Jeevankumar B, Thappa Devinder Mohan , Shivaswamy KN, Jayanthi S

Bioline International

Indian J Dermatol Venereol Leprol March-April 2004 Vol 70 Issue 2139QuizHow to cite this article: Jeevankumar B, Thappa DM, Shivaswamy KN, Jayanthi S. Ulcerated nodule over the chest. Indian J Dermatol VenereolLeprol 2004;70:139-41.Received: November, 2003. Accepted: January, 2004. Source of Support: Nil.Ulcerated nodule over the chestA 25-year-old male agricultural laborer presented witha non-healing ulcerated nodule located on the upperchest, of three months’ duration. The lesion had startedinsidiously as a single pea-sized nodule. It progressivelyincreased to achieve the present size and thensubsequently ulcerated about a month ago. There wasno history of preceding trauma or extrusion of bonychips from the lesion. Physical examination revealed asolitary ulcerated soft to firm nodule over the upperchest just medial to the right nipple (Figure 1). Itmeasured 3 x 3 cm in size and exuded aserosanguineous discharge. There was no regionallymphadenopathy. Systemic examination revealed noabnormality. A chest X-ray was normal.Histopathological examination of the ulcerated nodulerevealed a noncapsulated dense infiltrate of spindleshaped cells in the upper and mid-dermis (Figures 2and 3). Within this infiltrate, a few bizarre cells withpleomorphic hyperchromatic nuclei were also seen.Large bizarre multinucleated cells were arranged in avague fascicular pattern. Scattered inflammatory cellsand numerous small blood vessels were also present.WHAT IS YOUR DIAGNOSIS?Figure 1: Ulcerated nodule over the chestFigure 2: Non-capsulated tumor consisting of spindle shapedcells (H&E x100)Figure 3: Pleomorphic neoplastic cells, some of them withbizarre nuclei (H&E x200)Indian J Dermatol Venereol Leprol March-April 2004 Vol 70 Issue 2 140Diagnosis: Atypical fibroxanthoma.DISCUSSIONAtypical fibroxanthoma (AFX) is a pleomorphic spindlecell tumor that usually occurs in the sun-damaged skinof the head and neck area of elderly individuals. Helwig1first introduced AFX as a diagnostic term in 1960,emphasizing its high pleomorphic histologicappearance, which contrasted with a generally benigncourse.2 It has been earlier regarded as one of the‘pseudo-malignancies’ of the skin.3The etiology of AFX remains elusive.2-4 Solar radiationprobably represents a predisposing factor in thepathogenesis of this disease. This is supported by itscommon occurrence on actinically damaged skin. Otherless well-documented factors include occupational andtherapeutic irradiation.AFX has two forms of presentation.2 In the commonerform, the disease affects the elderly individual. Thetumor occurs on the exposed parts of the head andneck, particularly the nose, cheek and ear. In the lesscommon form, the tumor occurs on the limbs andtrunk of young persons. Grossly, these lesions aresolitary nodules or ulcers usually measuring less than2 cm in diameter. They may abut the epidermiscausing pressure atrophy or ulceration.2 Alternatively,a grenz zone of uninvolved dermis may be present.The tumor compresses the skin appendages laterallyand extends into the subcutis.Because of reported metastases to the regional lymphnodes and lungs, AFX is now widely regarded as alow-grade malignant neoplasm related to malignantfibrous histiocytoma, which it resembleshistologically.4 It has a more favorable prognosisbecause of its small size and superficial location,whereas malignant fibrous histiocytoma, apleomorphic sarcoma of middle and late adulthood,manifests in the subcutaneous tissue (superficialtumor) or entirely in the muscle (deep tumor) ascircumscribed multilobulated masses, most commonlyin the thigh and buttock, and carries a less favorableprognosis.3, 4Histologically, AFX resembles the pleomorphic formof malignant f ibrous histiocytoma. 3 I t  ischaracterized by bizarre cells arranged in ahaphazard or vague fascicular or rarely storiformpattern. The histological variants of AFX includespindle cell, clear cell, osteoid, osteoclastic,chondroid, pigmented and granular cell types.3 Thecells occasionally possess small deposits of neutralfat as well as PAS positive, diastase-resistantmaterial, probably reflecting degenerative changes.Features that denote a more aggressive behaviorinclude vascular invasion, deep tissue invasion,tumor necrosis and possibly impaired hostimmunity.5,6 If facilities are available, electronmicrocopy, immunoperoxidase studies for α1-antitrypsin, α1-antichymotrypsin, markers forvimentin and factor XIIIa may be done.7The histologic features of most cases of AFX arediagnostic.4 In some cases, especially the lesspleomorphic tumors,  immunohistochemicalstaining is helpful in distinguishing AFX from otherspindle cell neoplasms. AFX expresses vimentin andis non-reactive for cytokeratin, S-100 protein, andusually desmin, thereby excluding spindle cellsquamous cell carcinoma, spindle cell melanoma,and most cases of leiomyosarcoma. 4 Otherconditions sometimes mistaken for AFX includenoduloulcerative basal cell carcinoma and osteomacutis.1-3,8 Atypical fibroxanthoma is usually cured bysimple excision.8B. Jeevankumar, Devinder Mohan Thappa, K.N. Shivaswamy, *S. JayanthiDepartments of Dermatology and STD, and *Pathology, JawaharlalInstitute of Postgraduate Medical Education and Research(JIPMER), Pondicherry - 605006, India.Address for correspondence:Dr. Devinder Mohan Thappa, Professor and Head, Department ofDermatology and STD,  JIPMER, Pondicherry - 605006. India.E-mail: dmthappa@vsnl.net; dmthappa@jipmer.eduREFERENCES1. Helwig EB. Atypical fibroxanthoma. Texas J Med 1963;59:644-7.2. Enzinger FM, Weiss SW. Soft tissue tumors. 3rd ed. St. Louis:Mosby-Year Book; 1995. p. 351-80.QuizIndian J Dermatol Venereol Leprol March-April 2004 Vol 70 Issue 21413. Weedon D. Skin pathology. 2nd ed. London: ChurchillLivingstone; 2002. p. 917-54.4. Heenan PJ. Tumors of the fibrous tissue involving the skin. In:Elder D, Elenitsas R, Jaworsky C, Johnson B Jr, editors. Lever’sHistopathology of the skin. 8th ed. Philadelphia: Lippincott-Raven; 1997. p. 847-87.5. Helwig EB, May D. Atypical fibroxanthoma of the skin withmetastases. Cancer 1986;57:368-76.6. Kemp JD, Stenn KS, Arons M, Fischer J. Metastasizing atypicalQuizfibroxanthoma. Coexistence with chronic lymphocyticleukemia. Arch Dermatol 1978;114:1533-5.7. Altman DA, Nicholoff BJ, Fivenson DP. Differential expressionof factor XIIIa and CD34 in cutaneous mesenchymal tumors. J Cutan Pathol 1993;20:154-8.8. Calonje E, Wadden C, Wilson-Jones E, Fletcher CD. Spindle-cell non-pleomorphic atypical fibroxanthoma: analysis of aseries and delineation of a distinctive variant. Histopathology1993;22:247-54.

Ulcerated nodule over the chest

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