Malignant hemangiopericytoma of the condyle.

Hemangiopericytoma (HPC) is an uncommon vascular tumor arising from pericytic cells with variable malignant potential. Primary HPCs of the bone are extremely rare; however, involvement of the condylar process has not been reported. We presented a 21-year-old female patient with low-grade malignant HPC in the right mandibular condyle. Clinical examination and imaging findings revealed a well-defined soft mass, encapsulating the mandibular condyle from behind. The lesion and mandibular condyle were removed simultaneously; then, the costochondral graft was used to reconstruct the defect. Histopathologic examinations of the specimen confirmed the diagnosis. After 2 years of follow-up, the patient is free of disease, and mouth opening returned to normal.

Hemangiopericytoma of meningo-fronto-naso-orbito-maxillary complex.

Hemangiopericytoma (HPC) is a rare vascular tumour and difficult to diagnose clinically. Incidence is reported in fourth to fifth decade of life.With female predominance, 3%-5% cases affect the oral cavity, sinus lining and meninges. The patient presented with 8×6 cm swelling on her face, evaluation reported it to be HPC. Bilateral maxillary artery embolisation, wide local excision of the lesion, preserving the left eye and its function, was done. No recurrence is reported at 1-year follow-up. Response of such lesions to radiotherapy is questionable; with no lymphadenopathy and adequate encapsulation, embolisation of feeder vessel followed by a wide local excision of the lesion seems to be a fairly good option of treatment.

Rare nasosinusal tumors: case series and literature review.

Tumors of the nasal cavity and paranasal sinuses are unusual pathologies found in clinical practice. Approximately 0.8% of all human cancers are located in this area. Despite being rare, nasosinusal neoplasms usually manifest through nonspecific symptoms that are common to numerous inflammatory pathologies. The aim of this study is to describe a series of rare nasosinusal tumors, including esthesioneuroblastomas, central giant cell granulomas, extramedullary plasmocytomas, nasosinusal hemangiopericytomas, neurofibromas and cemento-ossifying fibromas, diagnosed at the Fortaleza General Hospital. We, hereby, briefly review each of the aforementioned pathologies, stressing the need for a precise histological diagnosis for proper treatment in each case.

Glomangiopericytoma of the pterygomandibular space: an unusual case.

We describe a case of glomangiopericytoma located in the pterygo-mandibular space, a rare anatomical region for this neoplasm to develop. The lesion is classified as a separate variant from the classic haemangiopericytoma, which is characterised by more aggressive biological behaviour.

Primary hemangiopericytoma in the axis bone: case report and review of literature.

OBJECTIVE AND IMPORTANCE: To the best of our knowledge, this is the first reported case of primary hemangiopericytoma in the axis bone. With this report, we attempt to better characterize this uncommon lesion in the vertebral column. CLINICAL PRESENTATION: This report describes a case of primary heman-giopericytoma in the axis bone of a 16-year-old female patient who presented with acute torticollis. Her neurological status was unimpaired. A lateral radiograph of the cervical spine demonstrated an odontoid neck fracture and a C1-C2 rotatory deformity. The magnetic resonance imaging study showed a hypodense lesion with moderate enhancement on T1- and T2-weighted images on the dens and body of the axis with an odontoid neck fracture. Reviewing the literature, primary hemangiopericytoma in the spine is rare; 9 cases of hemangiopericytoma with vertebral bone involvement and 44 cases of this tumor with intraspinal meningeal involvement have been reported. INTERVENTION: The patient was treated with odontoidectomy via the transoral approach, along with posterior fixation using the Halifax clamp. She was followed up 17 months after surgery, and no evidence of tumor recurrence was found. CONCLUSION: The treatment for osseous hemangiopericytoma is still controversial. At present, adequate surgical removal with postoperative radio-therapy is recommended. In addition, although hemangiopericytoma is rare in the spine, it should be kept in mind in the differential diagnosis of spinal tumors.

Benign lesions of the mandibular and maxillary region in children: characterization by CT and MRI.

The CT criteria for classifying lesions of the mandibular and maxillary regions in adults as benign or malignant are well recognized: irregular soft tissue margins and bone destruction. We reviewed the charts of 12 pediatric patients from 3 institutions to evaluate these criteria in children. These masses were evaluated by CT and/or MRI based solely on well-recognized and published criteria. The patients' ages ranged from 4 mo to 18 yr. The histological diagnoses were ameloblastoma (4), hemangioma (2), lymphangioma, desmoplastic fibroma, hemangiopericytoma, neurofibroma, fibrous dysplasia, and juvenile angiofibroma. All but one case was surgically proven. Among the 12 cases, 5 had bone destruction and 5 showed irregular soft tissue margins. Three cases satisfied both criteria. With well-recognized CT criteria (in adult patients), characterization of these processes led to an incorrect diagnosis of a malignant lesion in 8 of the 12 cases preoperatively. Therefore, CT and MR imaging should only be a guide to the planning and extent of surgical resection. Without clearly defined distal spread of the mass, the classification of these masses in children should be made with caution.

Head and neck hemangiopericytoma in a child: case report.

CONTEXT: Hemangiopericytoma is a relatively rare tumor, first described in 1942, with approximately 300 cases described in the literature to date. In most cases, it affects the trunk and lower extremities. The head and neck incidence is less than 20%, mostly in adults. We describe a case of malignant head and neck hemangiopericytoma in a child. TYPE OF STUDY: Case report. CASE REPORT: A twelve-year-old male patient noted the presence of a firm painless right-side retroauricular lymph node of 1 cm in diameter, which at first remained unchanged for six months, but subsequently enlarged progressively. He denied having had previous trauma at that site. In November 2000, he presented nasal obstruction and voluminous epistaxis that required hospitalization and blood transfusion. During dental treatment one month later, a cranial x-ray revealed bone alterations. A subsequent computed tomography scan showed an extensive lesion of soft tissue density that had invaded the maxillary fossa, eroding the skull base and middle and nasal fossa. The child was then referred to our service, where biopsy was performed, giving a diagnosis of hemangiopericytoma. Shortly afterwards, magnetic resonance imaging revealed that this lesion had undergone significant growth, while maintaining the same invasion pattern. The patient was submitted to conservative surgery in April 2001, with only partial resection of the tumor because of its extent. Histopathological examination of the specimen confirmed the presence of malignant hemangiopericytoma. Following the surgery, the patient presented fast regrowth of the lesion, with partial response to chemotherapy and radiotherapy.

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.

[Hemangiopericytoma of the maxillary sinus: apropos of a case]. / Hemangiopericitoma del seno maxilar: a propósito de un caso.

Haemangiopericytoma is a rare vascular tumour arising from the proliferation of pericytes which are cells surrounding the capillaries. Since 1949 only 54 cases of haemangiopericytomas of the nose and paranasal sinuses have been reported in the literature. Of the latter, only 8 cases originated from the maxillary sinus. We report a further case originated from the maxillary sinus.

Case report of fetal lingual tumor - perinatal care and neonatal surgical intervention.

Fetal tumors are rare and can be difficult to diagnose in utero. In this presentation, we report an infantile hemangiopericytoma of the tongue, and also review the clinical characteristics, imaging appearances, perinatal differential diagnosis, and potential treatments of this tumors. Prenatal diagnosis of lingual fetal tumors with two-dimensional (2D) ultrasound presents a challenge to sonographers and perinatologists because these lesions are rare, usually develop in the third trimester of pregnancy and need a multidisciplinary approach. We describe an lingual tumor and moderate polyhydramnios which were prenatally detected at 31 weeks of gestation with two- dimensional ultrasound. The application of the surface mode of three-dimensional ultrasound improved the visualization of this tumor. Fetal Magnetic resonance imaging was performed to help in prenatal differential diagnostic. The option of prenatal cytology gained by amniocentesis should also be considered. To our knowledge there are no prenatal reports of similar cases. However, in our experience every information provided during the pregnancy, a correct and early diagnose of fetal condition helps to cope with the demands of perinatal management and to explain to the parents and neonatologists the findings of the case.

Onyx embolization of an intracranial hemangiopericytoma by direct transcranial puncture.

Intracranial hemangiopericytomas are uncommon but highly vascular dural-based tumors which commonly derive arterial blood supply from both intracranial and extracranial circulations. Microsurgical resection of these lesions may result in excessive blood loss without the aid of pre-operative embolization. We describe a case of a large tentorial hemangiopericytoma for which initial resection was aborted due to excessive blood loss. After failed endovascular access, we performed a direct transcranial puncture of the hemangiopericytoma through the craniotomy defect and successfully embolized the tumor with Onyx. Post-embolization gross total resection was achieved with a limited amount of operative blood loss. Direct puncture embolization provides several advantages over traditional endovascular embolization including decreased procedural duration, circumventing challenging arterial anatomy, and a lower risk of stroke in the presence of extracranial-to-intracranial anastamoses. While direct puncture embolization has been described for tumors of the neck and skull base, this is the first reported case of an intracranial hemangiopericytoma successfully devascularized by direct transcranial puncture Onyx embolization.

Primary haemangiopericytoma of the parapharyngeal space: an unusual tumour and review of the literature.

Haemangiopericytoma is a rare soft tissue tumour, with great histological variability and unpredictable clinical and biological behaviour. The precise cell type origin is uncertain. One third of haemangiopericytomas occur in the head and neck area, but only a few cases have been reported regarding localization at the parapharyngeal space. Herewith, case is presented of a 54-year-old female, referred to our Department due to a parapharyngeal space tumour with non-specific imaging characteristics. The patient underwent radical excision of the tumour with a trans-cervical sub-mandibular approach. The histolopathologic examination revealed a neoplasm with the characteristic features of haemangiopericytoma. One year later, during the scheduled follow-up, the computerized tomography scan showed no evidence of recurrence or residual disease. The pre-operative evaluation of a haemangiopericytoma must include a thorough imaging evaluation with computerized tomography and magnetic resonance imaging, even if results may not be specific for haemangiopericytoma. Angiography and pre-operative embolization may be performed in cases of large tumours with significant vascularity. The treatment of choice is radical excision. The follow-up includes clinical evaluation every 6 months and annual magnetic resonance imaging for at least 3 years.

Hemangiopericytoma of the maxilla in a pediatric patient: a case report.

The hemangiopericytoma is a malignant vascular tumor arising from mesenchymal cells with pericytic differentiation. Hemangiopericytoma is most commonly seen in adults, and only 5% to 10% of cases occur in children. The tumor is extremely rare in the head and neck region (16%). Cytogenic abnormalities have been present in some hemangiopericytoma cases. Surgical resection remains the mainstay treatment. Adjuvant chemotherapy and radiotherapy is appropriate for cases of incomplete resections and life-threatening tumors particularly in children. Late relapses may occur and require long-term follow-up. A 4-year-old child patient with hemangiopericytoma of the maxilla presented with firm, recurrent, but painless jaw mass. Radiographic investigations revealed a poorly circumscribed radiolucency. The lesion biopsy showed wellcircumscribed multiple lobules of tumor mass consisting of tightly packed, spindleshaped cells. Chemotherapy and radiotherapy of the lesion was conducted. The role of the pediatric dental team is extensive in children with hemangiopericytoma, who require a regular dental review. The patient's oncologist should be immediately contacted if there is any suspicion of recurrence.

Hemangiopericytoma of maxilla in a pediatric patient: a case report.

The Hemangiopericytoma is a malignant vascular tumor arising from mesenchymal cells with pericytic differentiation. Hemangiopericytoma is most commonly seen in adults, and only 5% to 10% of cases occur in children. The tumor is extremely rare in the head and neck region (16%)1. Cytogenic abnormalities have been present in some hemangiopericytoma cases. Surgical resection remains the mainstay treatment. Adjuvant chemotherapy and radiotherapy is appropriate for cases of incomplete resections and life-threatening tumors particularly in children. Late relapses may occur and require long-term follow-up. A 4-year-old child patient with hemangiopericytoma of the maxilla presented with firm, recurrent, but painless jaw mass. Radiographic investigations revealed a poorly circumscribed radiolucency. The lesion biopsy showed well-circumscribed multiple lobules of tumor mass consisting of tightly packed, spindle-shaped cells. Chemotherapy and radiotherapy of the lesion was conducted. The role of the pediatric dental team is extensive in children with hemangiopericytoma, who require a regular dental review. The patient's oncologist should be immediately contacted if there is any suspicion of recurrence.

Myopericytoma of the lip: report of case.

BACKGROUND: Myopericytoma is a recently delineated entity showing a hemangiopericytoma-like vascular pattern. This rare mesenchymal neoplasm arises within the subcutaneous tissue of the extremities. Only 3 cases of myopericytoma involving the soft tissues of the mouth have been reported, and this case is the first, to the best of our knowledge, that involves the lips. METHODS: A 28-year-old black man had a slowly growing, painless, firm, ulcerated lower lip mass. Histopathologic examination and immunohistochemical analysis were performed, and the differential diagnosis included carcinoma and other well-circumscribed, vascular, slow-growing lesions of the mouth-like myofibroma, glomus tumor, and solitary fibrous tumor. RESULTS: The results of the histology combined with the immunohistochemical analysis led to a diagnosis of myopericytoma. CONCLUSIONS: This case may aid others in recognizing this very rare entity. As the myopericytoma shares morphologic features with other perivascular myoid neoplasms, it should be included in the differential diagnosis of well-circumscribed, vascular, slow-growing lesions of the mouth.

Diagnostic dilemma: solitary fibrous tumor or hemangiopericytoma of the submandibular region in a patient with multiple odontogenic keratocysts.

Solitary fibrous tumors (SFTs) are rare spindle cell neoplasms that originate from mesothelial-lined surfaces, mainly the pleura and peritoneum. SFT is a distinctive mesenchymal neoplasm of the pleura but can be found in extrapleural sites. The treatment of SFT is simple excision or wide local resection. This is the case study of a 14-year-old girl who presented with a large diffuse swelling in the left submandibular region, ultimately diagnosed with SFT and surgically excised.

Hemangiopericytoma of the cerebellopontine angle: a case report and review of the literature.

BACKGROUND: Intracranial hemangiopericytoma represents a rare intracranial tumor that is typically difficult to distinguish from meningioma based on clinical presentation and radiographic findings. These inherently aggressive neoplasms have been observed to occur in numerous intracranial compartments; however, isolated involvement of the CPA is essentially unreported. The authors present a case of a young lady with presumed right acoustic schwannoma, which proved to be HPC on histopathology. The case is described; and a review of the literature pertaining to the diagnosis, optimal management, and follow-up for these lesions is provided. CASE DESCRIPTION: A 37-year-old Asian woman presented with a 7-month history of right ear and mandible numbness, as well as subjective hearing loss involving the right ear. Magnetic resonance imaging demonstrated the presence of a homogeneously enhancing extraaxial lesion in the right CPA, radiographically suggestive of an acoustic schwannoma. The lesion proved to be an intracranial HPC on histologic sections. Review of the neurosurgical literature yielded only one prior detailed account of HPC confined to the CPA. The patient underwent right retrosigmoid craniotomy for gross total resection of the mass, followed by stereotactic radiotherapy several weeks postoperatively. CONCLUSION: Given the fundamentally different treatment approach for HPCs over other more common CPA tumors, it is imperative that the treating surgeon consider this rare diagnosis when evaluating patients with lesions localized to this area. Specifically, gross total resection, followed by adjuvant SRT, provides patients with the highest probability for disease-free survival, based on current evidence in the neurosurgical literature.

Sequential salvage chemotherapy for recurrent intracranial hemangiopericytoma.

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.

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.