Urgent surgical management of congenital intracranial hemangiopericytoma in a preterm neonate.

Hemangiopericytoma is a rare mesenchymal tumor originating from capillary pericytes, known as Zimmermann pericytes. The adult form is not uncommon and generally malignant but tumor is found rarely in children. Here we describe an intracranial hemangiopericytoma in a preterm newborn whose had the tumor resected successfully shortly after birth.

Haemangiopericytoma-like tumor of the nasal cavity treated by endoscopy - a case report.

Haemangiopericytoma (HPC) is an uncommon, vascular tumor derived from Zimmerman pericytes surrounding blood vessels. HPC constitute around 1% of all tumors of vascular origin and may appear anywhere, 5% of them can be situated in nasal cavity. Tumor location within the head and neck predispose to its benign character and improves prognosis. This case report presents the case of 33-year-old patient with haemangiopericytoma-like tumor of the nasal cavity, presented symptoms of impaired nasal breathing and recurrent epistaxis. Tumor was excised with 0 degree endoscope. The follow-up recurrence-free period was 2 year 6 months and shows this is effective way of treatment. Described in the literature late recurrences and metastases reminds that regular, life-long observation is mandatory.

[Hemangiopericytoma of the buccal mucosa. A case report]. / Hämangioperizytom der Wange. Ein Patientenbeispiel.

Hemangiopericytoma describes a rare mesenchymal neoplasm which can occur at any site on the body and represents 1% of vascular tumors. Histologically, it is characterized by a vascular pattern with high cellularity. These tumors occur in particular near the torso and surface variants are rare. Due to the unspecific clinical picture, it is often confused with other benign mesenchymal tumors. The heterogenous course and high risk of recurrence with hemangiopericytomas necessitate long-term clinical follow-up.

An infantile splenic hemangiopericytoma case treated with partial splenectomy.

Splenic hemangiopericytoma is a very rare tumor. So far only 10 patients (9 adults, 1 child) have been reported in the literature and all of them were treated with total splenectomy. Herein, we report the first infant case of the splenic hemangiopericytoma in a 10-month-old girl and the first case that was treated with partial splenectomy for splenic hemangiopericytoma.

Follicular acneiform eruption induced by bevacizumab.

Bevacizumab is a recombinant humanized monoclonal IgG1 antibody that binds to and inhibits the biologic activity of human vascular endothelial growth factor (VEGF). This antibody, in combination with other antineoplastic agents, is currently used to treat various neoplasms, including colorectal, lung, breast, kidney cancer, and glioblastoma. It is also being used as an off-label intravitreal agent in the treatment of proliferative (neovascular) eye diseases. We report the development of a skin rash with two different patterns in a patient with a hemangiopericytoma of the meninges, a rare aggressive sarcoma, who was treated with repeated intravenous injections of bevacizumab.

Preoperative radiation therapy in the management of recurrent orbital hemangiopericytoma.

A 77-year-old man presented for evaluation of a recurrent right orbital hemangiopericytoma. It had been excised twice over the past 6 years. After the second resection, the tumor rapidly recurred and was insensitive to systemic chemotherapy, and the patient was thus referred to the authors' institution. The patient had proptosis, restricted ocular movement, and binocular diplopia on presentation. Orbital imaging confirmed a well-circumscribed right extraconal mass in the medial orbit. Preoperative radiation therapy was given, which reduced the tumor volume considerably and allowed a successful complete surgical excision of the mass.

[Sinunasal hemangiopericytoma]. / Hämangioperizytom vom sinunasalen Typ.

A 65-year-old female was admitted to hospital for evaluation of recurrent, right-sided sometimes ensanguined, rhinorrhea and epistaxis. Clinical examination revealed a right-sidedunilateral polypoid nasal mass. After endoscopic sinus surgery a sinunasal hemangiopericytoma (SNHPC) was diagnosed. Revision surgery was performed without any residual tumor. To date the patient is free of recurrence. SNHPCs are uncommon neoplasms of vascular origin. The prognosis is mainly determined by initial tumor size and expansion as well as primary complete resection.

How to differentiate spinal cord hemangiopericytoma from common spinal cord tumor?

INTRODUCTION: Tumors of the spinal cord are rare and some can be confused with each other. We report a rare spinal cord solitary fibrous tumor/hemangiopericytoma (SFT/HPC), and propose keys to differentiate spinal cord tumors from each other. CASE REPORT: A 67-year-old man presented weakness with recent diffuse sensory disorders in the right lower limb. Spinal MRI revealed a T8-T9 intradural extramedullary mass with spinal cord compression. Gross total resection of a poorly vascularized intradural tumor was achieved. It was an encapsulated extramedullary tumor, which was difficult to separate from the spinal cord due to the presence of pial adhesions. Definitive diagnosis was grade 1 SFT/HPC of the spinal cord. One-year follow-up MRI revealed complete excision without any evidence of residual tumor. CONCLUSION: SFT/HPC is a very rare spinal tumor that can be extramedullary, intramedullary or both. It may perfectly mimic meningioma. The maximal resection is the best treatment, but can be challenging because of the tumor's firm consistency and pial adherences to the spinal cord. Outcome is good in case of gross total resection, but there is a risk of very late recurrence, requiring long-term follow-up.

Acute abdomen due to rupture of hemangiopericytoma of the greater omentum: case report.

Hemangiopericytoma (HP) is a vascular tumor that mostly develops in soft tissues. The greater omentum is a very rare site for its occurrence. We present herein the clinical evaluation and outcome of a very rare case of HP that caused acute abdomen. We evaluated a case of acute abdomen due to rupture of a HP of the greater omentum. The clinical and laboratory findings and treatment modality are reported. A 70-year-old patient with severe abdominal pain was operated with the diagnosis of acute abdomen. A semi-solid mass (12 x 10 x 6 cm) originating from the greater omentum was detected during surgery. There was active bleeding from the tumor. Pathologic evaluation of this lesion was reported as benign HP. HP of the greater omentum can be the cause of intraabdominal bleeding leading to acute abdomen. Surgical resection is the treatment of choice, especially in benign hemangiopericytomas.

[Hemangiopericytoma of the posterior fossa: case report]. / Hemangiopericitoma de la fosa posterior: a propósito de un caso.

Hemangiopericytoma is an uncommon mesenchymal neoplasm arising from Zimmerman's pericytes, which usually locates in soft tissues. Meningeal hemangiopericytoma accounts for less than 1% of all intracranial tumours. Typically, it behaves aggressively, showing distinct tendency to recur locally or distantly along the neural axis and to present extraneural metastases. We describe a 74-year-old patient who presented unspecific symptoms and whose physical exam revealed a painless retroauricular mass which was adhered to skin. Neuroimaging studies showed a large posterior fossa tumour with intense enhancement after contrast infusion that caused striking occipital-mastoid osteolysis and which was exclusively fed by external carotid artery branches. The patient underwent gross total resection of the tumour, and once the histological diagnosis of hemangiopericytoma was confirmed, she underwent initial adjuvant radiotherapy. Sixteen months after surgery, the patient remains recurrence free. The treatment of choice of intracranial hemangiopericytoma is gross total resection, which must be attempted when technically feasible, followed by adjuvant radiotherapy providing total doses over 50 Gy. This combination has demonstrated increasing recurrence- free interval in these patients. Close and longterm follow-up is mandatory in order to achieve early diagnosis of recurrence or metastases in these patients, since they can appear several years, even decades, after initial proper treatment.

[Patient with dyspnoea and gigantic tumor of the chest]. / Pacjentka z dusznoscia i olbrzymim guzem sciany klatki piersiowej.

We describe the patient with gigantic chest tumor admitted to the hospital because of dyspnoea. The patient was operated on after the elimination of suspicion of acute pulmonary embolism. Cardiovascular failure was caused by the huge arterial-venous fistula in the tumor and diastolic heart failure were the reasons for dyspnoea. On the basis of the microscopic examination hemangiopericytoma (HPC) was diagnosed--a mesenchymal tumor derived from pericytes. We present in our paper the rules applying to dealing with HPC.

Intraoperative Transpedicular Onyx Injection to Reduce Vascularity of a Thoracic Hemangiopericytoma After Unsuccessful Preoperative Endovascular Embolization: a Technical Report.

BACKGROUND AND IMPORTANCE: Hemangiopericytoma is a rare vascular tumor with central nervous system involvement representing only 1% of central nervous system tumors. They rarely affect the vertebral column. Complete surgical resection is the treatment of choice for hemangiopericytoma given their high rates of local recurrence. However, the high vascularity of such tumors with the risk of massive bleeding during surgery represents a significant challenge to surgeons. Therefore, preoperative endovascular embolization via the transarterial route has been advocated. CLINICAL PRESENTATION: In the current study, we present a case of a T12 hemangiopericytoma that was managed by a 2-stage surgical resection, with the use of intraoperative transpedicular onyx injection to reduce intraoperative blood loss following an unsuccessful trial of preoperative endovascular embolization. CONCLUSION: Preoperative endovascular embolization is not feasible in some cases due to the location of the segmental or radiculomedullary arteries in relation to tumor feeders and, rarely, small size of these arterial feeders. Percutaneous injection of onyx is an option. In this case report, we discuss direct intraoperative injection via a transpedicular route as a safe and effective method for decreasing the vascularity of some lesions and improving intraoperative blood loss.

Endonasal endoscopic laser-assisted resection of septal glomangiopericytoma.

Glomangiopericytoma is a rare vascular neoplasm characterised by a pattern of prominent perivascular growth with myoid phenotype. It is categorised as a borderline low-malignancy tumour by WHO and accounts for less than 0.5% of all sinonasal tumours. After curative resection, patients of glomangiopericytoma need long-term endoscopic follow-up due to high risk of recurrence.We report a case of a 23-year-old man complaining of nasal obstruction off and on and frequent epistaxis. A reddish mass in the right nasal cavity was observed on endoscopy and treated with endoscopic excision.Biopsy revealed this to be glomangiopericytoma arising from the septum of right nasal cavity, which was excised in toto with endonasal endoscopic approach using diode laser.

[Haemangiopericytoma of the nasal cavity as a cause of recurrent epistaxis--case report]. / Haemangiopericytoma nosa jako przyczyna nawracajacych krwawien--opis przypadku.

A case of haemangiopericytoma of the nasal septum in 73-year-old female admitted to the Department of Otolaryngology of Wroclaw Medical University with a history of recurrent epistaxis and progressive right monolateral nasal respiratory obstruction is reported. On admission presented smooth, cyanotic, easily bleeding lesion in the nasal cavity on the right. Nose CT scans identified presence of a soft-tissue mass arising from the nasal septum. She was treated with a complete surgical resection of the mass. The diagnosis was established based on the careful histopathological examination. Haemangiopericytomas are rare soft-tissue neoplastic lesions, occurring mainly in adults, originating from pericytes and primarily arising in the lower extremities and retroperitoneum. Haemangiopericytomas of head and neck are very unusual and comprise about 15 to 30%; of these, approximately 5% occur in the sinonasal area. Authors have presented etiology, symptomatology, histopathological features, differential diagnoses, diagnostic and therapeutic management. Malignant and benign clinical course have been described.

Tumor-induced hypophosphatemic osteomalacia associated with tertiary hyperparathyroidism: a case report.

BACKGROUND: Tumor-induced hypophosphatemic osteomalacia is a syndrome characterized by urinary phosphate wasting related to the presence of a slowly-growing tumor of mesenchymal origin. The characteristic laboratory findings are normal serum calcium, marked hypophosphatemia, increased serum alkaline phosphatase, markedly reduced renal tubular reabsorption of phosphorus and inappropriately low levels of 1,25-dihydroxyvitamin D [1,25-(OH)2D]. CASE PRESENTATION: A 65-year-old woman presented with a 17-year clinical history of musculoskeletal pain, muscular weakness in the pelvic girdle, spontaneous fractures and difficulty in walking. Over the ensuing years the patient suffered other multiple spontaneous fractures, surgically treated, and the muscular pains worsened until she became bedridden. During the years before hospital admission the patient received treatment with clodronate, oral calcium salts and vitamin D therapy. Standard laboratory, ultrasonography and scintigraphic findings provided a "convenient" diagnosis of primary hyperparathyroidism, but the low plasma level of phosphorus induced to perform an Indium111-octreotide scintigraphy. Scintigraphy visualized an area of pathologic increased signal uptake in the left groin, consistent with a mass containing a high density of somatostatin receptors. After surgery, histologic examination and immunostaining of the resected specimen indicated an hemangiopericytoma. Nevertheless, the persistently low blood phosphorus level, in association with the increased serum calcium and PTH levels, were attributed to the prolonged phosphate therapy the patient underwent over the years, and the persisting abnormal laboratory indexes indicated the development of a tertiary hyperparathyroidism. We performed a subtotal parathyroidectomy and intraoperative assay of serum PTH showed that levels had diminished by more than 80% from preoperative values. Over the ensuing months Ca+2, PTH and serum phosphorus values returned to normal, and the pain symptoms disappeared. CONCLUSIONS: Tumour-induced osteomalacia is a very rare syndrome associated in 5% of cases with tertiary hyperparathyroidism due to long-term therapy with phosphorus and vitamin D. The initial diagnosis of primary hyperparathyroidism, confirmed by the parathyroid MIBI-scintigraphy, would lead us to an inappropriate surgical treatment. Therefore we want to stress the importance of In111-octreotide scintigraphy in detecting tumours, rich in somatostatin receptors, in presence of an hypophosphatemic syndrome.

Intraoperative Intratumoral Embolization of a Complex Recurrent Hemangiopericytoma: Technical Report and Review of the Literature.

Objective Recurrent brain tumors represent a challenge for neurosurgeons because of the extensive blood loss and the time needed for surgical resection. Only a few hemostatic agents are useful to prevent the bleeding and thus facilitate the surgical resection. Fibrin sealant can be used to achieve sealing, tissue adherence, or hemostasis when other means of hemostasis are inadequate or inappropriate. We report the feasibility and positive effects of direct intratumoral injection of fibrin sealant during resection of a recurrent hemangiopericytoma. Material and Methods The intraoperative intratumoral injection of fibrin sealant changed the tumor properties of a recurrent hemangiopericytoma of the tentorium with infra- and supratentorial extension. From a loose friable briskly bleeding tumor, this complex lesion became a nonbleeding well-demarcated soft-firm tumor that could easily be dissected off the pial surface and totally resected without extensive bleeding. Results There are several benefits of intratumoral injection of fibrin sealant in hemangiopericytomas: (1) the extensive bleeding is diminished and blood loss minimized; (2) the restriction of the surgical view by the venous oozing is diminished, making the microsurgical dissection of the tumor capsule off the pial surface easier and safer; (3) the loose consistency of the tumor becomes firmer and facilitates the manipulation of the tumor and leads to a safer resection; and (4) a shorter operating time is needed. Conclusion The use of intratumoral fibrin glue injection is a safe and useful technique that could be used for hemostasis of highly vascularized tumors to facilitate a safer resection and to reduce blood loss.

[Haemangiopericytoma of the parotid gland]. / Haemangiopericytoma slinianki przyusznej.

Haemangiopericytoma is a vascular, soft tissue neoplasm with rare occurrence in the head and neck region. On the basis of literature we discuss etiology, symptomatology, histopathology, diagnosis, treatment and prognosis. We present the case of 66-old woman with haemangiopericytoma in the parotid gland treated by surgery.

Solitary fibrous tumor/hemangiopericytoma of the penis: Report of the first case.

Hemangiopericytoma (HPC) is an uncommon perivascular tumor, first described in 1942, occurring most frequently in the extremities (pelvis, meninges, head and neck), and rarely affecting the urogenital system. In 1870, Wagner published the first histological description of a Solitary Fibrous Tumor (SFT) of the pleura. It is now thought that the majority of lesions previously called hemangiopericytomas (HPCs) are essentially indistinguishable from solitary fibrous tumors (SFTs). Nowadays, the new WHO classification of soft tissue tumors categorizes most HPCs as SFTs. We report the first case of penile SFT-HPC in a 44-year-old man, presenting with a 3-year history of slow-growing penile mass. The patient underwent a tumor excision. Six months after surgery he is free of local recurrence and distant metastasis.