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 Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy): A Retrospective Study of 5 Cases

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Yanyan Niu ◽  
Yongjin Li ◽  
Jian Wang ◽  
Xiaofeng Jin ◽  
Dahai Yang ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Age Of Onset ◽  
Clinical Manifestations ◽  
Hormone Treatment ◽  
Good Prognosis ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Alive With Disease ◽  
Laryngeal Involvement

This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD) with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymmetric onset that was manifested by a laryngeal submucosal nodular mass. The patients were subjected to a regimen of hormone treatment combined with surgical resection. The median follow-up duration was 101 months (8–384 months). One case was lost, and the remaining 4 subjects are alive with disease. The follow-up examinations revealed that 4 subjects had stable laryngeal conditions, whereas one showed minor progression. RDD with laryngeal involvement is clinically rare and differs considerably from classical RDD in age of onset, gender composition, and extranodal involvement. The regimen of hormone treatment combined with surgical resection can stabilize the patient’s general condition and laryngeal lesion. Tracheotomies are recommended for patients with dyspnea. After their conditions stabilize, decannulation can be successfully performed in most cases. This therapeutic regimen generally delivers a good prognosis.

Rosai-Dorfman disease presenting as a parotid mass

1997 ◽  
Vol 111 (11) ◽  
pp. 1091-1093 ◽  
Author(s):  
L. Norman ◽  
A. C. Bateman ◽  
G. W. R. Watters ◽  
V. Singh ◽  
A. V. Spedding
Keyword(s):  
Cervical Lymphadenopathy ◽  
Benign Disease ◽  
Definitive Diagnosis ◽  
Unknown Aetiology ◽  
Fine Needle Aspirate ◽  
Cytological Examination ◽  
Fine Needle ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Gland Tumour

AbstractSinus histiocytosis with massive lymphadenopathy (SHML) is a rare, benign disease of unknown aetiology. This disease typically presents with massive, painless cervical lymphadenopathy but may occur in a wide variety of extranodal sites. Our report describes a 71-year-old man who presented with a discrete, unilateral parotid mass which was clinically suggestive of a primary salivary gland tumour. Initial cytological examination of a fine needle aspirate specimen taken from the mass demonstrated a discohesive cell population with nuclear atypia, raising the possibility of malignancy. However, excision of the mass and histological examination enabled a definitive diagnosis to be made.

scholarly journals Primary Rosai-Dorfman Disease of Bone: A Report of Two Cases

2019 ◽  
Vol 2019 ◽  
pp. 1-6
Author(s):  
Andrew B. Ross ◽  
Kirkland W. Davis ◽  
Darya Buehler ◽  
Brian Y. Chan
Keyword(s):  
Young Adults ◽  
Cervical Lymphadenopathy ◽  
Sinus Histiocytosis ◽  
Osseous Lesion ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Extranodal Disease

Rosai-Dorfman disease (RDD), sometimes known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder that most commonly presents as painless, massive cervical lymphadenopathy in young adults. Extranodal disease can occur in up to 40% of patients but primary involvement of bone is rare. We present two cases of primary RDD of bone: one case of multifocal osseous RDD presenting as a painful lesion in the elbow, and one case of a solitary osseous lesion presenting as a painful lesion in the wrist.

Diffuse Large Cell Lymphoma Transformed from Rosai-Dorfman Disease

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4968-4968
Author(s):  
David Jendiroba ◽  
Marcus Magnus Sampaio ◽  
Márcia Vilela Gonçalves ◽  
Juliana Alves Rodrigues ◽  
Daniella Barqueti Jendiroba ◽  
...  
Keyword(s):  
Lymph Node ◽  
Cell Lymphoma ◽  
Past History ◽  
Cervical Lymphadenopathy ◽  
Large Cell ◽  
Molecular Evidence ◽  
Diffuse Large Cell Lymphoma ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Large Cell Lymphoma

Abstract INTRODUCTION: Rosai-Dorfman disease, also known as Sinus Histiocytosis with Massive Lymphadenopathy, is an almost rare benign entity which mimics non-Hodgkin’s lymphoma, and is characterized by enlargement of lymph nodes. Knowing the difference between these two diseases is critical for the adequate therapeutic approach. The most common presentation of the disease is within the cervical basin, and most of the times, lymph node dissection and removal of their chains is enough for cure. PATIENT AND METHODS: We describe a 23-yo white woman with past history of persistent cervical lymphadenopathy, who underwent 4 surgical procedures to remove left cervical nodes, diagnosed as Rosai-Dorfman disease within the last 3 years. When relapse after the 4th surgery happened, the patient came to our Service and sought for a 2nd opinion consultation. RESULTS: Pathology reviews were done on the previously collected material, corroborating Rosai-Dorfman disease, except for the review of the slides of the last material, which showed inconclusiveness. Histopathology and immunohistochemistry were then repeated for the last surgical specimens at 2 different Pathology labs, and both confirmed diffuse large cell lymphoma. Patient started on chemo with CHOP 6×, and showed a partial response up to the 3rd cycle, when the tumor started progressing during the 4th cycle. Chemo was then changed to ESHAP, which was well-tolerated and promoted CR after 6 cycles. The patient is currently in CR and asymptomatic. CONCLUSION: Rosai-Dorfman disease is a proliferative reaction to an unknown, unidentified pathogen or specific cause, which leads to lymph node enlargement and extra nodal involvement in 25% of the cases. Some studies have attempted to correlate the presence of klebsiella as a proliferative stimulus present in this disease, but there is not enough evidence to confirm this hypothesis. Interestingly, it may seem that Rosai-Dorfman is a middle step between a truly benign process and malignancy itself because, not only other cases of lymphoma transformed after Rosai-Dorfman’s disease have been reported, but also extra nodal cancer following extra nodal presentation of the disease. Although many clues point to its involvement in the process of carcinogenesis, more clinical and molecular evidence are necessary to tie the link between Rosai-Dorfman disease and cancer.

Extranodal Rosai-Dorfman Disease with Multifocal Bone and Epidural Involvement Causing Recurrent Spinal Cord Compression

2005 ◽  
Vol 8 (5) ◽  
pp. 593-598 ◽  
Author(s):  
Khulood Al-Saad ◽  
Paul Thorner ◽  
Bo-Yee Ngan ◽  
J. Ted Gerstle ◽  
Abhaya V. Kulkarni ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Lymph Nodes ◽  
S100 Protein ◽  
Cervical Lymphadenopathy ◽  
Cord Compression ◽  
Diagnostic Dilemma ◽  
Clinical Behavior ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Consultation Services

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.

Intracranial Rosai—Dorfman disease treated with microsurgical resection and stereotactic radiosurgery

2003 ◽  
Vol 98 (1) ◽  
pp. 165-168 ◽  
Author(s):  
Constantinos G. Hadjipanayis ◽  
Ghassan Bejjani ◽  
Clayton Wiley ◽  
Toshinori Hasegawa ◽  
Melissa Maddock ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Cervical Lymphadenopathy ◽  
Residual Tumor ◽  
Content Type ◽  
First Case ◽  
Excess Morbidity ◽  
Rosai Dorfman Disease ◽  
Microsurgical Resection ◽  
Partial Labyrinthectomy ◽  
Fine Print

✓ Sinus histiocytosis or Rosai—Dorfman disease (RDD) is a rare idiopathic histioproliferative disorder typically characterized by painless cervical lymphadenopathy, fever, and weight loss. Extranodal, intracranial disease is uncommon. In this report the authors describe the first case of intracranial RDD treated with stereotactic radiosurgery after resection. This 52-year-old man with known RDD presented with a 7-day course of fever, headache, diplopia, left facial paresthesias, and difficulty swallowing. No cranial nerve deficits were evident on examination, but right submandibular and inguinal node enlargements were noted. On neuroimaging, the patient was found to have a homogeneously contrast-enhancing petroclival lesion with extension into the left cavernous sinus. The patient underwent a combined left petrosal craniotomy and partial labyrinthectomy with duraplasty for biopsy sampling and partial microsurgical resection of the lesion. Microscopic examination of the biopsy specimen revealed the presence of a mixed cellular population with predominant mature histiocytes consistent with RDD. The residual tumor was treated with stereotactic radiosurgery 2 months after resection. On follow-up imaging the lesion had regressed significantly, with only slight dural enhancement remaining. Microsurgical resection for histological diagnosis, followed by stereotactic radiosurgery for residual tumor represents one treatment alternative in the management of intracranial RDD in which a complete resection carries the potential for excess morbidity.

scholarly journals Case Report: Pericardial Effusion Treated With Pericardiectomy Plus Right Atrial Mass Resection: A 2-Year Follow-Up of Cardiac Rosai-Dorfman Disease

2021 ◽  
Vol 8 ◽  
Author(s):  
Edoardo Conte ◽  
Antonio Brucato ◽  
Francesco Petrella ◽  
Emanuela Passoni ◽  
Gianfranco Lauri ◽  
...  
Keyword(s):  
Pericardial Effusion ◽  
Immunohistochemical Analysis ◽  
Clinical History ◽  
Right Atrial ◽  
Atrial Mass ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Right Atrial Mass ◽  
Maximal Medical Therapy

Background: Rosai-Dorfman disease (RDD) is rare a sinus histiocytosis typically causing lymphadenopathy. Heart involvement is anecdotal, and <30 cases of cardiac RDD (cRDD) have been reported so far.Case Presentation: A 46-year old woman with positive clinical history for RDD was admitted to our cardiology department with transthoracic echocardiography diagnosis of severe pericardial effusion and right atrial masses. Pericardiocentesis with catheter insertion was performed 3 days after the admission due to clinical evidence of cardiac tamponade. After 10 weeks of maximal medical therapy for inflammatory pericarditis, including non-steroidal anti-inflammatory drugs (NSAIDs), colchicine, steroids, and anakinra, at least 100 ml of pericardial citric liquid has been daily drained suggesting no clinical improvement. Pericardial liquid analysis demonstrated no malignant cells, but immunohistochemical analysis resulted positive for AE1–AE3, D2–40, S100, and CD68 consistent with an RDD diagnosis. Surgical management was judged clinically indicated, and 2 months after admission, the patient underwent pericardiectomy and debulking of atrial mass with freezing of remaining atrial neoformation. Regular clinical and echocardiography evaluation was performed without pericardial effusion recurrence after 2 years of follow-up.Conclusions: This is the first case ever reported of cRDD who survived after 2 years of follow-up. Pericardiectomy could be feasible and effective for recurrent pericardial effusion in cRDD. Close follow-up and a multidisciplinary environment is needed to take care of cRDD patients.

scholarly journals Prenatal Diagnosis of Intracranial Tumors and the Difficulties in Prognostication: A Report of Three Cases

2015 ◽  
Vol 6 (2) ◽  
pp. 88-91
Author(s):  
Lavanya Rai ◽  
Akhila Vasudeva ◽  
Sapna V Amin ◽  
Rajagopal Kadavigere ◽  
Katta M Girisha
Keyword(s):  
Prenatal Diagnosis ◽  
Choroid Plexus ◽  
Choroid Plexus Papilloma ◽  
Good Prognosis ◽  
Neurological Involvement ◽  
Intracranial Tumors ◽  
Prenatal Mri ◽  
First Case ◽  
Rule Out

ABSTRACT Prenatal diagnosis of intracranial tumors generally implies a poor prognosis. We present three such cases, where prognostication was difficult. We attempted to correlate our prenatal counseling with postnatal follow-up/postabortal diagnosis. In the first case, tumor was diagnosed at 37 weeks. Ultrasound and fetal/neonatal MRI suggested a malignant intraventricular tumor. Anticipating guarded prognosis, parents refused neurosurgical intervention. At 1 year, child has normal neurodevelopment. Further magnetic resonance imagings (MRIs) show tumor shrinkage, pointing to a benign tumor. In case two, a choroid plexus tumor was diagnosed at midtrimester anomaly scan. Since it was difficult to rule out a malignant tumor, pregnancy was terminated. However, MRI, autopsy, and histopathology confirmed a choroid plexus papilloma, which is known to have good prognosis. In case three, prenatal MRI showed features of neurological involvement in tuberous sclerosis. However child has no neurological manifestations at 1 year of age. Hence, prognostication of prenatally diagnosed brain tumors remains a challenge. How to cite this article Vasudeva A, Amin SV, Kadavigere R, Girisha KM, Rai L. Prenatal Diagnosis of Intracranial Tumors and the Difficulties in Prognostication: A Report of Three Cases. Int J Infertil Fetal Med 2015;6(2):88-91.

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.

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

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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