eMedicine Specialties > Dermatology > Malignant Neoplasms

Stewart-Treves Syndrome: Differential Diagnoses & Workup

Author: Geover Fernandez, MD, FAAD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: Mar 28, 2007

Differential Diagnoses

Angioedema, Acquired
Lymphangioma
Angioedema, Hereditary
Lymphocytoma Cutis
Angioendotheliomatosis
Malignant Melanoma
Angiolymphoid Hyperplasia with Eosinophilia
Metastatic Carcinoma of the Skin
Kaposi Sarcoma
Lymphangiectasia

Other Problems to Be Considered

Telangiectatic metastatic breast disease to the skin
Hemangioendotheliomas
Hemangiopericytomas

Workup

Laboratory Studies

  • Although Stewart-Treves syndrome is also known as lymphangiosarcoma, ultrastructural and immunohistologic studies show that this malignancy arises from blood vessels rather than lymphatic vessels.
  • The following immunohistologic and ultrastructural findings can be used to confirm that the tumor originates from blood vessels.
    • Antibodies against factor VIII–related antigen are markers for endothelial cells. Although malignant endothelial cells may not always show positive staining with this marker, a more sensitive endothelial marker, lectin Ulex europaeus-I, is more likely to react with hemangiosarcoma tumor cells. However, the specificity of this marker is reduced in people with blood group O because normal epithelial cells and carcinomas also bind this lectin in these individuals.
    • CD34 antigen is a marker of vascular endothelial cells and does not react with the lymphatic endothelium.
    • Antikeratin antibodies show no evidence of keratin in this malignancy; this finding confirms that the tumor cells are nonepithelial in origin.
    • Positive staining for laminin, CD31, collagen IV, and vimentin can aid in diagnosing the tumors as angiosarcomas.

Imaging Studies

  • MRI is recommended to evaluate the local extent of angiosarcomas. However, its true value is in question because of poor results in delineating the margin of the tumor. It may be low in signal intensity on T2-weighting and short-tau inversion recovery (STIR) imaging, reflecting the densely cellular, fibrous stroma and sparsely vascularized tumor histology (Schindera, 2005). Additional administration of intravenous contrast medium may reveal significant enhancement of the tumorous lesions.
  • Chest CT should be performed to rule out metastatic disease to the lungs before the patient undergoes extensive surgery.
  • Chest radiography can help in identifying pulmonary metastases and pleural effusion.

Procedures

  • Analysis of a biopsy specimen is essential to the diagnosis of lymphangiosarcoma.
  • Fine needle aspiration is inadequate for diagnosis.

Histologic Findings

Histologically, angiosarcomas in Stewart-Treves syndrome are indistinguishable from angiosarcomas in nonlymphedematous sites. Postlymphedema angiosarcomas are characterized by proliferating vascular channels, which dissect the dermal collagen and, often, the obliterate appendages. Tumor endothelial cells lining these channels show marked hyperchromatism and pleomorphism. Mitoses are commonly seen in these tumor cells. The vascular endothelial cells appear round or oval, and they are protuberant and often project into the lumen. Erythrocytes can be seen inside these vascular channels. The overlying epidermis may be hyperkeratotic and acanthotic, or it may be atrophic. Prominent proliferation of reticular fibers can be seen in association with this malignancy.

At electron microscopic examination, lymphangiosarcoma cells are surrounded by a complete basal lamina. In some tumor cells, pinocytosis, intercellular junctions, and cytoplasmic intermediate filaments are observed. In addition, Weibel-Palade bodies and erythrophagocytosis are often present. These ultrastructural findings suggest a vascular endothelial origin rather than a lymphatic endothelial origin.

Staging

In 1959, McConnell and Haslam divided the course of development of lymphangiosarcoma into 3 stages. This staging system lacks universal application.

  • Stage 1 - Prolonged lymphedema
    • This stage is characterized by extensive edema that causes the degeneration of fat and collagen mainly in the deep part of the dermis.
    • Edema separates the collagen bands, creating a misperception of an increased amount of fibrous tissue in the area.
  • Stage 2 - Premalignant angiomatosis
    • This stage involves multiple foci of small, proliferating channels in the dermis and subdermis. These vessels are lined by hyperplastic endothelial cells, as well as normal, flattened cells.
    • The areas of angiomatosis vary in size, ranging from 100 µm to a couple of centimeters in diameter.
    • Superficial areas can be seen as bruises or vesicles, whereas deeper areas are seen as areas of induration and hemorrhage.
    • Early lesions show little evidence of malignancy, but more advanced lesions reveal early malignant transformation with an increased number of mitotic figures and pleomorphic cells.
  • Stage 3 - Frankly malignant angiosarcoma
    • These aggressive tumors develop from areas of premalignant angiomatosis.
    • The histologic features of this malignancy are described above in stage 2.

More on Stewart-Treves Syndrome

Overview: Stewart-Treves Syndrome
Differential Diagnoses & Workup: Stewart-Treves Syndrome
Treatment & Medication: Stewart-Treves Syndrome
Follow-up: Stewart-Treves Syndrome
References
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References

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Further Reading

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Keywords

cutaneous angiosarcoma, postmastectomy angiosarcoma, lymphangiosarcoma in postmastectomy lymphedema, postlymphedema angiosarcoma, hemangiosarcoma in chronic lymphedema, hemangiosarcoma in postmastectomy lymphedema, postmastectomy lymphedema, lymphangiosarcoma, Milroy disease, Milroy's disease, idiopathic lymphedema, congenital lymphedema, traumatic lymphedema, filarial lymphedema

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Contributor Information and Disclosures

Author

Geover Fernandez, MD, FAAD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School
Geover Fernandez, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society for MOHS Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Abby S Van Voorhees, MD, Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania
Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, National Psoriasis Foundation, Phi Beta Kappa, Sigma Xi, and Women's Dermatologic Society
Disclosure: Amgen Honoraria Consulting; Astellas Grant/research funds Other; Abbott Honoraria Consulting; Genentech Honoraria Consulting; Incyte Grant/research funds Other; Centocor Honoraria Consulting; Warner Chilcott  Consulting; Merck Salary Review panel membership

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Christen M Mowad, MD, Assistant Professor, Department of Dermatology, Geisinger Medical Center
Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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