Angiosarcoma

賴名耀 宗天一賴名耀 宗天一高雄榮總 皮膚科高雄榮總 皮膚科

AngiosarcomaAngiosarcoma Case ReportCase Report

An 85-year-old male suffered from asymptomatic to An 85-year-old male suffered from asymptomatic to mildly painful bruise-like skin eruption with oozing mildly painful bruise-like skin eruption with oozing over left forehead for 3 months.over left forehead for 3 months.

The lesion bled easily and progressed quickly from The lesion bled easily and progressed quickly from 2.5cm to 5.0cm in diameter.2.5cm to 5.0cm in diameter.

Several small purple satellite lesions around the main Several small purple satellite lesions around the main lesion were noted.lesion were noted.

There was no fever, no chills and no neck LAPThere was no fever, no chills and no neck LAP. .

HistopathologyHistopathology First biopsy:First biopsy:

Second biopsy (4 weeks later from the first one):Second biopsy (4 weeks later from the first one):

Special stain: CD34 (+), Factor VIII (-), S-100 (-)Special stain: CD34 (+), Factor VIII (-), S-100 (-)

Image StudyImage Study Chest X rayChest X ray Whole body bone scanWhole body bone scan Abdominal SonographyAbdominal Sonography Skull X raySkull X ray

ManagementManagement Radiotherapy: 6 MeV x 7000cGyRadiotherapy: 6 MeV x 7000cGy

Post-irradiation ulceration and pain over forehead Post-irradiation ulceration and pain over forehead and left side of face.and left side of face.

Surgical debridement and STSG for the post-Surgical debridement and STSG for the post-irradiation ulceration (one month after radiotherapy).irradiation ulceration (one month after radiotherapy).

Surgical intervention and chemotherapy were not Surgical intervention and chemotherapy were not considered because of old age.considered because of old age.

Follow-upFollow-up Two new lesions over left lower cheek and chin were Two new lesions over left lower cheek and chin were

noted after STSG was done for 1 month. noted after STSG was done for 1 month.

DiscussionDiscussion

Angiosarcoma (AS)Angiosarcoma (AS) A rare malignant vascular tumor (1-4% of all sarcoma).A rare malignant vascular tumor (1-4% of all sarcoma). The nomenclature:The nomenclature:

Angiosarcoma, hemangiosarcoma, malignant hemangio-Angiosarcoma, hemangiosarcoma, malignant hemangio-endothelioma, lymphangiosarcoma.endothelioma, lymphangiosarcoma.

ClassificationClassification Deep soft tissue tumorDeep soft tissue tumor Cutaneous/subcutaneous tumor (60%)Cutaneous/subcutaneous tumor (60%)

Cutaneous AS of face and scalp (most common)Cutaneous AS of face and scalp (most common) Cutaneous AS associated with lymphedemaCutaneous AS associated with lymphedema Post-irradiation ASPost-irradiation AS Primary AS of the breastPrimary AS of the breast

Cutaneous Angiosarcoma of the Face and ScalpCutaneous Angiosarcoma of the Face and Scalp Less than 0.1% of all head and neck cancers.Less than 0.1% of all head and neck cancers. The average age of onset: between 65-80 years.The average age of onset: between 65-80 years. Sex: Male : Female = 2:1Sex: Male : Female = 2:1 Most common site of AS: scalp, then upper face.Most common site of AS: scalp, then upper face. Pathogenesis of AS: unknown.Pathogenesis of AS: unknown. Predisposing factors:Predisposing factors:

Previous irradiation.Previous irradiation. Malignant change with preexisting benign lesions.Malignant change with preexisting benign lesions. Viral infection.Viral infection. Foreign bodies reaction.Foreign bodies reaction.

Clinical ManifestationClinical Manifestation Most common: asymptomatic bruise-like patches, Most common: asymptomatic bruise-like patches,

plaques or nodules with ill-defined margins and plaques or nodules with ill-defined margins and satellite lesions. satellite lesions.

Three clinical patterns Three clinical patterns (Darryl (Darryl et alet al, 1979), 1979):: Nodular typeNodular type Superficial spreading typeSuperficial spreading type Ulcerating typeUlcerating type

Median duration before diagnosisMedian duration before diagnosis(Aust (Aust et alet al, 1997), 1997): : 5 5 monthsmonths Median size: 7.0 cmMedian size: 7.0 cm

HistopathologyHistopathology The tumor shows varied differentiation The tumor shows varied differentiation

(angiomatous, spindle cell and undifferentiated(angiomatous, spindle cell and undifferentiated patterns), and most tumors are composed of various patterns), and most tumors are composed of various proportions of each patterns.proportions of each patterns.

In well-differentiated areas:In well-differentiated areas: Irregular anastomosing vascular channels were Irregular anastomosing vascular channels were

lined by a single layer of somewhat enlarged lined by a single layer of somewhat enlarged endothelial cells permeating between collagen endothelial cells permeating between collagen bundles.bundles.

Characteristic feature: Characteristic feature: “dissection of collagen”“dissection of collagen”.. Vascular lumens are generally bloodless and may Vascular lumens are generally bloodless and may

contain free-lying shed malignant cells.contain free-lying shed malignant cells.

HistopathologyHistopathology In less well-differentiated areas:In less well-differentiated areas:

Endothelial cells increase in size and number, Endothelial cells increase in size and number, forming intraluminal papillary projections.forming intraluminal papillary projections.

In poorly differentiated areas:In poorly differentiated areas: Solid sheets of large pleomorphic cells with little or Solid sheets of large pleomorphic cells with little or

no evidence of luminal differentiation, resemble no evidence of luminal differentiation, resemble metastatic carcinoma or melanoma.metastatic carcinoma or melanoma.

In other areas:In other areas: Spindle cell, epithelioid cell, interstitial Spindle cell, epithelioid cell, interstitial

hemorrhage, and widely dilated blood-filled spaces.hemorrhage, and widely dilated blood-filled spaces.

ImmunohistochemistryImmunohistochemistry Factor VIII and UEA-I: less value for diagnosis.Factor VIII and UEA-I: less value for diagnosis. CD34 and CD31: more reliable as sensitive markers.CD34 and CD31: more reliable as sensitive markers.

Differential DiagnosisDifferential Diagnosis In well-differentiated angiosarcomas:In well-differentiated angiosarcomas:

D/D from early macular lesions of Kaposi’s D/D from early macular lesions of Kaposi’s sarcoma , benign lymphangioendothelioma and sarcoma , benign lymphangioendothelioma and intravascular papillary endothelial hyperplasia.intravascular papillary endothelial hyperplasia.

In poorly differentiated angiosarcomas:In poorly differentiated angiosarcomas: D/D from adenoid squamous carcinoma with intra-D/D from adenoid squamous carcinoma with intra-

tumor hemorrhage.tumor hemorrhage.

ManagementManagement Recommendation: Recommendation:

Surgical wide excision combined with adjuvantSurgical wide excision combined with adjuvant

radiotherapy and/or chemotherapy.radiotherapy and/or chemotherapy. In the unresectable cases: palliative radiotherapy In the unresectable cases: palliative radiotherapy

and/or chemotherapy.and/or chemotherapy. Other treatment modalities:Other treatment modalities:

COCO22 laser laser Mohs surgeryMohs surgery ImmunotherapyImmunotherapy Intra-arterial doxorubicin HCl infusionIntra-arterial doxorubicin HCl infusion

PrognosisPrognosis High local recurrence rate: 33% ~ 90%.High local recurrence rate: 33% ~ 90%. Highest rate of lymph node metastases of all soft Highest rate of lymph node metastases of all soft

tissue sarcoma of the head and neck: 13%.tissue sarcoma of the head and neck: 13%. Common metastatic sites:Common metastatic sites:

Cervical lymph node, lung, liver and spleen.Cervical lymph node, lung, liver and spleen. Mean survival time: 10 ~ 24 months.Mean survival time: 10 ~ 24 months. 5-year survival rate: 12% ~ 33%.5-year survival rate: 12% ~ 33%. Independent prognostic factor:Independent prognostic factor:

tumor size (5 cm), mode of treatment, mitotic counts.tumor size (5 cm), mode of treatment, mitotic counts.

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