Sinus Histiocytosis with Massive Lymphadenopathy

1989 ◽  
Vol 98 (3) ◽  
pp. 241-242 ◽  
Author(s):  
Nour Sneige ◽  
John G. Batsakis

Sinus histiocytosis with massive lymphadenopathy is a lymphoreticular disorder of obscure pathogenesis with extranodal lesions and a variable clinical course. Clinical manifestations are most often in the head and neck, with 97% of patients showing cervical lymphadenopathy and a significant but lesser number having lesional infiltrates in the upper aerodigestive tracts, particularly the nasal cavity and salivary glands.

2007 ◽  
Vol 131 (7) ◽  
pp. 1117-1121 ◽  
Author(s):  
Sujata Gaitonde

Abstract Context.—This article provides an overview of the major pathologic manifestations of sinus histiocytosis with massive lymphadenopathy, including patient characteristics and current knowledge about its pathogenesis, with an emphasis on multifocal and extranodal presentation. Sinus histiocytosis with massive lymphadenopathy is a rare, nonneoplastic, idiopathic, proliferative histiocytic disorder; recognition of this disorder is important to avoid misinterpretation and subsequent unnecessary treatment. This is especially true for primary extranodal manifestation of this rare disorder. Although accurate diagnosis of this entity requires a correlation of clinical, radiologic, laboratory, and pathologic studies in most cases, it remains a disorder primarily defined by its histopathologic features and pathologic manifestations, which are key to the diagnosis. Objective.—To summarize the scientific literature, provide a concise review, and emphasize the diagnostic histopathologic features of extranodal sinus histiocytosis with massive lymphadenopathy. Data Sources.—A comprehensive literature review was undertaken to summarize the clinical and pathologic features of this disorder. Conclusions.—Sinus histiocytosis with massive lymphadenopathy is characterized by a rare, acquired, nonmalignant proliferation of distinctive histiocytes that present with lymphadenopathy or extranodal disease, primarily in children and young adults. It exhibits a broad range of clinical presentations, thus eliciting a wide differential diagnosis. The diverse clinical manifestations and frequent association with subtle or severe immunologic abnormalities suggest an immune-mediated cause. Additional studies are needed to characterize the interplay between death receptors and cytotoxic mediators and to further elucidate the loss of immune hemostasis that may underlie idiopathic histiocytic proliferations such as this.


1979 ◽  
Vol 88 (4) ◽  
pp. 486-494 ◽  
Author(s):  
Michael Setzen ◽  
Steven Sobol ◽  
James M. Toomey

The clinical manifestations of 29 recently encountered sarcomas of the head and neck were analyzed in an attempt to define more accurately the diagnostic characteristics and therapeutic responses of these unusual tumors. The host factors of age, sex distribution, race, habits and associated features differ sufficiently to distinguish the sarcoma from the carcinoma population. In addition, the clinical course of sarcoma patients as monitored by mode of presentation, site of involvement, tumor histology, diagnostic features and certain elements of management and outcome, further indicates that these tumors can be defined and managed as a distinct group of lesions. A review of the recent literature supports the major conclusions of this study.


1990 ◽  
Vol 102 (6) ◽  
pp. 764-767 ◽  
Author(s):  
Rahul K. Naidu ◽  
Mark L. Urken ◽  
Peter M. Som ◽  
Ardis Danon ◽  
Hugh F. Biller

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 ◽  
...  

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.


1999 ◽  
Vol 108 (11) ◽  
pp. 1095-1104 ◽  
Author(s):  
Antonino Carbone ◽  
Kenneth O. Devaney ◽  
Alberto Passannante ◽  
Alessandra Rinaldo ◽  
Annunziata Gloghini ◽  
...  

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.


1982 ◽  
Vol 96 (1) ◽  
pp. 89-94 ◽  
Author(s):  
E. O. Adekeye ◽  
M. B. Edwards ◽  
G. F. Goubran

SummarySinus histiocytosis with massive lymphadenopathy is a rare, benign lympho-proliferative disorder of unknown aetiology that chiefly affects the cervical lymphnodes of children. Various extranodal manifestations are recognized, especially in the head and neck. A case is described in a Nigerian child that illustrates problems of differential diagnosis.


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.


Sign in / Sign up

Export Citation Format

Share Document