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Cavernous Hemangioma

Cavernous Hemangiomas

Cavernous hemangioma is a benign intraorbital tumor. Even though it is benign, the mass effect of the tumor causes different manifestations, including painless protrusion of the eye, hyperopia and diplopia.


Presentation

Patients with cavernous hemangioma may not be aware of the condition because the benign tumor usually causes no symptoms other than a slowly progressing protrusion of the eye, which some people might not notice in early stages. Cavernous hemangioma usually presents in the middle age (around 40 years of age). The main symptom patients present with is a painless protrusion of the globe or around the eye progressing slowly, which might be accompanied by other symptoms, such as the feeling of fullness around the eye and changes in visual acuity including hyperopia and diplopia due to mass effect compression caused by the tumor on the extraocular muscles and surrounding tissues. Some studies suggest that the symptoms may be more severe among pregnant women [11]. The onset of symptoms may differ depending on the location of the tumor, for example an extradural cavernous hemangioma behaves differently from the intramedullary ones, as well as from an extradural one in the spine [12] [13].

Localized Pain
  • The initial presenting symptoms were local pain in 5 cases, radiculopathy in 6 cases, and myelopathy in 3 cases. Upon admission, 1 patient had radicular symptoms and 13 had myelopathic symptoms. The average symptom duration was 18 months.[ncbi.nlm.nih.gov]
  • Presenting symptoms: Eyeball protrusion (90%), visual impairment (65%), double vision (20%), local Pain (18%), headache (12%), eyelid fullness/swelling (5%). For more details on the case, please see associated PowerPoint Presentation.[morancore.utah.edu]
Splenectomy
  • Because of the presence of persistent fresh blood through abdominal drainage at a rate of 1 L/h, splenectomy was performed to control the bleeding again by sutures and Teflon patches. Finally, the abdomen was closed using a biologic mesh.[ncbi.nlm.nih.gov]
Sore Throat
  • If a lesion extends into the parapharyngeal space, otalgia and sore throat can result. Spontaneous regression is not a characteristic of cavernous hemangiomas. Surgical excision is a management option.[ncbi.nlm.nih.gov]
Pneumonia
  • The second one, died postoperatively from pneumonia. Cardiac cavernous hemangiomas, although rare and well tolerated require prompt management and surgery at discovery to avoid further complications which may put at risk the patient's life prognosis.[ncbi.nlm.nih.gov]
Acute Abdomen
  • Abstract We present the case of a 70-year-old patient admitted with strong abdominal pains and operated in our service for hemorrhagic acute abdomen. Intraoperative hepatic cavernous hemangioma was found with capsular rupture and hemoperitoneum.[ncbi.nlm.nih.gov]
Abdominal Mass
  • A 70-year old man was referred for an 8-cm abdominal mass incidentally discovered on a contrast-enhanced computed tomography (CT) performed to investigate a pulmonary nodule.[ncbi.nlm.nih.gov]
Vascular Disease
  • Retinal Perforations, Breaks Surgical Procedures and Postoperative Cases Toxic Retinopathies; see also Light Toxicitiy Trauma (Injury) Tumors, Neoplasma Vascular Diseases (see also: Systemic Immunologic Diseases) Sclera Strabismus, Ocular Motility Disorders[atlasophthalmology.net]
  • Occlusive cerebro-vascular disease 1144 33.1. Atherosclerotic cerebrovascular disease 1144 33.2. Cerebral arterial dissections 1160 33.3. Extracranial-intracranial (EC/IC) bypass 1165 33.4. Cerebrovascular venous thrombosis 1166 33.5.[worldcat.org]
Palpitations
  • The first patient was symptomatic and presented with palpitations. The second patient was referred after a random discovery at echocardiography. Chest CT and MRI were performed in the two cases and showed a mass located in the pericardial cavity.[ncbi.nlm.nih.gov]
Lacrimation
  • Abstract To describe a patient with coexistence of ipsilateral adenoid cystic carcinoma of the lacrimal gland and orbital cavernous hemangioma.[ncbi.nlm.nih.gov]
  • Cavernous hemangiomas have also been reported to present as an osseous lesion involving the orbital bones or as a tumor within the lacrimal gland. Imaging with time-resolved imaging with contrast kinetics MRI (ie.[eyewiki.aao.org]
  • Christian Davidson, MD, and Bhupendra Patel, MD Date: 09/28/2015 Keywords/Main Subjects: Cavernous hemangioma; Orbit; Orbital mass; Proptosis Secondary CORE Category: Orbit, Eyelids and lacrimal System / Orbital Neoplasms and Malformations / Vascular[morancore.utah.edu]
  • Anterior Chamber Brain and Optical Pathway Choroid Congenital Syndromes, Malformations and Abnomalities Conjunctiva Cornea Glaucomas, Ocular Hypertension Hypertensive Retinopathy Iris and Ciliary Body Lacrimal System Lens Lids Neuro-Ophthalmology Optic[atlasophthalmology.net]
Central Scotoma
  • An examination revealed a visual acuity of 1.4/10 and a central scotoma in the right eye.[ncbi.nlm.nih.gov]
Enophthalmos
  • The medial orbital wall may be simultaneously reconstructed to prevent diplopia and enophthalmos.[ncbi.nlm.nih.gov]
Epistaxis
  • The author wanted to discuss this rare disease by presenting a 78-year-old woman who presented with nasal obstruction and recurrent epistaxis to the author's clinic.[ncbi.nlm.nih.gov]
  • We report the case of a 77-year-old man who presented with severe epistaxis, nasal congestion, and olfactory dysfunction.[ncbi.nlm.nih.gov]
  • […] subsequent management options, and attempt to establish the findings as the basis of considering it as an important differential diagnosis of radiologically heterogeneous sinonasal mass with suspected bone erosions presenting with nasal obstruction and epistaxis[ncbi.nlm.nih.gov]
Stroke
  • CCMs can bleed and cause strokes, seizures, and headaches. CCMs are often caused by an inherited gene mutation (alteration) in one of three CCM genes (CCM1, CCM2, or CCM3).[clinicaltrials.ucsf.edu]
  • […] called a haemorrhagic stroke The severity and duration of symptoms can vary depending on the type of cavernoma and where it's located.[nhs.uk]
  • Stroke 1010 29.1. Cerebrovascular hemodynamics 1010 29.2. Strokes: general information 1011 29.3. Stroke in young adults 1024 29.4. Lacunar strokes 1026 29.5. Collateral circulation 1027 29.6. "Occlusion" syndromes 1027 29.7.[worldcat.org]
  • Evidence-based and transdisciplinary, the second edition covers the full spectrum of neurovascular pathologies, preoperatively and postoperatively, including: Ischemic Stroke and Vascular Insufficiency, Cerebral and Spinal Aneurysms, Cerebral Spinal Arteriovenous[worldcat.org]
  • Stroke 34(5):1163–1169 PubMed CrossRef Google Scholar 5.[doi.org]

Workup

The workup in order to diagnose cavernous hemangioma includes several tools, such as CT scans, ultrasound studies, doppler flow studies, and MRI images.

The CT scan usually shows a homogenous lesion, which is oval or round in shape and sharply marginated. It usually enhances after injection of contrast; however, enhancement is not mandatory. CT scans alone are not sufficient to reach a definite diagnosis. In addition, a uniform high-echogenicity may be found in an ultrasound. Doppler flow studies may show low blood flow in the hemangioma and a specific honeycomb pattern of alternating strong and weak echos [14] [15] [16]. Because of the static blood in the vascular spaces, a negative doppler phenomenon may be observed [17]. The bone usually appears normal on the CT scans [18] [19]. Phleboliths may be detected as calcification on the CT scan.

Cavernous hemangioma does not contain large vessels, and that is why hypervascularity is not demenostrated on MRI in these cases [18] [19]. T1-weighted MRI images usually show low intensity or they could be isointense, while T2-weighted images may show high intensity. Hypointensity suggests the presence of phleboliths. An extradural cavernous hemangioma is homogenous on CT and MRI studies because of its sinusoidal channels [21].

Angiography may be an option in the diagnosis of cavernous hemangioma; however, it is rarely used because CT scans, MRI images, and ultrasound studies suffice in reaching the diagnosis [15] [20].

Histopathologic studies may aid in the diagnosis of cavernous hemangioma, and they usually show engorged tightly knit vascular channels, which are lined by a layer of endothelial cells and separated by fibrous septae.

Multiple Pulmonary Nodules
  • A cardiac neoplasm and multiple pulmonary nodules were detected. The tumor was surgically removed, and biopsy specimens were taken from the lung nodules. Histopathologic examination confirmed that both lesions were cavernous hemangiomas.[ncbi.nlm.nih.gov]
Central Scotoma
  • An examination revealed a visual acuity of 1.4/10 and a central scotoma in the right eye.[ncbi.nlm.nih.gov]

Treatment

Cavernous hemangioma usually does not require any medical or surgical intervention, and observation with followup suffices. However, if mass effect symptoms develop, surgical intervention might be needed in order to relieve the compression on the other tissues and minimize the symptoms [22].

Several approaches and procedures can be used in the excision of the tumor and the choice depends on its location. They include midfacial degloving, lateral rhinotomy, trans-palatal, and trans-antral approach. Le Fort I osteotomy may also be used.

The most successful procedure involves complete excision of the tumor with ligation or cautery of the vessels feeding it [9]. Other procedures like cryotherapy or resection with YAG laser may also be used. Emoblization of the hemangioma may be successful in the treatment [23]. In case of partial removal of the tumor, a risk of recurrence remains. Therefore, it is advised to use radiotherapy as an adjuvant [24].

Lately, there have been advances in radiosurgery and radiosurgical equipment, which are becoming a good option for the management of cavernous hemangioma [25].

Radiotherapy was used in the past as a treatment for cavernous hemangioma but is now strongly advised against due to its adverse effects. It remains a treatment option where surgery is not possible.

Prognosis

Cavernous hemangioma is considered a benign tumor and has a very good prognosis. Usually, there is no need for any medical or surgical intervention and the tumor remains stable throughout the patient's life. However, in some cases, as the tumor grows slowly, it might cause mass effect and compress the surrounding tissues and structures, which affects the visual acuity. Furthermore, other symptoms like diplopia or hyperopia may be observed [10].

Surgical intervention is rarely required and observation can be sufficient; however, if mass effect symptoms occur and affect the patient, surgery may be needed in order to excise the tumor. Different surgical approaches may be used depending on the location of the tumor. The complete removal of the tumor ensures an excellent prognosis, whereas some risk of recurrence remains after partial excision. Some features and complications caused by the mass effect of the tumor, such as hyperopia, may persist even after successful full excision.

Etiology

The cause of cavernous hemangioma is the formation of new vessels and proliferation of the vessel wall tissue components, which result in hyperplasia of cellular elements [6].

The developed tumor, as it grows slowly, compresses the surrounding tissues, which causes the symptoms related to mass effect, such as changes in vision acuity, hyperopia and diplopia.

Epidemiology

Cavernous hemangioma is the most common intraorbital tumor in adults [7]. It appears that there is no difference in the incidence depending on race or gender; however, some studies suggest that the incidence is slightly higher among women than men [7] [8]. The tumor usually presents in the middle age (around the age of 40) [9].

Cavernous hemangioma is usually an intraorbital tumor. While hemangiomata are most commonly seen on the extremities or the trunk, they may also present on other parts of the body, such as the head and the neck.

Sex distribution
Age distribution

Pathophysiology

Even though cavernous hemangioma is a benign intraorbital tumor, it may be considered malignant because of its mass effect and the resultant complications, such as hyperopia and diplopia. The composition of cavernous hemangioma includes dilated cavernous vascular spaces separated by connective tissue stroma and lined with endothelium [10]. Phleboliths identified in CT scans is due to calcified thrombi within these vascular spaces. The vascular spaces and channels bud into its surroundings, which results in tumor growth and thus leading to displacement and incorporation of the surrounding structures and tissue into the tumor. Lymphangioma may show similar histopathologic features as those seen in cavernous hemangioma.

Prevention

The etiology of cavernous hemangioma is still being studied by researchers in order to understand more about the genes linked to the development of the condition. At the moment, it is not possible to prevent the condition. However, education and familial counseling are used to educate patients and families affected by the condition.

Summary

Cavernous hemangioma is the most common intraorbital tumor in adults. Patients usually complain about painless protrusion of the eye, which grows slowly. It belongs to a wide spectrum of hamartomas [1]. The tumor may occur in any part along the neuroaxis [2] [3]; however, the most common locations are the cerebellum, cerebral hemisphere and the brainstem, and it is most commonly found in supratentorial compartments [4].

There are two forms of cavernous hemangioma, familial and sporadic. Sporadic cases are more common than the familial ones, accounting for about 90% of the cases [5].

The incidence of the tumor is almost equal between both genders; however, some studies suggest that the incidence is slightly higher among women.

The symptoms and features of the tumor develop very slowly starting with painless protrusion or bulging of the globe, accompanied by a feeling of fullness in the eyelid and around the eye. Vision acuity might be affected by the tumor. Hyperopia and diplopia may develop as a result of mass effect and compression of the tumor on the surrounding and adjacent structures and tissues.

The tumor is benign and usually has good prognosis without significant complications. However, there might be some complications due to the mass effect and the compression of the tumor on the adjacent structures, such as changes in vision acuity. Prognosis after surgical excision of the tumor is good and there is almost no risk of recurrence, especially if the tumor was removed completely.

Different workup tools may be used in the diagnosis of cavernous hemangioma, such as computed tomography (CT) scans, magnetic resonance imagingn (MRI), and ultrasound studies. Angiography may be used as well; however, it is not mandatory for the diagnosis.

Cavernous hemangioma is a benign tumor and it usually requires no treatment other than observation and followup. However, if mass effect symptoms such as changes in vision acuity develop, then surgical intervention may be needed in order to excise the tumor and relieve the compression caused by the tumor. In the past, radiotherapy was used as a treatment for cavernous hemangioma; however, it is not used anymore due to its complications.

Prevention of cavernous hemangioma is not easy and researchers are still studying the related genes. However, counselling and education for affected families may be helpful in raising awareness about the tumor and the possible complications.

Patient Information

Cavernous hemangioma is a benign tumor, which usually affects women more than men and presents around the age of 40 years. It is a collection of spaces filled with blood and usually develops intraorbitally, which means around the globe of the eye.

There are two types of cavernous hemangioma, the most common type is sporadic, which has no previous familial history. The second type is familial, which is associated with familial genes and usually runs in families.

Patients who have this tumor may not be aware of the condition because it is painless. The main symptom that patients may notice is a protrusion in the globe or around the eye, which grows slowly and compresses the adjacent structures and tissues. This may lead to other symptoms like vision problems or the feel of fullness in the globe of the eye.

Several tools are used to make the diagnosis of cavernous hemangioma, such as CT scans, MRI images, and ultrasound studies.

Usually, cavernous hemangioma does not require any treatment or intervention. However, if symptoms like vision problems develop, surgical intervention may be considered as an option to relieve these symptoms and prevent other complications that may happen due to the compression of the tumor on the surrounding structures. Surgical intervention mainly involves complete excision of the tumor, which is done using different approaches, depending on the location of the tumor.

Cavernous hemangioma is a benign tumor and the outcome is very good. Patients who have the tumor can live a normal life without removing it. However, if surgery becomes necessary, the tumor is removed completely, resulting in almost no risk of recurrence.

It is hard to prevent the development of cavernous hemangioma because only little is known about the genes related to the condition. However, counselling and education for patients and their families may be useful to raise awareness about the condition, the outcomes, and the possible complications.

References

Article

  1. Graziani N, Bouillot P, Figarella-Branger D et al. Cavernous angiomas and arteriovenous malformations of the spinal epidural space: report of 11 cases. Neurosurgery. November 1994; 35(5): 856-63; discussion 863-4.
  2. Padovani R, Poppi M, Pozzati E, et al. Spinal epidural hemangiomas. Spine (Phila Pa 1976). July-August 1981; 6(4): 336-40.
  3. Robinson JR, Awad IA, Little JR. Natural history of the cavernous angioma. J Neurosurg. November 1991; 75(5): 709-14.
  4. Labauge P. [Familial forms of central nervous system cavernomas: from recognition to gene therapy]. Neurochirurgie. June 2007; 53(2-3 Pt 2): 152-5.
  5. Palur RS, Ravikumar CV, Satish S, et al. Developmental vascular abnormalities of the central nervous system-other than AVM's. In: Venkataraman S, ed. Progress in clinical neurosciences. Neurological Society of India. 1999; 151-72.
  6. Rosca TI, Pop MI, Curca M, et al. Vascular tumors in the orbit--capillary and cavernous hemangiomas. Ann Diagn Pathol. February 2006; 10(1): 13-9.
  7. Henderson GW. Vascular hamartomas, hyperplasias, and neoplasms. Henderson GW, ed. Orbital Tumors. New York: Raven Press. 1994; 94-100.
  8. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit: a clinicopathologic analysis of sixty-six cases. Jakobiec, ed. Ocular and Adnexal Tumors. Birmingham, Ala: 1978; 741-81.
  9. Iwata N, Hattori K, Nakagawa T, et al. Hemangioma of the nasal cavity: a clinicopathologic study. Auris Nasus Larynx. October 2002; 29(4): 335-9.
  10. Enzinger F, Weiss S. Soft tissue tumor. 2nd edition. St Louis: Mosby; 1988.
  11. Zauberman H, Feinsod M. Orbital hemangioma growth during pregnancy. Acta Ophthalmol (Copenh). 1970; 48(5): 929-33.
  12. Tekkök IH, Akpinar G, Güngen Y. Extradural lumbosacral cavernous hemangioma. Eur Spine J. August 2004; 13(5): 469-73. Epub December 20, 2003.
  13. Rovira A, Capellades J, Zauner M, et al. Lumbar extradural hemangiomas: report of three cases. AJNR Am J Neuroradiol. January 1999; 20(1): 27-31.
  14. Ko F, Dibernardo CW, Oak J, et al. Confirmation of and differentiation among primary vascular lesions using ultrasonography. Ophthal Plast Reconstr Surg. November 2011; 27(6): 431-5.
  15. Davis KR, Hesselink JR, Dallow RL, et al. CT and ultrasound in the diagnosis of cavernous hemangioma and lymphangioma of the orbit. J Comput Tomogr. June 1980; 4(2): 98-104.
  16. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit. J Neurosurg. August 1979; 51(2): 219-28.
  17. Levine RA. Orbital ultrasonography. Radiol Clin North Am. May 1987; 25(3): 447-69.
  18. Dillon WP, Som PM, Rosenau W. Hemangioma of the nasal vault: MR and CT features. Radiology. September 1991; 180(3): 761-5.
  19. Itoh K, Nishimura K, Togashi K, et al. MR imaging of cavernous hemangioma of the face and neck. J Comput Assist Tomogr. September-October 1986; 10(5): 831-5.
  20. Rootman J. Diseases of the orbit - A multi disciplinary Approach 2nd ed; 2003.
  21. Aoyagi N, Kojima K, Kasai H. Review of spinal epidural cavernous hemangioma. Neurol Med Chir (Tokyo). October 2003; 43(10): 471-5; discussion 476.
  22. Boari N, Gagliardi F, Castellazzi P, et al. Surgical treatment of orbital cavernomas: clinical and functional outcome in a series of 20 patients. Acta Neurochir (Wien). March 2011; 153(3): 491-8.
  23. Webb CJ, Porter G, Spencer MG, et al. Cavernous haemangioma of the nasal bones: an alternative management option. J Laryngol Otol. April 2000; 114(4): 287-9.
  24. Padovani R, Acciarri N, Giulioni M, et al. Cavernous angiomas of the spinal district: surgical treatment of 11 patients. Eur Spine J. 1997; 6(5): 298-303.
  25. Sohn MJ, Lee DJ, Jeon SR, et al. Spinal radiosurgical treatment for thoracic epidural cavernous hemangioma presenting as radiculomyelopathy: technical case report. Neurosurgery. June 2009; 64(6): E1202-3; discussion E1203. doi: 10.1227/01.NEU.0000345940.21674.AE.

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Last updated: 2019-06-28 11:07