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Table of Contents    
LETTER TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 2  |  Page : 348-349

Straddling across the neural foramina with a leash of blood vessels: Mason's vegetant intravascular hemangioendothelioma mimicking a schwannoma


1 Department of Neurosurgery, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, Punjab and Haryana, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, Punjab and Haryana, India

Date of Web Publication3-Mar-2016

Correspondence Address:
Ankur Kapoor
Department of Neurosurgery, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, Punjab and Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.177619

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How to cite this article:
Singla N, Kapoor A, Sodhi HB, Bal A, Chatterjee D. Straddling across the neural foramina with a leash of blood vessels: Mason's vegetant intravascular hemangioendothelioma mimicking a schwannoma. Neurol India 2016;64:348-9

How to cite this URL:
Singla N, Kapoor A, Sodhi HB, Bal A, Chatterjee D. Straddling across the neural foramina with a leash of blood vessels: Mason's vegetant intravascular hemangioendothelioma mimicking a schwannoma. Neurol India [serial online] 2016 [cited 2020 Nov 28];64:348-9. Available from: https://www.neurologyindia.com/text.asp?2016/64/2/348/177619


Sir,

Among the multiple causes of extramedullary lesions with focal spinal cord compression, a rarely described entity is Masson's vegetant hemangioendothelioma. It is a hyperproliferative non-neoplastic condition that appears histologically as bundles of papillae with one or more stalks attached to the wall of a dilated vascular space. [1],[2],[3] Though central nervous system involvement is less frequent as compared to extremities, a laterally located lesion in the spine can occasionally mimic a spinal schwannoma and cause diagnostic difficulties.

A 40-year-old man presented with complaints of backache and numbness of the right lower trunk for 2 years. Examination was unremarkable except for sensory deficit to touch at L1 dermatome on the right side. Magnetic resonance imaging (MRI) [Figure 1] showed a focal dense contrast-enhancing extramedullary lesion at D12 level at the right neural foramina causing widening of the foramina. The associated hyperintensity of vessels along the anterior aspect was remarkable. With a working diagnosis of schwannoma, the patient was taken up for surgery. Intraoperatively, a firm, vascular extradural lesion was seen well separated from the dura and the nerve root, near the right neural foramina with multiple vascular strands. Total excision was achieved, and the patient had an uneventful recovery. Histopathology [Figure 1] showed a dilated vascular space with organizing thrombi and exuberant reactive endothelial proliferation in the form of papillae. These papillae were lined by a single layer of endothelial cells, showing immunopositivity for CD31 and CD34 and had variable fibrosis and hyalinization, highlighted by Masson's trichrome stain. The patient continued to do well at 3 years of follow-up, and repeat MRI did not show any evidence of recurrence or residual disease.
Figure 1: (a and b) T1-weighted (W) axial plain and contrast MR showing the lesion along the right neural foramina that is hypo- to isointense on T1W, and with a dense contrast enhancement. (c) Sagittal T1W contrast image shows the lesion at D12–L1 level. (d) Low-power photomicrograph showing a large blood vessel containing multiple fibrin thrombi (black arrow) along with multiple delicate papillary structures projecting within the lumen (hematoxylin–eosin (H and E, ×40) (e). Higher magnification shows papillae lined by flattened endothelial cells (H and E, ×100). (f) HECD31 immunostain shows endothelial cells lining the papillae (IP, ×200)

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Masson's hemangioendothelioma, also known as intravascular papillary endothelial hyperplasia (IPEH) and intravascular angiomatosis, was first described by Masson in 1923 in a case of infected hemorrhoidal vein. [1],[2] The disease is considered a vascular proliferative condition that can occur as a pure lesion or focal change in a pre-existing cavernous hemangioma or pyogenic granuloma. Age distribution is wide, female individuals are more commonly affected, and the disease commonly occurs in the extremities, although it has also been reported in the larynx, pelvis, digestive tract, muscles, and liver. The size varies from 0.2–2 cm, and it exists as a sharply demarcated slightly elevated swelling. Histology is characterized by organizing thrombi surrounded by a fibrous pseudocapsule containing smooth muscles or elastic tissue of pre-existing blood vessels. Thrombi of different ages and sizes can be seen. The occasional high cellularity of the endothelium with little stroma can mimic a malignant vascular lesion, though mitotic figures and nuclear atypia are seldom seen. The disease is believed to arise as a result of an exuberant growth phase of a primary organizing thrombus.[1],[2],[4] Positivity for CD31, CD34, or Factor VIII helps in establishing endothelial cell as the cell of origin. The lesion in our patient was small, laterally located at the neural foramina, with a dense contrast enhancement, simulating a schwannoma. Despite thrombosis of vessels, the lesion had enough gap junctions that allowed penetration of contrast leading to its peculiar appearance on MRI. Histologically, differential diagnosis includes angiolymphoid hyperplasia, angiosarcoma, hemangioma, and Kaposi sarcoma.[3]

Although an extremely rare entity within the spinal axis, Masson's vegetant hemangioendothelioma must be considered in the differential diagnosis of contrast-enhancing extramedullary lesions. The dense contrast enhancement and a leash of vessels splayed along the neural foramina seem to be an important radiological finding. The distinction from other similar-appearing neoplastic lesions is imperative because it carries an excellent prognosis after excision and long-term recurrences are unknown.

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Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Porter DG, Martin AJ, Mallucci CL, Makunura CN, Sabin HI. Spinal cord compression due to Masson's vegetant intravascular hemangioendothelioma. Case report. J Neurosurg 1995;82:125-7.  Back to cited text no. 1
    
2.
Taricco MA, Vieira JO Jr, Machado AG, Ito FY. Intravascular papillary endothelial hyperplasia causing cauda equine compression: Case report. Neurosurgery 1994;45:1478-80.  Back to cited text no. 2
    
3.
Lanotte M, Molinaro L, Crudo V, Filosso PL, Crasto SG, Roncaroli F, et al. Spinal cord compression due to an extra-dural intra-vascular papillary endothelial hyperplasia of the thoracic spine. Acta Neurochir (Wien) 2010;152:877-80.  Back to cited text no. 3
    
4.
Ali SZ, Farmer PM, Black K, Rosenthal A. Masson's hemangioma of spinal meninges causing cord compression with paraplegia. Ann Clin Lab Sci 1994;24:371-5.  Back to cited text no. 4
    


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