation were given a trial of antibiotic and followed for two weeks were excluded from the study. Excisional biopsy of the lymph nodes was performed in all these patients with six weeks or more duration.RESULTSIn our study out of 270 patients, 158 (58.5%) were males and the majority of patients (63.3%) had ages from 11-40 years. Tuberculous cervical lymphadenopathy was diagnosed in 145 (53.7%) patients, reactive hyperplasia in 53 (19.6%), lymphoma in 32 (11.8 %,) metastasis to cervical lymph nodes in 30 (11.1%), sarcoidosis in 7 (2.6%) and other very rare conditions as Kawasaki, Kikuchie and Rosi Dorfman were found in only one of each (0.4%). About 136 (50.4%) of the patients had involvement of multiple lymph nodes while 134 (49.6%) had single swelling. The matted lymph nodes were found in 162 (60%) whereas discrete lymph nodes were found in only 108 (40%) cases. Cold abscess was found in 19 (7%) of patients.CONCLUSIONTuberculosis is the commonest cause of cervical lymphadenopathy, with the majority of these patients having multiple lymph node involvement. In children, usually cervical lymphadenopathy is reactive or infective while in older age mostly metastatic.


1984 ◽  
Vol 60 (4) ◽  
pp. 850-852 ◽  
Author(s):  
Michel Trudel

✓ An unusual case of an isolated histioproliferative lesion arising from the basal dura of the left middle cranial fossa is described. The presence of lymphophagocytosis suggests that this represents an extra-nodal, intracranial form of sinus histiocytosis with massive lymphadenopathy.


1996 ◽  
Vol 85 (4) ◽  
pp. 692-696 ◽  
Author(s):  
Richard K. Osenbach

✓ Sinus histiocytosis with massive lymphadenopathy (Rosai—Dorfman disease) is a nonneoplastic lymphoproliferative disorder that usually presents with massive painless cervical lymphadenopathy accompanied by fever and weight loss. Extranodal involvement occurs in over 40% of patients; however, isolated extranodal disease without lymph node involvement is unusual. Extranodal involvement of the nervous system is unusual and isolated central nervous system disease in the absence of nodal disease is distinctly rare. The author describes the case of a 35-year-old man who presented with paraplegia and an enhancing intramedullary lesion on magnetic resonance imaging that was suggestive of a primary spinal cord tumor. The lesion was completely resected and histologically proved to be consistent with an isolated extranodal case of Rosai—Dorfman disease. Although a number of cases of this entity involving the CNS have been published, the author believes this case represents the first report of intramedullary spinal cord involvement as the initial and only sign of disease.


Author(s):  
Kalpana Giri ◽  
Ashok Baral ◽  
Niva Tiwari ◽  
Krishna Sharma

Rosai-Dorfman disease (RDD) is referred to as Sinus Histiocytosis Massive Lymphadenopathy (SHML) of unknown origin. We are reporting a rare case of Rosai Dorfman disease, a case of 6 years old male with a history of multiple painless bilateral submandibular and cervical lymphadenopathy.


1988 ◽  
Vol 69 (4) ◽  
pp. 610-612 ◽  
Author(s):  
Akio Asai ◽  
Masao Matsutani ◽  
Takeshi Kohno ◽  
Takamitsu Fujimaki ◽  
Hideki Tanaka ◽  
...  

✓ An extremely rare case of isolated histioproliferative lesions arising from the subarachnoid space of the left occipital convexity and the orbit is presented. The presence of histiocytes showing lymphophagocytosis and positivity for S-100 protein staining confirmed that the lesions represented extranodal forms of sinus histiocytosis with massive lymphadenopathy.


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