Multidisciplinary Management of Metastatic Hemangiopericytoma Presenting with Hypoglycemia

Case Report

Ann Hematol Oncol. 2017; 4(3): 1140.

Multidisciplinary Management of Metastatic Hemangiopericytoma Presenting with Hypoglycemia

Hendrick L¹, Angel W², Besh S³, O’Brien T4, Goins N¹ and Dickson P¹*

¹Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, USA

²Memphis Radiological P.C, USA

³Division of Hematology/Oncology, University of Tennessee Health Science Center and West Cancer Center, USA 4Memphis Pathology Group, USA

*Corresponding author: Dickson PV, Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, 910 Madison, Ave. 3rd Floor, Memphis, TN 38163, USA

Received: January 26, 2017; Accepted: February 28, 2017; Published: March 10, 2017


Hemangiopericytomas (HPCs) are rare soft tissue tumors that arise from pericytes surrounding capillaries and venules and can occur throughout the body. We report the case of a 66-year old male with a history of a resected brain tumor that had been originally diagnosed as atypical meningioma. Two years later he presented with refractory hypoglycemia and was found to have multifocal liver metastases with biopsy revealing HPC. Evaluation of his hypoglycemia reveal suppressed insulin, C-peptide, and IGF-I levels and a normal IGF-II level. Biopsy of one of the liver masses revealed morphologic features consistent with HPC and re-review of his brain tumor pathology showed the same histomorphology. His liver metastases were treated with a combination of surgical resection and serial chemoembolization resulting in correction of his hypoglycemia. He has undergone continued serial treatments with chemoembolization with stabilization of his disease. Hepatic metastases from HPC are rare. Hypoglycemia in patients with HPC is likely driven by tumor production of pro-IGF-II peptide, a precursor of IGF-II. Given the paraneoplastic symptoms in these patients, cytoreduction is warranted. A well-coordinated multidisciplinary treatment plan and review of potential therapies is important in treating these unique patients.

Keywords: Hemangiopericytoma; Hypoglycemia; Liver metastases


WHO: World Health Organization; MRI: Magnetic Resonance Imaging; IGF: Insulin like Growth Factor; HPC: Hemangiopericytoma; CT: Computed Tomography; NICTH: Non-insulin-Cell Tumor Hypoglycemia

Case Presentation

A 66-year-old man was initially referred to our institution for progressive left sided weakness and imaging demonstrating a brain mass. His history included pre-referral resection of a brain tumor 5 years prior. Outside surgical and pathology reports were obtained and documented subtotal resection of a large right parafalcine meningioma, WHO grade I, which was hypercellular with Ki-67 index of 18% without histologic features of atypia. MRI at our hospital demonstrated a 3.6x4.2x2.9 cm recurrent tumor with associated compression of adjacent brain parenchyma and mild peritumoral edema. He underwent redo craniotomy with microsurgical resection of the recurrent tumor. Pathology was reported as an atypical meningioma, WHO grade II. He subsequently was treated with postoperative partial brain radiotherapy to a total dose of 54 Gy in 30 fractions.

Two years later he was brought to the emergency department after being found unconscious at home. On evaluation his blood glucose was 30 mg/dl, which initially responded to administration of 1/2 ampoule D50. However, shortly thereafter his blood glucose dropped back to 43 mg/d land he was started on a D10 infusion. On physical exam he became appropriately oriented, had mild but reportedly stable left sided weakness and his abdomen was soft but distended with a large palpable mass in the epigastrium. Evaluation for hypoglycemia revealed a suppressed insulin level of 1.1 milliunit/L (4.2-27.9) and C-peptide of 0.61 ng/ml (0.80-3.80), HbA1C 4.1% (4.0- 6.0), IGF-I 36 ng/ml (43-195), IGF-II 346 ng/ml (267-616), negative sulfonylurea screen, and normal random cortisol level. The IGF-II to IGF-I ratio was calculated at 9.6:1. Brain imaging demonstrated no evidence of locally recurrent tumor, however, abdominal CT and MRI revealed multiple bilobar hepatic masses (Figure 1A). There were no extra hepatic masses identified. Subsequent biopsy of one of the hepatic lesions revealed a neoplasm composed of monotonous cells with interspersed “staghorn” vessels highlighted by CD31 and CD34 immunoperoxidase stains, morphologically consistent with a diagnosis of hemangiopericytoma. Re-review of the brain tumor resection slides demonstrated similar morphologic findings (Figure 2A).