44222 Primary Cutaneous Adenoid Cystic Carcinoma: Report of a Rare Tumor with Review of the Literature

Document Type

Conference Proceeding

Publication Date

9-19-2023

Publication Title

J Am Acad Dermatol

Abstract

Background: A 53 year-old female presented with a 1-year history of a nodule on left parietal scalp. Physical examination revealed a 1cm subcutaneous nodule, with no overlying surface change. The lesion was presumed to be a cyst and treatment options included observation versus excision. The patient initially opted for observation, but returned 33 months later due to persistent enlargement. Punch biopsy revealed variably-sized cribriform nests of basaloid cells with abundant dermal mucin. Immunohistochemistry labeled tumor cells with CAM 5.2, CK7, P63, CD117, and EMA. Sequencing panel failed to detect a MYB fusion, though the constellation of findings were consistent with primary cutaneous adenoid cystic carcinoma (PCACC). Computed tomography imaging of head, neck, chest, and abdomen failed to reveal any metastasis. Treatment consisted of a wide local excision, which achieved clear histological margins and no perineural invasion. Compared to its more common salivary gland counterpart, PCACC represents a small subset of adenoid cystic carcinoma which can occur in various other organs. Approximately half of cases occur on head/neck, presenting as slow-growing, nondescript nodules that mimic more common benign cutaneous tumors and cysts. A subset of cases may develop local recurrences and rarely metastasis, often associated with one or more of the following features: perineural invasion, lesion diameter 2: 1 cm, involvement of subcutaneous fat, and widely infiltrative border. Due to the propensity for local recurrence, excision with two-centimeter margins has been advised. Small series have also demonstrated good outcomes with Mohs Micrographic Surgery, though sample sizes and follow up times were limited.

Volume

89

Issue

3

First Page

AB211

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