A Rare Neoplastic Growth on the Ear Lobe

Summary

We report a case of an 83 year  old previously healthy female patient presenting with a swiftly evolving erythematous violaceous, infiltrative, ulcerated onion like mass with hyperkeratotic surface on the left ear lobe. The lesion was excised and resulted as an atypical fibroxanthoma, an extremely rare neoplastic growth, being a superficial variant of pleomorphic malignant fibrous histiocytoma. A brief review of dia­gnosis, treatment and prognosis is discussed.

Key words:
skin –  clear cell atypical fibroxanthoma –  immunohistochemistry –  differential dia­gnosis –  head and neck neoplasms –  neoplasms – prognosis

Case report

An 83 year  old previously healthy female patient, agriculturist, presents with a history of an erythematous violaceous infiltrative, ulcerated onion like mass with hyperkeratotic surface on the left ear lobe (Fig. 1– 4). As the patient had a long history of chronic sun exposure and lived in one of the highest melanoma rates areas in the world [1], it was initially thought to be a metastatic melanoma by the surgeon.

The lesion was then completelly excised and sent for pathological analysis, with the result coming as a malignant ulcerated fusocellular neoplasia with negative margins. Further, an immunohistochemical analysis was performed and was negative for all markers, includ­ing protein S 100, all the cytokeratins, Melan A/ MART 1, protein p53, CD 23and desmin, compatible with an atypical fibroxanthoma, a very rare form of skin cancer. The atypical fibroxanthoma is a superficial variant of pleomorphic malignant fibrous histiocytoma [2]. Our case has followed the classic presentation as a head and neck tumor in an elderly individual, and to the best of our knowledge just one case in medical literature has been reported in a differ­ent topography  on the dorsum of the hand, described almost three decades ago [3].

In a retrospective analysis of Mohs surgery, only 0.2% of the malignant findings were fibroxantomas out of 42,279 patients [4].

Conclusion

Atypical fibroxanthoma normally ap­pears as a swiftly growing nodular or nodulo  ulcerative lesion. It may be composed predominantly of either pleomorphic, spindle, epithelioid cells, or a mixture of these cells. The differential dia­gnosis includes pleomorphic dermal sarcoma, squamous cell carcinoma, malignant melanoma and leiomyosarcoma [5].

It occurs mostly in older adults and in sun exposed areas [6], with male predominance [7] and is a dia­gnosis of exclusion. The treatment is surgical and the preferred method is the aforementioned Mohs surgery [8]. Even though fibroxanthoma may be locally aggres­sive [9], the prognosis is usually very good if margins are adequate and these tumors rarely metastasise [7,10].

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Rodrigo Kraft Rovere, MD

Oncology Unit

Santo Antonio Hospital

Rua Itajai 545

Blumenau, Santa Catarina

CEP 89050100 Brazil

e­-mail: rodrigorovere@hotmail.com

Submitted: 31. 3. 2014

Accepted: 1. 4. 2014