Resection of an orbital haemangiopericytoma after direct intraoperative injection of 40% N-butyl-2-cyanoacrylate and 60% Lipiodol.

Haemangiopericytoma is a highly vascular tumour, which is a rare soft tissue lesion that may arise anywhere in the body, including the orbit. During its surgical resection, it is too friable for the surgeon to handle and it can bleed severely causing many problems to the surgeon. That is why many surgical approaches have been reported till now, aiming at total excision with minimal blood loss. In this case, total resection of an orbital haemangiopericytoma in a 61-year-old Caucasian woman, using an intraoperative 23G needle injection of 40% n-butyl-2-cyanoacrylate and 60% lipiodol, is presented. The lesion was directly injected under fluoroscopic visualization, after which it became firm enough to be surgically removed without significant bleeding.

Sinonasal hemangiopericytoma caused hypophosphatemic osteomalacia: A case report.

RATIONALE: Tumor-induced osteomalacia (TIO) is a rare, paraneoplastic syndrome featured with fibroblast growth factor 23 (FGF23) secretion primarily by benign mesenchymal tumors and sometimes by malignancies. TIO diagnosis and treatment is often delayed because TIO usually has nonspecific generalized bone pain and weakness, and location of TIO tumor is quite challenging. Very few TIO caused by sinonasal hemangiopericytoma have been reported in the literature. PATIENT CONCERNS: A 40-year-old Chinese woman presented with diffuse bone pain for more than 1 year. Laboratory examination showed hypophosphatemia, hyperphosphaturia, hypocalcemia, an elevated serum alkaline phosphatase (ALP) level and bone-specific ALP level. Imaging studies revealed low bone mineral density (BMD) and multiple pseudofractures at the ribs. F-18 fluorodeoxyglucose positron emission tomography was negative in searching for tumors. Because no tumor was located, the patient was treated with oral phosphate, calcium, and alfacalcidol, and achieved great relief in her symptoms and improvement in BMD. Six years later, the patient had breast cancer surgery and received chemotherapy, and still had hypophosphatemia. During this time, nasopharyngo-fiberscope showed nasal mass in her left nasal cavity. Then she had her nasal polyps removed and surprisingly the serum phosphate became normal. DIAGNOSES AND INTERVENTIONS: The patient had the nasal mass resected, and pathological diagnosis of the nasal mass was sinonasal hemangiopericytoma. Immunohistochemical analysis was positive for FGF23. Thus the final diagnosis was osteomalacia induced by sinonasal hemangiopericytoma. Phosphate supplementation and alfacalcidol were discontinued. OUTCOMES: The patient had normal serum phosphate after 6-month follow-up. LESSONS: By presenting this case, we hope to remind clinicians that in patients with osteomalacia with undetermined reason and intranasal polypoid mass, sinonasal hemangiopericytoma should be suspected.

Tumor-induced osteomalacia: successful treatment by radio-guided tumor surgery.

Tumor-induced osteomalacia is a rare syndrome characterized by urinary phosphate loss with hypophosphatemic osteomalacia. The proposed pathogenetic mechanism is paraneoplastic secretion of phosphaturic factors (so-called phosphatonins).We describe a 34-year-old male patient who presented with severe pain of the spine and ribs for at least 2 years. Bone scintigraphy using Technetium hydroxymethylene diphosphonate (Tc HDP) showed multiple lesions suggesting metastatic disease. Bone biopsy however revealed osteomalacia. The patient had subnormal plasma phosphate levels (0.42 mmol/L; normal range, 0.87-1.45) and markedly increased phosphate clearance (82.8 mL/min; normal range, 5.4-16.2). The patient was treated with phosphate supplementation (up to 5 g daily) along with calcium (1000 mg daily) and calcitriol (1.5 microg daily). Although this therapy did not correct hypophosphatemia, it resulted in complete relief of pain within several months. (111)In pentetreotide scintigraphy showed a tiny lesion of 1-cm diameter, which could be localized to the left femoral neck in close vicinity to the greater trochanter by MRI and image fusion analysis. This lesion had not been visualized by Tc-99m HDP bone scintigraphy. Intraoperatively, use of a hand-held gamma probe after administration of (111)Indium pentetreotide ((111)In pentetreotide) clearly identified the tumor, which was completely removed and was shown to be a hemangiopericytoma. After removal of the tumor, phosphate metabolism normalized within 1 week without requirement of phosphate supplementation. Hypophosphatemic osteomalacia, although rare, raises an important differential diagnosis. An underlying tumor may be detected only by (111)In pentetreotide scintigraphy. Preoperative labeling with (111)In pentetreotide is a useful tool in detecting these tumors during surgery.This 34 year old man with osteomalacia had a small causative hemangiopericytoma detected in the indium pentetreotide scintography.

[Successful treatment of a huge meningeal hemangiopericytoma using Preoperative Autologous Transfusion and Hemodilutional Autologous Transfusion: case report].

We report successful operations for a meningeal hemangiopericytoma using sufficient amounts of Preoperative Autologous Transfusion (PAT) and Hemodilutional Autologous Transfusion (HAT). A 23-year-old woman with amenorrhea and bilateral visual field disturbance was found to have a huge intracranial tumor. MRI showed a well-enhanced cystic mass in the left middle fossa, suprasellar, intrasellar, sphenoidal sinus, and cavernous sinus. Preoperatively, the tumor was thought to be a cystic pituitary tumor or meningioma. Surgical removal was planned in three steps. The first operation was carried out via the transsphenoidal approach. Total blood loss was 1348 ml and 2 MAP infusion were required to control bleeding. Histopathological diagnosis was hemangiopericytoma. After preparation of PAT 400 ml and HAT 800 ml, we carried out the second partial removal operation mainly via the interhemispheric approach. Total blood loss was 1829 ml and required autologous transfusion only. After preparation of PAT 1200 ml and HAT 400 ml, the last total removal operation was carried out mainly via the pterional and subtemporal approach. Total blood loss was 1813 ml and required autologous transfusion only. We needed 2 MAP infusion in the first operation, but were able to perform total removal successfully without homologous blood transfusion because a sufficient amount of PAT and HAT had been prepared preoperatively. Hemangiopericytoma required postoperative radiation therapy to avoid local recurrence. After successful removal of the tumor surgically, postoperative radiation therapy was able to be carried out efficiently.

Glomangiopericytoma causing oncogenic osteomalacia. A case report with immunohistochemical analysis.

A 47-year-old woman suffered from gait disturbance due to back pain and muscle weakness. Laboratory data showed serum hypophosphatemia, elevated alkaline phosphatase, and a normal level of ionized calcium. Radiological examinations revealed multiple pathologic fractures in the ribs and pubic rami. She had had no episode of familial or any other notable disorder, and so she was initially treated with medication for adult-onset osteomalacia. However, 19 years later (when she was 66 years old), she noticed a soft-tissue tumor in her buttock. The tumor was excised. The histological features were those of glomangiopericytoma characterized by both glomus tumor-like and hemangiopericytoma-like structures. After removal of the tumor, her symptoms disappeared immediately. Laboratory data normalized 8 months later. To our knowledge, this is the first report of oncogenic osteomalacia caused by glomangiopericytoma.

Craniofacial hemangiopericytoma associated with oncogenic osteomalacia: case report.

A craniofacial hemangiopericytoma associated with oncogenic osteomalacia is described and the literature is reviewed. A 46 year-old male with multiple fractures and hypophosphatemia was found to have a craniofacial mass extending from the right ethmoid sinus into the right frontal lobe. Initial detection of the tumor was made with an 111Indium-pentreotide scan (Octreoscan). Gross total resection of the tumor was achieved and the patient received postoperative radiation therapy. One year after surgery, the patient remains free of tumor with significant increase in bone density and normal phosphate levels. This is the first report of a hemangiopericytoma invading the brain that was associated with paraneoplastic hypophosphatemia and osteomalacia. Also, this is the first reported detection of a hemangiopericytoma by an Octreoscan. Primary detection and secondary surveillance of hemangiopericytomas may be possible with serial Octreoscans.

Diagnostic utility of magnetic resonance imaging skeletal survey in a patient with oncogenic osteomalacia.

Oncogenic osteomalacia is a rare paraneoplastic syndrome characterized by hypophosphatemic osteomalacia due to renal phosphate wasting. The same biochemical features are found in patients with X-linked hypophosphatemic rickets/osteomalacia and sporadic hypophosphatemic osteomalacia with unknown etiology. Oncogenic osteomalacia is cured by resection of the responsible tumor. In contrast, patients with other types of hypophosphatemic rickets/osteomalacia need long-term treatment with large doses of active vitamin D3. Therefore, detection of the responsible tumor for oncogenic osteomalacia has great clinical importance. However, there is no standard method for detecting the tumor for oncogenic osteomalacia, and the responsible tumor is often very difficult to be found. We describe a patient with adult-onset osteomalacia due to renal phosphate wasting. Although oncogenic osteomalacia was suspected, cranial, chest, and abdominal computed tomography scanning, urological and otolaryngological examinations, and detailed palpation for soft tissue mass failed to detect the responsible tumor. However, magnetic resonance imaging skeletal survey revealed a tumor in the right femoral bone. Resection of the tumor resulted in normalization of serum phosphate and renal phosphate handling. Because the most frequent causes for oncogenic osteomalacia are tumors in bone or soft tissue, magnetic resonance imaging skeletal survey is a very powerful method for detecting the responsible tumor. Vigorous search for tumors with this method in patients with hypophosphatemic osteomalacia would be helpful not only for proper management of patients, but also for clarifying the identity of sporadic hypophosphatemic osteomalacia.

Surgically curable hypophosphatemic rickets. Diagnosis and management.

Childhood hypophosphatemic rickets (HR) is most often caused by a defect in renal tubular resorption of filtered phosphorus. However, HR can also be caused by secretion of a phosphaturetic factor from a tumor. The presentation of patients with the different HR syndromes may be identical. Distinguishing between the HR syndromes is essential, however, because HR caused by renal defect requires life-long therapy with Vitamin D and phosphate replacement, but tumor-associated HR is cured by removal of the tumor. A case of hemangiopericytoma occurring in bone and causing HR is reported. Children with HR typically have normal levels of serum calcium and parathyroid hormone but very low levels of serum phosphorus. In a child with HR, the following features should prompt a thorough evaluation for a causative tumor: lack of other family members who have hypophosphatemia; presence of aminoaciduria, particularly glycinuria. Causative lesions are most commonly found in the bone or skin.

[Oncogenic hypophosphatemic osteomalacia]. / Onkogene hypophosphatätmische Osteomalazie.

In the courses of six years a severe hypophosphataemic osteomalacia, painful motor impairment and multiple rib fractures developed in a 51-year-old man. The symptoms gradually improved within one year under treatment with 3 micrograms daily of 1,25-dihydroxycholecalciferol, 3 g phosphorus and 3 g calcium, and biochemical parameters and the bone scintigram became normal. Ultimately, computed tomography, scintigraphy and digital subtraction angiography revealed a highly vascularized tumour in the condylar aspect of the right femur, and it was chiselled out. Histologically it was a mesenchymal phosphaturic tumour of haemangiopericytoma type of questionable benignity. After the operation the patient was symptom-free for some weeks without any drug treatment, but the latter was then resumed because of renewed bone pain. By now, two years later, he is essentially without pain and has full mobility. However, repeat scintigraphy and angiography revealed renewed tumour growth in the right femoral condyle.

Hemangiopericytoma of the colon: report of a case and review of literature.

A patient with a hemangiopericytoma of the colon is discussed. This is the second such case reported in the English medical literature. Soon after discovery of the tumor, the patient presented with a colonic intussusception with the tumor serving as the lead point. This was reduced by a hypaque enema, but the intussusception recurred twice more, being reduced again by hypaque enema and finally having to be reduced by colonoscopy. At surgery a left hemicolectomy with primary anastomosis was performed. The microscopic, ultrastructural, and pathologic aspects of hemangiopericytoma are discussed with special attention to lesions of the gastrointestinal tract.

Preoperative vascular embolization as an adjunct to successful resection of large retroperitoneal hemangiopericytoma.

A large retroperitoneal hemangiopericytoma was resected successfully with the aid of preoperative control of the blood supply of the lesion with percutaneous intra-arterial gelfoam and ferromagnetic silicone embolization. We believe that this resection would not have been possible without this adjuvant technique. Future application of this combined technique will enable more aggressive surgical intervention in unresectable vascular tumors and arteriovenous malformations.