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Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a rare tumor derived from mesenchymal tissue. Although standard chemotherapy for SHT/HPC has not been established, temozolomide plus bevacizumab (TMZ+Bev) therapy for SFT/HPC has been reported. The effectiveness and safety of TMZ+Bev (temozolomide 150 mg/m 2 orally on days 1-7 and days 15-21 and bevacizumab 5 mg/kg intravenously on day 8 and day 22 on a 28-day cycle), which was administered from December 2013 until April 2019 to four patients with SFT/HPC, were retrospectively analyzed. Four patients with SFT/HPC received TMZ+Bev. The age of the patients ranged from 41 to 75 years. Two were male, and the primary tumor sites were the meninges in three patients and the pleura in one. One achieved partial response; the others, stable disease (SD). The progression-free survival time ranged from 9.4 to 29.6 months according to RECIST v1.1. One patient died 59 months after using TMZ+Bev, and the others survived for 17 to 64 months. All patients experienced Grade 3 or higher lymphopenia, and three had Grade 3 or higher leukopenia and neutropenia. One patient subsequently received doxorubicin; another, pazopanib. TMZ+Bev therapy for SFT/HPC is safe and effective for maintaining long-term SD., Competing Interests: Yuichi Ando received research funding from Chugai Pharmaceutical Co., Ltd., outside the submitted work. The other authors have no conflicts of interest to disclose.

Background: Patients with locally advanced, recurrent or metastatic solitary fibrous tumour are often treated with bevacizumab and temozolomide based on the clinical efficacy reported in a case series of 14 patients. Given the rarity of solitary fibrous tumour, large trials are not feasible. We report the efficacy of this regimen based on a population-based analysis., Methods: This was a population-based retrospective, multi-centre analysis using patient data from a provincial cancer registry and treatment database. Cases from June 2006 through October 2016 were identified for patients receiving bevacizumab and temozolomide for locally advanced, recurrent or metastatic solitary fibrous tumour or hemangiopericytoma, which is sometimes used to describe tumours arising from the meninges. The primary outcome was overall response rate. Secondary outcomes included time to response, progression free survival and overall survival estimated using the Kaplan-Meier method., Results: Fourteen patients were identified: median age 59 (range 44-70), male 78.6%. Diagnoses were solitary fibrous tumour in 10 (71.4%) and hemangiopericytoma in four (28.6%), with metastatic disease in 10 (72.7%) patients. The most common primary sites were meninges in four (28.6%) and pelvis in three (21.4%) patients. The median follow-up was 15.5 months, with median treatment of four months. Overall response rate was 21.4% (no complete response, 3 partial response), with median time to response of four months. Median progression free survival, six-month progression free survival and overall survival were 17 months, 65.0%, and 45 months, respectively., Conclusions: Efficacy of bevacizumab and temozolomide in solitary fibrous tumour appeared to be similar to that previously reported. Our findings confirmed that bevacizumab and temozolomide is an effective and tolerated treatment for this patient population.

Objective: Hemangiopericytoma is a tumor of vascular origin. It is very rare in the prostate. They are generally aggressive tumors. Currently, the treatment of choice is radical surgery. In the literature, there are less than 50 cases described, and neoadjuvant treatment has not been reported in any article. This treatment presents positive responses in another type of sarcomas. Our goal is to report two cases of malignant hemangiopericytoma. The first case was treated with radical surgery and the second case was treated with neoadjuvant chemotherapy before surgery., Methods: The first case is a 40 year old male. Obstructive urinary symptoms were his first symptoms. A prostate mass with tumor characteristics was seen on ultrasound. After transrectal biopsy, he was diagnosed with hemangiopericitoma. Because the tumor size, neoadjuvant chemotherapeutic prior to radical surgery was decided. The second case is a 77 year old male with an incidental diagnosis of renal mass. After radical nephrectomy, he was diagnosed with hemangiopericitoma. He did not receive adjuvant chemotherapy., Results: In the first case, after neoadjuvant therapy, tumor size was reduced significantly. A pelvic exenteration with radical cystoprostatectomy and rectal resection and Bricker type urinary diversion and colostomy were carried out. In the second case controls after radical nephrectomy were correct and he did not need any treatment., Conclusions: In urinary tumors, the prostate hemangiopericytoma is a rare entity. Currently, neoadjuvant chemotherapy is not established as treatment for these tumors. For other sarcomas neoadyuvant treatment has good response. In our case, a good result was obtained with neoadjuvant chemotheraphy before surgery. However, a greater number of cases are necessary to establish the use of neoadjuvant chemotherapy in urinary hemangiopericytomas.

The technique of direct intratumoral injection of permanent liquid agents has evolved significantly over the past few decades. The percutaneous direct puncture technique is being utilised more and more for embolisation of head and neck hypervascular lesions. We describe a case treated via the direct percutaneous transnasal (TN) puncture technique for embolisation of a sinonasal hypervascular tumour, performed in a 21-year-old male patient. Due to extensive vascularisation of the tumour, preoperative embolisation was requested by our otolaryngologist team in order to minimise intraoperative bleeding and reduce surgical morbidity. A primary attempt at trans-arterial embolisation through selective ophthalmic artery catheterisation was unsuccessful due to unfavourable anatomy. An extensive devascularisation of the nasal fossa lesion was obtained using a single injection of low-density polymeric embolic agent (SQUID®). Post-operative eradication was uneventful. Final histopathology exam revealed vascular tumour consistent with a rare sinonasal glomangiopericytoma. To our knowledge, this is the first reported case of a rare sinonasal glomangiopericytoma treated by direct TN injection, utilising the liquid polymer agent SQUID®.

Introduction: There is currently no treatment for solitary fibrous tumors/hemangiopericytomas (SFT/H) of the central nervous system recurring after multiple surgeries and radiotherapies. The NAB2-STAT6 gene fusion is the hallmark of these tumors, and upregulates Early Growth Factor, activating several growth pathways., Methods: We treated two patients presenting pluri-recurrent meningeal SFT/H with Pazopanib, a broad-spectrum tyrosine kinase inhibitor. We analyzed the exome and RNA sequencing data of one of them and, in addition to another meningeal SFT/H, compared it to the transcriptomic profiling of 5 systemic SFT/H., Results: A dramatic clinical and radiological response was observed in both cases, respectively 84 and 43% decrease after 3 months. As a comparison, Pazopanib has only a stabilizing effect in systemic SFT/H. Indeed, central nervous system SFT/H show overexpression of different tyrosine kinases targeted by Pazopanib., Conclusions: Two consecutive patients with untreatable central nervous system SFT/H showed a spectacular partial response to Pazopanib, an unprecedented result in SFT/H. This result could be explained by differences in expression profiles and calls for a confirmation in a larger cohort of patients.

A 74-year-old man with known metastatic haemangiopericytoma presented with neuroglycopaenic symptoms of confusion and light-headedness secondary to recurrent episodes of fasting hypoglycaemia, which resolved with oral carbohydrate intake. Investigations for hypoglycaemia revealed it to be non-insulin mediated, and subsequently due to a rare paraneoplastic phenomenon termed as non-islet cell tumour hypoglycaemia caused by tumoural overproduction of high molecular weight insulin-like growth factor-II. Despite his underlying malignancy being incurable, directed multimodality treatment involving regular oral carbohydrate intake, glucocorticoid therapy and recombinant human growth hormone was effective in relieving symptoms of hypoglycaemia. We discuss the importance of a systematic diagnostic approach to hypoglycaemia fulfilling Whipple's triad, as specific therapies can be invaluable to improving quality of life., (2015 BMJ Publishing Group Ltd.)

Introduction: Hemangiopericytoma of the spleen is a very rare tumor, with 14 isolated reports. It was our aim to review our experience and compare it with all the reported cases in an attempt to standardize surgical treatment, adjuvant treatment and follow-up protocol of this infrequent condition., Methods: A consecutive case series study, with a mean follow-up of 44 months. Five patients (mean age, 49 years) underwent simple splenectomy for hemangiopericytoma limited to the spleen followed by adriamycin-based chemotherapy in one patient., Results: All the patients are alive and free from disease., Conclusions: For tumors confined to the spleen, simple splenectomy can be considered curative, without any need for further adjuvant treatment. On review of the medical literature, cure can still be achieved with complete resection of recurrences, when feasible, with adjuvant chemotherapy being also indicated. The slow-growing pattern of the tumor suggests a 10-year follow-up., (Copyright © 2015 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.)

Haemangiopericytoma is a rare soft tissue tumour characterised by tightly packed tumour cells situated around thin walled endothelial lined vascular channels, ranging from capillary sized vessels to large gaping sinusoidal spaces. The tumour cells are surrounded by reticulin and are negative for muscle, nerve and epithelial markers. The diagnosis of extra-pulmonary intra-thoracic, extra-pleural mediastinal mass is difficult. It constitutes only 6% of all primary tumours and cysts of the mediastinum. We report the rare occurrence of primary intra-thoracic, extra-pulmonary mediastinal haemangiopericytoma of mesenchymal origin with perivascular localisation. The patient underwent right postero-lateral thoracotomy and post-operatively received chemotherapy with adriamycin (60 mg/m2) on day 1 and ifosfamide (1.5 g/m2) on day 1 to 3. Thirty-seven months after the operation, the patient has been well with evidence of a single recurrence in the left lower lobe.

Collision tumors in the paranasal region are extremely rare with limited literature data. To the best of our knowledge, this is the first report of associations of squamous cell carcinoma-esthesioneuroblastoma and lymphoma-hemangiopericytoma in the paranasal region. Preoperatively, radiological and clinical findings should be evaluated carefully for the diagnosis and two or more biopsy specimens should be taken from different morphological parts of the lesions. Adjuvant therapy should be planned according to two different histologies and special importance should be given to the tumor which indicates the prognosis of the patient. A multidisciplinary approach is required for the management of synchronous malignancies.

The medical treatment of adult soft tissue sarcomas is more and more dictated by the histological subtype, this applies to both cytotoxics and target therapies. Doxorubicin and ifosfamid are the two drugs used either in monotherapy or combination with the best established response rates in adult soft tissue sarcomas for several years. In addition to these compounds there is evidence of efficacy of new drugs such as taxanes in angiosarcoma, gemcitabine+taxanes combination in leiomyosarcomas, trabectedin in leiomyosarcomas and liposarcomas with an extremely high activity in myxoid liposarcoma. With regard to target therapy pazopanib seems especially active in leiomyosarcomas and synoviosarcomas, but totally inactive in liposarcomas, sunitinib and cediranib in alveolar soft part sarcomas, sunitinib and bevacizumab+temozolamide combination in solitary fibrous tumors, and sorafenib in angiosarcomas. mTOR inhibitors are active in PEComas (perivascular epitheloid cell tumors) and crizotinib in ALK rearranged inflammatory myofibroblastic tumors. The efficacy of imatinib and sunitinib in GIST tumors are established, and that of imatinib in dermatofibrosarcoma as well.

Haemangiopericytoma is a rare tumor of vascular origin. We report the case of patient with severe refractory anaemia due to peripheral destruction of red blood cells by spleen metastases. Anaemia was successfully treated by splenectomy. Afterwards, our patient developed liver and lung metastases and was treated, in a clinical trial, with gefitinib that stabilised the disease during nine years. These interesting features are discussed.

A 7-month-old female infant presented with proptosis of the left eye. Imaging showed an intraconal mass with marked post-contrast enhancement. Histopathology revealed a diagnosis of infantile hemangiopericytoma. The patient was treated with primary chemotherapy with no recurrence until 14 months after completion of treatment., (Copyright 2012, SLACK Incorporated.)

Background: Intravascular lymphoma (IVL) is a rare subtype of large-cell non-Hodgkin lymphoma, characterized by proliferation of lymphoma cells within the lumina of small vessels. There are no previously reported cases of IVL involving the pituitary gland presenting with neuro-ophthalmic findings., Methods: A 68-year-old female presented with headache, right third nerve palsy, and Horner syndrome. MRI showed a 1.4-cm sellar mass consistent with a pituitary macroadenoma. Two weeks later, despite treatment with dexamethasone, the patient developed complete bilateral ophthalmoplegia and ptosis. Repeat MRI showed invasion of the clivus and cavernous sinuses, and a transsphenoidal pituitary biopsy was undertaken., Results: The preliminary histopathology was consistent with bland pituitary apoplexy, but subsequent examination of an incidentally biopsied nasal polyp revealed endovascular malignant lymphoid cells that, on further scrutiny, were also present in the pituitary tissue. The diagnosis of IVL was confirmed, and the patient had an excellent clinical and radiological response to cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab (CHOP-R) chemotherapy., Conclusion: IVL may involve the pituitary gland, causing sellar mass effect, cavernous sinus infiltration, and pituitary ischemia, mimicking pituitary apoplexy with neuro-ophthalmic features. It can be effectively treated with CHOP-R chemotherapy.

Purpose: To present a case of Haemangiopericytoma (HPC), a rare neopalsm which originates from the vascular pericytes. HPC occurs most commonly in adults. Only 5-10% of cases occur in children. Congenital orbital HPC is generally unknown in the field of ophthalmology., Material and Methods: A case of congenital, large exophthalmus is reported in a 1 day old male neonate. Imaging studies demonstrated a vascular, orbital mass involving skull base and cranial fossa., Results: The diagnosis of HPC was established after histological exmination. Lesion did not qualify to surgical resection. The child was treated with chemotherapy for 10 months and a great regression of tumor was noted. There was no tumor recurrence during 4 years of a follow up., Conclusions: Chemotherapy may have a significant role in the treatment of infants with nonoperative malignant hemangiopericytoma.

Background: Hemangiopericytomas and malignant solitary fibrous tumors (HPC/SFT) are rare, closely related sarcomas with unpredictable behavior that respond infrequently to chemotherapy. An optimal systemic treatment strategy for advanced HPC/SFT has not yet been identified., Methods: We retrospectively analyzed the records of 14 patients with histopathologically confirmed HPC/SFT who were treated at The University of Texas MD Anderson Cancer Center between May 2005 and June 2007. All patients were treated with temozolomide 150 mg/m(2) orally on days 1-7 and days 15-21 and bevacizumab 5 mg/kg intravenously on days 8 and 22, repeated at 28-day intervals. Computed tomography assessment of tumor size and density (Choi criteria) was used to determine the best response to therapy. The Kaplan-Meier method was used to estimate progression-free survival., Results: The median follow-up period was 34 months. Eleven patients (79%) achieved a Choi partial response, with a median time to response of 2.5 months. Two patients (14%) had stable disease as the best response, and 1 patient (7%) had Choi progressive disease as the best response. The estimated median progression-free survival was 9.7 months, with a 6-month progression-free rate of 78.6%. The most frequently observed toxic effect was myelosuppression., Conclusion: Combination therapy with temozolomide and bevacizumab is a generally well-tolerated and clinically beneficial regimen for HPC/SFT patients. Additional investigation in a controlled, prospective trial is warranted., (Copyright © 2011 American Cancer Society.)

The objective of this phase II study was to evaluate the efficacy and safety of subcutaneous octreotide therapy for the treatment of recurrent meningioma and meningeal hemangiopericytoma. Octreotide is an agonist of somatostatin receptors, which are frequently expressed in meningioma, and reports have suggested that treatment with somatostatin agonists may lead to objective response in meningioma. Patients with recurrent/progressive meningioma or meningeal hemangiopericytoma were eligible for enrollment; those with atypical/anaplastic meningioma or hemangiopericytoma must have experienced disease progression despite radiotherapy or have had a contraindication to radiation. Patients received subcutaneous octreotide with a goal dose of 500 μg 3 times per day, as tolerated. Imaging was performed every 3 months during therapy. The primary outcome measure was radiographic response rate. Eleven patients with meningioma and 1 with meningeal hemangiopericytoma were enrolled during the period 1992-1998. Side effects included diarrhea (grade 1 in 4 patients and grade 2 in 2), nausea or anorexia (grade 1 in 4 patients), and transaminitis (grade 1 in 1 patient). One patient developed extra hepatic cholangiocarcinoma, which was likely unrelated to octreotide therapy. No radiographic responses were observed. Eleven of the 12 patients experienced progression, with a median time to progression of 17 weeks. Two patients experienced long progression-free intervals (30 months and ≥18 years). Eleven patients have died. Median duration of survival was 2.7 years. Immunohistochemical staining of somatostatin receptor Sstr2a expression in a subset of patients did not reveal a correlation between level of expression and length of progression-free survival. Octreotide was well-tolerated but failed to produce objective tumor response, although 2 patients experienced prolonged stability of previously progressive tumors.

Object: Hemangiopericytomas are rare tumors that behave aggressively with a high rate of local recurrence and distant metastases. With the aim of determining the outcome and response to various treatment modalities, a series of 39 patients who underwent microsurgical resection for primary meningeal hemangiopericytoma over a 24-year period is presented., Methods: Patients with hemangiopericytoma were identified from histopathology records and their medical records were analyzed retrospectively by 2 independent reviewers to collect data on surgical treatment, adjuvant therapy, postoperative course, local or distant recurrence, and follow-up., Results: Of the 39 patients, 19 were male and 20 were female. Mean patient age was 44.1 years. Thirty-four tumors were intracranial and 5 were spinal. The mean follow-up period was 123 months. Twenty-eight patients developed local recurrence. The recurrence rate at 1, 5, and 15 years was 3.5%, 46%, and 92%, respectively. Extraneural metastasis occurred in 8 patients (26%) at an average of 123 months after initial surgery. Recurrences and metastases were treated by surgical excision, external beam radiation therapy (EBRT), chemotherapy, and/or stereotactic radiosurgery. Adjuvant EBRT following initial surgery was found to extend the disease-free interval from 154 months to 254 months, although it did not prevent the development of metastasis. In those patients with EBRT and complete resection, the mean recurrence-free interval was found to be 126.3 months longer (p = 0.04) and overall survival 126 months longer than without EBRT. Furthermore, adjusting for resection, patients undergoing EBRT had 0.33 times increased risk of recurrence compared with those who did not (p = 0.03). A majority of patients remained able to live independently despite revision surgery for recurrence., Conclusions: The mean follow-up of this patient series represents the longest follow-up duration published to date and demonstrates extended survival in a significant number of patients with hemangiopericytoma. Gross-total resection followed by adjuvant EBRT provides patients with the highest probability of an increased recurrence-free interval and overall survival. Prolonged survival justifies long-term follow-up and aggressive treatment of initial, recurrent, and metastatic disease.

Infantile haemangiopericytoma of the CNS is a rare entity. We report the first case of a congenital haemangiopericytoma successfully treated by preoperative chemotherapy. The patient presented shortly after birth with the diagnosis of a haemangiopericytoma. As neurosurgery was too risky due to size, location and age of the patient an anthracycline-based chemotherapeutic regimen was applied and resulted in a significant decrease in tumour size, making a postchemotherapy complete surgical resection possible. Chemotherapy may benefit patients with congenital haemangiopericytoma especially if the tumour cannot initially be treated by complete neurosurgical resection.

Background: Auriculotemporal nerve syndrome is characterised by recurrent episodes of facial gustatory flushing and/or sweating along the cutaneous distribution of the auriculotemporal nerve. The condition is rare in children and is normally a sequel of perinatal birth trauma. We report a case of a sixteen-month-old boy referred by paediatric oncology with recurrent, unilateral facial flushing of the left cheek which had been present for 2 months. The flushing only occurred during mastication. The patient had also received treatment for a rare vascular tumour, congenital haemangiopericytoma, of the left cheek and parotid region. The possible association between auriculotemporal nerve syndrome and congenital haemangiopericytoma is discussed. Knowledge of the presentation, aetiology and management of Auriculotemporal Nerve Syndrome can provide much needed reassurance to those suffering with this condition.

Meningeal hemangiopericytoma is an uncommon and aggressive malignancy that, in contrast to meningiomas, shows a high propensity for local recurrence and the development of late extraneural metastases. The results of chemotherapy in advanced hemangiopericytoma have been disappointing, and they have been particularly poor in cases located in the meninges. We report a case of a heavily pretreated metastatic meningeal hemangiopericytoma in which fourth-line chemotherapy with trabectedin, a marine-derived antineoplastic agent effective in treating advanced soft tissue sarcomas, resulted in clinical benefit.

Hemangiopericytoma (HPC) is a rare vascular tumor arising from contractile cells around blood vessels, with the potential for malignant degeneration. Up to 10% of HPC occurs in children. Standard therapy for this tumor is surgical excision. We report the case of a 6-month-old infant with giant HPC involving the hand. Chemotherapy resulted in a decrease in tumor size, allowing for salvage of most of the hand and fingers. Preoperative chemotherapy should be considered in the care of HPC tumors involving the upper extremity in children., (Copyright 2010. Published by Elsevier Inc.)

Infantile fibrosarcoma represents less than 1% of all childhood cancers, but it is the most common soft-tissue sarcoma in those under 1 year of age. We report an infant with congenital infantile fibrosarcoma diagnosed as hemangiopericytoma. He was treated with chemotherapy and extremity-sparing surgery. Amputation was avoided.

Purpose: In previous studies, razoxane and vindesine together with radiotherapy was proved to be effective in soft tissue sarcomas (STS). Because razoxane leads to a redifferentiation of pathological tumor blood vessels, it was of particular interest to study the influence of this drug combination in vascular soft tissue sarcomas., Methods and Materials: This open multicenter Phase II study was performed by the Austrian Society of Radiooncology. Among 13 evaluable patients (10 angiosarcomas and 3 hemangio-pericytomas), 9 had unresectable measurable disease, 3 showed microscopic residuals, and 1 had a resection with clear margins. They received a basic treatment with razoxane and vindesine supported by radiation therapy. Outcome measures were objective response rates, survival time, and the incidence of distant metastases., Results: In nine patients with measurable vascular soft tissue sarcomas (eight angiosarcomas and one hemangiopericytoma), 6 complete remissions, 2 partial remissions, and 1 minor remission were achieved, corresponding to a major response rate of 89%. A maintenance therapy with razoxane and vindesine of 1 year or longer led to a suppression of distant metastases. The median survival time from the start of the treatment is 23+ months (range, 3-120+) for 12 patients with macroscopic and microscopic residual disease. The progression-free survival at 6 months was 75%. The combined treatment was associated with a low general toxicity, but attention must be given to increased normal tissue reactions., Conclusions: This trimodal treatment leads to excellent response rates, and it suppresses distant metastases when given as maintenance therapy.

Purpose: To evaluate adjuvant stereotactic radiosurgery (SRS) in the management of recurrent or residual intracranial hemangiopericytomas (HPCs), we assessed tumor control, survival, and complications in patients who had undergone gamma knife SRS as part of multimodal therapy., Methods and Materials: We retrospectively reviewed the records of consecutive 20 HPC patients who had undergone SRS for 29 tumors. The median patient age was 51.5 years (range, 8.9-80.2). All patients had undergone previous surgical resection of their tumors. In addition, 12 patients underwent fractionated radiotherapy before SRS. Of the 20 patients, 16 patients had low-grade HPCs (20 tumors) and 4 had high-grade anaplastic HPCs (9 tumors). The median radiosurgery target volume was 4.5 cm(3) (range, 0.07-34.3), and the median marginal dose was 15.0 Gy (range, 10-20)., Results: At an average of 48.2 months (range, 7.2-124.1), 5 patients had died of metastases and 3 patients had died of disease progression. The overall survival after radiosurgery was 100%, 85.9%, and 13.8% at 1, 5, and 10 years, respectively. The follow-up imaging studies demonstrated tumor control in 21 (72.4%) of 29 tumors. The progression-free survival rate after SRS at 1, 3, and 5 years was 89.1% for low-grade HPCs and 88.9%, 66.7%, and 0%, respectively, for high-grade HPCs. The factors associated with improved progression-free survival included lower grade and higher marginal dose. Eight patients had intracranial or extracranial metastasis after the initial diagnosis, which correlated with the shorter survival., Conclusion: The results of our study have shown that adjuvant SRS after tumor resection is an important management option for patients with residual or recurrent HPCs and is particularly effective for less-aggressive tumors.

Objective: Hemangiopericytoma (HPC) is an uncommon primary brain tumor with an almost invariable tendency to recur and metastasize. We undertook a retrospectively collected case series of recurrent intracranial HPCs treated with salvage chemotherapy with the primary objective of evaluating progression-free survival., Methods: Fifteen patients, ages 26 to 62 years, with recurrent HPC and who were previously treated with surgery and involved-field radiotherapy were studied. Eight (53%) of these patients had undergone re-resection before study entry. Ten patients (67%) were treated with stereotactic radiotherapy. Chemotherapy was administered to 5 patients at first relapse, 8 at second relapse, and 2 at third relapse (none of these patients were candidates for reoperation or stereotactic radiotherapy). Eight patients developed disseminated disease, all with multifocal intracranial disease (5 with cerebrospinal fluid dissemination, 4 with extraneural metastases). All patients were initially treated with cyclophosphamide, doxorubicin, and vincristine (CAV). After disease progression despite the administration of CAV in clinically appropriate patients, alpha-interferon (alpha-IFN) (9 patients) was administered. Five patients were treated with ifosfamide, cisplatin, and etoposide after they failed to respond to alpha-IFN. Neurological and neuroradiographic evaluations were performed every 8 weeks., Results: All patients were evaluable. A median of 4 cycles of CAV; 8 cycles of alpha-IFN; and 2 cycles of ifosfamide, cisplatin, and etoposide were administered. Chemotherapy-related toxicity included alopecia (100%), anemia (40%), thrombocytopenia (27%), and neutropenia (40%). Best response included 6 patients (40%) with a neuroradiographic partial response (2 with CAV, 4 with alpha-IFN), 14 (93%) had stable disease (9 with CAV, 5 with alpha-IFN), and 9 (60%) had progressive disease (4 with CAV, 5 with ifosfamide, cisplatin, and etoposide). The median overall survival was 14 months (range, 2-24 mo)., Conclusion: Salvage chemotherapy demonstrated modest efficacy with acceptable toxicity in this cohort of adult patients with recurrent surgery- and radiotherapy-refractory intracranial HPC.

Hemangiopericytoma (HP) is a rare mesenchymal tumor associated with the capillary wall. We describe a 62-year-old man with a 20-year history of recurrent metastatic HP, in which a favorable clinical benefit was observed with sunitinib, an oral VEGFR, and PDGFR kinase inhibitor. Disease progression was evident during the 3 months before starting sunitinib therapy. Radiologic evaluation indicated that metastatic lesions in the lungs and pelvis remained stable during the 6 months on sunitinib therapy. Histologic analysis of the tumor 3 weeks after active sunitinib therapy did not reveal tumor necrosis, but recanalized thrombi within tumor blood vessels indicated previous tumor insult. Additional studies are warranted to confirm the possible effects of sunitinib in controlling widespread metastatic HP.

Hemangiopericytoma is a rare vascular tumor, of which 5% to 10% occur in the pediatric population. Although usually benign in infants, local recurrence, metastasis, and deaths have been reported. Clonal chromosomal rearrangements have been described, most involving the long arm of chromosome 12. We report a case of a 6-month-old boy with an hemangiopericytoma of the left forearm initially incorrectly diagnosed as hemangioma. He was treated successfully with chemotherapy alone using vincristine, doxorubicin, actinomycin-D, and cyclophosphamide. Although cytogenetic analysis was not performed on his biopsy, it was later discovered that a prenatal karyotype had shown 46,XY,inv(12)(q15q24.1).

We describe here a patient with a recurrent hemangiopericytoma of the superior mediastinum 23 years after an initial complete resection. In the current biopsy specimen, the tumor cells were much more anaplastic than those seen 23 years ago. Although the patient was treated with chemotherapy, which consisted of ifosfamide and epirubicin, the tumor was unresponsive and he died 6 months later from disease progression. Careful long-term follow-up is mandatory for patients with hemangiopericytomas because recurrence with greater malignancy can develop following an extended disease-free interval.

Hemangiopericytoma (HPC) is a soft-tissue neoplasm composed of proliferating capillary pericytes. It has variable and unpredictable malignancy and most commonly occurs in the fifth or sixth decade of life. Diagnosis is based on the histological aspect. HPC is exceedingly rare in childhood. In both adults and children, curative surgery is the most important predictor of survival. The place of chemotherapy in the treatment of HPC is not well established. We describe a case of adult-type metastatic HPC of the thigh in a 13-year-old boy. The response to neoadjuvant chemotherapy was excellent, and local control of this initially unresectable tumor was achieved without radiation therapy or mutilating surgery. The child is alive and well and has had 8 years of follow-up after treatment.

Haemangiopericytoma is an uncommon soft tissue sarcoma of vascular origin. It occurs more frequently in the extremities than elsewhere in the body although it can arise in any organ. Wide surgical excision is the mainstay of treatment. However, adjuvant radiotherapy and chemotherapy are desirable because the malignant nature of this tumour is frequently unpredictable. Adjuvant therapy is recommended for metastases, recurrence and incomplete resection. Long term follow up is essential in all cases as recurrence can occur several years after treatment. Where little or no experience with managing this tumor exists, it is important to be aware of its clinical behaviour and the treatment options, hence this case reports.

Unlabelled: Malignant vascular tumours represent a rare group of neoplasms, usually treated according to protocols for soft tissue sarcomas., The Aim of the Study: To assess the clinical characteristics, disease course and outcome in Polish children with malignant vascular neoplasms., Material and Methods: The Polish Paediatric Rare Tumours Study retrospectively analysed multicentre data on 32 children with vascular tumours, registered between 1992 and 2002. On the basis of the histological type of the neoplasm these patients have been divided into three groups: group I -- 10 patients with angiosarcoma (ASA), group II -- 7 children with haemangioendothelioma (HE) and group III- 15 patients with haemangiopericytoma (HP), of both infantile (7 children) and adult-types (8 patients)., Results: Group I: 5 patients presented with local, 3 with systemic and 2 with regional disease. Primary complete resection (PRC) was performed in 7 patients, not resulting in local control in any of them. Radiotherapy (RTX) was administered in 5 patients, chemotherapy (CHT) in all. 7 patients relapsed, 3 never entered clinical remission. 9 of the 10 patients of group I, died of disease progression. Group II: PRC was performed in 5 patients and led to local control in 2. Adjuvant RTX was used in 2 and CHT in 4 patients. 2 children relapsed and died of the disease. Infantile HP: PRC was performed in 3 children, remaining 4 patients received adjuvant CHT All children entered complete remission and are disease-free. Adult-type HP: PRC was performed in 5 patients, resulting in local control in 4. Five children were given adjuvant RTX and six CHT Three patients relapsed and died of disease progression., Conclusions: The effectiveness of primary complete resection in all groups was doubtful. The high rate of metastatic relapses suggests that the currently given systemic therapy is not satisfactory. The only tumour with excellent prognosis was infantile type HP (all patients are alive and free of disease). Adequate treatment for children with angiosarcoma remains still unknown -- 9 of 10 patients died of disease progression. Prognosis in patients with haemangioendothelioma is intermediate, however the role of immunotherapy should be further investigated.

Hemangiopericytoma is a rare tumor with uncommon location in the central nervous system. It has only recently been included (WHO classification 1993) in a specific group of CNS tumors and subsequently (WHO classification 1997 and 2000) as a group by itself, while before it was confused with meningeal tumors. We report on a case of a 48-year-old woman affected by this tumor. The neoplasm was located in the posterior fossa. The patient underwent primary surgery in 1990, not followed by any adjuvant therapy because of the histopathological diagnosis of meningioma. After being free from disease for eight years she developed a local recurrence in 1998. Subtotal excision of the tumor, which was finally identified as a hemangiopericytoma, was carried out, followed by adjuvant radiotherapy (64 Gy). After six months multiple metastases were found in the liver and right kidney. A radical metastasectomy was performed, followed by systemic chemotherapy. One year later (2001) the tumor recurred again intracranially and a metastases was detected in the right breast, so the patient again underwent cranial irradiation (40 Gy) and second-line chemotherapy. She died in September 2002, 12 years after the diagnosis. We may conclude that, despite the tumor's natural tendency to recur several times and the ability of intracranial hemangiopericytoma to spread outside the CNS, it is possible to ensure a long survival time.

History and Admission Findings: A seventy-seven year-old woman with an unclear tumor of the liver suffered from recurrent hypoglycemia and was therefore admitted to our hospital. As diabetes mellitus, hyperinsulinism and reactive forms of hypoglycemia could be excluded, the presumptive diagnosis was non-islet-cell tumor hypoglycemia (NICTH)., Investigations: Postprandial glucose levels were normal. Fasting glucose levels were 30 - 50 mg/dl. Plasma insulin-like growth factor (IGF)-I was below the normal range, IGF-II was not elevated, although 34 % of plasma IGF-II was present as "big"-IGF-II. IGF-binding protein (IGFBP)-2 was extremely elevated, whereas IGFBP-3 was within the normal range. Histological examinations of the tumor revealed a hemangiopericytoma of the liver., Treatment and Course: : After a 2-month treatment with steroids and an experimental antiangiogenetic therapy, the glucose metabolism became stable. The tumour did not grow. Simultaneously, plasma IGF-II and "big"-IGF-II remained constant and plasma IGF-I level improved slightly. IGFBP-2, which is presumable produced by the tumor, increased, IGFBP-3 fell below the normal range., Conclusion: NICTH is a rare but important differential diagnosis of recurrent hypoglycemia. The tumor derived IGF-II has a higher than normal molecular weight ("big"-IGF-II) and shows different interactions with binding proteins, thus resulting in an increased bioavailability. An increased glucose uptake in different tissues as well as inhibition of hepatic gluconeogenesis and lipolysis lead to severe hypoglycemia. If surgical therapy of the tumor is not possible, symptomatic treatment with steroids may represent an effective alternative to control severe hypoglycemia.

Treating rare, primary cardiac soft-tissue sarcomas (C-STS) with heart transplantation (HTx) is controversial. Conventional tumor resection only partially alleviates the disease, and patients die of local recurrence of the tumor or of distant metastases. Heart transplantation offers the opportunity to eradicate the primary malignancy completely. In our experience of 4 patients with C-STS indicates, HTx followed by post-operative chemotherapy does not affect the long-term outcome. However, pre-operative chemotherapy can regress tumors in chemosensitive C-STS and potentially eradicate early micrometastases. Consecutive HTx for responders may then offer a chance of long-term survival.

The authors report on a 64-year-old woman with a huge recurrent skull base hemangiopericytoma, in whom they encountered severe difficulty in attaining intraoperative hemostasis. Standard surgical hemostatic methods and the administration of fresh-frozen plasma and prothrombin complex concentrates failed to stop diffuse bleeding from an inoperable tumor remnant. At a critical point during the operation, the intravenous administration of recombinant activated factor VII, combined with mechanical compression, finally led to satisfactory hemostasis. The rationale for using recombinant activated factor VII in situations of uncontrolled bleeding during neurosurgical procedures is discussed, along with the literature in which the use of recombinant activated factor VII as a maneuver of last resort is reported for hemostasis in other surgical fields.

Background: Hemangiopericytoma (HPC) is very uncommon in childhood and comprises two different clinical entities, the adult type and the infantile type, occurring in the first year of age. We report on a series of 27 pediatric patients treated from 1978 to 1999 by the Italian and German Soft Tissue Sarcoma Cooperative Group., Methods: Seven patients had infantile HPC; complete resection was achieved in the tumors of five patients and chemotherapy was given to four patients. Twenty children had adult type HPC; nine received complete tumor resection (four patients at diagnosis and five at delayed surgery). Post-operative radiotherapy was administered to 15 patients, chemotherapy to 19., Results: Six of seven patients with infantile HPC were alive in first remission; one patient died of disease. Chemotherapy achieved an objective response in four of four patients. Among the adult type HPC cases, 5-year event free survival was 64% (median follow-up 125 months); 12 patients were alive in first remission, eight patients relapsed and died of disease. Seven of 10 evaluable patients showed good response to chemotherapy. Statistically significant differences in outcome were observed in relation to Intergroup Rhabdomyosarcoma Study grouping, size, local invasiveness, and gender., Conclusions: Infantile HPC is a unique entity probably related to infantile myofibroblastic lesions and characterized by a high response to chemotherapy, which is required in case of unresectable, life-threatening tumors. In children over 1 year of age, HPC behaves like its adult counterpart; complete surgical resection remains the mainstay of treatment, but chemotherapy and radiotherapy seem effective and are recommended in all patients with incomplete tumor resection and/or locally invasive, large tumors., (Copyright 2001 American Cancer Society.)

Hemangiopericytoma (HPC) is a rare mesenchymal tumor generally occurring in adults and originating from the pericytes. The tumor more commonly affects the soft tissues of the extremities, the pelvis, and the retroperitoneum. We describe the computed tomographic and magnetic resonance appearance of a patient affected by HPC of the duodenum.

A 51-year-old man was admitted because of complaints of cough and bloody sputa. A chest CT scan revealed a giant mass lesion in the right middle and lower lobes of the lung and mediastinal lymphadenopathy. Bronchoscopic findings showed a tumor which almost completely obstructed the intermediate bronchus. Histopathological examination of a biopsy specimen demonstrated malignant hemangiopericytoma. Two courses of chemotherapy that combined cisplatin, ifosfamide and gemcitabine were performed every 3 weeks. Both primary lesion and mediastinal lymph node metastases showed marked reduction and toxicity was manageable.

Background and Objectives: Canine hemangiopericytomas are a commonly occurring neoplasm with a clinical course of recurrence after surgical removal. This study sought to evaluate Photochlor (HPPH) photodynamic therapy (HPPH-PDT) as an adjuvant therapy to prevent recurrence of tumor after surgical removal., Study Design/materials and Methods: Sixteen dogs with naturally occurring hemangiopericytomas were treated with surgical removal of the tumor followed by PDT using Photochlor as the photosensitizer. Photochlor was injected intravenously at a dose of 0.3 mg/kg. Forty-eight hours later the treatment consisted of surgical removal of the tumor followed by HPPH-PDT., Results: Nine dogs (56%) had recurrence of tumor from 2 to 29 (median 9) months after treatment. These results are comparable or not as good as other forms of therapy., Conclusions: Photochlor photodynamic therapy applied after surgery appears to have no advantage over other forms of therapy in regards to preventing recurrence. Delayed wound healing and infections are problematic and make HPPH-PDT an undesirable addition to surgery for the treatment of this tumor type., (Copyright 2001 Wiley-Liss, Inc.)

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.

We report a case of successful liver transplantation in a young man with metastatic hemangiopericytoma confined to the liver. He had a primary retroperitoneal hemangiopericytoma removed in 1982 and was diagnosed as having liver metastases 8 years later. Subsequently, he developed hypoglycemia, which did not respond to chemotherapy. Hepatectomy and transplantation resolved his hypoglycemic attacks. He remained well and symptom free for 4 years. Liver transplantation can provide effective palliation and should be considered in carefully selected patients with localized metastatic cancer.