[Solitary fibrous tumor/hemangiopericytoma of central nervous system: a clinicopathologic analysis of 71 cases].

Objective: As solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) share the same molecular genetics features, the 2016 WHO classification of central nervous system (CNS) tumors had created the combined term SFT/HPC and assigns three grades. This study aims to investigate the clinicopathologic characteristics, diagnosis, differential diagnosis and prognosis of CNS SFT/HPC. Methods: Seventy-one cases of CNS SFT and HPC were retrospectively reclassified and studied. Histopathological, immunohistochemical and imaging features were analyzed. The follow-up data were analyzed. Results: There were 37 male and 34 female patients. The median age was 48 years (range, 3-77 years). Twelve cases (17%) were WHO grade Ⅰ, 26 (37%) were WHO grade Ⅱ and 33 (46%) were WHO grade Ⅲ. Microscopically the tumor could show traditional SFT phenotype, HPC phenotype or mixed phenotype. Immunochemically, 97%(69/71) were positive for STAT6, with 96%(66/69)showing diffuse strong staining. Approximately 90% were diffusely positive for bcl-2, CD99 and vimentin. The expression rate of CD34 decreased with increasing tumor grade, and the mean expression rate was 78%. SSTR2a was variably expressed in 10% (7/71) of cases including one case showing strong cytoplasmic staining. A few cases expressed EMA, CD57 and S-100 focally. The Ki-67 index ranged from 1% to 50%. Thirty four patients were followed up for 8-130 months; 12 patients(35%)had recurrences, and two (6%) had liver metastases. Conclusions: CNS SFT/HPC is relatively uncommon. There was significant morphological overlap or transition between different grades. STAT6 is a specific marker for the diagnosis of this tumor. Surgical resection is the preferred treatment. WHO grade Ⅱ and Ⅲ SFT/HPC show rates of local recurrence and systemic metastasis, with liver being the most common site of extracranial metastasis.

[Solitary fibrous tumors and hemangiopericytomas of the meninges: Immunophenotype and histoprognosis in a series of 17 cases]. / Tumeurs fibreuses solitaires et hémangiopéricytomes des méninges : immunophénotype et évaluation du grade histopronostique dans 17 cas.

INTRODUCTION: The 2007 World Health Organization (WHO) classification of tumors of the central nervous system distinguishes meningeal hemangiopericytomas (HPC) from solitary fibrous tumors (TFS). In the WHO classification of tumors of soft tissue and bone, those neoplasms are no longer separate entities since the discovery in 2013 of a common oncogenic event, i.e. the NAB2-STAT6 gene fusion. A shared histopronostic grading system, called "Marseille grading system", was recently proposed, based on hypercellularity, mitotic count and necrosis. We evaluated the immunophenotype and histoprognosis in a retrospective cohort of intracranial HPC and TFS. METHODS: Fifteen initial tumors and 2 recurrences were evaluated by immunohistochemistry for STAT6, CD34, EMA, progesterone receptors and Ki67. The pronostic value of the WHO and the Marseille grading systems was tested on 12 patients with clinical follow-up. RESULTS: Initial tumors were 11 HPC and 4 SFT. STAT6 and CD34 were expressed in 16/17 tumors, EMA and progesterone receptors in 2 and 5 cases, respectively. The Ki67 labelling index was 6.25% in HPC and 3% in SFT. Half of the tumors recurred between 2 years and 9 years after initial diagnosis (mean time 5 years). No statistical difference in the risk of recurrence was associated with either grade (WHO or Marseille), in this small cohort. CONCLUSION: The diagnosis of HPC and TFS is facilitated by the almost constant immuno-expression of STAT6, and this justifies their common classification. The high rate of recurrence implies a very long-term follow-up because the current grading systems do not accurately predict the individual risk.

Tumor fibroso solitario dérmico: presentación de caso clínico / Dermal solitary fibrous tumor: a clinical case presentation

Objetivo: analizar la importancia de una metodología protocolizada para el diagnóstico y tratamiento de patologías de baja frecuencia, como el tumor fibroso solitario, y la del diagnóstico diferencial con otras entidades. Caso clínico: se trata de una paciente con lesión subcutánea de 13 cm x 3,5 cm en región geniana izquierda. Tras efectuar estudios clínicos por imágenes y anatomopatológicos, cuyo diagnóstico presuntivo era tumor de tejido blando vascularizado, se realizó la resección de la lesión y la reconstrucción de la zona intervenida, mediante un colgajo local. El diagnóstico definitivo fue tumor fibroso solitario dérmico. Conclusiones: los datos clínicos orientan las conductas diagnósticas complementarias. Los estudios por imágenes muestran las características de la lesión, pero no la definen. La planificación del tratamiento implica la preparación del paciente, la elección de la conducta terapéutica, la evaluación de las posibles complicaciones y el seguimiento del caso. (AU)

Tumor fibroso solitario dérmico: presentación de caso clínico / Dermal solitary fibrous tumor: a clinical case presentation

Objetivo: analizar la importancia de una metodología protocolizada para el diagnóstico y tratamiento de patologías de baja frecuencia, como el tumor fibroso solitario, y la del diagnóstico diferencial con otras entidades. Caso clínico: se trata de una paciente con lesión subcutánea de 13 cm x 3,5 cm en región geniana izquierda. Tras efectuar estudios clínicos por imágenes y anatomopatológicos, cuyo diagnóstico presuntivo era tumor de tejido blando vascularizado, se realizó la resección de la lesión y la reconstrucción de la zona intervenida, mediante un colgajo local. El diagnóstico definitivo fue tumor fibroso solitario dérmico. Conclusiones: los datos clínicos orientan las conductas diagnósticas complementarias. Los estudios por imágenes muestran las características de la lesión, pero no la definen. La planificación del tratamiento implica la preparación del paciente, la elección de la conducta terapéutica, la evaluación de las posibles complicaciones y el seguimiento del caso.

Use of electron microscopy to classify canine perivascular wall tumors.

The histologic classification of canine perivascular wall tumors (PWTs) is controversial. Many PWTs are still classified as hemangiopericytomas (HEPs), and the distinction from peripheral nerve sheath tumors (PNSTs) is still under debate. A recent histologic classification of canine soft tissue sarcomas included most histologic types of PWT but omitted those that were termed undifferentiated. Twelve cases of undifferentiated canine PWTs were evaluated by transmission electron microscopy. The ultrastructural findings supported a perivascular wall origin for all cases with 4 categories of differentiation: myopericytic (n = 4), myofibroblastic (n = 1), fibroblastic (n = 2), and undifferentiated (n = 5). A PNST was considered unlikely in each case based on immunohistochemical expression of desmin and/or the lack of typical ultrastructural features, such as basal lamina. Electron microscopy was pivotal for the subclassification of canine PWTs, and the results support the hypothesis that canine PWTs represent a continuum paralleling the phenotypic plasticity of vascular mural cells. The hypothesis that a subgroup of PWTs could arise from a pluripotent mesenchymal perivascular wall cell was also considered and may explain the diverse differentiation of canine PWTs.

Hemangiopericytomas in the spine: clinical features, classification, treatment, and long-term follow-up in 26 patients.

BACKGROUND: Intraspinal hemangiopericytoma (HPC) is a rare and malignant extra-axial tumor with a strong tendency to recur and metastasize. There is a paucity in the literature of large case series of patients with intraspinal HPCs. OBJECTIVE: We retrospectively analyzed the clinical radiological and histological features, classification, and treatment of 26 patients with HPCs in the spine. METHODS: Twenty-six patients with HPCs in the spine were treated at our institution between 1987 and 2010. Medical records were reviewed retrospectively to collect data on the clinical features, tumor morphology, surgical resection, recurrence, and follow-up. RESULTS: The 26 patients were predominantly male, and the mean age at diagnosis was 33.8 years. The intraspinal HPCs were divided into 3 types and 5 subtypes. Most of them involved the neighboring segments and/or caused bony erosion. All tumors were immunohistochemically positive for vimentin and negative for epithelial membrane antigen. All patients underwent at least 1 surgery, and most of them received postsurgical radiotherapy. The 5-year Kaplan-Meier rate of survival was 76%. The 5-year recurrence-free rate of survival was 29.4%. Only the tumor pathological grade was significantly associated with survival time and recurrence. CONCLUSION: High-grade tumors had a shorter survival time and recurred earlier than low-grade tumors. Surgical removal and postoperative radiotherapy are critical for the treatment of intraspinal HPCs. However, total resection may not necessary for these tumors. Stereotactic radiosurgery may be a good alternative to control the recurrent lesions.

Hemangiopericytomas grade II are not benign tumors.

BACKGROUND: Hemangiopericytomas (HPs) of the central nervous system are rare tumors and afflicted with a high propensity of recurrences and metastases. Histopathologically, HPs correspond to differentiated (WHO grade II) and anaplastic (WHO grade III) tumors. With respect to the available literature and our own experiences, the aggressiveness, especially of differentiated grade II HPs, seems to be underestimated. METHODS: Thus, in this retrospective study, we describe tumor behavior and examined the effect of radio- and chemotherapy on tumor control with respect to the WHO classification of grade II and III neoplasms. This study consists of 15 patients with cerebral (n = 10) and spinal (n = 5) HPs. RESULTS: Seven HPs were histopathologically classified as grade II and eight as anaplastic grade III tumors. Complete surgical resection could be achieved in 60% of cerebral and in 25% of spinal HPs. In total, local recurrences occurred in 20% of patients within 17.3 months after the primary operation. Recurrences occurred both from differentiated (n = 1) and anaplastic (n = 2) neoplasms. Treatment comprised re-operation followed by radio- and chemotherapy. Pointing out the importance of the extent of surgical resection, in this study, we could not detect a single patient showing any recurrences or systemic metastases after complete surgical resection of grade II HPs. During primary diagnostics, four patients showed systemic metastases. Although these tumors could be controlled via surgery, systemic metastases appeared in further four patients within 60.4 months. Interestingly, two of them were classified as differentiated tumors (WHO grade II). To control tumor progress, radiotherapy seemed to be partially effective. On the other hand, however, chemotherapy did not show any effect on tumor control. With respect to these results, screening investigations seem to be indispensable and are highly recommended during primary diagnostics and after the appearance of recurrences or metastases, independent of the histopathological staging of the tumor. CONCLUSION: With respect to our results, radical surgical resection offers the best treatment option to control tumor progress. In case of subtotal resection or histopathologically diagnosed anaplasia (WHO III), radiotherapy seems to be indicated; however, chemotherapy did not show effectiveness to control tumor progress.

[Solitary fibrous tumor and haemangiopericytoma: what is new?]. / Solitärer fibröser Tumor und Hämangioperizytom : Was ist neu?

Soft-tissue tumors with haemangiopericytoma (HPC)-like growth patterns can now be divided into three categories: (1) The solitary fibrous tumour (SFT) group with its variants; (2) lesions showing clear evidence of myoid/pericytic differentiation and corresponding to "true" HPCs (myopericytoma/glomangiopericytoma and a subset of sinonasal HPCs); (3) neoplasms that occasionally display HPC-like features (e.g. synovial sarcoma). In this study 268 intrathoracic and extrathoracic SFTs from the German consultation and reference center of soft tissue tumors in Jena were evaluated and analyzed immunohistochemically with antibodies CD34, Bcl-2, CD99, SMA, S100, PanCK and Ki-67. Furthermore, SFTs were categorized into the newly proposed SFT designation: Fibrous variant, cellular variant (more than 90% hypercellularity), fat-forming variant, giant cell-rich variant and malignant SFTs. This article should provide insights into the diagnosis of this entity with emphasis on the new international standard.

Intranasal myopericytoma. A tumour with perivascular myoid differentiation: the changing nomenclature for haemangiopericytoma.

We present a case report of a patient who developed a sinonasal myopericytoma treated by surgical excision through a lateral rhinotomy. Some aggressive features on pre-operative computed tomography scanning and the complexity of recent changes in the histological nomenclature for these tumours led to consideration of adjuvant therapy. The close histological relationship between myopericytoma, myofibromatosis, solitary myofibroma and infantile haemangiopericytoma is discussed. This group of lesions constitute a single morphological spectrum with differentiation towards perivascular myoid cells (pericytes). Currently myopericytoma is the most appropriate and accepted term embracing all these entities. A review of the literature has been reassuring in identifying these tumours as benign but with a reasonably high rate of local recurrence (17 per cent). The treatment of choice is surgical excision with further excisions for local recurrence.

[Congenital myofibroma. A true hemangiopericytoma. A neonatal case with immunohistochemical and ultrastructural studies]. / Miofibroma congénito. Un hemangiopericitoma verdadero. Un caso neonatal con estudio inmunohistoquímico y ultraestructural.

We report the case of a male newborn infant with a pedunculated dermic tumor, located in the right malar region; who underwent a complete surgical resection of the tumor and had a satisfactory postoperative evolution. The histopathologic findings disclosed a subcutaneous tumor with a nodular aspect and a subendothelial intravascular growth, constituted by a dual population of small cells and spindle-shaped cells, distributed in a biphasic pattern. All tumor cells showed a strong pericellular reaction for PAS. The immunohistochemical studies revealed: diffuse cytoplasmic positivity for CD34 and Vimentin in all tumor cells, and only spindle-shaped tumor cells and less differentiated isolated neoplastic cells, presented cytoplasmic positivity for the smooth muscle alpha-actin. The electronic microscopy demonstrated a layer of basal membrane and in the citoplasm, numerous intermediate filaments with focal condensations. Based on all these findings, we conclude that this is a myofibroma, a "true hemangiopericytoma" with myofibroblastic differentiation. For this reason, we propose the term myofibropericytoma, in order to highlight its pericytic origin and its myofibroblastic differentiation. We emphasize the need to recognize this entity, in view of its low frequency and the possibility of a diagnostic mistake with other soft tissues tumors that display haemangiopericytoma-like features.

[Vascular tumours and malformations, classification, pathology and imaging]. / Tumeurs et malformations vasculaires, classification anatomopathologique et imagerie.

The understanding of vascular anomalies (vascular tumours and vascular malformations) was obscured, for a long time, by confusion and uncertainties in nosology and terminology. The International Society for the Study of Vascular Anomalies (ISSVA) recently adopted a classification scheme, clearly separating vascular tumours (hemangiomas of different types) which result from active cell proliferation, from vascular malformations, which are inborn defects in vascular morphogenesis. These two types of lesions have different clinical behaviour and require different diagnostic and therapeutic strategies. The most frequent vascular tumour is infantile hemangioma. Its clinical aspects and evolution are well-known. New data have been recently obtained concerning the phenotype of tumour cells and its histogenesis. Of the numerous new vascular tumours, which have been recently described, only the congenital hemangiomas, the vascular tumours associated with the Maffucci syndrome and the tumours that may be complicated by a profound thrombocytopenia (Kasabach and Merritt phenomenon) will be considered. Vascular malformations can be classified according to the vessel(s) types they are composed of. A classification table is presented, separating the malformations of vascular trunks from tissular malformations which are more intimately embedded in the surrounding tissues. The different syndromes associated with vascular anomalies take also place in this table. The clinical, imaging and histological aspects of the most frequent malformations (capillary, venous, lymphatic and arteriovenous) are presented. This classification intend to clarify the nosology and terminology of the complex field of vascular tumours and malformation and to offer a common language to the different physicians and specialists contributing, preferably with a interdisciplinary approach, to the diagnosis and treatment of these difficult lesions.

[Vascular tumours and malformation of the orbit]. / Les lésions vasculaires de l'orbite.

Vascular diseases are an important part of orbital pathology. We describe vascular tumours of the orbit and vascular diseases with repercussion on the orbit, from intra or extra orbital origin. The classification of these abnormalities is difficult and several terms are used to describe the same histological entity. The objective of this work is, using the current classification, to illustrate the different imaging aspects of the most frequent vascular diseases of the orbit.

Hemangiopericitoma de cabeza y cuello

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Hemangiopericitoma en territorio de cabeza y cuello: a propósito de cuatro casos / Hemangiopericytoma of the head and neck area. A report of four cases

El hemangiopericitoma (Hpc) es un raro tumor que pertenece a la familia de los sarcomas y deriva de los pericitos de Zimmerman que rodean los vasos sanguíneos. Se trata de un tumor vascular (1 por ciento de los tumores vasculares) con un comportamiento paradójico, presentándose en la mayoría de los casos con características de un tumor benigno, aunque su índice de recurrencias y metástasis ascienden hasta un 50 por ciento. Por ello se trata como un tumor potencialmente maligno. En otras ocasiones presenta un comportamiento más agresivo. En este artículo aportamos cuatro nuevos casos a los ya publicados y hacemos un repaso meticuloso de la literatura en todo lo referente a esta neoplasia (AU)

Orbital hemangiopericytoma and solitary fibrous tumor: a morphologic continuum.

Solitary fibrous tumors (SFT) and hemangiopericytomas (HPC) are soft tissue tumors with known histologic and immunohistochemical overlap. A series of these tumors located in the orbit were analyzed in order to determine whether they could be re-classified based on currently recognized histologic criteria. Ten orbital spindle cell lesions, all of which were positive for CD34 antigen, were examined. Diagnostic criteria for SFT included a cytologically bland spindle cell lesion with variable cellularity and focal dense collagenization with diffuse, strong CD34 reactivity, while the criteria for HPC required a more monotonous cellular proliferation without significant variability in cellularity, a "staghorn" vascular pattern, minimal collagenization, and focal or absent CD34 staining. Tumors with typical histologic and immunohistochemical features of HPC or SFT were diagnosed as HPC and SFT, respectively. Those tumors with histologic or antigenic profiles not classic for HPC or SFT were defined as 'indeterminate.' Three lesions were classified as SFT and 1 tumor was diagnosed as HPC through use of the above-cited histologic criteria. All lesions showed positive staining of tumor cells with CD34 antigen in varying amounts and were negative for cytokeratin AE1-3, epithelial membrane antigen, CD68, and Factor XIIIa. One solitary fibrous tumor focally stained for S-100 protein and 1 hemangiopericytoma was focally positive for HHF-35. Of the 10 analyzed tumors, 6 were classified as 'indeterminate.' Furthermore, 1 lesion whose primary histology was that of an SFT recurred 9 years later with an appearance consistent with an 'indeterminate' lesion. Our results call into question the present histologic separation of HPC and SFT in the orbit. As in other sites, including deep soft tissue, these data suggest that SFT and HPC are 2 lesions whose morphologic features are best interpreted to exist along a continuum, rather than 2 lesions with distinctly defined histopathology.

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.

[Intracranial hemangiopericytoma 32 cases reports].

OBJECTIVE: To further elucidate the clinical feature, pathogenesis, treatment and prognosis of intracranial hemangiopericytomas (HPC). METHODS: Thirty-two cases of HPC treated in our department were analyzed retropectively. RESULTS: Grade I surgery, complet tumor removal with excision of involved dural and bone (7 cases); Grade II surgery, complet tumor removal with aggressively cauterization of dural and/or bone (13 cases); Grade III surgery, complet tumor removal but no satisfactory attempt to deal with involved dural or bone (7 cases); Grade IV surgery, tumor residual (7 cases). 13 cases were recurrent. CONCLUSION: The prognosis of intracranial hemangiopericytomas is dismal. The recurrent rates of HPC are very high. Microneurosurgery and adjuvant radiotherapy are main treatment. It is very important that counterchecking at regular intervals.

Lipomatous hemangiopericytoma. A histologic, ultrastructural and immunohistochemical study of a unique variant of hemangiopericytoma.

We report three cases of a unique, previously undescribed soft tissue tumor composed of mature adipocytes and hemangiopericytomatous areas, for which we propose the term lipomatous hemangiopericytoma. The tumors occurred in adults and were located in the sinonasal area, the soft tissue of the shoulder, and the retroperitoneum. The tumors ranged in size from 4 to 10 cm in greatest diameter and grossly were solid and ranged from tan to yellow. Histologically, they were composed of a variable admixture of benign lipomatous and hemangiopericytomatous components. Immunohistochemically, they stained with antibodies to vimentin and not to alpha-smooth-muscle actin, muscle-specific actin, desmin, S-100 protein, glial fibrillary acidic protein, epithelial membrane antigen, or keratin. Ultrastructurally, the cells constituting the hemangiopericytomatous areas had the features of pericytes, and no lipoblasts or transitional forms between lipocytes and pericytes were found. The histologic differential diagnosis of this neoplasm includes spindle-cell lipoma, angiolipoma, liposarcomas, tumors showing smooth muscle and adipocytic differentiation, and hemangiopericytoma infiltrating fat. Because of the small number of cases and the limited follow-up, we cannot be certain of their biologic behavior, although we expect that they are benign. Lipomatous hemangiopericytoma represents a distinctive pathologic entity that should be recognized and studied further.