Mucin-producing Malignant Tumor of Lower Eyelid Presenting in a 14-year-old Patient

Abstract

Malignancies of the ocular adnexa are rare, aggressive tumors with significant potential for local recurrence and metastases. Although basal cell carcinoma remains the most common malignancy of the eyelid, encompassing more than 90 percent of all periocular cancers, several other malignant neoplasms have been reported. Malignant ocular adnexal neoplasms are most commonly of sweat gland origin and include hidradenocarcinoma, mucinous eccrine adenocarcinoma, and apocrine adenocarcinoma of the glands of Moll. The different ocular adnexal adenocarcinomas share many of the same characteristics with regard to incidence, primary location, and rates of recurrence and metastases. As a result, these tumors are difficult to distinguish clinically and true diagnosis depends on histological findings. The highest incidence of the reported cases occurred during the sixth decade of life. The head, neck, and trunk are the most frequently reported primary locations. Ocular adnexal adenocarcinomas have a significant rate of metastasis to regional lymph nodes as well as distant sites. Standard treatment of care includes surgical excision of the tumor; however, these malignancies have a tendency to recur locally. The authors present a 14-year-old Caucasian girl with no significant past medical history who presented with a recurrent right lower eyelid tumor. She previously had multiple resections at an outside hospital; however, the margins could not be cleared. The patient was taken to the operating room for Mohs-type resection of the primary site as well as a right parotidectomy with facial nerve preservation and right modified radical neck dissection. The final pathology was consistent with a mucin-producing malignant tumor of ocular adnexa. Due to the great potential for local recurrence and metastases, the prognosis for ocular adnexal adenocarcinoma remains poor even with aggressive surgical management. The use of Mohs surgery for resection has increased as it has been linked to prolonged intervals of metastatic-free disease.

Figure 1

A 14-year-old Caucasian girl with a history of a recurrent right lower eyelid tumor

Figure 2

A 1.9×1.6×0.6cm portion of the right lower eyelid skin and conjunctiva was excised, extending to the lateral canthus.

Figure 3

A blue papule, measuring 0.4×0.2cm, was present within the excised skin.

Figure 4

Atypical epithelioid nests along with focal areas of fibrosis

Figure 5

Atypical epithelioid cells and goblet cells

Figure 6

Fourteen-month follow up after surgery