1/31. Acute promyelocytic leukaemia complicating multiple myeloma: evidence of different cell lineages.The association of leukemia and multiple myeloma is well described usually as a complication of chemotherapy but also in the absence of chemotherapy or at diagnosis. Such leukemias are typically acute myeloid leukemia (AML), particularly myelomonocytic subtype, and cases of acute promyelocytic leuke (APL) are rarely reported. Controversy exists as to whether myeloma and AML originate from a single haematopoietic progenitor or arise from different cell lineages. We report a case of a 58 year old female who developed APL 10 months following diagnosis of nonsecretory light chain (kappa) myeloma which had been treated with local spinal irradiation and low dose oral melphalan and prednisone. Clonality had originally been demonstrated by light chain restriction (kappa) of her bone marrow plasma cells whilst immunoglobulin heavy chain and T cell receptor genes were germ line. At development of APL cytogenetics revealed t(15;17) and PML-RAR fusion gene was detected by RT-PCR. The patient was treated with all-trans retinoic acid (ATRA) and received 2 cycles of consolidation chemotherapy with idarubicin. Following this therapy the t(15;17) and PML-RAR were both undetectable whilst the clonal population of kappa staining plasma cells persisted. This particular patient represents a rare case of APL complicating multiple myeloma with persistence of the myeloma clone but disappearance of PML-RAR alpha rna following therapy. This case study appears to support the argument that the APL and myeloma originated from distinct cell lineages.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/31. Severe osteoporosis in familial hajdu-cheney syndrome: progression of acro-osteolysis and osteoporosis during long-term follow-up.hajdu-cheney syndrome is an autosomal dominant inherited osteodysplastic bone disease with the hallmarks of acro-osteolysis, skull deformations, and generalized osteoporosis. Very few patients have been followed long-term with respect to the prognosis of acro-osteolysis and osteoporosis. Here we describe a 39-year-old woman and her 19-year-old daughter who are both affected with the hajdu-cheney syndrome. Skeletal lesions were followed in the mother between the ages of 22 and 39 years. The acro-osteolytic lesions progressed markedly and caused shortening of several fingers; some end phalanges had completely disappeared. Severe spinal osteoporosis with serial vertebral fractures was found at the age of 22 years. New vertebral fractures developed until the age of 33 years, but did not progress afterward. High turnover osteoporosis was found in the bone histology of iliac crest biopsies performed at the ages of 22 and 34 years. Bone mineral content (BMC) was strikingly decreased at the age of 34 years (T score -5.1 SD) and did not significantly change during further follow-up. In the daughter, BMC failed to increase between the ages of 12 and 19 years and was also markedly decreased (T score -4.4 SD). This suggests that osteoporosis in hajdu-cheney syndrome is related to a low peak bone mass and a high bone turnover, leading to insufficient bone formation compared with the increased bone resorption.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
3/31. Destructive hip disease complicating traumatic paraplegia.Recent progress in the management of spinal cord injury has provided longer survivals, and as a result the incidence of secondary bone and joint disorders has increased. Joint lesions due to syringomyelia complicating a cervical spinal cord injury are the most common of these disorders. We report a case of destructive hip disease 7 years after an injury responsible for complete paraplegia with sensory loss. The joint lesions were painless, and there was no local evidence of inflammation. hip radiographs disclosed atrophic osteoarthropathy with complete destruction of the femoral neck and head. This unusual case raises questions about the pathophysiology of neuropathic osteoarthropathy in paraplegics.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
4/31. Chronic cerebrospinal fluid leak into skull base causing intramedullary osteolysis.We present the rare case of a 74-year-old woman with extensive intramedullary osteolysis of the petroclival skull base straddling both sides of the basi-occiput and basi-sphenoid. She presented with clinical features of recurrent spontaneous cerebrospinal fluid (CSF) otorrhoea despite three previous attempts at repair of the CSF leak. Recent advances in imaging techniques enable accurate radiological diagnosis of skull base lesions. We performed T1- and T2-weighted magnetic resonance (MR) images, a fluid attenuated inversion recovery (FLAIR) sequence MR, and a diffusion scan to study the characteristics of the skull base pathology. This revealed extensive osteolysis, with cystic spaces within the clivus and the petrous pyramid extending also to the basi-occiput. The leak was sealed using the technique of subtotal petrosectomy with obliteration of the eustachian tube and blind pit closure of the ear canal. The patient has been followed up for six months with no recurrence of symptoms.- - - - - - - - - - ranking = 5.2043294717679keywords = spinal, canal (Clic here for more details about this article) |
5/31. spinal dysraphism presenting as acro-osteolysis: report of four cases.The acro-osteolyses are a heterogeneous group of disorders characterized by bone resorption. The disorder may occur as familial, idiopathic, or secondary to vascular, inflammatory, or neurologic conditions. acro-osteolysis is rare in association with spinal dysraphism. It is even rarer for it to be the presenting symptom in spinal dysraphism. We report here four patients in whom the diagnosis of spinal dysraphism was established while investigating for the various causes of acro-osteolysis. All four patients presented with trophic changes and acro-osteolysis. hyperhidrosis in the affected limb was seen in three patients. One patient had leg pain, the others had no sensory or motor deficits. magnetic resonance imaging showed spinal dysraphism in all four patients.- - - - - - - - - - ranking = 4keywords = spinal (Clic here for more details about this article) |
6/31. Leptomeningeal relapse of multiple myeloma following allogeneic stem cell transplantation.Progressive multiple myeloma may manifest features of 'de-differentiation', including a plasmablastic appearance, failure to secrete paraprotein, extramedullary involvement, and resistance to treatment. A 44-year-old woman with kappa-light chain myeloma underwent allogeneic stem cell transplantation (SCT). Twenty months later she developed paraspinal plasmablastic myeloma in the absence of paraprotein in urine or myeloma in the marrow. The paraspinal masses responded to chemotherapy. At 30 months she developed myelomatous meningitis, which proved resistant to intrathecal chemotherapy, irradiation, and donor lymphocyte infusion (DLI). The leptomeningeal disease led to death at 38 months. This is the first report of leptomeningeal relapse of myeloma after allografting.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
7/31. Hepatocellular carcinoma presenting with bone metastasis.Bone metastasis is an unusual complication of hepatocellular carcinoma. We report here 2 cases of patients with bone metastases of hepatocellular carcinoma at presentation. Patient No. 1 with liver cirrhosis and hepatocellular carcinoma was admitted with a bone metastasis in the rib. The patient was treated with hepatic arterial chemotherapy and rib resection. Patient No.2 was known to have an asymptomatic liver mass of uncertain histology for a year when he presented with back pain. Because of signs of spinal compression, laminectomy was performed, and the diagnosis of metastatic hepatocellular carcinoma was established. The presence of bone metastases in hepatocellular carcinoma at presentation is extremely rare. More frequently, bone lesions are observed after successful treatment of the primary liver tumor. Both surgery and radiotherapy are used as palliative treatment in bone metastases of hepatocellular carcinoma. The treatment of hepatocellular carcinoma presenting with bone metastasis by bone resection and intraarterial chemotherapy seems to be of limited impact on patient survival because of dissemination of micrometastases in other organs and the frequent presence of other comorbid conditions. However, effective palliation using this multimodality approach is feasible. Hepatocellular carcinoma should be considered in the differential diagnosis of bone metastases.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
8/31. Nontraumatic intradiploic arachnoid cysts--report of five cases.Five cases of nontraumatic intradiploic arachnoid cysts in elderly patients are reported. All cysts were located in the occipital bone and appeared as well-demarcated radiolucent lesions. The cysts were multiple in three cases. Presenting symptoms included headache or dizziness, but most lesions were asymptomatic and found incidentally. In the most recent three cases, magnetic resonance (MR) imaging revealed intradiploic cysts containing cerebrospinal fluid (CSF) with cerebellar herniation. Operation found the cysts filled with CSF and dural defects through which cerebellar tissue was herniating. In two patients, CSF leakage from the outer table occurred. Intradiploic arachnoid cyst seems to be congenital in origin but commonly found in the elderly. MR imaging is the most useful diagnostic method for differential diagnosis from other osteolytic skull lesions.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
9/31. Diagnostic value of 99mTc-HMDP bone scan in atypical osseous tuberculosis mimicking multiple secondary metastases.STUDY DESIGN: A case of atypical osseous tuberculosis (TB) mimicking multiple secondary metastases on radiologic and nuclear imaging is presented. OBJECTIVES: To emphasize the contribution of nuclear bone scanning for the assessment of osseous tuberculosis in typical and atypical presentations. SUMMARY AND BACKGROUND DATA: Skeletal locations of TB mostly involve the dorsolumbar spine and diagnosis is often delayed. The presence of multiple TB sites can mimic secondary metastases and biopsy remains the mainstay for final diagnosis. methods: Clinical symptoms, lab tests, and imaging data are presented. Possible diagnoses are discussed. A review of imaging characteristics in cases of typical and atypical presentations of osseous TB is proposed. RESULTS: A dorsal spine spondylitis was first diagnosed on a 56-year-old patient presenting neurologic deficit of the left arm. Fine needle aspiration identified bacterial infection but was negative for mycobacterium tuberculosis. Whole-body bone scan allowed the identification of an asymptomatic sacroiliac lesion, which was accessible to biopsy and gave a final diagnosis. CONCLUSION: Nuclear bone scanning should be kept in mind when assessing spinal pain in patients at high risk of TB infection or reactivation.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
10/31. Intraosseous malignant peripheral nerve sheath tumor (MPNST) of the thoracic spine: a rare cause of spinal cord compression.OBJECTIVE: To describe the management of a patient presenting with intraosseous MPNST of the thoracic spine causing cord compression. SUMMARY OF BACKGROUND DATA: Malignant peripheral nerve sheath tumors (MPNST) are uncommon tumors of cells of peripheral nerve sheath origin. MPNST typically present as an enlarging mass originating from a peripheral nerve root in the trunk, extremities, and head and neck region. methods.: A 59-year-old woman presented with midthoracic back pain, paraparesis, and a T4 sensory level. Magnetic resonance image scan revealed a large enhancing and destructive lesion at the T3 level with cord compression. RESULTS: Decompressive laminectomies, tumor debulking, and instrumentation was performed from a posterior approach. At surgery, the lesion was noted to originate from the T3 vertebral body, and separate from the dura and spinal nerve roots. Surgical excision was incomplete and the spine was stabilized with a Ti frame. MPNST was confirmed histologically. Despite adjuvant radiotherapy, she developed metastatic deposits in the spine and femur. CONCLUSIONS: Intraosseous MPNST causing spinal cord compression has not been described as yet and should be added to the differential diagnosis of primary bone tumors causing cord compression. prognosis with MPNST can be poor, especially in patients with large tumors, undergoing subtotal surgical resection and in association with neurofibromatosis.- - - - - - - - - - ranking = 6keywords = spinal (Clic here for more details about this article) |
| | Next -> |