Vertebrae Compression Fracture Due to Extra-nodal RDD Case

Vertebrae Compression Fracture Due to Extra-nodal RDD CaseINTRODUCTIONRDD was first exposit by Destombes1 in 1965 as a rare, separate entity of benign self-limiting sinus histiocytosis with gigantic lymphadenopathy later Rosai and Dorfman2 in 1969, confirmed it as a separate clinic-pathologic entity. Although RDD whitethorn occur in any age group, it is most frequently seen in children and young adults. Isolated intracranial illness tend to occur older patients. The disease is more general in males and in individuals of Afri apprise descent . The most frequent clinical manifestations include fever, night sweats and weight with bilateral massive cervical lymphadenopathy. Mediastinal, inguinal and retroperitoneal nodes may also be involved. RDD is potentially systemic disease and may affect extra nodal sites up to 43% of all RDD patients. The common extra-nodal sites include peel off, upper respiratory tract, and bone. Skeletal social occasion as a sole manifestation of RDD is ex tremely rare, occurring in fewer than 2% of all RDD patients and maturate most frequently in long bones such as tibia, femur, humerus, clavicle and bones of the hands. CNS elaborateness is rare (5%), with 75% of cases involving the brain and 25% involving the spinal anaesthesia canal. Involvement of the CNS without concurrent nodal involvement is rare.3 Laboratory findings are non-specific with leucocytosis, neutrophil, elevation of erythrocyte sedimentation rate (ESR), hypergammaglobulinemia being the most common findings.We impart a rare case of vertebrae compression fracture due to extra-nodal RDD without lymphadenopathy later developed metastases to brain.CASE REPORTA 45 year old maam presented with h/o sudden onset of paraplegia. Work up was done and spinal mass was found. Later the patient underwent surgical debulking to releave her symptoms. The patient gradually improved. Histopathologically it was diagnosed as RDD. The patient was symptom free for 6 months, then she dev eloped sudden onset of seizures and lower limb weakness. MRI showed multiple cranial and spinal metastasis, sideline which the patient underwent craniotomy. The patient was only treated with whole brain radiotherapy of 30Gy. There was no improvement of symptoms. The patient eventually expired 6 months interest the completion of radiotherapy.DiscussionRDD in its classic draw is a benign, self-limiting histiocytic proliferative disorder that is characterized by massive lymphadenopathy. Most patients with RDD are in their second or third gear decade of life, the mean age of onset of nodal disease is about 20years, with a male-to-female ratio of 1.41. It is commonly report in African-Americans. The etiology of RDD remains unknown resistive system dysfunction and an autoimmune process or viral infection such as Epstein-Barr virus and human herpes virus type 6 digest been attributed with the pathogenesis of RDD. The presentation of RDD can be categorised into three sub types 1) tho se with only lymph nodes enlargement with sudden increase and spontaneous regression and without any further recurrences 2) those with immunologic abnormalities at presentation have a more widespread nodal disease and a higher fatality rate4,5 3) those with several extra-nodal site involvement, multi-nodal disease and a protracted clinical course with multiple relapses and remissions for years. In these cases, the severity of disease depends on the type and number of extra-nodal sites.6Patients who present with or subsequently develop intracranial involvement, become symptomatic at a later mean age (34.9 years), with a strong male predominance.7 Involvement of CNS is rare (5%), with 75% of reported cases involving the brain and 25% involving the spinal canal. Involvement of the CNS in the absence nodal involvement is rare.3 The most common intra cranial presentation is solitary dural establish lesion, but multiple intracranial lesions have also been reported. The suprasellar region , cerebral convexity, para sagittal region, cavernous sinus, and petroclival regions are most common locations.7 Only 11 cases of spinal RDD have been reported, of which 10 patients had an extra-medullary tumor. Seven patients presented with epidural lesion, three presented with intra dural lesions.8 Spinal cord compression has been reported in a few patients with spinal involvement causing neurologic deficits.9 Compression fracture due to spinal involvement of RDD has not been reported. CNS RDD is a rare benign histiocytic proliferative disorder, with imaging findings typically screening a meningioma-like, dural-based extra-axial mass lesion, which can be solitary or multiple. Imaging studies typically show an enhancing meningeal-based mass with a variable amount of edema border the lesion.Skeletal involvement of RDD as a sole manifestation is extremely rare, occurs in less than 2% of all RDD patients and involves the long bones such as tibia, femur, humerus, clavicle and bones o f the hands or skull, and is usually multiple. Skeletal lesions of RDD are intramedullary osteolytic with either poorly or sharply defined margins. Spinal Rosai-Dorfman disease can be misdiagnosed as meningioma when arising from dura or as metastatic disease of another cause when presenting as vertebral body disease.10 The most common derivative instrument diagnosis of a primary bone RDD includes bacterial osteomyelitis, fungal infections such as histoplasmosis and Langerhans cell histiocytosis.Definitive diagnosis requires tissue examination. Microscopically, lymph node enlargement with sinuses containing fully grown numbers of histiocytes with phagocytosed lymphocytes. This is a typical picture of RDD. Immunopositivity is expressed by these cells for both CD68 and S-100 protein, and negative for CD1a. RDD has a typical finding in histopathologic studies, that is emperioplesis a phenomenon of phagocytosis of intact lymphocytes, blood plasma cells, erythrocytes or neutrophils.11 LCH is a close differential diagnosis both on radiology and histology. Both histiocytes of RDD and LCH are positive for CD68 and S100, but CD1a is positive in LCH. alike Langerhans cell histiocytosis does not exhibit emperioplesis.Treatment in the majority of cases is not indicated as it is a benign and self-limiting.12,13 Therapy is indicated in patients with bulky extra nodal disease with involvement of vital organs or causing life threatening complications.13 Surgical resection or debulking can be considered as an option only when the nodal mass iss compact airway or intra dural lesion.13 When stop resection cannot be achieved, adjuvant radiosurgery can be give successful partial resection.14 Surgical excision of resectable lesions induced complete remission (CR) in 8 out of 9 patients.13The role radiotherapy in the treatment has not been established. Various medical alternatives in the management have been tried with limited results. Short term Systemic corticosteroids and pr olonged course low dose oral prednisolone has been effectively used in RDD with skin and lymph nodal involvement with respiratory obstruction.15 Steroids tend to alleviate the symptoms and decrease nodal size, recurrences have been reported following withdrawal of steroids. Chemotherapeutic agents have been used without any encouraging results. confederacy chemotherapy of low dose MTX and 6-MP, MTX/6MP/vinblastine/6-thioguanine and acyclovir with thalidomide have been tried with limited benefit to patients. It is possible that different patients with RDD may respond to different drugs. Targeted therapy in the form of imatinib, interferon- , cladarabine and more recently rituximab has also been in the treatment of RDD.