Article

JOURNAL MENU
Cover
Journal Cover Page

RGUHS Nat. J. Pub. Heal. Sci Vol: 14  Issue: 2 eISSN:  pISSN

Case Report

Epitheloid Haemangioendothelioma Liver Coexisting with Retroperitoneal Rosai Dorfman Disease– A Rare Case Report

 Harish S Permi1 , Patil C.N2 , Shetty J.P3 , Makannavar J.H4

1Assistant Professor, Department of Pathology,

2Assistant Professor, Department of Oncology 1,

3Professor and Head, 

4Senior Professor

Department of Pathology, 1-Department of Oncology , K S Hegde Medical Academy of Nitte University, Deralakatte Mangalore, Karnataka, India, 575018.

Year: 2011, Volume: 1, Issue: 4, Page no. 57-60,
Views: 625, Downloads: 4
Licensing Information:
CC BY NC 4.0 ICON
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0.
Abstract

Epitheloid hemangioendothelioma is a rare tumor of vascular origin, which occurs at sites such as soft tissues, liver, or lung, and has a highly unpredictable malignant potential. It is an intermediate entity between well-differentiated hemangioma and angiosarcoma. Neoplastic cells express factor VIII related antigen, CD31 and CD34. Hepatic epitheloid hemangioendothelioma is a rare vascular tumor. The clinical course is unpredictable and different treatment modalities are offered depending on the patient’s conditions. Rosai-Dorfman disease, originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with massive cervical lymphadenopathy. However other nodal sites (mediastinal and inguinal) are also frequently involved. The retro-peritoneum is an infrequent site of involvement.

We report a case of a 41 year old man presented with recurrent pain abdomen diagnosed as epitheloid hemangioendothelioma liver coexisting with retroperitoneal Rosai Dorfman disease. He was managed symptomatically without any modalities of treatment with regular follow up every month since one year. This case highlights the diagnostic dilemma of retroperitoneal Rosai Dorfman disease which mimicked metastasis from hepatic neoplasm and its rare association with epitheloid hemangioendothelioma liver

<p>Epitheloid hemangioendothelioma is a rare tumor of vascular origin, which occurs at sites such as soft tissues, liver, or lung, and has a highly unpredictable malignant potential. It is an intermediate entity between well-differentiated hemangioma and angiosarcoma. Neoplastic cells express factor VIII related antigen, CD31 and CD34. Hepatic epitheloid hemangioendothelioma is a rare vascular tumor. The clinical course is unpredictable and different treatment modalities are offered depending on the patient&rsquo;s conditions. Rosai-Dorfman disease, originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with massive cervical lymphadenopathy. However other nodal sites (mediastinal and inguinal) are also frequently involved. The retro-peritoneum is an infrequent site of involvement.</p> <p>We report a case of a 41 year old man presented with recurrent pain abdomen diagnosed as epitheloid hemangioendothelioma liver coexisting with retroperitoneal Rosai Dorfman disease. He was managed symptomatically without any modalities of treatment with regular follow up every month since one year. This case highlights the diagnostic dilemma of retroperitoneal Rosai Dorfman disease which mimicked metastasis from hepatic neoplasm and its rare association with epitheloid hemangioendothelioma liver</p>
Keywords
Retroperitoneal, Rosai Dorfman disease, hemangioendothelioma liver.
Downloads
  • 1
    FullTextPDF
Article

none

Supporting File
No Pictures
References

none

We use and utilize cookies and other similar technologies necessary to understand, optimize, and improve visitor's experience in our site. By continuing to use our site you agree to our Cookies, Privacy and Terms of Use Policies.