Erythematous Patch With Central Papules on the Forehead of a Middle-aged Woman | Dermatology | JN Learning | AMA Ed Hub [Skip to Content]
[Skip to Content Landing]

Erythematous Patch With Central Papules on the Forehead of a Middle-aged Woman

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 55-year old woman presented with a 6-week history of an enlarging, tender, pruritic, 4-cm erythematous patch with central inflammatory papules distributed on the medial forehead (Figure, A). No inciting events, such as arthropod bite or trauma, were noted. She was initially treated for presumed shingles with valacyclovir, cephalexin, gabapentin, and hydrocortisone 2.5% topical ointment with no improvement in appearance or symptoms. No other cutaneous abnormalities were noted. A punch biopsy specimen of the left medial forehead was obtained, and histological and immunohistochemical analyses were performed (Figure, B and C).

Please finish quiz first before checking answer.

You answered correctly!

Read the answer below and download your certificate.

You answered incorrectly.

Read the discussion below and retake the quiz.

B. Angiosarcoma

A nodular and interstitial infiltrative dermal proliferation composed of atypical vascular channels and intersecting fascicles of poorly differentiated spindle cells was noted on the biopsy results. Vascular channels dissected collagen bundles and were lined by atypical endothelium demonstrating nuclear hyperchromasia (Figure, B). Focal confluence and piling up of endothelial cells lining the vascular channels were also noted. On immunohistological examination, tumor components included CD31, with diffuse expression of FLI-1 and ERG in both the vascular and spindle cell tumor components. Tumor cells were negative for epithelial membrane antigen, cytokeratin 5/6, p63, S100 protein, SOX10, and CD10.

Angiosarcoma (AS) is a rare malignancy of endothelial origin that is classified into cutaneous, visceral, and soft-tissue subtypes.1 This neoplasm generally occurs in adults, with the highest incidence in the seventh decade of life.2 Angiosarcoma commonly affects the face, scalp, and neck, presenting asymptomatically as a hematomalike lesion; whereas advanced lesions can mimic other cutaneous neoplasms such as squamous cell carcinoma.3 Because AS has an initial nonspecific clinical presentation and clinical features similar to those of other cutaneous malignant neoplasms, diagnosis is often delayed.

Survey Complete!

Sign in to take quiz and track your certificates

Buy This Activity

JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 CME Credit™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC

CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.

Article Information

Corresponding Author: Danielle Perna, BS, University of Louisville School of Medicine, 963 Schiller Ave, Louisville, KY 40204 (d0pern01@louisville.edu).

Published Online: February 20, 2020. doi:10.1001/jamaoncol.2019.6289

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information, Jeffery P Callen, MD, (University of Louisville School of Medicine) for contributing clinical photographs, and Jyoti Burruss, MD, and Jae Y Jung, MD, (University of Louisville School of Medicine) for contributing clinical information. These individuals were not compensated for their contributions.

References
1.
Hart  J , Mandavilli  S .  Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis.   Arch Pathol Lab Med. 2011;135(2):268-272.PubMedGoogle Scholar
2.
Meis-Kindblom  JM , Kindblom  LG .  Angiosarcoma of soft tissue: a study of 80 cases.   Am J Surg Pathol. 1998;22(6):683-697. doi:10.1097/00000478-199806000-00005PubMedGoogle ScholarCrossref
3.
Shustef  E , Kazlouskaya  V , Prieto  VG , Ivan  D , Aung  PP .  Cutaneous angiosarcoma: a current update.   J Clin Pathol. 2017;70(11):917-925. doi:10.1136/jclinpath-2017-204601PubMedGoogle ScholarCrossref
4.
Lima  M .  Cutaneous primary B-cell lymphomas: from diagnosis to treatment.   An Bras Dermatol. 2015;90(5):687-706. doi:10.1590/abd1806-4841.20153638PubMedGoogle ScholarCrossref
5.
Xue  Y , Thakuria  M .  Merkel cell carcinoma review.   Hematol Oncol Clin North Am. 2019;33(1):39-52. doi:10.1016/j.hoc.2018.08.002PubMedGoogle ScholarCrossref
6.
Udager  AM , Ishikawa  MK , Lucas  DR , McHugh  JB , Patel  RM .  MYC immunohistochemistry in angiosarcoma and atypical vascular lesions: practical considerations based on a single institutional experience.   Pathology. 2016;48(7):697-704. doi:10.1016/j.pathol.2016.08.007PubMedGoogle ScholarCrossref
7.
Lahat  G , Dhuka  AR , Hallevi  H ,  et al.  Angiosarcoma: clinical and molecular insights.   Ann Surg. 2010;251(6):1098-1106. doi:10.1097/SLA.0b013e3181dbb75aPubMedGoogle ScholarCrossref
8.
Sindhu  S , Gimber  LH , Cranmer  L , McBride  A , Kraft  AS .  Angiosarcoma treated successfully with anti-PD-1 therapy—a case report.   J Immunother Cancer. 2017;5(1):58. doi:10.1186/s40425-017-0263-0PubMedGoogle ScholarCrossref
9.
Florou  V , Rosenberg  AE , Wieder  E ,  et al.  Angiosarcoma patients treated with immune checkpoint inhibitors: a case series of seven patients from a single institution.   J Immunother Cancer. 2019;7(1):213. doi:10.1186/s40425-019-0689-7PubMedGoogle ScholarCrossref
Want full access to the AMA Ed Hub?
After you sign up for AMA Membership, make sure you sign in or create a Physician account with the AMA in order to access all learning activities on the AMA Ed Hub
Buy this activity
Close
Want full access to the AMA Ed Hub?
After you sign up for AMA Membership, make sure you sign in or create a Physician account with the AMA in order to access all learning activities on the AMA Ed Hub
Buy this activity
Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Close

Name Your Search

Save Search
Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Close

Lookup An Activity

or

Close

My Saved Searches

You currently have no searches saved.

Close

My Saved Courses

You currently have no courses saved.

Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Close