1/55. Management of sinonasal hemangiopericytomas.The purpose of the present study is to report four cases of sinonasal hemangiopericytoma (HP) diagnosed and treated in our department between 1987 and 1998. The pretreatment findings and the treatment are described and discussed in the light of the literature. HP are unusual vascular tumors, featuring pericytes distributed around normal vascular channels. Two of these four cases were located in the nasal cavity and the other two were located in the maxillary sinus. Inside the nasal cavity, HP presented as a protruding reddish-gray mass with marked bleeding on contact. Electron microscopy and immunohistochemical techniques are essential for diagnosis and to distinguish HP from other sarcomatous tumors. Preoperative assessment included routine CT, MRI, arteriography and selective embolization. These tumors must be treated surgically with complete excision. An endonasal approach was performed in two cases of intranasal HP, while a combined external-endonasal approach was required for the other two cases of HP.- - - - - - - - - - ranking = 1keywords = hemangiopericytoma (Clic here for more details about this article) |
2/55. Malignant hemangiopericytoma of the bone.hemangiopericytoma is a rare vascular tumor of pericyte origin with variable malignant potential. Very rarely, this tumor occurs as a primary bone lesion. We present a case of a highly malignant hemangiopericytoma of the proximal tibia. Current therapy consists of radical resection of the tumor with postoperative radiation therapy being recommended. Chemotherapy seems to be useful in disseminated disease. The prognosis correlates to the histological grading of the tumor. Early or late recurrence and distant metastases with fatal outcome, as shown in our case study, are not uncommon.- - - - - - - - - - ranking = 1keywords = hemangiopericytoma (Clic here for more details about this article) |
3/55. Clinicopathological features of solitary fibrous tumor of the meninges: An immunohistochemical reappraisal of cases previously diagnosed to be fibrous meningioma or hemangiopericytoma.Cases of solitary fibrous tumor (SFT) of the meninges are increasingly being reported. However, the real incidence of SFT among meningeal tumors has yet to be determined. We therefore clinicopathologically re-examined 64 meningeal tumors originally diagnosed to be either fibrous meningioma (FM group, n = 46) or hemangiopericytoma (HPC group, n = 18) while paying special attention to SFT. We thus reclassified one case from the FM group (2%) and one case from the HPC group (6%) to be SFT, both of which showed diffuse CD34-immunoreactivity and dense intercellular reticulin fibers but neither epithelial membrane antigen nor S-100 protein expression. The MIB-1 staining index of these cases were 6. 2% and 3.9%, respectively. The former recurred 15 years after the initial surgery and the patient underwent a second removal of the tumor. The patient has been alive with no evidence of recurrence for 7 years after the second surgery. The latter patient has been alive with no evidence of recurrence for 3 years postoperatively. The results confirmed that the incidence of SFT among meningeal tumors is relatively low, however, because of its clinically indolent nature, a careful histochemical examination is necessary to differentiate SFT from other neoplasms with a more aggressive nature. Our findings emphasize the need to clinically recognize this lesion as a distinct entity.- - - - - - - - - - ranking = 1keywords = hemangiopericytoma (Clic here for more details about this article) |
4/55. Extrapleural pneumonectomy for sarcomas report of two cases.AIMS AND BACKGROUND: Extrapleural pneumonectomy (EPP), which is a very uncommon surgical procedure, is electively indicated only in patients with early stages of malignant pleural mesothelioma, a rare condition. Two adults suffering from sarcomas and treated with EPP are described here. methods: A 29-year-old male with four left-sided lung metastases and ipsilateral pleural effusion from a chondrosarcoma of the mandibula and a 64-year old woman with a megamass in the left chest due to a local recurrence of a hemangiopericytoma underwent EPP. RESULTS: Extra-EPP-field multiorgan progression was diagnosed 14 months following surgery in the first patient who died at the 24th postoperative month but remained free of disease at the site of surgery. The second patient had a chest wall relapse at the forty-third month following EPP, which was treated by partial resection of the second and third ribs. She is alive and disease-free at the twelfth postoperative month. DISCUSSION: EPP may be considered for salvage treatment in selected patients with intrathoracic sarcomas not amenable to other effective therapies to achieve mid- to long-term disease control, even in the case of advanced spread.- - - - - - - - - - ranking = 0.2keywords = hemangiopericytoma (Clic here for more details about this article) |
5/55. Proliferation index as a prognostic marker in hemangiopericytoma of the head and neck.BACKGROUND: hemangiopericytoma (HPC) of the head and neck is a rare neoplasm whose biologic behavior is difficult to predict by means of conventional histologic parameters. methods: H & E-stained sections from 12 cases of HPC were reviewed. Proliferation index was assessed using an immunoperoxidase stain for MIB-1 (Ki-67). RESULTS: The study group consisted of 4 adult men, 5 adult women, and 1 infant male. necrosis, hypercellularity, and pleomorphism were found in 1, 5, and 6 case(s), respectively. The mitotic index per 10 high power fields varied from 0-1 to 15. Proliferation indices using MIB-1 ranged from 2.6% to 52.5%. Clinical follow-up revealed 3 cases with recurrence all possessing proliferation indices of approximately 10%. CONCLUSIONS: Standard histomorphologic features may be inadequate predictors of clinical outcome. A proliferation index of 10% or greater may indicate a more aggressive subset of HPC of the head and neck.- - - - - - - - - - ranking = 0.8keywords = hemangiopericytoma (Clic here for more details about this article) |
6/55. insulin-like growth factor ii-producing intra-abdominal hemangiopericytoma associated with hypoglycemia.We report a patient with insulin-like growth factor (IGF)-II-producing hemangiopericytoma with hypoglycemia in whom repeated intra-abdominal recurrences developed over a period of about 10 years and tumor resection was performed four times. A 67-year-old woman was admitted to our hospital in 1995 because of hypoglycemic attacks. In 1985, partial resection of the small bowel had been performed for a 17-cm abdominal tumor of the transverse mesocolon, and the pathological diagnosis was hemangiopericytoma. In 1991, left hemicolectomy had been performed for a mesosigmoidal tumor associated with hypoglycemia. In 1994, hysterectomy, bilateral adnexectomy, and resection of an intrapelvic tumor were performed. The fourth operation was performed in 1996, about 10 years after the first operation. The spleen was removed, together with more than 1500 tumors having a total weight of 1,660 g. The hypoglycemia was ameliorated after each operation. Before this operation, her serum IGF-I level was low, but her IGF-II level was within the normal range; however, the Western immunoblot method showed that most of the IGF-II was high-molecular-weight IGF-II. The tissue IGF-I level was also low, and the IGF-II level was high, suggesting an IGF-II-producing tumor. We suspect that the mechanism of the hypoglycemia in this patient was related to the high-molecular-weight IGF-II produced by the tumor. The patient died in 1997 because of tumor recurrence.- - - - - - - - - - ranking = 1.2keywords = hemangiopericytoma (Clic here for more details about this article) |
7/55. Tandem high-dose chemotherapy supported by autologous peripheral blood stem cell transplantation for recurrent soft tissue sarcoma.BACKGROUND: patients with recurrent soft tissue sarcoma (STS) are seldom curable, with 5-year survival rates of less than 10% in all large series. The role of high-dose chemotherapy (HDC) with hematopoietic stem cell support in this disease has not been established. CASE REPORT: We report on two patients with recurrent STS who were treated with tandem HDC supported by autologous peripheral blood stem cell transplantation (PBSCT). One patient with malignant fibrous histiocytoma recurred with multiple lung metastases. This patient achieved a partial response after two cycles of induction chemotherapy consisting of ifosfamide and epirubicin. During four cycles of induction chemotherapy, peripheral blood stem cells (PBSCs) were harvested. Tandem high-dose ice regimen (ifosfamide 3 g/m2 on days-7 to -3, carboplatin 400 mg/m2 on days-7, -5 and 3, etoposide 500 mg/m2 on days-7, -5 and 3) supported by autologous PBSCT gave rise to further regression of the tumors. Another patient with malignant hemangiopericytoma was treated by tandem high-dose ice regimen supported by autologous PBSCT after the 3rd removal of abdominal tumors. Relapse-free intervals until the 1st, 2nd and 3rd relapses were 40, 19 and 22 months, respectively. Tandem high-dose ice regimen might delay the relapse. CONCLUSION: These observations suggest that a tandem high-dose ice regimen with autologous PBSCT is feasible with some clinical efficacy in the control of refractory STS.- - - - - - - - - - ranking = 0.2keywords = hemangiopericytoma (Clic here for more details about this article) |
8/55. Orbital hemangiopericytoma--a case report.A 50-year-old male presented with progressive proptosis in the right eye for 10 years. Decreased visual acuity to hand-motion level was also noted. Orbital computed tomography revealed a large homogenously enhanced mass lesion in the right retrobulbar region with optic nerve compression. The remainder of the ocular examination was normal, except for right mature cataract. The tumor was completely removed through lateral orbitotomy. Histologic findings showed a picture of hemangiopericytoma. One month after cataract extraction and intraocular lens implantation in the right eye, the patient had restored vision. Through 2 years' follow-up, no more orbital tumor was noted. Orbital hemangiopericytoma may have potentially malignant behavior, and a high local recurrence rate if incompletely excised. Complete, intact removal prevents tumor recurrence and provides a good result.- - - - - - - - - - ranking = 1.2keywords = hemangiopericytoma (Clic here for more details about this article) |
9/55. Solitary fibrous tumor of the orbit: is it rare? Report of a case series and review of the literature.PURPOSE: The real incidence of solitary fibrous tumor (SFT) of the orbit is unknown, but it seems that since it was first described in 1994, orbital SFT has been increasingly recognized. We believe that the orbital SFT is a relatively common tumor and that it should be considered in the differential diagnosis of any orbital tumor. DESIGN: Interventional case series. PARTICIPANTS: Four new cases of orbital SFT. methods: Four patients affected by solitary fibrous tumor of the orbit are described. One patient experienced a recurrent SFT shortly after initial surgical excision performed elsewhere. Thirty-eight cases have been reported in the literature in 7 years. RESULTS: The number of orbital SFTs reported has been increasing, reaching an average of more than five tumors reported per year. Since the first orbital SFT was described in 1994, 37 cases have been reported in the literature. We add four new cases in our series, including a recurrent tumor. A total of 42 cases have now been described, eight with recurrences. Malignant transformation occurred in one case. CONCLUSIONS: We believe that before 1994, the diagnosis orbital SFT was confused with other benign orbital tumors, such as fibrous histiocytoma and hemangiopericytoma because of a lack of use of immunohistochemical techniques. This entity should now be considered relatively common and should be included in the differential diagnosis of orbital tumors in any age group. Local recurrences of SFT are possible and usually follow an incomplete initial excision. Recurrent tumors in the orbit have shown the tendency to infiltrate the surrounding tissues and the bone, rendering complete secondary excision more difficult. Recurrent orbital SFT also has the potential for malignant transformation. The treatment of choice of orbital SFT is complete surgical excision and careful follow-up. Considering the more aggressive course followed by recurrent tumor, correct diagnosis and management is essential.- - - - - - - - - - ranking = 0.2keywords = hemangiopericytoma (Clic here for more details about this article) |
10/55. myofibromatosis-like hemangiopericytoma metastasizing as differentiated vascular smooth-muscle and myosarcoma. Myopericytes as a subset of "myofibroblasts".A thyroid hemangiopericytoma that was resected in a 5-year-old boy recurred insidiously in the larynx 8 years later. Marked cicatricial mucosal inflammation prevented a definitive pathologic diagnosis of recurrence until a nodule grew to obstruct the airway 15 years after initial surgery. After excision of the nodule, a larger sarcomatous metastasis was discovered in the upper esophagus and resected, but the patient eventually succumbed to widespread disease at the age of 20 years. The original tumor contained atypical pericytes and bundles of hyalinizing smooth muscle abutting on "staghorn vessels," a pattern similar to infantile myofibromatosis. desmin immunostaining was negative in the pericytes but positive in smooth-muscle cells dispersed singly as well as in bundles. Both elements reacted strongly for vimentin and the alpha-isoform of actin (alpha-SMA) found in normal smooth muscle and pericytes. A third cell type showing dendritic processes and immunoreactivity for all three antigens was interpreted as a myopericyte. Spindled cells in multiple subsequent mucosal biopsy specimens stained retrospectively also positive for these antigens. Large bundles of vascular smooth muscle surrounded by radiating myocytes characterized the occluding laryngeal nodule. In the esophageal metastasis, which showed no histologic features typical of hemangiopericytoma, numerous mitotically active, small, vimentin , desmin , alpha-SMA cells often maintained shortened processes and tended to form nodular aggregates about capillaries. Single rows of pericytes accreted to endothelial tubes. Ultrastructurally, some cells contained myofilaments and irregular dense material or showed rare cell junctions and variable investment by a basal lamina.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 1.2keywords = hemangiopericytoma (Clic here for more details about this article) |
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