A case of hemangiopericytoma of the soft palate with articulate disorder and dysphagia

We report a case of hemangiopericytoma of the soft palate of 60-year-old patient, who noticed a mass of the soft palate and experienced difficulty in speaking. We found a pediculate, hard, elastic mass measuring 38 mm （cross-sectional diameter）. Computed tomography （CT） scans and dynamic magnetic resonance imaging （MRI） confirmed irregularly shaped mass and revealed a heterogeneous internal composition, consistent with vascular tumors. We excised the tumor under general anesthesia. Histopathological diagnosis was based on positive immunoreactivity of CD99 and vimentin and weak, positive staining of CD34. Three and half years following tumor excision, there is no recurrence or metastasis.

Angioarchitecture and CD133^+ tumor stem cell distribution in intracranial hemangiopericytoma A comparative study with meningioma

Angioarchitecture plays an important role in the malignant development of intracranial hemangiopericytoma. It remains poorly understood whether high frequency of hemorrhage during clinical surgery for intracranial hemangiopericytoma is associated with angioarchitecture. The present study utilized hematoxylin-eosin staining, and immunohistochemical staining with epithelial membrane antigen, vimentin, CD34, von Willebrand factor （vWF） and CD133 to observe characteristics of angioarchitecture. In addition, silver stains were used to demonstrate changes in reticular fibers in the wall of vessel channels in intracranial hemangiopericytoma and meningioma. Five patterns of angioarchitecture were identified in intracranial hemangiopericytoma, namely tumor cell islands, vasculogenic mimicry, mosaic blood vessels, sprouting angiogenesis, and intussusceptive angiogenesis. Several CD133＋ tumor cells were found to form tumor cell islands. A connection between vWF ^＋ and vWF channels was detected in the pattern of intussusceptive angiogenesis, and some vimentin^＋ tumor cells were embedded in the periodic acid-Schiff positive channel wall. Incomplete threads of reticular fibers formed the walls of larger pseudo-vascular channels and some tumor clumps or scattered tumor cells were detected ＂floating＂ in them. The angioarchitecture, specific markers and reticular fibers of intracranial hemangiopericytoma were significantly different from meningioma. Angioarchitecture provides a functional vascular network for vascular evolution in intracranial hemangiopericytoma and contributes to significant intra-operative bleeding.

Intracranial Hemangiopericytoma Focally Recurrent to the Pelvis

A 42-year-old male with a history of recurrent intracranial hemangiopericytoma presented to the Emergency Department with urinary retention. Ensuing work-up revealed a 10-centimeter circumscribed pelvic mass that appeared anatomically distinct from the sacrum. This metastasis was found 10 years after the initial resection of his intracranial lesion. An abdominal resection was performed, and the patient recovered uneventfully. Final pathology and staining findings were consistent with metastatic hemangiopericytoma. Hemangiopericytoma is a rare mesenchymal soft tissue neoplasm with potential for late local recurrence and metastasis. Here, we review the case and discuss the clinical relevance of narrowing the diagnosis to HPC or the growing umbrella of Solitary Fibrous Tumor (SFT). To our knowledge, this is the first report of intracranial HPC metastatic to the pelvis.

Infantile Hemangiopericytoma: Two Cases Report and Literature Review

Hemangiopericytoma is a rare vascular tumour of infants. Although generally considered to be benign, local recurrence and metastases can occur. Herein, we report on two full term girls, delivered with lumbosacral swelling and left thigh swelling respectively. Complete surgical excision with safety margins was performed for each lesion. Histologic examination of both lesions showed picture of infantile hemangiopericytoma. There is no evidence of local recurrence or distant metastasis during last 20 and 17 months for 1st case and 2nd case respectively. In conclusion;most infantile hemangiopericytoma follow a benign course. Rarely these tumours behave aggressively with local infiltration, recurrences and even distant metastases. Careful follow up is therefore essential.

Lumbar Paraspinal Hemangiopericytoma, An Unusual Location: Surgery with Preoperative Embolization

Background: Lumbar paraspinal hemangiopericytoma is rare. The hemorrhagic nature of the tumor causes problems of hemostasis and makes the resection delicate. We report a rare case of lumbar paraspinal hemangiopericytoma managed with preoperative embolization. Clinical Case: It is about a woman of 31 years, admitted for a large left lumbar paraspinal mass that evolved for 6 years. The clinical exam showed an asymmetry of the left paraspinal muscle. The mass painless was extended from the lumbar region. The neurological exam was normal. MRI showed the left large paraspinal mass tissue. It was extended from lumbar vertebrae, L1 to L4, and measured 100 × 50 × 50 mm. It was an iso-intense signal on T1-weighted with strong enhancement after gadolinium. It was hypervascular and supplied by left intercostal T12, L1, and L2 pedicles. The complete exclusion of the hyper-vascular left paraspinal tumor was obtained after selective embolization of the artery left L1 of the pedicle of the intercostal left L1 and trunks intercostal T12 and L2 left. The total resection of a mass encapsulated was performed. The operative outcome was good. The histology concluded to a hemangiopericytoma. No chemotherapy or radiotherapy was prescribed. After 5 years, the patient was asymptomatic. MRI control confirmed tumor resection with a residue at the level of the left intervertebral foramen L1 - L2. Conclusion: Lumbar paraspinal hemangiopericytoma is an extremely rare tumor. Selective preoperative embolization is recommended before the resection of large tumors to reduce vascular supply. A follow-up extended for these patients is necessary, given the frequency of recurrences.

Recurrent Sinonasal Hemangiopericytoma: A Rare Case Report

Hemangiopericytoma (HPC) is a rare vascular tumor arising from capillary pericytes. This tumor represents 3% - 5% of all soft tissue sarcomas and 1% of all vascular tumors. Only 15% - 30% of cases occur in the head and neck region, among which sinonasal hemangiopericytoma (HPC) is accounted for around 5% of cases. Hemangiopericytoma (HPC) has a tendency for recurrence. Here, a case of recurrent nasal hemangiopericytoma (HPC) after 12 years of treatment is reported.

A Rare Case of a Thoracic Spinal Hemangiopericytoma

Spinal hemangiopericytomas are rare tumors. To date, only 80 cases of spinal hemangiopericytomas have been reported in the literature. The postoperative evolution of this condition in rare cases can be complicated by a symptomatic compressive epidural hematoma that can be the source of serious neurological complications requiring emergency surgery. We report a rare case of intradural and extramedullary spinal hemangiopericytomas with favorable evolution after treatment in an 82 years old woman.

Chest Wall Hemangiopericytoma-Like Solitary Fibrous Tumor of the Pleura: Case Report with Computed Tomography Findings

We present a case report of a 65-year-old woman who underwent resection of a chest wall tumor. In contrast with computed tomography, the tumor exhibited hypervascularity and was fed from the 9th intercostals artery. Histologically, the tumor was diagnosed as a cellular variant of solitary fibrous tumor of the pleura, with branching “staghorn” vessels and hypervascularity. The tumor thus resembled a chest wall hemangiopericytoma.

Meningeal Hemangiopericytoma: A Rare Intracranial Tumor—About a Case

Introduction: Hemangiopericytoma is a very rare vascular tumor, difficult to diagnose, often confused with a meningioma. Imaging is not very specific and the diagnostic certainty remains histological. Observation: We report a case of meningeal hemangiopericytoma in a 48-year-old patient with a history of active smoking and whose sister died of glioblastoma. He was brought to the emergency room for a fall, confusion, and tonic-clonic seizures. The diagnosis was suspected on imaging (CT and MRI, in front of a syndrome of right temporal intraventricular mass measured at 37 mm in diameter, centered on the choroid plexus, with perilesional vasogenic edema and posterior white commissure. There was homogeneous enhancement of the lesion after gadolinium injection. The diagnosis was confirmed by immunohistochemical study, which showed diffuse expression of STAT6 and partial labeling for CD34, without significant expression of SSTR2. Expression of neurofilament protein was also found in the brain parenchyma. Conclusion: Hemangiopericytoma is a rare tumor, characterized by its malignant potential, its high rate of recurrence and distant metastasis. The management is based on a total microsurgical resection followed by radiotherapy.

Intracranial hemangiopericytoma with right-sided aortic arch: a case report and summary of experience

Hemangiopericytoma is clinically rare and easily confused with meningioma.Right-sided aortic arch is also a rare disease of congenital cardiovascular variability.The intracranial hemangiopericytoma associated with the right-sided aortic arch has not been reported in the literature.We are here to register a case of right aortic arch concurrent with intracranial hemangiopericytoma;the patient was initially thought to be a simple meningioma,but he was eventually diagnosed as a hemangiopericytoma with right aortic arch and multiple intracranial vascular stenosis and malformation.Whether there was a genetic correlation between the two provides a potential research direction for us.Therefore,the diagnosis and treatment of some rare diseases should be a more comprehensive analysis and evaluation.Adequate examination and accurate diagnosis are crucial,which will directly affect the efficacy of surgery and the prognosis of patients.

Clinical study and differential diagnosis of intracranial hemangiopericytomas

Objective Intracranial hemangiopericytomas (HPCs) were rare neoplasms with relatively high rates of recurrence and extracranial metastasis.The differential diagnoses from angiomatous meningiomas and solitary fibrous tumors(SFTs) are very important. This study aimes to reveal differences in the specific immunohistochemical features of HPCs,angiomatous meningiomas and SFTs by newly

Lipomatous hemangiopericytoma of the stomach:A case report and a review of literature

Lipomatous hemangiopericytomas(LHPCs) are rare soft-tissue tumors that are histologically characterized by hemangiopericytomatous vasculature and the presence of mature adipocytes.We present the clinicopathological features of a case of gastric LHPC in a 56-year-old female,along with a literature review.Endoscopy and endoscopic ultrasound showed a submucosal tumor 0.8 cm across in the greatest dimension in the lesser curvature side of the gastric antrum.Grossly,the well-defined mass had a solid and tan-white cut surface admixed with myxoid regions and yellowish areas.Histological examination revealed a submucosal well-circumscribed lesion composed of cellular nodules with the classic appearance of an hemangiopericytoma admixed with clusters and lobules of mature adipocytes.The ill-defined tumor cells had weakly eosinophilic cytoplasm and contained spindled nuclei with occasional small nucleoli.Nuclei atypia and mitoses were absent,and no cellular atypia,necrosis or vascular invasion was observed.Immunohistochemistry showed that the tumor cells were diffusely positive for CD34,CD99,and vimentin and were focally reactive for bcl-2.This is the first known report of an LHPC in the stomach.The patient was followed for 12 mo without any evidence of metastasis or recurrence.

Splenic hemangiopericytoma and serosal cavernous hemangiomatosis of the adjacent colon

A healthy 31-years-old man presented with a three-year history of abdominal discomfort. Radiological examinations revealed multifocal tumoral lesions in the spleen. The patient underwent splenectomy for differantial diagnosis and treatment. During the operation, in addition to the splenic masses, there were also multiple milimetric purpuric-like lesions on the colonic serosal surfaces adjacent to the splenic hilus. One of them was excised.Histologic examination showed hemangiopericytoma of the spleen and cavernous hemangioma of the adjacent colon. This is the first report showing the close association of these two distinct lesions with vascular origin in the literature. Despite not having any apparent evidence, there may be a sequential relationship between the hemangiopericytoma of the spleen and cavernous hemangiomas.

Combined Therapy against Recurrent Hemangiopericytoma:A Case Report

A patient with a seven-year history of recurrent metastatic hemangiopericytoma(HPC) was admitted.During his treatment,he received surgical resection,radiotherapy,radiofrequency hyperthermia and chemotherapy using combined doxorubicin,dacarbazin, vincristine,ginsenoside Rg3,and recombinant human endostatin.This synergistic method provides an encouraging model for treating HPC.

右侧颅骨肌周细胞瘤1例

患者女,25岁,无明显诱因间断性头痛3天;既往体健。查体和实验室检查均未见明显异常。头颅CT/MRI:右侧额顶骨交界处4.2 cm×3.1 cm×3.9 cm宽基底圆形不均匀高密度/信号肿块,向颅内膨胀性生长(图1A、1B),呈T1WI等-稍低信号、T2-液体衰减反转恢复(fluid attenuated inversion recovery,FLAIR)高信号(图1C、1D)、弥散加权成像(diffusion weighted imaging,DWI)低信号(图1E),增强后无明显强化,周围管状血管强化而呈“胡椒盐”征(图1F、1G);肿块包膜不完整,可见“皮髓质移位”征;考虑钙化型脑膜瘤。

Quantitative evaluation of benign meningioma and hemangiopericytoma with peritumoral brain edema by 64-slice CT perfusion imaging

Background Hemangiopericytomas （HPCs） have a relentless tendency for local recurrence and metastases,differentiating between benign meningiomas and HPCs before surgery is important for both treatment planning and the prognosis appraisal.The purpose of this study was to evaluate the correlations between CT perfusion parameters and microvessel density （MVD） in extra-axial tumors and the possible role of CT perfusion imaging in preoperatively differentiating benign meningiomas and HPCs.Methods Seventeen patients with benign meningiomas and peritumoral edema, 12 patients with HPCs and peritumoral edema underwent 64-slice CT perfusion imaging pre-operation.Perfusion was calculated using the Patlak method.The quantitative parameters, include cerebral blood volume （CBV）, permeability surface （PS） of parenchyma, peritumoral edema among benign meningiomas and HPCs were compared respectively.CBV and PS in parenchyma, peritumoral edema of benign meningiomas and HPCs were also compared to that of the contrallateral normal white matter respectively.The correlations between CBV, PS of tumoral parenchyma and MVD were examined.Results The value of CBV and PS in parenchyma of HPCs were significantly higher than that of benign meningiomas （P〈0.05）, while the values of CBV and PS in peritumoral edema of benign meningiomas and HPCs were not significantly different （P 〉0.05）.MVD in parenchyma of HPCs were significantly higher than that of benign meningiomas （P〈0.05）.There were positive correlations between CBV and MVD （r=0.648, P〈0.05）, PS and MVD （r=0.541, P〈0.05） respectively.Furthermore, the value of CBV and PS in parenchyma of benign meningiomas and HPCs were significantly higher than that of contrallateral normal white matter （P〈0.05）, the value of CBV in peritumoral edema of benign meningiomas and HPCs were significantly lower than that of contrallateral normal white matter （P〈0.05）, while the value of PS in peritumoral edema of benign meningiomas and HPCs were not significantly different with that of contrallateral normal white matter （P 〉0.05）.Conclusions CT perfusion imaging can provide critical information on the vascuiarity of HPC and benign meningiomas.Determination of maximal CBV and corresponding PS values in the parenchyma may be useful in the preoperative differentiating HPC from benign meningiomas.

Clinical and pathological characteristics of primary intraspinal hemangiopericytoma and choice of treatment

Background Primary intraspinal hemangiopericytoma is a rare malignant mesenchymal tumor with high rates of recurrence and metastasis. Surgery is the main therapeutic procedure for this lesion. This clinical research was undertaken to analyze the pathological characteristics, clinical course, and the choice of treatment for this lesion. Methods Twenty-three patients with primary intraspinal hemangiopericytomas were treated from 1987 to 2004. The clinical and imaging features, pathological findings, therapeutic procedures, and prognosis were analyzed retrospectively. Results Primary intraspinal hemangiopericytoma is more likely to attack middle-aged persons. The tumor mainly manifests as muscle weakness and sensor abnormalities. Microscopic examination showed slit-like vascular spaces and oral- or spindle-shaped cells with slightly acidic cytoplasm and oral nuclei. Tumors were subtotally resected in 11 patients, subtotally resected with postoperative radiotherapy in 4, totally resected in 5, and totally resected with postoperative radiotherapy in 3. Two patients were given spinal stabilization after total resection. Recurrence and metastatic rates were 50% and 0 in intradural patients. They were 73% and 27% in extradural patients, respectively. Conclusions The tumor should be resected en bloc with the neighboring dural mater to reduce recurrence and metastasis. Patients with subtotal resection need adjuvant radiotherapy. Patients with evident spinal involvement may benefit from spinal stabilization. The prognosis of the lesion arising from the dural mater is better.

Recurrent intracranial hemangiopericytoma with multiple metastases

Hemangiopericytoma （HPC） is a potential malignant tumor originating from Zimmermann＇s pericytes around capillaries and postcapillary venules, which is most common in soft tissues. Being rarely encountered, intracranial HPC accounts for 2%-4% in meningeal tumors or less than 1% in all intracranial tumors. The histogenesis of this tumor has been debated as a specific aggressive variant of meningioma or angioblastic meningioma for a long time.

Retroperitoneal hemangiopericytoma： case report and literature review

Retroperitoneal hemangiopericytoma is a kind of uncommon tumor. We report a case of 41-year-old man who was diagnosed retroperitoneal tumor without significant symptoms by abdominal ultrasonography. Abdominal CT and MRI scans show a 6 cmx 5 cm solid tumor delineated clearly from adjacent organs. Excision of the tumor was performed and the histopatholoCliCal examination confirmed the diaanosis of hemanoionericvtorna.

Unusual occurrence of orbital hemangiopericytoma in the zygomatic bone of an adolescent: a case report

Background:Hemangiopericytoma and solitary fibrous tumor are considered related variants on the same spectrum and both may essentially be the same tumor.They are infrequently encountered in the orbital region while the zygomatic bone is an extremely rare location for these neoplasms to occur.Case presentation:A 14-year-old boy presented with complaint of deformity of left infraorbital area and a firm,regular mass in the region.Orbital CT scan revealed a well-defined round isodense intraosseous lesion in the lowermost portion of the lateral orbital wall(zygomatic bone),expanding the bone and protruding anteriorly and medially.MRI showed the mass to be heterogenous and strongly enhancing with contrast medium.Inferior transconjunctival orbitotomy was performed and the mass was removed.The histopathologic examination and immunohistochemistry staining results(positive for CD34,CD31 and smooth muscle actin,but negative for CD99,S100,B-cell lymphoma 2(bcl-2)and desmin)confirmed the diagnosis of hemangiopericytoma.The postoperative course was uneventful,with no evidence of recurrence after 5 years follow up.Conclusions:This case represents the second hemangiopericytoma reported in the zygomatic bone.Although extremely rare,hemangiopericytoma/solitary fibrous tumor might be considered in the differential diagnosis of intraosseous lesions of the orbital and zygomatic region.