1/5. Histologically repeatedly confirmed gliosarcoma with long survival: review of the literature and report of a case.OBJECTIVE AND IMPORTANCE: A rare case of gliosarcoma in a 61-year-old woman is presented with a stable situation over 22 years with an excellent quality of life. CLINICAL PRESENTATION: The patient was initially symptomatic and was operated on in 1975 for a deep-seated left parietal gliosarcoma. During the following 20 years, she was clinically asymptomatic until she complained of increasing headache in 1995. Neuroradiological imaging showed a sharply demarcated lesion on MRI at the former operative site, which was operated on again. Four months later, the residual tumour did grow again. INTERVENTION: As radiation therapy could not stop tumour progression and the neurological status worsened, the patient was operated on again for a massive tumour mass in the left parieto-occipital region, filling out nearly all of the previous resection cavity. Despite radio-immunotherapy, the patient finally died 22 years after the first discovery of the tumour. CONCLUSION: The present case shows that, in rare instances, gliosarcomas may show prolonged survival, although the underlying pathogenetic mechanisms for this clinical behaviour are not understood.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/5. Multifocal cerebral hemangio-endothelioma. Case report and review of the literature.BACKGROUND: Intracranial hemangio-endotheliomas (HE) are rare vascular tumours that present histological features and biological behaviour of an intermediate malignancy. We report the first case of primary HE presenting as multiple intracranial masses. CASE DESCRIPTION: The patient is a 20-year-old female who presented with paresis of the left arm. magnetic resonance imaging (MRI) showed multiple lesions in both frontal lobes. Total surgical excision of the tumours was obtained; a histological diagnosis of hemangio-endothelioma was made. Five months later, MRI scan showed new intradiploic skull lesions whereas a scintigraphic study revealed multicentric extracranial bone involvement. Treatment based on interferon-alpha (IFN-alpha) administration was undertaken; this produced partial regression of the lesions. Approximately three years after the first operation, the patient is in good clinical condition. CONCLUSIONS: Surgery continues to play the leading role in the treatment of intracranial HE. However, adjuvant therapy with IFN represents a new and well-tolerated therapeutic method for residual intracranial lesions as well as multicentric extracranial disease.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/5. Recurrent cardiac myxoma: why it occurs. A case report with literature review.Two years after surgical removal of a right atrial myxoma, recurrences were found in both atria: multiple tumors in the right atrium and one tumor in the left atrium. All were subsequently removed. There was no further recurrence during the following 3 years. We report on an unusual case which, to our knowledge, is the first time that such a case appears in the literature. We also discuss the clinical symptoms, diagnostic aspects, as well as details of a literature review regarding recurring myxoma. We think the recurrence in our case was due to totipotent multicentricity of the tumor. Previous studies showed that the recurrence might be due to the following reasons: inadequate resection, or multifocal pattern behaviour of a benign myxoma, either in the same or in a different location as the primary tumor. Familial disposition may also play a role in recurrent development. The abnormal dna ploidy pattern of myxoma patients showed a high recurrence.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/5. Malignant transformation of pleomorphic xanthoastrocytoma.A 31-year-old woman presented with a pleomorphic xantho-astrocytoma (PXA) manifesting as epilepsy. The tumour was partially resected. Histological examination revealed cellular pleomorphism and cytoplasmic vacuolation consistent with PXA, but no mitoses, necrosis, or endothelial proliferation. Follow-up neuro-imaging showed the residual tumour had grown rapidly with dissemination in the spinal cord. The recurrent lesion was totally resected and was shown to be glioblastoma. The patient has survived without signs of recurrence for 36 months after adjuvant radiochemotherapy. The biological behaviour of PXA cannot be predicted based on the histological features and careful follow up is essential.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/5. Papillary thyroid carcinoma with exuberant nodular fasciitis-like stroma: treatment outcome and prognosis.Papillary thyroid carcinoma with nodular fasciitis-like stroma (PTC-NFS) is one of the extremely rare variants of papillary thyroid carcinoma. To date, the majority of reported cases have been published in the surgical pathology and cytopathology literature, addressing the diagnostic difficulties posed by the condition's extensive, reactive stromal proliferation. Because of the rarity of PTC-NFS among papillary thyroid carcinoma variants, it has been unexplored from a clinical viewpoint. A medline search on the clinical course, role of radioiodine, treatment outcome and long term follow up of this disease yielded no result.We report the clinicoradiologic and histopathologic profile, together with post-treatment long term follow up, in a 35-year-old woman harbouring this rare entity. To the best of our knowledge, this is the first report of a five-year follow up of this rare variant of PTC following total thyroidectomy and radioiodine treatment. Our follow-up findings reiterate the disease's favourable clinical course when managed in the same manner as a classical, differentiated papillary carcinoma of the thyroid, akin to that predicted by the pathologists, and emphasize the importance of differentiating PTC-NFS as a separate entity from the papillary carcinoma variants with aggressive histology. Given the rarity of this condition, the experience gained from the present case is a useful addition to the current knowledge on disease prognostication and management.A systematic review of the existing literature on PTC-NFS, including the case reported in the present paper, is also carried out, aiming to explore the patient characteristics and clinical behaviour pattern of this rare entity and to make appropriate recommendations on management strategy. The age of presentation ranges from 20 to 82 years, with a mean of 44.5 years. female preponderance was observed, with a female to male ratio of 3ratio1. No racial predilection was observed. Tumour size varied from 2 to 9 cm along its greatest diameter (mean = 4.3 cm). Metastasis to lymph nodes at presentation occurred in 25 per cent of cases. Metastasis to surrounding structures (e.g. parathyroid and skeletal muscle) was observed in 12.5 per cent. There have been no reports of pulmonary or skeletal metastasis at presentation.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |