An unusual tumor on the scalp of an elderly man

presented by

Henry Foong FRCP Edin (1)

Elizabeth M Taylor MBBS GDFPD (1)

J Andrew Carlson MD, FRCPC (2)

Ipoh,  Malaysia and Albany, NY, USA

March 26, 2009

(1) Foong Skin Specialist Clinic, Ipoh, Perak, Malaysia

(2) Professor, Divisions of Dermatology and Dermatopathology, Albany Medical College, Albany, NY, USA

Abstract 71 yo man with 6 month history of a enlarging scalp nodule.
71 yo man
6 months

This 71-year-old man presented with 6-month history of an enlarging nodule on the scalp. It was asymptomatic. He had seen two other general practitioners but did not improve. He did not have any fever, or other constitutional symptoms. His past medical history was insignificant. He had no previous radiation.

Physical Examination

Pertinent findings on examination were 4 x 4 cm smooth raised nodule with a soft central depression on the vertex of the scalp. It was firm and non-tender. Regional lymph nodes were not enlarged. Rest of examination was unremarkable. The thyroid was not enlarged and there was no hepatosplenomegaly.


(Please click image for higher resolution)

Laboratory Data

Blood counts and biochemistry normal

Tumor markers eg AFP, CEA and PSA were normal

Chest X Ray normal


Biopsy taken from the scalp

Section shows skin with infiltration of the dermis by nests and sheets of cells, large spindle to oval shaped with pleomorphic nuclei. Some have prominent nucleolus. In areas, slit-like vascular channels lined by similar malignant cells. No squamoid or glandular differentiation is seen. No melanin pigment is seen in the malignant cells. The epithelium is normal

Immunohistochemistry: The tumor cells are positive for vimentin and CD 34. They are negative for MNF 116, S-100, HMB 45 and CD 31.

Interpretation: Features are consistent with angiosarcoma epithelioid type


Epithelioid angiosarcoma of the scalp

Reasons Presented

Cutaneous epithelioid angiosarcoma of the scalp is indeed a very rare malignant tumor of the skin. It occurs most commonly in the elderly patients and is located on the scalp, face and legs. Post irradiation angiosarcoma is known to occur in association with lymphedema.

The behavior of the tumor is not well understood. It can recur locally but can also metastasize to regional lymph nodes or the lung. The 5-year survival rate is about 10-35%.

Main histologic features:

  1. vascular lumina that exhibit jagged outlines
  2. immunohistochemical stains for both the epithelial and endothelial markers ( cytokeratins and CD 31 and CD 34)

The main criteria for immunohistochemical diagnosis of cutaneous angiosarcoma are in the following.

  • expression with vascular markers ( CD31 and CD34)
  • inconsistent staining with cytokeratin (35% of the cases)

A careful and thorough histopathological examination and a high index of suspicion, along with appropriate immunohistochemical evaluation, can help reach a correct diagnosis and provide optimal patient care.


The epithelioid angiosarcoma can arise from any organs such as deep soft tissue, bone, thyroid, pharynx, adrenals, prostate, seminal vesicles, the breast and the skin.

In this patient, I think the tumor here is a primary tumor on the skin. Does anyone think this could be a metastatic tumor?

To what extent would one look for metastasis?

Which mode of treatment would be ideal for him? Mohs surgery vs radiotherapy vs chemotherapy? Would topical imiquimod have any role after excision of the tumor?

    Epithelioid tumor Dermatopathology: Practical & Conceptual. July - Sept 2006| Vol 12, #3
  2. Tien Anh Tran, Lydia Evans, Michael Mulvaney, J. Andrew Carlson, PITFALL Saved by the re-excision! What is your diagnosis? Dermatopathology: Practical & Conceptual July - Sept 1998 Vol 4, #3
  3. Mobini N. J Cutaneous epithelioid angiosarcoma: a neoplasm with potential pitfalls in diagnosis. Cutan Pathol 2009 Mar;36(3):362-9.

epithelioid angiosarcoma, cytokeratins, CD34

Comments from Faculty and Members

Azar Maluki, M.D., Asst. Professor and Head, Dept Dermatology, College of Medicine, University of Kufa, Al-Najaf, Iraq on March 28, 2009

I think that Moh's microsurgery would be a very good choice for this localized, yet, aggressive tumor. The use of topical imiquimod post operatively may aid in reducing tha rate of recurrence at the site. The good general condition of the patient, untill now, makes secondary metastasis a remote possibility. Carefull follow-up would be mandatory.

David Polsky, M.D., Assistant Professor, and Shane A. Meehan, M.D., Assistant Professor, Dermatopathology Section, Departments of Dermatology and Pathology, New York University School of Medicine, New York, NY, USA on March 31, 2009

Given that angiosarcoma is quite an aggressive neoplasm and negative margins are usually difficult to achieve even with surgery (please see ref below), I would think Moh's is too conservative an approach.  I would opt for a more aggressive therapy given the tumor size - wide local excision with xrt (at least).

Ref: Pawlik TM, Paulino AF, McGinn CJ, et al. Cutaneous angiosarcoma of the scalp: a multidisciplinary approach. Cancer. 2003;98:1716-1726.

Alfred Kopf, M.D., Professor Emeritus of Dermatology, Department of Dermatology, New York University School of Medicine, New York, NY, USA on March 31, 2009

I have seen only a few cases of this extremely aggressive type of angiosarcoma.  All patients died of this cancer.  I suggest radiologic examination of the cranium to look for bone invasion.  Very wide excision is needed.  Have there been any reports on imiquimod post surgery?

Michael Albom M.D, Clinical Professor of Dermatology, Ronald O. Perelman Department of Dermatology, New York University Medical Center, New York, NY, USA on April 8, 2009

Angiosarcoma is a rare and complex malignancy with a generally poor prognosis. There is a recent reference that specifically reviews angiosarcoma of the scalp ( and an overview of the angiosarcoma ( You have already included 4 other references. The microscopic pathology is well covered in: Soft Tissue Tumors by FM Enzinger and SW Weiss, Mosby 2001.

I have treated two cases of cutaneous angiosarcoma of the midface, one of the entire nose and one of the right nose, mid cheek and upper lip. In each case the neoplasm was multicentric and invaded skeletal muscle. I published these cases in Chapter 35 of Cancer of the Skin, Eds Friedman, Rigel, Kopf, et al, second edition, 1991 (WB Saunders). There are color photographs shown of the larger of the two cases.

The multifocal nature of the histology was striking. Immunohistochemistry revealed “skip areas” of disease. The microscopic tracing of non-contiguous disease is a near impossible challenge. I performed Mohs surgery in each case, once a day, over several days while the patients were hospitalized at New York University Medical Center. Frozen and permanent sections and immunostains were done. Immunostaining of permanent sections was done by Dr. A. Bernard Ackerman and his associate, Dr. Geoffrey Gottlieb. Histologically, several vascular channels revealed very little atypia of the endothelial cells. Thus, tracing the true surgical margins of this disease was very difficult.

Both patients did well with no gross signs of recurrent local disease for over 2 years when subsequently each man developed obvious cervical lymph node metastases. The patient (patient A) with the nasal neoplasm developed bilateral cervical lymph node metastasis at 25 months after surgery and the patient (patient B) with the larger lesion of the right nose, medial cheek and upper lip developed right-sided cervical metastasis at 32 months post surgery. Bilateral modified radical neck dissection was done in the former and a right-sided modified radical neck dissection was undertaken in the latter case.

Patient A survived for at least 10 years ultimately succumbing to a lymphoma. Patient B has survived for 25 years. I saw him 6 months ago to confirm his long-term survival.

I'm commenting on the present case by Drs. Foong, Taylor and Carlson because of several reasons. My colleagues, Drs. Polsky and Meehan state that Mohs surgery is too conservative an approach…and would opt for a more aggressive therapy given the tumor size (of the present case in VGRD) – wide local excision with xrt (at least). Dr. Kopf suggests a very wide excision is needed. Based upon my commentary above, what would my colleagues suggest for the dimensions of a wide excision for a multicentric disease which invades for many centimeters beyond gross clinical borders? A so-called wide excision for angiosarcoma is basically a fishing expedition. You throw your line in the water but you don't know if a fish is two feet in front of you or a mile away. Also, even if a so-called wide excision were to be done, the surgical specimen would have to undergo step-sectioning which would literally require examination of hundreds of histologic slides including immunostaining. Even then, less than 50% of the surgical specimen would be analyzed. At least with Mohs surgery, one has the opportunity to examine 100% of the margins of the surgical specimen. By the same token, because this disease has abnormalities of histologic multifocality and “skip areas,” no surgical technique including Mohs surgery can assure a successful extirpation of disease. In my two cases, successful local control was accomplished but metastatic disease to cervical lymph nodes occurred in both cases. Today, based upon more recent scientific papers, it would be prudent to consider concomitant radiation therapy in the surgical treatment of angiosarcoma.

This neoplasm is most likely a primary lesion but an oncologist could do a basic work-up to see if there is any other source. If the lesion is freely movable over the skull, it has probably not invaded bone. Radiological studies could be done if there is any question as to bony involvement of the lesion.

Adjuvant chemotherapy has had mixed results. Since these tumors are so rare, most reports contain a limited series of cases. It is difficult to establish an effective protocol under these circumstances.

Lastly, there is a question as to the value of topical imiquimod post-operatively in this case. This neoplasm often invades deeply into soft tissues as muscle. One would have to wonder how topical imiquimod would play any role in the management of this disease. If it has been done, the results, with long term follow-up should be reported.

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