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The Head-Tilt Maneuver A Clinical Aid in Recognizing Head and Neck Angiosarcomas FREE

Maryam M. Asgari, MD, MPH; Clay J. Cockerell, MD; Sarah Weitzul, MD
[+] Author Affiliations

Author Affiliations: Department of Dermatology, University of Texas Southwestern Medical Center, Dallas (Drs Asgari, Cockerell, and Weitzul); and Division of Research, Kaiser Permanente Northern California, Oakland (Dr Asgari).


Arch Dermatol. 2007;143(1):75-77. doi:10.1001/archderm.143.1.75.
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Published online

ABSTRACT

Background  Cutaneous angiosarcoma is a rare, life-threatening tumor that is often initially misdiagnosed. This delay in diagnosis can affect tumor growth, metastatic potential, and prognosis.

Observations  We describe the “head-tilt maneuver,” which highlights the vascular nature of these lesions and can be of potential benefit in early recognition and better appreciation of the clinical extent of this tumor.

Conclusion  Early recognition and aggressive management of these tumors can afford the best opportunity for cure.

Figures in this Article

Cutaneous angiosarcoma is a rare, aggressive endothelial sarcoma that arises most commonly on the head and neck of elderly men.1,2 Cutaneous angiosarcomas can arise on the extremities in patients with chronic lymphedema (Stewart-Treves syndrome) or on areas treated with irradiation as well as in preexisting vascular malformations. Little is known about the risk factors that lead to its development on the head and neck, although there is a strong predilection for elderly white men.3 Cumulative sun exposure does not seem to be a risk factor. The tumor is composed of a clonal proliferation of malignantly transformed cells that express markers for both lymphatic and vascular endothelium.3 Despite multimodal treatment, it has an extremely poor prognosis with a reported 5-year survival of less than 35%.2,4,5 Prognosis is closely linked to the size of the tumor and the depth of invasion,1,2,5,6 highlighting the importance of early recognition.

Cutaneous angiosarcomas can often mimic inflammatory conditions such as rosacea,7,8 erysipelas,9 or cellulitis.10 They can also be misdiagnosed as an arthropod bite11 or a bruise.12 The variety of ways in which cutaneous angiosarcomas manifest often delays diagnosis.

We present a sign, to our knowledge not previously described in the literature, that can aid in the diagnosis of these life-threatening tumors. The tumor's vascular etiology can be accentuated by performing what we call the head-tilt maneuver. In this maneuver, the patient is asked to place his or her head below the level of the heart for 5 to 10 seconds. If the area of involvement becomes markedly more violaceous and engorged, a vascular neoplasm should be suspected.

REPORT OF A CASE

We describe a previously healthy 46-year-old white man who presented to our clinic with a 2-month history of a progressively enlarging erythematous plaque on his left cheek. He noted that the erythema appeared to intensify with exertion or a rise in ambient temperature, as well as when he lowered his head below the level of his heart (head-tilt maneuver). The plaque was asymptomatic, although he had noted progressive induration of the area near his jaw line over the preceding 2 weeks.

Physical examination revealed an erythematous plaque on his left medial cheek with extension to the mandible (Figure 1 and Figure 2). The inferior portion of the plaque appeared edematous. When the patient lowered his head between his knees for 5 seconds, the involved areas became prominently violaceous and throbbed painfully. Violaceous patches were also noted in the glabella and right medial cheek (Figure 3) but were not visible when the patient was sitting upright.

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Figure 1. Erythematous plaque on the left cheek at baseline.

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Figure 2. Close-up view of the erythematous plaque of left cheek.

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Figure 3. Violaceous plaque on the cheek after head-tilt maneuver for 10 seconds.

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Histologic examination of the skin from the left cheek, right cheek, and glabella showed prominent dermal vascular proliferations of thin-walled vessels with irregular channels lined by atypical plump endothelial cells (Figure 4A). Atypical endothelial cells were also noted to float freely within the vascular spaces (Figure 4A, inset). CD31 staining further highlighted the abnormal endothelial channels (Figure 4B).

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Figure 4. Histologic examination of the skin. A, Proliferation of thin-walled vessels in the dermis (hematoxylin-eosin, original magnification ×2); inset, close-up of atypical vascular channels dissecting collagen bundles (hematoxylin-eosin, original magnification ×10); B, CD31 staining highlights endothelial origin of tumor cells (original magnification ×20).

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Owing to the extensive involvement of both cheeks and the glabella, surgery was excluded as a treatment option. The patient was seen by a hematologist-oncologist who recommended chemotherapy and radiation. The patient underwent 12 weeks of chemotherapy with paclitaxel. Toward the end of his chemotherapy course, the erythema on his glabella and right cheek resolved after the head-tilt maneuver, and the erythema on his left cheek was markedly improved. On completion of chemotherapy, the patient received local irradiation therapy and was doing well 6 months after his date of diagnosis.

COMMENT

Cutaneous angiosarcoma is a rare tumor with a poor prognosis and a high potential for metastasis. Prognosis is tightly linked to tumor size and depth of invasion, both of which can be decreased by early detection.6 We report this case to offer the head-tilt maneuver as a quick, easy, and noninvasive clinical aid that emphasizes the vascular etiology of these tumors and that can help facilitate early recognition. This maneuver may also aid in delineating the borders of the tumor as well as highlighting potential skip areas prior to surgical resection. This was particularly valuable in this patient because the contralateral cheek and glabellar involvement, which was not detectable without the head-tilt maneuver, resulted in the exclusion of surgery as a treatment option. In our patient, the violaceous color of the tumor decreased in intensity after commencing chemotherapy, which suggests that the head-tilt maneuver may be useful as a clinical aid in assessing tumor responsiveness to therapy.

ARTICLE INFORMATION

Correspondence: Maryam M. Asgari, MD, MPH, Kaiser Permanente, Division of Research, 2000 Broadway, Oakland, CA 94612 (maryam.m.asgari@kp.org).

Financial Disclosure: None reported.

Accepted for Publication: June 13, 2006.

Author Contributions:Study concept and design: Asgari. Acquisition of data: Weitzul. Analysis and interpretation of data: Cockrell. Drafting of the manuscript: Asgari. Critical revision of the manuscript for important intellectual content: Asgari, Cockrell, and Weitzul. Obtained funding: Asgari. Administrative, technical, and material support: Cockrell. Study supervision: Weitzul.

Acknowledgment: We thank Payam Abrishami, MD, for preparing the histopathologic photographs.

REFERENCES

Holden  CASpittle  MFJones  EW Angiosarcoma of the face and scalp, prognosis and treatment. Cancer 1987;591046- 1057
PubMed Link to Article
Morgan  MBSwann  MSomach  SEng  WSmoller  B Cutaneous angiosarcoma: a case series with prognostic correlation. J Am Acad Dermatol 2004;50867- 874
PubMed Link to Article
Bolognia  JedJorizzo  JedRapini  Red Dermatology.  New York, NY Mosby2003;
Mark  RJTran  LMSercarz  JFu  YSCalcaterra  TCJuillard  GF Angiosarcoma of the head and neck: the UCLA experience 1955 through 1990. Arch Otolaryngol Head Neck Surg 1993;119973- 978
PubMed Link to Article
Lydiatt  WMShaha  ARShah  JP Angiosarcoma of the head and neck. Am J Surg 1994;168451- 454
PubMed Link to Article
Aust  MROlsen  KDLewis  JE  et al.  Angiosarcomas of the head and neck: clinical and pathologic characteristics. Ann Otol Rhinol Laryngol 1997;106943- 951
PubMed
Panizzon  RSchneider  BVSchnyder  UW Rosacea-like angiosarcoma of the face. Dermatologica 1990;181252- 254
PubMed Link to Article
Mentzel  TKutzner  HWollina  U Cutaneous angiosarcoma of the face: clinicopathologic and immunohistochemical study of a case resembling rosacea clinically. J Am Acad Dermatol 1998;38837- 840
PubMed Link to Article
Verleysen  ADewolf  KGeerts  MLNaeyaert  JM Guess what! angiosarcoma of the face and scalp. Eur J Dermatol 2000;10403- 404
PubMed
Chan  LYTang  WYLam  WYLo  KK Guess what! angiosarcoma of the face and scalp. Eur J Dermatol 2001;11261- 263
PubMed
Cannavo  SPLentini  MMagliolo  EGuarneri  C Cutaneous angiosarcoma of the face. J Eur Acad Dermatol Venereol 2003;17594- 595
PubMed Link to Article
Rich  ALBerman  P Cutaneous angiosarcoma presenting as an unusual facial bruise. Age Ageing 2004;33512- 514
PubMed Link to Article

Figures

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Figure 1. Erythematous plaque on the left cheek at baseline.

Graphic Jump Location
Place holder to copy figure label and caption

Figure 2. Close-up view of the erythematous plaque of left cheek.

Graphic Jump Location
Place holder to copy figure label and caption

Figure 3. Violaceous plaque on the cheek after head-tilt maneuver for 10 seconds.

Graphic Jump Location
Place holder to copy figure label and caption

Figure 4. Histologic examination of the skin. A, Proliferation of thin-walled vessels in the dermis (hematoxylin-eosin, original magnification ×2); inset, close-up of atypical vascular channels dissecting collagen bundles (hematoxylin-eosin, original magnification ×10); B, CD31 staining highlights endothelial origin of tumor cells (original magnification ×20).

Graphic Jump Location

Tables

References

Holden  CASpittle  MFJones  EW Angiosarcoma of the face and scalp, prognosis and treatment. Cancer 1987;591046- 1057
PubMed Link to Article
Morgan  MBSwann  MSomach  SEng  WSmoller  B Cutaneous angiosarcoma: a case series with prognostic correlation. J Am Acad Dermatol 2004;50867- 874
PubMed Link to Article
Bolognia  JedJorizzo  JedRapini  Red Dermatology.  New York, NY Mosby2003;
Mark  RJTran  LMSercarz  JFu  YSCalcaterra  TCJuillard  GF Angiosarcoma of the head and neck: the UCLA experience 1955 through 1990. Arch Otolaryngol Head Neck Surg 1993;119973- 978
PubMed Link to Article
Lydiatt  WMShaha  ARShah  JP Angiosarcoma of the head and neck. Am J Surg 1994;168451- 454
PubMed Link to Article
Aust  MROlsen  KDLewis  JE  et al.  Angiosarcomas of the head and neck: clinical and pathologic characteristics. Ann Otol Rhinol Laryngol 1997;106943- 951
PubMed
Panizzon  RSchneider  BVSchnyder  UW Rosacea-like angiosarcoma of the face. Dermatologica 1990;181252- 254
PubMed Link to Article
Mentzel  TKutzner  HWollina  U Cutaneous angiosarcoma of the face: clinicopathologic and immunohistochemical study of a case resembling rosacea clinically. J Am Acad Dermatol 1998;38837- 840
PubMed Link to Article
Verleysen  ADewolf  KGeerts  MLNaeyaert  JM Guess what! angiosarcoma of the face and scalp. Eur J Dermatol 2000;10403- 404
PubMed
Chan  LYTang  WYLam  WYLo  KK Guess what! angiosarcoma of the face and scalp. Eur J Dermatol 2001;11261- 263
PubMed
Cannavo  SPLentini  MMagliolo  EGuarneri  C Cutaneous angiosarcoma of the face. J Eur Acad Dermatol Venereol 2003;17594- 595
PubMed Link to Article
Rich  ALBerman  P Cutaneous angiosarcoma presenting as an unusual facial bruise. Age Ageing 2004;33512- 514
PubMed Link to Article

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