scholarly journals Rosai-Dorfman Disease: A Case Report of Asymptomatic Isolated Renal Involvement

2021 ◽  
Author(s):  
Alireza Abrishami ◽  
Pardis Ziaeefar ◽  
Sara Ebrahimi ◽  
Nastaran Khalili ◽  
Akbar Nouralizadeh ◽  
...  
Keyword(s):  
Case Report ◽  
Mortality Rate ◽  
Renal Involvement ◽  
Cervical Lymphadenopathy ◽  
Good Prognosis ◽  
Malignant Histiocytosis ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare non-malignant histiocytosis disorder, commonly manifesting with massive painless cervical lymphadenopathy. Renal involvement develops in only four percent of patients with RDD. Generally, RDD is self-limiting and has a good prognosis; however, in patients with renal involvement, mortality rate can be as high as 40%.

scholarly journals A unique combination of Rosai-Dorfman disease and mycosis fungoides: a case report

2018 ◽  
Vol 6 ◽  
pp. 2050313X1877219 ◽  
Author(s):  
Amanda J Shelley ◽  
Nordau Kanigsberg
Keyword(s):  
Case Report ◽  
T Cell ◽  
Mycosis Fungoides ◽  
Cell Lymphoma ◽  
Cervical Lymphadenopathy ◽  
B Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Unknown Etiology ◽  
Unique Combination ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare histiocytic condition of unknown etiology. Patients with RDD classically present with massive painless cervical lymphadenopathy. However, extra-nodal disease occurs in approximately 40% of cases, with the skin being among the most commonly involved sites. Patients with isolated extra-nodal involvement may present without adenopathy. Reports of RDD occurring in patients with Hodgkin’s lymphoma, and B-cell lymphoma have been published, but there has only been one previous report of RDD in a patient with a T-cell lymphoma. This case report documents a unique combination of RDD and mycosis fungoides (MF), a cutaneous T cell lymphoma. This report also highlights diagnostic challenges in RDD due to the rarity of the condition and its variable presentation.

scholarly journals Rosai-Dorfman disease as a rare cause of cervical lymphadenopathy – case report and literature review

2018 ◽  
Vol 43 (3) ◽  
pp. 341-345 ◽  
Author(s):  
Aleksandra Miękus ◽  
Joanna Stefanowicz ◽  
Grażyna Kobierska-Gulida ◽  
Elżbieta Adamkiewicz-Drożyńska
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Cervical Lymphadenopathy ◽  
Rosai Dorfman Disease

scholarly journals Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature

2020 ◽  
Vol 7 ◽  
Author(s):  
Yi Zhang ◽  
Jie Liu ◽  
Jianyu Zhu ◽  
Xiang Zhou ◽  
Kun Zhang ◽  
...  
Keyword(s):  
Systematic Review ◽  
Case Report ◽  
Pediatric Patient ◽  
Cervical Lymphadenopathy ◽  
Sellar Region ◽  
Rosai Dorfman Disease ◽  
The Individual ◽  
Mass Lesions ◽  
Endocrine Abnormality

Rosai-Dorfman disease (RDD) is an extremely rare histiocytic disorder characterized by cervical lymphadenopathy, while the involvement of sellar region is less observed. Here we report a pediatric patient who was initially suspected as sellar germinoma but later identified as RDD. We also conducted a systematic review about RDD involving sellar region. A total of only 14 cases were included and analyzed in our study in terms of clinical presentation, endocrine abnormality, radiological features, pathology, treatment, and follow up. The most common neurological manifestations of sellar RDD is diabetes insipidus and visual changes. Two typical kinds of MRI manifestations were presented in sellar RDD; one is like meningioma-like mass lesions, another showing infiltrative pattern that demonstrates hyperintense areas on T2WI. Currently, the treatment of RDD is tailored to the individual clinical circumstances. For sellar RDD, surgical treatment can be considered to completely remove or debulk the tumor.

scholarly journals Rosai-Dorfman disease presenting with extensive cutaneous manifestation - Case report

2013 ◽  
Vol 88 (2) ◽  
pp. 256-259 ◽  
Author(s):  
Paula Azevedo Borges Leal ◽  
Adrilena Lopes Adriano ◽  
Marcelle Parente Breckenfeld ◽  
Igor Santos Costa ◽  
Antônio Renê Diógenes de Sousa ◽  
...  
Keyword(s):  
Case Report ◽  
Lymph Nodes ◽  
Cervical Lymphadenopathy ◽  
Cutaneous Manifestation ◽  
Common Site ◽  
Atypical Case ◽  
Inguinal Lymph Nodes ◽  
Rosai Dorfman Disease ◽  
Polyclonal Gammopathy ◽  
Extranodal Manifestations

Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been reported in 43% of cases. In this study, we report an atypical case of Rosai-Dorfman disease in a female with massive cutaneous manifestation on the thigh, associated with a minimal lymphadenopathy limited to the regional inguinal lymph nodes.

scholarly journals Rosai-Dorfman disease of the subdural spine with a long segment lesion: A case report and literature review

2017 ◽  
Vol 45 (2) ◽  
pp. 875-881 ◽  
Author(s):  
Ji Tu ◽  
Wen-Tian Li ◽  
Cao Yang
Keyword(s):  
Cervical Lymphadenopathy ◽  
Good Prognosis ◽  
Massive Lymphadenopathy ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Puncture Biopsy ◽  
Appropriate Therapy ◽  
Systemic Manifestations ◽  
Cluster Of Differentiation

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare benign disorder usually characterized by massive painless cervical lymphadenopathy and systemic manifestations. Extranodal involvement, especially spinal involvement, is extremely rare. We report a 41-year-old man who presented with only intermittent dorsodynia. His condition was diagnosed as non-specific inflammatory disease on the basis of preoperative puncture biopsy results. We performed total surgical resection. Histopathological findings showed distinctive emperipolesis and immunohistochemistry results were positive for cluster of differentiation CD68 and S100 and negative for CD1a. A good prognosis was confirmed at the 3-month follow-up visit. This is the first case of RDD of the subdural spine with such a long segment lesion. There is still no consensus regarding appropriate therapy for this type of RDD and the preoperative diagnosis remains challenging. The unusual presentation of our case serves as a reference when diagnosing and treating RDD.

scholarly journals Rosai-dorfman disease of the fibula: A case report

2019 ◽  
Vol 26 (2) ◽  
pp. 108-113
Author(s):  
Glen Purnomo ◽  
Jesus Adrian A Dueñas ◽  
Richard S Rotor ◽  
Rafael S Claudio
Keyword(s):  
Case Report ◽  
Lymph Nodes ◽  
Immunohistochemical Staining ◽  
Cervical Lymphadenopathy ◽  
Skeletal System ◽  
Rosai Dorfman Disease

Rosai–Dorfman disease (RDD) is usually characterized by painless bilateral cervical lymphadenopathy associated with fever and leukocytosis. Although the disease may occur outside lymphnodes, manifestation of skeletal system occurs in less than 8% of cases. In addition, presentation of this disease in a purely skeletal form without lymph nodes involvement is extremely uncommon. This case report describes a 17-year-old female with a pure skeletal presentation of RDD in the fibula. Trocar biopsy was performed, and immunohistochemical staining using S100 and CD68 was done to confirm the diagnosis.

scholarly journals Isolated Intracranial Rosai-Dorfman Disease

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Md. Taufiq ◽  
Abul Khair ◽  
Ferdousy Begum ◽  
Shabnam Akhter ◽  
Md. Shamim Farooq ◽  
...  
Keyword(s):  
Case Report ◽  
Ct Scan ◽  
Clinical Improvement ◽  
Cervical Lymphadenopathy ◽  
Rare Condition ◽  
Unknown Etiology ◽  
Nodal Involvement ◽  
Rosai Dorfman Disease ◽  
Parasellar Region ◽  
Significant Clinical Improvement

Background. Rosai-Dorfman disease (RDD) is a benign histiocytic proliferative disorder of unknown etiology. This rare condition commonly causes massive cervical lymphadenopathy. Intracranial RDD without any nodal involvement is extremely rare.Case Report. A young Bangladeshi male complained of bilateral complete blindness with left sided deafness for about three years. There was no lymphadenopathy. MRI and CT scan of brain suggested an inflammatory/neoplastic (?meningioma) lesion located at left parasellar region which extended frontally to encircle both optic nerves and also to left prepontine area. Histopathologically the lesion was diagnosed as RDD. The patient was treated with steroid and significant clinical improvement observed.Conclusion. The prognosis of intracranial RDD is not poor. It can be treated with surgery with or without corticosteroids, chemotherapy, and so forth. But as the condition is extremely rare and often misdiagnosed, the clinician, radiologist, and histopathologist should have a suspicion in their mind about the possibility of RDD.

scholarly journals Tumor de Frantz: desafios diagnósticos e terapêuticos: relato de caso / Frantz’s tumor: diagnostic and therapeutic challenges: case report

2019 ◽  
Vol 64 (1) ◽  
pp. 65
Author(s):  
Fernando Cascelli Oliva ◽  
Gabriel José Dos Santos ◽  
Gustavo Hideki Hideki Orikasa ◽  
Milena Martello Cristófalo ◽  
Rafael Reis dos Santos ◽  
...  
Keyword(s):  
Case Report ◽  
Mortality Rate ◽  
Pancreatic Fistula ◽  
Young Women ◽  
Pancreatic Neoplasms ◽  
Pancreatic Pseudocyst ◽  
Good Prognosis ◽  
Solid Pseudopapillary Tumor ◽  
Carcinoma Papilar ◽  
Rate Case

Introdução: O tumor sólido pseudopapilar do pâncreas ou Tumor de Frantz, é uma neoplasia rara, que acomete preferencialmente mulheres jovens e apresenta bom prognóstico, com baixas taxas de mortalidade. Relato de Caso: O presente trabalho apresenta um caso de uma paciente da Santa Casa de São Paulo, com Tumor de Frantz, seu diagnóstico, tratamento e complicações pós pancreatectomia.Descritores: Pâncreas, Neoplasias pancreáticas, Carcinoma papilar, Pseudocisto pancreático, Fístula pancreáticaAbstractIntroduction: The solid pseudopapillary tumor, or Frantz’s tumor, is a rare neoplasm that occurs mainly in young women and have a good prognosis, with a low mortality rate. Case report: This study aims to describe the diagnosis, treatment and post pancreatectomy complications of a Frantz’s tumor case in Santa Casa of São Paulo.Keywords: Pancreas, Pancreatic neoplasms; Carcinoma, pappilary; Pancreatic pseudocyst; Pancreatic fistula  

scholarly journals Rosai-Dorfman Disease Presenting as an Isolated Extranodal Mass of the Carotid Sheath: A Case Report

2007 ◽  
Vol 86 (10) ◽  
pp. 624-627 ◽  
Author(s):  
Lisa Lee ◽  
Christine M. Glastonbury ◽  
Doris Lin
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Head And Neck ◽  
Cervical Lymphadenopathy ◽  
Granulomatous Disease ◽  
Carotid Sheath ◽  
Common Diseases ◽  
Rosai Dorfman Disease ◽  
Histopathologic Diagnosis ◽  
Extranodal Manifestations

Rosai-Dorfman disease is a rare, benign granulomatous disease that typically presents with massive cervical lymphadenopathy. In less than 50% of cases, other soft-tissue manifestations may also be found in the head and neck. Rosai-Dorfman disease can be difficult to diagnose because of its rarity and its ability to mimic, both clinically and radiologically, more common diseases such as lymphoma. The histopathologic diagnosis can also be difficult to make, particularly when the disease exhibits extranodal manifestations. We present a case of isolated extranodal Rosai-Dorfman disease involving the carotid sheath, without the typical massive adenopathy.

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