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Radiotherapy for large symptomatic hemangiomas.

Between 1974 and 1988, 13 patients presented with large (3 to greater than 20 cm) symptomatic unresectable or partially resected hemangiomas. Tumor sites were extremities (five cases), vertebral bodies (three cases), face (two cases), pituitary fossa (one case), pelvic bones (one case), and bladder (one case). Symptoms included severe pain in eight patients, inability to use an extremity in five, vision problems in three, weakness in two, and hematuria with decreased urinary stream in one. Many patients had more than one symptom. Two of the cases were associated with life-threatening consumptive coagulopathies (Kasabach-Merritt syndrome). Therapy was delivered with both orthovoltage and megavoltage photons. Doses ranged from 6.25 to 40.0 Gy in 1.6- to 2.5-Gy fractions. Follow-up ranged from 2 to 15 years (median, 13 years). Tumor shrinkage was noted in 9 (82%) of the 11 cases in which tumor size data were collected before and after therapy. Complete response of tumor mass occurred in 4 (36%) of 11, partial response occurred in 5 (45%), and no response occurred in 2 (18%). No tumor grew after radiotherapy. Some relief in symptoms occurred in all 13 patients; 10 (77%) had complete resolution of symptoms. In both patients with life-threatening cytopenias, hematologic values returned to normal after treatment. Because the majority of the patients responded to all dose levels administered, no firm dose-response relationship was evident. However, objective (measurable) complete responses occurred in two (50%) of the four patients receiving doses of 30 Gy or greater compared with only two (29%) of the seven patients who received lesser doses. No long-term morbidity occurred. Radiotherapy of large unresectable and partially resected hemangiomas yields long-term relief of symptoms and tumor shrinkage in the majority of patients treated.

Adolescent↗

Base of skull and cervical spine chordomas in children treated by high-dose irradiation.

PURPOSE: To evaluate the outcome of children with base of skull or cervical spine chordomas treated by high dose irradiation. METHODS AND MATERIALS: Eighteen children, 4 to 18 years of age, with base of skull or cervical spine chordomas, received fractionated high-dose postoperative radiation using mixed photon and 160 MeV proton beams. The median tumor dose was 69 Cobalt Gray-equivalent (CGE) with a 1.8 CGE daily fraction. RESULTS: The median follow-up was 72 months. The 5-year actuarial survival was 68% and the 5-year disease-free survival (DFS) was 63%. The only significant prognostic factor was the location: patients with cervical spine chordomas had a worse survival than those with base of skull lesions (p = 0.008). The incidence of treatment-related morbidity was acceptable: two patients developed a growth hormone deficit corrected by hormone replacement, one temporal lobe necrosis, and one fibrosis of the temporalis muscle, improved by surgery. CONCLUSION: Chordomas in children behave similarly to those in adults: children can receive the same high-dose irradiation as adults with acceptable morbidity.

Adolescent↗

Radiation therapy for chordomas of the base of skull and cervical spine: patterns of failure and outcome after relapse.

PURPOSE: To determine the patterns of failure and outcome following relapse of chordomas of the base of skull and cervical spine. METHODS AND MATERIALS: Between November 1975 and October 1993, 204 patients were treated for chordoma of the base of skull or cervical spine, of which 63 have developed relapse. These 63 patients constitute the main focus of this study. Forty-five patients presented with base of skull and 18 with cervical spine tumors. All patients received combined proton and photon beam radiation. The median prescribed dose was 70.1 cobalt-Gray equivalent (CGE) (range 66.6-77.4). There were 25 males and 38 females, with a median age of 41 years (range 7-66). Median follow-up was 54 months (range 8-158). RESULTS: Sixty-three of the 204 patients treated (31%) had treatment failure. Among the 63 patients who relapsed, 60 (95%) experienced local recurrence, and in 49 patients (78%), this was the only site of failure. Two of 63 patients (3%) developed regional lymph node relapse and 3 of 63 (5%) developed surgical pathway recurrence (1 left neck, 1 palate and 1 nasal cavity). Thirteen of 204 patients relapsed in distant sites, accounting for 20% (13 of 63) of all patients with recurrence in this series. The most common metastatic sites were lungs and bones presenting in 7 of 13 and 6 of 13 patients, respectively. Only 2 of 13 patients failed with isolated distant metastasis. The actuarial 3- and 5-year survival rates after local relapse (60 patients) were 44 and 5%, respectively. Following distant failure (13 patients), the 3- and 5-year survival rates were 25 and 12%, respectively. After any relapse (63 patients) the corresponding survival rates were 43 and 7%. Following local relapse, 49 of 60 patients underwent salvage therapy consisting of subtotal resection in most patients (46 of 49). The remaining 11 of 60 patients received supportive care only. Salvage therapy resulted in stable or improved status without subsequent disease progression in 26 of 49 (53%), and progressive disease in 16 of 49 patients (33%). The actuarial 2- and 5-year overall survival rates following relapse for the 49 patients who underwent salvage treatment were 63 and 6%, which favorably compared to the 2-year survival rate of 21% for those who received supportive care only (p = 0.001). CONCLUSION: Local relapse is the predominant type of treatment failure for chordomas of the base of skull and cervical spine. Salvage treatment may relieve symptoms; however, most patients will ultimately succumb to their disease. Poor long-term survival rates following relapse emphasize the importance of a combined treatment approach with experienced surgeons and radiation oncologists at the time of primary treatment. For most patients, only permanent local tumor control will offer a chance of cure.

Adolescent↗

Computerized tomography in the assessment of diseases of the thorax.

Many of the diseases of the chest wall, heart, mediastinum, pleura and lungs can be successfully imaged by computerized tomography. Radiation therapy ports can be calculated and transcutaneous biopsies monitored. This paper deals with the many uses that we have had for computerized tomography in assessing diseases within the chest and thorax.

Adult↗

Computed tomography of the spinal canal and cord.

Selective cases have been presented to demonstrate the usefulness of computed tomography in disorders of the spine and spinal cord. The osseous structures are more readily demonstrated than the soft tissues. The cord is difficult and inconsistently identified. However, cystic lesions (syringomyelia) may, at times, be demonstrated. The transverse projection of the CT scan is an added benefit in demonstrating bony spinal lesions. The use of metrizamide with faster scanners should improve the usefulness of this technique in evaluating intraspinal lesions.

Adult↗

Computed tomography in the diagnosis and management of paravertebral masses.

The role of computed tomography (CT) in the detection, evaluation, and management of paravertebral masses was studied in 32 consecutive patients with such lesions. The characteristics of the patients and masses were tabulated, and follow-up data were collected. There were 25 neoplasms (of which 24 were malignant), 5 inflammatory lesions, and two cases without proven diagnosis. The CT provided unique diagnostic information in 9 of the 32 cases (28%), and information that affected patient management in 13 cases (41%). Overall, CT proved of benefit in 19 of the 32 patients (59%). These findings indicate that CT can make important contributions to the diagnosis and management of paravertebral masses.

Adolescent↗

[Chordomas of the base of the skull and upper cervical spine. 100 patients irradiated by a 3D conformal technique combining photon and proton beams].

OBJECTIVE: To define prognostic factors for local control and survival in 100 consecutive patients treated by fractionated photon and proton radiation for chordoma of the skull base and upper cervical spine. PATIENTS AND METHODS: Between December 1995 and August 2002, 100 patients (median age: 53 years, range: 8-85, M/F sex-ratio: 3/2), were treated by a combination of high-energy photons and protons. The proton component was delivered by the 201 MeV proton beam of the Centre de Protonthérapie d'Orsay (CPO). The median total dose delivered to the gross tumour volume was 67 Cobalt Gray Equivalent (CGE) (range: 60-71). A complete surgery, incomplete surgery or a biopsy was performed before the radiotherapy in 16, 75 and 9 cases, respectively. RESULTS: With a median follow-up of 31 months (range: 1-87), 25 tumours failed locally. The 2 and 4-year local control rates were 86.3% (+/-3.9%) and 53.8% (+/-7.5%), respectively. According to multivariate analysis, less than 95% of the tumour volume encompassed by the 95% isodose line (P=0.048; RR: 3.4 IC95% [1.01-11.8]) and a minimal dose less than 56 CGE (p=0.042; RR: 2.3 IC95% [1.03-5.2]) were independent prognostic factors of local control. Ten patients died. The 2 and 5-year overall survival rates were 94.3% (+/-2.5%) and 80.5% (+/-7.2%). According to multivariate analysis, a controlled tumour (P=0.005; RR: 21 IC95% [2.2-200]) was the lonely independent favourable prognostic factor for overall survival. CONCLUSION: In chordomas of the skull base and upper cervical spine treated by surgical resection followed by high-dose photon and proton irradiation, local control is mainly dependent on the quality of radiation, especially dose-uniformity within the gross tumour volume. Special attention must be paid to minimise underdosed areas due to the close proximity of critical structures and possibly escalate dose-constraints to tumour targets in future studies, in view of the low toxicity observed to date.

Adolescent↗

Image-guided radiotherapy using megavoltage cone-beam computed tomography for treatment of paraspinous tumors in the presence of orthopedic hardware.

PURPOSE: This report describes a new image-guided radiotherapy (IGRT) technique using megavoltage cone-beam computed tomography (MV-CBCT) to treat paraspinous tumors in the presence of orthopedic hardware. METHODS AND MATERIALS: A patient with a resected paraspinous high-grade sarcoma was treated to 59.4 Gy with an IMRT plan. Daily MV-CBCT imaging was used to ensure accurate positioning. The displacement between MV-CBCT and planning CT images were determined daily and applied remotely to the treatment couch. The dose-volume histograms of the original and a hypothetical IMRT plan (shifted by the average daily setup errors) were compared to estimate the impact on dosimetry. RESULTS: The mean setup corrections in the lateral, longitudinal, and vertical directions were 3.6 mm (95% CI, 2.6-4.6 mm), 4.1 mm (95% CI, 3.2-5.0 mm), and 1.0 mm (95% CI, 0.6-1.3 mm), respectively. Without corrected positioning, the dose to 0.1 cc of the spinal cord increased by 9.4 Gy, and the doses to 95% of clinical target volumes 1 and 2 were reduced by 4 Gy and 4.8 Gy, respectively. CONCLUSIONS: Megavoltage-CBCT provides a new alternative image-guided radiotherapy approach for treatment of paraspinous tumors in the presence of orthopedic hardware by providing 3D anatomic information in the treatment position, with clear imaging of metallic objects and without compromising soft-tissue information.

Female↗

Chordoma cutis.

Chordomas are rare malignant primary bone tumors, which most often occur in the sacral area. These tumors uncommonly affect the skin, and may not be recognized by dermatologists. We present a case of an adult woman with cutaneous metastasis of a primary sacral chordoma.

Aged↗

Imaging of a thoracic spinal nerve haemangioblastoma by three-dimensional digital angiography.

Haemangioblastomas occur sporadically or in association with Von Hippel-Lindau (VHL) disease. Occasionally, they are associated with intrathecal cauda equina nerve roots and rarely with spinal nerve roots. The occurrence of a completely extrathecal, thoracic spinal nerve haemangioblastoma is exceptional, with only one case previously described in the English-language literature. We report an extradural thoracic spinal nerve haemangioblastoma in a patient with VHL disease causing a progressive thoracic radiculopathy. The advantages of three-dimensional digital angiography will be discussed. Three-dimensional reconstruction of the images acquired by rotational digital subtraction angiography is one of the most promising developments to improve surgical planning for complex vascular lesions.

Adult↗

Retroperitoneal fibrosis and ankylosing spondylitis: which links?

OBJECTIVES: To report a case of retroperitoneal fibrosis (RPF) in a patient with ankylosing spondylitis (AS) and to review the medical literature for similar cases to detect possible links between the 2 diseases. METHODS: The presentation, clinical course, diagnostic work-up, and treatment of our patient are described, and the English and French medical literature from 1960 to 2000 is reviewed using a MEDLINE search for cases with coexisting RPF and spondyloarthritis. RESULTS: Our patient with AS had RPF, which mimicked rectal cancer with retroperitoneal and vertebral metastases. Special attention is paid to the unusual clinical presentation, the multistep diagnostic process, and the therapeutic strategy which was both radiologically and histologically successful. Literature review revealed 18 cases of concomitant RPF and spondyloarthritis, mainly AS. Patients were more frequently male (M/F, 3/1) and developed spondyloarthritis several years before RPF. CONCLUSIONS: RPF may result from a local immune response to products of aortic atheromatous plaques, with subsequent periaortic deposition of fibrous tissue. However, the clinical features and the frequent association with other fibrosing disorders suggest that RPF is a systemic inflammatory condition. The role of AS-associated aortitis in the development of RPF warrants consideration.

Aortitis↗

Nuclear magnetic resonance in oncology.

The application of nuclear magnetic resonance (NMR) techniques to the diagnosis of cancer was first explored by Damadian, who proposed that benign and malignant tissues could be differentiated on the basis of characteristic differences in spin-lattice and spin-spin relaxation times (T1 and T2) as determined in vitro with NMR spectrometers. Damadian's thesis was very controversial and never gained widespread acceptance. Not all investigators were able to confirm his findings. Moreover, it was improbable that NMR would ever play an important role in the diagnosis of malignancy as long as biopsy was necessary to obtain material for analysis. However, the potential usefulness of NMR in oncology was enhanced considerably by the work of Lauterbur, who showed that NMR signals could be spatially encoded to produce images of the examined object. NMR imaging has made feasible the measurement of the T1 and T2 of lesions without biopsy. Unfortunately, initial efforts at characterizing tissues by in vivo determination of proton relaxation times have yielded disappointing results. Nonetheless, NMR imaging will be a powerful tool for evaluating patients with malignant disease because of the unique anatomic information it can provide without exposure of the patient to ionizing radiation. In vivo NMR spectroscopy of 31P and other sensitive nuclei may add a new dimension to clinical and experimental oncology.

Animals↗

Ewing's sarcoma presenting as a posterior mediastinal mass: a lesson learned.

Thoracic vertebral body hemicorpectomy and chest wall resection was performed in a 17-year-old male patient with a posterior mediastinal tumor thought to be neurogenic in origin. No preoperative tissue diagnostic endeavor was made. Final pathologic diagnosis showed this tumor to be Ewing's sarcoma. This communication alerts the thoracic surgeon to the need for definitive diagnosis of posterior mediastinal masses with vertebral body involvement, particularly in children. Induction chemotherapy is the accepted standard of management of these sarcomas.

Adolescent↗

Nontraumatic spinal cord injury: incidence, epidemiology, and functional outcome.

OBJECTIVES: To identify and compare the incidence, demographics, neurologic presentation, and functional outcome of individuals with nontraumatic spinal cord injury (SCI) to individuals with traumatic SCI. DESIGN: A 5-year prospective study. SETTING: Level I trauma center of a Regional SCI Model System. PATIENTS: Two hundred twenty adult SCI admissions. MAIN OUTCOME MEASURES: Demographics, etiology, level and completeness of injury, Functional Independent Measure (FIM) scores. RESULTS: Of SCI admissions, 39% were nontraumatic in etiology (spinal stenosis, 54%; tumor, 26%). Compared to subjects with traumatic SCI, those individuals with nontraumatic SCI were significantly (p < .01) older and were more likely married, female, and retired. Injury characteristics revealed significantly more paraplegia and incomplete SCI within the nontraumatic SCI group (p < .01). Both nontraumatic and traumatic SCI individuals had significant FIM changes from rehabilitation admission to discharge (p < .01). Those with tetraplegia-incomplete nontraumatic SCI had significantly higher admission motor FIM scores and shorter rehabilitation length of stay than in the traumatic group (p < .05). Paraplegic-complete and paraplegic-incomplete nontraumatic SCI subjects had lower discharge motor FIM scores, FIM change, and FIM efficiency than those with traumatic SCI. Similar discharge-to-home rates were noted in both nontraumatic and traumatic SCI groups. CONCLUSIONS: These data suggest that individuals with nontraumatic SCI represent a significant proportion of SCI rehabilitation admissions and, although differing from those with traumatic SCI in demographic and injury patterns, can achieve similar functional outcomes.

Adult↗