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CASE REPORT
Year : 2008  |  Volume : 20  |  Issue : 3  |  Page : 116-120 Table of Contents   

Angiosarcoma in the mandible: Report of a rare case


Department of Oral Medicine and Radiology, Kothiwal Dental College, Research Centre and Hospital, Moradabad - 244 001, Uttar Pradesh, India

Date of Web Publication16-Jun-2009

Correspondence Address:
M Srinivasa Raju
Department of Oral Medicine and Radiology, Kothiwal Dental College, Research Centre and Hospital, Moradabad - 244 001, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.52780

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   Abstract 

Angiosarcoma in the oral cavity, pharynx, or paranasal sinuses comprises less than 5% of the total cases of neoplasms. Age of presentation varies from 1 day to 67 years. Intraorally, the most preferred site is the mandible and the least common is the maxilla.
Histologically, all angiosarcomas show a wide spectrum of differentiation of endothelial cells. Treatment is surgery in combination with radiation therapy. However, the prognosis for patients is generally poor, with one half of the patients dying within 15 months of diagnosis. Here is a rare case of angiosarcoma of the body of the mandible reported in an 80-year-old male patient.

Keywords: Angiosarcoma, mandible, endothelial cells


How to cite this article:
Vashishth S, Raju M S, Reddy S, Suma G N, Raviprakash S M, Singh N N. Angiosarcoma in the mandible: Report of a rare case. J Indian Acad Oral Med Radiol 2008;20:116-20

How to cite this URL:
Vashishth S, Raju M S, Reddy S, Suma G N, Raviprakash S M, Singh N N. Angiosarcoma in the mandible: Report of a rare case. J Indian Acad Oral Med Radiol [serial online] 2008 [cited 2021 Sep 19];20:116-20. Available from: https://www.jiaomr.in/text.asp?2008/20/3/116/52780


   Introduction Top


Angiosarcoma in the oral cavity, pharynx, or paranasal sinuses comprises less than 5% of the total cases of neoplasms. [1],[2]

Most cases of intraoral lesions have shown involvement of the mandible, with the maxilla being affected far less frequently. Others are the parotid gland, lips, tongue, floor of the mouth, cheek and palate, as well as the antrum. The site in the discussed case also lies within the most commonly affected region. [1]

Prognosis for patients with angiosarcoma is generally considered rather poor, although tumor size, site, and the histopathologic grade may influence survival. [1],[3],[4],[5]

Investigations have indicated that one half of the patients die within 15 months of diagnosis, with only 12% surviving 5 years or longer. [1],[4],[5],[6]

An 80-year-old male patient reported to the outpatient department with a chief complaint of swelling over the left lower third of the face for 3-4 months [Figure 1].

After complete history and all investigations confirmed histopathologically, a diagnosis of angiosarcoma of the mandible was made.


   Case Report Top


An 80-year-old patient reported to the outpatient department with a history of a 4-month-old swelling in the left mandibular molar region, which was spontaneous in onset and grew rapidly to reach to the present size. Swelling was asymptomatic. History for numbness, discharge, bleeding, ulceration, difficulty in mastication, and presence of any other swelling was negative.

There was a history of fall from the roof and injury of his left arm 17 years back. Other medical, surgical, and dental history was not significant.

On general examination, the patient was poorly built and debilitated (weight 42 kg).

Extraorally, solitary swelling present on the left lower facial region extending from the external lip commissure to about 2 cm in front of the angle of the mandible and about 3.5 cm from the Ala-tragus line to about 0.5 cm below the inferior border of the mandible was present. The swelling was about 5 4 cm, rhomboidal, with ill-defined borders and there was no change in the surface color and temperature [Figure 1] and [Figure 2].

On palpation, the inspectory findings were confirmed. The swelling was bony hard, skin over the swelling was pinchable, and there was no tenderness.

A step in the continuity of the left inferior border of the mandible showed a pathological fracture (i.r.t 34, 35 regions). There were no palpable pulsations.

Intraorally, the lobulated swelling extended from the 33 to the 37 region, obliterating the vestibule and causing the buccolingual expansion of 4 cm.

Overlying mucosa was normal, without any visible pulsation.

On palpation, the swelling was nodular, firm to hard in consistency, non-tender, non-compressible, non-pulsatile, and there was no sensory loss [Figure 3] and [Figure 4].

The left and right submandibular lymph nodes were palpable and the left submandibular lymph node was about 2-3 cm in size, soft to firm in consistency, mobile, and non-tender.

A provisional diagnosis of squamous cell carcinoma was made with differential diagnosis of:

  • Osteosarcoma.
  • Intraosseous carcinoma.
  • Angiosarcoma.
  • Metastatic carcinoma.


The patient was subjected to:

  • Routine blood examination.
  • Serum alkaline phosphate.
  • Serum calcium estimation.
  • Conventional radiological examinations.
  • Histopathological examination.
  • Color Doppler.


All the values were within normal physiological limits.

Radiographic examination of OPG and occlusal radiograph revealed the presence of a destructive lesion in the body of the mandible (i.r.t. 34-37) of size about 3.8 2.9 cm. The borders were ill defined, with lack of cortication and infiltrative. The shape was irregular.

The lesion was homogeneously radiolucent, with few residual islands of bone present, with no evidence of bone production, giving a characteristic "moth eaten" appearance to the lesion. Pathological fracture of the inferior border of the mandible was also appreciated.

The integrity of the alveolar canal in the site of the lesion was lost and inferiorly displaced [Figure 5],[Figure 6],[Figure 7].

High-resolution ultrasonography and color Doppler of the swelling with a multifrequency 10 MHz probe revealed a large ill-defined heteroechoeic mass in the left side of the mandible with an internal vascularity that measured about 7 6 cm in size.

An arterial feeder at the lateral part of the mass could be due to the inferior alveolar artery, which was also encased by the mass [Figure 8] and [Figure 9].

Histopathological examination revealed a superficial hyperplastic parakeratinized squamous epithelium.

Deep areas of connective tissue exhibiting sheets of round malignant endothelial cells of varying sizes were seen. Within these sheets of cells a few giant cells were seen.

Nuclei appeared vescicular with prominent nucleoli.

Capillary-like spaces lined by plum endothelial cells showed a few mitoses [Figure 10] and [Figure 11].

The overall histopathological features were suggestive of angiosarcoma.

After correlating clinical, radiological, (includes color Doppler) and histopathological findings, a final diagnosis of angiosarcoma was made and the patient was referred immediately to the Cancer Institute for further evaluation and management.


   Discussion Top


Angiosarcoma of the head and neck usually affects the scalp of the elderly men. Uncommonly, the oral cavity is the site of the primary lesion. [1],[2] In fact, a primary angiosarcoma in the oral cavity, pharynx, or paranasal sinuses comprises less than 5% of the total reported cases of neoplasm. A literature review of angiosarcomas in the oral region has shown an age of presentation ranging from 1 day to 67 years. [1] In this case, the age of diagnosis is 80 years, which is more than the age at which most primary oral angiosarcomas are detected. Mc Carthy and Pack hypothesized that these tumors arise from the capillaries of the granulation tissue in traumatized areas. [7]

Most cases of intraoral lesions have shown involvement of the mandible, with the maxilla being affected far less frequently. Others are the parotid gland, lips, tongue, floor of the mouth, cheek and palate, as well as the antrum. The site in the discussed case also lies within the most commonly affected region.

Angiosarcoma is a clinically heterogeneous group of soft tissue sarcomas that exhibit endothelial cell differentiation.

Angiosarcomas can be divided into four clinically distinct groups according to the site where they arise and their manner of presentation:

  • Cutaneous tumors occurring without pre-existing lymphoedema.
  • Cutaneous tumors arising in lymphadematous tissues.
  • Angiosarcoma of the breast.
  • Angiosarcoma of the deep tissues.


Cutaneous angiosarcomas are usually found in the head and neck area of elderly patients, particularly in the scalp and face.

These tumors have the appearance of ill-defined plaques or diffusely infiltrative nodules that often extend to subcutaneous tissues. Angiosarcomas arising in lymphadematous extremities are either congenital or acquired. Their clinical behavior is aggressive and death as the result of widespread metastasis to internal organs is the rule.

Angiosarcomas of the breast are ill-defined hemorrhagic nodules and masses. They occur only in women and mostly in those who are menopausal (30-50 years of age).

Angiosarcomas of deep soft tissues are rare and present as combined hemorrhagic and necrotic masses and are mostly found deep in the extremities or in the retroperitonium.

Histologically, all angiosarcomas show a wide spectrum of differentiation. The well-differentiated tumors show irregular anastomosing, blood-filled vascular channels lined by variably atypical endothelial cells that often show papillary intraluminal tufting.

Less-differentiated tumors may show solid sheets of undifferentiated cells that may resemble a carcinoma or a lymphoma.

Treatment of angiosarcoma is greatly complicated by the diffuse infiltration with which these tumors are typically seen. Various interventions have been attempted to stem the disease process. [8],[9]

In a recent detailed treatment analysis, it has been indicated that surgery in combination with radiation therapy affords the most favorable opportunity for the control of angiosarcoma. [1],[3],[8],[9]

Prognosis for patients with angiosarcoma is generally considered rather poor, although tumor size, site, and the histopathologic grade may influence survival. [1],[3],[4],[5]

Investigations have indicated that one half of the patients die within 15 months of diagnosis, with only 12% surviving 5 years or longer. [1],[4],[5],[6]

However, patients presenting with lesions less than 10 cm in diameter respond better to therapy.

Recurrence after local treatment manifests itself primarily in local failure. Yet, distant metastasis is not insignificant in the post-treatment group. [10],[11]

These factors, both local and distant spread, are reflective of the highly aggressive nature of this illness and serve to explain the poor survival statistics.

 
   References Top

1.Loudon JA. Angiosarcoma of the Mandible: Case report and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:471-6.  Back to cited text no. 1    
2.Frick WG, Angiosarcoma of tongue: Report of a case. J Oral Maxillofac Surg 1988;46:496-8.  Back to cited text no. 2    
3.Mark RJ, Poen. Angiosarcoma: A report of 67 patients and a review of literature. Cancer 1996;77:2400-6.  Back to cited text no. 3    
4.Mentrez T. Cutaneus Angiosarcoma of the face: Clinicopathological and immunohistochemical study of a case resembling rosacea clinically. J Am Acad Dermatol 1998;38:837-40.  Back to cited text no. 4    
5.Holden CA. Angiosarcoma of the face and scalp, Prognosis and treatment. Cancer 1987;59:1046-57.  Back to cited text no. 5    
6.Haustein UF. Angiosarcoma of the face and scalp. Am J Dermatol 1991;30:851-6.  Back to cited text no. 6    
7.Triantafillidou K, Lazaridis N, Zaramboukas T. Epitheliod angiosarcoma of the maxilla: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94:333-7.  Back to cited text no. 7    
8.Fetcher RE. Atlas of tumor Pathology; Tumors of bone and joints. Washington DC: Armed forces Institute of Pathology; 1992. p. 138.  Back to cited text no. 8    
9.Kemposa RL. Atlas of tumor Pathology; Tumors of soft tissue. Washington DC: Armed forces Institute of Pathology; 2001. p. 99.  Back to cited text no. 9    
10.Daiani S. Primary Angiosarcoma of the parotid gland arising from benign congenital hemangioma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003,96:66-9.  Back to cited text no. 10    
11.Orchard GE. Immunocytochemistry in the diagnosis of Kaposi sarcoma and Angiosarcoma. Br J Biomed Sci 1995;52:35-49.  Back to cited text no. 11    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]



 

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