Malignant peripheral nerve sheath tumor of the cauda equina

Only one case of malignant peripheral nerve sheath tumor (MPNST) affecting the cauda equina region has been reported earlier. A 32-year-old male with congenital multiple subcutaneous swellings presented with low back pain, progressive paraparesis and bladder-bowel dysfunction. Magnetic resonance imaging (MRI) demonstrated a heterogenously enhancing intradural lesion at L2–L4. At operation, on opening the dura, multiple nodular, firm matted masses attached to the lumbosacral nerve roots were encountered. Peripheral lesions were partially excised. Histopathological exam revealed varied cellularity with necrosis and pleomorphic nuclei suggestive of MPNST. MRI features, pathophysiological characteristics and the literature are reviewed. Received: 2 February 2001 / Accepted in revised form: 13 June 2001     

Metastatic malignant peripheral nerve sheath tumor of the cauda equina

Malignant peripheral nerve sheath tumor (MPNST) of the cauda equina is rare, with only four reported patients in the literature. We present a 70-year-old woman with a history of left sciatic nerve MPNST that had been treated by left hemipelvectomy 4 years prior. She then presented with disabling right leg weakness that progressed over 7 months. Spine imaging revealed an intradural extramedullary lesion from L5 to S1 with compression of the cauda equina. She underwent an L3-S1 laminectomy with subtotal resection of the lesion, which was diagnosed as a metastatic MPNST. At the 3-month postoperative visit, her lower extremity strength had returned to baseline. The only notable postoperative complication was a superficial wound infection that resolved with antibiotic treatment and wet-to-dry dressing changes. Thus, although uncommon, the spinal canal and cauda equina should be considered as possible locations for metastatic MPNST in the appropriate clinical scenario. To our knowledge, this is the first report of a patient with a metastatic MPNST to the cauda equina.     

Malignant peripheral nerve sheath tumor of the cauda equina with craniospinal metastasis

Intradural spinal malignant peripheral nerve sheath tumors (MPNST) are extremely rare, with only 20 adult patients reported to our knowledge, and only four primary tumors arising from the cauda equina. A 49-year-old man presented with back pain, constipation, and lower extremity weakness and was found to have a large intradural lesion involving the cauda equina. Imaging of the rest of his neuraxis revealed additional small left temporal lobe, cervical, and thoracic lesions. The patient underwent laminectomy for tumor debulking and biopsy, as gross total resection was not possible due to envelopment of the cauda equina. Histopathology revealed a MPNST with high cellularity, elevated proliferative indices, and nerve fascicle invasion. After the debulking, the patient reported improvement in his symptoms. However, 6 weeks later, the patient began having severe headaches, and his left temporal lobe lesion was found to have increased significantly in size, requiring craniotomy for palliative resection. The authors report the first adult patient with sporadic spinal MPNST with craniospinal metastasis to our knowledge. Imaging of the entire neuraxis is recommended for initial workup of these lesions, which are capable of intradural spread. The best treatment approach is unclear, but total surgical resection should be attempted, barring infiltration and engulfment of the nerve roots or widespread dissemination.     

Primitive neuroectodermal tumor of the cauda equina. Report of a case

Primitive neuroectodermal tumors are malignant neoplasms whose classification has been controversial. Spinal primitive neuroectodermal tumors are rare and at the cauda equina are even less common. We report the case of a 40 year-old man with a history of progressive back pain and gait difficulty. After the diagnosis of a primitive neuroectodermal tumor of the cauda equina, the patient presented seeding to the intracranial subarachnoid space followed by a poor outcome. We review the literature of primary spinal primitive neuroectodermal tumors.     

Paraganglioma of the cauda equina: a case report.

This case report describes a paraganglioma of the cauda equina in a 63 year old woman. Very few examples of paraganglioma have been recorded in this site. Histologically these neoplasms may have considerable similarity with ependymoma, the most common neoplasm of the lower spinal cord, and the diagnosis can be easily missed unless special techniques are employed. The clinical and pathological data obtained from this and the other reported examples suggests that paragangliomas of the cauda equina are benign, slowly growing neoplasms. In contrast to ependymomas and to paragangliomas elsewhere, they are well circumscribed, amenable to complete resection and have an excellent prognosis.     

Selective hypertrophy of the cauda equina nerve roots

Two patients are described with a previously unreported hypertrophic radiculopathy of the cauda equina with preservation of peripheral nerve function. In one removal of an associated bronchial carcinoid tumour led to marked improvement in the neurological condition suggesting a paraneoplastic cause. The second patient later developed an external ophthalmoplegia but the underlying aetiology remains obscure. Received: 19 February 2001, Received in revised form: 25 July 2001, Accepted: 2 August 2001     

Paraganglioma of cauda equina: a case report

Histopathologically and immunologically confirmed case of paraganglioma of cauda equina region is described.     

Sclerosing fibrous tumor of the cauda equina: a fibroblastic variant of peripheral nerve tumors?

Summary The case of a 43-year-old man with an unusual mesenchymal tumor of the cauda equina is presented. A well-circumscribed firm tumor was found in the lower spinal canal at L1 level. Although a nerve root was involved, the adjacent dura mater or filum terminale was unrelated to the tumor. Microscopically, the tumor was rich in collagen and made up of irregularly intertwining fascicles of fibroblastic spindle cells lacking in nuclear atypia or mitotic activity. Partial broad hyalinization of collagen was another histological feature of the tumor. Histological and immunohistochemical studies failed to reveal any findings that suggested known fibrous neoplasms, such as schwannoma, neurofibroma and meningioma, originating in the nervous system. Ultrastructural features of the tumor cells were consistent with those of fibroblasts. Hence, the present tumor is regarded as a unique pure fibroblastic tumor (fibroma) derived from the interstitium of a nerve root in the cauda equina.     

Discrimination of a nerve fiber that is the origin of a cauda equina tumor using acetylcholinesterase staining

Spinal nerve sheath tumors are well known to typically originate from dorsal sensory nerve roots. However, it is difficult to anatomically identify the origin in the case of cauda equina tumors. In this study, we aimed to ascertain whether a cauda equina nerve root removed with a nerve sheath tumor was a motor nerve using acetylcholinesterase (AchE) staining. Nerve rootlet sections removed with tumors were stained for AchE using the AchE Rapid Staining Kit. Additionally, we performed intraoperative motor‐evoked potential (MEP) monitoring following either transcranial electrical stimulation (TES) or electrical stimulation of nerve rootlets. The muscular strength of the lower extremities was assessed bilaterally before and after surgery using manual muscle testing. An AchE‐positive motor nerve rootlet that was the origin of a cauda equina tumor was observed in one of the 12 patients. In this patient, a MEP in the right quadriceps evoked by electrical stimulation of this rootlet was detected. TES‐MEP showed a 30% decrease in the amplitude in the right quadriceps evoked after tumor resection with this nerve rootlet. However, the motor strength in both lower extremities did not change after surgery. AchE staining and intraoperative MEP monitoring could detect the motor nerve rootlet that was the origin of a cauda equina tumor. Nerve sheath tumors originating from the motor nerve might be rare even in cauda equina.     

Enterogenous cyst of the cauda equina. A case report and a review of the literature

The clinico-pathological findings in a 38-year-old man with a progressive cauda equina syndrome and with a dorsal dermal fistula in the lower back are presented. The X-rays of the spine revealed fusion of the vertebrae L4-L5 and L5-S1, and the myelogram showed an intradural tumour, extending upto the level of L2. An enterogenous cyst, adherent to the right third lumbar radix was completely removed. The clinical and pathological data are compared to previous published cases and discussed in relation to the origin of the tumour.     

Chemodectoma of the cauda equina: case report

Well-differentiated paragangliomas have been found in unusual sites where conspicuous paraganglions are not normally found. A patient with a chemodectoma of the cauda equina is described. The importance of differentiating such tumors from those far more commonly seen in the region of the cauda equina, namely, ependymomas, hemangioblastomas, and metastatic carcinomas is emphasised.     

Tumours of the cauda equina.

A retrospective study of 70 consecutive patients with a cauda equina tumour who were admitted to Neurosurgical Department at the Radcliffe Infirmary, Oxford is presented. The diagnosis of these tumours is often difficult and delayed. The quality of life largely depends upon the neurological disability at presentation. The diagnostic features and investigations are discussed together with the treatment and prognosis.     

Features of intracranial hypertension as a manifestation of cauda equina tumor

A 61-year-old man was admitted to the Department of Neurology, Medical University of Bia?ystok with signs of intracranial hypertension associated with headaches and papilledema, with no other signs of the nervous system dysfunction. CT and MRI brain scans were normal, cerebrospinal fluid contained 1620 mg/dl of proteins. One month later epileptic seizures with a loss of consciousness occurred. Brain CT and MRI scans showed no focal pathology and failed to disclose any intracranial tumor. The acute pain in the lumbar spine region made us perform an MRI scan of the lower spinal cord, which disclosed cauda equina tumor. The tumor was surgically removed and it was histopathologically concluded to be ependymoma. After the operation there were neither headaches nor seizures and we noticed an improvement in his vision acuity. In the postoperative course the patient suffered from urinary and bowel dysfunction. In this article we discuss a pathogenesis of intracranial hypertension occurring in spinal cord tumors and stress the need for a diagnosis of spinal cord lesions in patients with the elevated intracranial pressure.