1/12. Antiangiogenic therapy of a recurrent giant cell tumor of the mandible with interferon alfa-2a.We report a 5-year-old girl with a large rapidly growing giant cell tumor of the mandible that recurred 2 months after the first surgical excision and 3 months after a second resection. An angiogenic protein, (bFGF), was abnormally elevated in her urine. The patient was treated with interferon alfa-2a for 1 year because this agent inhibits angiogenesis by suppressing bFGF overexpression in infantile hemangiomas and in other human tumors. During this time the bone tumor regressed and disappeared, the urinary bFGF fell to normal levels, and the mandible regenerated. She has remained tumor-free and has been off therapy for 3 years at this writing. This first successful use of interferon alfa-2a to treat a mandibular tumor in a child demonstrates: 1) low grade tumors that overexpress bFGF may respond to interferon alfa-2a, in a manner similar to life-threatening infantile hemangiomas; 2) antiangiogenic therapy, given without interruption for 1 year, was safe and effective in this patient; and 3) treatment may be continued for 1 year without the development of drug resistance.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/12. life-threatening giant mediastinal goiter: a surgical challenge.Mediastinal goiter is a well known benign disease, usually resectable through a cervical approach with minimal morbidity and mortality. Only occasionally a median sternotomy or a lateral thoracotomy may be required. The present case is worthy of presentation because of the exceptional dimension of the disease and the surgical challenge that it presented. In a 72-year-old woman a large intrathoracic goiter of the right thorax caused a severe dyspnoea due to an important contralateral mediastinal shift with compression of the lung, superior vena cava system and trachea. At surgical exploration, through a cervico-sternotomic approach, the mediastinal structures dislocation and the strong adherences between the anomalous neovascularized capsula of the mass and the surrounding structures, complicated the surgical dissection. An accidental lesion of the innominate artery required its reimplantation on the ascending aorta. An immense mass, was finally removed and pathological examination revealed a rare case of neovascularized, pseudosarcomatoid capsula among a benign hyperplastic proliferation. In spite of its benign nature, a giant goiter caused a life-threatening compression of the respiratory tract and lung parenchyma in this patient. The dimension of the lesions, the mediastinal anatomy alterations and the severe intraoperative haemorrhage represented major technical difficulties during surgical resection.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
3/12. Evolution of incidentally-discovered fusiform aneurysms of the vertebrobasilar arterial system: neuroimaging features suggesting progressive aneurysm growth.This study investigated the natural history and biological behavior of incidental fusiform aneurysms in four patients with incidental fusiform aneurysms of the vertebrobasilar arterial system who had been followed up for more than 3 years (mean 3.5 years). Two lesions remained the same size, and two lesions gradually grew. angiography showed the non-growing fusiform aneurysms as a circumferentially or unilaterally fusiform dilatation of a short segment of the vertebral artery with smooth walls and a steep slope of the dilatation, and the growing fusiform aneurysms as unilaterally fusiform involving a long segment of the vertebral artery or basilar artery with irregular walls and a gentle slope of dilatation. Magnetic resonance (MR) imaging demonstrated the non-growing fusiform aneurysms as a signal-void area, and the growing fusiform aneurysms as high and intermediate signals in addition to the normal flow void. The heterogeneous MR intensities probably correspond to turbulent flow, laminar flow, thrombosis, or intramural hematoma. Differentiation of growing and non-growing fusiform aneurysms is very difficult at the initial diagnosis. However, enlargement of the fusiform aneurysms is consistent with hemorrhage into the aneurysmal wall, which is confirmed by MR imaging. Fusiform aneurysms with the characteristics of the growing aneurysms cannot be overlooked because of the potential to develop into giant fusiform aneurysms which are very difficult to manage therapeutically.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
4/12. Successful antiangiogenic therapy of giant cell angioblastoma with interferon alfa 2b: report of 2 cases.We describe 2 cases of angioblastoma, a rare, destructive pediatric tumor, treated with interferon alfa 2b (IFNalpha2b). The first patient is a 10-month-old male who presented with an ulcerated palatal neoplasm that could not be completely resected. The second is a male neonate with a congenital tumor of the right hand that invaded the hypothenar eminence, destroying the fourth and fifth metacarpals. biopsy in both patients was interpreted as giant cell angioblastoma. Angioblastoma is rare; there is only 1 reported case that necessitated amputation of an upper extremity, also initially recommended for our patient. Because there is little experience with chemotherapy, permission was granted to employ an antiangiogenic regimen of IFNalpha2b. The angiogenic protein, basic fibroblast growth factor (bFGF), was abnormally elevated in both patients. Both patients received IFNalpha2b. In the first child, it was used after incomplete resection, because biopsy-proven tumor was present at the margin and in the nasopharynx. Biopsies 15 months after initiation of IFN2alphab were negative for tumor. Therapy was stopped after 3 years. Eighteen months later, the patient remains disease-free. In the second child, IFNalpha2b was started after debridement of the ulcerated tumor. Over 11 months, the tumor completely regressed and there was bony regeneration of the metacarpals. The fifth digit was amputated because of damage to the metacarpophalangeal joint by the tumor. IFNalpha2b therapy was discontinued after 1 year of treatment, and the child remains disease-free 2 years and 8 months later. In conclusion, this report demonstrates that: 1) a bFGF-overexpressing low-grade tumor can respond to IFNalpha2b in a manner similar to life-threatening infantile hemangiomas, 2) urinary bFGF levels can help guide IFNalpha dosage in such patients, and 3) although bFGF-mediated tumor angiogenesis is inhibited by IFNalpha, physiologic angiogenesis seems to be unaffected.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/12. Aggressive giant fibroepithelial lesion with unusual vascular stroma--a case report.The stroma of fibroadenoma and phyllodes tumor usually consists of fibroblastic proliferation. Rarely the stroma contains bundles of smooth muscle. Pseudoangiomatous hyperplasia of the mammary stroma has been described in fibroadenomas. However, true benign vascular stroma has not been reported. We report a case of a 34-year-old Chinese woman who presented with a large mass occupying the entire left breast. Left mastectomy was performed and showed a large, well-circumscribed, lobulated, rubbery-firm tumor measuring 13 x 10 x 6 cm. Microscopic examination revealed a fibroepithelial tumor formed by an organoid pattern of ductal structures with a very striking stromal appearance composed of extensive vascular proliferation and that demonstrated strong immunoreactivity for CD31, CD34, and factor viii. Ultrastructural examination revealed intercellular junctions, basal lamina, pinocytotic vesicles, and weibel-palade bodies in the cells lining the vascular spaces, confirming their endothelial nature. These findings rule out the diagnosis of pseudoangiomatous hyperplasia. The patient developed local recurrence a year later, and the resection showed malignant phyllodes tumor with ductal carcinoma in situ.The extensive vascular stroma noted in the primary tumor may have played a role in the malignant transformation of the epithelial and stromal components in this tumor.- - - - - - - - - - ranking = 0.8keywords = giant (Clic here for more details about this article) |
6/12. Giant-cell tumor of bone, stage II, displaying translocation t(12;19)(q13;q13).A new case of giant-cell tumour (GCT) of bone with benign histological features, clinical stage II, has been reviewed with immunohistochemistry and electron microscopy. After short-term tissue culture the karyotype, using G-banding techniques, presented a consistent translocation t(12;19)(q13;q13). Nude mice xenografts of the tumour were unsuccessful after 6 months of follow-up. Presence of such chromosomal rearrangement may be related to locally aggressive, histologically benign giant-cell tumors of bone.- - - - - - - - - - ranking = 0.4keywords = giant (Clic here for more details about this article) |
7/12. Ocular findings in incontinentia pigmenti.incontinentia pigmenti is an uncommon disorder affecting females, and is associated with ocular, dental, skeletal, and central nervous system abnormalities. Ocular abnormalities are found in 35% of cases. The authors present an eight-month-old girl with neonatal history of skin bullae, who was noted to have esotropia, leukocoria, and a fixed pupil. Histologic examination of the enucleated globe reveals total retinal detachment with retinal neovascularization into the collapsed vitreous, reactive retinal gliosis, and scattered giant nodular proliferations of retinal pigment epithelium. It is suggested that the underlying defect is at the level of the retinal pigment epithelium and that the neurosensory retinal changes are secondary.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
8/12. hemangiosarcoma of the thyroid gland. Histological diagnosis problems.A bleeding tumor of the thyroid gland in a 69-year-old woman is presented. Histologically, it was a hemangiosarcoma with proliferation and vessel neoformation: vascular-like clefts or distinct capillaries with tumoral endo- and perithelial structures were present. With reference to the published data, problems of differential diagnosis in the undifferentiated and giant cell carcinomas of the thyroid gland are discussed.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
9/12. Granulomatous reaction to Bruch's membrane in age-related macular degeneration.The histopathologic features of a granulomatous reaction in one eye of a patient with neovascular age-related macular degeneration are presented. Multiple multinucleated giant cells were found in intimate association with Bruch's membrane and at the margin of Bruch's membrane defects. Multinucleated giant cells appear to participate in the breakdown of Bruch's membrane and, together with diffuse disease of the retinal pigment epithelium and changes in the physicochemical properties of Bruch's membrane, may provide angiogenic stimulus for choroidal neovascularization in age-related macular degeneration.- - - - - - - - - - ranking = 0.4keywords = giant (Clic here for more details about this article) |
10/12. anterior chamber angle vascularization in sturge-weber syndrome. Report of a case.The case of a 20-year-old woman with a left-sided facial hemangioma and a homolateral glaucoma is reported, complete with the histology of a trabeculectomy specimen. Her left eye had an episcleral hemangioma and goniodysgenetic features in the anterior chamber angle, while the intraocular pressure was measured to be 45 mmHg. The left optic disc showed a large cupping and the left visual field was constricted. The right eye had no glaucomatous changes. Histological examination of the trabeculectomy specimen by both light and electron microscopy showed multiple congenital anomalies. There was a cluster of blood vessels in the trabecular meshwork. Abnormal accumulations of fine granular extracellular matrixes were observed in both the juxtacanalicular connective tissue and around the vascular structures. The lumen of Schlemm's canal was subdivided into three or four parts with few giant vacuole structures. The endothelial cells lining the inner wall of Schlemm's canal contained a well-formed basal lamina with many villi projecting into the lumen. These findings suggest that the multiple anomalies observed in the trabecular tissue may contribute to the manifestation of glaucoma in sturge-weber syndrome.- - - - - - - - - - ranking = 0.2keywords = giant (Clic here for more details about this article) |
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