Chronic indurated ulceration of the leg, epithelioid sarcoma with lymph node metastasis

ARTICLE

Auteur(s) : S. ATUGODA, H. AUDRING, C. VOIT, W. STERRY, U. BLUME-PEYTAVI

Department of Dermatology and Allergy University Medical Center Charité Humboldt-University of Berlin Schumannstr. 20-21 10117 Berlin, Germany

Article accepted on 11/08/2003

Epithelioid sarcoma (ES) is a distinctive, rare, malignant soft tissue neoplasm of debatable pathogenesis with recent evidence suggesting a primitive mesenchymal cell histogenesis with epithelial cell differentiation capacity. Commonly predelictive of an extremity, the tumor occurs mostly in young adults as a slowly growing dermal or subcutaneous nodule with frequent ulceration of superficial lesions. Deep-seated tumors tend to show an insidious multifocal growth pattern of local spread along tendons, nerves and facial structures, classically characterized by multiple locoregional recurrences as well as pulmonary and lymph node metastases. The 5 year survival rate is approximately 50%.
The difficulty of early recognition of the tumor is compounded by initial erroneous diagnosis as e.g. granulomatous processes and infections.

Case report

A 71-year-old male in very good health, appearing considerably younger than his chronological age was referred to our department with non-tender, partly ulcerated lesions on the lateral aspect of his right lower leg. Clinical examination revealed two fetid ulcera around the lateral malleolus and four barely palpable nodules distributed evenly around the right gastrocnemial region. The ulcera had first become apparent about 5 weeks prior to first presentation at our department as small nodular erosions which progressively enlarged until they had reached diameters of approximately 5 cm each at presentation (Fig. 1). The patient reported that he had incurred a penetrative injury to the side of the lower right leg as a child, which apparently took many years to heal properly, and later caused intermittent bouts of mild ankle edema. During the months prior to first presentation at our department, the patient had run the gamut of leg vasculature studies without proof of any abnormality. Additionally, the patient had enlarged inguinal lymph nodes ipsilaterally.

Histopathology (Fig. 2) was able to depict a population of diffusely distributed epithelioid cells with large nuclei reaching from the dermis right through to the upper subcutis, with lymph vessel invasion.

Immunohistochemistry demonstrated negative staining for pancytokeratin, CK7, CK8/18, CK20, CK19, MelanA, HMB45, CD68, CD34, CD31, LCA, MNF116, AE1/3, CEA as well as muscle-specific actin (Figs. 4a,  4b) and positive staining for vimentin as well as epithelial membrane antigen (EMA) (Figs. 5a,  5b).

Ultrasound B-scan in combination with power mode to show vascularization of the hilum region and periphery allowed detection of lymph node involvement in the dependent groin lymph node basin (Fig. 3).

These findings, together with the identical histopathology of the affected ipsilateral inguinal lymph nodes made it possible to identify the tumor as an epithelioid sarcoma of histological grade 3, with lymph node metastases, giving this particular tumor stage group IVa.

The negative staining for cytokeratins and CD34, which is rather typical for synovial sarcoma, was excluded as a possible diagnosis in view of the striking dermal involvement and ulceration exhibited in our patient, which is more commonly indicative of epithelioid sarcoma.

Treatment consisted of limb saving conservative surgery with wide surgical margins, with autologous split skin grafts being used to cover the skin defects. The lymph node metastases were completely surgically removed. The follow up investigations five months after first presentation at our department showed no signs of distant metastases with one local recurrence on one of the excision margins.

Discussion

Recognized by Laskowski [1] in 1960 and described in detail ten years later by Enzinger [2], epithelioid sarcoma is a rare tumor characterized by an innocuous presentation of a non-tender nodule or cluster of nodules, typically located on a distal extremity, although presentation in other areas of the body such as the penis and vulva have also been reported [3-5]. Classically, it has been described as having a high propensity for nodal spread [6, 7], as well as relentless local and distant progression. The name sarcoma aponeuroticum, coined by Laskowski, reflects its tendency for growth along fascial planes, tendon sheaths, and aponeuroses [6]. Typically, the disease occurs in young adults, more frequently among males, with affected females having the better prognosis [8]. Because of its innocuous presentation, diagnosis is often delayed, on histological examination, epithelioid sarcoma can often be confused with granulomatous processes, synovial sarcoma, or ulcerating malignancies of epithelial origin, and frequently multiple biopsies have to be performed before the correct diagnosis [9].
Although classically the combination of immunohistological reactivity for cytokeratins, vimentin and EMA has been described in the majority of cases of ES, other imunohistochemical profiles which veer from this constellation are also possible as described by Miettinen [10], where typical and variant (angiomatoid, fibroma-like, large cell-rhabdoid) ESs were evaluated, with almost all typical ESs being positive for K8, 72% for K19, 48% for intermediate- and high-molecular-weight keratins and 22% for K7.
The reported outcome of patients with epithelioid sarcoma has been limited to a few surgical series and clinical-pathologic reviews. Most reports include a spectrum of surgical procedures from excision with extended margins to limb amputation, infrequent adjuvant therapy, and relatively short follow-up. These limitations have impeded the formulation of well-founded treatment policies, although it is apparent that improvements in both local and distant disease control are warranted. The role of amputation as the best means of treatment for extremity ES has in recent years been questioned as conservative surgery combined with radiotherapy and chemotherapy have also in the long run brought about comparative results [11, 12].
The long-term survival rates, ranging from 42% to 79%, are comparable to those reported for other sarcomas, depending on histological subtype and stage of presentation [6, 10, 13-15]. Local failure rates, as exemplified by our patient, however, are worse than expected, varying from 30% to 77% [6, 13, 15-18], confirming the local aggressiveness of the tumour. This case clearly illustrates the importance of a significantly broad differential diagnosis in the evaluation of chronic leg ulcers. n

References

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