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Epithelioid hemangioma of occipital condyle and clivus: Unusual location of a rare bony tumor with presentation in perigestational period
Clinical Neurology and Neurosurgery 115 (2013) 1137–1140
Contents lists available at SciVerse ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Epithelioid hemangioma of occipital condyle and clivus: Unusual location of a rare bony tumor with presentation in perigestational period Narayanam Anantha Sai Kiran a,∗ , Nandita Ghosal b , Sunil Valentine Furtado a , Sandhya Ramachandran c , Alangar S. Hegde a a
Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Bangalore, India Department of Neuropathology, Sri Sathya Sai Institute of Higher Medical Sciences, Bangalore, India c Sri Sathya Sai Institute of Higher Medical Sciences, Prasanthigram, India b
a r t i c l e
i n f o
Article history: Received 18 March 2012 Received in revised form 18 August 2012 Accepted 3 September 2012 Available online 20 September 2012 Keywords: Angiolymphoid hyperplasia Epithelioid hemangioma Far lateral approach Occipital condyle Pregnancy
1. Introduction Epithelioid hemangioma (EH) usually involves skin and rarely involves bone [1–3]. EH is usually considered as a benign lesion though few consider it as a locally aggressive lesion with a potential for local recurrence and lymph node metastasis [2,3]. Very few cases of perigestational presentation of osseous EH have been reported [1,4]. EH involving cranial bones is rare and involvement of occipital condyle and clivus is extremely uncommon [2,5]. Authors describe this unusual location of EH with presentation in perigestational period. 2. Case report
of tongue to the left side suggestive of left XII nerve palsy. Rest of the neurological and systemic examination was normal. Examination of the neck was normal without any deformity, torticollis, or muscle wasting. Range of neck movements was normal. Neck movements were associated with severe pain. 2.2. Imaging features CT scan (Fig. 1a–d) revealed an expansile bony lytic lesion destroying most of the left occipital condyle. MR imaging (Fig. 1e and f) revealed a diffuse lesion involving left occipital condyle with supero medial extension to the left side of clivus. Lesion was isointense on T1 and T2 weighted images with enhancement on contrast.
2.1. History and examination 2.3. Operation and postoperative course A 21-year old lactating female presented with history of suboccipital neck pain and wasting of the left side of tongue for past 3 months with symptoms starting at 8 months of gestation. Examination revealed wasting of left side of the tongue along with deviation
∗ Corresponding author at: Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Whitefield, Bangalore 560066, India. Tel.: +91 08904779819; fax: +91 8411503. E-mail address: [email protected] (N.A. Sai Kiran). 0303-8467/$ – see front matter © 2012 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.clineuro.2012.09.003
Lesion was excised by left far lateral approach. Lesion was completely extradural, destroying most of the left occipital condyle with anterior extension to the clival region (Fig. 2). Condylar part was completely excised and the clival part was partially excised. The anterior and superior part of the lesion involving clivus which could not be reached by this approach was left over. Bone dust collected during craniectomy was used to fill the cavity in the bone after tumor excision. Later occipitocervical fusion was done as the lesion was almost completely destroying left occipital condyle. Occiput to
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Fig. 1. CT scan coronal (a) and axial (b–d) sections showing an expansile lytic bony lesion destroying most of the left occipital condyle (solid white arrows in a and b) with extension to left side of clivus (broken arrow in d). (e) Contrast MR T1 weighted images coronal section showing a diffuse enhancing lesion involving left occipital condyle (arrow in e). (f) Axial T2 weighted MR image showing isointense lesion.
C1–C2 fusion was done with contoured titanium rod, sub laminar cables and iliac crest graft. Post operative course was uneventful. Post operative imaging revealed complete excision of the condylar part with small residual clival component (Fig. 3). Patient at 3 months follow-up was asymptomatic except for persisting XII nerve palsy. Patient was advised regular follow-up with repeat imaging after 1 year to look for any increase in the size of the residual lesion. 2.4. Histopathology Histopathological examination (Fig. 4) revealed fragments of bone with highly vascular lesion with numerous closely packed capillary sized channels lined by plump endothelial cells with epithelioid morphology separated by stroma rich in eosinophils with admixed lymphocytes and histiocytes. Smaller vascular channels showed slit like lumina. A few large vessels showed distinct, plump endothelial cells with hobnailing giving it a tomb stone appearance. Thicker vessels with hyperplastic medial coat were seen, intersecting the tumor. No hyalinized or myxoid stroma was
seen. Immunohistochemistry for CD31 and vimentin were focally positive (Fig. 5). Overall features were suggestive of epithelioid hemangioma with eosinophilia. 3. Discussion Epithelioid hemangioma previously known as angiolymphoid hyperplasia (ALH) with eosinophilia and histiocytoid hemangioma commonly involves skin and subcutaneous tissue of face and scalp. Other areas of involvement of EH being orbit, lacrimal gland, colon, oral mucosa, etc. EH can rarely involve bones [3]. Bones commonly involved by EH include long/short tubular bones of the distal lower extremity, flat bones, vertebrae and small bones of the hands [2]. Involvement of cranial bones is extremely uncommon and only one case of EH involving occipital condyle has been reported [5]. Osseous EH usually present as contrast enhancing expansile lesion with bone destruction as seen in the present case [2]. EHs are generally considered as benign lesions. Majority of EHs present as solitary lesions [2]. Few (18%) of them present as multifocal lesions [2]. Nielsen et al. [2] reported the aggressive
Fig. 2. Intraoperative images. (a) Intradural – initial intradural exploration did not reveal any intradural lesion. Dura (white arrow) inferior to jugular foramen was elevated suggestive of underlying tumor. J – jugular foramen, L – lower cranial nerves, S – spinal portion of XI nerve, B – brain stem, C – cerebellum, DF – dural flap reflected laterally, P – loop of posterior inferior cerebellar artery. (b) – Extradural – dural flap (DF – black arrow) was reflected medially and lesion excised extradurally. Lesion (white arrow) was highly vascular destroying the occipital condyle completely.
N.A. Sai Kiran et al. / Clinical Neurology and Neurosurgery 115 (2013) 1137–1140
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Fig. 3. (a–d) Postoperative CT scan coronal (a) and axial (b–d) showing complete excision of condylar part (solid white arrows in a and b) with bone dust in the operative cavity and residual clival lesion (broken white arrow in d). (e) X-ray CVJ lateral view showing occipitocervical fusion with contoured rod and sublaminar wires. (f–h) – Follow-up CT scan (f and g) and contrast MR imaging (h) 3 months after surgery showing no residual lesion in the region of condyle and a small residual in the region of clivus (broken white arrow in h).
Fig. 4. Photomicrograph of the lesion. (a) Paraffin section showing predominantly capillary sized vessels. (b) Numerous eosinophils (straight arrow) in the stroma separating the vascular channels. (c) Few vessels showing prominent endothelial cells with hob nailing (curved arrow) [hematoxylin and eosin: (a) and (b) 100×; (c) 400×].
nature of these lesions with potential for local recurrence (8%) and lymph node metastasis (2%). Verbeke and Bovée [3] consider EH as intermediate (locally aggressive, rarely metastasizing) grade lesion in their classification of primary vascular tumors of bone into benign, intermediate and malignant tumors. Patients with these lesions require a close follow-up after surgical
excision in view of their potential for local recurrence and lymph node metastasis. EHs must be differentiated from malignant lesions like hemangioendothelioma and angiosarcoma. EH is characterized by lobular growth pattern of capillary sized channels lined by plump endothelial cells with epithelioid morphology separated by stroma rich
Fig. 5. Immunohistochemistry showing (a) vimentin positivity in the vascular and stromal component. (b) CD31 positivity in the endothelial cells (curved arrow) [avidin biotin complex immunoperoxidase method: (a) and (b) 400×].
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in eosinophils with admixed lymphocytes and histiocytes as seen in the present case [2,3]. Myxoid and hyalinized stroma characteristic of epithelioid hemangioendothelioma is absent in these lesions [2,3]. Atypia, atypical mitosis and solid growth pattern characteristic of angiosarcoma is not seen in EH [3]. The recurrent t(1;3)(p36;q25) chromosomal translocation, resulting in WWTR1-CAMTA1 fusion, is identified as a consistent abnormality in epithelioid hemangioendothelioma but not in EH or other epithelioid vascular neoplasms like epithelioid angiosarcomas [6]. Molecular analysis for the presence of WWTR1-CAMTA1 fusion may serve as a useful diagnostic tool in challenging cases of epithelioid vascular tumors with morphological overlap. Common occurrence of vascular neoplasia in women and their tendency for rapid growth during pregnancy are suggestive of possible hormonal influence on these lesions. Various studies demonstrated the presence of estrogen receptors in these lesions. Deyrup et al. [7] in their immunohistochemical evaluation of 53 vascular neoplastic lesions reported the presence of estrogen receptor beta in all the cases. Spontaneous remission of a multifocal EH presenting in perigestational period reported by Lewis et al. [4] and few other reported cases of osseous EHs presenting in perigestational period suggest a gestation related proliferation stimulus on these lesions [1]. Presentation in perigestational period in the present case strongly suggests the influence of hormones on the proliferation of these lesions. Complete excision, whenever feasible is the treatment of choice for EH [2]. Present case was a diffuse lesion involving most of the occipital condyle with extension to clivus. Complete excision of such extensive lesion by single approach is difficult [8]. Various options in such cases include extended (expanded) endoscopic endonasal approach, far lateral approach, extensive skull base approach with petrosectomy and mastoidectomy [5,8]. Though extended endonasal endoscopic approach is minimally invasive it has various limitations. By endoscopic approach alone, it is difficult to achieve gross total excision of the lesions which are predominantly centered at lower clivus with significant lateral extension to occipital condyle as seen in the present case [8]. Far lateral approach offers better degree of tumor exposure and removal in such cases with significant lateral extension to occipital condyle. Dehdashti et al. [8] combined far lateral approach with extended endoscopic endonasal approach in such extensive lesions. Occipito cervical fusion was required in the present case as the occipital condyle was almost completely destroyed by the lesion. We preferred far lateral approach as it offers better exposure for the condylar extension of the tumor along with exposure for performing occipito cervical fusion. Further excision of the residual clival component in the present case requires an extensive skull base approach with petrosectomy and mastoidectomy or an extended endonasal endoscopic approach. Further excision of the residual lesion was not attempted in view of the benign nature of this lesion, proximity of the residual highly vascular firm lesion to the petrous segment of internal carotid artery (Fig. 1f), and morbidity associated with extensive skull base approaches. Experience with radiotherapy (RT) in the management of these cases is limited and only very few cases of bony EH were given
RT. Lewis et al. [4] reported spontaneous clinical and radiological remission in a patient with multifocal EH of bone with presentation in perigestational period and advised close observation rather than radiotherapy for the management of EH. In view of limited experience with RT in the management of these lesions and indolent course in majority of these lesions we did not give RT in the present case. Regular follow-up was advised to look for any increase in the size of residual lesion. 4. Conclusion Epithelioid hemangioma is a rare bony tumor and its involvement of occipital condyle and clivus is extremely uncommon. Presentation in perigestational period in the present case strongly suggests the influence of hormones on the proliferation of these vascular lesions. Patients with these lesions require a close followup after surgical excision in view of their potential for local recurrence. Source of support The authors have not received any support, in the form of grant, from any source for preparation of this article. Neither do the authors, nor does the institute have any personal or institutional financial interest in drugs, materials, or devices described in their submissions. Disclosure This paper has not been published before and has not been submitted for publication to any other journal in part or full. The authors concur with this submission and there is no conflict of interest arising from this article. References [1] Lamovec J, Bracko M. Epithelioid hemangioma of small tubular bones: a report of three cases, two of them associated with pregnancy. Modern Pathology 1996;9:821–7. [2] Nielsen GP, Srivastava A, Kattapuram S, Deshpande V, O’Connell JX, Mangham CD, et al. Epithelioid hemangioma of bone revisited: a study of 50 cases. American Journal of Surgical Pathology 2009;33:270–7. [3] Verbeke SL, Bovée JV. Primary vascular tumors of bone: a spectrum of entities? International Journal of Clinical and Experimental Pathology 2011;4: 541–51. [4] Lewis VO, Montag AG, Simon MA. Epithelioid hemangiomas of bone: spontaneous clinical and radiographic remission. Clinical Orthopaedics and Related Research 2003;407:167–72. [5] Mazzoni A. The petro-occipital trans-sigmoid approach for lesions of the jugular foramen. Skull Base 2009;19:48–56. [6] Errani C, Zhang L, Sung YS, Hajdu M, Singer S, Maki RG, et al. A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites. Genes, Chromosomes and Cancer 2011;50:644–53. [7] Deyrup AT, Tretiakova M, Khramtsov A, Montag AG. Estrogen receptor b expression in vascular neoplasia: an analysis of 53 benign and malignant cases. Modern Pathology 2004;17:1372–7. [8] Dehdashti AR, Karabatsou K, Ganna A, Witterick I, Gentili F. Expanded endoscopic endonasal approach for treatment of clival chordomas: early results in 12 patients. Neurosurgery 2008;63:299–309.
Contents lists available at SciVerse ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Epithelioid hemangioma of occipital condyle and clivus: Unusual location of a rare bony tumor with presentation in perigestational period Narayanam Anantha Sai Kiran a,∗ , Nandita Ghosal b , Sunil Valentine Furtado a , Sandhya Ramachandran c , Alangar S. Hegde a a
Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Bangalore, India Department of Neuropathology, Sri Sathya Sai Institute of Higher Medical Sciences, Bangalore, India c Sri Sathya Sai Institute of Higher Medical Sciences, Prasanthigram, India b
a r t i c l e
i n f o
Article history: Received 18 March 2012 Received in revised form 18 August 2012 Accepted 3 September 2012 Available online 20 September 2012 Keywords: Angiolymphoid hyperplasia Epithelioid hemangioma Far lateral approach Occipital condyle Pregnancy
1. Introduction Epithelioid hemangioma (EH) usually involves skin and rarely involves bone [1–3]. EH is usually considered as a benign lesion though few consider it as a locally aggressive lesion with a potential for local recurrence and lymph node metastasis [2,3]. Very few cases of perigestational presentation of osseous EH have been reported [1,4]. EH involving cranial bones is rare and involvement of occipital condyle and clivus is extremely uncommon [2,5]. Authors describe this unusual location of EH with presentation in perigestational period. 2. Case report
of tongue to the left side suggestive of left XII nerve palsy. Rest of the neurological and systemic examination was normal. Examination of the neck was normal without any deformity, torticollis, or muscle wasting. Range of neck movements was normal. Neck movements were associated with severe pain. 2.2. Imaging features CT scan (Fig. 1a–d) revealed an expansile bony lytic lesion destroying most of the left occipital condyle. MR imaging (Fig. 1e and f) revealed a diffuse lesion involving left occipital condyle with supero medial extension to the left side of clivus. Lesion was isointense on T1 and T2 weighted images with enhancement on contrast.
2.1. History and examination 2.3. Operation and postoperative course A 21-year old lactating female presented with history of suboccipital neck pain and wasting of the left side of tongue for past 3 months with symptoms starting at 8 months of gestation. Examination revealed wasting of left side of the tongue along with deviation
∗ Corresponding author at: Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Whitefield, Bangalore 560066, India. Tel.: +91 08904779819; fax: +91 8411503. E-mail address: [email protected] (N.A. Sai Kiran). 0303-8467/$ – see front matter © 2012 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.clineuro.2012.09.003
Lesion was excised by left far lateral approach. Lesion was completely extradural, destroying most of the left occipital condyle with anterior extension to the clival region (Fig. 2). Condylar part was completely excised and the clival part was partially excised. The anterior and superior part of the lesion involving clivus which could not be reached by this approach was left over. Bone dust collected during craniectomy was used to fill the cavity in the bone after tumor excision. Later occipitocervical fusion was done as the lesion was almost completely destroying left occipital condyle. Occiput to
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Fig. 1. CT scan coronal (a) and axial (b–d) sections showing an expansile lytic bony lesion destroying most of the left occipital condyle (solid white arrows in a and b) with extension to left side of clivus (broken arrow in d). (e) Contrast MR T1 weighted images coronal section showing a diffuse enhancing lesion involving left occipital condyle (arrow in e). (f) Axial T2 weighted MR image showing isointense lesion.
C1–C2 fusion was done with contoured titanium rod, sub laminar cables and iliac crest graft. Post operative course was uneventful. Post operative imaging revealed complete excision of the condylar part with small residual clival component (Fig. 3). Patient at 3 months follow-up was asymptomatic except for persisting XII nerve palsy. Patient was advised regular follow-up with repeat imaging after 1 year to look for any increase in the size of the residual lesion. 2.4. Histopathology Histopathological examination (Fig. 4) revealed fragments of bone with highly vascular lesion with numerous closely packed capillary sized channels lined by plump endothelial cells with epithelioid morphology separated by stroma rich in eosinophils with admixed lymphocytes and histiocytes. Smaller vascular channels showed slit like lumina. A few large vessels showed distinct, plump endothelial cells with hobnailing giving it a tomb stone appearance. Thicker vessels with hyperplastic medial coat were seen, intersecting the tumor. No hyalinized or myxoid stroma was
seen. Immunohistochemistry for CD31 and vimentin were focally positive (Fig. 5). Overall features were suggestive of epithelioid hemangioma with eosinophilia. 3. Discussion Epithelioid hemangioma previously known as angiolymphoid hyperplasia (ALH) with eosinophilia and histiocytoid hemangioma commonly involves skin and subcutaneous tissue of face and scalp. Other areas of involvement of EH being orbit, lacrimal gland, colon, oral mucosa, etc. EH can rarely involve bones [3]. Bones commonly involved by EH include long/short tubular bones of the distal lower extremity, flat bones, vertebrae and small bones of the hands [2]. Involvement of cranial bones is extremely uncommon and only one case of EH involving occipital condyle has been reported [5]. Osseous EH usually present as contrast enhancing expansile lesion with bone destruction as seen in the present case [2]. EHs are generally considered as benign lesions. Majority of EHs present as solitary lesions [2]. Few (18%) of them present as multifocal lesions [2]. Nielsen et al. [2] reported the aggressive
Fig. 2. Intraoperative images. (a) Intradural – initial intradural exploration did not reveal any intradural lesion. Dura (white arrow) inferior to jugular foramen was elevated suggestive of underlying tumor. J – jugular foramen, L – lower cranial nerves, S – spinal portion of XI nerve, B – brain stem, C – cerebellum, DF – dural flap reflected laterally, P – loop of posterior inferior cerebellar artery. (b) – Extradural – dural flap (DF – black arrow) was reflected medially and lesion excised extradurally. Lesion (white arrow) was highly vascular destroying the occipital condyle completely.
N.A. Sai Kiran et al. / Clinical Neurology and Neurosurgery 115 (2013) 1137–1140
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Fig. 3. (a–d) Postoperative CT scan coronal (a) and axial (b–d) showing complete excision of condylar part (solid white arrows in a and b) with bone dust in the operative cavity and residual clival lesion (broken white arrow in d). (e) X-ray CVJ lateral view showing occipitocervical fusion with contoured rod and sublaminar wires. (f–h) – Follow-up CT scan (f and g) and contrast MR imaging (h) 3 months after surgery showing no residual lesion in the region of condyle and a small residual in the region of clivus (broken white arrow in h).
Fig. 4. Photomicrograph of the lesion. (a) Paraffin section showing predominantly capillary sized vessels. (b) Numerous eosinophils (straight arrow) in the stroma separating the vascular channels. (c) Few vessels showing prominent endothelial cells with hob nailing (curved arrow) [hematoxylin and eosin: (a) and (b) 100×; (c) 400×].
nature of these lesions with potential for local recurrence (8%) and lymph node metastasis (2%). Verbeke and Bovée [3] consider EH as intermediate (locally aggressive, rarely metastasizing) grade lesion in their classification of primary vascular tumors of bone into benign, intermediate and malignant tumors. Patients with these lesions require a close follow-up after surgical
excision in view of their potential for local recurrence and lymph node metastasis. EHs must be differentiated from malignant lesions like hemangioendothelioma and angiosarcoma. EH is characterized by lobular growth pattern of capillary sized channels lined by plump endothelial cells with epithelioid morphology separated by stroma rich
Fig. 5. Immunohistochemistry showing (a) vimentin positivity in the vascular and stromal component. (b) CD31 positivity in the endothelial cells (curved arrow) [avidin biotin complex immunoperoxidase method: (a) and (b) 400×].
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in eosinophils with admixed lymphocytes and histiocytes as seen in the present case [2,3]. Myxoid and hyalinized stroma characteristic of epithelioid hemangioendothelioma is absent in these lesions [2,3]. Atypia, atypical mitosis and solid growth pattern characteristic of angiosarcoma is not seen in EH [3]. The recurrent t(1;3)(p36;q25) chromosomal translocation, resulting in WWTR1-CAMTA1 fusion, is identified as a consistent abnormality in epithelioid hemangioendothelioma but not in EH or other epithelioid vascular neoplasms like epithelioid angiosarcomas [6]. Molecular analysis for the presence of WWTR1-CAMTA1 fusion may serve as a useful diagnostic tool in challenging cases of epithelioid vascular tumors with morphological overlap. Common occurrence of vascular neoplasia in women and their tendency for rapid growth during pregnancy are suggestive of possible hormonal influence on these lesions. Various studies demonstrated the presence of estrogen receptors in these lesions. Deyrup et al. [7] in their immunohistochemical evaluation of 53 vascular neoplastic lesions reported the presence of estrogen receptor beta in all the cases. Spontaneous remission of a multifocal EH presenting in perigestational period reported by Lewis et al. [4] and few other reported cases of osseous EHs presenting in perigestational period suggest a gestation related proliferation stimulus on these lesions [1]. Presentation in perigestational period in the present case strongly suggests the influence of hormones on the proliferation of these lesions. Complete excision, whenever feasible is the treatment of choice for EH [2]. Present case was a diffuse lesion involving most of the occipital condyle with extension to clivus. Complete excision of such extensive lesion by single approach is difficult [8]. Various options in such cases include extended (expanded) endoscopic endonasal approach, far lateral approach, extensive skull base approach with petrosectomy and mastoidectomy [5,8]. Though extended endonasal endoscopic approach is minimally invasive it has various limitations. By endoscopic approach alone, it is difficult to achieve gross total excision of the lesions which are predominantly centered at lower clivus with significant lateral extension to occipital condyle as seen in the present case [8]. Far lateral approach offers better degree of tumor exposure and removal in such cases with significant lateral extension to occipital condyle. Dehdashti et al. [8] combined far lateral approach with extended endoscopic endonasal approach in such extensive lesions. Occipito cervical fusion was required in the present case as the occipital condyle was almost completely destroyed by the lesion. We preferred far lateral approach as it offers better exposure for the condylar extension of the tumor along with exposure for performing occipito cervical fusion. Further excision of the residual clival component in the present case requires an extensive skull base approach with petrosectomy and mastoidectomy or an extended endonasal endoscopic approach. Further excision of the residual lesion was not attempted in view of the benign nature of this lesion, proximity of the residual highly vascular firm lesion to the petrous segment of internal carotid artery (Fig. 1f), and morbidity associated with extensive skull base approaches. Experience with radiotherapy (RT) in the management of these cases is limited and only very few cases of bony EH were given
RT. Lewis et al. [4] reported spontaneous clinical and radiological remission in a patient with multifocal EH of bone with presentation in perigestational period and advised close observation rather than radiotherapy for the management of EH. In view of limited experience with RT in the management of these lesions and indolent course in majority of these lesions we did not give RT in the present case. Regular follow-up was advised to look for any increase in the size of residual lesion. 4. Conclusion Epithelioid hemangioma is a rare bony tumor and its involvement of occipital condyle and clivus is extremely uncommon. Presentation in perigestational period in the present case strongly suggests the influence of hormones on the proliferation of these vascular lesions. Patients with these lesions require a close followup after surgical excision in view of their potential for local recurrence. Source of support The authors have not received any support, in the form of grant, from any source for preparation of this article. Neither do the authors, nor does the institute have any personal or institutional financial interest in drugs, materials, or devices described in their submissions. Disclosure This paper has not been published before and has not been submitted for publication to any other journal in part or full. The authors concur with this submission and there is no conflict of interest arising from this article. References [1] Lamovec J, Bracko M. Epithelioid hemangioma of small tubular bones: a report of three cases, two of them associated with pregnancy. Modern Pathology 1996;9:821–7. [2] Nielsen GP, Srivastava A, Kattapuram S, Deshpande V, O’Connell JX, Mangham CD, et al. Epithelioid hemangioma of bone revisited: a study of 50 cases. American Journal of Surgical Pathology 2009;33:270–7. [3] Verbeke SL, Bovée JV. Primary vascular tumors of bone: a spectrum of entities? International Journal of Clinical and Experimental Pathology 2011;4: 541–51. [4] Lewis VO, Montag AG, Simon MA. Epithelioid hemangiomas of bone: spontaneous clinical and radiographic remission. Clinical Orthopaedics and Related Research 2003;407:167–72. [5] Mazzoni A. The petro-occipital trans-sigmoid approach for lesions of the jugular foramen. Skull Base 2009;19:48–56. [6] Errani C, Zhang L, Sung YS, Hajdu M, Singer S, Maki RG, et al. A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites. Genes, Chromosomes and Cancer 2011;50:644–53. [7] Deyrup AT, Tretiakova M, Khramtsov A, Montag AG. Estrogen receptor b expression in vascular neoplasia: an analysis of 53 benign and malignant cases. Modern Pathology 2004;17:1372–7. [8] Dehdashti AR, Karabatsou K, Ganna A, Witterick I, Gentili F. Expanded endoscopic endonasal approach for treatment of clival chordomas: early results in 12 patients. Neurosurgery 2008;63:299–309.