cytes. These capillaries were of varying size and weredistributed throughout the iris stroma (Figure 2).Similar capillaries were noted within all sectors ofthe iris, from the pupillary border to the iris root,and in the anterior part of the ciliary body (Figure2) and ciliary processes. These hemangiomas werehistopathologically similar to those seen in otherorgans. Erythrocytes not associated with normalcapillaries were present in the trabecular structuresand within the ciliary muscle. The lens, retina, andchoroid were within normal limits. Retinal or sub-retinal hemangiomas were not identified. The opticnerve disclosed the presence of erythrocytes in thesubdural and subarachnoid spaces. The left eye wasunremarkable.

To our knowledge, this is the first histopathologicdescription of the eye in diffuse neonatal hemangi-omatosis. The hemangiomas in the iris, ciliary body,and ciliary processes of the right eye of this patientwere similar to cutaneous and visceral lesions de-scribed previously.1,3 Thus, many ocular structuresdevelop hemangiomas in diffuse neonatal hemangi-omatosis. The molecular origin of diffuse neonatalhemangiomatosis is unclear. Vascular endothelialgrowth factor is an endothelial cell-specific growthfactor and a regulator of physiologic and pathologicangiogenesis. Studies to characterize the vascularendothelial growth factor gene, its promotor, recep-tor, and cellular immunohistochemical distributionin this patient are under way.

REFERENCES

1. Golitz LE, Rudikoff J, O’Meara OP. Diffuse neonatal heman-giomatosis. Pediatr Dermatol 1986;3:145–152.

2. Stratte EG, Tope WD, Johnson CL, Swanson NA. Multi-modal management of diffuse neonatal hemangiomatosis.J Am Acad Dermatol 1996;34:337–342.

3. Haik BG, Clancy P, Ellsworth RM, Perina A, Zimmerman K.Ocular manifestations in diffuse neonatal hemangiomatosis.J Pediatr Ophthalmol Strabismus 1983;20:101–105.

4. Ronan SG, Solomon LM. Benign neonatal eruptive hemangi-omatosis in identical twins. Pediatr Dermatol 1984;1:318–321.

Cutaneous Angiosarcoma WithEyelid InvolvementKaan Gunduz, MD, Jerry A. Shields, MD,Carol L. Shields, MD, Ralph C. Eagle, Jr, MD,and Faith Nathan, MD

PURPOSE: To report a clinicopathologic correlationof angiosarcoma affecting the eyelid skin.METHODS: An 82-year-old man developed multiplebruise-like maculopapular lesions, subcutaneousnodules, and diffuse edema over his scalp, face,and eyelids. Biopsy disclosed angiosarcoma, andthe patient was treated with wide-field externalbeam radiotherapy (5,000 cGy).RESULTS: He responded to radiotherapy with par-tial regression of the tumor. Twelve months later,he developed extensive lymphedema secondary tothe residual tumor. He was also found to haveparotid gland and bone metastases, treated withradiotherapy. The patient died of widespread bonemetastases 3 months later.CONCLUSIONS: Angiosarcoma is a rare skin tumorthat has a poor prognosis despite treatment. Itshould be considered in the differential diagnosisof eyelid tumors and edema because it may affectonly the central portion of the face. (Am JOphthalmol 1998;125:870–871. © 1998 byElsevier Science Inc. All rights reserved.)

ANGIOSARCOMA IS A MALIGNANT NEOPLASM

that most commonly arises in the skin andsuperficial soft tissues but can also affect the deeptissues and viscera.1,2 Angiosarcoma of the face andscalp is a distinct subgroup that generally occurs inpatients older than 55 years.1 The majority oftumors arise in the upper part of the face or scalp.The central part of the face, including the eyelids, isinvolved less often. The lower face, including themandibular region, is rarely affected.1 The clinicalappearance of angiosarcoma is variable. It canpresent with maculopapular lesions resembling ec-chymoses, edema, cellulitis, or ulceration, or in anodular form.1–3 The skin lesions are usually red orblue, suggestive of their vascular nature, althoughthey occasionally are tan or resemble normal skincolor. The diagnosis of angiosarcoma is often de-layed because of its varied clinical features. We

Accepted for publication Dec 12, 1997.Oncology Service (K.G., J.A.S., C.L.S.) and Pathology Department

(R.C.E.), Wills Eye Hospital, and Department of Medicine, Division ofNeoplastic Diseases and Hematology (F.N.), Thomas Jefferson Univer-sity. Supported by the Paul Kayser International Award of Merit inRetina Research, Houston, Texas (J.A.S.), and Eye Tumor ResearchFoundation, Philadelphia, Pennsylvania (J.A.S., C.L.S.).

Inquiries to Jerry A. Shields, MD, Oncology Service, Wills EyeHospital, 900 Walnut St, Philadelphia, PA 19107; fax: (215) 928-1140.

AMERICAN JOURNAL OF OPHTHALMOLOGY870 JUNE 1998

report a rare case of angiosarcoma of the upper facialregion that also affected the eyelids.

A 82-year-old healthy man developed multipleecchymotic maculopapular lesions on his scalp, face,and eyelids. Subcutaneous nodules and diffuse cuta-neous edema subsequently developed. A scalp bi-opsy showed bizarrely shaped vascular channelslined by atypical endothelial cells, consistent withangiosarcoma (Figure 1). The extensive cutaneouslesion of the left scalp and face was treated withwide-field external beam radiotherapy (5,000 cGy).He responded to radiotherapy with partial regres-sion of the tumor. Twelve months later, he devel-oped extensive lymphedema secondary to the resid-ual tumor (Figure 2). He was also found to havemetastases in the right parotid gland, spine, and leftribs, treated with radiotherapy. The patient died ofwidespread bone metastases 3 months later.

The pathogenesis of angiosarcoma is obscure.1 Itdevelops de novo in most cases although it has alsobeen reported to arise after irradiation of somevascular tumors4 or from persistent chroniclymphedema in the extremities (Stewart-Trevessyndrome).5 The tumor may originate from thelymphatic or vascular system, or both.1 Althoughthe histopathologic findings in our case were con-sistent with the vascular derivation, the presence ofextensive lymphedema suggested a lymphatic com-ponent clinically.

The mainstay of treatment in angiosarcoma pa-

tients is wide-field radiotherapy.1 In selected caseswith circumscribed tumors, surgical debulking maybe performed before radiotherapy.1 Our patient wastreated with radiotherapy because of the diffusenature of his lesion. Facial angiosarcoma frequentlymetastasizes to the cervical lymph nodes as well asto the lung, liver, and other sites.1–3 In our patient,the tumor metastasized to the bone and parotidgland with no clinically apparent lung or liverinvolvement. Despite treatment, the overall sur-vival with angiosarcoma is poor. No more than halfof the affected patients survive more than 1 year,and the 5-year survival rate is only 12%.1

In conclusion, angiosarcoma should be in-cluded in the differential diagnosis of eyelidtumors or edema. The tumor may present withdiffuse eyelid swelling or as a subcutaneous nod-ule. Adjacent skin involvement with diffuse in-filtration is often found.

REFERENCES

1. Holden CA, Spittle MF, Jones EW. Angiosarcoma of theface and scalp: prognosis and treatment. Cancer 1987;59:1046–1057.

2. Lever WF, Schaumburg-Lever G. Histopathology of theskin. Philadelphia: Saunders, 1990:708–711.

3. Mackenzie IJ. Angiosarcoma of the face. Arch Dermatol1985;121:549–550.

4. Bennett RG, Keller JW, Ditty JF Jr. Hemangiosarcomasubsequent to radiotherapy for a hemangioma in infancy. JDermatol Surg Oncol 1978;4:881–883.

5. Wilson Jones E. Malignant vascular tumors. Clin ExpDermatol 1976;1:287–331.

FIGURE 1. Photomicrograph demonstrating the vascu-lar neoplasm consisting of bizarrely shaped vascularspaces lined by atypical endothelial cells, consistentwith angiosarcoma (hematoxylin and eosin, 3150).

FIGURE 2. Photograph showing the residual tumor(arrow) with diffuse lymphedema on the left eyelids 1year after radiotherapy.

BRIEF REPORTSVOL. 125, NO. 6 871