|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 ( http://emedicine.medscape.com/article/1296442-overview) and an overview of the angiosarcoma ( http://emedicine.medscape.com/article/276512-overview). 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.