Do not break a sweat: avoiding pitfalls in the diagnosis of sweat gland tumors

The group of malignant sweat gland neoplasms is characterized by a wide biologic spectrum, including tumors with indolent behavior, low-grade malignant potential with locally destructive tumor growth and high local recurrence rates and high-grade malignant potential characterized by risk for disseminated disease and disease-related mortality. Reliable diagnosis to predict behavior may be challenging for a number of reasons. The clinical presentation is often nonspecific. Many of the tumors are rare, and they are only infrequently encountered in routine diagnostic practice. A significant subset of tumors shows bland and innocuous histologic features. They are easily mistaken for benign tumors despite their potential for destructive growth and aggressive disease course. At the other end of the spectrum the tumors may resemble poorly differentiated carcinoma or adenocarcinoma and recognition relies entirely on sampling and carful histological examination. The tumors may be inseparable from cutaneous metastases from visceral primaries by morphology and immunohistochemistry, requiring careful clinical correlation and work-up. Conversely, cutaneous metastases are readily mistaken for cutaneous primary tumors. While the presence of a myoepithelial layer is a helpful feature in excluding metastatic deposits, it does not imply benign behavior of sweat gland tumors in general. The above issues and challenges are exemplified in the discussion of selected sweat gland carcinoma in this manuscript, with a focus on recently described entities and those with novel findings.