Primary Pancreatic Sinus Histiocytosis With Massive Lymphadenopathy (Rosai-Dorfman Disease): An Unusual Extranodal Manifestation Clinically Simulating Malignancy

2010 ◽  
Vol 134 (2) ◽  
pp. 276-278 ◽  
Author(s):  
Mark Podberezin ◽  
Ronald Angeles ◽  
Grace Guzman ◽  
David Peace ◽  
Sujata Gaitonde

Abstract Sinus histiocytosis with massive lymphadenopathy (SHML), also called Rosai-Dorfman disease, is a rare entity. Its etiology and pathogenesis are still essentially unclear. The histologic hallmark of this disease is proliferation of distinctive histiocytes within lymph node sinuses and in extranodal sites. Approximately 23% of patients with SHML, documented in the SHML Registry, presented with disease primarily in extranodal sites, and very few cases of SHML (<1%) involving the gastrointestinal system have been described in the literature. We report an unusual case of primary pancreatic SHML with infiltration of the process into peripancreatic, perinephric, and perisplenic adipose tissue, simulating malignancy.

2011 ◽  
Vol 135 (3) ◽  
pp. 390-393
Author(s):  
Changlee S Pang ◽  
David D Grier ◽  
Michael W Beaty

Abstract Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a rare self-limiting disorder of histiocytes with unknown etiology. Sinus histiocytosis with massive lymphadenopathy is most common in children and young adults and is characterized by painless lymphadenopathy. Histologically there is a proliferation of sinus histiocytes with lymphophagocytosis or emperipolesis. On rare occasions, SHML has been associated with lymphoma, usually involving different anatomic sites and developing at different times. We report a case of concomitant SHML and nodal marginal zone lymphoma involving the same lymph node without involvement of other nodal or extranodal sites. The presence of concomitant SHML within the lymph node involved by nodal marginal zone lymphoma may represent the responsiveness of SHML histiocytes to B-cell–derived cytokines in lymphoproliferative disorders. To our knowledge, this is the first description of concomitant occurrence of SHML and nodal marginal zone lymphoma.


1992 ◽  
Vol 106 (1) ◽  
pp. 61-62 ◽  
Author(s):  
R. G. Courteney-Harris ◽  
M. J. Goddard

AbstractA case of subglottic narrowing caused by an extranodal deposit of sinus histiocytosis in an eight-year-old child with Rosai-Dorfman disease is presented. The airway obstruction was initially managed with a tracheostomy, and then on confirmation of the diagnosis by lymph node biopsy, by excision of the extranodal deposit with a CO2 bronchoscopic laser.


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


2012 ◽  
Vol 43 (4) ◽  
pp. 626-629 ◽  
Author(s):  
Minerva A. Romero Arenas ◽  
Aatur D. Singhi ◽  
Ralph H. Hruban ◽  
Andrew M. Cameron

2001 ◽  
Vol 69 (1) ◽  
pp. 67-71 ◽  
Author(s):  
Alessandro Pulsoni ◽  
Gabriel Anghel ◽  
Paolo Falcucci ◽  
Roberta Matera ◽  
Edoardo Pescarmona ◽  
...  

Rare Tumors ◽  
2010 ◽  
Vol 2 (1) ◽  
pp. 5-7 ◽  
Author(s):  
Munenori Ide ◽  
Takayuki Asao ◽  
Takatomo Yoshida ◽  
Junko Hirato ◽  
Tatsuo Shimura ◽  
...  

Rosai-Dorfman disease (RDD) was formerly known as “sinus histiocytosis with massive lymphadenopathy”, and cases involving the gastrointestinal tract are rare. We present a case of pure extranodal RDD, resected as a polypoid lesion in colonoscopic study. The patient was a 62-year old woman with a history of sigmoidectomy for unexplained peritonitis. Microscopic study of the polypoid lesion showed the submucosal mass with histological and immunological features of RDD. The whole body computed tomography revealed neither lymphadenopathy nor tumor-like mass.


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