1/38. Pediatric eighth cranial nerve schwannoma without evidence of neurofibromatosis.Schwannomas of the eighth cranial nerve are rare in children. We report a 4 10/12 - year-old girl with no evidence of neurofibromatosis who presented with facial droop. Radiographic studies revealed a large cerebellopontine angle tumor. At surgery, the tumor was attached to the eighth cranial nerve and histologically was a schwannoma. This is the youngest reported case of unilateral eighth cranial nerve schwannoma in a patient without the stigmata of neurofibromatosis.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
2/38. meningioma with meningioangiomatosis: a condition mimicking invasive meningiomas in children and young adults: report of two cases and review of the literature.Meningioangiomatosis is a malformative meningovascular proliferation that occurs sporadically and in patients with neurofibromatosis type 2. Its histologic features of perivascular proliferation of elongated fibroblast and meningothelial cells trapping islands of gliotic cortex may be erroneously interpreted as invasion when an overlying meningioma is present. We report two cases of meningioangiomatosis associated with meningioma and review the literature on the subject for a total of six cases. The age of patients ranged from 9 months to 33 years. All cases were single lesions, and none had clinical evidence of neurofibromatosis type 2. Meningiomas in children have been regarded as having more aggressive behavior than their adult counterparts, with more frequent invasion of the underlying brain. The lack of correlation between brain invasion and recurrence observed in series of meningiomas in young patients may suggest that some of these lesions are meningioangiomatosis associated with meningioma rather than invasive meningiomas.- - - - - - - - - - ranking = 0.33333333333333keywords = fibromatosis (Clic here for more details about this article) |
3/38. Malignant peripheral nerve sheath tumour in the maxilla associated with von Recklinghausen's disease.Malignant transformation of neurofibromatosis is one of the most serious complications of von Recklinghausen's disease (VRD). The most common associated malignancy is the malignant peripheral nerve sheath tumour (MPNST). Few cases of MPNST associated with VRD in the maxillary region have been reported. This report describes a rare case of MPNST in the maxilla and the aggressive nature of MPNST associated with VRD.- - - - - - - - - - ranking = 0.16666666666667keywords = fibromatosis (Clic here for more details about this article) |
4/38. Aggressive congenital fibromatosis of the small intestine in the newborn. Report of a case and review of the literature.A case of so-called congenital fibro(leio)myosarcoma of the small intestine in a 18-day-old female baby, treated only with surgical resection, was studied by immunohistochemistry and electron microscopy in order to investigate the proliferating cell type. The tumour cells showed positivity only for vimentin and CD 34 and were negative for smooth muscle actin, desmin, alpha-sarcomeric actin, factor VIIIR: Ag and S-100 protein. Ultrastructural findings showed oval nuclei with prominent nucleoli, rare intracytoplasmic mitochondria and well developed rough endoplasmic reticulum. According to histoimmunological and electron microscopy findings the proliferating cells were likely to be of fibroblastic origin. A 7-year follow-up showed a favourable clinical evolution thus confirming that surgical resection can be a sufficient therapeutic approach. The morphological findings and clinical behaviour suggest that more appropriate terminology for this tumour would be "aggressive congenital fibromatosis" which better highlights its local progressive invasion without metastases.- - - - - - - - - - ranking = 0.83333333333333keywords = fibromatosis (Clic here for more details about this article) |
5/38. Pediatric acoustic schwannoma showing rapid regrowth with high proliferative activity.Acoustic schwannoma is a slow-growing tumor and usually occurs in adult patients. We report a rare pediatric case of acoustic schwannoma with high proliferative potential. A 10-year-old boy was diagnosed as having a right cerebellopontine angle tumor. The tumor was subtotally resected. Histological examination revealed a typical acoustic schwannoma with a few mitotic figures. Chromosomal analysis showed no abnormality on the long arm of chromosome 22 associated with neurofibromatosis type 2. The lesion re-grew rapidly as an acoustic schwannoma, necessitating subtotal resection on three occasions and CyberKnife radiosurgery. The immunohistochemical MIB-1 staining indices of the specimens obtained at the first, second, and third operations were 2.3%, 4.6% and 14.7%, respectively. The immunohistochemical proliferative potential of acoustic schwannoma is discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = fibromatosis (Clic here for more details about this article) |
6/38. Six cases of fibromatosis of the head and neck in children.Six cases of aggressive fibromatosis occurring in the head and neck in children are described. Fibromatosis is a rare, benign but locally infiltrative condition. Primary excision is not always possible in the head and neck areas. It is not always appropriate to treat children using the same modalities as adults due to associated growth problems. The case histories illustrate the management difficulties that can be encountered when treating the paediatric population.- - - - - - - - - - ranking = 0.83333333333333keywords = fibromatosis (Clic here for more details about this article) |
7/38. Sporadic unilateral vestibular schwannoma with islets of meningioma: case report.OBJECTIVE AND IMPORTANCE: Vestibular schwannomas with meningioma islets have been rarely reported in the literature; they have been observed only among patients with neurofibromatosis Type II. We present a case of a sporadic mixed tumor in a patient without neurofibromatosis Type II that was not suspected before surgery. CLINICAL PRESENTATION: A 59-year-old female patient presented with clinical signs of progressive loss of hearing. Her family history did not include evidence of neurological diseases. magnetic resonance imaging scans revealed a typical unilateral vestibular schwannoma. INTERVENTION: The tumor presented with invasion of the surrounding arachnoid membrane, as well as cranial nerves VII and VIII. Preservation of the facial nerve with complete removal of the tumor was not possible. Therefore, Cranial Nerve VII reconstruction was performed. CONCLUSION: The concomitant occurrence of schwannomas and meningiomas infiltrating the arachnoid membrane might be related to poor clinical outcomes for patients with neurofibromatosis Type II, with respect to preservation of facial and acoustic nerves. Among sporadic schwannomas, this phenomenon is extremely rare.- - - - - - - - - - ranking = 0.5keywords = fibromatosis (Clic here for more details about this article) |
8/38. Malignant peripheral nerve sheath tumor in the parapharyngeal space: tumor spread through the eustachian tube.SUMMARY: We report the CT and MR findings in a patient with malignant peripheral nerve sheath tumor in the parapharyngeal space who had neither a family history nor stigmata of neurofibromatosis. A high-resolution CT scan of the temporal bone revealed bony erosion and widening of both the foramen ovale and the bony portion of the eustachian tube. A temporal bone MR image showed an intensely enhancing solid mass in the parapharyngeal space, which extended into the middle ear cavity via the eustachian tube.- - - - - - - - - - ranking = 0.16666666666667keywords = fibromatosis (Clic here for more details about this article) |
9/38. brain-invasive meningioma in a 16-month-old boy.A previously well 16-month-old boy presented to Sydney Children's Hospital with generalised seizures. There was no history of neurofibromatosis and no family history. Imaging revealed a well-demarcated, frontal intracranial mass at the grey-white matter junction with surrounding oedema. At operation, the tumour was adherent to the underlying brain on its deep aspect but superficially connected to the dura. There was an associated peritumoural cyst. The tumour was biopsied for intra-operative frozen section opinion and excised in its entirety. The specimen consisted of a 25 x 25 x 17 mm nodule of firm cream tissue and separate fragments. Histologically, the tumour was a meningioma with frequent mitoses, necrosis and brain invasion. Optimal treatment, in view of the recurrence risk, includes complete excision and postoperative irradiation. The latter was deferred because of the young age. At follow-up at 26 months, the child was well and free of disease on MRI scan. Paediatric meningiomas are rare, with very few brain-invasive examples in reported series. In infants and young children, few meningiomas have been reported. There are several notable differences between meningiomas in children and adults. This report illustrates these differences.- - - - - - - - - - ranking = 0.16666666666667keywords = fibromatosis (Clic here for more details about this article) |
10/38. Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1--case report.A 26-year-old man with neurofibromatosis type 1 (NF1) presented with a giant malignant schwannoma of the sciatic nerve. The differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) was based on clinical, radiological, and histological evidence. The tumor apparently originated in a spinal plexiform neurofibroma. The lesion was resected totally without neural damage to the sciatic nerve. However, the tumor recurred within 2 months. The patient died of unknown factors probably associated with the spinal involvement. MPNST associated with NF1 has a poor prognosis due to recurrence or metastasis despite complete macroscopic removal.- - - - - - - - - - ranking = 0.83333333333333keywords = fibromatosis (Clic here for more details about this article) |
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