Thigh pain due to intraspinal neurilemmoma.
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PURPOSE: To retrospectively analyze the long term results of treatment and the patterns of failure for patients with chordoma of the sacrum, base of skull and mobile spine treated predominantly with postoperative photon irradiation. MATERIALS AND METHODS: Forty-eight adult patients with chordoma of the sacrum (23), base of skull (20) or mobile spine (5), were seen between 1958-1992. Forty-four were referred post operatively with overt disease and 31 of these were irradiated with conventionally fractionated radiation to a median dose of 50 Gy/25 fractions/5 weeks (range 25-60 Gy). Eight received a hyperfractionation protocol of 1 Gy, 4 hourly, 4 times a day (median 40 Gy/44 fractions/14 days), two sacral patients were treated with a hypofractionation protocol and three cases with skull base tumours were referred elsewhere for proton therapy. Endpoints measured were survival from diagnosis, objective response rate, symptomatic response rate and clinical or radiological progression-free survival from radiotherapy. RESULTS: Median survival was 62 months (range 4-240 month) from diagnosis with no difference between clival and non-clival presentations. One complete and no partial responses were identified in 23 assessable patients. A subjective response was recorded for 12/14 (85%) with pain and 10/23 (45%) with neurological signs or symptoms, and the median time to progression for those with overt disease was 35 months (range 5-220 months). There was no survival advantage to patients receiving radiation doses > 50 Gy (median 60 Gy) compared to doses < 50 Gy (median 40 Gy). There was no difference between the conventional or hyperfractionation regimens with respect to the degree or duration of symptomatic response, or in progression-free survival. Fourteen patients who progressed after irradiation were retreated with surgery (6), irradiation (7) or both modalities (1). Median survival after retreatment was 18 months, and the only two symptomatic responses seen were with reirradiation, and after failure of relatively low dose initial therapy. At last follow-up, 35 were dead of or with disease, seven are alive with disease, and two are disease-free. Thirty-eight had local disease persistence as the sole site of failure, and four developed distant metastases initially or subsequently. DISCUSSION: Overt residual chordoma is rarely cured with conventional external beam irradiation, but treatment does provide useful and prolonged palliation of pain for most patients. Chordoma is a disease with low metastatic potential, and better local control may improve survival. Complete resection rates may be improved for patients with sacral disease by using planned excisions in centres experienced in treating this rare disease. Because radiation therapy may prove to be more successful in controlling microscopic disease, it should be considered as a pre- or postoperative adjuvant to a macroscopically complete resection. Patients with skull base disease should also be resected in centres specializing in this surgery, but complete excision is unlikely. These patients will not obtain local control with conventional photon irradiation, and suitable patients should be considered for irradiation with stereotactic photon or particle beam therapy. For patients who progress after irradiation, there is limited symptomatic benefit to retreatment with surgery or reirradiation, and this should be limited to treating life-threatening complications.
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A paraganglioma is a rare tumor composed of chromaffin cells. Malignant paraganglioma is a very rare presentation diagnosed by local recurrence after total resection of the primary mass or findings of distant metastasis. We report a case of a 30-year-old man who had undergone a carotid body tumor resection 10 years before and who presented to us with recurrence of the tumor associated with lymph node, vertebra and skull metastasis. The diagnosis of malignant paraganglioma was based on loco-regional and distant metastasis.
INTRODUCTION: Due to the occurrence of osteoblastic metastases in the course of various cancers, particularly in the course of prostate cancer, we are faced with diagnosis and follow-up issues different from those associated with lytic metastasis. We therefore analyzed the respective advantages of imaging techniques. CURRENT KNOWLEDGE AND KEY POINTS: Most of the time, osteoblastic metastases are evidenced by standard radiography. Due to its ability to demonstrate metastases localization, extent and signs, CT scan is not only of value when osteoblastic metastases are suspected but also for patient's follow-up. MRI provides further information in regard to both the lesion content and osteoblastic degree. Though MRI must be performed after all other imaging procedures, it is of value for multiplanar study of the whole spine. FUTURE PROSPECTS AND PROJECTS: Studies focusing on either the lesion content and volume or helical CT are in progress and aim at better monitoring follow-up, while the objective of dynamic MRI studies is to better analyze lesion content.
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PURPOSE: Conformal and intensity-modulated radiotherapy (IMRT) plans for 9 patients were compared based on characterization of plan quality and effects on the oncology department. METHODS AND MATERIALS: These clinical cases, treated originally with conformal radiotherapy (CRT), required extraordinary effort to produce conformal treatment plans using nonmodulated, shaped noncoplanar fields with multileaf collimators (MLCs). IMRT plans created for comparison included rotational treatments with slit collimator, and fixed-field MLC treatments using equispaced coplanar, and noncoplanar fields. Plans were compared based upon target coverage, target conformality, dose homogeneity, monitor units (MU), user-interactive planning time, and treatment delivery time. The results were subjected to a statistical analysis. RESULTS: IMRT increased target coverage an average of 36% and conformality by 10%. Where dose escalation was a goal, IMRT increased mean dose by 4-6 Gy and target coverage by 19% with the same degree of conformality. Rotational IMRT was slightly superior to fixed-field IMRT. All IMRT techniques increased integral dose and target dose heterogeneity. IMRT planning times were significantly less, whereas MU increased significantly; estimated delivery times were similar. CONCLUSION: IMRT techniques increase dose and target coverage while continuing to spare organs-at-risk, and can be delivered in a time frame comparable to other sophisticated techniques.
Twenty-five patients with gross residual chordoma after partial excision or biopsy were treated by radical radiotherapy at Royal Marsden Hospital between 1952 and 1981. The median duration of freedom from local progression following radiotherapy was 32 months. Twenty-four of the 25 patients (96%) had stabilization or reduction in symptoms. All 19 patients in whom pain was a major symptom had relief. The overall actuarial 5- and 10-year survival rates were 44% and 17%, respectively. The corresponding progression--free survival rates were 33% and 20%. Permanent cure was unusual with any radiotherapy dose, but doses higher than 55 Gy or a TDF of 90 were associated with a statistically significant improvement in duration of local control. Seven of 17 (41%) patients who received greater than 55 Gy had freedom from local progression for 5 or more years compared with only 1 of 8 patients receiving less than 50 Gy. Subtotal excision prior to radiotherapy did not appear to improve median survival or probability of long-term local control. Distant metastases were seen in 16% of the patients, derived entirely from sacrococcygeal lesions, but only half of these caused significant morbidity or death. Local control is obviously the overwhelming problem. High dose, generous volume radiotherapy is advocated.
Neuroblastoma is the second most common malignant solid tumor in the pediatric population. Recent advances in treatment options and identification of prognostic subsets have made early detection important. Early classification into a favorable stage and subset may carry a 10-year survival of about 90% (1). With newer treatment regimens, the probability of long-term survival in patients with metastatic disease has also increased about fourfold since 1979 (2). Emergency physicians can contribute to improved morbidity and mortality with appropriate evaluation and disposition of the patient presenting with neuroblastoma. Two patients, a 6-month-old and a 2-week-old, presented to the Emergency Department with weakness. Both had a presumptive diagnosis of neuroblastoma made by the emergency physician. A brief discussion of the incidence, presentation, and diagnosis of neuroblastoma follows.
Although rare, direct infiltration of the cauda equina by non-Hodgkin's lymphoma (NHL) must be considered as a potential cause of subacute onset paraplegia. We present two cases of lymphomatous involvement of the cauda equina presenting with subacute paraplegia, each associated with a palsy of an oculomotor nerve. We highlight the need to use gadolinium-enhanced magnetic resonance imaging (MRI) when investigating potential cauda equina lesions, since it is crucial in detecting and outlining the extent of disease. Differential diagnoses and treatment options for NHL involving the cauda equina are also discussed.
Advances in imaging have greatly improved the ability to display spine anatomy and pathology. This article describes the imaging findings in degenerative, traumatic, infectious, and neoplastic disease. Anatomic information, however, must be tempered by an understanding of asymptomatic disease and interpreted in the context of the clinical syndrome.
PURPOSE: A prospective evaluation to determine if percutaneous needle biopsy yields enough tissue to establish the diagnosis of lymphoma and initiate therapy. PATIENTS AND METHODS: Lymphoma was diagnosed in 43 patients for the first time. Patients underwent either a core needle biopsy (n = 41) or an aspiration-type needle biopsy (n = 2) performed with imaging guidance. Immunochemical studies were performed on specimens from 39 of 43 patients (91%); flow cytometry was performed formed on specimens from 10 patients (23%). Patient progress was followed to see if biopsy results were used as a basis for treatment or if additional material was obtained with an open surgical procedure. RESULTS: On the basis of treatment decisions, needle biopsy results were sufficient for a diagnosis to be made in 36 of 43 patients (84%). In seven patients (16%), needle biopsy specimens were considered nondiagnostic, suspicious for lymphoma, or insufficient. None of the 43 patients responded to therapy in a manner that suggested the diagnosis of lymphoma to be incorrect. CONCLUSION: Image-guided needle biopsy should be the first procedure performed in the diagnosis of lymphoma, except in easily accessible superficial neck, inguinal, and axillary nodal sites.
Meningiomas are unusual in childhood, and the intraspinal location is very uncommon. Those arising from lumbar dura matter are the most rare among spinal neoplasms of meningeal origin. We present the case of a 12-years-old girl with a cauda equina meningioma. As in previously reported cases, the initial complain was back pain with radicular irradiation. The girl had few neurological findings, with pyramidal signs of both lower limbs as single neurological impairment. She underwent surgical treatment through an open door laminoplasty and the tumour was completely removed without neurological deficit. After 2-year of follow-up she remains asymptomatic.