Primary columellar angiosarcoma: a case report.

Abstract

Angiosarcoma of the head and neck, a rare malignancy, is associated with a high degree of invasiveness and poor survival. A high level of suspicion followed by histopathologic and immunohistochemical studies is warranted in order to arrive at a well-timed and accurate diagnosis. We report the case of a 56-year-old man who developed an unusually small neoplasm in the nasal columella. Rapid diagnosis allowed for simple treatment with surgical excision, a rare circumstance because most of these tumors require extensive surgery. Close long-term follow-up of patients with angiosarcoma of the head and neck is vitally important.

Introduction

Malignant vasoformative (angiogenic) tumors of the soft tissues of the head and neck account for fewer than 0.5% of all sarcomas. (1,2) When they do occur, they have a predilection for the skin and superficial soft tissue. (3) Primary angiosarcomas of the head and neck are most prevalent among elderly men, and they are well known for their aggressive behavior and a poor prognosis. (4) Very few cases of primary angiosarcoma in the nose and paranasal sinuses have been reported in the literature, and little information is available regarding treatment options, survival rates, and prognostic factors. (5)

In this article, we describe a new case of primary angiosarcoma of the head and neck, and we review those reports of this neoplasm that do exist.

Case report

A 56-year-old man was referred to our hospital for assessment of a 1-month history of anterior epistaxis from a nodular lesion in the right nasal vestibule. Examination of the nose revealed that the lesion involved the right lateral margin of the nasal columella. No palpable adenopathy was detected. The bleeding was controlled with topical care, and the lesion was removed 5 days later.

Three weeks later, the patient presented to us with a small (<1 cm) recurrent lesion (figure 1). We performed a wider excision that extended to the cartilaginous plane and removed a small anteroinferior piece of the cartilage. Histopathologic and immunohistochemical studies were positive for CD31 and Ulex europaeus; these findings established a conclusive diagnosis of angiosarcoma (figure 2). The patient underwent further testing with laboratory, serologic, and immunologic studies, as well as computed tomography and a bone scan. The imaging studies detected no evidence of involvement of the nose, paranasal sinuses, neck, lungs, or bone.

[FIGURE 1 OMITTED]

After discussing this case at the weekly meeting of the Oncology Group at our hospital, we decided to follow the patient with close surveillance and to hold off extensive surgery and/or radiotherapy unless the patient exhibited evidence of a new neoplasm. Two years and 7 months later, the patient remained completely asymptomatic.

Discussion

Angiosarcoma of the head and neck is unique. (6) Although the oncogenesis of this type of vascular tumor is still unknown, the tumor appears to be related to an endothelial primitive cell. (7) No definitive etiologic factors have been identified, but associations with previous radiation, local trauma, vascular malformations, and exposure to vinyl chloride have been reported. (4,8,9)

When angiosarcoma of the head and neck occurs, it usually arises from the scalp and the superior half of the face. (10) Primary lesions of the sinonasal cavities are exceedingly rare; when they occur, they usually originate in the maxillary sinus. (5,11-13) We did not find any report in the literature describing an angiosarcoma of the nasal columella.

The clinical variability of these tumors in terms of their size and morphologic characteristics is often confusing. Some masses resemble benign lesions. (10) Both symptomatic and asymptomatic lesions can be flat, raised, or nodular; their colors range from blue to purple to red. Angiosarcomas grow quickly in a centrifugal pattern. Local extensions are often missed because of their subcutaneous extension. (10) This probably explains our finding of recurrence after the first excision. Local and distant recurrences are common; some arise years after an apparent remission. (4-14)

Diagnosis of an angiosarcoma is often difficult and requires a high level of suspicion. Histologic studies are the mainstay of diagnosis. Immunohistochemical studies--including assays for factor VIII-related antigen, CD31, and U europaeus--are necessary to identify the endothelial nature of the neoplastic cells. (10)

The prognosis for patients with angiosarcoma of the head and neck is poor; reported 5-year survival rates range from a low of less than 10% to a high of 30%. (4,7) No definitive prognostic factors have been identified, but tumors larger than 5 to 7 cm have been associated with poorer outcomes. (4,10)

No consensus on treatment has been achieved because no single institution has gained enough experience to establish guidelines. (4,8) Most authors believe that surgery should always be undertaken if possible; however, neck dissection should be performed only when there is evidence of cervical node involvement. (8) The treatment of choice appears to be wide surgical removal combined with external radiotherapy. (4-6,10) Palliative radiotherapy alone can be administered to patients whose tumors are too large to be removed and in those who cannot tolerate surgery. (10)

Studies conducted at the Mayo Clinic suggest that lesions smaller than 1.5 cm may be treated with surgical resection alone, although the authors advise that this recommendation might be premature. (4) For our patient, we chose not to perform extensive surgery or administer radiotherapy in view of the small size of the lesion (< 1 cm) and the absence of locoregional spread. Instead, we monitored the patient with close follow-up, and he exhibited no evidence of recurrence at 2 years and 7 months. In view of this favorable outcome, we believe that simple excision is sufficient for small localized angiosarcomas of the head and neck.

In conclusion, we should keep in mind the counsel of Gallardo et al regarding "the importance of the histological study of any erythematous enlargement of the skin on the nose, especially when it adopts tumorous characteristics." (10) Our patient had an unusually small angiosarcoma of the columella, which we were able to successfully treat with simple excision. Our experience suggests that this strategy could become a valid option for the treatment of lesions smaller than 1.5 cm. Finally, we cannot overemphasize the importance of close follow-up, especially if radiotherapy is not performed.

References

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(11.) Zakrzewska JM. Angiosarcoma of the maxilla--A case report and review of the literature including angiosarcoma of maxillary sinus. Br J Oral Maxillofac Surg 1986;24:286-92.

(12.) Wong KF, So CC, Wong N, et al. Sinonasal angiosarcoma with marrow involvement at presentation mimicking malignant lymphoma: Cytogenetic analysis using multiple techniques. Cancer Genet Cytogenet 2001;129:64-8.

(13.) Sobol SM, Matthieu DE, Jr., Agee JH. Angiosarcoma of the maxillary sinus. Ear Nose Throat J 1990;69:813-18.

(14.) Rosai J, Sumner HW, Kostianovsky M, Perez-Mesa C. Angiosarcoma of the skin. A clinicopathologic and fine structural study. Hum Pathol 1976;7:83-109.

From the Department of Otorhinolaryngology (Dr. Oliveira, Dr. Correia, Dr. Castro, and Dr. Silva) and the Department of Pathology (Dr. Almeida), Centro Hospitalar de Vila Nova de Gala, Portugal.

Reprint requests: Pedro Oliveira, Rua Principal, 1024 30[degrees] Esq., 4405-397 Sao Felix Marinha, Portugal. Phone: [phone]; fax: [phone]; e-mail: [e-mail] or [e-mail]