Malignant acrospiroma

Signs and symptoms

Usually, lesions appear as slow-growing nodules that have the potential to drain and ulcerate. Their sizes vary from 0.5 to 10 cm. Typically, they affect the head, neck, and limbs; the breasts and chest are less frequently affected.

Causes

Malignant acrospiromas can develop spontaneously or evolve from a preexisting benign lesion.

Diagnosis

The diagnosis of malignant acrospiroma is primarily based on histological, immunohistochemical, or ultrastructural characteristics.

Histological traits include a pronounced nodular (lobular) pattern. Normally, there is no link between the epidermis and the tumour, but the superficial epithelium may become ulcerated. The tumour may have enhanced mitotic activity and localized regions of necrosis.

The differential diagnosis for malignant acrospiroma includes additional cancers with clear cell changes, such as trichilemmal carcinoma, sebaceous carcinoma, clear cell changes in squamous cell carcinoma, basal cell carcinoma, balloon cell melanoma, porocarcinoma, and metastatic renal clear cell carcinoma.

Treatment

The primary therapeutic method is wide surgical excision. Because of the aggressive nature and proclivity for lymphatic invasion, several authors suggest prophylactic lymph node dissection. Adjuvant chemotherapy's effectiveness is not well established.

Outlook

Within the first two years, the tumour has a 50% chance of local recurrence and a 60% chance of metastatic spread. Disease-free 5-year survival rates have been reported to be less than 30%.

Epidemiology

Malignant acrospiromas account for around 6% of all malignant eccrine tumours. The disease typically appears in middle ages. There does not appear to be a clear gender preponderance, despite some reports suggesting that malignant acrospiromas are more common in women.

References

References

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