Primary cutaneous adenoid cystic carcinoma of the upper lid: a case report and literature review

Abstract

To report on the diagnostic challenge of an adenoid cystic carcinoma arising from the eyelid. A 77-year-old male was referred to our center with a clinical diagnosis of upper eyelid chalazion for a lesion that had appeared 2 years before. A loss of cilia was observed over the cutaneous area of induration, but there was no reddening or ulceration. Incisional biopsy was performed and the specimen was submitted in formalin for histopathological examination. On light microscopy, the lesion was composed of basaloid epithelial and myoepithelial cells that were arranged in strands or nests and associated with cystic spaces that contained a deeply eosinophilic secretory substance and an Alcian blue-positive material, characteristic of adenoid cystic carcinoma. After histological diagnosis, tumor re-excision was performed to ensure adequacy of resection margins, as well as a sentinel lymph node procedure, resulting in complete excision of the malignant tumor. No recurrence was observed during the first 18 months after surgery. Adenoid cystic carcinoma is a rare and aggressive epithelial malignancy, which tends to grow slowly and should be considered in the differential diagnosis of eyelid tumors simulating chalazion.

Primary cutaneous adenoid cystic carcinoma of the eyelid and literature review

“…Cacchi et al (6) reported that the diagnosis of PCACC is typically made approximately 10 years after onset and at a patient mean age of 59 years. While Cavazza et al (1) reported that ACC of the eyelid characteristically emerges with painful nodules together with eyelash loss or irregularity, the mass lesion observed in our case was small and not accompanied by any significant eyelash changes or nodules. We hypothesize that this diffe rence in appearance resulted from the establishment of diagnosis four rather than 10 years after onset and when the patient was 52 rather than 59 years of age.…”

“…Analysis of these pathological findings resulted in the diagnosis of PCACC. the origin of the remaining three was not reported (1,7) . Cavazza et al (1) concluded that both eyelids were equally involved, as four cases developed from the upper eyelid and four from the lower eyelid.…”

“…Adenoid cystic carcinoma (ACC) is an epithelial tumor seen mainly in the major salivary glands and rarely in the minor salivary glands, esophagus, bronchial glands, skin, breast, lungs, vulva, cervix, and pros tate (1)(2)(3) . First described by Boggio et al in 1975, primary cutaneous ACC (PCACC) is a rare type of ACC, with only approximately 60 PCACC cases reported in the literature (4)(5)(6) .…”

“…The mean duration between the onset of disease and diagnosis is 10 years, and patient mean age at the time of detection is 59 years (6) . Although local recurrence is frequently observed, distant metastasis of PCACC, mainly to the lungs, has rarely been reported (1,6) . Although PCACC most commonly originates from the palpebral part of the lacrimal gland, eyelid involvement has also been observed.…”

“…Although PCACC most commonly originates from the palpebral part of the lacrimal gland, eyelid involvement has also been observed. PCACC of the eyelid is very rare, and almost all cases described in the literature were treated surgically for chalazion and the diagnosis of PCACC was established either pathologically or due to postoperative recurrence (1,7) . To further explore the nature of and findings associated with PCACC, we present data on treatment of a case of PCACC admitted to our eye clinic presenting with mass lesions in the lower eyelid and review the scientific literature relevant to PCACC.…”