BACKGROUND: Adnexal carcinomas (ACs) are rare cutaneous malignancies of sweat gland or pilosebaceous origin. Optimal treatment and metastatic potential of AC are poorly defined. Mohs micrographic surgery (MMS) has been increasingly used to treat AC.
OBJECTIVE: To review selected primary cutaneous AC and their treatment outcomes with MMS.
METHODS: Literature review using PubMed search for articles related to primary cutaneous ACs.
RESULTS: Sebaceous carcinoma treated with MMS recurred and metastasized in 6.4% and 3.7%, respectively. Primary cutaneous mucinous carcinoma treated with MMS recurred and metastasized in 9.6% and 6.4%, respectively. After MMS, 4.7% of microcystic AC recurred with no reported metastases. After MMS, recurrences and metastases of trichilemmal carcinoma or hidradenocarcinoma have not been reported. Two (4.2%) regional lymph node metastases but no distant metastases or local recurrences have been reported in eccrine porocarcinoma. Squamoid eccrine duct tumor, pilomatrix carcinoma, and spiradenocarcinoma treated with MMS are also reviewed.
CONCLUSIONS: The rarity of ACs and the lack of comparative data on treatment makes conclusive recommendations on treatment difficult. Recent large case series and reviews suggest MMS is a useful and possibly superior treatment for AC and should be considered if primary cutaneous disease is suspected.

Digital papillary adenocarcinoma is a rare malignant tumor of the sweat glands, most commonly encountered in adult males. Only few pediatric cases have been reported, with an apparent predominance of females and none on the ankle. We report a case of a 15-year-old girl with a slowly growing, non-tender mass on the right ankle presenting with difficulty in walking; clinically believed to be a keloid. Excision of the mass revealed a glandular neoplasm with largely eccrine and focally apocrine differentiation. Histologic features do not reliably predict biologic behavior. Because of the existing confusion over the nomenclature, we propose that such tumors be called \'papillary adnexal neoplasms of distal extremities\'.

Proliferating trichilemmal tumor (PTT) is an uncommon appendageal skin tumor that affects mainly elderly women. It arises from the external root sheath of the hair follicle and is most commonly observed on the scalp. Although most cases pursue a favorable clinical course and surgical excision is curative, malignant transformation has rarely been reported in these lesions. Because of limited number of cases reported in the literature, management of malignant PTT is controversial and mainly entails wide local excision. Many other adjuvant modalities have been tried. In this report, we present a case of malignant PTT in a 65-year-old male patient who presented with ulceroproliferative growth over occipital region since 4 months.

Microcystic adnexal carcinoma (MAC) is an uncommon, locally aggressive cutaneous neoplasm that usually presents as a slow-growing, asymptomatic lesion on the head or neck. Microcystic adnexal carcinoma frequently is misdiagnosed due to its histologic appearance on superficial biopsy specimens mimicking other follicular neoplasms. Herein, we highlight a case in which a slow-growing lesion was initially diagnosed as a trichoadenoma following superficial biopsy; however, after surgical excision the pathology revealed a locally aggressive MAC.

OBJECTIVE: Trichoblastomas are rare and benign tumors that arise from rudimentary hair follicles. Presentation varies from superficial plaques to papular or nodular lesions. Trichoblastomas usually arise on the head or neck. A few cases of other vulvar trichoblastic tumors such as trichofibromas and trichoepitheliomas have been reported to this day, but no such report of vulvar trichoblastoma exists.
METHODS: We report the case of a 61-year-old woman who presented with a vulvar trichoblastoma. The woman presented with a lump of the labium majus. She was managed surgically, first by wide excision of the mass followed by a second surgery consisting of a partial vulvectomy. Because surgical margins were positive and there is a potential for malignant transformation, a third surgery was performed. The margins came back negative. A literature review on trichoblastomas was performed, including its potential for malignant transformation, management, and immunohistochemistry to differentiate it from basal cell carcinoma.
RESULTS: Trichoblastomas are benign tumors that have a potential for malignant transformation. These tumors can present aggressive characteristics. Differential diagnosis from basal cell carcinoma can be difficult but is facilitated using immunohistochemistry.
CONCLUSIONS: This is the first case of vulvar trichoblastoma reported in the literature. Although it is a benign tumor, excision with negative margins is recommended because it can be difficult to distinguish from basal cell carcinoma and it has a potential for malignant transformation.

Microcystic adnexal carcinoma of the skin is a very rare malignant tumour arising from the sweat glands. As far as we know, the MRI features of this tumour have not been described in the literature before. In this report we present the MRI features and pathological description of a case of a microcystic adnexal carcinoma in the cheek that was incidentally imaged during brain MRI examination. A review of the relevant literature as well as a discussion of MRI of skin tumours is also presented.

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Trichoepithelioma is a rare benign skin lesion arising from hair follicles. It most commonly arises on the face and scalp. It is exceptionally rare on the vulva. We present a case and review the literature.

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The secondary skin malignancies arising in seborrheic keratosis (SK) are uncommon, and the causal association between the pre-existing lesion and subsequent malignant transformation remains uncertain. Among these enigmatic conditions, trichilemmal differentiation and/or neoplasms in SK have rarely been reported thus far. Herein, we describe a case of invasive trichilemmal carcinoma arising in a long-standing SK of the abdominal skin, and clinicopathologically review this rare complication with a computerized medical published work search (PubMed) and citations from earlier reports. To our knowledge, only four cases of trichilemmal tumors arising in SK have been observed, and, interestingly, all cases, including ours, were Japanese. Four of five cases (80%) developed the tumors in non-sun-exposed SK, and indeed had no apparent actinic damage in the histology. The pre-existing SK itself is more likely to act as the primary pathogenic event for developing the secondary trichilemmal tumors than a coincidental phenomenon and a consequence of skin damage by cumulative sun exposure.