Acta Neurochir (2010) 152:877–880 DOI 10.1007/s00701-009-0502-3
CASE REPORT
Spinal cord compression due to an extra-dural intra-vascular papillary endothelial hyperplasia of the thoracic spine Michele Lanotte & Luca Molinaro & Valentina Crudo & Pier Luigi Filosso & Stefania Greco Crasto & Federico Roncaroli & Paola Cassoni
Received: 24 July 2009 / Accepted: 17 August 2009 / Published online: 9 September 2009 # Springer-Verlag 2009
Abstract We present a case of spinal cord compression in a 33-year-old male due to a T6–T7 paravertebral mass extending through the intervertebral foramen into the vertebral canal. Histopathological feature was consistent with an intra-vascular papillary endothelial hyperplasia. Differential diagnosis of the lesion and review of the literature are presented.
ation of endothelial cells occurring within an organising thrombus. IPEH has been described in a variety of sites, mainly in the skin and subcutaneous soft tissues [4, 6]; only three cases involving the spine have been reported [1, 7, 8].
Keywords Intra-vascular papillary endothelial hyperplasia . IPEH . Spinal cord compression . Paravertebral mass
Clinical summary
Intra-vascular papillary endothelial hyperplasia (IPEH) is a benign, reactive lesion consisting of a florid prolifer-
M. Lanotte (*) Department of Neuroscience, University of Turin, Via Cherasco, 15, 10126 Turin, Italy e-mail:
[email protected] L. Molinaro : V. Crudo : P. Cassoni Department of Biomedical Sciences and Human Oncology, University of Turin, Turin, Italy P. L. Filosso Department of Thoracic Surgery, University of Turin, Turin, Italy S. G. Crasto Division of Radiodiagnostic 2, San Giovanni Battista Hospital, Turin, Italy F. Roncaroli Division of Neuroscience and Mental Health, Imperial College, London, UK
We describe a 33-year-old man who presented with a 2-week history of back pain, hypoesthesia and progressive weakness of both legs and hesitancy in micturition. One month prior to the onset of symptoms, he accidentally fell, causing himself a traumatic injury to the right flank, knee and ankle. No vertebral lesions were detected at X-rays at that time. On present admission, neurological examination showed weakness in the lower limbs, which was more prominent to the right side, left-sided hypoesthesia and hyperesthesia of the right side, with a level at D6–D8. Magnetic resonance imaging (MRI) disclosed a large, well-demarcated and multilobular paravertebral mass of 4.5×2.5×2.5 cm at D6 level causing enlargement of the D6–D7 intervertebral foramen, extending into the vertebral canal and compressing the spinal cord. The lesion was slightly hypointense against the cord parenchyma on the T1-weighted sequences and hyperintense on T2weighted images (Fig. 1). With the aim of relieving cord compression and obtaining tissue for histopathological diagnosis, the patient underwent an urgent D6 laminectomy with excision of the intracanalar portion of the mass. One month later, he underwent a posterolateral thoracotomy to radically resect the
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Fig. 1 Axial (a), sagittal (b) and coronal (c) T2-weighted magnetic resonance images showing a hyperintense extra-dural mass compressing the spinal cord and extending through the intervertebral foramen
to the thoracic cavity. No gadolinium-enhanced images are available as the patient did not consent to the use of contrast agents
intra-thoracic portion of the lesion, which was mostly originating from the paravertebral space. Following this latter operation, his pain and weakness remarkably improved, and he fully recovered 6 months after surgery. The samples from both operations showed similar histopathological features that were consistent with IPEH. The lesion was confined within a vessel and consisted of multiple layers of connective tissue, anastomosing vascular spaces of different calibre and collagenised papillae lined by endothelial cells and projecting within the lumen of vascular structures. Haemorrhagic changes including collections of haemosiderin-laden macrophages were observed (Fig. 2). Immunohistochemical stains with anti-CD31 antibodies (monoclonal antibody QBnd/10, prediluted, Ventana, Tucson, AZ, USA) and anti-CD34 antibodies CD31 (monoclonal antibody JC/70A, prediluted, Ventana) were positive in endothelial cells. Immunostains for CD31 and smooth muscle actin highlighted a second cell population suggestive of pericytes. Immunostains for D240 (monoclonal antibody D240, diluted 1:100, Signet, Dedham, MA, USA), a marker of lymphatic endothelium, and cytokeratins (monoclonal antibody PCK26, prediluted, Ventana) were negative.
Enzinger in 1976 [2] and is currently considered an exuberant organised thrombus. Differential diagnosis includes cavernous and capillary haemangioma, well-differentiated angiosarcoma and retiform and kaposiform haemangioendothelioma. In particular, IPEH can be misdiagnosed as angiosarcoma, but its intra-vascular location, the lack of nuclear pleomorphism in endothelial cells and the absence of mitotic activity are helpful features to differentiate between the two lesions. Whether the present lesion occurred within a paravertebral or an intra-spinal extra-dural vessel cannot be established, although its predominant paravertebral location would suggest an extra- rather intra-spinal origin. Only three cases involving the vertebral canal have been described [1, 7, 8]. Ali et al. [1] reported a 42-year-old man with a T8 epidural mass who developed acute cord compression resulting in sudden paraplegia. Porter et al. [7] presented a spinal cord compression in a 16-year-old boy due to a T6 posterior epidural mass. Taricco et al. [8] described a 17-year-old boy with a cauda equina compression due to a T12–L1 epidural lesion expanded into the neural foramen. In all three cases, the lesions were removed with good recovery of the patients after the surgical treatment. Preoperative diagnosis of spinal IPEH is challenging. Intermediate or hypointense signal on MRI T1-weighted images and variable signal on T2 images reflecting the different stages of thrombus formation [3] along with avid uptake of gadolinium may suggest the diagnosis of IPEH, although these neuroimaging features are not specific and may be seen in peripheral nerve sheath tumours that undergo cystic/haemorrhagic changes. The
Discussion IPEH is a rare lesion first described by Pierre Masson in 1923 and named “hemangioendotheliome vegetant intravasculaire” to describe a tumour-like papillary proliferation of the endothelium within an infected hemorrhoidal vein [5]. This lesion was renamed IPEH by Clearkin and
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Fig. 2 Histological and immunophenotypic features. a Haematoxylin and eosin stain: at ×5 magnification, the lesion appeared well delimited within a blood vessel wall: haematic deposits and collagenised areas can be observed as well. b At higher magnification
(×20), distinctive papillary features can be noticed. c, d Both CD31 and CD34 stains (e, f) were intensively positive, thus confirming the endothelial origin of the cells (c, e ×10; d, ×20 of the c inset; f, ×40)
mainstay of therapy for IPEH compressing the spinal cord is surgical resection; the prognosis is good, and no recurrence after a radical excision has been yet reported.
References
Acknowledgments This paper was supported by grants from the Ricerca Sanitaria Finalizzata Regione Piemonte and MIUR (ex 60%).
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