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The classification of malignant sweat gland neoplasms has undergone changes over time.
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Malignant sweat gland neoplasms have variable malignant potential, with some behaving as indolent tumors and others with high metastatic potential.
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Many of the sweat gland neoplasms have overlapping features but knowledge of the characteristic findings of these entities is important.
Malignant Sweat Gland Tumors
Section snippets
Key points
Clinical Features
Porocarcinoma, also known as eccrine porocarcinoma and malignant poroma, is a malignant tumor derived from the intraepidermal portion of the eccrine sweat duct. It is typically seen in older patients, although there are reports of occurrence in all ages. There seems to be no gender nor racial predilection.1 Lesions tend to occur on the legs in up to 60% of cases, particularly favoring the thigh and knee. Other sites of involvement include the head, neck, and trunk. Few reports in the literature
Clinical Features
Primary cutaneous tubular carcinoma, also known as apocrine adenocarcinoma and apocrine tubular carcinoma, is a malignant apocrine neoplasm that typically arises in areas where there is a high density of apocrine glands, such as the axillae and groin, although cases have been reported in the eyelid, scalp, nipple, lip, and ear canal, and within preexisting nevus sebaceous.14, 15 They appear more frequently in women in the fourth to sixth decades of life. This entity presents as a solitary firm,
Clinical Features
Papillary carcinoma, also described as aggressive digital papillary adenocarcinoma, is notable for its preferential location on the digits. Initially, papillary adenomas were distinguished from adenocarcinomas based on the degree of differentiation. However, Duke and colleagues20 found that the histopathologic criteria initially proposed did not differentiate benign from malignant entities, because lesions previously designated as benign were found to subsequently metastasize. This group
Clinical Features
Adenoid cystic carcinoma (ACC) are malignant neoplasms that are commonly described in the salivary glands and other organs, such as the trachea, breast, Bartholin glands, and prostate. Primary cutaneous ACC was first described in 1975.25 This lesion is observed in adults, affecting men and women equally. The most common location is the scalp, although lesions on the face, trunk, and extremities have been described. Lesions are often asymptomatic with nonspecific clinical features, usually
Clinical Features
Cribriform carcinoma is a rare cutaneous adenocarcinoma exhibiting apocrine differentiation. First described in 1998 by Requena and colleagues,30 this malignant sweat gland neoplasm is observed in young to middle-aged adults. Women are more frequently affected than men, and the average age at the time of diagnosis is 47 years.31 The most common locations are the upper and lower extremities, with few reports on the head and trunk. Clinically, lesions present with nonspecific features, usually as
Clinical Features
Apocrine hidradenocarcinoma is an entity that has been reported under numerous designations, including clear cell hidradenocarcinoma, clear cell malignant acrospiroma, nodular hidroadenocarcinoma, and malignant eccrine acrospiroma, among others. This is a rare and aggressive malignancy, observed slightly more frequently in men than in women and primarily in adults. Clinically, this neoplasm presents as an asymptomatic subcutaneous red to violaceous nodule and tends to favor the face, hands, and
Clinical Features
Malignant mixed tumor of the skin, also known as malignant chondroid syringoma, are uncommon, with less than 50 cases described in the literature. The nomenclature is derived from the fact that this neoplasm is a tumor of epithelial and mesenchymal origin.37 It can occur de novo or arise within a preexisting benign chondroid syringoma. Clinically, lesions are nonspecific, presenting as a subcutaneous nodule with possible overlying ulceration and firm attachment to underlying tissue. It is seen
Clinical Features
Syringoid carcinoma, first designated as an eccrine epithelioma, but also referred to as malignant syringoma, syringoid eccrine carcinoma, basal cell epithelioma with eccrine differentiation, and sclerosing syringoma, is a rare malignant neoplasm that is characterized by its similar histopathologic appearance to syringoma. This tumor is observed in men and women equally and presents on the face and scalp most frequently.40 They are often slow-growing, and present as tender nodules or plaques.
Clinical Features
This rare neoplasm was first described in 1929 by Wiedman. Cylindrocarcinomas typically occur on the scalp, and the presence of multiple cylindromas seems to be the most common predisposing factor for developing cylindrocarcinoma.42 Like other sweat gland neoplasms, this tumor lacks distinctive clinical features, but the most common presentation is a nodule of rapid growth arising in patients with multiple long-standing cylindromas. The sudden rapid growth often leads to ulceration of the
Clinical Features
Spiradenocarcinomas, also known as malignant eccrine spiradenomas, are rare cutaneous adnexal neoplasms that arise from longstanding benign spiradenomas. A history of trauma to preexisting spiradenoma has also been reported. Rarely, they are seen in the setting of Brooke-Spiegler syndrome. They are seen in men and women equally, and usually observed in adults. The most common locations are the extremities, although there are reports of occurrences on the face, scalp, and chest. The most common
Clinical Features
Primary cutaneous mucinous carcinoma are rare low-grade malignant neoplasms thought to be of eccrine origin. Most mucinous carcinomas involving the skin are metastatic from breast, gastrointestinal tract, salivary glands, lacrimal glands, nasal sinuses, bronchi, or ovaries. One of the greatest challenges in diagnosing primary cutaneous mucinous carcinoma is differentiating this entity from metastatic disease. Both entities are nearly identical with similar gross and histologic features.47
Clinical Features
Polymorphous sweat gland carcinoma is a rare neoplasm, with only 12 cases described in the literature. The lesion demonstrates a predilection for the extremities and is often misdiagnosed as a dermatofibroma or cyst. Based on few available reports, it seems to occur in middle aged to older patients, and affects men and women equally. In most case reports, patients present with an asymptomatic nodule after a prolonged clinical course. It is characterized by variegate histopathologic findings and
Clinical Features
MAC is an uncommon, locally aggressive adnexal neoplasm. It may be an underdiagnosed entity caused by shared clinical and histologic features with other cutaneous neoplasms.52 MAC affects men and women equally and can occur in a wide range of ages from the first to eighth decades of life. It is seen more frequently in white persons, and favors the periorbital and nasolabial regions. Less commonly, they are found on the nipple, axilla, vulva, hands, tongue, and extremities. It presents as a
Clinical Features
Secretory carcinoma of the skin is a rare neoplasm with only few reports to date. It appears as a solitary dermal or subcutaneous nodule and has most frequently been observed in the axillae. It affects females more often and is associated with an ETV6-NTRK3 translocation, which is a characteristic finding of secretory breast carcinoma and mammary analogue secretory carcinoma.54
Histopathologic Features
At scanning magnification, secretory carcinoma of the skin is poorly circumscribed and is composed of dense neoplastic
Clinical Features
Signet ring cell carcinoma or primary cutaneous histiocytoid carcinoma is a rare neoplasm that is typically located on the eyelids of adults. In a review of 39 cases, there was a male predominance, and most lesions were located on the eyelids, followed by the axillae. Clinically, lesions present as diffuse thickening of the involved eyelid or a nodule or induration in the axillae. Treatment options include surgical removal, which may be followed by adjuvant and maintenance chemotherapy.55, 56,
References (57)
- et al.
Porocarcinoma; presentation and management, a meta-analysis of 453 cases
Ann Med Surg (Lond)
(2017) - et al.
Complete response of a metastatic porocarcinoma treated with paclitaxel, cetuximab and radiotherapy
Eur J Cancer
(2018) - et al.
Eccrine porocarcinoma of the face
J Am Acad Dermatol
(1992) - et al.
An immunohistochemical comparison of cytokeratin 7, cytokeratin 15, cytokeratin 19, CAM 5.2, carcinoembryonic antigen, and nestin in differentiating porocarcinoma from squamous cell carcinoma
Hum Pathol
(2012) - et al.
Primary apocrine carcinoma of scalp: report of primary scalp cutaneous apocrine carcinoma indistinguishable from cutaneous metastasis of breast carcinoma
J Plast Reconstr Aesthet Surg
(2012) - et al.
Aggressive digital papillary adenocarcinoma
J Am Acad Dermatol
(1990) - et al.
Primary cutaneous adenoid cystic carcinoma in the United States: incidence, survival, and associated cancers, 1976 to 2005
J Am Acad Dermatol
(2010) - et al.
Nodular hidradenoma and hidradenocarcinoma. A 10-year review
J Am Acad Dermatol
(1985) - et al.
A rare case of eccrine porocarcinoma of the eyelid
Semin Ophthalmol
(2015) - et al.
Subungual eccrine porocarcinoma: rare but possible
Dermatol Surg
(2017)
Eccrine porocarcinoma of the vulva: a case report and review of the literature
J Med Case Rep
Porocarcinoma: clinical evolution
Dermatol Surg
Eccrine porocarcinoma: new insights and a systematic review of the literature
Dermatol Surg
Treatment of porocarcinoma with Mohs micrographic surgery: the Mayo Clinic experience
Dermatol Surg
Treatment of advanced malignant eccrine poroma with locoregional chemotherapy
Br J Dermatol
Porocarcinoma of the heel. A case report with unusual histologic features
Cancer
The squamous variant of eccrine porocarcinoma: a clinicopathological study of 21 cases
J Clin Pathol
Apocrine intraductal carcinoma in situ in nevus sebaceus: two case reports
J Cutan Pathol
A case report of a primary apocrine adenocarcinoma of the eyelid with literature review
Orbit
Apocrine gland carcinoma of the axilla: review of the literature and recommendations for treatment
Am J Clin Oncol
Apocrine axillary adenocarcinoma: an aggressive adnexal tumor in middle-age individuals
Dermatol Surg
Primary apocrine adenocarcinoma of the axilla
Cutis
Aggressive digital papillary adenocarcinoma (aggressive digital papillary adenoma and adenocarcinoma revisited)
Am J Surg Pathol
Aggressive digital papillary adenoma and adenocarcinoma. A clinicopathologic study of 57 patients with histochemical, immunopathological and ultrastructural observations
J Cutan Pathol
Cutaneous digital papillary adenocarcinoma: a clinicopathologic study of 31 cases of a rare neoplasm with new observations
Am J Surg Pathol
Aggressive digital papillary adenocarcinoma: population-based analysis of incidence, demographics, treatment, and outcomes
Dermatol Surg
Adenoid cystic carcinoma of the scalp
Arch Dermatol
Primary cutaneous adenoid cystic carcinoma of the scalp with p16 expression: a case report and review of the literature
Am J Dermatopathol
Cited by (29)
Adnexal and Sebaceous Carcinomas
2023, Dermatologic ClinicsCitation Excerpt :Primary cutaneous secretory carcinoma (PCSC) is most often seen in the axilla as a dermal or subcutaneous nodule. Prognosis is excellent following surgical excision, without reported cases of metastasis or LN involvement.47,48 Apocrine adenocarcinoma (AA) is hypothesized to arise from apocrine hyperplasia and is seen in apocrine-rich areas such as the axilla as a slow-growing nontender nodule or plaque.49,50
Clinicopathological and genomic copy number variation analysis in nodular hidradenoma and hidradenocarcinoma with focus on prognostically important features
2022, Human PathologyCitation Excerpt :The malignant counterpart, hidradenocarcinoma, first described in the 1950s [2,11], was considered a rare malignant skin adnexal tumor with clinically aggressive behavior. Histologically, it may exhibit infiltrative growth, necrosis, perineural invasion, angiolymphatic invasion, increased mitotic activity, and significant nuclear pleomorphism [1–5,12–19]. Owing to the subjectivity of diagnostic criteria, histologic distinction between benign hidradenoma, hidradenocarcinoma, and ambiguous atypia can be arbitrary and challenging at times.
[Translated article] Primary Cutaneous Cribriform Carcinoma
2022, Actas Dermo-SifiliograficasUpdate on immunohistochemical techniques in dermatopathology. A focus on adnexal neoplasms.
2022, Seminars in Diagnostic PathologyCitation Excerpt :It is composed of basaloid cells in strand or nested configuration with duct formation and “tadpole” or “paisley-tie” appearance (Fig. 5). EC has similar histomorphological features to those of syringoma, but with distinctive infiltrative/destructive growth pattern, stromal fibrosis/sclerosis, cytologic atypia, inconspicuous mitosis, and perineural invasion 19, 20 (Fig. 6). In many instances, these tumors can be poorly differentiated and show no overlying epidermal or adnexal connection, making a diagnosis of a definitive primary cutaneous carcinoma a histologic challenge (Fig. 7).
Primary Cutaneous Cribriform Carcinoma
2022, Actas Dermo-SifiliograficasPrimary cutaneous adenoid cystic carcinoma: Characterizing US demographics, clinical course, and prognostic factors
2021, Journal of the American Academy of Dermatology
Disclosure Statement: The authors have no commercial or financial conflicts of interest to disclose.