Elderly woman referred for unusual upper eyelid lesion

A 15-mm raised sausage-shaped lesion was observed along the right upper lid margin.

Jeffrey Chang

Vivek Chaturvedi

A 74-year-old Caucasian woman was referred to the oculoplastics service at the New England Eye Center for an unusual lesion on her right upper lid. The patient was asymptomatic until 1 month before presentation, when she noted redness at the margin of her right upper lid. She saw her internist who treated her with an antibiotic ointment. The lesion continued to grow in size, and she was referred for treatment of the lesion after being seen by an ophthalmologist.

She described only mild itching and irritation in the area of the lesion, and denied pain, discharge or bleeding. She had a history of a skin lesion on her ear, which was removed several years prior, but was unsure whether the lesion was cancerous. The remainder of her clinical history was unremarkable.

Examination

On examination, a 15-mm raised sausage-shaped lesion was noted along the right upper lid margin (Figure 1). The lesion was reddish in color with overlying telangiectasia. Its margins were distinct. The skin surface was intact with no ulceration.

An incisional biopsy was performed in the office, and the specimen was sent for histologic analysis. Hematoxylin and eosin staining showed large nests of monomorphic cells in a trabecular arrangement with hyperchromatic nuclei and scant cytoplasm (Figure 2). A peripheral palisading pattern was absent. The tissue stained positive with cytokeratin-20 histochemical marker (Figure 3). With chromogranin neuroendocrine marker staining, the cytoplasm appeared dark, identifying and localizing these granules. Thyroid transcription factor-1 immunostaining was negative.

Figure 1. External photo of raised sausage-shaped lesion along right upper lid margin with telangiectasias. Images: Patron ME, Laver NM, Kapadia MK

Figure 2. Hematoxylin and eosin stain showing large nests of monomorphic cells in trabecular pattern with hyperchromatic nuclei and scant cytoplasm.

Figure 3. Positive cytokeratin-20 histochemical marker.

What is your diagnosis?

Right upper lid lesion

Based on this pathology, a diagnosis of Merkel cell carcinoma of the eyelid was made. Hematoxylin and eosin staining showed characteristics of a cutaneous small cell carcinoma. Merkel cell carcinoma and metastatic small cell carcinoma of the lung are examples of small cell lesions that may present in the eyelid. Positive staining with cytokeratin-20 and chromogranin markers suggested Merkel cell carcinoma, and negative staining with TTF-1 ruled out lung origin.

Discussion

Merkel cell carcinoma is an uncommon, aggressive primary cutaneous tumor. The tumor often exhibits early spread to regional lymph nodes or distant sites, and has a 5-year mortality rate of 50% in most published studies. The tumor arises from Merkel cells, specialized receptors involved in the sensation of touch. Most patients are elderly Caucasians, with women affected more often than men. The overall incidence of this disease is estimated to be about 0.23 per 100,000 people. Approximately 40% of all Merkel cell tumors are located in the head and neck region, with 5% to 10% of all tumors located in the eyelids.

Clinically, Merkel cell carcinoma presents as a solitary, painless nodule. The lesion is most commonly deep red in color, but may range from pink to violaceous. The skin surface is usually intact without ulceration and frequently has telangiectasias. The margins of the tumor often appear well-demarcated, but the tumor may invade deeper tissues. In the eyelids, Merkel cell tumors arise near the lid margin, most often in the upper lid, and may cause eyelash loss.

Because of the rarity of this disease, most lesions are initially misdiagnosed as chalazion or basal cell carcinoma. Diagnosis is established through biopsy of the tumor and histologic analysis.

Treatment

The treatment for Merkel cell carcinoma starts with complete surgical resection under frozen section control. Postoperative radiation appears to decrease the rate of local tumor recurrence and is advocated by most authorities. Formal lymph node dissection or sentinel node mapping is often performed for staging and therapeutic purposes.

References:

• Allen PJ, Bowne WB, Jaques DP, Brennan MF, Busam K, Coit DG. Merkel cell carcinoma: prognosis and treatment of patients from a single institution. J Clin Oncol. 2005;23(10):2300-2309.
• Kivelä T, Tarkkanen A. The Merkel cell and associated neoplasms in the eyelids and periocular region. Surv Ophthalmol. 1990;35(3):171-187.
• Missotten GS, de Wolff-Rouendaal D, de Keizer RJ. Merkel cell carcinoma of the eyelid: review of the literature and report of patients with Merkel cell carcinoma showing spontaneous regression. Ophthalmology. 2008;115(1):195-201.
• Peters GB 3rd, Meyer DR, Shields JA, et al. Management and prognosis of Merkel cell carcinoma of the eyelid. Ophthalmology. 2001;108(9):1575-1579.
• Singh AD, Eagle RC Jr, Shields CL, Shields JA. Merkel cell carcinoma of the eyelids. Int Ophthalmol Clin. 1993;33(3):11-17.
• Veness MJ, Palme CE, Morgan GJ. Merkel cell carcinoma: a review of management. Curr Opin Otolaryngol Head Neck Surg. 2008;16(2):170-174.

• Mark E. Patron, MD, Nora M. Laver, MD, and Mitesh K. Kapadia, MD, PhD, can be reached at Tufts Medical Center, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.

• Edited by Jeffrey Chang, MD, and Vivek Chaturvedi, MD. Drs. Chang and Chaturvedi can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.