1/78. Benign vascular neoplasms of the spleen with myoid and angioendotheliomatous features.AIMS: To present the clinical light microscopic and immunophenotypic features of a distinctive vascular neoplasm of the spleen. methods AND RESULTS: Two of the splenic lesions arose in children, and one was found in an adult. They ranged from 19 to 40 mm diameter and histologically were quite similar. Sheets of large epithelioid cells with a spectrum of nuclear configurations ranging from oval and vesicular to twisted and hyperchromatic were noted in each case. Distinct or prominent nucleoli were present in many cells, and occasional cells had nuclear pseudoinclusions. In two cases, bands of basophilic, fibroblast-rich stroma with scattered chronic inflammatory cells were present. The mitotic rate ranged from 0/10 high-power fields (HPF) to 0.5/10 HPF in these epithelioid cells. The vascular nature of these tumours was manifested as a sieve-like array of round, erythrocyte-filled spaces, most with attenuated and cytologically bland lining cells. The polygonal, epithelioid cells exhibited the following phenotype: smooth muscle actin (SMA) , muscle specific actin (MSA) , vimentin , CD31-, CD34-, CD21-, CD8-, CD68- (2/3 cases), S100-, while the lining cells were CD34 , vimentin and SMA-, with variable CD31 and factor viii related antigen expression. Elongated SMA , MSA cell processes were evident in one case, reminiscent of previously characterized myoid elements of the normal spleen. An uneventful follow-up was noted for all three patients. CONCLUSIONS: The histology and immunophenotype set these neoplasms apart from classic hamartomas, haemangiomas and previously characterized (haem)angioendotheliomas of the spleen, and may represent proliferations of myoid elements native to the spleen.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/78. forehead lipoblastoma mimicking a hemangioma.A case of forehead lipoblastoma simulating a hemangioma in a male infant is reported, to alert pediatricians to this rare tumor and to increase the index of suspicion in atypical hemangiomas. A 2-month-old male infant developed a protruding forehead mass with increased vascularity. It demonstrated progressive and accelerated growth over the subsequent 6 months, unresponsive to steroid therapy. A magnetic resonance imaging scan supported the diagnosis of hemangioma because of the hypervascular nature of the lesion. Surgical excision was performed because of visual obstruction. Pathologic examination of the specimen was consistent with a very primitive lipoblastoma. This tumor is a rare, benign lesion of immature fat cells that is found almost exclusively in the pediatric population. Lipoblastomas are more common in males than females and frequently present as asymptomatic, rapidly enlarging, soft lobular masses on the extremities. Complete surgical excision is the definitive treatment. In the vast majority of reported cases, however, the preoperative diagnosis was incorrect, underscoring the diagnostic dilemma presented by these rare tumors.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/78. A case of malignant transformation in thoracic vertebral hemangioma following repetitive irradiation and extraction.We report a rare case of thoracic vertebral hemangioma which developed into angiosarcoma during the course of repetitive operations and irradiation. A 44 year old female was operated on for hemangioma of the first thoracic vertebra. The diagnosis of hemangioma was confirmed histopathologically with the specimen from the first operation. The tumor developed multiple lesions later in the clinical course after the first operation, these lesions were removed in four consecutive operations and each histological diagnosis was that of hemangioma. Throughout the period of these operations, the patient was treated with steroid, and with radiotherapy simultaneously. The patient underwent the fifth operation for the recurrence of the tumor on 26 March 1990, and the histopathological diagnosis was not hemangioma but hemangiosarcoma which was considered a malignant transformation. The tumor cells immunohistochemically revealed positive staining with UEA-I, Factor-VIII, as the tumor immunohistochemically showed a vascular endothelioid nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/78. astrocytoma with angiomatoid vascular proliferation ("angiomatous astrocytoma").We report a surgical case of unusual anaplastic astrocytoma which was accompanied by an exuberant proliferation of abnormal blood vessels with features resembling those of capillary telangiectasis or cavernous angioma. The patient was a 39-year-old man, who presented with a generalized convulsive seizure, and neuroradiological examination revealed a tumor in the left frontal lobe. The resected tumor showed the features of an anaplastic astrocytoma, grade 3. The proliferation of abnormal blood vessels with dilated lumina and thin walls was seen throughout the tumor, and in the central area these vessels were densely packed and almost replaced the neoplastic astrocytic tissue. Although these dense vascular aggregates in the central area closely simulated capillary telangiectasis or cavernous angioma, they were considered to be of a reactive nature. The term "angiomatous astrocytoma", which is analogous to angiomatous meningioma, seemed to be the most appropriate for the present tumor.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/78. Solitary skeletal hemangioma of the extremities.OBJECTIVE: To report the clinicopathologic features of solitary skeletal hemangioma of the extremities and to review previous cases in the English language medical literature. patients: In addition to five of our own cases, 34 literature cases with substantial and 75 with partial clinicopathologic information were found. RESULTS: Our patients, three men and two women, ranged in age from 37 to 83 years (mean 65.6 years). The lesion was an incidental radiologic finding in two patients, while three were symptomatic. In no case was a correct preoperative radiologic diagnosis made, a malignant process being considered as a possibility in all. The hemangiomas were medullary; two involved a metacarpal, two the fibula, and one the humerus. In contrast, previously reported patients were younger (mean age 32 years), predominantly female (60%), and symptomatic in over 90% of cases. The lesion is rare in those younger than age 10 years or older than age 60 years. As in our patients, the long bones are most frequently involved (75%), with the diaphysis or metadiaphysis, as in four of our patients, the most common locations. Although 20% of cases occur in the hands or feet, metacarpal involvement is rare. Medullary origin, as in all of our cases, is most frequent, but 45% of cases are either periosteal (33%) or intracortical (12%). In the literature, cavernous hemangioma is the most frequent type. Three of our hemangiomas were cavernous, one capillary, and one venous, the latter being rarely reported in extremity bones. CONCLUSIONS: Due to the diversity of radiologic patterns produced by skeletal hemangioma, a correct preoperative diagnosis is rarely made. Almost all patients do well, even those with less than complete removal of the lesion; local recurrence is rare. All of our patients were well following either therapeutic or simple diagnostic procedures. Due to the destructive nature of some biopsy procedures, the histologic diagnosis of hemangioma may at times also be problematic.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/78. Thyroid hemangioma.A case is presented of a fifty-six year old man with a thyroid hemangioma presenting as a neck mass with tracheal deviation and unilateral vocal cord paralysis. A standard thyroid scan yielded equivocal findings. The diagnosis was determined preoperatively by the use of a 99m-technetium angiogram that disclosed the vascular nature of the lesion. Subsequent arteriography demonstrated the main arterial supply of the mass to be from both inferior thyroid arteries. Operative removal of the mass was accomplished through a standard transverse cervical incision. Pathologic study revealed that the vascular tumor involved both the surrounding normal thyroid parenchyma and also a microfollicular adenoma. This case points out the value of utilizing all available diagnostic means in atypical cases of thyroid disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/78. Intramuscular hemangioma of posterior neck muscles.Intramuscular hemangiomas of the head and neck are rare tumors, sparsely reported. They usually present themselves as a mass which enlarges suddenly. A case of intramuscular hemangioma involving the posterior neck muscles is presented. Computed tomography scanning, magnetic resonance imaging and angiography revealed the vascular nature of this lesion. Surgery consisted of a wide excision. The patient is free of disease after a 4-year follow-up.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/78. thoracic outlet syndrome caused by first rib hemangioma.We report a case of first rib hemangioma that caused thoracic outlet syndrome. A 50-year-ole woman who was admitted to our hospital with a clinical diagnosis of thoracic outlet syndrome presented with fullness and easy fatigue of her right arm. Her right arm discomfort was associated with intermittent engorgement of superficial veins over the shoulder girdle. A chest radiograph revealed an enlargement of the anterior aspect of the first rib with fine bony trabeculations. Computed tomography scan showed contrast enhancement over the enlarged rib. Our tentative preoperative diagnosis was a benign first rib hypertrophic change, such as an old fracture with exuberant callus formation. A right-arm venogram revealed a patent subclavian vein with an extrinsic compression, which occluded on arm abduction. The findings of neural conduction studies of both upper extremities were symmetric and normal. The patient agreed to surgery because of the occlusive condition of the subclavian vein on arm abduction and progressive arm weakness in recent months. Segmental transection of the offending portion of the enlarged first rib was complicated by difficulty in isolating the whole length of the compressed but normal-appearing subclavian vein by our initial transaxillary and infraclavicular approaches because the medial aspect of the subclavian vein was obstructed by the enlarged first rib, which extended medially to the junction of the right jugular and subclavian veins. Successful segmental transection of the enlarged first rib was finally accomplished by combined transaxillary, infraclavicular, and supraclavicular approaches. A moderate amount of rib bleeding from resection ends was noted during segmental resection of the enlarged first rib, resulting in local hematoma formation. A 470-mL bloody discharge was collected from the vacuum ball inserted via the transaxillary route during her 12-day hospitalization. Pathologic examination revealed an intraosseous hemangioma. The patient had a prolonged course to partial recovery of her arm numbness, but signs of venous compression were much improved at 6 months' follow-up. Although hemangioma is benign, its hypervascular nature may cause catastrophic intraoperative bleeding.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/78. Left atrial cardiac hemangioma associated with shortness of breath and palpitations.We present a patient with a left atrial hemangioma associated with shortness of breath and irregular heart-beats. Imaging modalities used to evaluate this rare tumor demonstrated characteristic features of its vascular nature. After successful surgical excision of the tumor under cardiopulmonary bypass, there were no clinical or echocardiographic evidence of recurrence at 18 months.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/78. Report of a child with aortic aneurysm, orofacial clefting, hemangioma, upper sternal defect, and marfanoid features: possible PHACE syndrome.We report a female patient who had a scalp hemangioma, a cleft uvula, an upper sternal defect, pectus excavatum, arachnodactyly, pes planus, and joint hypermobility. She had rupture of an aortic aneurysm after minor trauma at 11 years of age. At 17 years of age, elective repair of a dilated, ectatic aorta was complicated by cerebral ischemia. Other vascular abnormalities in the proband included an aneurysm of the left subclavian artery, atresia of the right carotid artery, and calcified cerebral aneurysms. We believe that the proband's physical anomalies are best described by the PHACE (posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) phenotypic spectrum. This spectrum of physical anomalies also includes sternal clefting and hemagiomas as part of the sternal malformation/vascular dysplasia (SM/VD) association, as found in our patient, and the acronym PHACES has also been used. We consider that the PHACE phenotypic spectrum is likely to be broader than previously recognized and includes orofacial clefting and aortic dilatation and rupture. Our patient also had skeletal anomalies that lead to consideration of marfan syndrome as a diagnosis. It should be recognized that there is clinical overlap between PHACE syndrome and marfan syndrome when aortic dilatation is present. We would also like to emphasize the minor nature of the cutaneous findings in our patient despite her severe vascular complications. This is in contrast to previous reports of large or multiple hemangiomas in PHACE syndrome.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
| | Next -> |