Treatment of spinal cord tumors

Opinion statement  Tumors associated with the spinal cord can have devastating effects on patient function and quality of life. Most of these tumors are from metastatic disease, usually to the epidural space. Less frequently, the tumors are intrinsic to the spinal cord itself (ie, primary tumor). Regardless of the etiology, spinal cord tumors often present first with progressive local or radicular pain, or both. Other symptoms include weakness, sensory changes, or sphincter dysfunction. The timeliness of diagnosis of spinal cord tumors and promptness of treatment are important, as they directly affect outcome. Dexamethasone, a corticosteroid, is used as a temporizing measure to improve or stabilize neurologic function until definitive treatment. For nonambulatory patients with epidural metastatic tumors, surgery followed by radiation therapy maximizes neurologic function and modestly lengthens survival. However, palliative radiotherapy alone is recommended for those with neurologic deficits lasting longer than 48 hours, survival prognosis less than 3 months, inability to tolerate surgery, multiple areas of compression, or radiosensitive tumors. An ambulatory patient with a stable spine should be considered for radiation treatment only. The role of chemotherapy for epidural metastatic tumors is not well established. For intramedullary metastases, the role of surgery and chemo therapy remains controversial and radiation is the mainstay. For low-grade or benign primary spinal cord tumors, resective surgery is of benefit and can be curative. For high-grade tumors, the benefit of resection is less clear, and radiotherapy and/or chemotherapy may be helpful. The use of chemotherapy for primary spinal cord tumors has rarely been assessed. Agents reported in the literature for treatment of spinal cord gliomas include temozolomide, irinotecan, cisplatin, and carboplatin. A multidisciplinary approach is often required to maximize the therapeutic and functional outcome of patients with metastatic and primary spinal cord tumors.     

Intramedullary spinal cord germinoma--2 case reports

BACKGROUND: Primary intramedullary spinal cord germinoma is very rare. We encountered 2 patients with primary intramedullary spinal cord germinoma. We describe herein our comprehensive management system for CNS germinoma, including intramedullary spinal cord germinoma, along with a review of the literature. This is the first report to describe successful application of ICE chemotherapy for intramedullary germinoma. CASE DESCRIPTIONS: A 16-year-old adolescent girl (case 1) experienced lumbago and subsequently noticed gait disturbance that aggravated gradually. On admission, paraparesis and urinary retention were noted. Magnetic resonance imaging demonstrated marked cord swelling between T9 and T12, with slight enhancement in the spinal cord. Astrocytic tumor was initially suspected, and partial removal was performed. However, pathologic examination identified germinoma. Successful treatment with ICE chemotherapy and radiotherapy was implemented, with no evidence of recurrence apparent at 48 months postoperatively. A 34-year-old woman (case 2) presented with paraparesis and sensory disturbance. Magnetic resonance imaging demonstrated cord swelling between T8 and T10, with slight Gd-DTPA enhancement. Because the lesion did not respond to steroid pulse therapy, spinal cord tumor was suspected and biopsy was performed. Pathologic examination verified primary germinoma of the spine. Successful treatment with ICE chemotherapy and radiotherapy was implemented with no exacerbation of neurologic deficits. No evidence of recurrence was apparent at 36 months postoperatively. CONCLUSION: Correct diagnosis of very rare primary intramedullary spinal cord germinoma is important, because these patients can be treated successfully using chemo- and radiotherapy without neurologic deterioration.     

Spinal Cord Compression

OPINION STATEMENT: Malignant epidural spinal cord compression (MESCC) remains a common neuro-oncologic emergency with high associated morbidity. Despite widespread availability of MRI, the diagnosis frequently goes unmade until myelopathy supervenes, which is unfortunate because the strongest predictor of neurologic outcome with treatment is the neurologic status when treatment is initiated. Once the diagnosis of MESCC is suspected, patients with neurologic deficits should be started on high-dose corticosteroids (eg, dexamethasone, 10-100?mg intravenously, followed by 16 to 100?mg/d in divided doses). Definitive therapy of MESCC almost always includes radiation therapy and in some cases surgical intervention; factors considered include the patient's performance status and extent of visceral and skeletal disease, spinal stability, the tumor's underlying radiosensitivity, and the degree of spinal cord compression. Patients with spinal instability or radioresistant tumors are likely to have a much better neurologic outcome with tumor resection and spinal stabilization prior to radiation; the same may also pertain to patients with moderately radiosensitive tumors who have good life expectancy in terms of their systemic tumor. Conventional radiation has historically been beneficial after surgery and in patients who are not surgical candidates. Recent studies suggest a role for stereotactic body radiation therapy in patients with spinal metastasis from radioresistant tumors and in patients who have received prior standard radiotherapy, so long as the spinal cord has been decompressed.     

Intratumoral hemorrhage because of primary spinal mixed germ cell tumor presenting with atypical radiological features in an adult

Background context

Germ cell tumors are known to arise in the central nervous system, usually in the intracranial regions. However, primary spinal mixed germ cell tumors are extremely rare.

Purpose

This is the first reported case of intratumoral hemorrhage because of a primary spinal mixed germ cell tumor consisting of germinoma and immature teratoma in the conus medullaris of an adult patient that presented with rapid changes on magnetic resonance image (MRI). We report this rare case and discuss the clinical manifestations of an intramedullary spinal mixed germ cell tumor in adult.

Study design

A case report.

Methods

A 42-year-old woman experienced buttock numbness, and a spinal cord tumor was observed on the conus medullaris on MRI. The patient was scheduled for an operation in 1 month, but she developed sudden-onset neurologic deterioration. Rapid progression of the tumor was observed on follow-up MRI. The tumor was removed by emergency surgery and was identified as a primary mixed germinoma and immature teratoma.

Results

The patient received adjuvant chemotherapy and radiotherapy after gross total resection. The neurologic deficit of the patient was relieved, and recurrence of the tumor was not observed 26 months after the surgery.

Conclusions

We present this rare case and emphasize the necessity of precise diagnosis and early treatment of primary spinal germ cell tumor. Close observation on MRI is required after surgery, and adjuvant chemotherapy and radiotherapy should be considered according to the pathologic features.     

Primary malignant B-cell-type dural lymphoma: Case report

BACKGROUND: The primary malignant dural lymphoma of B-cell type is rare. Our review of the literature (24 cases) indicated that patients with this tumor had female predominance, immunocompetency, and longer survival compared with those with primary malignant intracerebral lymphomas. Based on its clinicopathological features, this dural lymphoma may be classified differently from other types of malignant lymphomas in the central nervous system. CASE DESCRIPTION: The authors report an example of a patient who had a favorable course of malignant dural lymphoma. A 59-year-old woman presented with primary malignant dural lymphoma in the frontal area. Histological diagnosis was a diffuse large B-cell-type lymphoma of intermediate malignancy, but MIB-1 index was extremely high. Subtotal tumor removal was followed by 4 courses of chemotherapy. Thirty months after surgery, the patient is asymptomatic with no MRI evidence of recurrence. A combination of subtotal resection and chemotherapy for a primary malignant dural lymphoma is an effective means to attempt cure of this tumor. CONCLUSION: These rare tumors may be managed successfully by subtotal resection and postoperative treatment. We believe that chemotherapy is the treatment of choice after subtotal resection of malignant dural lymphomas, as experienced in our case.     

Primary spinal epidural Burkitt's lymphoma

Three Japanese children suffered from symptoms of spinal cord compression, and myelography demonstrated extradural masses compressing the spinal cord at different levels. Decompressive laminectomy revealed epidural tumors which were resected as completely as possible. Histologic pictures of the tumors showed Burkitt's lymphoma, and the diagnosis of primary epidural Burkitt's lymphoma with clinical stage A was made in each case after postoperative examination. Though postoperative radiotherapy and systemic chemotherapy were administered to all patients, they only survived for a short period of time.     

Primary spinal epidural mantle cell lymphoma: case report

OBJECTIVE AND IMPORTANCE: Mantle cell lymphoma is a distinct clinicopathological type of non-Hodgkin's lymphoma that often presents at an advanced stage, with systemic spread. Spinal involvement is uncommon and generally occurs as part of advanced disease or generalized relapses. Primary spinal epidural lymphoma is a rare initial manifestation of non-Hodgkin's lymphoma, and mantle cell lymphoma with initial presentation in the spinal epidural space is extremely rare, having been previously reported in only two cases. CLINICAL PRESENTATION: We report a case of a 71-year-old man who presented with increasing weakness and numbness of the legs. Magnetic resonance imaging revealed a spinal epidural mass in the lumbosacral region. INTERVENTION: The patient underwent a partial L4 and L5-S1 laminectomy, with incomplete resection of the mass for spinal decompression and tissue diagnosis. Mantle cell lymphoma was diagnosed in the pathological examination. CONCLUSION: After radiotherapy, the disease recurred with a soft-tissue mass in the anterior maxillary area of the face. The patient underwent restaging and was treated with chemotherapy, with only a partial response. Mantle cell lymphoma with primary spinal epidural presentation is rare. This diagnosis can be established and other causes of spinal cord compression can be ruled out by obtaining tissue for proper histopathological examinations. Because of its aggressive behavior and poor prognosis, mantle cell lymphoma should be treated using a combined-modality approach.     

Extraosseous extension of vertebral hemangioma, a rare cause of spinal cord compression.

STUDY DESIGN: Case report. OBJECTIVE: To illustrate a rare cause of thoracic spinal cord compression, its diagnosis, and its management. SUMMARY OF BACKGROUND DATA: Asymptomatic vertebral hemangiomas are common, but extraosseous extension causing spinal cord compression with neurologic symptoms is rare, and few cases appear in the English-language literature. METHOD: A previously asymptomatic 63-year-old man sought medical attention for acute back pain and thoracic myelopathy of 6 week's duration. Magnetic resonance imaging confirmed the presence of a mass in the T10 vertebral body with paravertebral and intracanalicular extension contributing to cord compression. Decompression and reconstructive surgery were performed and radiotherapy administered after surgery. Preoperative angiography was not performed because of the patient's rapidly progressive neurologic deterioration and the consideration that the differential diagnosis of vertebral hemangioma was less likely. RESULTS: The diagnosis of benign capillary hemangioma was made histologically. Neurologic recovery was complete except for minor residual sensory changes in the legs. At follow-up 10 months after surgery the patient had returned to his usual active life and motor mower repairing business. CONCLUSION: Extraosseous extension of vertebral hemangiomas is a rare cause of thoracic spinal cord compression. As such, the available data are derived from reports based on series involving only a small number of cases, rather than on results of randomized controlled trials. Those causing progressive neurologic symptoms should be surgically decompressed, with the specific procedure determined by the extent and site of the lesion. Preoperative angiography is recommended, but embolization is not always necessary or even possible. Postoperative radiotherapy is recommended when tumor removal is subtotal.     

Leptomeningeal neoplasms

Opinion statement  Leptomeningeal metastasis is becoming an increasingly important late complication of cancer as survival from systemic disease increases, and due to the fact that many novel cancer drugs fail to achieve therapeutic concentrations in the central nervous system. It occurs when neoplastic cells enter cerebrospinal fluid (CSF) pathways, causing diffuse infiltration of the subarachnoid space of the brain and spinal cord. Definitive diagnosis is established by the demonstration of malignant cells in the CSF. However, in certain circumstances the presence of leptomeningeal enhancement on brain or spinal MRI may be sufficient to make the diagnosis. Early diagnosis and aggressive treatment may delay neurologic progression and can lead to prolonged survival and improvement of neurologic function in certain patients. The prognosis depends on the underlying malignancy but is often poor, with a median survival of 4 months, and most treatment interventions are palliative. Nevertheless, some patients respond to treatment, and some survive beyond 1 or 2 years after diagnosis. Areas of radiographic bulky disease or symptomatic tumor should receive radiotherapy. Intrathecal chemotherapy is most effective in patients with lymphoma, leukemia, or breast cancer and without evidence of bulky disease on neuroimaging. Intrathecal chemotherapy requires normal CSF flow, and the most commonly used agents are methotrexate, cytarabine, and thiotepa. In lieu of intrathecal therapy, systemic chemotherapy may occasionally be indicated in select patients in part based on its ability to penetrate into bulky disease. When hydrocephalus occurs, ventriculoperitoneal shunting frequently leads to rapid clinical improvement. There is hope that progress in diagnostic modalities and the development of more effective intrathecal antineoplastic drugs may decrease neurologic morbidity and improve quality of life and survival.     

Intramedullary spinal cord tumors

Opinion statement The three most common types of intramedullary spinal cord tumors are low-grade astrocytomas, ependymomas, and high-grade astrocytomas. Surgical extirpation is the necessary and sufficient primary treatment for most intramedullary spinal cord tumors. Radiation therapy may also have a role in the management of persistent, recurrent, or progressive low-grade astrocytomas and ependymomas. The current treatment of spinal cord high-grade astrocytomas, which includes surgical debulking, radiation therapy, and possibly chemotherapy, is clearly inadequate. Chemotherapy may have a potential role for certain progressive spinal cord tumors, but the role is undefined at present. Recent reports have described the use of stereotactic radiosurgery for extramedullary spinal tumors, and stereotactic radiosurgery may someday be useful in the management of intramedullary spinal cord tumors. Rehabilitation programs are an important component of the multidisciplinary care of patients with spinal cord tumors. Finally, more work, especially the inclusion of adults and children with intramedullary spinal cord tumors into prospective clinical trials, is needed to improve the therapy of intramedullary spinal cord tumors and rehabilitation after diagnosis of a spinal cord tumor.     

Primary breast lymphoma in a male patient

Primary lymphoma of the male breast is extremely rare. We report a case of a diffuse large B-cell lymphoma in a male patient. A 67-year-old man presented with a palpable mass in the right breast and ipsilateral axillary lymphadenopathy. At operation a 6 x 5 x 4-cm mass was excised, and a frozen section demonstrated malignancy. A modified radical mastectomy was then performed, together with axillary lymph node clearance. Histological examination established the diagnosis of a primary non-Hodgkin's lymphoma of the breast. The patient was referred for chemotherapy and died a year later from systemic disease involving the adrenals. The importance of early diagnosis is emphasized; this should be based on an excisional biopsy or aspiration cytology. As patients with primary breast lymphoma (PBL) have a better prognosis than those with carcinoma of the breast or patients with extranodal lymphomas, a multidisciplinary approach including surgery, radiotherapy, and chemotherapy when needed would result in a more favorable outcome.     

Primary malignant lymphoma of the spine: clinical aspects and therapeutic advances

Malignant lymphomas are occasionally encountered in the spine, where they are usually secondary deposits. The authors report the case of a primary non-Hodgkin lymphoma of the L1 vertebra in whom surgical treatment (two operations and double approach) was followed by radiotherapy and chemotherapy. The diagnosis is often made at a late stage, when neurological deficits produced by epidural compression become evident. The surgical treatment is only palliative but has several goals: obtaining a biopsy, improving the neurological symptoms through decompression, stabilizing and "rebuilding" the spinal column; it is performed using posterior, anterior or combined approaches which are discussed. The combined surgical, radiotherapeutic and polychemotherapeutic treatment is associated with a 5-year survival rate of 60-80%. Such a prognosis justifies the risk of surgery which will lead to a stable and lasting reconstruction.     

Primary tumors of the spine. Radiotherapy and chemotherapy

Complete surgical excision of a primary spinal tumor without contamination of the surgical wound is not always possible, and adjuvant therapy by chemotherapy and/or radiation therapy is important to improve local and survival disease free. The efficacy of radiation therapy is limited above L2 because of the risk of radionecrosis of the spinal cord if one exceeds 45 Gy over 4.5 weeks. According to drugs and radiation sensitiveness, three groups can be distinguished: i) malignant tumors with low sensitiveness: chordoma, osteo-, chondro-, and fibro-sarcoma; ii) malignant tumors with high sensitiveness: Ewing's sarcoma, plasmocytoma, and non Hodgkin lymphoma; iii) benign tumos, occasionally aggressive like giant-cells tumor. For each type of tumor, a literature review and our personal experience is presented.     

Primary intramedullary lymphoma. Case report

A 66-year-old female presented primary intramedullary spinal cord lymphoma. This patient was referred for lower limbs weakness, which had developed six weeks earlier and right C5 radiculalgia. Physical examination revealed a medullary syndrome with Claude-Bernard-Horner syndrome. The diagnosis was established after MRI and biopsy (dorsal myelotomy). The patient was given chemotherapy and craniospinal adjuvant radiotherapy (30 Grays). The clinical, radiological and therapeutic features are discussed.     

Primary renal lymphoma of mucosa-associated lymphoid tissue

Mucosa-associated lymphoid tissue-type lymphomas have recently been recognized as a distinctive form of B-cell malignant lymphoma. In contrast to other types of low-grade lymphomas, these tumors have a tendency to be localized at diagnosis and to be curable with local therapy. We report an unusual case of primary localized low-grade lymphoma of mucosa-associated lymphoid tissue arising in the kidney. The patient underwent radical nephrectomy and was free of disease at 28 months of follow-up without additional treatment. Once properly staged and classified, lymphoma of mucosa-associated lymphoid tissue involving the kidney can be managed by radical nephrectomy and follow-up.     

Primary non-Hodgkin large B-cell lymphoma of bladder. Report of a case

Introductionprimary genitourinary lymphomas are uncommon. Among them, bladder lymphomas are extremely unusual tumors, with clinico-radiological features similar to urothelial carcinomas of bladder. Histopathological, immunohistochemical and molecular studies are compulsory for the diagnosis. We report a case of this tumorClinical caseAn 80-year-old woman was admitted to our hospital with hematuria. Abdominal ultrasound and cystoscopy revealed an infiltrating bladder tumor involving the right lateral wall. After transuretral biopsy, a diagnosis of non-Hodgkin large B-cell lymphoma was made. Neither clinical symptoms nor radiological findings showed disseminated disease, indicating that the tumor was localized in the bladder. After chemotherapy, the patient is disease-free after 9 months follow-upCommentif a bladder tumor with uncommon histopathological features is found, lymphoma should be excluded, because chemotherapy avoids cystectomy     

Unusual T- and T/NK- cell non-Hodgkin's lymphoma of the larynx: a diagnostic challenge for clinicians and pathologists.

BACKGROUND: Lymphomas of T-cell and T/natural killer (T/NK)-cell types rarely involve the larynx. Early symptoms are subtle and nonspecific, and confirmation of the diagnosis is often difficult. Because of the rarity of this disease entity, the optimal management remains controversial. METHODS: The case records of patients with the diagnosis of lymphoma involving the larynx were retrospectively reviewed. Details of the pathologic condition, the difficulties in diagnosis, and treatment were presented. RESULTS: One case of T-cell lymphoma (Lennert's lymphoma subtype) and two cases of T/NK-cell lymphomas involving the larynx were presented. The diagnoses were all difficult to establish. All three patients received either multiagent chemotherapy or radiotherapy as the first-line treatment. They all died of the disease within 32 months of diagnosis. CONCLUSIONS: T- and T/NK-cell non-Hodgkin's lymphoma of the larynx is a diagnostic challenge for clinicians and pathologists. Conventional chemotherapy or radiotherapy has not been effective. A more aggressive therapy should be considered.     

Primary lymphoma of the breast: a case of marginal zone B-cell lymphoma

A case of primary marginal zone B-cell lymphoma in an elderly female patient is presented. Primary breast lymphomas are rare, comprising less than 1 per cent of all breast malignancies. These tumors have no clinical, pathologic, or radiologic pathognomonic features to distinguish them from breast adenocarcinoma. The diagnosis is usually made with an excisional biopsy, and more extensive surgery should be avoided. Delivery of radiation therapy and chemotherapy is tailored according to the histologic grade, stage of disease, and overall patient condition. This report summarizes the current knowledge reflected in the literature.     

Primary brain lymphomas after kidney transplantation: presentation and outcome

BACKGROUND: Non-Hodgkin's lymphoma is the second most frequent neoplasia following solid-organ transplantation. The objective of this study is to describe the clinical, histologic, and radiologic features of primary posttransplantation brain lymphomas (PTBL) in addition to their outcome. METHODS: Twenty-five kidney transplant patients with histologically proven PTBL from 11 French centers were retrospectively investigated. RESULTS: Immunosuppressive regimen included induction with antithymocyte globulins (ATG) in 20 patients. Median overall delay between transplantation and lymphoma was 18 months (4-264). Six of 10 patients with late posttransplantation brain lymphomas (PTBL) occurrence (>3 years) had been recently switched from azathioprine to mycophenolate mofetil (median switch lymphoma delay 14 months). Cerebral computed tomography (CT) scans and magnetic resonance imaging (MRI) revealed multifocal lesions (n=18), with a ring contrast enhancement (n=20) similar to cerebral abscesses, as observed in HIV-related brain lymphomas. Histology showed large B-cell non-Hodgkin's lymphoma in 87.5% of cases; Epstein-Barr virus (EBV) was detected in 95%. After lymphoma diagnosis, immunosuppressive treatment was reduced in all patients, and all but one received complementary treatment by surgery (n=2), anti-CD21 antibodies (n=2), chemotherapy including high-dose intravenous methotrexate (n=7), encephalic radiotherapy (n=5), or chemotherapy plus radiotherapy (n=8). Median overall survival was 26 months. Patients with a radiotherapy-based regimen seemed to have a longer survival (36 vs. 7 months, P<0.005). CONCLUSIONS: Our study showed that PTBL are EBV-induced large B-cell lymphomas, which mimic cerebral abscesses on imaging and whose occurrence may be influenced by immunosuppression modifications. Treatment by radiotherapy is associated with better survival.     

The diminishing role of surgery in the treatment of gastric lymphoma

OBJECTIVE: This article reviews the pathogenesis, diagnosis, and treatment of patients with primary gastric lymphoma, with special attention to the changing role of surgery. SUMMARY BACKGROUND DATA: Primary gastric lymphomas are non-Hodgkin lymphomas that originate in the stomach and are divided into low-grade (or indolent) and high-grade (or aggressive) types. Low-grade lesions nearly always arise from mucosa-associated lymphoid tissue (MALT) secondary to chronic Helicobacter pylori (H. pylori) infection and disseminate slowly. High-grade lesions may arise from a low grade-MALT component or arise de novo and can spread to lymph nodes, adjacent organs and tissues, or distant sites. METHODS: A review of the relevant English-language articles was performed on the basis of a MEDLINE search from January 1984 to August 2003. RESULTS: About 40% of gastric lymphomas are low-grade, and nearly all these low-grade lesions are classified as MALT lymphomas. For low-grade MALT lymphomas confined to the gastric wall and without certain negative prognostic factors, H. pylori eradication is highly successful in causing lymphoma regression. More advanced low-grade lymphomas or those that do not regress with antibiotic therapy can be treated with combinations of H. pylori eradication, radiation therapy, and chemotherapy. Nearly 60% of gastric lymphomas are high-grade lesions with or without a low-grade MALT component. These lymphomas can be treated with chemotherapy and radiation therapy according to the extent of disease. Surgery for gastric lymphoma is now often reserved for patients with localized, residual disease after nonsurgical therapy or for rare patients with complications. CONCLUSION: The treatment of gastric lymphoma continues to evolve, and surgical resection is now uncommonly a part of the initial management strategy.