Haemangioma of the Zygoma Report of two Cases with a Review of the Literature
Luigi Clauser, Fabio Meneghini, Manuela Riga, Leone Rigo
Dept. of Maxillofacial Surgery, Centre for Craniofacial Deformities (Head: Prof. C. Curioni, M.D., D.M.D.), San Bortolo Hospital, Vicenza, Italy
Submitted 17.4. 91; accepted 14. 8.91
Summary
Zygomatic haemangioma is a rare benign neoplasm oc- curring most frequently in adult females. The authors, after reviewing the available literature, report two cases which presented with a swelling of the zygoma. Resec- tion of the neoplasm, immediate reconstruction with cranial bone grafts and internal rigid fixation was the treatment of choice. Results two years after surgery are presented.
Key words
Haemangioma - Orbital tumours - Cranial bone grafting - Internal rigid fixation
Introduction
Vascular neoformations of bony structures, whether benign or not, have a low rate of incidence (Jaffe, 1958). At the Mayo Clinic, in a review of 400 cases of bone tu- rnouts, only 69 were of vascular origin and of these, 56 were haemangiomas, 4haemangiopericytomas and 9 haem- angioendotheliomas (Unni et al., 1971). A topographic dis- tribution of the 56 cases of bony haemangiomas showed that most of these neoformations occurred in the maxillo- facial region, jaw included, and vertebrae. Regarding the age distribution, bone haemangiomas appear in all age
groups. The incidence is higher in females (Unni et al., 1971; Schindel et al., 1978). The zygomatic site of a bone haemangioma is extremely rare. Besides the two cases we report, other cases have also been reported in the literature by Schofield (1950), Davis and Morgan (1974), Marshak (1980), Schmidt (1982) and Har-El et al. (1986). The neoformation occurs more fre- quently in adult females. The initial signs frequently consist of a hard protruding mass that elicits pain either spontane- ously or during palpation. The case of a zygomatic haeman- gioma with endosinus development, reported by Har-El et
Tables 1 a and b Clinical findings and treatment data of the haemangiomas of the zygoma reported in the literature.
Table l a Clinical findings of haemangiomas of the zygoma reported in the literature.
Cases Sex and Symptoms Rx pattern Size age, years
Schofield 1950 M, 1 Firm, non-pulsating, non-tender lump "Irregular" Davis 1974 F, 47 Bony hard mass, not painful "Reticulated" Marshak 1980 F, 53 Bony hard mass, occasionally painful "Honeycomb" Marshak 1980 F, 35 Painful bony hard mass "Reticulated" Schmidt 1982 F, 43 Bony hard mass "Sunburst" Har-EI 1986 M, 60 Hemifacial pain, mass in the maxillary sinus Not mentioned Clauser 1990 F, 46 Firm bony hard mass, not painful "Honeycomb" Clauser 1990 F, 35 Firm bony hard mass, not painful "Sunray"
20 x 20 mm 20x20 mm 20x20 mm 20x20 mm
5x 7mm 40x30 mm 30x20 mm 25x20 mm
Table 1 b Treatment data of the haemangioma of the zygoma reported in the literature.
Cases Treatment Surgical approach Bone graft Intraoperative Histology bleeding
Schofield 1950 Excision Transverse incision over the malar No Not mentioned Capillary Davis 1974 Excision Infraorbital incision Rib autograft (delayed) 250 ml Cavernous Marshak 1 9 8 0 Excision Infraorbital incision No "Minimal" Capillary Marshak 1 9 8 0 Excision Infraorbital incision No 100 ml Capillary Schmidt 1982 Curettage Infraorbital incision No "Minimal" Not mentioned Hat-El 1986 Excision CaldweII-Luc approach No More than 100 ml Cavernous Clauser 1990 Resection Infraorbital and coronal incision Split cranial bone "Minimal" Cavernous Clauser 1990 Resection Infraorbital and coronal incision Split cranial bone "Minimal" Cavernous
354 J. Cranio-Max.-Fac. Surg. 19 (1991) L. Clauser et al.
Fig.1 Case 1. Preoperative appearance of a 47-year-old female with a right zygomatic haemangioma.
Fig.2 Postoperative appearance of the 3atient.
Fig.3 Coronal computed tomogram shows the lesion invading the zygomatic body and orbital floor ("Honeycomb" appearance).
Fig.4 External carotid arteriogram does not show any pathological blood supply.
al. (1986), had a completely different clinical evolution. In this case, the lesion did not produce facial deformity; it pre- sented as pain involving the entire half of the face. An an- giogram was done in just two cases, neither of which were considered appropriate for a presurgical biopsy. All the patients underwent excision or resection. Just in one case was a homolateral external carotid artery ligation per- formed. The bleeding, as judged by the surgeons, was never defined as dangerous. Three cases required bone graft re- construction in order to obtain normal facial and/or mor- phological contours of the orbital area. None of the au- thors reported long term morphological or functional alter- ations or recurrences. Table 1 (a and b) report the principal clinical findings and treatment data of the haemangiomas of the zygoma report- ed in the literature.
Case Reports
Case 1 A 56-year-old female was referred on account of a hard swelling located in the body of the right malar bone (Fig. 1). This neoformation, present for 4 years, had grown progres- sively in size but was not accompanied by any pain. The clinical examination revealed a round formation, about 3 cendmetres in diameter. It was covered by mobile, nor- mally coloured skin. Palpation revealed a hard mass, fixed to the surrounding bony structures, and no pain was elicit- ed. The patient did not report any paraesthesia in the zygo- matic region. The needle aspirate from the mass was mostly blood. A CT scan of the patient's facial bones was performed (Fig.3) and an arteriogram of the right external carotid which did not reveal any pathological blood supply (Fig. 4). The neoformation was exposed by means of a combined
Haemangioma of the Zygoma J. Cranio-Max.-Fac. Surg. 19 (1991) 355
Fig.5 Bicoronal appraoch. Arrows indicate the neoformation.
Fig.6 Reconstructive stage. Split cranial grafts have been stabilized with microplates and micro lag screws. Onlay bone graft augmented the malar prominence.
bicoronal and right subciliary incision (Fig. 5). The entire mass, including the malar body and the anterolateral por- tion of the orbital floor was removed by means of an ostec- tomy, performed on non-involved bone at the level of the orbital floor, zygomatic shelf and base of the malar tempor- al process. During resection, bleeding was never excessive. For reconstruction, the neurosurgeon harvested a full thick- ness calvarial graft which was then split. The inner cortex was repositioned at the donor site and fixed with wire osteosynthesis. The external cortex, after careful modelling, was used to reconstruct the resected area. The grafts were then fixed with microplates and microscrews. A more pro- nounced zygomatic projection was obtained by means of an additional onlay graft fixed with microscrews. Lateral canthopexy and suturing completed the operation (Fig. 6). A follow-up CT scan and X-ray performed after surgery confirmed the anatomical reshaping of the zygomatic area (Fig. 7). Histological examination of the surgical specimen confirmed the diagnosis of "cavernous" bony haemangio- ma (Fig. 8). The post-operative period was uneventful. The aesthetic result has been satisfactory (Fig. 2).
Case 2 A 35-year-old female came under our observation, present- ing a hard swelling at the left lower lateral orbital angle, 2.5 cm in diameter, which had been present for about one year and pain was elicited on palpation (Fig. 9). The patient underwent a skull X-ray and CT scan of the orbitofacial skeleton (Fig. 10). The external carotid angio-
Fig.7 CT scan and X-ray show the reconstruction of the zygomatic body and orbital floor.
Fig.8 Cavernous haemangioma. Thin-walled large blood vessels (arrows) are interspersed among osseous trabeculae (arrows). HEx 125.
graphy was negative for abnormal blood supply. The needle aspirate was mostly blood. The operation was performed through a bicoronal and left subciliary incision. The resection included the malar body, the floor, lateral wall and the corresponding orbital ridges. The reconstruction was accomplished using a partial thick- ness calvarial graft fixed with micromesh and microscrews. The histological examination of the surgical specimen con- firmed the diagnosis of "bony cavernous haemangioma". The post-operative period was free from complication and the patient had a good morphological and functional re- covery (Fig. 11).
Discussion and Conclusions
Haemangiomas are neoformations of endothelial origin considered by authors to be truly benign neoplasms origi- nating from an initial cellular proliferation which manifests
356 J. Cranio-Max.-Fac. Surg. i9 (1991) L. Clauser et aL
Fig.9 Case 2. Patient with left zygomatic haemangioma. FIg.11 Postoperative view.
Fig.10 Axial computed tomogram shows the haemangioma de- stroying the left zygomatic body ("sun-ray" appearance).
itself in the production of blood vessels (Shira and Guern- sey, 1965). Others instead believe they should be consid- ered to be hamartomas (La Dow et al., 1976). Macroscopically, the bony haemangiomas are subdivided into two groups. The first consists of peripheral angiomas, which originate from periostium vessels and then involve calcified structures secondarily. The second group is repre- sented by central haemangiomas which arise and extend within the spongiosa (Smith, 1959). The histological classification of bony haemangiomas takes into consideration the calibre of the newly formed vessels (capillary, cavernous or mixed haemangiomas), the pres- ence of solid endothelial cell clusters (cellular haemangio- mas), and the eventual preponderance of the connective tis- sue stroma (scirrhous haemangiomas). Such a subdivision does not correlate well with the neoformation clinical be-
haviour, therefore a simple differentiation into cavernous and capillary haemangiomas is preferred (Shklar and Mey- er, 1965; Afshin and Sharmin, 1974). Cavernous haemangiomas are characterized by large, thin- walled, blood filled spaces. These walls are lined by a single layer of flattened endothelial cells which adhere to a con- nective tissue stroma that is also very thin and lies along the long bony trabeculae. On the other hand, capillary haemangiomas appear to be composed of a capillary loop lined with small endothelial cells with a very narrow lumen and are sustained by a con- nective stroma of variable thickness in this latter histotype, one may find solid masses of endothelial cells in which, un- like the case of haemangioendothelioma (angiosarcoma) there are no evident signs of mitotic figures (Unni et al., 1971; Afshin and Sharmin, 1974; Gamez-Araujo et al., 1974). Bony haemangioma symptomatology is variable and de- pends on the site, extension, rate of growth and the mass's haemodynamics. Important diagnostic findings include the presence of paraesthetic areas, a subjective sense of throb- bing and the presence of bruits (Baum et al., 1972; Gamez- Araujo et al., 1974; Schindel et al., 1978; Schmidt, 1982; Hat-El et al., 1986; Hoffman and Israel, 1990). Frequently, neither the patient's medical history nor physi- cal examination lead one to suspect a vascularized mass, unless certain other signs are also present such as a bluish- coloured area, spontaneous haemorrhage, throbbing or a bruit. The natural evolution of the neoformation, along with the symptomatology, can be quite variable. In some cases it may remain stable in its dimensions and symptomatology even for as long as ten years, whereas in other cases its growth, usually intermittent, may lead to quite severe facial deformity (Baum et al., 1972; Afshin and Sharmin, 1974). Frequently the first diagnostic investigation is a plain radio- graphic examination. The bony haemangioma image is not
Haemangioma of the Zygoma J. Cranio-Max.-Fac. Surg. 19 (199I) 357
pathognomonic and may appear similar to that of other neoformations. The majority of haemangiomatous lesions appear as well circumscribed, radiolucent areas traversed by trabeculae. In some cases the margins are not well-defined and the result- ing bone destruction leads one to suspect a primitive or me- tastatic malignant lesion. The bony contour may appear enlarged, thin or eroded, whereas the bony trabeculae are arranged so that they give a radiological image resembling a "sun-ray", "honeycomb" or "soap-bubble" appearance. Much more rarely, instead the centre of the mass may contain well-defined structures whose opacity is greater than that of normal bone (Most, 1985). Therefore, based on the site and radiographic image char- acteristics, the differential diagnosis should include aneu- rysmal bone cysts, arteriovenous fistulae, haemangiopericy- toma, eosinophilic granuloma, giant cell reparative granulo- ma, epithelial and odontogenic cysts, ameloblastoma, hy- perparathyroidism, Paget's disease, fibrous dysplasia, me- tastatic carcinoma, multiple myeloma, meningioma, osteo- ma and osteosarcoma (Caldarelli and Sperling~ 1976; Hoff- man and Israel, 1990). A C T scan employing contrast provides valuable details re- garding its relationship with the surrounding anatomical structures (Ali and Campbell, 1983). The arteriogram confirms the presence or absence of arteri- al supply, venous drainage and haemodynamic characteris- tics of the haemangioma (Zou et al., 1983; Greene et al., 1990). Whenever possible, an explorative needle aspiration should preceed any surgical manoeuvres, even biopsies. The emission of blood under pressure is indicative of a cav- ernous haemangioma, thereby permitting the surgeon to re- evaluate the case and proposed therapeutic procedure (Hayward, 1981). Numerous therapeutic approaches have been described in the literature. The main therapeutic protocols include cortisone adminis- tration (in infants), radiotherapy, injection of sclerosing so- lutions into the lesion, embolism of the main afferent ves- sels and surgery. In the past, radiotherapy was considered the treatment of choice in cases where surgery was not feasible or too muti- lating (Smith, 1959; Macansh and Owen, 1972). Wilde et al. (1966) state that the mature endothelial cells of the haemangioma should be considered radiation resistant. Many negative aspects regarding the use of radiotherapy should be considered. Among them: the difficulty in prop- erly evaluating the exposure necessary for a lesion buried in bone, the problems involved in irradiating a growth area and tooth buds during their growth period, impairment of salivary glands, and last but not least, the transformation in- to a malignant histiotype (Sadowsky et al., 1981). The intralesional injection of sclerosing solutions has fre- quently been employed for haemangiomas of the soft tis- sues of the head and neck (Morgan and Schow, 1974; Chin, 1983). Hayward (1981) published four cases of central angiomas of which two had been successfully treated with intraspon- giosum injection of sodium morrhuate following the surgi- cal creation of a small breach in the bone cortex. According to this author, the procedure should be reserved for cases in which the blood flow within the neoplasm is very slow (in the absence of bruits and important arteriovenous
shunts in the arteriogram). The advantage offered by this method is the destruction of the endothelium while pre- serving bone and its regeneration potential. In cases where there is an abnormal blood supply, emboli- zation may be performed in the course of an arteriogram in an attempt to prevent the risk of intraoperation haemor- rhage. Some authors have proposed embolization as the on- ly therapy, even though long term recurrence cases have been reported (Hoey et al., 1970; La Dow et al., 1976; Braun et al., 1985; Greene et al., 1990). In cavernous haemangioma, angiography does not usually reveal any pathological blood supply, hence embolization is not rational. Moreover, it is not curative even though it can be repeated (Greene et al., 1990). Surgery is probably the most frequently used treatment. The techniques vary from simple curettage of the lesion to a more or less wide bone resection. Once the excision or resection has been performed, recon- struction is mandatory in order to maintain the normal bone morphology. Different solutions have been proposed in the past. If the defect is small, a free or pedicled autolo- gous fat graft can be used to fill in the space (Marshack, 1980), a hydroxylapatite block or fragments (Greene et al., 1990), or layers of Surgicel can be applied beneath the peri- osteum (Schmidt, 1982). The zygoma is a keystone in facial aesthetics. Defects of prominence are immediately recognized, thus curettage is not the right choice for a protruding zygomatic haemangio- ma. The best method of treatment is immediate reconstruction using a bone graft fixed with plates and screws. The newly developed microsystem (Luhr, 1990) has found valid indi- cations in orbital reconstruction. In centres doing craniofacial surgery, in the last ten years, cranial bone has become a frequent source of material for grafting. The generally accepted clinical impression is that there is less reabsorption of cranial bone than when using ribs or iliac crest (Zins and Whitaker, 1983; Kusiak et al., 1985). Moreover, cranial bone has an easy accessibility to the don- or site particularly when working in the craniofacial region. The two cases described have had a two-year clinical and radiological follow-up. Results have been satisfactory both from a functional and morphological standpoint.
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L. Clauser, M.D., D.M.D. F. Meneghini, M.D. M. Riga, M.D. L. Rigo, M.D. Department of Maxillofacial Surgery Centre for Craniofacial Deformities San Bortolo Hospital 36100 Vicenza, Italy
