Immediate umbilical reconstruction after a Mohs micrographic surgery for primary cutaneous adenoid cystic carcinoma arising in the umbilicus

Adenoid cystic carcinoma (ACC) is a malignant neoplasm of glands commonly occurs in salivary glands. Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare form of ACC that primarily presents on the skin. Herein, we represent a rare case of PCACC occurred in the umbilicus in a 66-year-old Korean male patient. The patient visited our center with erythematous indurated patch on the umbilicus diagnosed as ACC by incisional biopsy at another center. The diagnosis of PCACC was confirmed by additional histopathologic examination and imaging study. We proceeded Mohs micrographic surgery and reconstructed umbilicus with tacked purse string suture. Local recurrence and distant metastasis were not observed during 30-month follow-up. We report this rare case of PCACC on the umbilicus so that dermatologist can aware of the rare disease. Furthermore, we recommend MMS and tacked purse string suture as effective methods for treatment of PCACC and immediate umbilical reconstruction.