Intracranial ependymomas

Clinically significant spinal implants develop in intracranial ependymomas. Spinal subarachnoid implants developed in 11 of 32 patients who received local irradiation at the University of Michigan between 1955 and 1972 for intracranial ependymoma. Seven of these patients received spinal axis irradiation for neurological disturbances resulting from the implants, and implants were found in the other 4 patients postmortem. Ten patients had infratentorial tumors, and one had a supratentorial tumor; nine tumors were poorly differentiated, two were well differentiated. The authors conclude that spinal irradiation is required if the tumor is poorly differentiated, or located in the infratentorial fossa.     

Treatment of intracranial ependymomas of children: review of a 15-year experience

PURPOSE: There are still major controversies in the optimal management of children with intracranial ependymomas. To assess the impact of tumor site, histology, and treatment, the outcome of children treated at the Institut Gustave Roussy was reviewed retrospectively. METHODS AND MATERIALS: Between 1975 and 1989, 80 children aged 4 months to 15.8 years were seen at the Institut Gustave Roussy for postoperative management of an intracranial ependymoma. Location of tumor was infratentorial in 63 cases and supratentorial in 17. Surgical treatment consisted of complete resection in 38, incomplete resection in 38 and biopsy only in 4. Postoperative irradiation was done in 65 patients and chemotherapy in 33. Surviving patients have been followed from 12-197 months with a median of 54 months. RESULTS: The 5-year actuarial survival and event-free survival are 56% and 38%, respectively. Thirty-four patients relapsed from 3-72 months after diagnosis (median 25 months). In 20 patients, the only site of failure was the original tumor site. Three patients failed locally and at distance, while 10 others failed only at distance. Survival at 5 years was significantly better for patients who had complete resection of the tumor (75% vs. 41%, p = 0.001) and for those who received radiation therapy (63% vs. 23%, p = 0.003). Event-free survival at 5 years was superior in patients with complete resection of the tumor (51% vs. 26%, p = 0.002) and in patients who received radiation therapy (45% vs. 0%, p < 0.001). Sex and tumor site had no impact on survival or event-free survival. There was no difference in survival, event-free survival, or pattern of failure between patients treated with local field, whole brain or craniospinal irradiation, while severe longterm sequelae were noted predominantly in the latter two groups. CONCLUSION: Considering that failures were predominantly local and that there was no apparent benefit from prophylactic irradiation, we recommend local field irradiation with doses above 50.0 Gy for all children with intracranial ependymomas, without meningeal dissemination at diagnosis. Special considerations are necessary for children < 3 years of age.     

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.     

Effect of negative middle-ear pressure on transient-evoked otoacoustic emissions

OBJECTIVE: To seek an optimal treatment plan from the results of treatment for metastatic disease of the spine in children. DESIGN: An 8-year retrospective study of children with metastatic disease of the spine. Imaging studies were reviewed and treatment modalities analysed. SETTING: The divisions of pediatric orthopedics and pediatric neurosurgery at the Children's Hospital of Eastern Ontario, Ottawa. PATIENTS: All children seen between April 1980 and December 1987 who had lesions metastatic to the spine by hematogenous or direct extension. There were 20 children (15 boys, 5 girls) with a mean age at the time of diagnosis of 9.5 years. Follow-up ranged from 2 weeks to 108 months. One child was lost to follow-up. INTERVENTIONS: Eleven children underwent laminectomy and decompression. Of the 14 neurologically compromised children, 5 received chemotherapy and radiotherapy and 9 received chemotherapy, radiotherapy and surgery. MAIN OUTCOME MEASURES: Type of metastatic lesion, vertebrae involved and response to therapy. RESULTS: Vertebrae involved with metastases were as follows: cervical (3), thoracic (5), lumbar (8) and multilevel (2). Meninges were involved in 2 cases. The most common causes of metastatic spinal involvement were neuroblastoma (4 cases) and astrocytoma (6 cases). Pathologic fractures occurred in 4 children and kyphoscoliosis in 4. Spinal cord paresis developed in 14 of the 20 children. Of the 6 children who survived from 48 to 108 months, 5 had tumours of neural origin, 4 being astrocytomas. Children with neuroblastoma or leukemic infiltration had a good initial response to chemotherapy. Five of the 6 surviving children had astrocytomas, and 5 were treated by surgical decompression. CONCLUSIONS: Metastatic disease of the spine in children secondary to astrocytoma should be treated aggressively, but from the experience gained from this study it is impossible to devise a rigid treatment plan for each type of metastatic tumour. The choice of chemotherapy, radiotherapy or surgery depends on the type of tumour, the age of the child and whether or not the spinal cord is compromised.     

Extra osseous tumors of the spine in children and adolescents. Spinal complications

PURPOSE OF THE STUDY: The delay in diagnosis of spinal tumors is not rare. The chief complaint may include pain, walking disability, and spinal or limb deformities. The purpose of our study is to analyze the spinal deformities associated with non osseous intraspinal tumors, to assess the complications of treatment, and to set out a preventive protocol. MATERIALS: We reviewed retrospectively 40 consecutive cases of non osseous intraspinal tumors treated between 1972 and 1991. There were 32 intradural, 2 extradural, and 6 intra and extradural combined tumors. At the first consultation, the age ranged between 4 months and 15 years, and only 16 patients showed neurologic deficit. Spinal deformity existed in 9 patients, 7 of which had no neurologic deficit. All the tumors were treated surgically. Laminectomy was done in 28 cases, and osteoplastic laminotomy in 12 cases. The number of levels included in the laminectomy ranged from 3 to 18. Bilateral arthrectomy at least at one level was undertaken in 15 cases. A postoperative brace was worn in all cases for an average period of 4 months. An adjuvant radiotherapy was undertaken in 12 cases for an incomplete resection. METHODS: The incidence and pattern of spinal deformity was assessed before tumor treatment and ultimately after laminectomy or osteoplastic laminotomy (or laminoplasty). RESULTS: Among the 9 cases with preexisting spinal deformity, the curve magnitude increased after laminectomy in 4. A kyphotic, kyphosoliotic or scoliotic deformity developed in 18 cases after surgery for tumor resection. Among these 18 patients, only one had had an adequate osteoplastic laminotomy. The treatment of spinal deformities was surgical in 12 cases, and done by either posterior or anterior and posterior combined arthrodesis. DISCUSSION: Spinal deformity may be the main complaint of a patient who has intraspinal tumor. Prevention of postlaminectomy spinal deformity is mandatory, and could be done by osteoplastic laminotomy and the use of a brace during a minimum period of 4 to 6 months after surgery. CONCLUSION: Diagnosis of intraspinal tumors in children and adolescents should be done early, and laminoarthrectomy should be replaced by osteoplastic laminotomy.     

Randomized trial of epirubicin and cisplatin chemotherapy followed by pelvic radiation in locally advanced cervical cancer. Cervical Cancer Study Group of the Asian Oceanian Clinical Oncology Association

PURPOSE: Pelvic radiation is standard treatment for women with stage IIb to IVa cervical cancer, but treatment results are disappointing, particularly for women with bulky tumors. We investigated the role of primary chemotherapy followed by pelvic radiotherapy in a randomized trial. PATIENTS AND METHODS: Two hundred sixty patients with stage IIb and IVa cervical cancer received either standard pelvic radiotherapy or primary chemotherapy with cisplatin 60 mg/m2 and epirubicin 110 mg/m2 administered at 3-week intervals for three cycles, followed by pelvic radiotherapy. RESULTS: Ninety-nine patients have relapsed with a median follow-up duration of 1.3 years; in 62 patients, the first site of progressive disease was the pelvis. Patients who received primary chemotherapy had a significantly higher pelvic failure rate than those who received radiotherapy alone (P < .003). Seventy-six patients have died, and those who received primary chemotherapy had significantly inferior survival compared with those who received radiotherapy alone (P = .02). Tumor response following chemotherapy was observed in 63%. After radiotherapy, tumor response occurred in 72% of those who received combined modality treatment, compared with 92% of those who received radiotherapy alone. CONCLUSION: Primary chemotherapy with epirubicin and cisplatin, although resulting in tumor response in a significant proportion of patients, is accompanied by an inferior local control rate and survival compared with standard pelvic radiotherapy alone.     

Primitive cerebral neuroectodermal tumors excluding medulloblastomas: a retrospective study of 30 cases

We present a retrospective study of 30 cases of primitive cerebral neuroectodermal tumors (PNET), excluding medulloblastomas, referred to us postoperatively for additional therapy to evaluate prognostic factors and treatment efficiency. The histologic types were: pinealoblastomas (n = 7); ependymoblastomas (n = 2); medulloepitheliomas (n = 4), and other PNET (n = 17). The tumor was located in the supratentorial area in 24 patients and in the posterior fossa in 6 patients. Among the supratentorial tumors, 8 were metastatic. Maximal surgical resection was performed. Sixteen of 30 patients had no measurable disease after surgery and were considered as standard-risk (SR) cases, and 14 with a local residue or metastasis as high-risk (HR) cases. The objective of postsurgical treatment was to avoid radiotherapy in children below 4 years of age. It consisted of radiotherapy alone in 6 patients, chemotherapy alone in 17, and radiotherapy with chemotherapy in 7. Furthermore, high-dose chemotherapy (busulfan, thiotepa) and autologous bone marrow transplantation, performed in 6 patients, yielded a response rate of 3/6. Event-free survival (EFS) of SR patients was 37% at 3 years (95% confidence interval (CI) 14-60%) and overall survival 44% (95% CI 26-62%). Only 1 of the HR patients achieved a complete remission and all of them died early. The critical prognostic factors appear to be the completeness of initial surgical resection and absence of metastasis. These tumors have a poor prognosis. Novel strategies (high-dose chemotherapy) are needed to improve their outcome because the children concerned are very young and the effects of radiotherapy are particularly deleterious when tumors are situated in the supratentorial area.     

Neurovascular considerations in surgery of glomus tumors with intracranial extensions

Paragangliomas of the skull base, by virtue of their location, locally infiltrative behavior, and vascular nature, are difficult tumors to resect. Surgical removal is especially complicated when intracranial extensions are encountered. Our experience with a one-stage resection of intracranial extensions of glomus tumors in 20 patients is presented. These 20 patients had a total of 29 paragangliomas: 23 glomus jugulare or tympanicum tumors, 5 carotid body tumors, and 1 pterygopalatine lesion. Ten patients had intradural extension; the other 10 had intracranial extradural tumors. The primary complicating treatment factor was the loss of surgical planes in 6 patients with prior surgery and or radiotherapy. The presence of multiple paragangliomas (20%) and catecholamine secretion by the tumors (15%) complicated surgical treatment as well. Surgical morbidity was primarily related to deficits of lower cranial nerves (50%).     

Combined neck dissection and postoperative radiation therapy in the management of the high-risk neck: a matched-pair analysis

PURPOSE: Our purpose was to determine the sensitivity, specificity, and receiver operator characteristic (ROC) curve of a fast screening MR protocol in children and adolescents with suspected intracranial tumors. METHODS: One hundred forty-one patients (mean age, 9.7 years; range, 2 months to 23.5 years) with suspected brain tumor were entered in a case-control study. Eighty-seven patients had intracranial tumors (31 suprasellar/hypothalamic, 27 supratentorial, 26 infratentorial, and three pineal) and 54 patients in the control group had other disorders. Two neuroradiologists reviewed blindly a detailed three-sequence conventional protocol (acquisition time, 8 minutes 27 seconds) and a two-sequence fast screening MR protocol (acquisition time, 4 minutes 44 seconds). RESULTS: Sensitivity and specificity of the fast screening protocol for intracranial tumors was 100% and 92.6%, respectively. The areas under the ROC curves were 0.966 for the fast screening and 0.980 for the conventional MR protocol. No diagnostic performance difference was found between the ROC curves using the Az index. A kappa statistic of .93 for both examinations indicated excellent interobserver agreement. Additional MR sequences and other neuroimaging studies were not deemed necessary to exclude the presence of an intracranial tumor. CONCLUSION: A fast dual-plane brain MR protocol may be adequate to screen children and adolescents thought to have an intracranial tumor. The less than 5 minute acquisition time allows a complete examination (including preparation) to be performed in 10 to 15 minutes. Future studies are recommended before this time-efficient neuroimaging examination is incorporated into clinical practice.     

Multiple sclerosis of the spinal cord: magnetic resonance appearance

OBJECTIVE: To determine the MR appearance of spinal cord multiple sclerosis (MS) plaques in patients presenting with myelopathy by using a high-field (1.5 T) imager. MATERIALS AND METHODS: We studied 119 patients who underwent high-field (1.5 T) MR studies of the spinal cord for evaluation of myelopathy. All 119 patients were thought to have possible findings of spinal cord MS at the time of the MRI interpretation. RESULTS: Sixty-four plaques were studied in 47 patients with clinically definite MS and adequate quality MRI. Of these patients 68% had a single spinal cord plaque, 19% had two plaques, and 13% had three or more plaques. Sixty-two percent of the plaques occurred in the cervical spinal cord and most frequently involved the posterior (41%) and lateral (25%) aspects of the spinal cord. None of the 64 lesions involved the entire thickness of the spinal cord. The lesion length varied from 2 to 60 mm, with 84% of the lesions < 15 mm in length. The spinal cord diameter was unchanged in 84% of plaques, enlarged at the level of the lesion in 14%, and atrophic in 2%. Just over half (55%) of the plaques enhanced with intravenously administered gadolinium. Of the patients who received synchronous head and spinal cord examinations on the same day, 24% had normal findings on the MR study of the head. Follow-up spinal cord studies were available in nine patients. New lesions developed in two patients, while previously described lesions resolved. In three patients only new lesions developed. In four patients no change occurred in the existing number of cord plaques. CONCLUSION: Spinal cord demyelinating plaques present as well-circumscribed foci of increased T2 signal that asymmetrically involve the spinal cord parenchyma. Knowledge of their usual appearance may prevent unnecessary biopsy. An MR examination of the head may confirm the imaging suggestion of spinal cord demyelinating disease, because up to 76% of patients have abnormal intracranial findings. In the remaining 24% of cases in which the clinical diagnosis is not certain and MR findings in the head are negative, a follow-up spinal cord study is recommended, because these lesions evolve and change over time.     

Prognostic factors in anterior decompression for metastatic cord compression. An analysis of results

During the 10-year period 1981-1990, 59 patients suffering from spinal cord or cauda equina compression underwent anterior spinal decompression and in most cases spinal restabilization with methylmethacrylate cement and/or instrumentation. Follow-up in 55 patients showed that 75% were improved neurologically by the procedure, one-third of these showing complete recovery from the spinal cord compression. The results in younger female patients suffering from metastatic breast cancer were considerably better than those of older men with prostatic metastases. Other genitourinary system tumors also had a relatively poorer prognosis. There was a significantly better result for metastatic lesions of the thoracic or thoracolumbar spine than for lesions in the lumbar spine causing cauda equina paralysis.     

Mammographic findings of recurrent breast cancer after lumpectomy and radiation therapy: comparison with the primary tumor

PURPOSE: To compare the mammographic findings of recurrent breast cancer with those of the primary tumor in patients who underwent lumpectomy and radiation therapy. MATERIALS AND METHODS: Mammograms were reviewed of primary and recurrent tumors in 25 patients (26 lesions). Mammographic appearance, location, and histopathologic characteristics were retrospectively compared between primary and recurrent tumors. RESULTS: Primary and recurrent tumors were mammographically similar in 21 (81%) of the 26 lesions. Of 14 primary tumors with calcifications, 12 (86%) recurred with calcifications, and of the 12 masses, nine (75%) recurred as masses. Recurrent tumors that occurred in the lumpectomy quadrant were more often similar in mammographic appearance to the primary tumor (20 of 22 tumors) than those in other quadrants (one of four tumors) (P < .02). CONCLUSION: After conservative treatment of breast cancer, the majority of recurrent tumors appear to be mammographically similar to primary tumors. It is prudent to review preoperative mammograms during follow-up of patients after lumpectomy and radiation therapy.     

Brain metastases as the only manifestation of an undetected primary tumor

BACKGROUND: The prognosis of patients with brain metastasis as the only manifestation of an undetected primary tumor generally is considered to be poor. Therefore, most treatment is palliative. The authors reviewed the clinical outcomes and treatment results of patients presenting with brain metastasis from an undetected primary tumor at The University of Texas M. D. Anderson Cancer Center. METHODS: Between 1977-1996, 220 patients were referred to the study department for the treatment of brain metastasis from an undetected primary tumor. The patients' records were reviewed to identify those for whom brain metastasis was the only manifestation of the primary tumor. The majority of patients were excluded from the current analysis because extracranial metastasis also were present. Thirty-nine patients qualified for this retrospective review. The level of neurosurgical excision varied, but all patients received radiotherapy. Tumor control in the brain and survival were analyzed by various tumor-related and treatment-related factors. RESULTS: In 31 patients, the brain metastasis were adenocarcinomas, whereas the remaining patients had tumors of various other histologies. In 12 patients, the primary tumor eventually was found, most commonly in the lung. The median survival time for all patients was 13.4 months. Overall survival rates (OS) at 1, 3, and 5 years were 56%, 19%, and 15%, respectively. Intracranial disease control was 72% at 5 years. Patients who received gross total resection (GTR) and radiotherapy had significantly better OS than patients who received radiotherapy alone. The OS of patients whose primary tumor was identified was similar to that of patients in whom the primary tumor remained occult. CONCLUSIONS: Brain metastasis as the only manifestation of an unknown primary tumor is a distinct clinical entity. The prognosis for patients with this presentation is better than that of patients with brain metastasis in general. Although the majority of patients die of extracranial disease, a few will achieve long term survival. Treatment to the brain is effective in controlling local disease; aggressive treatment with GTR and radiotherapy is recommended.     

The fate of patients with locally advanced bladder cancer treated conservatively with neoadjuvant chemotherapy, extensive transurethral resection and radiotherapy: 10-year experience

PURPOSE: We assess the results of bladder preservation for infiltrating bladder cancer. The potential for neoadjuvant chemotherapy followed by extensive transurethral resection and radiotherapy was evaluated in 40 patients with T2-T4a G2-G3 bladder carcinoma. MATERIALS AND METHODS: From 1983 to 1995, 40 patients with bladder cancer underwent bladder sparing treatment, consisting of neoadjuvant chemotherapy, extensive transurethral resection and radiotherapy. Most patients had T3G3 cancer. A deep transurethral resection biopsy was performed before and after chemotherapy, and an extensive transurethral resection was repeated at the end of radiotherapy. Of the patients 30 received cisplatin and methotrexate and 10 also received vinblastine. Total dose of radiotherapy was 60 to 65 Gy. Recurrent superficial tumors were treated transurethrally. Radical cystectomy was considered for persistent or recurrent invasive disease. RESULTS: Complete response occurred in 19 patients (47.5%) after chemotherapy, and in 8 patients after transurethral resection and radiotherapy (67.5%). Within 10 years 8 responding patients (30%) had local recurrences and 3 underwent cystectomy. Of the patients 14 (35%) are alive, including 13 with no evidence of disease (mean survival 65 months), 5 died of unrelated disease and 21 (52.5%) died of distant metastases (mean survival 28 months). Of the 21 patients 14 had residual tumor after radiotherapy, 3 presented with distant metastases after vesical infiltrating recurrence and 4 had distant metastases in the absence of locoregional recurrence. In 22 patients (55%) the bladder was salvaged. Patients with complete response to chemotherapy had a low risk for recurrent infiltrating tumors and metastases. CONCLUSIONS: Complete tumor control was maintained at 5 years in more than 50% of the patients treated conservatively. Bladder salvage is feasible in select patients.     

Primary transitional cell carcinoma in vesical diverticula

OBJECTIVES: To evaluate the treatment and prognosis of primary tumors in bladder diverticula. METHODS: The cases of 611 patients treated for bladder tumors at a single medical center were retrospectively reviewed. RESULTS: Eight patients had primary intradiverticular transitional cell carcinoma. Five patients had Stage Ta tumor, and 3 had Stage T1 tumor. Most patients were treated by local resection and adjuvant intravesical chemotherapy. All patients with initial Ta disease are disease free at the time of this writing. One patient with T1 disease died, 1 patient's disease recurred several times, and 1 patient showed positive cytology without apparent disease. CONCLUSIONS: Superficial intradiverticular tumors may be treated conservatively. Routine cystoscopy for patients with a bladder diverticulum is warranted for early diagnosis of possible intradiverticular tumor.     

Microsurgery of intramedullary cervical cord tumor

OBJECTIVE: To evaluate whether intramedullary tumor of the cervical spinal cord is amenable to aggressive surgery and to clarify surgical timing and important points for tumor removal. PATIENTS AND METHODS: Fifty-eight patients with intramedullary tumor of the cervical spinal cord were examined by magnetic resonance imaging and treated by microsurgery in Huashan Hospital between May 1988 and December 1994. The results were analysed by F or Chi square tests. RESULTS: Tumors were totally resected in 50 cases (86.2%), subtotally resected in 7 and partially resected in 1. Forty-five (77.6%) patients had their neurological status improved postoperatively. Most patients with moderate neurological deficit can recover remarkably after total tumor removal. Laser surgery is especially helpful for treating lipoma. CONCLUSIONS: Intramedullary tumor of the cervical spinal cord is amenable to total tumor removal. Operation is suitable when a patient presents moderate neurological deficit. Proficient surgical technique and standards for total tumor resection are essential for good results. Preoperative radiotherapy contributes to difficult surgery and poor prognosis, and is not recommended.     

Chemotherapy for spinal cord astrocytoma: can natural history be modified?

Standard treatment of spinal cord astrocytomas is based upon surgery, followed by radiotherapy when resection is incomplete or when histology is of high grade. Owing to the major consequences of radiotherapy on the spine in childhood, alternative therapies must be explored. The potential role of chemotherapy in the management of spinal cord astrocytoma remains to be defined. Two patients are described. The first was a 19-month-old child with an anaplastic astrocytoma of the cervical spinal cord that progressed rapidly after initial partial resection. Chemotherapy was begun according to the UKCCSG Baby Brain Protocol, with marked clinical improvement. Reassessment by MRI at 4 months showed improvement, and at the end of treatment no evaluable disease remained. The second was a 4-year-old child with a recurrent low-grade astrocytoma. Chemotherapy according to the SIOP Protocol for Low Grade Gliomas was administered for 3 months, after which marked tumour regression was seen, with neurological recovery. These patients demonstrate the potential value and low morbidity of chemotherapy in spinal cord astrocytoma. The management of this rare tumour is discussed.     

En bloc resections of bone tumors of the thoracolumbar spine. A preliminary report on 29 patients

STUDY DESIGN: Twenty-nine patients with primary bone tumors and solitary metastases of the thoracolumbar spine treated with en bloc resection are reviewed retrospectively. OBJECTIVE: To demonstrate the possibility to apply in the spine the same principles of surgical oncology adopted for primary bone tumors of the limbs. SUMMARY OF BACKGROUND DATA: The surgical oncologic staging systems currently applied in limb tumor surgery are difficult to apply to spinal tumors. The anatomic conditions make extralesional surgery difficult or impossible, which has restrained a more common use of resection surgery in the spine. Focus is put on a new surgical staging system and en bloc vertebral resection. METHODS: Twenty-five primary malignant and aggressive benign bone tumors and four solitary metastases were treated. The patients were submitted to oncologic and surgical staging for surgical planning. The primary tumors were classified according to Enneking system: three Stage IA, six Stage IB, eight Stage IIB, eight Stage 3 benign. Staging according to the Weinstein-Boriani-Biagini system was also done. Thirteen lesions involved the vertebral body; nine lesions developed in the posterior arch, or part of it, and seven lesions occupied part of the body and part of the arch. A careful anesthesiologic evaluation was performed as well as a continuous intraoperative on-line monitoring of the vital parameters. The en bloc resections (multisegmental in five patients) were performed in 10 thoracic, in 16 lumbar, in two thoracolumbar lesions, and in one lumbosacral lesion. Reconstruction was performed, aiming to replace the resected columns. The specimens were submitted to histologic study of the margins. All the patients were followed, and their status was defined on clinical and imaging studies. RESULTS: In 20 patients, a wide margin was achieved, in eight a marginal margin, in one an intralesional margin. The margin was contaminated in seven patients. Surgical time was 3-21 hours (average, 12 hours). No patient died during surgery or from surgical complications. Three mechanical failures of the implants required additional surgery. One deep infection arose. The only neurologic problems observed were related to the nerve roots sectioned for oncologic purpose. No local recurrence was found at follow-up evaluation after 6-134 months (average, 30 months). CONCLUSIONS: En bloc resection can be performed in selected tumors of the spine; the indication to such major surgery must be based on the oncologic stage, and the procedure must be carefully planned. For this purpose, the Weinstein-Boriani-Biagini system could be a helpful tool. Long-term results must be weighed before a definitive statement of the indications can be made.     

Intramedullary spinal cord ependymomas in children: treatment, results and follow-up

A retrospective review of 20 pediatric patients with intramedullary spinal cord ependymomas, all of whom underwent operative resection between 1985 and 1996, was undertaken to determine surgical results, long-term follow-up and tumor recurrence. Twelve children operated on in the same period with filum or cauda equina ependymomas were not included in this study. Nine children had had previous treatment before referral. Gross total resection was achieved in 14 patients and subtotal in 6. None of these had a post-operative radiation therapy. The median follow-up period was 67 months (range 25-177 months). All children were clinically evaluated before and after operation and at the last follow-up. The clinical grade at the last follow-up showed improvement in 8 patients (40%), was unchanged in 10 (50%) and deteriorated in 2 (10%). Three patients had a recurrence, 2 at the primary site (2 and 3 years after our surgery) and 1 at a distant site (3 years after). The actuarial 5- and 10-year survival rates were both 90%; 5- and 10-year progression-free survival rates were 93 and 70%, respectively. We conclude that a complete removal can be achieved in almost all cases of intramedullary spinal cord ependymomas in children, and that the long survival rates justify avoiding post-operative radiation therapy.     

Malignant vascular tumors in young patients

BACKGROUND: To the authors' knowledge there are few published series of malignant vascular tumors in patients age < or = 21 years. METHODS: The authors retrospectively documented the clinical presentation, pathology, treatment, and outcome of patients age < or = 21 years with malignant vascular tumors treated between 1970-1995 at Memorial Sloan-Kettering Cancer Center. The histologic sections were rereviewed to confirm the diagnosis. RESULTS: Four patients were identified with angiosarcoma and two with malignant hemangioendothelioma. Five patients were female and one was male; the median age at diagnosis was 11.8 years (range, 8 months-21 years). The tumor involved the skin in one patient, soft tissue in one patient, bone in two patients, and internal organs in two patients. One patient had associated diffuse angiomatosis and another had the Klippel-Trenaunay-Weber syndrome. This patient received prior radiation therapy to the primary site with the subsequent development of a vascular sarcoma. None of the patients had distant metastases at diagnosis. Resection was attempted in five patients and completed in four. Chemotherapy alone was given to three patients whereas radiation therapy alone and radiation plus chemotherapy were administered to one patient each. The median follow-up was 4.9 years (range, 1 month-12 years). There were three deaths from progressive disease and two deaths from locoregional recurrences. Reexcision and radiotherapy controlled one local recurrence. Another patient developed recurrence to regional lymph nodes and further dissemination. The primary tumor in all three survivors was excised completely whereas two of the three patients who died of progressive disease underwent an incomplete excision or biopsy alone. CONCLUSIONS: Malignant vascular tumors are rare in the first two decades of life and when they do occur are very aggressive. Complete resection is curative for patients with localized lesions.