Adenoid Cystic Carcinoma of Cutaneous Tissues

Adenoid cystic carcinoma of cutaneous tissues is a rare malignancy arising primarily in the skin, distinct from its more common salivary gland counterpart. It typically presents as a slow-growing nodular or ulcerated lesion on sites such as the scalp, chest wall or periocular skin in middle-aged and older adults. Histologically, it is characterised by dual populations of epithelial and myoepithelial cells arranged in tubular, cribriform and solid patterns. A hallmark feature is perineural invasion, which contributes to local recurrence and neurological symptoms. Diagnosis relies on histopathology complemented by immunohistochemistry to distinguish it from other adnexal tumours and metastatic disease. Standard management involves wide local excision or Mohs micrographic surgery to achieve clear margins, often supplemented by radiotherapy in cases of positive margins or perineural spread. Recent efforts have focused on elucidating the molecular drivers—such as MYB gene rearrangements—and exploring targeted therapies to improve long-term control and reduce morbidity.

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Adenoid Cystic Carcinoma of Cutaneous Tissues publication trend

The graph below shows the total number of articles in adenoid cystic carcinoma of cutaneous tissues across all publications each year (not limited to Nature Index journals).

Technical terms

Primary cutaneous adenoid cystic carcinoma (PCACC): a malignant tumour of the skin displaying characteristic cribriform, tubular and solid architectures derived from eccrine or apocrine structures.

Perineural invasion: infiltration of cancer cells along nerves, often resulting in pain, sensory changes and higher rates of local recurrence.

Immunohistochemistry: a diagnostic method using labelled antibodies to detect specific proteins in tissue sections, aiding tumour classification.

Mohs micrographic surgery: a precise surgical technique in which layers of skin are removed and examined microscopically until clear margins are achieved, minimising tissue loss.

References

1. Two Different Cell Populations Is an Important Clue for Diagnosis of Primary Cutaneous Adenoid Cystic Carcinoma: Immunohistochemical Study. Case Reports in Pathology (2017).
2. A Unique Case of Primary Cutaneous Adenoid Cystic Carcinoma Associated with Aplasia Cutis Congenita in a Four-Year-Old Female: A Case Report. Children (2022).
3. Primary Cutaneous Adenoid Cystic Carcinoma of the Back: A Case Report. Cureus (2023).