scholarly journals Sinonasal Manifestation of Rosai-Dorfman Syndrome

2015 ◽  
Vol 8 (1) ◽  
pp. 43-45
Author(s):  
Kiran Mishra ◽  
Lakshmi Vaid ◽  
Vivek Gogia

ABSTRACT Sinus histiocytosis with massive lymphadenopathy (SHML) (also known as Rosai-Dorfman syndrome) is a rare, idiopathic, benign and self-limiting histiocytic proliferative disorder. It most commonly involves the cervical lymph nodes. The disease has a benign course and involvement of the nasal cavity as an extranodal site is exceptional. Here, we report a case of bilateral sinonasal mass with subsequent involvement of cervical lymph nodes in 22-year-old lady. Histological examination of the cervical lymph nodes and nasal mass biopsy demonstrated evidences of Rosai-Dorfman disease (RDD). The clinical presentation, histologic characteristics, radiographic findings and treatment of the disease are discussed. How to cite this article Vaid L, Jain N, Gogia V, Mishra K. Sinonasal Manifestation of Rosai-Dorfman Syndrome. Clin Rhinol An Int J 2015;8(1):43-45.

2021 ◽  
Vol 12 (1) ◽  
pp. 72-75
Author(s):  
Hubert Daisley Jr ◽  
Sailaja Golamari ◽  
Lilly Paul ◽  
Deandra Thomas-Romain ◽  
Dawn Meyers ◽  
...  

estombes–Rosai–Dorfman disease—often simply referred to, in the literature, as Rosai–Dorfman disease (RDD)—is a rare, nonmalignant disorder of histiocyte proliferation typically involving the cervical lymph nodes. A subset of patients with RDD, however, display extranodal manifestations highly variable in presentation, more challenging to diagnose, and less likely to spontaneously regress when compared to the nodal manifestations. This study describes the case of a young African male presenting himself with multiple nodules involving the anterior abdominal wall, who was found to have extranodal RDD. The current mode of diagnosis and the clinical management of RDD are reviewed.


2020 ◽  
pp. 1-3
Author(s):  
Rinsha Surendranath ◽  
M Rajani

Rosai Dorfman Disease also called as Sinus Histiocytosis with Massive Lymphadenopathy is a benign proliferative disorder of histiocytes which typically affects lymph nodes of cervical region ,however extranodal sites are also being reported by many authors[1].FNAC is the investigation of choice as is it less invasive and more reliable.Aims :To study the various sites of involvement and diagnostic significance of FNAC along with the pattern of site and age distribution .We studied a series of 11 cases for a period of one year.Materials and methods: Eleven cases studied in the department of Pathology Govt. Siddhartha Medical College,Vijayawada.Material collected from Govt. General Hospital,Vijayawada.Results:The present study showed two extranodal,one axillary lymph node,one inguinal lymph node and seven cervical lymph node involment.Conclusion:Rosai- dorfman disease main clinical manifestion is cervical lymph node enlargement ,but other lymph nodes and extranodal site can also be involved.Most common age of presentation was between 20-40years


2005 ◽  
Vol 8 (5) ◽  
pp. 593-598 ◽  
Author(s):  
Khulood Al-Saad ◽  
Paul Thorner ◽  
Bo-Yee Ngan ◽  
J. Ted Gerstle ◽  
Abhaya V. Kulkarni ◽  
...  

Sinus histocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare histiocytic disorder that typically presents with chronic, self-limiting, cervical lymphadenopathy. We present a case, a diagnostic dilemma for multiple consultation services, of an otherwise well 17-year-old boy without lymphadenopathy who, 8 months after excision of a T9 lytic vertebral lesion and epidural mass that caused cord compression, again presented with cord compression from progressive vertebral disease, recurrent epidural mass, and development of a paraspinal mass and tibial lesion. The excised vertebral and epidural lesions, 2 paraspinal biopsies, and tibial biopsy were interpreted as chronic inflammation until large histiocytes were noted, which were positive for CD68, S100 protein, fascin, and MAC387, and demonstrated characteristic emperipolesis (lymphophagocytosis) that was diagnostic of Rosai-Dorfman disease. This atypical clinical behavior and sites of involvement of multiple bones but not of lymph nodes is unusual and constitutes the aggressive end of the clinical spectrum and a rare cause for cord compression.


2007 ◽  
Vol 81 (9) ◽  
pp. 4482-4491 ◽  
Author(s):  
Anthony E. Kincaid ◽  
Jason C. Bartz

ABSTRACT Animals that naturally acquire the prion diseases have a well-developed olfactory sense that they utilize for a variety of basic behaviors. To assess the potential for the nasal cavity to serve as a point of entry for prion diseases, a small amount of prion-infected brain homogenate was placed inferior to the nostrils of hamsters, where it was immediately sniffed into the nasal cavity. Hamsters extranasally inoculated with the HY strain of transmissible mink encephalopathy (TME) agent had an incubation period that was not significantly different from per os inoculation of the same dose of the HY TME agent. However, the efficiency of the nasal route of inoculation was determined to be 10 to 100 times greater based on endpoint dilution analysis. Immunohistochemistry on tissues from hamsters killed at 2-week intervals after inoculation was used to identify the disease-associated form of the prion protein (PrPd) to determine the route of prion neuroinvasion. Nasal mucosa-associated lymphoid tissue and submandibular lymph nodes initially accumulated PrPd as early as 4 weeks postinfection. PrPd was first identified in cervical lymph nodes at 8 weeks, in the mesenteric lymph nodes, spleen, and Peyer's patches at 14 weeks, and in the tongue 20 weeks after inoculation. Surprisingly, there was no evidence of PrPd in olfactory epithelium or olfactory nerve fascicles at any time after inoculation. Therefore, the HY TME agent did not enter the central nervous system via the olfactory nerve; instead, PrPd accumulated in elements of the cranial lymphoreticular system prior to neuroinvasion.


Author(s):  
Ashwini Khadatkar ◽  
Nishant Ghodake

Rosai Dorfman disease is a rare histiocytic disorder of unknown etiology commonly involving the cervical lymph nodes first described in 1969, Extranodal involvement is very rare. Clinical course is variable.


Pharmaceutics ◽  
2021 ◽  
Vol 13 (9) ◽  
pp. 1363
Author(s):  
Tomoyuki Furubayashi ◽  
Daisuke Inoue ◽  
Shunsuke Kimura ◽  
Akiko Tanaka ◽  
Toshiyasu Sakane

A well-developed lymphatic network is located under the nasal mucosa, and a few drugs that permeate the nasal mucosa are absorbed into the lymphatic capillaries. Lymph from the nasal cavity flows to the cervical lymph nodes (CLNs). In this study, we evaluated the pharmacokinetics of the direct transport of intranasally administered drugs to CLNs through the nasal mucosa of Wistar rats using methotrexate as a model drug. The drug targeting index, which was calculated based on the areas under the concentration–time curves after intravenous and intranasal administration, was 3.78, indicating the benefits of nasal delivery of methotrexate to target CLNs. The direct transport percentage, which was indicative of the contribution of the direct nose–CLN pathway of methotrexate after intranasal administration, was 74.3%. The rate constant of methotrexate from the nasal cavity to CLNs was 0.0047 ± 0.0013 min−1, while that from systemic circulation to CLNs was 0.0021 ± 0.0009 min−1. Through pharmacokinetic analysis, this study demonstrated that the direct nasal–CLN pathway contributed more to the transport of methotrexate to the CLNs than the direct blood–CLN pathway.


2020 ◽  
Vol 28 (5) ◽  
pp. 541-548
Author(s):  
Sindhu Shetty ◽  
Nidhi Sharma ◽  
Christine N. Booth ◽  
Olaronke Oshilaja ◽  
Erinn P. Downs-Kelly ◽  
...  

Rosai-Dorfman disease is a rare proliferative histiocytic disorder of lymph nodes that is descriptively known as sinus histiocytosis with massive lymphadenopathy. Extranodal involvement of the parenchyma of the breast is uncommonly reported, with fewer than 50 cases of mammary extranodal disease detailed in the English-language literature. We characterize a retrospective series of adult female patients from a single institution with Rosai-Dorfman disease of the breast and axillary lymph nodes. Because Rosai-Dorfman disease of the breast and axillary lymph nodes may clinically, radiographically, and histologically mimic breast carcinoma and other conditions, we present an illustrated review of the disease and its relevant differential diagnoses in hopes of raising awareness and allowing for accurate management of affected patients.


2010 ◽  
Vol 89 (7) ◽  
pp. E28-E30 ◽  
Author(s):  
S.C. Gupta ◽  
Jain Sachin ◽  
Saxena Savyasachi ◽  
Jaiswal Ritesh ◽  
Garg Neha ◽  
...  

Benign schwannomas of the nasal cavity are rare. When they do occur, their clinical presentation is variable, and some are fairly vascular. We report the case of a 17-year-old boy who presented with epistaxis associated with a unilateral nasal mass that extended into the nasopharynx. The tumor was removed via a transpalatal approach. In most such cases, a clinical diagnosis of angiofibroma is made. In this case, however, histopathologic examination identified the lesion as a schwannoma.


1993 ◽  
Vol 102 (1) ◽  
pp. 11-15 ◽  
Author(s):  
Carlos E. García ◽  
Harsha V. Girdhar-Gopal ◽  
David M. Dorfman

Kikuchi-Fujimoto disease (KFD) is a rare entity of uncertain cause that commonly presents with persistently enlarged cervical lymph nodes unresponsive to antibiotic therapy. Although it usually follows a benign course, KFD has been repeatedly misdiagnosed as malignant lymphoma; hence, clinicians and pathologists alike need to be aware of this disease entity. The newest developments regarding the etiology and course of KFD are presented through a review of the literature and a recent illustrative case.


2006 ◽  
Vol 16 (Suppl 1) ◽  
pp. 312-315
Author(s):  
B. J.L. Leiva ◽  
R. M. Bofill ◽  
B. U. Sevin ◽  
X. J. Geiger

Rosai–Dorfman disease (RDD) is a rare histiocytic disorder of unknown cause. RDD most commonly involves the cervical lymph nodes, but extranodal involvement has been described. We report the case of a patient with extranodal RDD that presented as a retroperitoneal mass obstructing the left ureter. The patient underwent surgical resection of the mass, followed by a 5-month course of vinblastine. There was no evidence of progressive disease 22 months after surgery.


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