Year : 2008 | Volume
: 20 | Issue : 1 | Page : 19--22
Angiosarcoma of the mandible
MC Shashikanth1, Neelkamal Sharda2, IM Ali1, U Deepak1, Sheeba Mohindra3,
1 Department of Oral Medicine and Radiology, College of Dental Sciences Davangere, Karnataka, India
2 Department of Oral Medicine and Radiology, Santosh Dental College, Ghazibad, Uttar Pradesh, India
3 Department of Oral Medicine, BRS Dental College, Haryana, India
77 Pushpanjali, Vikas Marg New Delhi 110092
Angiosarcoma is a rare malignancy of vascular endothelium which may arise from either blood or lymphatic vessels. These tumors are aggressive with a tendency to recur locally and also have a high rate of lymph node and systemic metastases. Angiosarcomas occur more frequently in skin and soft tissue. Occurrence of a primary or metastatic angiosarcoma in the oral cavity is extremely rare. The purpose of this article is to document a case of primary angiosarcoma in the right mandible of a 20 year old woman. The tumor presented as a rapidly enlarging lobulated soft tissue mass which was associated with an irregular radiolytic defect in the right mandible. Histological features of the lesion were suggestive of a high grade angiosarcoma. The patient was successfully treated with a combination therapy involving surgery and chemotherapy. There were no signs of tumor recurrence or metastasis after one and a half year of follow up.
|How to cite this article:|
Shashikanth M C, Sharda N, Ali I M, Deepak U, Mohindra S. Angiosarcoma of the mandible.J Indian Acad Oral Med Radiol 2008;20:19-22
|How to cite this URL:|
Shashikanth M C, Sharda N, Ali I M, Deepak U, Mohindra S. Angiosarcoma of the mandible. J Indian Acad Oral Med Radiol [serial online] 2008 [cited 2022 Aug 17 ];20:19-22
Available from: https://www.jiaomr.in/text.asp?2008/20/1/19/44356
Angiosarcomas are malignant tumors composed of endothelial cells of either blood or lymphatic vessel origin. , The cells of these tumors demonstrate many of the functional or morphological properties of normal endothelium.  Histologically, angiosarcoma may vary from highly differentiated tumors that resemble hemangiomas to those whose aplasia makes them difficult to distinguish from carcinomas or melanomas. Consequently, the literature is replete with various terms such as hemangioendothelioma, lymangioendothelioma, hemangioblastoma, lymphangio-sarcoma, and hemangiosarcoma that attest to the wide morphological spectrum of this tumor. ,
Angiosarcomas are collectively one of the rarest forms of soft tissue neoplasms. They comprise less than 1% of all sarcomas, which by themselves constitute only 1% of all cancers. ,
The tumor has been described in many different locations such as spleen, long bones, liver, mammary glands, striated muscle, skin and subcutaneous tissue. Its occurrence as a primary or metastatic tumor in the oral cavity is extremely unusual. Only 4% cases have been found in the pharynx, oral cavity or paranasal sinuses. [4, 50% of cutaneous angiosarcoma are known to occur in the head and neck region; however the intraossoeus type is seldom reported in the jaws. Because of its rarity, angiosarcoma is seldom included in the differential diagnosis of the jaw swellings and almost never diagnosed clinically. ,
This rarity of localization prompted us to report a case of primary angiosarcoma in the right mandible of a 20 year old female patient.
A 20 year old female patient presented to the department of Oral Medicine and Radiology, College of Dental Sciences, Davangere, Karnataka, India, with a mild, intermittent throbbing type of nonradiating pain associated with a swelling in the right mandible since 20 days. The swelling was small in size at the time of onset, however it progressed rapidly to attain the present size. Patient gave no history of previous such swellings or presence of any lumps or swellings elsewhere in the body. There was no history of trauma but the patient did report loss of weight and appetite over the last one month.
On general examination, patient was found to be moderately built and nourished with a normal skin and gait. There were no signs of pallor, icterus, cyanosis and edema. A solitary right submandibular lymph node was palpable, tender, measured 1.5 x 1.5 cm in size; it was soft to firm in consistency and mobile. The vital signs were within the normal limits.
Extra orally, facial asymmetry could be appreciated because a diffuse swelling was present along the right mandible. The swelling was roughly oval with a smooth surface. On palpation, tenderness was elicited and the overlying facial skin could be lifted over the swelling. The lips were incompetent and the anterior portion of the intra-oral swelling could be seen [Figure 1].
Intra-oral examination revealed the presence of a solitary swelling in the right mandible extending buccally from mesial of 32 to distal of 47 anteroposteriorly and from the level of the occlusal plane to the depth of the vestibule superoinferiorly. The swelling measured approximately 7 x 6.5 cm in size. The swelling was whitish pink in color, and spherical in shape with lobulated surface and ill defined edges.
On palpation, the swelling was mildly tender and the inspectory findings regarding the size, shape and extent were confirmed. The swelling was soft in consistency and there was bleeding on manipulation. There were no signs of fluctuation, translucency, reducibility, compressibility, pulsatility and the swelling appeared to be arising from with in the bone.
Considering the patient's young age, the rapidity of growth of the swelling with an associated feature of loss of weight and appetite, allied with the clinical presentation of an ill defined lobulated mass associated with bleeding on manipulation, a provisional diagnosis of a sarcoma involving the right mandible was made.
Angiosarcoma, chondrosarcoma, osteosarcoma and Ewing's sarcoma were considered for differential diagnosis. Among other malignancies squamous cell carcinoma and a metastatic tumor were considered for differential diagnosis.
Investigations included a complete hemogram, radiographs and excisional biopsy. Hemogram showed a normal blood count with an elevated Erythrocyte sedimentation rate (ESR). A complete physical examination ruled out any other tumor site.
The panoramic view, posterio-anterio (PA) mandible and the occlusal radiographs revealed the presence of a radiolucent lesion with ill defined and irregular margins which was seen 1-1.5 cm apical to 43, 44, 45 and 46. The radiolucency measured 2.5 x 3 cm in size. The lesion had caused thinning of the inferior border of the mandible and there was widening of periodontal ligament space with respect to 43, 44, 45 and 46 and loss of lamina dura with respect to 44, 45 and 46. The radiographic findings were also suggestive of a malignant condition [Figure 2].
Patient was surgically treated under general anesthesia and a hemimandibulectomy was performed extending from the region of 33 to 48. The excised specimen was subjected to microscopic evaluation and the histologic features were suggestive of a high grade angiosarcoma. Patient was then referred to Bangalore Cancer Institute where she was given 3 rounds of chemotherapy. Patient was reevaluated after a period of 1½ years following surgery and there was no evidence of any recurrence or metastatic disease at the time of the follow up visit [Figure 3] and [Figure 4].
Angiosarcoma is a rare tumor and oral presentations of this condition are even rarer.  These tumors are malignancies of the vascular endothelium characterized by the formation of irregular vascular channels lined by one or more layers of atypical endothelial cells, often of immature appearance, and accompanied by solid masses of poorly differentiated or anaplastic tissue.  The exact etiology of the tumor is not known. However, trauma, longstanding lymphedema and radiation appear to be contributory factors.
These tumors tend to occur in the elderly, but can be seen in any age group.  Zacharides et al . discussed 46 cases of primary angiosarcoma of the oral cavity, among these 32% of the patients were younger than 19 years, 28% were between 26 and 42 years and 17% between 50 and 67 years.  The youngest patient was 1 day old and the oldest was 67 years old. In our case, the patient was a 20 years old female.
The incidence of angiosarcomas is generally reported to be almost the same for males and females. However, Bundens and Brighton have reported that lesions in the bone occur almost twice as often in males as in females while Zacharides et al. reported a female predominance. 
Angiosarcomas can be solitary or multicentric.  One third of the cases of oral angiosarcomas are primary bone lesions with most affecting mandible and these are commonly unifocal. , The tumor in the presented case involved the right mandible. The other sites of reported oral involvement include the parotid gland, lips, tongue, floor of the mouth, cheek, palate and the antrum.  Metastatic oral angiosarcomas are exceedingly rare lesions, and these have a tendency to localize in the gingiva. 
As the appearance of these tumors in the oral cavity is quite alarming, 80% patients seek help with in the first month.  Our patient reported with a swelling associated with mild pain. Zacharides et al . also reported swelling or growth as the principle chief complaint, followed by facial pain while the most common presenting features in Zakrzewska's series was spontaneous bleeding after trivial trauma. 
The clinical appearance of the oral lesions usually consists of an epulis type sessile mass which may be both soft and compressible or of moderately firm consistency. Some lesions may be nodular and lobulated, whereas others are more diffuse. The surface may be ulcerated, and the color of the mass may be normal or may have a purplish red or violaceous hue.  Our case showed a rapidly enlarging lobulated soft tissue mass, a finding which was consistent with previous observations.
The tumor also commonly has superficial ulceration and a bleeding tendency due to secondary trauma. These features may lead to a misdiagnosis of pyogenic granuloma, "pregnancy tumour" or peripheral or central giant cell reparative granuloma. Kaposi's sarcoma, melanoma, undifferentiated neoplasia and metastatic tumor must also be ruled out. 
The radiographic appearance of angiosarcoma involving the facial bones is generally that of a destructive osteolytic lesion.  The radiographic changes in our patient also revealed an irregular radiolytic defect in the right mandible. However, radiopaque areas and a mild periosteal reaction have also been noted in rare instances. Jaw lesions may show widening of periodontal ligament and erosion of the alveolar bone, sometimes to the extent that it results in a "tooth floating in space" appearance. 
Angiosarcomas cannot be diagnosed easily on the basis of clinical and radiologic findings. The diagnosis can be made only by histologic and histochemical criteria. 
Histologically, angiosarcomas are characterized by irregular vascular channels lined by atypical immature endothelial cells. The margins are ill defined with infiltration into the soft tissues.  From a microscopic view point, the differential diagnosis may include pyogenic granuloma, hemangioma, hemangioendothelioma, papillary endothelial hyperplasia, angiolymphoid hyperplasia with eosinophilia, Kaposi's sarcoma or malignant melanoma.  The immunohistochemical findings help exclude other diagnostic possibilities by demonstrating positive immunoreactivity for factor VIII related antigen, vimentin, CD-31, CD37 and negative reactivity for smooth muscle action, desmin, keratin, and oestrogen/progesterone receptors. ,
Angiosarcomas spread by local infiltration and by early hematogenous metastasis. Lymphatic spread may also occur. The literature reports that angiosarcomas of the bone are less aggressive than their soft tissue counterparts. It is also important for clinicians to remember that head and neck angiosarcoma may also be metastatic in origin. Carr and Green reported that head and neck was a location to which an angiosarcoma metastasized in almost 1/3 rd of the cases. 
Treatment of angiosarcomas is greatly complicated by the diffuse infiltration with which these tumors are typically seen. Various interventions have been attempted to stem the disease process. In a detailed treatment analysis, it has been indicated that surgery in combination with radiation therapy affords the most favorable opportunity to control angiosarcoma while some authors claim wide surgical resection followed by chemotherapy is the best line of treatment. Various agents like doxorubicin, etc. have been used in these patients. ,, Radical neck dissection is used only for those patients with detectable metastasis to cervical lymphnodes.  Our patient was successfully treated with a combination therapy involving surgery and chemotherapy.
The prognosis for patients with angiosarcomas is generally considered to be rather poor, although tumor size (and hence, stage), site and the histopathologic grade may influence survival.  These tumors have a strong tendency to recur locally and to metastasize. Patients with metastases have the poorest prognosis. The main cause of death in these patients is pulmonary metastases. Investigations have indicated that one half of the patients die within 15 months of diagnosis, with only 12% surviving 5 years or longer.  The survival rate for oral and jaw angiosarcomas is better than that for angiosarcomas in the rest of the body, this may be a reflection of low grade malignancy in this area or a shorter time between diagnosis and treatment.  In our case, the patient did not show any signs of tumor recurrence or metastasis after one and a half year of follow up.
Angiosarcoma of the head and neck is a rare malignancy, and it is associated with a high degree of invasiveness and poor survival. A high level of suspicion followed by histopathologic and immunohistochemical studies are warranted in order to arrive at a well-timed and accurate diagnosis. An early diagnosis can aid in providng early treatment to the patient which is of vital importance in improving the chances of patient's survival. Finally, the importance of a close and regular follow up of these patients cannot be overemphasized.
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