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.     

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.     

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     

Trigeminal neuralgia and chiropractic care: a case report

The following case describes a 68 year-old woman with a 7½ year history of worsening head and neck pain diagnosed as trigeminal neuralgia following surgical resection of a brain tumor. After years of unsuccessful management with medication and physical therapies, a therapeutic trial of chiropractic was carried out. Chiropractic care included ultrasound, manual therapies (manipulation and mobilization), soft tissue therapies, and home stretching exercises. After an initial treatment period followed by 18 months of supportive care the patient reported satisfactory improvement. It became evident that there were at least three sources of her symptoms: mechanical and/or degenerative neck pain, temporomandibular joint syndrome, and trigeminal neuralgia. While never completely pain-free, the patient continued to report that her pains reduced to minimal at times. At the most recent follow-up, the pain had not returned to pre-treatment intractable levels. This case study demonstrates the importance of diagnosing and treating multiple sources of pain and the positive role chiropractic care can have in the management of patients with these clinical conditions. The potential for convergence of sensory input from the upper three cervical segments and the trigeminal nerve via the trigeminocervical nucleus is discussed.     

Cerebral blood flow changes induced by electrical stimulation of the Gasserian ganglion after experimentally induced subarachnoid haemorrhage in pigs

Summary The effect of trigeminal electrical stimulation on cerebral blood flow has been studied in conditions of normal or reduced cerebral blood flow (CBF).Autologous blood was injected into the subarachnoid space of ten Pittmann-Moore pigs to induce subarachnoid haemorrhage (SAH) accompanied by cerebral blood flow (CBF) reduction. One week later, in six of ten animals, a considerable decrease of CBF was noted as evaluated by means of a recording-system monitoring over the right parieto-temporal calvarium the washout of¹³³ Xenon injected into the internal carotid artery after the external carotid had been clamped. Continuous electrical stimulation of the Gasserian ganglion performed in the six animals with severely induced CBF reduction produced a remarkable cerebrovascular dilation and increase of CBF lasting over 3 h.Electrical stimulation of the Gasserian ganglion produced a similar pattern of vasodilation in six pigs in which no blood was injected and no reduction of CBF was evident.The mechanisms and the anatomical pathways which underlie these results are discussed.     

Frontotemporal epidural approach to trigeminal neurinomas

Summary From 1980 through 1993 an series of 44 patients with trigeminal neurinomas were treated. Five of them were operated on for the remainder of the tumour as they had undergone previous surgery elsewhere, 35 were operated on for the first time, and 4 were not operated on for various reasons.An epidural approach to the neurinomas originating in the branches of the Vth nerve peripheraly to the Gasserian ganglion (GG) was used. In the neurinomas originating in the GG or in the root of the Vth nerve, either an epidural-transdural approach or an epidural-transdural-transpetrous approach was used.All tumours operated on using the approach described in this article were completely removed. In 10 patients, the Vth nerve sensory deficits increased in comparison with preoperative deficits; in 9 their state remained unaltered; and in 11 the sensory function of the Vth nerve improved. In those patients who had experienced pre-operative atypical trigeminal pain, the pain disappeared after surgery.There was no additional treatment: radiosurgery, irradiation or chemotherapy. Histopathological examination did not reveal any malignant changes in the tumours in any of the patients.Based on our own experience and on the published data it is believed tht the best treatment for trigeminal neurinomas is complete microsurgical removal of the lesion.     

Osteochondroma: review of the literature and case report.

Osteochondroma is the most common bone tumor of the foot. The podiatric surgeon should have an organized protocol to diagnose and treat suspicious osseous lesions in order to prevent undertreatment of malignant lesions, or overtreatment of benign lesions. The presence of a solitary osteochondroma is not sufficient indication for surgical removal. Surgical removal is indicated if the tumor produces pain, has radiographic features of malignancy, or shows abnormal increase in size. This case involves an osteochondroma of the fifth digit that was treated by digital amputation.     

Athelia: case report and review of the literature.

The absence of nipple-areola complex is a rare entity and is always associated with other anomalies. This paper described a case of bilateral athelia without other alterations. The atrophy of the dense mesenchyme due to absence of parathyroid hormone-related protein produced in epithelium may lead to nipple involution. Further cases should be studied to corroborate this theory.     

Mesenteric fibromatosis: case report and literature review.

Mesenteric fibromatosis is a rare, benign fibrous lesion found in the bowel mesentery or the retroperitoneum. Its biological behavior is intermediate between benign fibrous tissue proliferation and fibrosarcoma. Fibromatosis characteristically is locally invasive and tends to recur but does not metastasize. Most reported cases have been in older individuals, and there is a frequent association with familial polyposis coli, previous trauma, and hormonal imbalance. The authors report a case of mesenteric fibromatosis in a 32-month-old girl with a 1-month history of abdominal pain who was discovered to have an abdominal mass. After appropriate investigations, the mass was excised. The pathology report confirmed the diagnosis of fibromatosis. Mesenteric fibromatosis in children, as in adults, presents a management challenge for the surgeon.     

Kaposiform haemangioendothelioma: case report and review of the literature.

Kaposiform haemangioendothelioma (KHE) is a rare vascular tumour, predominantly of infancy and early childhood, that has a close association with Kasabach-Merritt syndrome. Despite benign histology, this tumour frequently behaves aggressively, causing significant morbidity and mortality as a result of the compression and invasion of surrounding structures as well as from associated haematological and lymphoproliferative syndromes. There is a need for a high index of suspicion when presented with large, enlarging or abnormal vascular lesions in infancy and, less commonly, in adulthood. An early diagnosis of KHE can lead to prompt treatment, which may be life saving. To date, there have been only four reported cases of KHE occurring in patients over the age of 18 years. We report an otherwise well 26-year-old woman who presented with a KHE of the left thigh, and briefly review the literature. We believe this to be the second reported case of KHE in the UK and the first in an adult patient.     

Trigeminal nerve schwannoma with ancient change. Case report and review of the literature

Ancient change in a schwannoma is a histological variant typically found in longstanding tumors. Histologically, the tumor has biphasic features typical of a schwannoma with evidence of degenerative changes that may complicate diagnosis. The authors report on a 23-year-old man with no features of neurofibromatosis who presented with headaches, blurred vision, and ataxic gait. Magnetic resonance imaging demonstrated a rim-enhancing lesion in the cerebellopontine angle with displacement of brainstem structures and no supratentorial hydrocephalus. Using a lateral suboccipital approach together with image guidance and intraoperative neurophysiological monitoring, a gross-total macroscopic excision was performed. At surgery, the tumor was found to arise from the inferior division of the trigeminal nerve. The final histological diagnosis was schwannoma with ancient change. Note that ancient change in schwannomas is a histological variant thought to result from degenerative changes in longstanding tumors. To the authors' knowledge, this is the first independent report of this histological variant in an intracranial schwannoma.     

Supernumerary nostril: case report and review of the literature.

Supernumerary nostrils are extremely rare. A review of the literature revealed 21 published cases since 1906. We present a case of supernumerary nostril located above and to the side of the left nostril in a male patient. We discuss differences between published reports, surgical techniques proposed, and the outcome of our treatment approach.     

Intramedullary meningioma: case report and review of the literature.

Intramedullary meningioma is a rarely reported clinical entity. As far as we know, only three cases have been reported to date. We describe a further case at the cervical level and review the few published cases.     

Psoas abscess: case report and review of the literature.

Psoas muscle abscess is rare and presents a diagnostic challenge requiring a high index of suspicion. We report an unusual case of primary psoas abscess caused by Proteus mirabilis. Primary psoas abscess is most commonly present in children and is usually caused by Staphylococcus aureus. A total of 434 cases of psoas abscess has been reported. The subject is discussed noting the differences between primary and secondary cases, and emphasizing the importance of ultrasound and computerized tomography guided drainage of psoas abscess. A flow chart for the evaluation and management of primary and secondary abscess is presented, taking into consideration the success rate of the various methods of treatment described in the literature.     

Paracecal hernia: case report and review of the literature.

A case of paracecal hernia successfully treated operatively is described. Review of the literature revealed 145 previously reported cases. Our case is added. Pertinent anatomy predisposing to paracecal hernia is reviewed. Roentgenographic signs of paracecal hernia are presented. Clinical signs and symptoms are discussed.