Angiosarcoma 賴名耀 宗天一 高雄榮總 皮膚科

Angiosarcoma  Case Report  An 85-year-old male suffered from asymptomatic to mildly painful bruise-like skin eruption with oozing over left forehead for 3 months.  The lesion bled easily and progressed quickly from 2.5cm to 5.0cm in diameter.  Several small purple satellite lesions around the main lesion were noted.  There was no fever, no chills and no neck LAP.

 Histopathology  First biopsy:  Second biopsy (4 weeks later from the first one):  Special stain: CD34 (+), Factor VIII (-), S-100 (-)  Image Study  Chest X ray  Whole body bone scan  Abdominal Sonography  Skull X ray

 Management  Radiotherapy: 6 MeV x 7000cGy  Post-irradiation ulceration and pain over forehead and left side of face.  Surgical debridement and STSG for the post- irradiation ulceration (one month after radiotherapy).  Surgical intervention and chemotherapy were not considered because of old age.  Follow-up  Two new lesions over left lower cheek and chin were noted after STSG was done for 1 month.

Discussion

Angiosarcoma (AS)  A rare malignant vascular tumor (1-4% of all sarcoma).  The nomenclature: Angiosarcoma, hemangiosarcoma, malignant hemangio- endothelioma, lymphangiosarcoma. Angiosarcoma, hemangiosarcoma, malignant hemangio- endothelioma, lymphangiosarcoma.  Classification  Deep soft tissue tumor  Cutaneous/subcutaneous tumor (60%)  Cutaneous AS of face and scalp (most common)  Cutaneous AS associated with lymphedema  Post-irradiation AS  Primary AS of the breast

 Cutaneous Angiosarcoma of the Face and Scalp  Less than 0.1% of all head and neck cancers.  The average age of onset: between years.  Sex: Male : Female = 2:1  Most common site of AS: scalp, then upper face.  Pathogenesis of AS: unknown.  Predisposing factors:  Previous irradiation.  Malignant change with preexisting benign lesions.  Viral infection.  Foreign bodies reaction.

 Clinical Manifestation  Most common: asymptomatic bruise-like patches, plaques or nodules with ill-defined margins and satellite lesions.  Three clinical patterns (Darryl et al, 1979) :  Nodular type  Superficial spreading type  Ulcerating type  Median duration before diagnosis (Aust et al, 1997) : 5 months  Median size: 7.0 cm

 Histopathology  The tumor shows varied differentiation (angiomatous, spindle cell and undifferentiated patterns), and most tumors are composed of various proportions of each patterns.  In well-differentiated areas:  Irregular anastomosing vascular channels were lined by a single layer of somewhat enlarged endothelial cells permeating between collagen bundles.  Characteristic feature: “dissection of collagen”.  Vascular lumens are generally bloodless and may contain free-lying shed malignant cells.

 Histopathology  In less well-differentiated areas:  Endothelial cells increase in size and number, forming intraluminal papillary projections.  In poorly differentiated areas:  Solid sheets of large pleomorphic cells with little or no evidence of luminal differentiation, resemble metastatic carcinoma or melanoma.  In other areas:  Spindle cell, epithelioid cell, interstitial hemorrhage, and widely dilated blood-filled spaces.

 Immunohistochemistry  Factor VIII and UEA-I: less value for diagnosis.  CD34 and CD31: more reliable as sensitive markers.  Differential Diagnosis  In well-differentiated angiosarcomas:  D/D from early macular lesions of Kaposi’s sarcoma, benign lymphangioendothelioma and intravascular papillary endothelial hyperplasia.  In poorly differentiated angiosarcomas:  D/D from adenoid squamous carcinoma with intra- tumor hemorrhage.

 Management  Recommendation: Surgical wide excision combined with adjuvant radiotherapy and/or chemotherapy.  In the unresectable cases: palliative radiotherapy and/or chemotherapy.  Other treatment modalities:  CO 2 laser  Mohs surgery  Immunotherapy  Intra-arterial doxorubicin HCl infusion

 Prognosis  High local recurrence rate: 33% ~ 90%.  Highest rate of lymph node metastases of all soft tissue sarcoma of the head and neck: 13%.  Common metastatic sites: Cervical lymph node, lung, liver and spleen.  Mean survival time: 10 ~ 24 months.  5-year survival rate: 12% ~ 33%.  Independent prognostic factor: tumor size (5 cm), mode of treatment, mitotic counts.

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