Novel Insights from Clinical Practice Received: November 13, 2013 Accepted after revision: April 30, 2014 Published online: July 24, 2014
Pediatr Neurosurg 2013;49:229–231 DOI: 10.1159/000363328
Congenital Intraosseous Cavernous Hemangioma of the Skull: An Unusual Case Makhdoomi Rumana a Nayil Khursheed b Mustafa Farhat a Salim Othman a Laharwal Masood b
Departments of a Pathology and b Neurosurgery, Sheri-Kashmir Institute of Medical Sciences, Kashmir, India
Established Facts • Congenital intraosseous cavernous hemangioma of the skull is a rare entity presenting as a skull mass.
Novel Insights • It needs to be distinguished from other childhood skull swellings; with appropriate diagnosis and complete excision the results are excellent.
Key Words Hemangioma · Skull · Congenital intraosseous hemangioma
Abstract Intraosseous hemangiomas are benign vascular malformations mostly seen in the spine. They rarely occur in the skull. The usual age-group involved is the 2nd to 4th decades, and females outnumber males. We hereby report a rare case of congenital intraosseous cavernous hemangioma of the skull bone in a male infant. The patient underwent total excision of the lesion. © 2014 S. Karger AG, Basel
© 2014 S. Karger AG, Basel 1016–2291/14/0494–0229$39.50/0 E-Mail [email protected] www.karger.com/pne
Introduction
Intraosseous hemangiomas of the skull are rare vascular lesions; usually young females are affected. These hemangiomas present as expansile lesions producing local swelling and pain [1]. Radiology shows asymmetric enlargement of the skull with a few classic radiological signs [2]. Intraosseous hemangiomas of the skull need to be differentiated from a number of other neonatal skull swellings [3]. Total excision of the lesion achieves the cure. Histopathology is characteristic and reveals dilated blood spaces with hemorrhage [4].
Dr. Nayil Khursheed, MS, MCh Department of Neurosurgery Sheri-Kashmir Institute of Medical Sciences Srinagar 190011 (India) E-Mail nayilkhursh @ gmail.com
Case Report
Fig. 1. CT scan showing asymmetric enlargement of the right parietal bone. The inner table is irregular and the outer table is thinned and expanded. The diploic space is also increased.
A 3-month-old male baby delivered by lower segment cesarean section was noted to have a small soft swelling over the parietal area at birth. There was no history suggestive of perinatal insult. The swelling remained constant in size for the next 2 months. Over the course of the next month the parents noticed that the swelling increased in size and became harder. With these complaints they reported to our neurosurgical outpatient department. On examination there was a bony hard swelling about 6 × 5 cm in size over the right parietal area. There were no other findings and no neurological deficit. A head CT scan with bone windows showed asymmetric enlargement of the skull over the right parietal area with increased diploic space of the right parietal bone. The inner table was irregular and the outer table seemed to be thinned and expanded. There was no associated soft tissue component (fig. 1). The patient underwent parietal craniectomy with total excision of the lesion. The bone was bluish in color and had expanded. The outer table was not eroded. Normal surrounding bone was also nibbled. There was minimal blood loss during the surgery. The dura was normal. The patient was planned for autologous rib cranioplasty. The histopathological examination revealed thin-walled, dilated, irregular vascular channels lined by a single layer of flattened endothelial cells interspersed among bony trabeculae, consistent with an intraosseous cavernous hemangioma of the bone (fig. 2).
Discussion
Fig. 2. Photomicrograph showing dilated vascular channels filled with blood infiltrating the bone. HE. ×20.
Table 1. Case reports of congenital cavernous hemangioma of the
skull in the literature References
Year
Age at Gender presentation
Site
Yoshida [5] Honda [8] Vural [9] Martinez-Lage [10] Yucel [11] Present case
1999 2003 2009 2010 2011 2013
4 months at birth 6 months 12 months 4 months 3 months
parietal temporal parietal parietal parietal parietal
230
Pediatr Neurosurg 2013;49:229–231 DOI: 10.1159/000363328
male male male female male
Primary intraosseous hemangiomas are benign vascular malformations that account for approximately 1% of all primary bone neoplasms. The skull bones are lamellar at birth. The diploic veins and the diploic space develop around the 2nd year of age. Abnormal differentiation of capillary vessels during the intrauterine period may cause the formation of intraosseous hemangiomas. Trauma has not been thought to be a predisposing factor [1, 5]. These tumors are mostly found in vertebral bodies and are rarely seen in the calvarium [6]. The usual age of presentation is the 2nd to 4th decades of life. Females outnumber males by a ratio of 4:1 [7, 8]. Our patient was a male child and he had the swelling over the parietal area since birth. In a review of the literature only 5 cases of congenital cavernous hemangiomas of the skull have been reported in infants and surprisingly most of the patients had parietal bone involvement (table 1) [5, 8–11]. Hemangiomas are classified histopathologically into venous, capillary and cavernous subtypes [12]. Capillary hemangiomas lack fibrous septa, have smaller vessel lumens and mostly are seen in the vertebral column, while cavernous hemangiomas have thin-walled blood vessels and variable amounts of intervening collagenous fibrosis and may show calcification [4, 13]. Calvarial hemangiomas present as expansile lesions producing local swelling. Rumana /Khursheed /Farhat /Othman / Masood
The swelling may be associated with pain [4]. Our patient also had a swelling over the right parietal area since birth which had increased in size over the last month. Cavernous hemangiomas of the skull usually affect the frontal and parietal bones [14, 15]. Radiologically these lesions are well circumscribed, expansile with areas of rarefaction owing to osteoblastic remodeling of trabecular bone following osteoclastic activity of the tumor resulting in a typical sunburst appearance on X-ray or a honeycomb or polka-dot appearance on axial CT scan [2, 11, 16]. On MRI intraosseous hemangiomas have heterogeneous signal on both T1- and T2-weighted sequence depending basically on the amount of venous blood, content of red marrow and fatty marrow [16]. However, of note is that smaller calvarial hemangiomas may not exhibit all the classic radiological signs except for the rarefaction of the bone, and the diagnosis may be made only after histopathology [17]. Glucose transporter protein (GLUT1) immunoreactivity is a highly selective and diagnostically useful marker for infantile capillary hemangiomas as was demonstrated in a retrospective study on hemangiomas [18]. Propranolol can be tried as first-line therapy in infantile hemangiomas irrespective of age of presentation, location of the lesion, extent and phase of growth. Treatment with propranolol might also help in downgrading the size, making the lesion more amenable to surgical ex-
cision [19]. Indications for surgical removal include cosmetic deformity, neurological deficit, headache or need for definitive diagnosis. Usually en bloc resection is done with normal bony margins [2]. Aneurysmal bone cyst, osteoma, giant cell tumor, fibrous dysplasia, sarcoma, meningioma, metastatic disease, Paget’s disease, dermoid and epidermoid cyst should be taken into consideration in the differential diagnosis of cavernous hemangioma of the skull [19–22]. The differential diagnosis especially in children also includes fibrous dysplasia, cephalohematomas, desmoplastic fibroma, cranial fasciitis, and dermal eosinophilic granuloma [3]. Recurrence after surgery is rare [2]. The role of radiotherapy is limited, it may provide some help after subtotal resection [4].
Conclusion
Congenital intraosseous cavernous hemangioma of the skull is an unusual neoplasm; it is a treatable condition and should be included in the differential diagnosis of neonatal skull swellings. Osteoblastic remodeling with trabecular bone following osteoclastic activity of the tumor results in a typical sunburst appearance on X-ray or CT scan.
References 1 Maraire JN, Awad IA: Intracranial cavernous malformations: lesion behavior and management strategies. Neurosurgery 1995; 37: 591– 605. 2 Liu JK, Burger PC, Harnsberger HR, Couldwell WT: Primary intraosseous skull base cavernous hemangioma: case report. Skull Base 2003; 13:219–228. 3 Gibson SE, Prayson RA: Primary skull lesions in the pediatric population: a 25-year experience. Arch Pathol Lab Med 2007;131:761–766. 4 Peterson DL, Murk SE, Story JL: Multifocal cavernous hemangioma of the skull: report of a case and review of the literature. Neurosurgery 1992;30:778–781. 5 Yoshida D, Sugisaki Y, Shimura T, Teramoto A: Cavernous hemangioma of the skull in a neonate. Childs Nerv Syst 1999;15:351–353. 6 Gologorsky Y, Shrivastava RK, Panov F, Mascitelli J, Signore AD, Govindaraj S, Smethurst M, Bronster DJ: Primary intraosseous cavernous hemangioma of the clivus: case report and review of the literature. J Neurol Surg Rep 2013;74:17–22. 7 Heckl S, Aschoff A, Kunze S: Cavernomas of the skull: review of the literature 1975–2000. Neurosurg Rev 2002;25:56–62. 8 Honda M, Toda K, Baba H, Yonekura M: Congenital cavernous angioma of the temporal
Congenital Intraosseous Cavernous Hemangioma of the Skull
9
10
11 12
13 14
15
bone: case report. Surg Neurol 2003; 59: 120– 123. Vural M, Acikalin MF, Adspinar B, Atasoy MA: Congenital cavernous hemangioma of the calvaria. Case report. J Neurosurg Pediatr 2009;3:41–45. Martinez-Lage JF, Torroba MA, Cuartero Perez B, Almagro MJ, Lopez Lopez-Guerrero A, de la Rosa P: Cavernous hemangioma of the cranial vault in infants: a case-based update. Childs Nerv Syst 2010;26:861–865. Yucel E, Akkaya H, Gurkanlar D, Ergun T: Congenital cavernous hemangioma of the skull. Turk Neurosurg 2011;21:645–647. Adler CP, Wold L: Haemangioma and related lesions; in Fletcher CDM, Unni KK, Mertens F (eds): World Health Organization Classification of Tumors: Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon, IARC Press, 2002, pp 320–321. Sade B, Lee DK, Prayson RA, Hughes GB, Lee JH: Intraosseous cavernous angioma of the petrous bone. Skull Base 2009;19:237–240. Clauser L, Meneghini F, Riga M, Rigo L: Haemangioma of the zygoma. Report of two cases with a review of the literature. J Craniomaxillofac Surg 1991;19:353–358. Moore SL, Chun JK, Mitre SA, Som PM: Intraosseous hemangioma of the zygoma: CT
16
17
18
19 20
21
22
and MR findings. AJNR Am J Neuroradiol 2001;22:1383–1385. Vanhoenacker FM, De Praeter G, Kools D, Voormolen M, Parizel PM: Unusual lesion of the clivus: diagnosis and discussion. Skeletal Radiol 2011;40:243–244. Khanam H, Lipper MH, Wolff CL, Lopes MB: Calvarial hemangiomas: report of two cases and review of the literature. Surg Neurol 2001; 55:63–67. North PE, Waner M, Mizeracki A, Mihm MC Jr: GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol 2000;31:11–22. Gunturi N, Ramgopal S, Balagopal S, Scott JX: Propranolol therapy for infantile hemangioma. Indian Pediatr 2013;50:307–313. Banerji D, Inao S, Sugita K, Kaur A, Chhabra DK: Primary intraosseous orbital hemangioma: a case report and review of the literature. Neurosurgery 1994;35:1131–1134. Buhl R, Barth H, Dorner L, Nabavi A, Rohr A, Mehdorn HM: De novo development of intraosseous cavernous hemangioma. J Clin Neurosci 2007;14:289–292. Suzuki Y, Ikeda H, Matsumoto K: Neuroradiological features of intraosseous cavernous hemangioma – case report. Neurol Med Chir (Tokyo) 2001;41:279–282.
Pediatr Neurosurg 2013;49:229–231 DOI: 10.1159/000363328
231
Copyright: S. Karger AG, Basel 2014. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
Pediatr Neurosurg 2013;49:229–231 DOI: 10.1159/000363328
Congenital Intraosseous Cavernous Hemangioma of the Skull: An Unusual Case Makhdoomi Rumana a Nayil Khursheed b Mustafa Farhat a Salim Othman a Laharwal Masood b
Departments of a Pathology and b Neurosurgery, Sheri-Kashmir Institute of Medical Sciences, Kashmir, India
Established Facts • Congenital intraosseous cavernous hemangioma of the skull is a rare entity presenting as a skull mass.
Novel Insights • It needs to be distinguished from other childhood skull swellings; with appropriate diagnosis and complete excision the results are excellent.
Key Words Hemangioma · Skull · Congenital intraosseous hemangioma
Abstract Intraosseous hemangiomas are benign vascular malformations mostly seen in the spine. They rarely occur in the skull. The usual age-group involved is the 2nd to 4th decades, and females outnumber males. We hereby report a rare case of congenital intraosseous cavernous hemangioma of the skull bone in a male infant. The patient underwent total excision of the lesion. © 2014 S. Karger AG, Basel
© 2014 S. Karger AG, Basel 1016–2291/14/0494–0229$39.50/0 E-Mail [email protected] www.karger.com/pne
Introduction
Intraosseous hemangiomas of the skull are rare vascular lesions; usually young females are affected. These hemangiomas present as expansile lesions producing local swelling and pain [1]. Radiology shows asymmetric enlargement of the skull with a few classic radiological signs [2]. Intraosseous hemangiomas of the skull need to be differentiated from a number of other neonatal skull swellings [3]. Total excision of the lesion achieves the cure. Histopathology is characteristic and reveals dilated blood spaces with hemorrhage [4].
Dr. Nayil Khursheed, MS, MCh Department of Neurosurgery Sheri-Kashmir Institute of Medical Sciences Srinagar 190011 (India) E-Mail nayilkhursh @ gmail.com
Case Report
Fig. 1. CT scan showing asymmetric enlargement of the right parietal bone. The inner table is irregular and the outer table is thinned and expanded. The diploic space is also increased.
A 3-month-old male baby delivered by lower segment cesarean section was noted to have a small soft swelling over the parietal area at birth. There was no history suggestive of perinatal insult. The swelling remained constant in size for the next 2 months. Over the course of the next month the parents noticed that the swelling increased in size and became harder. With these complaints they reported to our neurosurgical outpatient department. On examination there was a bony hard swelling about 6 × 5 cm in size over the right parietal area. There were no other findings and no neurological deficit. A head CT scan with bone windows showed asymmetric enlargement of the skull over the right parietal area with increased diploic space of the right parietal bone. The inner table was irregular and the outer table seemed to be thinned and expanded. There was no associated soft tissue component (fig. 1). The patient underwent parietal craniectomy with total excision of the lesion. The bone was bluish in color and had expanded. The outer table was not eroded. Normal surrounding bone was also nibbled. There was minimal blood loss during the surgery. The dura was normal. The patient was planned for autologous rib cranioplasty. The histopathological examination revealed thin-walled, dilated, irregular vascular channels lined by a single layer of flattened endothelial cells interspersed among bony trabeculae, consistent with an intraosseous cavernous hemangioma of the bone (fig. 2).
Discussion
Fig. 2. Photomicrograph showing dilated vascular channels filled with blood infiltrating the bone. HE. ×20.
Table 1. Case reports of congenital cavernous hemangioma of the
skull in the literature References
Year
Age at Gender presentation
Site
Yoshida [5] Honda [8] Vural [9] Martinez-Lage [10] Yucel [11] Present case
1999 2003 2009 2010 2011 2013
4 months at birth 6 months 12 months 4 months 3 months
parietal temporal parietal parietal parietal parietal
230
Pediatr Neurosurg 2013;49:229–231 DOI: 10.1159/000363328
male male male female male
Primary intraosseous hemangiomas are benign vascular malformations that account for approximately 1% of all primary bone neoplasms. The skull bones are lamellar at birth. The diploic veins and the diploic space develop around the 2nd year of age. Abnormal differentiation of capillary vessels during the intrauterine period may cause the formation of intraosseous hemangiomas. Trauma has not been thought to be a predisposing factor [1, 5]. These tumors are mostly found in vertebral bodies and are rarely seen in the calvarium [6]. The usual age of presentation is the 2nd to 4th decades of life. Females outnumber males by a ratio of 4:1 [7, 8]. Our patient was a male child and he had the swelling over the parietal area since birth. In a review of the literature only 5 cases of congenital cavernous hemangiomas of the skull have been reported in infants and surprisingly most of the patients had parietal bone involvement (table 1) [5, 8–11]. Hemangiomas are classified histopathologically into venous, capillary and cavernous subtypes [12]. Capillary hemangiomas lack fibrous septa, have smaller vessel lumens and mostly are seen in the vertebral column, while cavernous hemangiomas have thin-walled blood vessels and variable amounts of intervening collagenous fibrosis and may show calcification [4, 13]. Calvarial hemangiomas present as expansile lesions producing local swelling. Rumana /Khursheed /Farhat /Othman / Masood
The swelling may be associated with pain [4]. Our patient also had a swelling over the right parietal area since birth which had increased in size over the last month. Cavernous hemangiomas of the skull usually affect the frontal and parietal bones [14, 15]. Radiologically these lesions are well circumscribed, expansile with areas of rarefaction owing to osteoblastic remodeling of trabecular bone following osteoclastic activity of the tumor resulting in a typical sunburst appearance on X-ray or a honeycomb or polka-dot appearance on axial CT scan [2, 11, 16]. On MRI intraosseous hemangiomas have heterogeneous signal on both T1- and T2-weighted sequence depending basically on the amount of venous blood, content of red marrow and fatty marrow [16]. However, of note is that smaller calvarial hemangiomas may not exhibit all the classic radiological signs except for the rarefaction of the bone, and the diagnosis may be made only after histopathology [17]. Glucose transporter protein (GLUT1) immunoreactivity is a highly selective and diagnostically useful marker for infantile capillary hemangiomas as was demonstrated in a retrospective study on hemangiomas [18]. Propranolol can be tried as first-line therapy in infantile hemangiomas irrespective of age of presentation, location of the lesion, extent and phase of growth. Treatment with propranolol might also help in downgrading the size, making the lesion more amenable to surgical ex-
cision [19]. Indications for surgical removal include cosmetic deformity, neurological deficit, headache or need for definitive diagnosis. Usually en bloc resection is done with normal bony margins [2]. Aneurysmal bone cyst, osteoma, giant cell tumor, fibrous dysplasia, sarcoma, meningioma, metastatic disease, Paget’s disease, dermoid and epidermoid cyst should be taken into consideration in the differential diagnosis of cavernous hemangioma of the skull [19–22]. The differential diagnosis especially in children also includes fibrous dysplasia, cephalohematomas, desmoplastic fibroma, cranial fasciitis, and dermal eosinophilic granuloma [3]. Recurrence after surgery is rare [2]. The role of radiotherapy is limited, it may provide some help after subtotal resection [4].
Conclusion
Congenital intraosseous cavernous hemangioma of the skull is an unusual neoplasm; it is a treatable condition and should be included in the differential diagnosis of neonatal skull swellings. Osteoblastic remodeling with trabecular bone following osteoclastic activity of the tumor results in a typical sunburst appearance on X-ray or CT scan.
References 1 Maraire JN, Awad IA: Intracranial cavernous malformations: lesion behavior and management strategies. Neurosurgery 1995; 37: 591– 605. 2 Liu JK, Burger PC, Harnsberger HR, Couldwell WT: Primary intraosseous skull base cavernous hemangioma: case report. Skull Base 2003; 13:219–228. 3 Gibson SE, Prayson RA: Primary skull lesions in the pediatric population: a 25-year experience. Arch Pathol Lab Med 2007;131:761–766. 4 Peterson DL, Murk SE, Story JL: Multifocal cavernous hemangioma of the skull: report of a case and review of the literature. Neurosurgery 1992;30:778–781. 5 Yoshida D, Sugisaki Y, Shimura T, Teramoto A: Cavernous hemangioma of the skull in a neonate. Childs Nerv Syst 1999;15:351–353. 6 Gologorsky Y, Shrivastava RK, Panov F, Mascitelli J, Signore AD, Govindaraj S, Smethurst M, Bronster DJ: Primary intraosseous cavernous hemangioma of the clivus: case report and review of the literature. J Neurol Surg Rep 2013;74:17–22. 7 Heckl S, Aschoff A, Kunze S: Cavernomas of the skull: review of the literature 1975–2000. Neurosurg Rev 2002;25:56–62. 8 Honda M, Toda K, Baba H, Yonekura M: Congenital cavernous angioma of the temporal
Congenital Intraosseous Cavernous Hemangioma of the Skull
9
10
11 12
13 14
15
bone: case report. Surg Neurol 2003; 59: 120– 123. Vural M, Acikalin MF, Adspinar B, Atasoy MA: Congenital cavernous hemangioma of the calvaria. Case report. J Neurosurg Pediatr 2009;3:41–45. Martinez-Lage JF, Torroba MA, Cuartero Perez B, Almagro MJ, Lopez Lopez-Guerrero A, de la Rosa P: Cavernous hemangioma of the cranial vault in infants: a case-based update. Childs Nerv Syst 2010;26:861–865. Yucel E, Akkaya H, Gurkanlar D, Ergun T: Congenital cavernous hemangioma of the skull. Turk Neurosurg 2011;21:645–647. Adler CP, Wold L: Haemangioma and related lesions; in Fletcher CDM, Unni KK, Mertens F (eds): World Health Organization Classification of Tumors: Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon, IARC Press, 2002, pp 320–321. Sade B, Lee DK, Prayson RA, Hughes GB, Lee JH: Intraosseous cavernous angioma of the petrous bone. Skull Base 2009;19:237–240. Clauser L, Meneghini F, Riga M, Rigo L: Haemangioma of the zygoma. Report of two cases with a review of the literature. J Craniomaxillofac Surg 1991;19:353–358. Moore SL, Chun JK, Mitre SA, Som PM: Intraosseous hemangioma of the zygoma: CT
16
17
18
19 20
21
22
and MR findings. AJNR Am J Neuroradiol 2001;22:1383–1385. Vanhoenacker FM, De Praeter G, Kools D, Voormolen M, Parizel PM: Unusual lesion of the clivus: diagnosis and discussion. Skeletal Radiol 2011;40:243–244. Khanam H, Lipper MH, Wolff CL, Lopes MB: Calvarial hemangiomas: report of two cases and review of the literature. Surg Neurol 2001; 55:63–67. North PE, Waner M, Mizeracki A, Mihm MC Jr: GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol 2000;31:11–22. Gunturi N, Ramgopal S, Balagopal S, Scott JX: Propranolol therapy for infantile hemangioma. Indian Pediatr 2013;50:307–313. Banerji D, Inao S, Sugita K, Kaur A, Chhabra DK: Primary intraosseous orbital hemangioma: a case report and review of the literature. Neurosurgery 1994;35:1131–1134. Buhl R, Barth H, Dorner L, Nabavi A, Rohr A, Mehdorn HM: De novo development of intraosseous cavernous hemangioma. J Clin Neurosci 2007;14:289–292. Suzuki Y, Ikeda H, Matsumoto K: Neuroradiological features of intraosseous cavernous hemangioma – case report. Neurol Med Chir (Tokyo) 2001;41:279–282.
Pediatr Neurosurg 2013;49:229–231 DOI: 10.1159/000363328
231
Copyright: S. Karger AG, Basel 2014. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
