UPMC Dermatopathology "Case of the Month" Presentations
UPP - Department of Dermatology, Dermatopathology Unit
Case Authors: Jason G. Whalen, MD, Jau-Shyong Deng, MD, Drazen Jukic MD PhD
MAY 2004 CASE OF THE MONTH
DISCUSSION & DIAGNOSIS
DIAGNOSIS
Final Diagnosis: Angiosarcoma
DISCUSSION
Angiosarcomas are clonal proliferations
of malignantly transformed cells which are differentiated towards endothelial
cells. They are rare neoplasms that usually occur in adults and are more common
in Caucasians and males.
There are three typical clinical settings in which angiosarcomas are seen. They
are 1) idiopathic angiosarcoma of the face, neck, and scalp, 2) Lymphedema-associated
angiosarcoma, and 3) Postirradiation angiosarcoma. The idiopathic angiosarcoma
form is most frequently seen in elderly males. They present as multifocal patches
or plaques on the face, neck and scalp. This was also the form diagnosed in
our patient described above. The lymphedema-associated angiosarcoma form typically
occurs in chronic edematous areas due to lymphatic malformation, post-mastectomy
(Stewart-Treves syndrome), filariasis, and trauma. Post-irradiation angiosarcoma
occurs at sites where radiotherapy was performed for benign and malignant conditions,
typically many years (mean=6) later.
Histopathological examination reveals irregular vascular channels
dissecting through the collagen. The vessels are lined by pleomorphic endothelial
cells with hyperchromatic nuclei. RBC’s may or may not be present in these
vascular channels. Papillary processes within the lumen and mitoses are frequently
present. Most angiosarcomas react with CD31 immunostaining as this is the most
sensitive and endothelium-specific marker. CD34 and Factor VIII-related antigen
are also helpful in diagnosis, but are less specific stains.
Prognosis is poor for all forms.
Treatments include surgical excision with wide margins followed
by radiotherapy for limited disease. For extensive disease, chemotherapy and
radiotherapy are only palliative. Recurrences are common.
This patient was referred to Hematology-Oncology and Radiation-Oncology for
evaluation and treatment. Due to the multifocal nature of this lesion, surgery
was not a reasonable option.
REFERENCES
1. Holden CA, et al. Angiosarcoma
of the face and scalp, prognosis and treatment. Cancer. 1987;59:1046-57.
2. Meis-Kindblom JM, et al. Angiosarcoma of soft tissue: a study of 80 cases.
Am J Surg Pathol. 1998;22:683-97.
3. Morrison WH, et al. Cutaneous angiosarcoma of the head and neck. Cancer. 1995;76:319.
