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

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.

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De novo appearance of intracranial Rosai-Dorfman disease during long-term follow-up: A case report

Interdisciplinary Neurosurgery ◽

10.1016/j.inat.2020.100891 ◽

2020 ◽

Vol 22 ◽

pp. 100891

Author(s):

Nobuhiro Nakagawa ◽

Norihito Fukawa ◽

Kiyoshi Tsuji ◽

Naoki Nakano ◽

Amami Kato

Keyword(s):

Case Report ◽

De Novo ◽

Rosai Dorfman Disease ◽

Long Term Follow Up

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

SAGE Open Medical Case Reports ◽

10.1177/2050313x18772195 ◽

2018 ◽

Vol 6 ◽

pp. 2050313X1877219 ◽

Cited By ~ 1

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.

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

Central European Journal of Immunology ◽

10.5114/ceji.2018.80055 ◽

2018 ◽

Vol 43 (3) ◽

pp. 341-345 ◽

Cited By ~ 2

Author(s):

Aleksandra Miękus ◽

Joanna Stefanowicz ◽

Grażyna Kobierska-Gulida ◽

Elżbieta Adamkiewicz-Drożyńska

Keyword(s):

Case Report ◽

Literature Review ◽

Cervical Lymphadenopathy ◽

Rosai Dorfman Disease

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A Rare Presentation of Synovial Sarcoma as Cervical Lymphadenopathy in a Pediatric Patient—a Case Report

Indian Journal of Surgical Oncology ◽

10.1007/s13193-017-0687-5 ◽

2017 ◽

Vol 9 (1) ◽

pp. 62-64 ◽

Cited By ~ 1

Author(s):

Ashutosh Gupta ◽

Gunjan Agrawal ◽

Vivek Chaudhary ◽

Heena Mazhar ◽

Santanu Tiwari

Keyword(s):

Case Report ◽

Synovial Sarcoma ◽

Pediatric Patient ◽

Cervical Lymphadenopathy ◽

Rare Presentation

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Pregnant patient with extranodal CNS Rosai-Dorfman disease with new-onset seizures: the conundrum of differentiating disease progression from eclampsia

BMJ Case Reports ◽

10.1136/bcr-2020-239427 ◽

2021 ◽

Vol 14 (2) ◽

pp. e239427

Author(s):

Katie Swor ◽

Deepti Zutshi ◽

Elizabeth Dubey ◽

Bernard Gonik

Keyword(s):

Cervical Lymphadenopathy ◽

Pregnant Patient ◽

Late Gestation ◽

Critically Ill Patient ◽

Hypertensive Disorder ◽

Rosai Dorfman Disease ◽

Extranodal Disease ◽

Cns Lesions ◽

New Onset

A 31-year-old woman at 30 weeks gestation presented to the emergency department following multiple seizures. Her history was significant for extranodal Rosai-Dorfman Disease (RDD) with central nervous system (CNS) lesions. RDD, a rare form of non-Langerhans cell histiocytosis, commonly presents with non-tender cervical lymphadenopathy. CNS involvement accounts for a small number of cases in those with extranodal disease. Patients with CNS RDD can have a variety of neurological symptoms, including seizures. Eclampsia, a relatively rare obstetric hypertensive disorder, is always within the differential diagnosis for patients presenting with late gestation seizures. We present the challenging evaluation and treatment of a patient whose clinical picture did not clearly differentiate eclampsia from new onset seizures related to progression of her RDD. This conundrum perhaps resulted in unnecessary preterm operative delivery of a critically ill patient. Only follow-up of the patient helped clarify the likely antepartum diagnosis.

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A Solitary Rosai-Dorfman Disease of The Talus: The First Case Report of Bisphosphonate Therapy with Imaging Follow-up

Journal of the Medical Association of Thailand ◽

10.35755/jmedassocthai.2021.12.13087 ◽

2021 ◽

Vol 104 (12) ◽

pp. 1988-1991

Keyword(s):

Case Report ◽

Clinical Symptoms ◽

Bisphosphonate Therapy ◽

Histological Feature ◽

Ankle Pain ◽

First Report ◽

Osseous Lesion ◽

First Case ◽

Rosai Dorfman Disease

Rosai-Dorfman disease (RDD) manifesting as a solitary osseous lesion especially of talus bone is rare. The authors reported a 31-year-old Thai man who had chronic left ankle pain and the biopsy of his talar lesion demonstrated emperipolesis, the typical histological feature of RDD. He was treated with curettage and adjuvant bisphosphonate and appeared to show improvement in clinical symptoms and radiological evidence. To the authors’ knowledge, this is the first report of an intraosseous RDD lesion treated with bisphosphonate with imaging follow-up. Keywords: Rosai-Dorfman disease; Talus; Solitary; Radiology; Bisphosphonate

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Initial Treatment of CD20+ Lymphocyte Predominant Hodgkin Lymphoma with Chemotherapy and the Monoclonal Antibody Rituximab-Mabthera Is Effective and Well Tolerated - Case Report.

Blood ◽

10.1182/blood.v108.11.4696.4696 ◽

2006 ◽

Vol 108 (11) ◽

pp. 4696-4696

Author(s):

Sonja G. Genadieva-Stavrik ◽

Lubomir Ivkovski ◽

Aleksandra Pivkova ◽

Lidija Cevreska

Keyword(s):

Case Report ◽

Complete Remission ◽

Patient Population ◽

Mediastinal Mass ◽

Early Stage ◽

Cervical Lymphadenopathy ◽

Malignant Cells ◽

Limited Information ◽

Patients Experience

Abstract Patients with lymphocyte-predominant Hodgkin disease (LPHD) often present at an early stage with slow disease progression. Although 96% of LPHD patients experience a complete remission (CR) upon first line treatment, many relapses occur. CD20+ is strongly expressed by malignant cells in LPHD, but so far there is limited information regarding the efficacy of rituximab in this patient population. We report 46-year-old woman who presented in 2004 with left cervical lymphadenopathy and weight loss. Case report: Biopsy confirmed a diagnosis of LPHD(stage IIB disease), immunohistochemistry revealed that >30% of malignant cells were CD20+, and a bulky mediastinal mass was observed by computer tomography (CT). Blood counts, erythrocyte sedimentation rate, albumin and lactate dehydrogenate were normal. The patient was treated with rituximab and ABVD (doxorubicin 25mg/m2, bleomycin 10mg/m2, vinbalstine 6mg/m2, dacarbazine 375mg/m2, six cycles). Rituximab was administered (375mg/m2) one day before chemotherapy (50mg/h for one hour and gradually escalated to a maximum of 400mg/h). Treatment was well tolerated and there were no complications. The patient was assessed immediately following treatment and every three months for the following year, by physical examination, CT, bone marrow biopsy and routine analyses. A detectable tumor mass was no observed by CT for the follow up of 2 years, the patient remains in complete remission. Conclusion: Our case report suggests that Rituximab is both safe and effective in patients with CD20+ LPHD. Further studies with Rituximab are warranted in this patient population.

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

Anais Brasileiros de Dermatologia ◽

10.1590/s0365-05962013000200014 ◽

2013 ◽

Vol 88 (2) ◽

pp. 256-259 ◽

Cited By ~ 2

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.

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