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1/210. p53 and p16INK4A mutations during the progression of glomus tumor.

    Glomus tumors are significantly rare tumors of carotid body. The great majority of these tumors are benign in character. Here we present two brothers with hereditary glomus jugulare tumor who had consanguineous parents. radiotherapy was applied approximately 8 and 10 years ago for treatment in both cases. Eight years later, one of these cases came to our notice due to relapse. The mutation pattern of p53, p57KIP2, p16INK4A and p15NK4B genes which have roles in the cell cycle, was analyzed in tumor samples obtained from the two affected cases in the initial phase and from one of these cases at relapse. The dna sample obtained from the case in initial diagnosis phase revealed no p53, p57KIP2, p16INK4A or p15INK4B mutation. He is still in remission phase. Despite the lack of p53, p57KIP2, p16INK4A and p15INK4B mutation at initial diagnosis the tumor dna of the other case in relapse revealed p53 codon 243 (ATG-->ATC; met-->ile) and p16 codon 97 (GAC-->AAC; asp-->asn) missense point mutations. No loss of heterozygosity in p53 and p16INK4A was observed by microsatellite analysis of tumoral tissues in these cases. P53 and p16INK4A mutations observed in relapse phase were in conserved regions of both genes. No previous reports have been published with these mutations in glomus tumor during progression. The mutation observed in this case may due to radiotherapy. In spite of this possibility, the missense point mutations in conserved region of p53 and p16INK4A genes may indicate the role of p53 and p16INK4A in tumor progression of glomus tumors.
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2/210. Rapid progression of squamous cell carcinoma of the cervix after hyperbaric oxygenation.

    The role of hyperbaric oxygenation in the treatment of radiation-induced sequelae and chronic ulcer is well established. On the contrary, a possible cancer-causing or growth-enhancing effect by hyperbaric oxygenation was highly controversial. Herein, we present a 55-year-old Chinese woman with recurrent squamous cell carcinoma of the cervix on her left inguinal area. She received concurrent chemoradiation therapy followed by radical inguinal lymphadenectomy due to persistent tumor mass. The patient was complicated with severe radiation fibrosis and unhealed wounds, so she was treated with hyperbaric oxygenation (HBO). However, the patient died of complications of the disease after completing HBO therapy I month later and autopsy of the patient showed carcinomatosis of the abdominal cavity and lower abdominal wall. Because previous studies have been inconclusive regarding the effect of HBO on tumor cells, we reviewed the possible relation between the HBO and tumor cells.
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3/210. Clinical and radiographic response in three children with recurrent malignant cerebral tumors with high-dose tamoxifen.

    The purpose of this study was to deliver tamoxifen as antiangiogenic therapy to children with recurrent progressive malignant brain tumors. tamoxifen was administered orally in very high dosage to one child as monotherapy and to two children in combination with oral etoposide and dexamethasone. One boy was diagnosed with high-grade astrocytoma in the brain stem, one girl with anaplastic ependymoma of the fourth ventricule, and one girl with high-grade astrocytoma in the midbrain. Conventional treatment with multiple surgeries, first- and second-line chemotherapy, and external beam therapy had failed. Tumor reduction was seen in radiographic images together with clinical improvement in 2 children, and clinical and radiographic halting of tumor progression was demonstrated in the patient with anaplastic ependymoma. None of the patients developed complications from the treatment. Follow up of the patients ranged from 15 to 30 months with a mean of 17 months. These encouraging preliminary results suggest a potential for this type of therapy. More studies are needed to start clinical trials and prove that angiostatic activity may contribute to the therapeutic effect of antiestrogens in estrogen receptor-negative tumors.
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4/210. Histologic transformation of polymorphous low-grade adenocarcinoma of salivary gland.

    Polymorphous low-grade adenocarcinoma of salivary gland origin (PLGA) was initially described in 1984 and has since become an established clinicopathologic entity. Owing to the indolent nature of PLGA and its relatively recent description, the full clinicopathologic spectrum of this entity has not been elucidated fully. Transformation to a histologically different-appearing lesion or progression to a higher histologic grade has not been reported. We describe 2 PLGAs arising in the palate and associated with multiple locoregional recurrences that were treated with excision and radiation therapy. This was followed by histologic transformation to a higher grade neoplasm after 17 and 26 years, respectively. The histologic appearance after transformation was characterized by a predominantly solid and cystic growth pattern, nuclear atypia with prominent nucleoli, and foci of necrosis. High-grade transformation of PLGA may occur after a protracted clinical course with multiple recurrences of typical PLGA. The possible role of radiation therapy as an initiator of this transformation merits further study. Tumors with these histologic features should not be included under the rubric of typical PLGA. Segregation of these neoplasms will allow further study of their biologic potential, particularly with regard to possible increased rates of local recurrence and metastasis.
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5/210. Sarcomatoid carcinoma of the urinary bladder: 3 case reports.

    Three cases of sarcomatoid carcinoma of the urinary bladder are considered. The patients were 2 males, aged 68 and 81 years, and a 79-year-old female. Transurethral resection of the bladder tumor (TUR-BT) and consecutive radical cystectomy were performed in all patients. Local progression of bladder tumors just after TUR-BT was recognized in the 2 younger patients, each of whom died due to recurrence after radical cystectomy. Because of the extremely aggressive malignant potential of sarcomatoid carcinomas, the indications for TUR-BT should be carefully assessed and suitable therapeutic strategies should be examined further.
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6/210. Chondromyxoid fibroma of skull base: a tumour prone to local recurrence.

    Chondromyxoid fibroma of the skull base is extremely uncommon. Sometimes involvement of the nasal cavity may occur and the patients may present with nasal symptoms. The biological behaviour of this tumour has not been well studied, primarily because of the limited number of reported cases and the short duration of follow-up. We report a histologically confirmed case of chondromyxoid fibroma of the skull base that recurred repeatedly over a 10-year period after the initial operation. Histologically it showed identical morphology to the original tumour with no evidence of histological progression or dedifferentiation. Ultrastructurally, the spindle tumour cells in the fibromyxoid area showed dual chondroblastic and fibroblastic differentiation, suggesting that these spindle fibroblastic cells and the better differentiated chondroid cells were of the same cell type with different histological morphology.
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7/210. Primary hepatic carcinoid tumors confirmed with long-term follow-up after resection.

    Primary carcinoid tumor of the liver is very rare. Only 30 cases have been reported in the English literature. However, most of those cases were diagnosed only on the basis of diagnostic imaging and surgical exploration, their follow-up periods being up to 5 years. Considering the slow progression of the tumor, long-term follow-up is required to exclude occult extrahepatic primaries which may manifest afterwards, and to determine the clinical course of this disease. We experienced 3 patients with primary hepatic carcinoid tumors who underwent total resections. They all survived more than 7 years and were clinically confirmed as hepatic primary by failing to detect other primaries during their courses. One patient is disease-free for more than 7 years after resection. The other 2 were found to have recurrence in the remnant liver; 1 underwent transcatheter arterial embolization several times and is alive more than 4 years after recurrence, and the other underwent 2 more operations and survived 8 years after the 1st recurrence. Surgery might offer a possible chance of cure for primary hepatic carcinoid tumor, and transcatheter arterial embolization might be a good treatment option when an unresectable disease is confined to the liver.
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8/210. Locally recurrent malignant fibrous histiocytoma: a rare and aggressive genitourinary malignancy.

    OBJECTIVE: In this study, 22 cases of locally recurrent urological malignant fibrous histiocytoma were reviewed considering therapeutic options, follow-up and prognosis. patients AND methods: In the available literature on this topic we identified 19 cases of locally recurrent genitourinary malignant fibrous histiocytoma. Three additional cases are discussed, primarily arising from the kidney, the bladder and the paratesticular region. RESULTS: The prognosis of locally recurrent urological malignant fibrous histiocytoma was found to be extraordinarily poor. Only 2 of 22 patients have survived for longer than 3.5 years. One of them reported herein is still alive 10 years after extensive lymphatic spread accompanying the first local recurrence. In this case, late local recurrence occurred after an 8-year interval free of disease. CONCLUSION: Malignant fibrous histiocytoma is an unusual urological malignancy with a high rate of local recurrence. The latter is frequently accompanied by metastatic disease and unrelenting progression. Despite the poor prognosis early detection of local failure and aggressive salvage therapy might offer the chance of long-term survival in selected cases. Close and life-long follow-up is advisable for patients once treated for recurrent urological malignant fibrous histiocytoma.
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9/210. Utility of a moveable 1.5 Tesla intraoperative MR imaging system.

    OBJECTIVE: This study demonstrates the utility of a newly-developed moveable 1.5 Tesla intraoperative MR imaging system using a case report of a multi-lobulated parafalx meningioma. CLINICAL PRESENTATION: A 43-year-old female presented with progression of a multi-lobulated anterior parafalx meningioma several years following resection of a large left frontal convexity meningioma. INTERVENTION AND TECHNIQUE: Surgical excision of the lesion was undertaken. Following apparent total resection, intraoperative MR imaging revealed two residual dumbell shaped lobules. Using these updated MR images, the tumour was readily identified and removed. CONCLUSION: The moveable 1.5 Tesla intraoperative MR system used in the present case provides rapid, high resolution MR images during neurosurgical procedures. Moving the magnet out of the surgical field during surgery permits the use of all standard neurosurgical instruments. The ease of use and quality of images combined with minimal interference on well-established surgical techniques makes this system a valuable adjunct in the neurosurgical treatment of intracranial disease.
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10/210. Solitary bone plasmacytoma: management of isolated local relapse following radiotherapy.

    radiotherapy is the prime treatment modality for solitary plasmacytomas of bone (SPB). Although local control rates are excellent, progression to multiple myeloma is frequent, albeit with varying latency. Local failure in the absence of dissemination is rare and thus management is poorly documented. We discuss such a patient who presented 3 years after local radiation for a pelvic SPB and review the relevant literature. radiation doses, portals employed and prognostic factors that may predict progression to myeloma are discussed. This report shows that an isolated recurrence of SPB in a previously irradiated field was successfully treated with orthopaedic surgery. This resulted in good pain relief and mobility for the patient, who remains free of disease 6 months after operation.
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