ROSAI-DORFMAN DISEASE CAUSING SPINAL CORD COMPRESSION

Neurosurgery ◽  
2008 ◽  
Vol 62 (4) ◽  
pp. E977-E978 ◽  
Author(s):  
Grégory Dran ◽  
David Rasendrarijao ◽  
Fanny Vandenbos ◽  
Philippe Paquis

Abstract OBJECTIVE Rosai-Dorfman disease is a rare idiopathic, histiocytic, proliferative disease characterized by massive, painless cervical lymphadenopathy. Extranodal involvement is rare and central nervous system involvement is unusual. We present a patient with Rosai-Dorfman disease with spinal cord compression. Very few cases have been reported in the literature. CLINICAL PRESENTATION A 17-year-old man presented with a 1-month history of progressive fatigue of the legs. His medical history was significant for Rosai-Dorfman disease diagnosed 7 months earlier. Clinical examination was consistent with a pyramidal syndrome and proprioceptive disturbances on his lower limbs without sensory level. A magnetic resonance imaging scan revealed an intradural extramedullary space-occupying lesion at the T1-T4 level with dural insertion and spinal cord compression. INTERVENTION A T1–T4 laminotomy was performed. Upon opening the dura, a reddish-gray mass was encountered, which encased the dorsal and lateral arachnoidal membrane. The lesion was relatively well circumscribed and was easily dissected from the underlying arachnoid. Pathological examination of the compressive soft tissue was consistent with Rosai-Dorfman disease. Postoperatively, the patient showed substantial improvement in neurological function. He was followed for 18 months with no complaints and no recurrence. CONCLUSION Neurosurgeons should consider this rare etiology of spinal cord compression. They must be aware that this lesion can occur in front of an intraspinal lesion, mimic meningiomas, occur in young people, and can potentially be associated with other locations of disease, including intracranial lesions. Surgery is the treatment of choice.

2012 ◽  
Vol 10 (4) ◽  
pp. 508-511 ◽  
Author(s):  
Leonardo Giacomini ◽  
Roger Neves Mathias ◽  
Andrei Fernandes Joaquim ◽  
Mateus Dal Fabbro ◽  
Enrico Ghizoni ◽  
...  

Paraplegia is a well-defined state of complete motor deficit in lower limbs, regardless of sensory involvement. The cause of paraplegia usually guides treatment, however, some controversies remain about the time and benefits for spinal cord decompression in nontraumatic paraplegic patients, especially after 48 hours of the onset of paraplegia. The objective of this study was to evaluate the benefits of spinal cord decompression in such patients. We describe three patients with paraplegia secondary to non-traumatic spinal cord compression without sensory deficits, and who were surgically treated after more than 48 hours of the onset of symptoms. All patients, even those with paraplegia during more than 48 hours, had benefits from spinal cord decompression like recovery of gait ability. The duration of paraplegia, which influences prognosis, is not a contra-indication for surgery. The preservation of sensitivity in this group of patients should be considered as a positive prognostic factor when surgery is taken into account.


2014 ◽  
Vol 24 (S4) ◽  
pp. 529-535 ◽  
Author(s):  
Sara Sciacca ◽  
Kostantinos Barkas ◽  
Lauren Heptinstall ◽  
Christopher McNamara ◽  
Rohit Shetty

Medicine ◽  
2018 ◽  
Vol 97 (40) ◽  
pp. e12722 ◽  
Author(s):  
Yongning Li ◽  
Xin Wang ◽  
Jun Gao ◽  
Shuangni Yu ◽  
Zhimin Li

2019 ◽  
Vol 2019 ◽  
pp. 1-7
Author(s):  
Nooraldin Merza ◽  
Ahmed Taha ◽  
John Lung ◽  
Anthony W. Benderman ◽  
Stephen E. Wright

Immunoglobulin G4-related disease (IgG4-RD) is known for forming soft tissue mass lesions that may have compressive effects. It is an extremely rare disease that most frequently affects the pancreas causing autoimmune pancreatitis. It can also affect the gallbladder, salivary glands, and lacrimal glands causing respective organ-specific complications. In our report, we describe an IgG4-RD case that affected the spinal cord. A 60-year-old female presented with cervical spinal cord compression caused by IgG4-RD leading to several neurological deficits. Pathological examination of the excisional biopsy of the mass revealed dense lymphoplasmacytic cells infiltration and stromal fibrosis with IgG4 and plasma cells. The patient showed a dramatic response to the administration of systemic steroids with almost resolution of her neurological symptoms. This case highlights the first case in literature for IgG4-RD of the extradural tissue causing spinal compression. Hereby, we also demonstrate the dramatic response of IgG4-RD to the administration of systemic steroids as the patient had no recurrence after 5 years of close follow-up, the longest reported period of follow-up reported in the literature to date.


2005 ◽  
Vol 8 (5) ◽  
pp. 593-598 ◽  
Author(s):  
Khulood Al-Saad ◽  
Paul Thorner ◽  
Bo-Yee Ngan ◽  
J. Ted Gerstle ◽  
Abhaya V. Kulkarni ◽  
...  

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.


2021 ◽  
Author(s):  
Yafei Cao ◽  
Yihong Wu ◽  
Weiji Yu ◽  
Weidong Liu ◽  
Shufen Sun ◽  
...  

Abstract Background: Lower limb sensory disturbance presentation can be a false localizing cervical cord compressive myelopathy (CSM). It may lead to delayed or missed diagnosis, resulting in the wrong management plan, especially in the presence of concurrent lumbar lesions.Case presentation:Three Asian patients with lower limb sensory disturbances presentation were treated ineffectively in the lumbar. Magnetic resonance imaging (MRI) showed cervical disc herniation and cervical level spinal cord compression. Anterior cervical discectomy surgery and zero-p interbody fusion were performed. After operations, imagings showed that the spinal cord compression were relieved, and the lower limbs sensory disturbances were also relieved. Three-months follow-up after operation showed good recovery.Conclusions:These three cervical cord compression cases of lower limb sensory disturbance presentation were easily misdiagnosed with lumbar spondylosis. Anterior cervical discectomy and fusion operation had a good therapeutic effect. Therefore, cases that present with lower limb sensory disturbance, but in a non-radicular classical pattern, should always alert a suspicion of a possible cord compression cause at a higher level.


Neurosurgery ◽  
1990 ◽  
Vol 26 (4) ◽  
pp. 678-681 ◽  
Author(s):  
João de Sá ◽  
José Pimentel ◽  
Mamede Carvalho ◽  
Pedro Evangelista ◽  
Paulo Martins

Abstract Retroperitoneal fibrosis is a rare disorder in which the abnormal fibrotic tissue compresses retroperitoneal organs. In the majority of patients no obvious cause can be found, hence the name idiopathic retroperitoneal fibrosis. The process can also arise from other anatomic areas, suggesting a multifocal origin. We report a case of dorsal epidural compression in a 63-year-old patient harboring idiopathic retroperitoneal fibrosis. A magnetic resonance imaging scan revealed the precise location of the lesion. It is suggested that retroperitoneal fibrosis should be considered in the differential diagnosis of epidural spinal cord compression.


2019 ◽  
pp. 326-328
Author(s):  
A. Khelifa ◽  
I. Assoumane ◽  
S. Bachir ◽  
L. Berchiche ◽  
A. Morsli

Background. Spinal angiolipoma (SAL) is a rare tumour with double component mature adipose tissue and proliferating abnormal blood vessels, which result in spinal cord compression requiring an urgent surgical removal. We report a case of woman with spinal angiolipoma. Case presentation. The patient is a 26 years old woman with past medical history of a low grade urothelial bladder carcinoma removed 4 months before she consults at our department, 2 months later the patient presented a lower limbs weakness. The clinical exam at the admission found a patient with paraparesis, hypoesthesia at the level of Th4 and urinary urgency. The spinal MRI objectified a spinal cord compression by a lesion located at the epidural space from Th2 to Th4. The patient was operated and a fatty well vascularized tumour distinct from the epidural fat was removed through a Th2 to Th4 laminectomy. The pathology study was in favour of an angiolipoma. Days after the operation the patient recovered totally, the weakness and the urinary urgency disappeared. The patient is flowed since 24 months she got pregnant. Conclusion. Spinal angiolipoma is a rare tumour with a clinic of spinal cord compression, MRI is the gold standard in diagnosis it shows a fatty lesion with a large enhancement, surgery is the perfect treatment with good outcome and exceptional recurrence.


Neurosurgery ◽  
2002 ◽  
Vol 50 (2) ◽  
pp. 399-403 ◽  
Author(s):  
Edward Kachur ◽  
Lee-Cyn Ang ◽  
Joseph F. Megyesi

ABSTRACT OBJECTIVE AND IMPORTANCE Castleman's disease is a rare lymphoproliferative disorder most often found in the mediastinum. Localized forms are usually benign, whereas multicentric forms may be aggressive . We report a patient with Castleman's disease who presented with spinal cord compression, and we review previously published cases of Castleman's disease involving the central nervous system. To our knowledge, this is only the second case of Castleman's disease presenting as a spinal epidural mass with cord compression. CLINICAL PRESENTATION A 44-year-old otherwise healthy woman presented acutely with difficulty walking. Examination revealed mild myelopathy in her legs. Magnetic resonance imaging revealed a posterior epidural mass compressing the thoracic spinal cord at T3–T5. INTERVENTION Thoracic laminectomy and gross total resection of the lesion were performed. Pathological examination of the lesion identified the hyaline-vascular type of Castleman's disease. The patient's symptoms resolved postoperatively. CONCLUSION Castleman's disease presenting as a spinal epidural mass lesion with cord compression is rare. Surgical treatment can result in an excellent outcome.


Author(s):  
Tony El Murr

A 59 year old male with a past medical history of cholecystectomy and essential hypertension, presented to the emergency department with severe back pain, abdominal discomfort, poor appetite, generalized fatigue and progressive weight loss of 10 kgs over a three-month period. He has never been a smoker and drinks alcohol occasionally. He has no known allergies and no familial history of cancer. His current home medication includes beta blocker; angiotensin receptor blocker and low dose acetylsalicylic acid. His back pain started two months prior to presentation; it is not well localized to specific vertebra, not irradiating to lower limbs and sometimes related to weight lifting and cough. It is rarely exacerbated at night. His orthopedic surgeon attributed it to osteoarthritis since the patient used to practice hard manual work and weights lifting at his shop. He has been treated since that time with NSAIDs, muscle relaxant and opioids without complete analgesic response. At presentation, he had no fever, chills or night sweats. He had no urinary or sexual complaints that would suggest prostatic disease and was otherwise asymptomatic. The physical examination showed diffuse pain on lumbar vertebral percussion and abdominal tenderness in the right upper quadrant. He has no skin lesions and no palpable peripheral lymph nodes. His neurologic examination was also normal. On admission, significant laboratory findings showed hemoglobin 11.8 g/dl, platelets of 97,000 k/ul, white blood count 4,400 k/ul, ESR=40, creatinine 0.4 mg/dl,CRP 7.5 mg/L, lactate dehydrogenase 2256 U/L, alanine aminotransferase (ALAT) 70 U/L, gamma-glutamyl transferase (GGT) 1500 U/L, total bilirubin 2.32 mg/dl, and a creatine kinase (CK) 167 U/L. Prostatic specific antigen (PSA) was normal and equal to 1.17 ng/ml. Peripheral smear displayed normal pattern and thyroid tests were all within normal ranges. He was negative for salmonellosis, brucellosis and HIV. Tuberculin PPD test was negative as well. His chest X-ray was normal and abdominopelvic ultrasound showed only multiple liver nodules and mild prostate hypertrophy without ascites or significant abdominal lymph nodes. Lumbar MRI done one week before his admission revealed multiple vertebral lytic lesions without spinal cord compression. PET/CT done on the third day at hospital revealed a significant uptake of radiotracer by multiple small nodules in the right lung, in the liver and by multiple lymph nodes within the abdominal cavity. Moreover there were many lytic lesions in the dorsal and lumbar vertebrae. Surprisingly, the scan also showed focal, intense uptake of the prostate with a SUVmax of 8, 28 with evidence of seminal vesicles invasion. Both gastroscopy and colonoscopy was normal as well as his brain MRI. Ultrasound-guided liver nodules biopsies performed on the fifth day after stopping the acetylsalicylic acid revealed neuroendocrine small cell carcinoma as it showed immunohistochemical (IHC) positivity for synaptophysin and CD56. IHC was negative for TTF1 and PSA. Ultrasound-guided biopsy of the prostate was not performed for medical reasons as on his hospital day 7, the patient started feeling numbness and muscle weakness in his lower limbs more severe on the left side associated with urinary incontinence and revealing a spinal cord compression by secondary bone lesions. Regarding this rapidly progressive disease, high dose dexamethasone subcutaneous therapy and ten sessions of focused proton beam radiotherapy has been conducted on daily basis to release the spinal cord compression without significant improvement. Meanwhile, and based on liver biopsy findings, treatment by octreotide 100mg S/C twice daily was started, followed by chemotherapy with Carboplatin, Etoposide, and Atezolizumab (TECENTRIQ) 1200mg. Few days after, the patient developed severe prolonged pancytopenia requiring blood and platelets transfusions and treatment with double dose of granulocyte stimulating growth factor. His prolonged neutropenia was complicated by CMV colitis with unretractable diarrhea, bilateral pneumonia and pseudomonas aeroginosa septicemia. After one month of large spectrum antibiotics, antiviral and antifungal treatment, and while he was still neutropenic, bone marrow biopsy revealed severe infiltration by neuroendocrine small cell carcinoma. The patient was still deteriorating clinically and showing further weight and appetite loss, total muscle weakness and asthenia. At this point, he was no longer a candidate for chemotherapy but only symptomatic treatment was maintained. He died 45 days after his admission.


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