Primary amyloidoma of the axis and acute spinal cord compression: a case report

Background Localized primary amyloidosis is a disease characterized by a single tumor and localized amyloid deposit (amyloidoma) with no evidence of generalized amyloidosis. The occurrence of an amyloidoma in the spine is rare and only three cases affecting the axis have been previously reported. We describe the case of a 79-year-old woman presenting with a mass involving the odontoid process, responsible for an acute tetraparesia. Diagnosis of local primary amyloidosis was made after surgical excision.Results Despite the critical presentation, outcome was excellent after total excision of the mass. This case can be classified as a primary localized amyloidoma. The patient did not exhibit any infection, tumor or inflammatory disease, and continued investigations failed to demonstrate other amyloid deposit after one-year follow-up.Conclusions Amyloidoma must be discussed in presence of a tumor-like mass of the odontoid process and may be responsible, as in our case, for spinal cord compression.     

Primary cervical amyloidoma: a case report and review of the literature

Background context

Primary solitary amyloidosis or amyloidoma is a disease process characterized by the focal deposition of amyloid in the absence of a plasma cell dyscrasia with normal serum protein measurements. Solitary amyloidomas affecting the vertebrae are very uncommon but typically affect the thoracic spine. Primary cervical amyloidosis is an exceedingly rare entity with exceptionally good prognosis, but requires diligence of the treating physician to establish the diagnosis and implement the appropriate surgical intervention.

Purpose

This study aimed to present a rare case of primary cervical amyloidosis with long-term follow-up and review the clinical presentation, characteristic imaging findings, diagnostic pathology, differential diagnosis, treatment algorithm, and prognosis of the disease entity. This case demonstrates the progressive resorption of the amyloidoma over time after surgical stabilization. Previous reported cases of primary cervical amyloidosis will also be reviewed.

Study design

This study is a report and review of the literature.

Methods

A 77-year-old woman presented with a several-week history of gradual progressive weakness in her upper and lower extremities. Computed tomography and magnetic resonance imaging demonstrated a retro-odontoid nonenhancing soft-tissue mass, with erosive bony changes and severe mass effect on the upper cervical cord. The patient was taken to the operating room for decompression and posterior spinal stabilization.

Results

Intraoperative tissue specimens demonstrated amyloidosis and extensive systemic workup did not reveal any inflammatory processes, systemic amyloidosis, or plasma cell dyscrasia. Postoperatively, the patient regained full strength and ambulatory status. The patient remains asymptomatic at a 2-year follow-up. A postoperative follow-up magnetic resonance imaging demonstrated complete resorption of the residual amyloidoma.

Conclusions

Primary solitary amyloidosis is a rare form of amyloidosis that is important to differentiate given its excellent prognosis with surgical management. Treatment should include surgical decompression and spinal stabilization. This is the first case report to clinically and radiographically demonstrate the progressive resorption of a primary amyloidoma over time after surgical stabilization in the upper cervical spine. It is imperative that surgeons encountering such lesions maintain a high suspicion for this rare disease entity and advise their pathologists accordingly to establish the correct diagnosis.     

Primary amyloidoma of the thoracic spine

Amyloidoma is a nodular mass of amyloid with no evidence of generalized amyloidosis. Primary amyloidoma of the spine is very rare and has a predilection for the thoracic region. The tumor-like appearance and behavior make it difficult to diagnose on imaging studies. Despite its rarity and nonspecific radiologic findings, primary spinal amyloidoma should be included in the differential diagnosis of an osteolytic and calcified mass of the spine. We report the case of a 38-year-old woman with amyloidoma involving the thoracic spine and describe the main characteristics of this condition.     

Multiple myeloma-associated solitary epidural amyloidoma of C2-C3 without bony connection or myelopathy: case report and review of the literature

BACKGROUND: A large solitary amyloidoma in the cervical epidural space without bony connection and with minimal spinal cord compression and no myelopathy, as a first manifestation of disseminated amyloidosis in a multiple myeloma patient, has not been reported previously; this case is thereby distinct from the seven prior reports in the world literature, of a solitary amyloidoma of the cervical spine. CASE DESCRIPTION: A 72-year-old male, recently diagnosed with multiple myeloma, presented with a neuropathy that prompted a screening MRI of the cervical spine. This disclosed a large mass in the upper cervical epidural area felt to pose a high morbidity/mortality risk, leading to successful surgical removal of the amyloidoma mass. This then led to further testing that showed previously unsuspected widespread amyloidosis, which was subclinical up to that time. CONCLUSIONS: In patients with a newly discovered amyloidoma, whether or not in the setting of known multiple myeloma, further evaluation should be done to detect the presence of amyloidosis. If the mass is discovered de novo, then an investigation should be carried out to determine if multiple myeloma, as well as amyloidosis, is present.     

Primary amyloidoma of the thoracic spine

Focal amyloidosis (amyloidoma) involving the vertebral spine without an underlying systemic disorder is rare. Only six cases been reported in the literature so far, one involving the cervical spine, the rest occurring in the thoracic region. We present a patient with amyloidoma involving the thoracic spine and describe the magnetic resonance imaging features of this condition.     

Primary amyloidoma in epidural and paravertebral space of the lumbar spine

Background context

Localized amyloid deposits result in a mass, that is, so-called amyloidoma; it has been reported in every anatomic site, although systemic amyloid deposition is much more common. However, primary lumbar epidural amyloidoma without bony involvement is extremely rare. To the best of our knowledge, only one case has been reported previously.

Purpose

To report and review the clinical presentations, imaging studies, and treatment of epidural and paravertebral amyloidoma.

Study design

A case report and review of the literature.

Methods

Lumbar epidural and paravertebral amyloidoma in a 75-year-old man with neurologic compromise is presented. Laminectomy with mass resection was performed.

Results

After surgery, almost complete neurologic improvement was observed. Histologically, definite diagnosis was obtained only after the specific staining of tissue. No sign of local recurrence was evident 1 year after surgery.

Conclusions

Primary amyloidoma, although rare, should be included in the differential diagnosis of epidural mass of the spine. Diagnosis before surgery is difficult as there were no characteristic findings in clinical and imaging studies. Special histologic technique and stains are useful to make a definite diagnosis.     

Late esophageal perforation complicating anterior cervical plate fixation in ankylosing spondylitis: a case report and review of the literature

Esophageal perforation in ankylosing spondylitis (AS) is a rare complication in anterior cervical spine surgery and has not been reported before. A 50-year-old patient with AS developed incomplete tetraplegia after minimal trauma. C5 pedicle fracture was diagnosed and treated predominantly by physical therapy until neurological symptoms progressed. Cervical spine MRI showed C6/7 fracture and spinal cord compression. The patient underwent dorsal laminectomy, C5–7 anterior cervical fusion using allograft iliac crest and CASPAR-plate fixation. Delayed esophageal perforation appeared 10 months postoperatively when he came first to our hospital. He complained of dysphagia and developed acute dyspnea. Posterior stabilization with two plates was performed followed by removal of the ventral plate and screws. The esophageal laceration was sutured. The patient was treated with antibiotics and percutaneous endoscopic gastrostomy. Position of fracture and implants were accurate at 18 months postoperatively. The patient had persistent minor neurological deficits (Frankel D) at last follow-up. We conclude that esophageal perforation after anterior spinal fusion is a rare complication. Minor traumas in patients with AS are unstable and can result in significant spinal injury. Dorsoventral stabilization should be performed to avoid further complications.     

Solitary epidural amyloidoma of C2–4 without osteolysis of the spine in a multiple myeloma patient

Spinal cord compression sometimes occurs in patients with multiple myeloma (MM), and it usually involves bone infiltration from proliferative plasma cells leading to vertebral collapse, with the occasional extradural extension of a plasmacytoma [1]. However, a localized amyloid tumor, also known as an amyloidoma in the spine, is rare, and a few patients [2, 3] had underlying multiple myeloma (MM). The present case was unique in the form without osteolysis or vertebral collapse, which did not originate from adjacent spinal vertebrae, in a patient with a known diagnosis of MM.     

Amyloidoma of the chest wall: a rare entity

Amyloidoma (tumoral amyloidosis) is defined as a solitary localized tumor-like deposit of amyloid, in the absence of systemic amyloidosis. Amyloidoma is the least common presentation of tissue amyloid deposition, reported in many anatomic sites including the respiratory, genitourinary and gastrointestinal tracts, as well as the central nervous system, skin, breast and soft tissue. Amyloidoma of the chest wall is extremely rare, and to date only one case has been reported in literature. The authors present a case of a chest wall tumor that causes local destruction, being an amyloidoma on histopathologic examination. It was treated with wide local excision, with no recurrence during almost two years of follow-up. A search for occult systemic disease is recommended and was also performed.     

Paraplegia caused by painless acute aortic dissection

OBJECTIVES: Painless acute aortic dissection in which paraplegia is the only presenting sign is rare, with limited reported cases. CASE REPORT: The authors report a patient with painless acute aortic dissection who presented with sudden onset paraplegia. Ischemic diseases of the spinal cord were suspected as the cause. MRI revealed extensive acute aortic dissection with an intramural hematoma. The patient was treated conservatively by strictly controlling his blood pressure. The treatment was successful, although the motor function of the lower extremities could not be rescued. Although 3% to 5% of patients with acute aortic dissection present with paraplegia as a result of spinal cord infarction, most of these patients experience severe pain prior to presentation. CONCLUSION: Painless acute aortic dissection in which paraplegia is the only presenting sign is very rare. However, aortic diseases, including acute aortic dissection, should always be considered as a differential diagnosis of patients with sudden onset, painless paraplegia.     

Paraplegia caused by vertebral compression fractures in senile osteoporosis

Paraplegia followed spontaneous deformed osteoporotic spine is very rare. Our own 3 cases treated conservatively in two and operatively in one with success and 11 cases reported in the literatures were analyzed. All patients were recovered from paraplegia. One patient was died one and a half year after the recovery of her paralytic episode. Thorough autopsy study of this patient showed no organic lesion in the regarded spinal cord due to such kind of spine deformity.     

Postoperative recovery of complete sudden paraplegia due to lumbar schwannoma. Case report

The authors present a rare case of acute complete paraplegia due to a lumbar schwannoma. The clinical presentation, magnetic resonance imaging features, and management strategy are discussed. A 29 year-old man presented with acute complete paraplegia and bladder and bowel incontinence. He had a history of stable back pain and a 4-year history of lower-extremity numbness bilaterally. Magnetic resonance imaging revealed an enhancing extraaxial mass filling the spinal canal over two segments below the conus medullaris. An L1-3 laminectomy was performed and the tumor was completely removed. Pathological evaluation showed features characteristic of a schwannoma. The patient regained almost complete motor function after 6 months. To the authors' knowledge, this is the first reported case of acute complete paraplegia secondary to lumbar schwannoma. Possible mechanisms of this occurrence are discussed.     

Acute flaccid paraplegia: a rare complication of meningococcal meningitis

STUDY DESIGN: A case report of spinal cord dysfunction following meningococcal meningitis. OBJECTIVES: To describe a rare complication of meningococcal meningitis. SETTING: Spinal Unit, Armed Forces Institute of Rehabilitation Medicine, Rawalpindi, Pakistan. METHODS: A young healthy male developed meningococcal meningitis followed by acute onset low thoracic flaccid paraplegia with complete motor and sensory loss and sphincter disturbance. He responded well to antibiotics but was not investigated for causes of paraplegia. While at home in a rural area, he developed pressure ulcers, anemia and depression. Magnetic resonance imaging of the whole spine and computed tomography scan of the brain performed after 4 and 10 weeks were normal. RESULTS: The patient had a comprehensive rehabilitation at our institute. Recovery was complicated by ossification in the right thigh, which responded well to radiotherapy. At 1-year follow-up, the motor deficit and neurogenic bladder and bowel persisted and the patient remained wheelchair dependent for mobility. CONCLUSION: Several mechanisms have been proposed to explain spinal cord damage after meningitis. These include spinal cord infarction; autoimmune-mediated inflammatory myelopathy and direct infection of the cord. Most probable cause of spinal cord dysfunction in this case was thoracic myelopathy.     

Primary osteosarcoma of the L2 lamina presenting as “silent” paraplegia: case report and review of the literature

Summary Primary osteosarcomas of the vertebral column are not common, and to our knowledge a total of 78 cases, mostly located in the vertebral body, have been previously reported. We report a primary osteosarcoma of the spine with an extremely rare location — the lamina of the second lumbar vertebra. The patient, a 38-year-old woman, was admitted with paraplegia of a short duration without pain. Preoperatively, the patient underwent CT scanning for staging (Enneking IIB) followed by a needle biopsy and local preoperative arterial embolization. An emergency decompressive laminectomy was performed, and stabilization was carried out using methylacrylate. The patient showed a complete neurologic recovery. Combined chemotherapy and local irradiation did not prevent tumor recurrences, which occurred 12 and 19 months after the initial intervention and were associated with recurrent neurologic impairment. The patient died 19 months after the initial presentation, while in paraplegia, from lung metastases. Based on our unique observation, it seems that in primary osteosarcomas located in the posterior elements of the spine, the symptoms are not specific, and the disease may only become manifest when the tumor is no longer resectable. When the tumor is associated with neurologic impairment, spinal canal decompression should be performed even though it does not radically resect the tumor because it significantly improves the quality of the patient's life.     

脊椎肿瘤的外科治疗

目的：探讨采用不同经路切除脊椎肿瘤与改进的人工椎体和自体植骨术治疗脊椎不同节段肿瘤的疗效。方法：对13例脊椎肿瘤采取切除瘤椎、人工椎体置换和自体植骨术;肿瘤包括颈椎4例,胸椎3例,腰椎6例。术前颈脊髓瘫痪1例,不完全瘫痪4例,肿瘤的术后病理学诊断有骨巨细胞瘤3例,骨囊肿、Ewing瘤、骨神经鞘瘤、软骨瘤、骨成纤维细胞瘤、骨纤维细胞瘤、骨嗜酸性肉芽肿、骨转移瘤、骨肉瘤和骨血管瘤各1例。结果：13例安全度过围手术期,无脊髓神经症状加重。除1例3个月恢复良好后失访外,余12例获平均4．3年随访,瘫痪5例完全恢复,12例均恢复家务和工作,X线片显示假体无松动、移位,植骨融合。结论：应根据肿瘤的脊柱节段和类型,选择手术治疗方式,采用人工椎体置换和自体植骨术,对完全切除脊椎肿瘤和重建脊椎稳定性是十分有益的。     

Multiple spinal extradural meningeal cysts presenting as acute paraplegia. Case report and review of the literature

Multiple spinal extradural meningeal cysts are rare. To the authors' knowledge, there have been only four reported cases in the world literature. The authors report a case of multiple spinal extradural meningeal cysts in a 31-year-old woman presenting with acute paraplegia. Magnetic resonance imaging of the thoracolumbar spine revealed multiple extradural cystic lesions extending from T-7 to T-8 and from T-12 to L-3. Intraoperative findings demonstrated a white, fibrous, and tense cyst filled with cerebrospinal fluid-like colorless fluid. Excision of the posterior wall of the symptomatic cyst was followed by immediate neurological improvement. The examination of the pathological specimen showed a thick duralike layer of collagen and an inner membrane of arachnoid that is often not found in these lesions. The final diagnosis was based on combined imaging, intraoperative, and histopathological findings. The authors review the literature and discuss the etiological, diagnostic, and therapeutic aspects of this lesion.     

Als Spondylodiszitis imponierende vertebrale Sarkoidose

Sarcoidosis is a multisystem disorder of unknown cause characterized by non-caseating granulomatous infiltration affecting multiple organs. Osseous involvement has been estimated to occur in up to 13% cases. Due to its rare occurrence, only a few cases of vertebral sarcoidosis have been reported. Including our case, there are only three reports of vertebral sarcoidosis causing instability being treated by surgical fusion. The 54-year-old male patient presented with severe back pain, which had significantly impeded his mobility for months. He was unresponsive to analgesic and non-steroidal anti-inflammatory drugs (NSAID) and also reported weight loss and progressive weakness. Laboratory tests showed no specific alterations. When an MRI revealed destructive vertebral lesions of the lower thoracic spine, a tentative diagnosis of pyogenic discitis was made. For spinal stabilization as well as infection control, sequential operations were performed: dorsal spinal fusion by means of an internal fixation system was followed by excision of vertebrae 7/8 and filling of the defect with an autologous tricortical iliac crest bone graft through a ventral approach. Histological section of the excised vertebrae demonstrated a non-caseating granulomatous infiltration. Once this was determined, treatment with corticosteroids was started. The patient made a complete recovery and remains pain-free.     

Dumbbell-type solitary fibrous tumor in the cervical spine

 We report a dumbbell-type solitary fibrous tumor in the cervical spine. Eight spinal solitary fibrous tumors have been reported previously, but the cervical location is rare and this may be the first report of a dumbbell-type tumor. Histopathological examination showed that the tumor was composed of spindle cells in a collagen-rich matrix, although regional variability was noted. Diffuse immunostaining for CD34 and vimentin was noted in the cytoplasm of the tumor cells. This rare tumor should be recognized in the differential diagnosis of cervical spinal tumors. Received: October 8, 2002 / Accepted: January 22, 2003 RID="*" ID="*" Offprint requests to: K. Endo     

Pneumocephalus secondary to cerebrospinal fluid leak associated with a lumbar pressure ulcer in a man with paraplegia

BACKGROUND: Pneumocephalus is a well-known condition following head trauma, but is uncommon in injuries or surgeries of the spine. Even more unusual is its occurrence in association with an eroding pressure ulcer and the subsequent penetration of the intrathecal space. This article reports such a case in a man with spinal cord injury. No previously reported cases of pneumocephalus and subarachnoid-pleural fistula secondary to a pressure ulcer are known. METHODS: Case presentation and literature review. FINDINGS: A 75-year-old man with with paraplegia, T2-level spinal cord injury, impairment score on the American Spinal Injury Association (ASIA) scale of ASIA A, and multiple pressure ulcers developed dural leak via a tract extending from a thoracolumbar ulcer to the T11 -T12 vertebrae. The resultant pneumocephalus was diagnosed based on acute neurologic symptoms and computed tomography scan. He underwent excision of the pressure ulcer and a T10 through L1 laminectomy, during which the dural leak was located and sealed with fibrin glue. CONCLUSION: Deep pressure ulcers overlying the spine should be managed aggressively to avoid life-threatening complications. Signs of meningeal irritation and/or mental status change in a patient with a deep posterior midline pressure ulcer with drainage suggestive of cerebrospinal fluid may indicate invasion of the intramedullary canal. Imaging studies are diagnostic of pneumocephalus and surgical closure of the spinal fluid leak is imperative when meningeal or other adverse neurologic signs are present.     

Giant soft tissue amyloidoma of lumbar region: A case report and literature review

Localized amyloidoma is characterized by a tumour mass with amyloid deposit in a patient who has no detectable abnormal serum proteins or plasma cell dysfunction. In patients with no history of cancer or dialysis, soft tissue localized amyloidoma is a very rare benign condition and can achieve considerable dimensions. We report a soft tissue localized amyloidoma of the lumbar region in an otherwise healthy patient, the largest of its kind found in literature, and further review those cases previously published of this specific condition. We discuss our literature review, concerning the aetiology and diagnosis of this kind of amyloidoma. Level of Evidence: Level V, diagnostic study