lilt. J. Oral Maxillofac. Surg , 1986: 15: 357-360

(Key words: sarcoma; angiosarcoma ; malignancy. oral; surgery. oral and maxillofacial)

Maxillary angiosarcoma

NICHOLAS ZACHARIADES AND PANAYIOTA ECONOMOPOULOU

Oral and Maxillofacial Department, "Apostle Paul's" Accidents Hospital , Kiflssia, Athens, Greece andDepartment of Oral Pathology, Dental School, National University, Ath ens, Greece

ABSTRACT - A malignant tumor of mesenchymal origin, angiosarcoma, isa rare entity deriving from the endothelium of the blood vessels, that veryinfrequ ently is encountered in the jaws. 46 such cases have only beenreported.

(Accepted for publication Zil March 1985)

Angiosarcoma is a very rare tumor con­sidering the abundance of blood vessels inthe human body. It is known to develop asa primary tumor or from irradiated benignhemangioma1.6.16,21,23,27,31 . It usually affectsyounger people' . Although the cutaneoustype is usually encountered in the face andscalp, the intraosseous type is seldom re­ported in the jawsl,6,26,36. Because of its rarity,it is seldom included in difTerential diag­nosis and almost never diagnosed clinically.Pyogenic granuloma is the more likely diag­nosis. The central type will appear as a radi­olucent lesion in radiographic examination,which in some cases may simulate a benignlytic lesion ',23,26,31. Even microscopically, it isnot always easy to distinguish between thebenign and the malignant forms , except forthe atypical endothelial cells that character­ize the latter, Yet it is still difficull to dis­tinguish between hemangioendotheliosarco­rna and hemangiopericytosarcoma, and inmore anaplastic forms, between these tu­mors and leiomyosarcoma or fibrosarco-

ma S,22,24,27,28,33,36,39. The tumor spreads by localinvasion and metastasizes mainly by thebloodstream. The best treatment consistsof wide surgical resection followed bychemotherapy. Prognosis is poor; few pa­tients survive longer than 2 years. Pulmon­ary metastasis is the main cause ofdea th 1.14,21,23,26,28,31,36,39 .

Report of a caseA 68-year old woman was admitted to the hospi­tal because of a painless swelling of the left infra­orbital region (Fig. I) of 2 months duration. Theswelling was associated with infraorbital nervehypesthesia and could also be palpated . intra­orally at the corresponding muccobuccal fold.No regional lymph nodes were palpable. Radio­graphic examination (Fig. 2) revealed that a dif­fuse rad iolucency occupied the area between theupper left central incisor and the canine, alsoextending towards the premolars. The left lateralincisor had been extracted and the left centralincisor and canine had had root canal therapiesperformed a month previously. (There was noevidence of caries in the central incisor.) The den-

358 ZACHARIADES AND ECONOMOPOULOU

Fig. J. Appearance of the patient upon admis­sion. Note the swelling of the left infraorbitalregion.

tal work was carried out because these teeth werepainful and the swelling of the -area was con­sidered to be an abscess originating from them.Antibiotics had been prescribed, but the swellingdid not subside. Suspecting a malignancy, it wasdecided to admit the patient for a detailed clinicaland laboratory examination, which provedwithin nonnal limits. 2 days later, under local

. anesth esia, a mucoperiosteal nap was raised anda biopsy was performed. The pathologist de­scribed sheets of mesenchymal cells that re­sembled endothelial cellsand newly fonned fibro­blasts. There were a number of mitoses as wellas pleomorphism of cells. Vascular channels werealso seen in the specimen (Figs. 3, 4). The diag­nosis was angiosarcoma; the patient, however,discharged herself following diagnosis and hasnot been seen since.

We have tried to locate her and we learned thatshe visited a specialized clinic abroad, where anew biopsy was taken, which verified the original

"..' . ... _ ;.... . - ~

4' • •

'. , ,, ~

'-" ... .. . -

Fig. 2. Radiograph of the involved area showing adiffuse radiolucency extending between the upperleft central incisor and the premolars of the sameside.

Fig . 3. Photomicrograph showing atypical capil­laries lined with anaplastic endothelial cells. Thelumina of most of the capillaries are filled withneoplastic cells. (H.&E. x 160.)

MAXILLARY ANGIOSARCOMA 359

III10 20 30 40 50 60 70

age

Fig. 5. Age distribution of the cases presented,when indicated in the reports.

987

1Il 6~ 5:34

32

1 ............~~~~~.....~.......~.....~~_

Fig. 4. Irregular vascular channels lined with pro­liferating endothelial cells that ar e pleomorphicand show large hyperchromatic nuclei. (H.&E.x 63.)

dia gnosis of angiosarcoma. (We were able to secthe pathologist's report but the micro scopic slideswere not available.) The tumor was cons ideredinoperable and chemotherapy was adm inistered .The patient stayed abroad for approximately 6months. There was some remission of her symp­tom s for up to about a year, after which shereturned home . At that time she started to de­velop dyspnea, back pain and headaches. Shevisited the same clinic abroad, where recurrenceof the tumor was verified, associated with widespread metastasis that involved both lungs, thebra in, the liver and the lumbar spinal column.No further biopsies were taken this time andaccording to her relatives chemotherapy and ir­radiation were administered. The patient stayedabroad for a month and was brought back hometo die, 15 days later, approximately 2 years afterthe initial biopsy was taken by us.

DiscussionAngiosarcoma is a very rare neoplasm; only46 cases have so far been reported in theoral cavity and the surrounding tis­suesl-l.7-21.2+-26,29-J2,.l-l-39. The age of the pa-tients, when recorded, varied, the youngestbeing I-day old' and the oldest (current re­port) 68 (Fig.5). The male/female ratio isalmost 1:2 and the mandible and the maxillaare affected with approximately the samefrequency. There is no difference between

the right and the left side of the face. Theradiographic picture, when published in re­ports, is always osteolytic. The best treat­ment appears to consist of wide surgicalremoval of the tumor regardless of the com­bination of irradiation. The 2-year survivalrate is 50% and the 5-year rate is only 25%.We had the opportunity to encounter an­other such rare case in OUr Institution".

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