Hemangiopericytoma: Incidence, Treatment, and Prognosis Analysis Based on SEER Database.

BACKGROUND: Hemangiopericytomas are rare tumors derived from pericytes surrounding the blood vessels. The clinicopathological characteristics and prognosis of hemangiopericytoma patients remain mostly unknown. In this retrospective cohort study, we assessed the clinicopathological characteristics of hemangiopericytoma patients, as well as the clinical usefulness of different treatment modalities. Material and Methods. We collected the clinicopathological data (between 1975 and 2016) of hemangiopericytoma and hemangioendothelioma patients from the Surveillance, Epidemiology, and End Results (SEER) database. Incidence, treatment, and patient prognosis were assessed. RESULTS: Data from 1474 patients were analyzed in our study cohort (hemangiopericytoma: n = 1243; hemangioendothelioma: n = 231). The incidence of hemangiopericytoma in 2016 was 0.060 per 100,000 individuals. The overall survival (OS) and cancer-specific survival (CSS) did not differ between patients with hemangioendothelioma and those with hemangiopericytoma (P = 0.721, P = 0.544). The tumor grade had no effect on the OS of hemangiopericytoma patients. Multivariate analysis revealed the clinical usefulness of surgery in hemangiopericytoma patients (HR = 0.15, 95% confidence interval: 0.05-0.41, P < 0.001). In contrast, radiotherapy did not improve OS (P = 0.497) or CSS (P = 0.584), and chemotherapy worsened patient survival (P < 0.001). Additionally, the combination of surgery and radiotherapy had a similar effect with surgery alone on hemangiopericytoma patient survival (OS: P = 0.900; CSS: P = 0.156). Surgery plus chemotherapy provided a worse clinical benefit than surgery alone (P < 0.001). CONCLUSIONS: Our findings suggested that hemangiopericytoma had a similar prognosis with hemangioendothelioma. Surgery was the only effective treatment that provided survival benefits in hemangiopericytoma patients, while the clinical usefulness of adjuvant chemotherapy or radiotherapy was limited.

Surveillance for metastatic hemangiopericytoma-solitary fibrous tumors-systematic literature review on incidence, predictors and diagnosis of extra-cranial disease.

BACKGROUND: Intracranial hemangiopericytomas (HPC) and solitary fibrous tumors (SFTs) (HPC-SFT) are rare vascular tumors that resemble meningioma on imaging and predominantly affect young adults. HPC-SFT have a high rate of local recurrence with well-known propensity for extracranial metastases. This provides clinical dilemmas frequently encountered in oncology: (i) How should these patients be monitored long term? (ii) Which primary tumors are more likely to metastasize? OBJECTIVES: This systematic review aims to identify the incidence, common locations and time to presentation of extra-cranial metastases of HPC-SFT. We will assess the effect of primary tumor location, treatment, grade, patient age, gender and effect of local recurrence on rates of extra-cranial metastasis and discuss the ideal techniques by which patients with intracranial HPC-SFT should be monitored for extra-cranial metastases. METHODS: Using PRISMA guidelines the authors searched Pubmed. Search terms included hemangiopericytoma, HPC, solitary fibrous tumor/ tumour, SFT, HPC-SFT, extra-cranial metastases, metastases, recurrence, monitoring, follow-up. Studies were identified up to 1st February 2018. Reference lists of identified articles were reviewed to detect other relevant citations. Data were extracted using a standard data collection form and results organized into (i) general study/patient characteristics, (ii) location of extra-cranial metastases, (iii) methods by which metastases were detected and followed up and (iv) characteristics of primary tumors. RESULTS: Seventy-one studies were identified. Mean recorded follow up ranged from 4 to 312 months. Mean age at diagnosis was 42.0 years. The overall rate of extra-cranial metastasis was 28% (n = 251/904). The minimum time to extracranial metastases was 3 months and the maximum time was 372 months. In the 71 studies identified, where site of extra-cranial metastasis was specified, there were 347 metastases in 213 patients. The most common sites for metastases were bone (location not specified) (19.6%) followed by lung and pleura (18.4%), liver (17.6%), and vertebrae (14.1%). Extra-cranial metastatic disease is typically diagnosed following symptomatic presentation. There is little documentation of methods used to monitor patients with extra-cranial HPC-SFT and no clear surveillance paradigm observed. Higher primary tumor grade (WHO Grade III) was associated with a 1.88 (p = 0.016) increased risk of extra-cranial metastasis. Location and treatment of primary tumor, local recurrence, patient age and gender were not. CONCLUSION: Patients with intracranial HPC-SFT require periodic, long term monitoring for extra-cranial metastases. Metastases occur in any age group and can occur early and late. They vary in location and are typically diagnosed following symptomatic presentation. There is no suggested imaging modality for surveillance. Higher grade primary tumors have a greater risk of metastasis. Regular clinical review is essential with early imaging for symptoms of recurrence/metastasis with imaging modality dependent on clinical concern. Quality evidence for an imaging surveillance protocol in this heterogeneous group of patients is lacking. A multicenter study on appropriate surveillance may be of benefit.

Solitary-fibrous tumor/hemangiopericytoma of the central nervous system: a population-based study.

The World Health Organization (WHO) classification of tumors of the central nervous system (CNS) was recently updated, restructuring solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) into one combined entity. This is the first population-based study to examine outcomes of SFT/HPC based on the new WHO guidelines. The Surveillance, Epidemiology, and End Results (SEER) database (1998-2013) was queried to examine age-adjusted incidence and prognostic factors associated with overall survival in 416 surgically resected cases. Age-adjusted incidence was calculated to be 3.77 per 10,000,000 and was rising. Median survival was 155 months, with 5- and 10-year survival rates of 78 and 61%, respectively. Younger age, Asian/Pacific Islander versus white race, benign histology, tumor location, gross-total resection (GTR), and GTR plus radiation (RT) versus subtotal resection were significantly associated with survival. In multivariable analysis, older age (HR = 1.038, p < 0.0001), infratentorial location (HR = 2.019, p = 0.038), GTR (HR = 0.313, p = 0.041), and GTR + RT (HR = 0.215, p = 0.008) were independent prognostic factors. In the HPC and borderline/malignant subgroups, GTR + RT was associated with significantly increased survival compared with GTR alone (HR = 0.537, p = 0.039 and HR = 0.525, p = 0.038). After eliminating patients that died within 3 months of diagnosis, GTR + RT was still associated with an incremental increase in survival (HR = 0.238, p = 0.031) over GTR alone (HR = 0.280, p = 0.054). GTR + RT may be optimal in the management CNS HPC and SFT/HPC tumors with borderline/malignant features. This study, in combination with existing literature, warrants further investigation of adjuvant radiation through a prospective clinical trial.

A population-based analysis of Head and Neck hemangiopericytoma.

OBJECTIVES/HYPOTHESIS: Hemangiopericytomas (HPC) are tumors that arise from pericytes. Hemangiopericytomas of the head and neck are rare and occur both extracranially and intracranially. This study analyzes the demographic, clinicopathologic, treatment modalities, and survival characteristics of extracranial head and neck hemangiopericytomas (HN-HPC) and compares them to HPCs at other body sites (Other-HPC). METHODS: The Surveillance, Epidemiology, and End Results (SEER) database (1973-2012) was queried for HN-HPC (121 cases) and Other-HPC (510 cases). Data were analyzed comparatively with respect to various demographic and clinicopathologic factors. Disease-specific survival (DSS) was analyzed using the Kaplan-Meier model. RESULTS: There was no significant difference in age at time of diagnosis between HN-HPC and Other-HPC. Head and neck HPC was most commonly located in the connective and soft tissue (18.4%), followed by the nasal cavity and paranasal sinuses (8.5%). Head and neck HPCs were smaller than Other-HPC (P < 0.0001) and more likely to be a lower histologic grade (P < 0.0097). The primary treatment modality for HN-HPC was surgery alone, used in 55.8% of cases. The 5-, 10-, and 20-year DSS for HN-HPC were 84.0%, 79.4%, and 69.4%, respectfully. Higher histologic grade and the presence of distant metastases were poor prognostic factors for HN-HPC. CONCLUSION: Head and neck HPCs are rare tumors. This study represents the largest series of HN-HPCs to date. Surgery alone is the primary treatment modality for HN-HPC, with a favorable prognosis. Adjuvant radiotherapy does not appear to confer a survival benefit for any body site. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:643-650, 2016.

Intracranial hemangiopericytoma and the role of radiation therapy: a population based analysis.

BACKGROUND: Intracranial hemangiopericytoma (HPC) is a rare malignancy for which treatment recommendations vary. OBJECTIVE: We sought to characterize outcomes of HPC patients treated with postoperative external beam radiotherapy (PORT). METHODS: A retrospective analysis was conducted using the Surveillance, Epidemiology and End Results (SEER) Program of the US National Cancer Institute. We identified patients with intracranial hemangiopericytoma who underwent surgery alone or PORT. RESULTS: We identified 88 patients with a diagnosis of HPC between 1982 and 2009 treated with surgery alone or PORT. The majority of patients were female (53%) and white (84%) with a median age of 50.5 years (range, 0-92 years). Gross total resection (GTR) was achieved in 55%, and PORT was delivered to 48% of the entire cohort. The median overall survival (OS) and cause-specific survival (CSS) were 111 months and 161 months, respectively. On univariate analysis, age older than 50 years correlated with poor OS (hazard ratio [HR]: 3.43; 95% confidence interval [CI]: 1.70-6.95; P = .001) and CSS (HR: 2.77; 95% CI: 1.18-6.48; P = .019). On multivariate analysis (MVA), age >50 years correlated with poor OS (HR: 3.69; 95% CI: 1.72-7.93; P = .001) and CSS (HR: 2.67; 95% CI: 1.08-6.59; P = .034). On MVA, GTR correlated with improved OS (HR: 0.28; 95% CI: 0.11-0.71; P = .007) and CSS (HR: 0.23; 95% CI: 0.07-0.76; P = .016). In addition, PORT correlated with improved OS (MVA HR: 0.02; 95% CI: 0.00-0.31; P = .005) and CSS (MVA HR: 0.02; 95% CI: 0.00-0.45; P = .015). Patients undergoing STR with PORT compared favorably with those undergoing GTR alone with respect to OS (HR: 0.43; 95% CI: 0.15-1.26; P = .13) and CSS (HR: 0.51; 95% CI: 0.15-1.78; P = .29). CONCLUSION: In intracranial HPC, both PORT and GTR independently correlate with improved OS and CSS.

Meningeal hemangiopericytomas and hemangiopericytoma/solitary fibrous tumors of extracranial soft tissues: a comparison.

The current World Health Organization (WHO) classification of central nervous system tumors lists meningeal hemangiopericytomas (HPC) and meningeal solitary fibrous tumors (SFT) as separate entities. On the contrary, SFT and HPC of soft tissues are regarded in the WHO soft tissue fascicle as features of the same entity. The clinical data, histology, and immunohistochemistry of 18 cases of meningeal HPC and 12 cases of peripheral soft tissue HPC-SFT were compared. Both intracranial and soft tissue lesions had significant similarities that included staghorn vasculature, necrotic areas, cytologic atypia, and positivities for CD99, collagen IV, and reticulin. Nevertheless, intracranial tumors were more cellular than HPC-SFT of soft tissues and had fewer collagen bands. Meningeal HPC in addition had more mitoses, higher Ki67 index, stained less intensely for CD34 and B-cell lymphoma 2 (BCL2) than HPC-SFT of soft tissues. Meningeal HPCs recurred in 13 out of 14 cases (92.9%). One of the patients died in the postoperative period for a recurrent lesion 5 years after the diagnosis, and another patient developed an extracranial metastasis 13 years after surgery. None of the six cases of HPC-SFT of soft tissues available for follow-up recurred. Both meningeal and soft tissue tumors appear to represent different features of the same entity. A more aggressive phenotype of the tumor together with incomplete surgical resection of intracranial lesions might explain the noticeable clinical difference between HPC of the meninges and HPC-SFT of soft tissues.

Incidence and familial risks in pituitary adenoma and associated tumors.

Reliable data on familial risks are important for clinical counseling and cancer genetics. We wanted to study incidence trends and familial risks for pituitary adenomas and associated tumors through parental and sibling probands, using the nation-wide Swedish Family-Cancer Database on 10.5 million individuals, containing families with parents and offspring. Cancer data were retrieved from the Swedish Cancer Registry from years 1958 to 2002, including 3239 pituitary tumor patients. Familial risk for offspring was defined through standardized incidence ratio (SIR), adjusted for many variables. The incidence of pituitary adenoma has increased moderately from 1958 to the 1990s and declined thereafter. There were only three offspring-parent pairs with a concordant pituitary tumor, the SIR was not significant. Parental skin cancer (SIR 1.60) and leukemia (1.90, chronic lymphatic leukemia 2.59) were associated with offspring pituitary adenoma diagnosed at any age up to 70 years. There was a strong association of pituitary adenomas with nervous system hemangiopericytomas, SIR 182. The only significant association among siblings was between pituitary tumors and breast cancer (1.46). The risk of pituitary adenoma was marginally increased in individuals whose siblings were diagnosed with colorectal cancer. The results suggest an association of pituitary adenomas with nervous system hemangiopericytomas and breast and colorectal cancers, in addition to some other tumor types. Whether these associations can be explained by the recently identified pituitary adenoma predisposing gene, AIP, remains to be established.

A clinicopathological study of eyelid malignancies from central India.

BACKGROUND: Eyelid malignancies are completely treatable if detected early. The treatment depends on the invasiveness of the cancer which in turn depends on the type of malignancy. AIM: The aim of the study was to characterize the distribution of the types of eyelid malignancies in central India. SETTINGS AND DESIGN: The study was conducted in the Department of Plastic and Maxillofacial Surgery at a tertiary care hospital. MATERIALS AND METHODS: We report a series of 27 cases of eyelid malignancies. In the same case series, we also include a case of malignant hemangiopericytoma which is an extremely rare form of eyelid malignancy worldwide. STATISTICAL ANALYSIS: Depending on the underlying statistical distribution, either analysis of variance (ANOVA) or the Kruskal-Wallis (K-W) test was used to assess the differential distribution of these variables across the types of eyelid malignancies observed in this study. RESULTS: We observed that sebaceous cell carcinoma (approximately 37%) was almost as prevalent as basal cell carcinoma (approximately 44%) in the study subjects and had an earlier age of occurrence and a more rapid clinical course. CONCLUSIONS: Sebaceous cell carcinoma of the eyelid is almost as common as basal cell carcinoma in a large tertiary care centre in central India.

Maxillary haemangiopericytoma: a case report.

BACKGROUND: Haemangiopericytoma is a very rare slow-growing vascular tumour with a variable malignant potential, constituting less than 1% of all neoplasms. It may arise from any blood vessel and in any organ of the body. Primary haemangiopericytoma of bone is even rarer, constituting about 0.1% of bone tumours. The tumour is extremely rare in Africans and particularly in the head and neck region. STUDY DESIGN: We describe the case of a 66-year old Nigerian with haemangiopericytoma of the maxilla, who presented with a recurrent but painless jaw mass. RESULTS: Surgical resection of this tumour is potentially bedevilled with the risk of torrential haemorrhage and high rate of recurrence. This risk may be substantially reduced by wide surgical resection with a careful microscopical examination of the resection margins and the institution of adjuvant radiotherapy in incompletely resected tumours. Chemotherapy has no known role in the management of haemangiopericytoma. Postoperative radiation therapy appears to be effective against tumour recurrence. CONCLUSION: Even then, long-term follow-up is essential in all cases. To our knowledge, this is the first report of this entity in an African.

Hemangiopericitoma de cabeza y cuello

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Haemangiopericytoma of the nasal cavity.

Haemangiopericytomas (HPC) are rare vascular tumours originating from a pericytes, a term coined by Zimmermann to refer to the main location of this cell line in the pericapillary connective tissue. HPC may arise in any part of the body. We report a 29-year-old man with a histologically proven nasal haemangiopericytoma-like tumour. The lesion was embolised through the ophthalmic artery before it was removed surgically. The main symptoms of nasal HPC are epistaxis and obstruction of the nose. Malignant and benign clinical courses have been described. Local recurrence and metastases may be observed years after initial diagnosis.

Primary malignant hemangiopericytoma of the breast: report of a case.

A 70-year-old woman presented with a huge tumor in her left breast, which had rapidly increased in size causing cutaneous erosion. She underwent a simple mastectomy, at which time the tumor was found to be an encapsulated multilocular cystic lesion containing dark fluid. Histological examination disclosed the characteristic features of malignant hemangiopericytoma, including the perivascular lamellar growth of atypical mesenchymal cells with vimentin-positive cytoplasm. Multiple irregular vascular luminal formation was also conspicuous. The characteristics of this lesion are discussed together with a review of the previous literature on this unusual neoplasm.

Hemangiopericytoma of the head and neck.

OBJECTIVE/HYPOTHESIS: Hemangiopericytomas are uncommon neoplasms of vascular origin that may arise in the head and neck. Their rare occurrence and variable malignant potential have limited attempts to characterize their clinical behavior. This study reviews the experience in treating hemangiopericytomas of the head and neck at a single institution. STUDY DESIGN: Retrospective. METHODS: The records of 12 patients with hemangiopericytomas of the head and neck presenting between 1979 and 1995 were reviewed. Site of origin included the neck (4), oral cavity (3), parotid (2), orbit (1), maxillary sinus (1) and mandible (1). Five patients had lesions characterized as high or intermediate grade histologically, and six had lesions characterized as low grade. RESULTS: Nine patients were treated with curative intent; three presented either with pulmonary metastasis (2) or unresectable primaries (1) and were treated with radiation therapy and/or palliative Adriamycin-based chemotherapy. Patients treated with curative intent underwent a variety of surgical resections dictated by tumor location and size. Four patients received postoperative radiation therapy to a median dose of 60 Gy, for positive surgical margins (2), high-grade histology (1) or a recurrent lesion (1). Five-year overall survival in patients treated surgically was 87.5%. A single mortality occurred in a patient with a recurrent high-grade lesion who failed at local, regional, and distant sites. Median follow-up of survivors was 73 months. CONCLUSION: The clinical behavior of hemangiopericytomas appears to be related to their histological grade. Aggressive local therapy including surgery and radiation therapy appears to be effective in providing tumor control.

Long-term survival after repetitive surgery for malignant hemangiopericytoma of the lung with subsequent systemic metastases: case report and review of the literature.

Malignant hemangiopericytoma represent less than 1% of all vascular and about 5% of all sarcomatous tumors. We are reporting the uncommon clinical case of primary pulmonary localization, of which approximately 100 cases have been published in the literature. A 54-year-old male underwent left upper lobectomy seven years ago for a tumor which proved to be a malignant hemangiopericytoma. Six months later a metastasis in the abdominal wall in the epigastrium appeared and was completely excised without further treatment. During a follow-up of 16 months another metastasis developed in the mesenterium causing ileus and leading to laparotomy with resection of 120 cm small bowel. After this procedure followed by two cycles of chemotherapy the patient remained free of disease. Details of the clinical course and a review of the literature are presented.

Hemangiopericytoma of the masticator space.

Hemangiopericytomas are rare tumors of the head and neck. The benign presentation of this tumor belies its high local recurrence rate, local aggressiveness, and malignant potential. In view of these characteristics, workup to provide a diagnosis preoperatively is of significant importance. Diagnostic imaging is helpful in planning operative management, detecting metastases, and narrowing the list of differential diagnoses. However, because of the variety and lack of specificity of radiologic findings, it is generally difficult to provide a diagnosis. A history of a painless, slowly growing, otherwise asymptomatic mass, together with the radiologic findings of a vascular neoplasm, should enhance the suspicion of an HPC as a diagnosis. Hemangiopericytoma should be included in the differential diagnosis of any vascular soft tissue lesion presenting in the head and neck, and plans for surgical intervention should include the possibility of aggressive, wide local resection in order to adequately treat such a lesion should it be encountered.

Resection of primary pulmonary hemangiopericytoma: a report of two cases.

We report herein the cases of two patients who underwent resection of a primary pulmonary hemangiopericytoma. The first patient was a 58-year-old man found to have a mass-like shadow of about 5 cm in diameter in the right hilum by a routine chest X-ray. Subsequent magnetic resonance imaging (MRI) showed a probable mediastinal tumor, and surgery was performed. Postoperative pathological examinations confirmed the diagnosis of pulmonary hemangiopericytoma. The second patient was a 21-year-old woman found to have a mass-like shadow of about 2 cm in diameter in the left middle lung field. As a preoperative diagnosis could not be made, exploratory surgery was performed and the left S6 segment was excised. A definitive diagnosis of pulmonary hemangiopericytoma was established postoperatively by pathological examination. Primary pulmonary hemangiopericytoma is an extremely rare type of tumor, with only 36 cases having been reported in the Japanese literature to date, including out 2 cases. A discussion following the case reports examines the sex, age, initial manifestations, sites, and methods of surgery employed in these 36 cases.

[Computed tomography in malignant primary soft tissue tumors]. / Computertomographie bei malignen primären Weichteiltumoren.

The results of CT examinations in 36 patients suffering from histologically confirmed malignant primary tumours of the soft tissues are presented (6 rhabdomyosarcomas, 4 leiomyosarcomas, 6 liposarcomas, 4 malignant schwannomas, 5 malignant fibrous histiocytomas, 4 malignant haemangiopericytomas, 3 angiosarcomas, 1 fibrosarcoma, 1 renal sarcoma, 2 malignant mesenchymal tumours without histologically clear classification). The CT image alone will not yield information on the type of tumour or on the tumour status. However, CT continues to rank in the diagnosis of tumours of the soft tissues and is even superior to MR especially in the identification of gas accumulations due to infection in a tumour of the soft tissues that is otherwise unclear. Comparing the literature conclude that MR is now the imaging method of choice in the diagnosis of soft tissue tumours.

[Primary pulmonary hemangiopericytoma in a child]. / Hémangiopéricytome pulmonaire primitif de l'enfant.

The authors report a case of a child, aged fourteen, who had a primary malignant pulmonary haemangiopericytoma. The diagnosis could only be made following the histological study of the excised pneumonectomy specimen and after silver staining. There was a fatal outcome some six months after the operation, despite satisfactory surgery and complementary chemotherapy. The rarity of this type of tumour, notably in children, justifies the presentation of this case report.