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.     

Magnetic resonance imaging of a temporal lobe cerebral amyloidoma

Amyloidomas are focal solitary amyloid masses without systemic involvement that have been observed to occur in various body locations. When presenting intracranially, they pose a challenging diagnostic and therapeutic course given their location and rarity. We report a case of a 62-year-old man with a 4-year history of seizure and headaches. Magnetic resonance imaging was initially inconclusive but revealed an ill-defined right temporal lobe lesion. Biopsy later confirmed a cerebral amyloidoma. We also review the current literature on the pathogenesis, imaging findings, prognosis, and treatment of cerebral amyloidomas.     

Soft-tissue amyloidoma of the extremities: a case report and review of literature

Amyloidosis is a heterogeneous group of disorders characterized by extracellular deposition of unique protein fibrils. Amyloidosis may be hereditary or acquired, and the deposits may be focal, localized, or systemic in distribution. The least common presentation of an amyloid deposition is as a discrete mass called amyloidoma or amyloid tumor. Although described at various body sites, soft-tissue amyloidoma in an extremity is exceedingly rare. We report such a case of a large amyloidoma in the thigh, which simulated a soft-tissue sarcoma. In spite of attaining a very large size over a course of more than 20 years, the clinical course and the histology of this lesion were benign. Awareness of this entity will allow this rare diagnosis to be considered, prevent confusion with malignant disease, and allow appropriate management and patient reassurance. A review of literature on soft-tissue amyloidomas of extremities is also being presented.     

DIALYSIS-RELATED CERVICAL AMYLOIDOMA PRESENTING WITH QUADRIPLEGIA

We evaluated the intraplatelet and plasma levels of transforming growth factor β (TGF-β) in patients with or without renal osteodystrophy (ROD) who were undergoing hemodialysis (HD). Intraplatelet and plasma levels of TGF-β were examined before and after HD, and compared with those from healthy controls. Patients undergoing HD had significantly higher mean intraplatelet levels of TGF-β before and after HD than did the healthy subjects (22.7 ±7.8 and 29.5 ±15.8 vs. 18.7 ±7.9 ng/10⁵ platelets; p <.05). The mean intraplatelet and plasma levels of TGF-β in patients after HD were significantly increased than those before HD and in healthy subjects (p <.05). Moreover, patients with ROD showed a significantly higher mean intra-platelet and plasma levels of TGF-β than that without ROD (p <.05). To investigate the effects of TGF-β on ROD in HD patients, we evaluated such parameters as parathyroid hormone (PTH) and alkaline phosphatase (ALP), which reflect the lesions of ROD. The mean intraplatelet level of TGF-β was not correlated with either parameter. Meanwhile, no correlation was observed between the intraplatelet level of TGF-β and the hematocrit (Hct). Similarly, no correlation was observed between the intraplatelet levels of TGF-β and the dose of erythropoietin (EPO)administered. These findings indicate that metabolism of TGF-β occurs during HD and overproduction of TGF-β may play an important role in the pathogenesis of ROD.     

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.     

前纵隔内孤立性淀粉样瘤1例

正患者女,42岁,因"头晕、头痛1天"入院,既往有干燥综合征病史4年余。查体:神清,无明显脱发,牙齿片状脱落,舌干裂,舌苔消失,皮肤遇光变红。CT:平扫示前纵隔肿块,边缘光整,最大截面约8.1cm×4.5cm,其内密度不均,可见点状钙化及大小不等低密度区,CT值40～76HU(图1A),增强扫描病     

Isolated pulmonary amyloidomas: Report of 3 cases with histologic and imaging findings

Amyloid tumors presenting as lung masses are rare. We report 3 patients seen over a 2-year period with multiple lung masses, 2 that were suspicious for metastasis, and one in a patient with chest pain. Pathologic evaluation demonstrated amyloid tumor in each case. Two demonstrated a prominent macrophage giant cell reaction; scattered polyclonal plasma cells were present in two of the cases. PET scanning of 2 of the patients revealed an SUV of 1.9 and 4.0, respectively. Short-term follow-up revealed that none of the 3 cases were associated with lymphoproliferative disorders. This small series and a literature review suggest that pulmonary amyloidomas are usually isolated lesions, and that PET may show increased uptake simulating a neoplasm.     

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.     

Gastric amyloidoma in patient after remission of Non-Hodgkin's Lymphoma

Amyloidosis is commonly systemic, occasionally organ-limited, and rarely a solitary localized mass. The latter, commonly referred to as tumoral amyloidosis, is described as occurring in nearly every organ/tissue. Only a few reports of gastric amyloidosis exist today. We describe a 72 year-old black male from Barbados presenting with 3 d of diffuse abdominal pain. His medical history included Non-Hodgkin's Lymphoma diagnosed five years ago, status-post six rounds of cyclophosphamide, adriamycin, vincristine, prednisone chemotherapy, and currently was in remission. On computed tomography scan of the abdomen, thickening and calcification of the gastric wall was noted along with pneumatosis. On esophagogastroduodenoscopy, a large circumferential friable mass was seen from the gastroesophageal junction to the body. A large non-bleeding 3 cm polyp was also seen in post bulbar area of duodenum. Biopsies were stained with Congo red and gave green birefringence under polarized light, consistent with tumoral amyloidosis. Positron emission tomography scan revealed diffuse gastric mucosa uptake compatible with gastric malignancy without metastatic foci. Treatment for gastric amyloidomas has presently been one of observation or, at most, resection of the amyloid mass. It is not known if our patient required the same approach or if this warranted the re-institution of chemotherapy for Non-Hodgkin's Lymphoma. Until more reports of tumoral amyloidosis are made known, treatment as well as prognosis remain uncertain.