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 of the thoracic spine presenting with acute paraplegia

BACKGROUND

Primary solitary amyloidoma of the spine is a rare disease characterized by localized deposition of amyloid. To the best of our knowledge, there have been only 14 cases previously reported in the literature. Patients with focal spinal amyloidoma usually have relatively long symptomatic periods preoperatively, ranging from 3 weeks to 6 years (mean: 12 months). Only two reported patients had acute paraplegia. We add a third case of a thoracic spine amyloidoma presenting with acute paraplegia.

CASE DESCRIPTION

A 65-year-old man presented with a three-day history of progressive paraplegia and urinary retention. He was found to have severe cord compression at T2 on magnetic resonance imaging. He underwent emergent decompressive laminectomy with instrumentation for spinal stabilization. Histopathology revealed abundant amyloid deposits. A systemic work-up was negative for amyloidosis. The patient showed marked neurological improvement with residual mild spastic gait after 1 year.

CONCLUSIONS

Primary spinal amyloidoma with acute paraplegia is rare. One-stage surgery combining prompt decompression and stabilization of the spinal column is mandatory in cases of spinal amyloidoma with acute myelopathy, because primary solitary amyloidoma carries a good prognosis.     

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 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 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.     

Primary amyloidoma of the thoracic spine causing paraparesis

A 52-year-old lady presented with progressive paraparesis. MRI revealed collapse of D9 vertebrae which was infiltrated by a low signal lesion both on T1 and T2. She underwent vertebrectomy and insertion of moss cage. The histology came back as amyloidoma. The presentation, investigation and management of primary amyloidoma of the spine is discussed along with a review of the literature.     

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     

A solitary osteochondroma of the pediatric thoracic spine: a case report and review of the literature

The objective of this study design is to describe the diagnosis and successful treatment of a pediatric patient with an osteochondroma of the thoracic spine. An osteochondroma is a bone tumor that rarely occurs in the thoracic spine, especially in the pediatric population. A simple painless mass may be the only presenting symptom. The laboratory findings are usually nonspecific. Radiographs may be nondiagnostic in certain cases, and computed tomography is the imaging modality of choice. The diagnosis, treatment, and outcome of a pediatric patient with an osteochondroma of the thoracic spine, including a possible genetic predisposition, are reviewed, along with a complete review of the literature. Anteroposterior and lateral plain radiographs illustrated a well-defined solid mass arising from the posterior elements of the tenth thoracic vertebrae. A computed tomography (CT) scan further delineated that the mass arose from the spinous process with no obvious impingement of the nerve roots. After excision of the lesion, gross pathological and histologic evaluation was consistent with an osteochondroma. The use of CT allowed accurate diagnosis of the osteochondroma. This led to appropriate surgical intervention, resulting in definitive treatment.     

Treatment of myelopathy due to cervicothoracic OPLL via open door laminoplasty

STUDY DESIGN: Postoperative long-term follow-up study of open door laminoplasty for the ossification of the posterior longitudinal ligament (OPLL) in the thoracic spine. OBJECTIVES: Techniques and outcomes of open door laminoplasty were described. The efficacy of this procedure was discussed and compared with other surgical methods for thoracic OPLL reported in the literature. SUMMARY OF BACKGROUND DATA: OPLL of the thoracic spine is often associated with cervical OPLL or ossification of the yellow ligament (OYL) of the thoracic spine; therefore, it is extremely difficult to determine the most appropriate surgical therapeutic procedure. There are very few detailed reports about extensive laminoplasty for OPLL of the thoracic spine. METHODS: The subjects included in this study consisted of 13 consecutive patients of thoracic OPLL who were surgically treated between 1994 and 2003 by the open door laminoplasty using the spinal processes and ligament complex as spacers for the open side. The number of manipulated lamina, including the cervical spine, was from 7 to 14 (mean 10 laminae), the follow-up period was 75 months on average. We evaluated the clinical symptoms by the JOA scoring method and postoperative bone union and thoracic kyphosis by plain x-ray photograph and computed tomography. RESULTS: Postoperatively, the JOA score improved from an average of 5.5 to 8.5 out of a maximum of 11 points and the mean recovery rate by Hirabayashi method was 54.5%. In all cases, bone union was seen at the hinge side between the opened lamina and the lateral mass. Neither restenosis of the opened lamina nor marked progression of kyphosis were seen on the final follow-up observation in any patient. There was no postoperative spinal cord injury. CONCLUSIONS: Open door laminoplasty is a useful procedure for OPLL of the thoracic spine. This method enables wide-range posterior decompression, especially for the continuous type OPLL extending from the cervical spine to the thoracic spine, even if the apex of the thoracic kyphosis is included.     

Unusual presentation of osteoblastoma in a patient with idiopathic scoliosis after posterior spinal fusion

BACKGROUND CONTEXT: Few studies have described the diagnosis of osteoblastoma of the spine as a cause of scoliosis. These reports have described the tumor in conjunction with initial presentation of painful scoliosis. This case report presents a case of osteoblastoma 9 years removed from diagnosis and fusion of idiopathic scoliosis in the thoracic spine. PURPOSE: To report the late presentation of an osteoblastoma of the thoracic spine 9 years after posterior spinal fusion for scoliosis. STUDY DESIGN: Case report. METHODS: A 25-year-old man presented with thoracolumbar back pain and progressive neurological deficit 9 years after posterior spinal fusion for idiopathic scoliosis. Magnetic resonance imaging of the thoracic spine indicated the presence of a mass in the spinal canal causing cord compression. The patient underwent decompression with resection of the mass which was found to be an aggressive osteoblastoma. RESULTS: The patient enjoyed a full neurological recovery and has subsequently developed a recurrence at 13 months. CONCLUSIONS: We present osteoblastoma as a possible cause of low back pain and neurological deficit postfusion that should be considered in a differential diagnosis.     

Transdiaphragmatic amyloidoma

The term "amyloidoma" has been used to describe localized pulmonary nodular amyloidosis when it is a solitary lesion. Amyloidoma is an uncommon and infrequently reported cause of benign pulmonary lesions. We report the case of a 45-year-old man with hemoptysis, eosinophilia, and a large mass involving both lobes of the left lung, the chest wall, and, via extension through the diaphragm, the liver. Clinical suspicion of echinococcal cyst led to treatment via en bloc excision rather than attempting tissue biopsy for diagnosis. Complete resection of the isolated pulmonary amyloidoma was achieved with no evidence of recurrence.     

Amyloidoma in the gasserian ganglion: case report

BACKGROUND: Amyloidoma in the central nervous system is extremely rare. We describe a rare case of amyloidoma in the gasserian ganglion manifesting as trigeminal neuropathy. METHODS: A 41-year-old woman was admitted to our hospital with progressive numbness and hypalgesia in the distribution of the second and third divisions of the left trigeminal nerve. There was no evidence of chronic inflammatory disorder or immunological abnormalities. Magnetic resonance images showed a mass in the left Meckel's cave that was brightly enhanced with gadolinium. RESULTS: A reddish, firm mass was successfully removed via a left temporal craniotomy. Histologically, the tumor was composed of larger acellular deposits of eosinophilic material. The acellular deposits were positive for potassium permanganate-resistant Congo red staining, showing apple-green birefringence under polarized light and expression of immunoglobulin lambda light chain-derived proteins (A lambda) immunohistochemically. CONCLUSION: The present case revealed an A lambda amyloidoma in the left gasserian ganglion. Although the incidence is rare, amyloidoma should be suspected in patients who complain of progressive trigeminal neuropathies and show an enhanced lesion in the gasserian ganglion on MR images.     

A plate-rod device for treatment of cervicothoracic disorders: comparison of mechanical testing with established cervical spine in vitro load testing data

Posterior cervical internal fixation has long been accomplished using wires, hooks, and rods. More recently, the cervical lateral mass screw and plate or rod systems have been used effectively in unstable lower cervical spine disorders. Each form of fixation has its advantages and disadvantages. Interspinous wiring and lateral mass screw placement obviate canal penetration in the cervical region but are associated with a potential neurologic risk as a result of canal encroachment. Minor canal intrusion by laminar hooks in the thoracic spine pose a lesser neurologic risk than in the cervical region. To exploit the benefits and safety features of spinal instrumentation, a combination plate rod construct (PRC) has been developed that obviates canal penetration in the cervical region by way of lateral mass and cervical pedicle screw fixation and hooks or wires in the thoracic spine. A biomechanical analysis of the PRC device was performed and compared with the in vivo maximal load data of the cervical spine and established maximal load data of the Roy-Camille posterior cervical fixation system. The PRC has greater strength and resistance to failure than is necessary to sustain maximal in vivo cervical spine loads, and it has also compared favorably with the parameters of the Roy-Camille system. The PRC device, or variations on it, is an excellent option for spinal fixation across the cervicothoracic junction because of its superior biomechanical qualities and versatility in stabilizing a complex anatomic junction of the spine.     

Dumbbell hydatid cyst of the spine: case report and review of the literature

STUDY DESIGN: A case report of a hydatid cyst in the retropleural space at T7-T8 with an extension into the spinal canal through an intervertebral foramen complicated by paraparesis due to thoracic cord compression. OBJECTIVE: To confirm that hydatid cyst should be considered in the differential diagnosis for any mass discovered in the human body. SUMMARY OF BACKGROUND DATA: Appearance of a dumbbell-shaped mass in the thoracic spine is highly suggestive of neurofibroma. Hydatid cyst, although very rare, may have the same appearance. The spine is involved in 50% of hydatid disease of the bone. A hydatid cyst of the bone and muscle is always primary. Neurologic recovery is possible because it is a slow-growing lesion. METHOD: Clinical symptoms, differential diagnosis, and treatment are reviewed. The patient was treated successfully by total surgical excision of the lesion through a posterior retropleural approach. No medical treatment was prescribed. RESULTS: Surgical excision is effective in the treatment of hydatid disease of the spine, particularly if there is no extensive bony destruction and it is possible to achieve neurologic recovery after decompression of the thoracic spine. CONCLUSION: Extra spinal hydatidosis may lead to spinal cord compression by extension through the intervertebral foramen. The possibility of cure is high when there is no bony involvement.     

Dextrocardia and coronal alignment of thoracic curve: a population study

The objective of this study was to evaluate the coronal alignment of the thoracic spine in persons with dextrocardia. Generally, the thoracic spine is slightly curved to the right. It has been suggested that the curve could be triggered by pulsation forces from the descending aorta. Since no population study has focused on the alignment of the thoracic spine in persons with situs inversus, dextrocardia, and right-sided descending aorta, we compared the radiographs of the thoracic spine in persons with dextrocardia to those having normal levocardia. Among 57,440 persons in a health survey, 11 cases of dextrocardia were identified through standard radiological screening. The miniature chest radiographs of eight persons were eligible for the present study. The study was carried out as a nested case–control study. Four individually matched (age, gender, and municipality) controls with levocardia were chosen for each case. Coronal alignment of the thoracic spine was analyzed without knowledge of whether the person had levo- or dextrocardia. A mild convexity to the left was found in all persons with dextrocardia and right-sided descending aorta (mean Cobb angle 6.6° to the left, SD 2.9). Of the 32 normal levocardia persons, 29 displayed a convexity to the right, and the remaining three had a straight spine (mean Cobb angle 5.2° to the right, SD 2.3). The difference (mean 11.8°, SD 3.5) differed significantly from unity (P = 0.00003). In conclusion, it seems that a slight left convexity of the thoracic spine is frequent in dextrocardia. We assume that the effect of the repetitive pulsatile pressure of the descending thoracic aorta, and the mass effect of the heart may cause the direction of the convexity to develop opposite to the side of the aortic arch.     

Subaxial cervical and cervicothoracic fixation techniques--indications, techniques, and outcomes

The subaxial and cervicothoracic junction is a relatively difficult area for spine surgeons to navigate. Because of different transitional stressors at the junction of the smaller cervical vertebrae and the larger thoracic segments, proximity to neurovascular structures, and complex anatomy, extreme care and precision must be assumed during fixation in these regions. Lateral mass screws, pedicle screws, and translaminar screws are currently the standard of choice in the subaxial cervical and upper thoracic spine. This article addresses the relevant surgical anatomy, pitfalls, and pearls associated with each of these fixation techniques.     

A splenic cyst causing a viscerosomatic reflex in the thoracic spine. A case report

IntroductionMusculoskeletal pain in the thoracic spine is a common complaint and often seen in the osteopathic clinic. An underlying cause may be pain originating from an internal organ referring to the segmental related somatic structures, i.e. a viscerosomatic reflex. A splenic cyst causing a viscerosomatic reflex in the thoracic spine is a rare but serious differential diagnosis for thoracic musculoskeletal pain that may be identified during routine osteopathic assessment and treatment.Case presentationA 36-year-old women was referred for osteopathy with pain in the thoracic spine as the main complaint. The pain was aggravated by twisting motions, prolonged standing and sitting. The physical examination identified hyperalgesia, hypertonic paraspinal muscles with trophic changes at T6-10 and hypomobility at T6-8. On palpation the diaphragm muscle was hypertonic. In the abdomen, a palpable mass was found in the left upper quadrant, which was suspected to be an enlarged spleen. A normal size spleen is not palpable; therefore, the patient was referred for further investigations. An X-ray and CT scan identified a splenic cyst of approximately 10 cm which was removed with a splenectomy as advised, leading to resolution of the thoracic pain.ConclusionA splenic cyst mimicking mechanical pain in the thoracic spine is rare; however, this case report highlights the importance of comprehensive history taking and screening of red flags, combined with careful physical examination of both musculoskeletal and visceral structures.     

Unifocal amyloidosis: a rare cause of spinal cord compression

A 75-year-old man presented with a 3-month history of progressive paraparesis due to an extradural mass causing cord compression at the T7 level of the thoracic spine. He underwent decompressive surgery, and later vertebrectomy and cage fixation. Histologically, the lesion was a localized mass of amyloid associated with a clonal plasma cell infiltrate. Localized 'amyloidomas' of the spine are rare, evolve slowly and often have a good prognosis following surgery.     

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.