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Haemangiopericytoma diagnosed from a metastasis 11years after surgery for "atypical meningioma"

¹Department of Radiotherapy, Singleton Hospital and ²Department of Pathology, Morriston Hospital, Swansea, UK

Correspondence: Dr B R Prakasha, Department of Radiotherapy, Velindre Hospital, Whitchurch, Cardiff CF14 2TL

	Abstract

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Meningeal haemangiopericytoma (HPC) is a rare tumour often mistakenly reported as "vascular meningioma". Unlike meningiomas, HPC has a high rate of local recurrence and distant metastases, which may occur several years after initial treatment. We report a patient in whom a HPC was misdiagnosed as benign vascular meningioma and the patient discharged from follow-up. HPC was diagnosed 11 years later from biopsy of a skeletal metastasis. Histological review of the meningeal tumour confirmed the diagnosis of meningeal HPC. Meningeal HPCs resemble meningiomas clinically, radiologically and even light microscopically. As a result, they can be reported as atypical meningioma, as in this case. HPC's are more aggressive than typical meningiomas, with a high rate of recurrence and distant metastasis, often late in the course of the disease. Management of meningeal HPC differs from that of typical meningioma, with a need for post-operative radiotherapy and long-term follow-up.

	Introduction

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Meningeal haemangiopericytoma (HPC) is a distinct class of meningeal tumour with an aggressive natural history. HPCs resemble typical meningiomas clinically, radiologically and even light microscopically, and hence are sometimes reported as "atypical meningiomas". However, HPC has a high rate of local recurrence and distant metastasis, often late in the course of the disease. We report a patient with metastatic skeletal HPC 11 years after surgery for what was considered to be a vascular meningioma.

	Case report

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A 49-year-old male was admitted to hospital in April 1997 with pain in the right hip, paraesthesiae, weakness of the right leg and numbness of the lateral border of the right foot. Clinical examination showed a previous craniotomy scar in the left parietotemporal area. His right leg was held in flexion and hip movements were painful. The deep tendon reflexes were sluggish bilaterally and there was sensory impairment along the L5 dermatome in the right leg.

11 years prior to presentation the patient had undergone craniotomy and complete excision of a tumour arising from the lateral sphenoid wing on the left side. On histological examination, the tumour had been reported as a highly cellular, vascular meningioma with no features of malignancy. He had no post-operative treatment, was followed up for 5 years and then discharged.

Plain radiographs of the right hip performed at the current presentation showed a pathological fracture of the proximal femur. MRI demonstrated a destructive bony lesion of the L5 vertebral body involving the posterior two-thirds of the body, with direct invasion of the spinal canal, resulting in severe compromise of the theca and the nerve roots at that level (Figure 1). There was also an expansile lytic lesion involving the subtrochantric and intertrochantric area of the right proximal femur. Radionuclide bone scan showed increased uptake in the proximal right femur but not in the L5 vertebral body.

View larger version (109K): [in this window] [in a new window]   Figure 1. MRI of the lumbar spine showing collapse of the L5 vertebral body and stenosis of the spinal canal, with cauda equina compression.

Biopsy from the intertrochantric area of the femur showed a solid and richly vascular tumour. The tumour was composed of small spindle cells with indistinct fibrillary cytoplasm, arranged in a storiform pattern with the cells individually enmeshed in a dense reticulin network (Figure 2). On immunohistochemistry, the tumour was negative for vimentin, broad spectrum cytokeratin, smooth muscle actin, glial fibrillary acid protein and S-100 protein. A diagnosis of HPC was made on the excised specimen. The histology slides of the original tumour removed from the sphenoid were reviewed and a revised diagnosis of meningeal HPC was made.

View larger version (168K): [in this window] [in a new window]   Figure 2. Photomicrograph of the excised tumour from the femoral trochanter (H&E stain, x 20 objective).

The patient is alive at the time of reporting, with asymptomatic bony metastases but no intracranial disease.

	Discussion

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The term haemangiopericytoma was coined by Stout and Murray [1] to describe a vascular neoplasm arising from pericapillary cells or Zimmerman's pericytes. These tumours arise most commonly in the lower extremities, pelvis and retroperitoneum, but can develop from capillaries at any site. Pericytes lack readily identifiable cellular features. The diagnosis of HPC rests on recognition of the architectural pattern and on immunohistochemistry.

Meningeal HPC, which constitutes 2% of meningeal tumours, was first described in 1928 by Bailey et al [2], and was considered an angioblastic variant of meningioma. Immunohistochemical, ultrastructural and genetic studies subsequently showed HPC to be distinct from other variants of meningioma [3]. Meningeal HPC was reclassified under "sarcomas" in the World Health Organization classification of tumours of the central nervous system [4]. HPC is typically cellular and composed of small oval cells, with numerous branching thin walled vessels of varying calibre, giving a characteristic "staghorn" vascular pattern [3]. Reticulin stain may be helpful in showing individual cell enmeshing. Lack of epithelial membrane antigen and S-100 protein in the presence of positive staining for vimentin, factor 13-a and leu-7 has been considered sufficient to distinguish meningeal HPC from other histological mimics, including fibrous meningiomas [3].

Meningeal HPCs are locally aggressive tumours with a high rate of local recurrence and distant metastasis. Mena et al [5], in their review of 94 cases, reported a recurrence rate of 70% and a metastasis rate of 27%. Bone, lung and liver are common sites of metastases, but there are reports of metastases at other sites [5]. Although metastases typically occur at 63–99 months from diagnosis, they can occasionally be delayed until 20 years after diagnosis [6]. Bone metastases manifest as osteolytic lesions on plain radiographs. The role of radionuclide bone scan is limited, as metastases may not take up the isotope [7], as in one of the lesions in this case. Local recurrence tends to pre-date the onset of distant metastasis in the majority of cases [8].

Surgery is currently regarded as the main modality of treatment for meningeal HPC, and pre-operative tumour embolisation has been reported to be useful in reducing the vascularity [9]. Local recurrences are common even after complete excision, with up to 50% recurrence rates reported by McMaster and Stout [10]. Post-operative radiotherapy appears to reduce the incidence of local recurrence. Guthrie et al [11] in their series of 44 patients treated with post-operative radiotherapy recorded a significantly longer recurrence-free interval and longer survival. Conservative surgery and post-operative radiotherapy has been suggested, especially for tumours in unfavourable locations [12]. A dose of 50–60 Gy is thought to be adequate to reduce the rate of local recurrence [13].

Chemotherapy has generally been disappointing in the management of metastatic disease [13]. In the series reported by Galanis et al [14], only one patient out of seven showed a partial response to doxorubicin. There are, however, isolated case reports of tumour control using a combination of ifosfamide and epirubicin [15].

From current evidence, meningeal HPC is best treated by surgical excision. Post-operative radiotherapy is recommended to improve local control, particularly in patients where complete surgical clearance of tumour is not possible owing to the unfavourable location of the tumour. Patients need prolonged follow-up, as distant metastases are generally a late phenomenon. These tumours are sometimes misdiagnosed as atypical meningiomas, but an accurate histological diagnosis of meningeal HPC is important at the time of initial presentation, as these tumours have a different natural history to atypical meningiomas and should be managed accordingly.

	Acknowledgments

 
We thank Dr Robin Reid of the University of Glasgow for his confirmatory second opinion on the histology.

Received for publication January 15, 2001. Revision received March 28, 2001. Accepted for publication April 17, 2001.

	References

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