scholarly journals Mercaptopurine Treatment in an Adult Man with Orbital and Intracranial Rosai-Dorfman Disease

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Valentina Arnao ◽  
Marianna Riolo ◽  
Giovanni Savettieri ◽  
Paolo Aridon
Keyword(s):  
Cervical Lymphadenopathy ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Polyclonal Hypergammaglobulinemia ◽  
Surgical Debulking ◽  
The Central Nervous System ◽  
Children And Young Adults ◽  
Patient Refusal ◽  
Orbital Region ◽  
Cns Lesions

Background. Rosai-Dorfmann disease (RDD) is a rare, idiopathic non-Langerhans cell histiocytosis, affecting children and young adults, that commonly presents as painless, massive cervical lymphadenopathy with fever, weight loss, and polyclonal hypergammaglobulinemia. Cervical lymphadenopathy and extranodal involvement are the main presentations. On the contrary, ophthalmic involvement and localisation in the central nervous system are rare.Case Report. An old man was admitted to our hospital for first seizure. Brain imaging studies revealed on the left an extra-axial thickening of the dura mater with enhancement and perilesional oedema, infiltrating the sphenoorbital fissure and an isointense mass with enhancement in the orbital region with dislocation of the optic nerve. Pathological and immunohistochemistry examination of the bioptical specimen was consistent with a diagnosis of RDD. Treatment with levetiracetam and steroids was started obtaining only remission of seizures. Because of the patient refusal of the surgical debulking, therapy with mercaptopurine was started, stopping disease progression.Conclusion. So far, very few cases of extranodal RDD with multiple CNS lesions involving the orbital region have been described. Our case is significant because it is the first case in which the efficacy of mercaptopurine treatment has been documented in an adult patient with isolated ocular and intracranial RDD.

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.

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.

Regression of intracranial Rosai–Dorfman disease following corticosteroid therapy

2006 ◽  
Vol 104 (5) ◽  
pp. 840-844 ◽  
Author(s):  
Christopher M. McPherson ◽  
Justin Brown ◽  
Angela W. Kim ◽  
Franco Demonte
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Corticosteroid Therapy ◽  
Histopathological Analysis ◽  
Extranodal Involvement ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Effective Option ◽  
The Central Nervous System ◽  
Skull Base Lesions

✓ Rosai–Dorfman disease (RDD) is an idiopathic histioproliferative disorder usually presenting with massive, painless lymphadenopathy. Extranodal involvement has been reported including at least 50 cases affecting the central nervous system (CNS). The treatment of CNS RDD as reported in the literature has primarily involved a surgical technique. The authors report on the case of a 53-year-old man presenting with multiple skull base lesions mimicking meningiomas. The patient suffered visual deterioration and underwent a right orbitopterional craniotomy as well as optic nerve decompression. Histopathological analysis revealed histiocytic cells and emperipolesis consistent with RDD. Following surgery, corticosteroid agents were administered, leading to marked resolution of both the remaining surgically untreated lesions and the balance of the patient's symptoms. This report represents the first case of the resolution of intracranial RDD following corticosteroid therapy. Corticosteroid agents should be considered an effective option in the treatment of CNS RDD.

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: A Rare Cause of Cervical Lymphadenopathy

2014 ◽  
Vol 5 (3) ◽  
pp. 152-154
Author(s):  
Adharsh Anand ◽  
Kanchan Lanjewar ◽  
Vipin Ram Ekhar ◽  
Ritesh N Shelkar ◽  
Sachin Rane ◽  
...  
Keyword(s):  
Pathological Condition ◽  
Cervical Lymphadenopathy ◽  
Needle Aspiration ◽  
Benign Condition ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Life Threatening ◽  
Children And Young Adults ◽  
Needle Aspiration Cytology ◽  
Extranodal Manifestations

ABSTRACT Rosai-Dorfman disease is also known as sinus histiocytosis with massive lymphadenopathy (SHML) is a rare clinico-pathological condition. It is a benign condition which causes significant cervical lymphadenopathy in children and young adults. The clinical presentation varies from isolated nodal involvement to significant extranodal manifestations. The clinical features are usually mild, but rarely life-threatening complications can occur in some individuals depending on the site of involvement. Here, we present two cases of Rosai-Dorfman disease, both diagnosed on fine needle aspiration cytology (FNAC) and histopathology and responded well to steroids. One of the patients had extranodal site involvement in the form of bilateral nasal mass which is very rare. How to cite this article Ekhar VR, Shelkar RN, Rane S, Anand A, Lanjewar K, Jain SKT. Rosai-Dorfman Disease: A Rare Cause of Cervical Lymphadenopathy. Int J Head Neck Surg 2014;5(3): 152-154.

scholarly journals Rosai-Dorfman disease mimicking images of meningiomas: Two case reports and literature review

2021 ◽  
Vol 12 ◽  
pp. 292
Author(s):  
Rafael Trindade Tatit ◽  
Paulo Eduardo Albuquerque Zito Raffa ◽  
Giovana Cassia de Almeida Motta ◽  
André Alexandre Bocchi ◽  
Júlia Loripe Guimaraes ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Case Reports ◽  
Central Nervous System Involvement ◽  
Cervical Lymphadenopathy ◽  
Surgical Excision ◽  
Definitive Diagnosis ◽  
Rosai Dorfman Disease ◽  
Intracranial Involvement ◽  
Radiological Images ◽  
The Central Nervous System

Background: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytic proliferative disorder classically as a massive cervical lymphadenopathy. However, over the years, extranodal locations were confirmed with the central nervous system involvement in less than 5% of cases, which is marked as a significant differential diagnosis of meningiomas, with which they are widely confused due to the similarity of their radiological images. Case Description: We report a 37-year-old man and 45-year-old man who were diagnosed with intracranial RDD but whose radiological images mimic meningiomas, requiring anatomopathological and tumor’s immunohistochemistry for definitive diagnosis. Moreover, a review of 184 publications with 285 cases of intracranial involvement of this disease was also performed, comparing these findings with those brought in the previous studies. Conclusion: Intracranial Rosai-Dorfman tumors should always be remembered as differential diagnosis of meningiomas since they are similar radiologically and macroscopically. Once remembered and diagnosed, the lesion must be treated following the same pattern of resection done in meningiomas and, treatment’s differences will not occur in the surgical excision technique, but in complementary chemotherapy implementation, radiotherapy, and even with radiosurgery aid, depending on the case. Thus, it is possible to obtain better results than with just the isolated surgical procedure.

Pediatric Fronto-Orbital Skull Reconstruction

2021 ◽  
Author(s):  
Andrew M. Ferry ◽  
Rami P. Dibbs ◽  
Shayan M. Sarrami ◽  
Amjed Abu-Ghname ◽  
Han Zhuang Beh ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Facial Trauma ◽  
Limited Data ◽  
Significant Morbidity ◽  
Craniofacial Skeleton ◽  
Optimal Care ◽  
Orbital Reconstruction ◽  
Safety And Efficacy ◽  
The Central Nervous System ◽  
Orbital Region

AbstractCraniofacial surgery in children is a highly challenging discipline that requires extensive knowledge of craniofacial anatomy and pathology. Insults to the fronto-orbital skeleton have the potential to inflict significant morbidity and even mortality in patients due to its proximity to the central nervous system. In addition, significant aesthetic and ophthalmologic disturbances frequently accompany these insults. Craniosynostosis, facial trauma, and craniofacial tumors are all pathologies that frequently affect the fronto-orbital region of the craniofacial skeleton in children. While the mechanisms of these pathologies vary greatly, the underlying principles of reconstruction remain the same. Despite the limited data in certain areas of fronto-orbital reconstruction in children, significant innovations have greatly improved its safety and efficacy. It is imperative that further investigations of fronto-orbital reconstruction are undertaken so that craniofacial surgeons may provide optimal care for these patients.

Cutaneous Rosai-Dorfman Disease With Increased Number of Eosinophils: Coincidence or Histologic Variant?

2011 ◽  
Vol 135 (12) ◽  
pp. 1597-1600 ◽  
Author(s):  
John J Cangelosi ◽  
Victor G Prieto ◽  
Doina Ivan
Keyword(s):  
Differential Diagnosis ◽  
Pituitary Gland ◽  
Plasma Cells ◽  
S100 Protein ◽  
Clinical Context ◽  
Cutaneous Involvement ◽  
Eosinophilic Infiltrate ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Skin Biopsies

Rosai-Dorfman disease (RDD) is characterized histologically by a dense histiocytic infiltrate with emperipolesis and associated lymphocytes, plasma cells, and neutrophils. Eosinophils are not commonly associated. We report a patient with initial thymus and pituitary gland involvement by RDD, who later developed papules on the groin and axilla. Skin biopsies showed admixed histiocytic infiltrates (lymphocytes, neutrophils, and plasma cells) without emperipolesis. A prominent eosinophilic infiltrate was also observed, a feature not, to our knowledge, previously reported. Immunohistochemistry revealed positivity for CD68 (most cells) and S100 protein (scattered cells) and was negative for anti-CD1a. The diagnosis of RDD was established in the clinical context after comparison with the thymic and pituitary lesions (similar histologic features, albeit with fewer eosinophils, and immunohistochemical profiles). We present the first case, to our knowledge, of multicentric RDD with cutaneous involvement and associated prominent eosinophilic infiltrate. Thus, RDD should be included in the differential diagnosis of mononuclear infiltrates containing eosinophils.

Robot-Assisted Stereotactic Shunting as a Novel Treatment for Pontine Glioependymal Cysts

2021 ◽  
Author(s):  
Simon Schieferdecker ◽  
Stefan Hunsche ◽  
Faycal El Majdoub ◽  
Mohammad Maarouf
Keyword(s):  
Abducens Nerve ◽  
Ependymal Cells ◽  
Robot Assisted ◽  
Quick Recovery ◽  
Glioependymal Cyst ◽  
Novel Treatment ◽  
First Case ◽  
Mri Scans ◽  
The Central Nervous System ◽  
Magnetic Resonance Imaging Mri

AbstractIn this case report, the authors describe the first case of a glioependymal cyst of the brainstem managed by robot-assisted, stereotactic, cysto-ventricular shunting. Glioependymal cysts are rare congenital cystic lesions that are thought to form by displacement of ependymal cells during the embryonal period. Glioependymal cysts have been reported in a variety of different locations within the central nervous system. However, glioependymal cysts of the brainstem have only been described once before. Here, we report the case of a 53-year-old man who was referred to our department due to hemiparesis, hemihypesthesia, and hemidysesthesia, as well as facial and abducens nerve palsy. A large pontine glioependymal cyst was confirmed via magnetic resonance imaging (MRI) scans. The cyst was subsequently decompressed by connecting the cyst with the fourth ventricle via robot-assisted stereotactic shunt placement. In the postoperative course, the patient made a quick recovery and did not report any permanent neurologic deficits.

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