Central nervous system complications by malignant lymphomas: radiation schedule and treatment results

During a 10-year period, 28 patients with spinal cord compression due to epidural malignant lymphoma and 47 patients with cerebral involvement of lymphoma were treated with radiation at our institution. Fifty-four percent of the patients with spinal cord compression had this complication at the time of initial presentation of the disease, whereas only 4% with cerebral involvement presented with CNS symptoms. Only one patient had primary lymphoma solely located in the brain. Characteristically, a majority of the patients with spinal cord compression complained of back pain several months before developing neurological symptoms. Because only one-third of the patients had positive spine roentgenograms at the time of spinal cord compression, a CT scan is suggested in patients with malignant lymphoma suffering from back pain in order to verify a paraspinal lymphoma. Thus spinal cord compression may be avoided by early diagnosis and treatment. Among the patients with spinal cord compression, Hodgkin's and non-Hodgkin's histology were equally represented, whereas only 6% had Hodgkin's lymphoma among the patients with cerebral involvement of lymphoma. The response to treatment defined as improvement in neurological deficit in the patients with spinal cord compression was approximately 90% in both the Hodgkin's and the non-Hodgkin's group. No difference in response was found among patients who had laminectomy compared to patients who did not. Patients receiving high dose, short-term treatment (5 Gy X 5-6) responded equally to patients receiving low dose, long-term treatment (2 Gy X 18-20). The median survival from initiation of radiation therapy in patients developing spinal cord compression or cerebral involvement during relapse was 30 months. In patients with spinal cord compression at initial presentation of the disease, median survival had not been reached after 5 years. Among patients with cerebral involvement 50% had improvement of neurological symptoms with no difference between patients receiving high dose, short-term and patients receiving low dose, long-term treatment. It is concluded that high dose, short-term irradiation is as effective as low dose treatment. Especially in patients with neurological complications at relapse, this treatment schedule is preferred because of the extremely short survival of these patients.     

Spinal cord compression in small cell lung cancer: a retrospective study of 610 patients

The records of 610 consecutive patients with small cell lung cancer, treated on a common protocol in a multicentre trial, were reviewed and 24 (4%) cases of spinal cord compression identified. Five hundred patients had isotope bone scans performed at presentation, and in 131 (26%) there was abnormal isotope uptake in the spinal column; only 7% of these patients developed spinal cord compression. However, of the 24 patients who presented with back pain and had a positive bone scan affecting the spine, 36% developed cord compression. Cerebral metastases occurred at some stage in 19.5% of all patients and in 45% of patients with cord compression. The combination of cerebral metastases and a positive bone scan gave a 25% chance of developing spinal cord compression. There were two distinct forms of clinical presentation. Six patients (group A) presented with cord compression: All had back pain and positive bone scans, five out of six had sphincter disturbance, and median survival from cord compression was 30 weeks. Eighteen patients (group B) developed cord compression while on treatment: 28% had positive initial bone scans, 44% back pain and 61% sphincter disturbance, and median survival from cord compression was 4 weeks. Spinal cord compression is an important cause of morbidity and mortality in small cell lung cancer. We suggest that it may be possible to select patients who should receive radiotherapy to the spine to try to prevent the development of this complication.     

Presenting symptoms of neoplastic spinal cord compression

The cases of 28 patients with neoplastic spinal cord compression were reviewed. The most common presenting symptoms were: back pain (68%), bilateral leg weakness (61%), urinary retention (36%), and bilateral leg numbness (32%). Twelve patients (43%) had known neoplastic disease prior to diagnosis of spinal cord compression. Only two patients (7%) were diagnosed within one week of the onset of major spinal symptoms. The commonest symptoms associated with delay in diagnosis were again back pain (50%) and bilateral leg weakness (38%). However, when certain symptoms were present, diagnosis was almost always delayed, particularly with unilateral leg weakness or pain (100%), ataxic gait (80%), and back pain (68%). Symptoms in the neck, chest, and arms were also always associated with delayed diagnosis.     

Management of metastatic spinal cord compression

Metastatic spinal cord compression, diagnosed in 3-7% of cancer patients, is one of the most dreaded complications of metastatic cancer. It is an oncologic emergency, which must be diagnosed early and treated promptly to achieve the best results and avoid progressive pain, paralysis, sensory loss and sphincter incontinence. Patients who are ambulatory at the time of the diagnosis have a higher probability of obtaining good response to treatment and a longer survival. In clinical practice, back pain accompanies metastatic spinal cord compression in most cases, even in patients with no neurologic deficits. Magnetic resonance imaging is the best tool for diagnosing metastatic spinal cord compression and is able to identify spinal cord compression in 32-35% patients with back pain, bone metastases and normal neurologic examination. Moreover, magnetic resonance imaging gives the extension of the lesion, can diagnose other unsuspected clinical metastatic spinal cord compression sites, and is useful for the radiation oncologist in defining the target volume. Radiotherapy is the treatment of choice in most cases, whereas surgery is advised only in selected patients (ie, if stabilization is necessary, if radiotherapy has already been given in the same area, when vertebral body collapse causes bone impingement on the cord or nerve roots, when there are diagnostic doubts, or when computed tomography-guided percutaneous vertebral biopsy cannot be performed). Laminectomy should be abandoned in favor of more aggressive surgery (ie, posterior, anterior, and/or lateral approach, tumor mass resection, and stabilization of the spine). Generally, radiotherapy must be administered 7-10 days after surgery. The optimal radiation schedule has not been defined. However, as recently suggested by some clinical trials, even the hypofractionated radiotherapy regimens are effective and can be used without increasing radiation-induced myelopathy. Moderate doses of dexamethasone should be used in the early phases of therapy. After radiotherapy, spinal recurrence is generally found in sites different from the first compression area. A close post-treatment follow-up is suggested using clinical parameters (pain, motor and sphincter function), and magnetic resonance imaging should be performed only when a second metastatic spinal cord compression and/or myelopathy are clinically suspected.     

Malignant spinal cord compression in cancer patients may be mimicked by a primary spinal cord tumour

Although it is quite rare, second primary neoplasms in cancer patients may present with the signs and symptoms of malignant spinal cord compression. Primary spinal cord tumours in the cancer patients may be deceptive and considered as the recurrent first cancer. Therefore, it should be precisely differentiated and appropriately managed. We report such a case of intramedullary ependymoma of the cervical spinal cord mimicking metatstatic recurrent lymphoma and causing cord compression. A 50-year-old man developed intramedullary ependymoma of the cervical spinal cord 1.5 years following chemoradiation for Waldeyer's ring lymphoma. He presented with a 2-month history of neck pain, progressive upper- and lower-extremity numbness and weakness, and bowel and bladder dysfunction. Magnetic resonance imaging revealed an intramedullary expansive lesion extending from C4 to C6 levels of the cervical spinal cord. The clinical and radiological findings were suggestive of malignant process. A comprehensive investigation failed to detect another site of disease. He underwent operation, and the tumour was subtotally resected. The patient's neurological deficits improved subsequently. The development of the intramedullary ependymoma following treating lymphoma has not been reported. We describe the clinical, radiological and pathological findings of this case and review the literature.     

Systematic review of the diagnosis and management of malignant extradural spinal cord compression: the Cancer Care Ontario Practice Guidelines Initiative's Neuro-Oncology Disease Site Group.

PURPOSE: This systematic review describes the diagnosis and management of adult patients with a suspected or confirmed diagnosis of extradural malignant spinal cord compression (MSCC). METHODS: MEDLINE, CANCERLIT, and the Cochrane Library databases were searched to January 2004 using the following terms: spinal cord compression, nerve compression syndromes, spinal cord neoplasms, clinical trial, meta-analysis, and systematic review. RESULTS: Symptoms for MSCC include sensory changes, autonomic dysfunction, and back pain; however, back pain was not predictive of MSCC. The sensitivity and specificity for magnetic resonance imaging (MRI) range from 0.44 to 0.93 and 0.90 to 0.98, respectively, in the diagnosis of MSCC. The sensitivity and specificity for myelography range from 0.71 to 0.97 and 0.88 to 1.00, respectively. A randomized study detected higher ambulation rates in patients with MSCC who received high-dose dexamethasone before radiotherapy (RT) compared with patients who did not receive corticosteroids before RT (81% v 63% at 3 months, respectively; P = .046). There is no direct evidence that supports or refutes the type of surgery patients should have for the treatment of MSCC, whether surgical salvage should be attempted if patient is progressing on or shortly after RT, and whether patients with spinal instability should be treated with surgery. CONCLUSION: Patients with symptoms of MSCC should be managed to minimize treatment delay. MRI is the preferred imaging technique. Treatment for patients with MSCC should consider pretreatment ambulatory status, comorbidities, technical surgical factors, the presence of bony compression and spinal instability, potential surgical complications, potential RT reactions, and patient preferences.     

Frequency, diagnosis, and prognosis of spinal cord compression in small cell bronchogenic carcinoma. A review of 817 consecutive patients

A retrospective review of 817 consecutive patients with small cell bronchogenic carcinoma disclosed 29 cases of spinal cord compression (frequency, 3.5%). Twelve patients (group 1) presented with symptom of cord compression, whereas the remaining 17 patients (group 2) developed this complication during therapy. Clinical features differed in the two groups: pain was present in 83% of the patients in group 1 and in 47% of those in group 2. Pain preceded motor dysfunction in 75% and 12% of the two groups, respectively. Radiologic bone destruction of the spine was found in 11 of 12 patients in group 1 and in 3 of 16 patients in group 2. Myelography was performed in 8 patients in group 1 and in 14 patients from group 2. A complete block was found in seven and none of the patients in each of the two groups, respectively. Treatment with irradiation and/or laminectomy rarely resulted in a significant improvement of preexisting neurologic impairment but it frequently prevented the progression of symptoms, leading to preservation of sphincter control and ambulatory function in eight of nine and seven of eight patients, respectively. Careful evaluation is mandatory for patients presenting with back pain and radiographic evidence of bone destruction, as 11 of 26 such patients were found to have spinal cord compression. It is concluded that spinal cord compression presents in two distinct ways. One with early onset, pain, evidence of bone destruction, and complete myelographic block. The second is characterized by motor impairment with no evidence of bone destruction and only partial myelographic block. Treatment rarely ameliorates symptoms but prevents deterioration, making early diagnosis important.     

Spinales Kompressionssyndrom

Acute spinal cord compression syndrome caused by metastases constitutes an oncological emergency. Patients with corresponding symptoms (progressive back pain, spinal cord compression syndrome) should be promptly referred to a center which can offer complete diagnostic tests and has the appropriate departments (neurosurgery, radio-oncology, orthopedics, hematology). On principle, the further course of action (surgical intervention, if necessary followed by postoperative radiotherapy, or immediate radiotherapy alone) should be decided individually for each patient by the interdisciplinary team.     

Spinal Burkitt's lymphoma in adults

Adult Burkitt's lymphoma is an uncommon disease. Few cases of spinal involvement in adults with sporadic Burkitt's lymphoma are reported in the literature. We present a case of a middle-aged man who was found to have an epidural mass in the thoracic spine when investigated for back pain and lower extremity weakness. He underwent a laminectomy with resection of the epidural mass. Histologic examination revealed a primary Burkitt's lymphoma of the spinal cord. He was treated with aggressive chemotherapy and is now experiencing remission of his disease. We also present a review of the literature for the etiology and clinical features of other spinal lymphomas and Burkitt's lymphoma involving the spine in adults.     

硬脊膜外原发性恶性淋巴瘤

本文报告5例原发于脊髓硬膜外腔的恶性淋巴瘤,病人均为男性,年龄20～63岁。肿瘤均发生于胸段。发病时,病人多有类似感冒症状或背腰部疼痛等征,继而下肢麻木及瘫痪等。脊椎X线摄片和椎管碘油造影检查有助于某些椎管内疾患的鉴别诊断,但确诊需依赖病理检查。治疗可采用广泛切除肿瘤及术后补加放疗或化疗。     

Intramedullary spinal cord metastases of melanoma

Intramedullary spinal cord metastases (ISCMs) are extremely rare. An exact diagnosis may be difficult even when the primary tumour is known. Patients usually present with back pain and signs and symptoms of spinal cord compression, such as hemiparesis or hemisensory impairments. Magnetic resonance imaging (MRI) is considered to be the main diagnostic tool for intramedullary lesions as it is very sensitive, but non-specific, in distinguishing between ISCMs and primary cord tumours. Optimal treatment in patients with ISCMs remains controversial. We report a case of ISCMs of melanoma, with a review of the clinical and radiological characteristics of these medullary lesions and their prognosis, as well as the different therapeutic approaches.     

Spinal cord compression in malignant lymphoma. Treatment and results.

The natural history, treatment, and results of 73 spinal cord compressions caused by malignant lymphomas are analyzed. It is found that the spinal cord compression caused by malignant lymphomas is generally a late manifestation of the illness, although primary or early involvements are occasionally seen. In our study, reticulum cell sarcoma is the most frequently variety followed by Hodgkin's disease and lymphosarcoma. The dorsal spinal cord is the most frequently involved segment and pain, weakness, and paresthesia are cardinal symptoms. Radiation treatment delivered in the early phase of the compression is commonly successful in reversing the neurologic symptoms and a dose above 2500 rads appears to be optimal for local control of disease. The low incidence of cerivcal cord compression in Hodgkin's disease patients may be related to frequent manifestation and irradiation of the neck nodes in these patients. Early detection of disease in the deep seated areas along the spinal cord and irradiation of these areas may prevent progression of tumor to the epidural space.     

Spinal cord compression in prostate cancer

Approximately 200,000 men will be diagnosed with prostate cancer in 1994. While localized disease is potentially curable with surgery or radiation therapy, metastatic disease is incurable. The most frequent site of metastasis is bone. Spinal cord compression occurs in approximately 7% of men with prostate cancer. Back pain often heralds the diagnosis of spinal cord compression. In prostate cancer patients with back pain or signs of myelopathy or radiculopathy, plain radiographs of the spine and magnetic resonance imaging should be performed. Early diagnosis is of utmost importance. The neurologic status prior to treatment is the major determinant influencing outcome. Following diagnosis, corticosteroid therapy should begin immediately. Hormonal therapy should be instituted in those patients who have not previously undergone hormonal manipulation. The standard approach to definitive therapy is radiation. Surgical decompression plays a role in patients with severe myelopathy, spinal instability, and in those patients whose neurologic status deteriorates during or after radiation therapy.     

Non-Hodgkin's lymphoma presenting with spinal epidural involvement

Cord compression was noted at presentation in 10 of 453 (2.2%) previously untreated non-Hodgkin's lymphoma patients seen at the Northern Israel Oncology Center between 1968 and 1983. A prodromal phase of local back pain occurred in eight patients, persisting up to 1 year, followed by a second phase of rapidly progressive signs of cord compression. Five of the ten patients presented with primary spinal epidural involvement (Stage IE), whereas the others had Stage IIE and IIIE (one patient each) and Stage IV, with bone and bone marrow involvement (three patients). All patients had unfavorable histologic diagnoses, mostly of the intermediate grade malignancy types according to the Working Formulation. The patients were treated by radiotherapy (two patients), chemotherapy (three patients), or both modalities (five patients). Seven of the ten patients achieved complete remission, but four of them have subsequently had relapses (two patients in bone, one in central nervous system, and one in mediastinum). The 5-year actuarial survival and 3-year relapse-free survival were 66% and 32%, respectively. Median survival has not been reached after a mean follow-up of 34 months. Non-Hodgkin's lymphoma with spinal epidural involvement at presentation is an aggressive disease. An intensive treatment combining irradiation with chemotherapy, and surgery as needed, is suggested in order to achieve good local response and long-term survival.     

Spinal cord compression in breast cancer

Myelography was performed on 78 patients with breast cancer who had signs or symptoms compatible with spinal cord compression. Of 42 patients (54%) with extradural defects, 21 (50%) had a complete block. All patients with positive myelograms (M+) had a positive bone scan and 41 of 42 (97%) had positive skeletal x-rays. Except for paraplegia, paraparesis, or a sensory level abnormality, signs and symptoms were usually not precise enough to accurately predict patients with cord lesions; however, back pain, paresthesias, and bladder or bowel dysfunction were significantly more common in M+ patients. Cerebrospinal fluid (CSF) protein was elevated in almost all M+ patients but also in approximately half of the M- group. Cytology and glucose analysis of CSF were not of value in predicting cord involvement. Response to treatment was better for patients with fewer sites of metastatic disease and a shorter time from diagnosis to treatment. There was no notable difference in survival between M+ and M- patients. Myelography remains the most precise tool for diagnosing spinal cord lesions. Unfortunately, the prognosis of patients with metastatic breast cancer is poor regardless of whether spinal cord compression is present.     

Spinal cord compression complicating gynecological malignancy--report of four cases

Five episodes of spinal cord compression (SCC) in four patients with gynecological malignancy are described. Two patients had a recurrent cervical cancer, one had a recurrent uterine sarcoma, and one had a primary ovarian cancer. All presented with back pain, and the site of compression was at the thoracic spine. Three patient were treated with radiotherapy, and one underwent decompression laminectomy followed by radiotherapy. One patient improved, but the other three did not. SCC is an oncologic emergency, which should be borne in mind by all physicians who take care of cancer patients.     

Vertebral collapse with quadraparesis due to metastatic gliobla multiforme: case report and review of the literature

A case is reported of a patient rendered quadraparetic following collapse of a cervical vertebra due to neoplastic invasion by metastatic glioblastoma multiforme. The case is discussed in light of a review of the world literature regarding the clinical incidence and significance of metastasis of glial tumors. It is recommended that all patients with high grade glial tumors who complain of back pain be evaluated with plain radiographs and MRI of the spine or⁹⁹Tc bone scan. The management of pathologic spine fractures from metastatic glial tumors with accompanying spinal instability or spinal cord compression due to intracanalicular bone should aim for immediate surgical decompression and stabilization followed by involved field irradiation.     

Non-Hodgkin's tumor and Pancoast's syndrome

A 60-year old man presented with Horner's syndrome, and acute right hand and lower extremity weakness. Chest X-ray and MRI revealed a right apical lung tumor (presumed to be a primary lung cancer), with brachial plexus infiltration and spinal cord compression. Emergent radiotherapy was initiated for spinal cord compression and a biopsy was obtained 24 h later. A careful review of pathology demonstrated a non-Hodgkin's lymphoma. The patient subsequently received chemotherapy, and is now in remission. This case illustrates the importance of a tissue diagnosis before initiating therapy for a Pancoast's tumor.     

Non-pharmacological interventions in patients with spinal cord compression: a systematic review

Spinal cord compression is a complex and challenging condition that greatly affects the quality of life. Non-pharmacological techniques have only been studied to a very lesser extent; although they are evidence to be beneficial. We performed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) analysis of the scientific literature in several databases (Medline, Cochrane, Scopus, Cuiden, Pubmed, Lilacs and Embase); using the following keywords: spinal cord compression, spine compression, mobilization, positioning, brace and bracing. Eleven studies met the inclusion criteria and were finally included in the systematic review. 3 of them were related to metastatic spinal cord compression, 5 to spinal cord compression due to other causes and the last 3 of them regarded the health professional´s knowledge in oncology patients. In all cases, it seems possible to manage spinal cord compression by using external mobilization and braces and that this treatment is beneficial to patients. Positioning plays a massive role in the disease and can improve or worsen the condition when used improperly; the supine position is overused and can have a negative impact both physically and psychologically. Non-pharmacological interventions could be useful for pain management, cardiovascular alterations and patients’ well-being. One randomized clinical trial demonstrated that massage therapy, using either broad compression massage or light contact touch massage improved pain control. There is an urgent need of randomized clinical trials with these interventions in order to achieve an improved care of these patients.     

A case of primary leptomeningeal gliomatosis confined to the spinal cord

We report a case of primary leptomeningeal gliomatosis limited to the spinal cord occurring in a 52 years-old patient, who presented with back pain and leg weakness. MRI-scan of the craniospinal axis revealed an enhancing cervicothoracic lesion confined to the leptomeninges. A diagnostic biopsy was taken followed by a six level cervicothoracic laminoplasty with the aim of debulking of the dorsal portion of the tumour and relieving cord compression. Two weeks following surgery, the patient developed bilateral arm weakness. Repeat imaging revealed extension of the lesion to the level of the lower medulla. This patient underwent spinal radiotherapy and concomitant chemotherapy with Temozolomide, but she died of pneumonia 8.5 months after the time of diagnosis. No post-mortem was performed. Primary leptomeningeal gliomatosis exclusively involving the spinal cord is an extremely rare condition bearing a dismal prognosis. It can be suspected on neuroimaging, but histopathological examination is required for the final diagnosis. No current treatment protocols are available. Decompressive surgery may have a role in relieving symptoms, but no substantial benefit has been proven in administering radio-chemotherapy.