1/26. Spontaneous remission of chiasmatic/hypothalamic masses in neurofibromatosis type 1: report of two cases.We report two children with neurofibromatosis type 1 showing enhancing masses on MRI suggesting neoplasms in the chiasm and hypothalamic region. In both patients no visual or endocrinal dysfunction was present. On serial MRI spontaneous partial remission was found, implying that a cautious approach to therapeutic management of similar cases should be taken.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
2/26. Solitary form of infantile myofibromatosis: a histologic, immunohistochemical, and electronmicroscopic study of a regressing tumor over a 20-month period.We present the repeated clinical, histologic, immunohistochemical, and ultrastructural observations on a cutaneous myofibromatous tumor over a 20-month period. A 6-day-old Japanese female had a solitary tumor on her left wrist at birth. A biopsy was first performed at 16 days of age, when the tumor was likely fully developed. Thereafter, the tumor gradually regressed. A second biopsy was performed at 58 days of age, when the tumor was already in a phase of early regression. Finally, the tumor was resected at 20 months of age, when it was in a phase of late regression. Our study demonstrated that undifferentiated immature histiocytic cells predominated over spindle cells in the first biopsy specimen, but thereafter the former cells decreased or disappeared in parallel with the increase in the latter cells, which showed characteristics similar to myofibroblasts, in regressing lesions. This evidence suggests that the undifferentiated immature histiocytic cells are precursors of the spindle cells. Spindle cells in the phase of early regression also showed many vacuoles and lipid-like droplets in the cytoplasm, even though they actively produced massive amounts of glycogen. These findings also suggest that tumor regression results from cytoplasmic vacuolation and disruption of spindle cells. Our results are considered to demonstrate, for the first time, the clinical and histologic features of the different developmental or regressive phases of infantile myofibromatosis.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
3/26. Spontaneous improvement of optic pathway lesions in children with neurofibromatosis type 1.Two cases of spontaneous improvement of optic pathway lesions in neurofibromatosis type 1 are reported. At time of diagnosis the children were aged 21 and 32 months respectively; they have been followed by both MRI and clinical evaluation for 5 and 4 years. MRI findings of the first 19 months of follow-up for Case 1 have been described by us before. On MRI serial evaluation, Case 1 showed an almost complete normalization of the size of lesions and a resolution of enhancement, whereas Case 2 showed a slight decrease in the size of lesions and a resolution of enhancement. From the clinical point of view Case 1 showed a normalization of his clinical signs, whereas in Case 2, a visual improvement was only slight, if at all present. In Case 1, the clinical improvement seemed to follow the spontaneous regression of the lesions detected by MRI.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
4/26. Spontaneous regression of low-grade astrocytomas in childhood.An 8-year-old boy with neurofibromatosis type 1 (NF1) and a biopsy-proven juvenile pilocytic astrocytoma of the hypothalamic/chiasmatic region was followed with serial MRIs over 4 years. Spontaneous tumor regression was followed by progression and biopsy; 6 months later, the tumor regressed again. This bimodal regression is rare, but highlights the variable natural history of low-grade gliomas in children with NF1 and the difficulty in evaluating response of such tumors to therapy.- - - - - - - - - - ranking = 0.2keywords = fibromatosis (Clic here for more details about this article) |
5/26. Early spontaneous regression of a hypothalamic/chiasmatic mass in neurofibromatosis type 1: MR findings.A patient with neurofibromatosis type 1 was found to have an enhancing mass in the hypothalamus and in the anterior optic pathway. A 3-month MR study showed a reduction in the size and enhancement of the mass. At a 9-month MR follow-up the mass disappeared and ceased to enhance. This report shows the unusual behaviour of a hypothalamic/chiasmatic mass confirming that in such asymptomatic cases the conservative management can be considered the treatment of choice.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
6/26. Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging.OBJECTIVE: To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1). methods: Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-ophthalmology Society (NANOS) and through requests on the NANOSNET internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink. RESULTS: Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2. CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.- - - - - - - - - - ranking = 0.2keywords = fibromatosis (Clic here for more details about this article) |
7/26. Solitary infantile myofibromatosis in the lateral orbit floor showing spontaneous regression.Infantile myofibromatosis is a rare benign tumour usually occurring early in infancy. We describe the case of a 10-year-old boy with solitary infantile myofibromatosis in the left lateral orbit floor which regressed spontaneously. Although our patient was older than previously reported cases and showed bony destruction confirmed by computed tomography (CT), this tumour was diagnosed as infantile myofibromatosis based on immunohistochemical findings. The tumour disappeared spontaneously six months after incisional biopsy, that also indicated this tumour was an infantile myofibromatosis.- - - - - - - - - - ranking = 1.6keywords = fibromatosis (Clic here for more details about this article) |
8/26. Infantile myofibromatosis with visceral involvement and complete spontaneous regression.Infantile myofibromatosis is an unusual mesenchymal disorder characterized by the proliferation of tumors in the skin, muscle, bone, and viscera. Two types can be distinguished; the solitary type, defined by the presence of one nodule in the skin, muscle, bone or subcutaneous tissue; and the multicentric type which can be divided into two sub-types. In the first sub-type the lesions are multicentric but without visceral involvement, while in the second, visceral involvement is present. The prognosis of the disease depends on whether visceral involvement is present. Solitary and multicentric nodules without visceral involvement usually have excellent prognosis with spontaneous regression of lesions within 1 to 2 years of diagnosis. On the other hand, visceral lesions are associated with a significant morbidity and mortality, resulting from vital organ obstruction, failure to thrive, or infection. death in these cases often occurs at birth, or soon after, and is usually due to cardio-pulmonary or gastrointestinal complications. The case being reported here, is that of a female newborn who had multiple skin, subcutaneous tissue, skeletal muscle, bone, and lung lesions immediately after bith. At the age of three years, the child is in good health, her psychomotor development is in accordance with her age group, and the lesions have completely regressed. This is a case of the multicentic type of infantile myofibromatosis with visceral involvement, where all lesions have spontaneously regressed. This is a rarity since the prognosis in the majority of such cases is poor.- - - - - - - - - - ranking = 1.2keywords = fibromatosis (Clic here for more details about this article) |
9/26. Spontaneous involution of a non-optic astrocytoma in neurofibromatosis type I: serial magnetic resonance imaging evaluation.A patient with neurofibromatosis type I (NF1) was followed-up with serial magnetic resonance imaging (MRI) studies over a period of 6 years. A contrast-enhancing lesion of the internal capsule, histologically proven to be pilocytic astrocytoma through stereotactic brain biopsy with mass effect and associated edema, was detected to reveal spontaneous involution on follow-up MRI studies. Although spontaneous regression of gliomas of the optic pathway-hypothalamus in patients with NF1 is relatively common in the literature, spontaneous involution of non-optic (i.e. areas other than optic pathways and hypothalamus) gliomas is rarely reported. Conservative management with follow-up MRI studies should be considered for non-optic glial tumors and tumor-like masses in patients with NF1, and surgical treatment should not be considered unless the lesions exhibit a rapid or unrelenting growth on serial MRI studies or produce significant clinical deterioration.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
10/26. A case of infantile digital fibromatosis showing spontaneous regression.A case of infantile digital fibromatosis (IDF) in a 3-year-old girl is reported. Two tumours were present on the left fourth toe and one over the lateral aspect of the left heel. Histological examination revealed that the tumor cells contained intracytoplasmic inclusion bodies, characteristic for this condition. The tumours showed spontaneous regression without therapy.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
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