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A Dusky-Red Rash on the Forehead and Scalp of an Elderly Patient – A Case Report of Angiosarcoma
BULLETIN FOR MEDICAL PRACTITIONERS
Dr Loh Teck Hiong, Registrar
Dr Chua Sze Hon, Consultant
Dr Tan Suat Hoon, Consultant
National Skin Centre
Introduction
Angiosarcoma is a rare malignant tumour of vascular origin that affects predominantly the skin and soft tissues. Its benign initial appearance and insidious onset often translates to a delayed diagnosis in many cases. Biologically, it is an aggressive malignancy with a poor prognosis when diagnosed late. It is hoped that early diagnosis and intervention will result in a better overall prognosis. We describe a case seen at our Centre recently to raise awareness of this condition when evaluating an elderly patient with a persistent or progressive dusky-red rash on the forehead and scalp.
Case Report
The patient, a 60-year-old Chinese male presented to the National Skin Centre with a three month history of a progressive red rash on the scalp and forehead (Fig. 1). The rash was previously managed by his family physician as ‘eczema’ and ‘herpes zoster’. There was no associated pruritis or pain. There was no history of previous trauma or irradiation to the affected area. On examination, he had a bruise-like dusky red plaque over his right frontal scalp measuring approximately 7cm x 9cm. The borders of the lesion were ill defined and lesion extended into his right upper eye-lid and left forehead. There was no associated cervical lymphadenopathy.
A skin biopsy done revealed bizarre vascular spaces in the upper dermis. The vascular spaces formed an irregular anastomosing network that permeated between the collagen bundles and were lined by a single layer of atypical endothelial cells (Fig 2.). The histology confirmed the diagnosis of a well-differentiated angiosarcoma. Upon diagnosis, the patient was referred to the National Cancer Centre for co-management. As the tumour was too extensive for complete surgical excision, he was treated with a course of wide-field radiotherapy with complete clinical clearance. A recurrence of the malignancy with right cervical lymphadenopathy however occurred two months later for which the patient is currently undergoing chemotherapy.
Discussion
The term angiosarcoma presently covers what was previously termed as haemangiosarcoma, lymph-angiosarcoma and malignant angioendothelioma. This terminology is deemed more precise as the exact origin of the malignant endothelial cells is not known. The occurrence of angiosarcoma is very rare - it constitutes about two percent of soft tissue sarcomas, which in turn make up less than 1% of all cancers 1. It usually occurs in the sixth and seventh decades of life and is more common in men. Childhood cases are rare but have been reported 2. The skin and superficial soft tissues are by far the commonest primary sites affected; with the deep tissues and viscera such as the heart, liver, spleen, lung and kidney being very rarely involved. The head and neck regions are most commonly affected, in particular the scalp, temple and cheek.
The etiology is still largely unknown. Angiosarcoma is associated with several conditions including chronic lymphoedema, post-irradiation, trauma and exposure to vinyl chloride 3 . More recently, mutation in the p53 tumour suppressor gene 4, human herpes virus type 8 5 , and defects in normal regulation of angiogenesis 6 have been shown to play roles in the pathogenesis of the tumour.
Histologically, there are different grades of differentiation although different biopsies from the same tumour, or even different fields within the same biopsy may show varied differentiation. This complicates the accurate grading of the tumour. In well-differentiated lesions, there are irregular anastomosing vascular channels which permeate between collagen bundles (dissection of bundles), and are lined by a single layer of atypical endothelial cells. In moderately differentiated lesions, there is an increase in the number of malignant endothelial cells forming intra-luminal papillary projections. In poorly differentiated lesions, there are solid sheets of large pleomorphic cells with no evidence of luminal differentiation. The histopathological changes typically extend well beyond the limits of the apparent clinical lesions (dermal spread).
Clinically, almost all cutaneous angiosarcomas arise under three settings: 1. Idiopathic angiosarcoma of the head and scalp of the elderly (in more than 50% of cases), 2. Angiosarcoma secondary to chronic lymphoedema (about 10% of cases) and 3. Angiosarcoma occurring in previously irradiated sites.
The characteristic appearance is that of a bruise-like dusky red, purplish macule or plaque with variable degree of swelling, and ill-defined edges that merge with the normal skin. Some lesions may be nodular. Larger lesions tend to be associated with ulceration and bleeding. The lesions are often multi-focal and satellite lesions may be present. Most patients with cutaneous angiosarcoma are asymptomatic which contribute to the delayed presentation. Some unusual presentations that have been reported include rosacea-like 7 , recurrent angioedema 8 , eyelid xanthoma-like plaque with ptosis 9 , scarring alopecia 9 and chronic facial oedema. The variable clinical presentation often delays the definitive diagnosis. Angiosarcomas are often misdiagnosed initially as cellulitis, herpes zoster, bruise/haematoma, contact allergy, port-wine stain or Kaposi sarcoma.
The tumour grows rapidly. There is both horizontal and vertical growth. The horizontal growth has a dermal component that typically spreads well beyond the margins of the apparent clinical lesion. The vertical infiltrative growth can extend into the underlying fat, muscle, bone, and even brain contributing to much of the morbidity and mortality at the advanced stage.
Angiosarcoma has the highest incidence of lymph node metastasis amongst the sarcomas (13.5%). Distant metastasis also occurs frequently and the commonest sites are the lungs, liver and bones. Late local recurrence and distant metastasis can happen years after apparent clinical remission.
Current treatment includes wide local surgical excision, radiotherapy and systemic chemotherapy. Radical surgical excision, followed by a course of adjuvant radiotherapy may offer a better prognosis than either surgery or radiotherapy alone 1,11 . However, the delayed diagnosis coupled with the highly infiltrative nature of the tumour often renders the lesion too extensive for primary resection. In such cases, wide-field radiotherapy is beneficial 11 . In cases where surgical and radiotherapy has failed, or where distant metastases has occurred, chemotherapy with agents such as cyclophosphamide, actinomycin, doxorubicin, methotrexate are used as a palliative treatment. Chemotherapy, once regarded as ineffective 1 is now a promising option with newer agents such as alpha interferon 2a with 13-cis-retinoid acid 12 , and paclitaxel being shown to be effective 13 .
The prognosis of the tumour is poor. Death is normally due to extensive uncontrollable local disease, or distant metastasis. In the largest series so far, Holden 11 demonstrated a dismal 5 year survival rate of only 12% in a follow-up of 72 patients. Small tumour size is the only consistently reported favorable prognostic factor 1,11 . Histological grade has not been shown consistently to be of prognostic significance 1,11 . This is unusual in sarcomas; and may be due to difficulty in accurately grading the tumour as both well differentiated and poorly differentiated histological features can often be found in the same tumour at different sites.
Conclusion
A bruise-like dusky red-purplish skin lesion on the forehead in the elderly may be an angiosarcoma. Awareness coupled with a high degree of suspicion is the key to an early diagnosis of this rare but aggressive vascular malignancy. Early diagnosis is essential, as curative surgical excision is possible only with very small early tumours.
References
- Mark RJ, Poen JC, Tran LM et al. Angiosarcoma: A report of 67 patients and a review of literature. Cancer 1996:77:2400-2406.
- Kaul BK, Sinhai CD, Rastogi BL. Hemangiosarcoma in Children: two case reports. Am Surg 1974;40: 643-6.
- Rubin DF Occupational history and angiosarcoma. Arch Dermatol 1981;117(8):425-3.
- Naka N, Tomita Y, Nakanishi H, et al. Mutation of the p53 tumour suppressor gene in angiosarcoma. Int J Cancer 1997;71:952-5.
- MacDonagh DP, Liu J, Gaffey MJ et al. Detection of Kaposi’s sarcoma-associated herpesvirus DNA sequences in angiosarcoma. AM J Patholol 1996;149:1363-8
- Naito S, Shimizu K, Nakashima M et al. Overexpression of Ets-1 transcription factor in angiosarcoma of the skin. Pathol Res Pract 2000;196(2):103-109.
- Mentzel T, Kutzner H, Wollina U et al. Cutaneous angiosarcoma of the face: Clinicopathologic and immunohistochemical study of a case resembling rosacea clinically. J Am Acad Dermatol 1998;38(5):837-840.
- Tay YK, Ong BH. Cutaneous angiosarcoma presenting as recurrent angio-oedema of the face. Br J Dermatol. 2000 Dec;143(6):1346-8.
- Lapidus CS, Sutula FC, Stadecker MJ et al. Angiosarcoma of the eyelid: yellow plaques causing ptosis. J Am Acad Dermatol1996;34(2):308-310.
- Knight TE, Robinson HM, Sina B. Angiosarcoma of the scalp. An unusual case of scarring alopecia. Arch Dermatol 1980;116(6):683-6.
- Holden CA, Spittle MF, Jones EW. Angiosarcoma of the face and scalp: Prognosis and treatment. Cancer 1987;59:1046-57.
- Spieth K, Gille J, Kaufmann R et al. Therapeutic Efficacy of Interferon Alfa-2a and 13 cis-retinoid in recurrent angiosarcoma of the head. Arch Dermatol 1999; 135:1035-37.
- Fata F, O’Reily E, Ilson D et al. Paclitaxel in the treatment of patients with angiosarcoma of the scalp and face. Cancer 1999;86(10):2034-7.
DEDICATED TO EXCELLENCE IN DERMATOLOGY
By National Skin Centre (Singapore)
Copyright (C) 1995 - National Skin Centre (Singapore)
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