The NOMS approach to metastatic tumors: Integrating new technologies to improve outcomes / El enfoque de NOMS para los tumores metastásicos: integración de nuevas tecnologías para mejorar los resultados

Los paradigmas de tratamiento para pacientes con metástasis de columna vertebral han evolucionado significativamente en las últimas dos décadas. El cambio más transformador de estos paradigmas ha sido la integración de la radiocirugía estereotáctica espinal (sSRS). La sSRS permite la administración de dosis de radiación lítica con preservación de los órganos cercanos en riesgo, particularmente la médula espinal. La evidencia apoya la seguridad y la eficacia de la radiocirugía, ya que actualmente ofrece un control tumoral local duradero con bajas tasas de complicaciones, incluso para tumores que anteriormente se consideraban radiorresistentes a la radioterapia convencional de haz externo. El papel de la intervención quirúrgica sigue siendo consistente, pero se ha observado una tendencia hacia técnicas menos agresivas, a menudo mínimamente invasivas. Utilizando tecnologías modernas e instrumentación mejorada, los resultados quirúrgicos continúan mejorando con una morbilidad reducida. Además, los agentes dirigidos, como los productos biológicos y los inhibidores de puntos de control, han revolucionado la atención del cáncer al mejorar tanto el control local como la supervivencia del paciente. Estos avances han dado lugar a la necesidad de nuevas herramientas de pronóstico y a una revisión más crítica de los resultados a largo plazo. La naturaleza compleja de los esquemas de tratamiento actuales requiere un enfoque multidisciplinario que incluya cirujanos, oncólogos médicos, oncólogos radioterápicos, intervencionistas y especialistas en dolor. Esta revisión recapitula los datos actuales basados en la evidencia sobre el tratamiento de las metástasis espinales e integra estos datos en un marco de decisión, NOMS, que se basa en cuatro pilares centinela de la toma de decisiones en tumores metastásicos de la columna vertebral: estado neurológico, comportamiento oncológico del tumor, estabilidad mecánica, y carga sistémica de la enfermedad y comorbilidades médicas.(AU)

Spinal metastases are a common oncologic challenge as 20–40% of cancer patients are affected during the course of their illness and up to 20% of those will become symptomatic from spinal cord compression.1–5 The magnitude of this problem is expected to grow commensurate with the exponential rise in the use of targeted therapies which have demonstrated markedly improved survivals for virtually all malignant tumors. Additionally, the increased availability of advanced diagnostic imaging such as magnetic resonance imaging and 18-FDG PET scans will also serve to increase detection of spine metastatic disease. Despite extended survivals conveyed by biologics and checkpoint inhibitors, the treatment goals for patients with spine metastases remain palliative and focused on the preservation or restoration of neurological function and spinal stability, improved pain control and health related quality of life (HRQOL), and durable tumor control. Scoring systems such as the Tomita score6 and Tokuhashi revised score7 historically have been used to estimate survival and dictate treatment but increasingly have become obsolete due to their inability to incorporate and account for advances in all domains of cancer treatment.(AU)

El enfoque de NOMS para los tumores metastásicos: integración de nuevas tecnologías para mejorar los resultados / The NOMS approach to metastatic tumors: Integrating new technologies to improve outcomes

Los paradigmas de tratamiento para pacientes con metástasis de columna vertebral han evolucionado significativamente en las últimas dos décadas. El cambio más transformador de estos paradigmas ha sido la integración de la radiocirugía estereotáctica espinal (sSRS). La sSRS permite la administración de dosis de radiación lítica con preservación de los órganos cercanos en riesgo, particularmente la médula espinal. La evidencia apoya la seguridad y la eficacia de la radiocirugía, ya que actualmente ofrece un control tumoral local duradero con bajas tasas de complicaciones, incluso para tumores que anteriormente se consideraban radiorresistentes a la radioterapia convencional de haz externo. El papel de la intervención quirúrgica sigue siendo consistente, pero se ha observado una tendencia hacia técnicas menos agresivas, a menudo mínimamente invasivas. Utilizando tecnologías modernas e instrumentación mejorada, los resultados quirúrgicos continúan mejorando con una morbilidad reducida. Además, los agentes dirigidos, como los productos biológicos y los inhibidores de puntos de control, han revolucionado la atención del cáncer al mejorar tanto el control local como la supervivencia del paciente. Estos avances han dado lugar a la necesidad de nuevas herramientas de pronóstico y a una revisión más crítica de los resultados a largo plazo. La naturaleza compleja de los esquemas de tratamiento actuales requiere un enfoque multidisciplinario que incluya cirujanos, oncólogos médicos, oncólogos radioterápicos, intervencionistas y especialistas en dolor. Esta revisión recapitula los datos actuales basados en la evidencia sobre el tratamiento de las metástasis espinales e integra estos datos en un marco de decisión, NOMS, que se basa en cuatro pilares centinela de la toma de decisiones en tumores metastásicos de la columna vertebral: estado neurológico, comportamiento oncológico del tumor, estabilidad mecánica, y carga sistémica de la enfermedad y comorbilidades médicas.(AU)

Spinal metastases are a common oncologic challenge as 20–40% of cancer patients are affected during the course of their illness and up to 20% of those will become symptomatic from spinal cord compression.1–5 The magnitude of this problem is expected to grow commensurate with the exponential rise in the use of targeted therapies which have demonstrated markedly improved survivals for virtually all malignant tumors. Additionally, the increased availability of advanced diagnostic imaging such as magnetic resonance imaging and 18-FDG PET scans will also serve to increase detection of spine metastatic disease. Despite extended survivals conveyed by biologics and checkpoint inhibitors, the treatment goals for patients with spine metastases remain palliative and focused on the preservation or restoration of neurological function and spinal stability, improved pain control and health related quality of life (HRQOL), and durable tumor control. Scoring systems such as the Tomita score6 and Tokuhashi revised score7 historically have been used to estimate survival and dictate treatment but increasingly have become obsolete due to their inability to incorporate and account for advances in all domains of cancer treatment.(AU)

The NOMS approach to metastatic tumors: Integrating new technologies to improve outcomes. / El enfoque de NOMS para los tumores metastásicos: integración de nuevas tecnologías para mejorar los resultados.

Treatment paradigms for patients with spine metastases have evolved significantly over the past two decades. The most transformative change to these paradigms has been the integration of spinal stereotactic radiosurgery (sSRS). sSRS allows for the delivery of tumoricidal radiation doses with sparing of nearby organs at risk, particularly the spinal cord. Evidence supports the safety and efficacy of radiosurgery as it currently offers durable local tumor control with low complication rates even for tumors previously considered radioresistant to conventional external beam radiation therapy. The role for surgical intervention remains consistent, but a trend has been observed toward less aggressive, often minimally invasive techniques. Using modern technologies and improved instrumentation, surgical outcomes continue to improve with reduced morbidity. Additionally, targeted agents such as biologics and checkpoint inhibitors have revolutionized cancer care by improving both local control and patient survival. These advances have brought forth a need for new prognostication tools and a more critical review of long-term outcomes. The complex nature of current treatment schemes necessitates a multidisciplinary approach including surgeons, medical oncologists, radiation oncologists, interventionalists and pain specialists. This review recapitulates the current state-of-the-art, evidence-based data on the treatment of spinal metastases and integrates these data into a decision framework, NOMS, which is based on four sentinel pillars of decision making in metastatic spine tumors: neurological status, Oocologic tumor behavior, mechanical stability and systemic disease burden and medical co-morbidities.

The NOMS approach to metastatic tumors: Integrating new technologies to improve outcomes.

Treatment paradigms for patients with spine metastases have evolved significantly over the past two decades. The most transformative change to these paradigms has been the integration of spinal stereotactic radiosurgery (sSRS). sSRS allows for the delivery of tumoricidal radiation doses with sparing of nearby organs at risk, particularly the spinal cord. Evidence supports the safety and efficacy of radiosurgery as it currently offers durable local tumor control with low complication rates even for tumors previously considered radioresistant to conventional external beam radiation therapy. The role for surgical intervention remains consistent, but a trend has been observed toward less aggressive, often minimally invasive techniques. Using modern technologies and improved instrumentation, surgical outcomes continue to improve with reduced morbidity. Additionally, targeted agents such as biologics and checkpoint inhibitors have revolutionized cancer care by improving both local control and patient survival. These advances have brought forth a need for new prognostication tools and a more critical review of long-term outcomes. The complex nature of current treatment schemes necessitates a multidisciplinary approach including surgeons, medical oncologists, radiation oncologists, interventionalists and pain specialists. This review recapitulates the current state-of-the-art, evidence-based data on the treatment of spinal metastases and integrates these data into a decision framework, NOMS, which is based on four sentinel pillars of decision making in metastatic spine tumors: Neurological status, Oncologic tumor behavior, Mechanical stability, and Systemic disease burden and medical co-morbidities.

Management of radiated reoperative wounds of the cervicothoracic spine: the role of the trapezius turnover flap.

Reoperation for malignant disease of the cervicothoracic spine can lead to compromised wound healing secondary to poor tissue quality from previous operations, heavily irradiated beds, and concomitant steroid therapy. Other complicating factors include exposed dura and spinal implants. Introducing well-vascularized soft tissue to obliterate dead space is critical to reliable wound healing. The purpose of this study was to determine the efficacy of the trapezius turnover flap in the management of these complex wounds. This study is a retrospective review of all patients undergoing trapezius muscle turnover flaps for closure of complex cervicothoracic wounds after spinal operations for metastatic or primary tumors. Six patients (3 male/3 female) were operated over an 18-month period (mean patient age, 43 years). Primary pathologies included radiation-induced peripheral nerve sheath tumor (N = 2), chondrosarcoma (N = 1), nonsmall-cell lung cancer (N = 1), paraganglioma (N = 1), and spindle cell sarcoma (N = 1). Trapezius muscle turnover flaps were unilateral and based on the transverse cervical artery in every patient. Indication for flap closure included inability to perform primary layered closure (N = 3), open wound with infection (N = 2), and exposed hardware (N = 1). All patients had previous operations of the cervicothoracic spine (mean, 5.8 months; range 2-9 months) for malignant disease and prior radiation therapy. Exposed dura was present in all patients, and 2 patients had dural repairs with bovine pericardial patches. Spinal stabilization hardware was present in 4 patients. All patients underwent perioperative treatment with systemic corticosteroids. All flaps survived, and primary wound healing was achieved in each patient. The only wound complication was a malignant pleural effusion communicating with the back wound, which was controlled with a closed suction drain. All wounds remained healed during the follow-up period. Four patients died from progression of disease within 10 months of surgery. The trapezius turnover flap has been used successfully when local tissue conditions prevent primary closure, or in the setting of open, infected wounds with exposed dura and hardware. The ease of flap elevation and minimal donor site morbidity make it a useful, single-stage reconstructive option in these difficult wounds.

Tension pneumocephalus resulting from iatrogenic subarachnoid-pleural fistulae: report of three cases.

BACKGROUND: Symptomatic pneumocephalus may result from a cerebrospinal fluid leak communicating with extradural air. However, it is a rare event after thoracic surgical procedures, and its management and physiology are not widely recognized. METHODS: During the past 2 years, we have identified 3 patients who developed pneumocephalus after thoracotomy for tumor resection. Only 1 patient had a discernible spinal fluid leak identified intraoperatively. Two patients experienced delayed spinal fluid drainage from their chest tubes and subsequently developed profound lethargy, confusion, and focal neurologic signs. The third patient was readmitted to the hospital with a delayed pneumothorax and altered mental status. Radiographic imaging in all patients showed significant pneumocephalus of the basilar cisterns and ventricles. RESULTS: The first 2 patients were managed by discontinuation of the chest tube suction and bedrest. The third patient underwent surgical reexploration and nerve root ligation. All 3 patients had resolution of their symptoms within 72 hours. CONCLUSIONS: Pneumocephalus is a rare, but serious, complication of thoracotomy. Previous patients reported in the literature have been managed with reoperation to ligate the nerve roots. However, the condition resolved nonoperatively in 2 of our patients. Discontinuation of chest tube suction may be definitive treatment and is always the important initial management to decrease cerebrospinal fluid extravasation into the pleural space and allow normalization of neurologic symptoms.

Stereotactic intensity-modulation radiation therapy for vertebral body and paraspinal tumors.

Therapeutic doses of radiation for paraspinal tumors are often limited by the dose-related tolerance of the spinal cord. Intensity-modulated radiation therapy (IMRT) is an advanced form of three-dimensional conformal radiation therapy that provides improved coverage of tumor volumes while reducing the radiation dose to the spinal cord. Computer-controlled multileaf collimation provides high conformality, which makes it feasible to treat tumors of any shape, even those that are wrapped around the spinal cord. The use of a newly developed, noninvasive body frame, the capability of fusing computerized tomography and magnetic resonance images, and on-line portal films provide precise target immobilization and target identification. In this paper the authors discuss their preliminary experience in six cases in which IMRT was used to treat paraspinal lesions in patients who harbored locally recurrent tumors and/or tumors that previously received the maximum doses of radiation that could be tolerated by the spinal cord.

Intralesional resection of primary and metastatic sarcoma involving the spine: outcome analysis of 59 patients.

OBJECTIVE: Surgery plays an important role in achieving local tumor control and cure for primary and metastatic tumors of the spine. As has been established with regard to sarcomas at extraspinal sites, these goals may best be achieved by en bloc resection with negative histological margins. Unfortunately, sarcomas of the spine often present with tumor patterns that are amenable only to intralesional resection, if neurological preservation is a priority. This study is a retrospective analysis of the long-term outcomes of patients who had operations for sarcomas of the spine using modern surgical approaches, intralesional resections, and spinal instrumentation. METHODS: Between 1985 and 1997, 59 patients had spinal operations for sarcoma involving the extrasacral spine. Data regarding tumor histology, grade, surgical indications, patterns of spinal tumor involvement, and neurological and functional outcomes were reviewed at presentation and at tumor recurrence. RESULTS: Thirty-five patients underwent a single operation, and 24 patients required reoperation for locally recurrent tumors. At presentation, only nine patients (15%) had tumors that were amenable to marginal or wide resections. Functional outcomes after initial spinal surgery and after operations performed at first tumor recurrence showed that 95% of patients had maintained or regained ambulation. Intradural extension of tumor was seen in 5 of 12 patients who had three or more operations for locally recurrent disease. The median survival from first spine operation was 18 months, and the median event-free interval between the first and second spine operations was 13 months. CONCLUSION: Surgery for sarcoma of the spine is useful for maintaining or improving neurological and functional outcomes, but local tumor recurrences are common. Because of the anatomy of the tumor at presentation and concern for neurological preservation, few patients are candidates for marginal or wide resections.

Endoscopic biopsy for tumors of the third ventricle.

Twelve patients underwent endoscopic biopsy of a tumor involving the third ventricle. Nine patients had no significant medical history while 3 had a history of cancer. Unique characteristics of each case dictated the optimal surgical technique. Endoscopic tumor biopsy was combined with additional procedures in 9 cases; shunt insertion (3), shunt insertion with endoscopic septostomy (5), and transcallosal craniotomy (1). Diagnosis was established in 11 patients (92%); 6 primary brain tumors, 3 metastatic central nervous system tumors, 1 metastatic systemic cancer, and 1 region of post-treatment gliosis. One case was aborted due to poor visualization. Therapy was directly influenced by endoscopic biopsy in 11/12 cases (92%) and craniotomy for tumor resection was avoided in 10/12 patients (83%). Of the 5 patients who underwent endoscopic septostomy, 4 required no subsequent procedures for hydrocephalus. There were no complications, and hospital stay averaged 1.78 days for patients who underwent successful endoscopic biopsy. Tumors of the third ventricle are amenable to endoscopic biopsy with excellent diagnostic yield and low morbidity. The procedure must be tailored depending upon the tumor location within the third ventricle, the degree of ventriculomegaly, and the need to perform a septostomy. Singularly or combined with other endoscopic procedures, patients can be spared multiple and more invasive surgical procedures.

Sclerosing epithelioid fibrosarcomas involving the neuraxis: report of three cases.

OBJECTIVE AND IMPORTANCE: Sclerosing epithelioid fibrosarcoma (SEF) is a rare mesenchymal neoplasm composed of rounded, vimentin-immunoreactive tumor cells disposed in nests and cords within a hyalinized collagenous matrix. Most examples arise in the deep skeletal muscles of adults. The cases recorded to date have been characterized by protracted clinical evolutions with a tendency for stubborn local recurrence, followed by late metastasis. Accordingly, SEF has been regarded as a low-grade sarcoma. A single instance of brain and vertebral metastasis has been described. We report three examples of SEF distinguished by primary involvement of the neuraxis at initial presentation. CLINICAL PRESENTATION: Two tumors had intracranial, calvarial and extracalvarial, soft-tissue components, whereas the third tumor manifested as a paraspinal mass with extension into the T12-L1 neural foramen and invasion of the T12 nerve root. INTERVENTION: All three affected patients experienced local recurrence and distant metastasis after resection of the primary site. These complications appeared early in the disease course in two cases. In no case was there a response to adjuvant chemotherapy or radiotherapy. CONCLUSION: Our experience indicates that SEFs arising along the neuraxis may demonstrate unexpectedly aggressive clinical behavior, compared with those arising in the more typical location of deep skeletal muscles.

An integrated functional magnetic resonance imaging procedure for preoperative mapping of cortical areas associated with tactile, motor, language, and visual functions.

OBJECTIVE: To evaluate an integrated battery of preoperative functional magnetic resonance imaging (fMRI) tasks developed to identify cortical areas associated with tactile, motor, language, and visual functions. METHODS: Sensitivity of each task was determined by the probability that a targeted region was activated for both healthy volunteers (n = 63) and surgical patients with lesions in these critical areas (n = 125). Accuracy of each task was determined by the correspondence between the fMRI maps and intraoperative electrophysiological measurements, including somatosensory evoked potentials (n = 16), direct cortical stimulation (n = 9), and language mapping (n = 5), and by preoperative Wada tests (n = 13) and visual field examinations (n = 6). RESULTS: For healthy volunteers, the overall sensitivity was 100% for identification of the central sulcus, visual cortex, and putative Wernicke's area, and 93% for the putative Broca's area (dominant hemisphere). For patients with tumors affecting these regions of interest, task sensitivity was 97% for identification of the central sulcus, 100% for the visual cortex, 91% for the putative Wernicke's area, and 77% for the putative Broca's area. These sensitivities were enhanced by the use of multiple tasks to target related functions. Concordance of the fMRI maps and intraoperative electrophysiological measurements was observed whenever both techniques yielded maps and Wada and visual field examinations were consistent with fMRI results. CONCLUSION: This integrated fMRI task battery offers standardized and noninvasive preoperative maps of multiple critical functions to facilitate assessment of surgical risk, planning of surgical routes, and direction of conventional, intraoperative electrophysiological procedures. Thus, a greater range of structural and functional relationships is brought to bear in the service of optimal outcomes for neurosurgery.

Single-stage posterolateral transpedicle approach for spondylectomy, epidural decompression, and circumferential fusion of spinal metastases.

STUDY DESIGN: Retrospective review of prospectively maintained institutional spine database. OBJECTIVES: To assess the pain, neurologic, and functional outcome of patients with metastatic spinal cord compression using a posterolateral transpedicular approach with circumferential fusion. SUMMARY OF BACKGROUND DATA: Patients with spinal metastases often have patterns of disease requiring both an anterior and posterior surgical decompression and spinal fusion. For patients whose concurrent illness or previous surgery makes an anterior approach difficult, a posterior transpedicular approach was used to resect the involved vertebral bodies, posterior elements, and epidural tumor. This approach provides exposure sufficient to decompress and instrument the anterior and posterior columns. METHODS: During the past 15 months, 25 patients were operated on using a posterolateral transpedicular approach. The primary indications for surgery were back pain (15 patients) and neurologic progression (10 patients). All patients had vertebral body disease, and 21 patients had high-grade spinal cord compression from epidural disease as assessed by magnetic resonance imaging. Seven patients underwent preoperative embolization for vascular tumors. In each patient, the anterior column was reconstructed with polymethyl methacrylate and Steinmann pins and the posterior column with long segmental fixation. RESULTS: All patients achieved immediate stability. Pain relief was significant in all 23 patients who had had moderate or severe pain. Neurologic symptoms were stable or improved in 23 patients. One patient with an acutely evolving myelopathy was immediately worse after surgery, and one patient had a delayed neurologic worsening, progressing to paraplegia. CONCLUSIONS: The posterolateral transpedicular approach provides a wide surgical exposure to decompress and instrument the anterior and posterior spine. This technique avoids the morbidity associated with anterior approaches and provides immediate stability. Vascular tumors may be removed safely after embolization. Patients can be mobilized early after surgery.

Ommaya reservoirs for the treatment of leptomeningeal metastases.

OBJECTIVE: Ommaya reservoirs are frequently used to deliver intraventricular chemotherapy in cancer patients with leptomeningeal metastases. We review techniques of catheter placement and complication avoidance. METHODS: Between January 1995 and June 1998, Ommaya reservoirs were placed in 107 patients for the treatment or prophylaxis of leptomeningeal metastases at the Memorial Sloan-Kettering Cancer Center. Patients with slit ventricles (total, 25) underwent preoperative pneumoencephalography for ventricular dilation. Intraoperative fluoroscopic guidance was used in 77 patients to confirm the catheter tip position at the foramen of Monro. Other intraoperative aids included endoscopy in 21 patients, ultrasound in 7, and stereotaxy in 6. No aids were used in 3 patients, more than one aid was used in 9, and the technique could not be determined retrospectively in 3. RESULTS: The median survival of patients treated for leptomeningeal metastases was 9 months (Kaplan-Meier method). Eight patients developed hydrocephalus requiring conversion of the Ommaya reservoir to a ventriculoperitoneal shunt and precluding delivery of chemotherapeutic agents. An additional 11 patients referred for Ommaya reservoir placement demonstrated elevated intracranial pressure requiring an initial ventriculoperitoneal shunt. Complications of Ommaya reservoir placement occurred in 10 patients (9.3%) and included two infections, five catheter malpositions, and three intracranial hemorrhages. Two deaths occurred secondary to intracranial hemorrhage: one after postoperative anticoagulation for a mechanical heart valve, and one attributed to treatment-related thrombocytopenia. Nine patients (8.4%) had treatment-related imaging abnormalities; seven were asymptomatic and two developed symptomatic leukoencephalopathy. CONCLUSION: Complications associated with Ommaya reservoirs can be minimized by intraoperative confirmation of the catheter position with fluoroscopic guidance and/or endoscopy. We recommend postoperative computed tomographic scans before initiation of intraventricular chemotherapy. Patients with elevated intracranial pressure may require shunting procedures in lieu of Ommaya reservoir placement.

Transpedicular approach for thoracic disc herniations.

OBJECT: Patients with symptomatic herniated thoracic discs may require operation for intractable radiculopathy or functionally disabling myelopathy. In the past, laminectomy was the procedure of choice for the treatment of thoracic herniations, but it was found that the approach was associated with an unacceptably high rate of neurological morbidity. Several strategies have been developed to excise the disc without manipulating the spinal cord. The focus of this paper is the transpedicular approach. METHODS: The author retrospectively reviewed the cases of 20 consecutive patients presenting with herniated thoracic discs in whom surgery was performed via a transpedicular approach. Fourteen patients presented with acute myelopathy and six with radiculopathy. Of those with myelopathy six of six regained ambulation and six of seven regained normal bladder function. No patient with myelopathy experienced neurological worsening. In four patients presenting with radiculopathy postoperative pain resolved, and in two it remained unchanged. Three minor complications (15%) occurred. No patient suffered postoperative spinal instability-related pain or delayed kyphosis. CONCLUSIONS: As experience accumulates in the use of multiple approaches for the treatment of thoracic disc herniations, the role of each is becoming more clearly defined. The transpedicular approach is most applicable to lateral or centrolateral calcified or soft discs. The more anterior (transthoracic or thoracoscopic) and lateral (costotransversectomy or lateral extracavitary) approaches may be more useful for excision of central calcified discs.

Primary intracranial neoplasms in patients with HIV.

OBJECTIVE: To report a series of HIV-infected patients with intracranial tumors not known to be associated with immunodeficiency. BACKGROUND: The spectrum of HIV-associated diseases is changing with improved treatments and prolonged patient survival. Although primary central nervous system lymphoma (PCNSL) and toxoplasmosis continue to be the most common intracranial lesions in HIV-infected patients, the recognition of other pathologic entities is increasingly important. METHODS: The clinical characteristics and outcome of eight HIV-infected patients with nine intracranial neoplasms other than PCNSL are reported. In addition, all available pathologic specimens were tested for evidence of either HIV or Epstein-Barr virus (EBV) infection. An additional 28 patients reported in the literature are summarized. RESULTS: Five of eight patients had a glioblastoma multiforme; other tumors included an anaplastic ependymoma, a low-grade glioma, a subependymoma, and a leiomyosarcoma. More than half of the patients developed their tumor > or =6 years after the diagnosis of HIV infection. Patient prognosis and survival was best predicted by tumor histology. Treatment response and outcome did not appear to be influenced by HIV infection. Only the leiomyosarcoma demonstrated evidence of latent EBV infection. CONCLUSIONS: HIV-infected patients are at risk for intracranial neoplasms other than PCNSL, and benefit from aggressive tumor-specific therapy. It is possible that gliomas are occurring at a higher rate than in the general population. There was no evidence of HIV or EBV infection in any glial tumor.

The diagnosis and treatment of metastatic spinal tumor.

Metastases to the spine represent a challenging problem in an oncology practice. Treatment decisions require multidisciplinary review. Radiation therapy remains the primary treatment for metastatic spinal tumor, but advances in radiation therapy, chemotherapy, and surgery have changed the roles of each and lead to improved patient outcomes. Regardless of the treatment, diagnosis and treatment before the development of significant neurologic and functional deficits improve outcomes. Physician awareness and appropriate imaging greatly assist in the early detection of tumor.

Subdiaphragmatic and intrathoracic paraspinal malignant peripheral nerve sheath tumors: a clinicopathologic study of 25 patients and 26 tumors.

BACKGROUND: To determine the effects of anatomic site on the presentation and diagnosis of malignant peripheral nerve sheath tumors (MPNSTs) and on the treatment and outcomes of the patients, the authors initiated a study of these tumors at different sites. An earlier report described MPNSTs of the buttock and lower extremity, and the current series analyzes those presenting at intrathoracic (IT) and subdiaphragmatic (SD) paraspinal sites. METHODS: The authors reviewed data on patients with paraspinal MPNSTs who were seen at Memorial Hospital during the period 1960-1995 and for whom histologic slides were available. Various clinicopathologic parameters and their effects on patient outcomes were examined. RESULTS: Twenty-five patients with 26 tumors were evaluated. Seven tumors were IT and 19 were SD; 60% of the patients had neurofibromatosis type 1 (NF1). Most patients presented with pain, and a diagnostic delay (of 3 months to 2 years) was often noted. Mean tumor sizes for SD and IT tumors were 14.3 cm and 6.6 cm, respectively. Most MPNSTs were composed of spindle cells in fascicles. Twenty-seven percent exhibited divergent differentiation. Twenty-four tumors were high grade, and a low grade component was identified in 8 tumors. Surgical resection was attempted for 23 tumors (88%), but complete resection was achieved in only 6 cases (23%). Eighty percent of the patients died of their tumors, 2-year and 5-year survival rates were 35% and 16%, and median survival was 8.5 months. Significant prognostic factors were tumor size <5 cm, the presence of a low grade component, and complete tumor resection. CONCLUSIONS: Paraspinal MPNSTs have more aggressive behavior than peripherally located tumors, mainly because of the difficulty encountered in resecting them completely. Prognoses of patients with MPNST at this site appear to be affected by resection status, tumor size, and tumor grade.

Function-sparing surgery for desmoid tumors and other low-grade fibrosarcomas involving the brachial plexus.

OBJECTIVE: Low-grade fibrosarcomas and desmoid tumors present a surgical challenge in that they have a strong tendency for local invasion, surgical margins are poorly delineated, and complete resections are difficult. Nowhere is this more evident than in those lesions involving the brachial plexus. We review our experience with these difficult lesions. METHODS: From a prospective database of 2900 patients admitted for treatment of sarcoma between 1982 and 1996, we identified 15 patients with involvement of the brachial plexus by a low-grade fibrosarcoma or desmoid tumor. All patients underwent resection, with 13 of 15 receiving adjuvant radiotherapy. The 15 patients had a mean age at initial operation of 47 years. The male-to-female ratio was 8:7. The mean follow-up period was 65 months (median, 53 mo). RESULTS: Gross total resection was achieved in 12 patients (80%), although 11 of these patients had positive surgical margins. Overall, 64% of the tumors have recurred locally. There were no distant metastases, and no patients died as a result of their disease. One patient died as a result of unrelated cancer. An assessment of the functional outcomes revealed seven patients with normal function or mild neurological deficits and eight who were suffering from significant weakness, debilitation, or chronic pain. One patient required forequarter amputation. CONCLUSION: Surgical resection plus postoperative radiotherapy is the treatment of choice for low-grade fibrosarcomas and desmoid tumors involving the brachial plexus. However, aggressive surgical management with the goal of achieving a gross total resection with negative histological margins can produce unnecessary morbidity. Preserving function should be a primary goal of the operations, although this will be associated with residual disease and will risk local recurrence but rarely death resulting from the disease.

Craniofacial resection for malignant tumors involving the anterior skull base.

OBJECTIVES: To review our experience with craniofacial resection for malignant neoplasms of the anterior skull base and report long-term results, and to analyze survival in terms of the overall experience, tumor histological diagnoses, and tumor extent. Also, to report complications of this surgical procedure. DESIGN: Retrospective review. SETTING: Tertiary cancer facility. PATIENTS: We evaluated 115 consecutive patients undergoing craniofacial resection for malignant neoplasms involving the anterior skull base. Forty-five (39%) presented with recurrent or persistent disease after prior therapy. MAIN OUTCOME MEASURES: Survival was evaluated with the Kaplan-Meier product limit method and comparisons between individual subgroups were performed using the log-rank test. RESULTS: The operative mortality rate was 3.5%. Major complications occurred in 40 patients (35%). For the entire group, disease-specific survival rates were 58% and 48% at 5 and 10 years, respectively. The highest survival rate was observed in patients with esthesioneuroblastoma and lowest in those with mucosal melanoma. Survival was significantly better for those whose tumors could be excised with a limited resection in comparison with those requiring an extended procedure (P = .009). CONCLUSIONS: A 23-year experience with craniofacial resection performed for malignant tumors involving the anterior skull base confirms the durable results obtained with this intervention. The diversity of histological diagnoses, site of origin, extent of tumor invasion, and impact of prior therapy hampers any attempt at reporting meaningful survival statistics for comparison with other series or other means of treatment.