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P07: Muir–Torre syndrome associated with treatment‐resistant actinic keratosis and microsatellite instability

P06 A nontender, well-circumscribed nodule on the left forehead C. Drumm, S. Flanagan, J. Ralph, C. D’Arcy and A. Alsharqi St Vincent’s University Hospital and St James’s Hospital, Dublin, Ireland A 75-year-old man presented with a nontender, slow-growing nodule on his left forehead. His medical history included idiopathic Parkinson disease, resected colon cancer, Janus kinase 2-positive polycythaemia rubra vera and a right caudate nucleus infarct. Physical examination revealed a 11 9 11 mm glassy nodule with some erythema and a pale centre on the patient’s left forehead near the hairline. The examination was otherwise normal. Histopathological examination of a punch biopsy from the 11-mm nodule revealed a lobular tumour located in the dermis composed of pale cells that formed well-circumscribed glands containing mucinous material. There was no epidermal connection and the overlying epidermis was normal. Mitoses were inconspicuous and the pleomorphism was mild. The lesion showed peripheral retraction artefact focally. The slides from the patient’s previous colorectal carcinoma resection were also reviewed and the current tumour was found to be morphologically different. Immunohistochemistry (IHC) showed that the tumour cells were strongly positive with cytokeratin 5/6 and p63 immunohistochemical preparations. Cytokeratin 7 showed patchy positivity. There was negative staining with colorectal markers. Mismatch repair IHC showed no loss of staining (9MLH1, PMS2, MSH2, MSH6). The patient was examined by the ear, nose and throat surgeons for an alternative primary neoplasm. No other primary site was identified, which confirmed the diagnosis of primary cutaneous adenoid cystic carcinoma (PCACC). The lesion was excised with a 5-mm margin and a subsequent advancement flap with no complications. Adenoid cystic carcinoma is a rare, slow-growing malignant adnexal tumour. It may appear in any location in which glands are located but it is most commonly found on the head or neck of people > 50 years old and affects men and women equally. The skin is a rare site for an adenoid cystic carcinoma. PCACC was first reported by Boggio in 1975 and an incidence of 0 23 per 1 million person-years was described in the US Surveillance, Epidemiology and End Results (SEER) programme. Clinically, the tumour may appear as a slow-growing, skin-coloured nodule with ill-defined edges. It characteristically consists of basophilic cells with a distinct adenoid or cribriform pattern in the mid-to-deep reticular dermis. It is important to exclude other primary tumours, particularly of the salivary glands. Treatment usually consists of wide local excision. Owing to high rates of local recurrence, long-term clinical follow-up is required. We present this rare presentation of an adenoid cystic carcinoma successfully treated with wide local excision.