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PubMed2006

[Tumors of the craniocervical junction].

In this review, the authors describe the most common tumors of the craniocervical junction and their management. Early recognition is important in view of the unspecific symptomatology and much better surgical results in patients operated before the appearance of neurological deficits. In the last 15 years, different craniobasal approaches have been developed which permit the radical excision of many of these lesions with no or acceptable morbidity. Representative cases are illustrated.

Adolescent↗
PubMed2000

Management issues in chordoma: a case series.

This study presents a combined case series of chordomas from two Canadian institutes. Twenty-seven patients were identified for the period 1954-1998. Management issues with regard to diagnostic pitfalls, selection of charged particle treatment and retreatment of recurrences are discussed. The diagnosis of early stage chordoma requires a high index of suspicion. One patient in the series presented with hoarseness and is described in detail. The diagnosis was made coincidentally by a computed tomographic scan of the head, performed after a motor vehicle accident. The planning of both surgery and radiotherapy was optimized by using magnetic resonance imaging. A review of the literature supports the use of a combined surgical and radiotherapeutic approach.

Adult↗
PubMed2005

Multifocal epithelioid hemangioendothelioma in a 16-year-old boy.

We report on a 16-year-old white male presenting with a 4-month history of syncopal episodes and occasional headaches. The CT and MRI studies revealed numerous lytic lesions of the skull base and cervical spine; subsequently, similar lesions were demonstrated in all areas of the skeleton, and CT showed numerous lesions in the lungs, liver, and kidneys. Excisional biopsy from several sites confirmed the diagnosis of epithelioid hemangioendothelioma. We conclude that epithelioid hemangioendothelioma should be added to the differential diagnosis for lytic lesions of bone that are clustered in the same anatomic region and that might also present with visceral involvement.

Adolescent↗
PubMed2001

Vertebral osteoid osteoma masquerading as a malignant bone or soft-tissue tumor on MRI.

PURPOSE: Four pediatric patients were sent to our institution with the diagnosis of soft-tissue/malignant bone tumor. In all cases an MRI was the initial study performed for neck or back pain. All were surgically proven to have an osteoid osteoma/osteoblastoma (OO) as a final diagnosis. The MRI findings are reviewed. METHODS: Four patients, three boys and one girl, ranging in age from 5 to 17 years, presented with symptoms of neck or back pain for 2 months to 2 years. Two had neurological findings. All patients underwent MRI. RESULTS: All MRIs demonstrated decreased T1 signal and increased T2 signal in the soft tissues and bone surrounding the lesions consistent with edema. Enhancement was observed in the adjacent soft tissues and in the lesion nidus retrospectively. CONCLUSION: Investigating neck or back pain with an initial MRI may lead to misleading diagnoses unless the radiologist is aware of the typical MRI appearance of vertebral osteoid osteoma.

Adolescent↗
PubMed2004

Three-dimensional trabecular bone architecture of the lumbar spine in bone metastasis from prostate cancer: comparison with degenerative sclerosis.

OBJECTIVE: Prostate cancer frequently metastasizes to bone, inducing osteosclerotic lesions. The objective of this study was to clarify the three-dimensional (3D) trabecular bone microstructure in bone metastasis from prostate cancer by comparison with normal and degenerative sclerotic bone lesions, using microcomputed tomography (micro-CT). DESIGN AND MATERIALS: A total of 32 cancellous bone samples were excised from the lumbar spine of six autopsy patients: 15 metastatic samples (one patient), eight degenerative sclerotic samples (four patients) and the rest from normal sites (three patients). The samples were serially scanned cross-sectionally by micro-CT with a pixel size of 23.20 microm, slice thickness of 18.56 microm, and image matrix of 512 x 512. Each image data set consisted of 250 consecutive slices. The volumes of interest (96 x 96 x 120 voxels) were defined in the original image sets and 3D indices of the trabecular microstructure were determined. RESULTS: The trabecular thickness (Tb.Th) in degenerative sclerotic lesions was significantly higher than that in normal sites, whereas no significant difference was observed in trabecular number (Tb.N). By contrast, in metastatic lesions, the Tb.N was significantly higher with increased bone volume fraction (BV/TV) than in normal sites, and no significant difference was found in Tb.Th. The characteristics of the trabecular surface in the metastatic samples showed concave structural elements with an increase in BV/TV, indicating osteolysis of the trabecular bone. In 3D reconstructed images, increased trabecular bone with an irregular surface was observed in samples from metastatic sites, which were uniformly sclerotic on soft X-ray radiographs. CONCLUSION: These results support, through 3D morphological features, the strong bone resorption effect in bone metastasis from prostate cancer.

Adult↗
PubMed2003

Unusual skeletal metastases from myxoid liposarcoma only detectable by MR imaging.

We present two cases of skeletal metastases from myxoid liposarcoma, occurring several years after treatment of the primary tumors in the lower limb. The present two case reports have unusual radiological features only detectable by MR imaging and not by plain radiographs or bone scans. From the present two cases, we found that a negative plain radiograph of the spine or a negative bone scan could not exclude skeletal metastases from myxoid liposarcoma, and MRI was a more sensitive screening procedure for their detection, especially in T1-weighted images. Unusual radiological features of skeletal metastases from myxoid liposarcoma are not well documented and only a few cases have been previously reported. Our aim is to document two more patients exhibiting the unusual radiological features of skeletal metastases from myxoid liposarcoma to improve their early detection and management.

Adult↗
PubMed1998

CT and MRI appearances of a thoracic chordoma.

A case of a chordoma in the thoracic spine is presented. This is a very rare tumour in this location and should be considered in the differential diagnosis of any posterior mediastinal mass. The appearances on CT and MRI were similar to chordomas described in other locations. On T2-weighted images septae of low signal intensity radiated throughout the large high-signal mass. This feature may be of use in differentiating chordomas from other posterior mediastinal masses.

Adult↗
PubMed2001

Double ependymoma of the filum terminale.

Myxopapillary ependymoma of the filum terminale and conus medullaris are relatively common spinal intradural neoplasms in adults, only 8-14% affecting children. We describe a case of a 15-year-old girl with two myxopapillary ependymomas of the filum terminale. Although cases of two such tumours have been described in adults, we have not found any similar paediatric cases in the literature.

Adolescent↗
PubMed1998

Extracranial neuroblastomas and neurological complications.

Between October 1989 and March 1997, 25 pediatric inpatients were treated for primary extracranial neuroblastoma (NB; n=20) or ganglioneuroblastoma (GNB; n=5) at the University of Istanbul, Institute of Pediatric Oncology, and these children were the subjects of this retrospective study. Seventeen (68%) of these patients experienced 19 neurological complications during the course of their disease. Fourteen had nervous system metastases or invasion. Nonmetastatic complications, including CNS infections (n=3) and new onset of seizures (n=2) secondary to metabolic encephalopathy were seen in 5 cases. By the time of the final analysis of the results, 8 of the 17 patients with neurological complications had died, 7 had either been lost to follow-up (n=4) or were in the terminal stage of their disease (n=3), and 2 were in remission. Both of the patients who were in remission had dumbbell neuroblastoma (DNB), and 1 of them, with congenital DNB, also had neurological sequelae, characterized by paraplegia and neurogenic bladder. Neurological complications occurred in 68% of NB and GNB cases. Metastatic complications were more common than nonmetastatic complications and had a poor prognosis. Neurological complications were the primary cause of mortality in this study, mortality being related to neurological complications in 63% of cases, and the final outcome was worse than expected. However, regardless of any differences in social, economic and geographic factors and different treatment protocols for NB in different pediatric oncology institutions, neurological complication rates in pediatric NB are similar in all.

Adolescent↗
PubMed2000

Giant schwannoma of the back.

We treated a patient with giant schwannoma of the back. The tumor measured 35 x 25 x 12 cm and weighted 1840 g. Histological diagnosis was benign schwannoma. To our knowledge, there is no previous report of such a large schwannoma of the back.

Humans↗
PubMed2002

Management strategies in neurofibromatosis type 2.

The objective of this study was to review the experience of the Neurotology and Skull Base Surgery Unit of Addenbrooke's Hospital in Cambridge, England, in the management of patients with neurofibromatosis type 2 (NF2). This was a retrospective review of the institution's series conducted at a neurotological tertiary referral centre. Over a 17-year period (1984-2001), 35 patients with NF2 were managed. These patients presented with multiple cranio-spinal neoplasms, including 62 cerebellopontine angle tumours of which 59 were vestibular schwannomas (nine patients with unilateral tumours and 25 patients with bilateral tumours). Clinical presentation, diagnosis and patient management were reviewed. The outcome parameters measured were tumour progression, the incidence of complications (hearing deterioration and facial nerve palsy) and the need for secondary intervention. Five vestibular schwannomas were treated with stereotactic radiosurgery (gamma-knife), all of which showed evidence of progression in size and/or deterioration in hearing. Thirty-one VS were conservatively treated with annual surveillance. In nine cases, the tumours had a follow-up shorter than 6 months and were therefore excluded from the results. In 13 cases the VS did not progress in size, and the hearing remained stable, while in the remaining nine cases, tumour progression was evident. In fifteen cases, surgery was performed at the authors' institution. In 11 cases a translabyrinthine approach was adopted, and in the remaining four cases a retrosigmoid approach was preferred. In all these cases, tumour removal was total and facial nerve function was House-Brackmann grade I-III in 55%. Successful hearing preservation was elusive in those patients in whom a hearing preservation approach was attempted. NF2 remains an extremely challenging disorder for the neurotologist and the patient alike. Early diagnosis offers distinct advantages to the patients, their families and the community at large. Of the treatment modalities, surgery unequivocally offers the most superior tumour control. Hearing preservation remains a challenge in these patients, but is optimised by the early detection of tumours in the NF2 patient.

Adolescent↗
PubMed2006

Epithelioid hemangioendothelioma derived from the spine region: case report and review of the literature.

Epithelioid hemangioendotheliomas (EHE) are rare vascular tumors which generally originate from soft tissues and visceral organs. Primary bone EHEs, especially those occurring in the spine region, are extremely rare. Our case is that of a 30-year-old man who was admitted to hospital with low back pain, difficulty in walking, post-voiding urinary incontinence and numbness in the caudal area. X-ray showed a lytic process affecting the vertebra L2 and collapse of L1. Vertebrectomy of L1 and gross total tumor resection were performed. Histopathological and immunohistochemical findings of the tumor tissue supported the diagnosis of EHE. The case, which to the best of our knowledge is only the fifth such reported case, is presented with its clinicopathological findings and a review of the literature.

Adult↗
PubMed2006

Epithelioid sarcoma in the thoracic spine.

Epithelioid sarcoma is a rare and highly malignant soft tissue tumor that is commonly found in the extremities and rarely in the trunk area. This malignant tumor often mimics granuloma or nodular fasciitis, which causes a delay in establishing the diagnosis. This type of cancer has a high recurrence rate. Surgical treatment requires wide radical resection. The objective of this case report is to highlight the unique location of a rare neoplasm and to illustrate the relentless course of epithelioid sarcoma despite initial radical resection. A 14-year-old boy was admitted to our facility with a soft tissue mass on the right lower thoracic spine. The large tumor mass had deeply penetrated into the muscles, infiltrated the neuroforamen of T9-T10 level, and compressed the dural sac. Immunohistological study of the biopsy was highly consistent with an epithelioid sarcoma. Wide excision of the mass, laminectomy and spine fusion with instrumentation was performed. The patient received chemotherapy and irradiation. The first recurrence of the neoplasm was seen as a contralateral metastasis 21 months after the resection. On the last follow-up, 3 years postoperatively, the patient was in a good general condition. However, further progression of the sarcoma had to be recognized. Our case encompasses multiple features that represent negative prognostic factors. Initial wide excision of the neoplasm and adjuvant therapy including chemotherapy and irradiation seem to slow down the relentless course of epithelioid sarcoma in the trunk.

Adolescent↗
PubMed2003

Management of benign craniovertebral junction tumors.

BACKGROUND: We report our surgical experience in the treatment of fifteen consecutive patients with benign craniovertebral junction tumors, observed from 1993 to 2000 at our department. METHOD: We treated 7 meningiomas, 3 epidermoids, 3 C1 neurinomas and 2 neurinomas of the lower cranial nerves. Clinical results were evaluated by Karnofsky Performance Scale and all patients underwent preoperative neuroradiological evaluation with CT, MRI and MRA; angiography was not routinely performed and was considered for each individual case. FINDINGS: 11 partial transcondilar and 4 retrocondilar approaches were performed. Total removal was achieved in 11 cases (73,3%) and subtotal removal in 4 patients (26,7%). None of the patients required occipitocervical fusion. Patients were followed for an average period of 24+/-31 months. Clinical and radiological follow-up showed no recurrence in cases with total removal. In all patients a statistically significant postoperative increase of KPS scores was recorded. The treatment of epidermoid tumors presented particular issues: debulking the lesion, we obtained a surgical window, avoiding a large removal of bone. In Nakasu grade 1 or 2 meningiomas, we carried out total removal by piecemeal resection and without complete condylectomy and bone fixation. INTERPRETATION: The choice of these approaches and the extent of bone resection should be defined according to the tumor's location and size. Moreover we emphasize that preoperative neuroradiological evaluations on presumptive tumor type could be helpful to the surgeon in order to tailor the technique to different lesions, providing the required exposure, without unnecessary surgical steps.

Adolescent↗
PubMed2005

Intracisternal neurinoma of the C1 posterior root.

We report a rare intracisternal C1 posterior root neurinoma in a 35-year-old man without neurofibromatosis who presented with headache, nuchal pain, bilateral motor weakness of the upper extremities, and numbness in the right distal upper extremity. CT and MRI study showed a 20-mm intracisternal lesion at the foramen magnum. At surgery, there was an anastomosis between the C1 posterior root and a spinal accessory nerve at the site of the tumor; the root from the collateral sulcus of this C1 root was absent. Postoperatively, the patient remains free of symptoms. Foramen magnum neurinomas have been described as accessory nerve tumors. We present new anatomical consideration regarding this lesion.

Accessory Nerve↗