J Korean Ophthalmol Soc.  2018 Sep;59(9):861-866. 10.3341/jkos.2018.59.9.861.

Poorly Differentiated Neuroendocrine Carcinoma of the Eyelid

• Cho K
• Kim JH
• Lee DC
• Suh YL
• Woo KI
• Kim YD

• KMID: 2420335
• DOI: http://doi.org/10.3341/jkos.2018.59.9.861

Abstract

PURPOSE
To report a case of poorly differentiated neuroendocrine carcinoma of the eyelid.
CASE SUMMARY
A 70-year-old male presented with a 5-month history of a right upper eyelid mass. The mass appeared as 1.2 × 1.2 cm on the right upper eyelid. A mass excision was performed under frozen section control. The tumor was completely excised with a safety margin clearance and an upper eyelid reconstruction was performed. Histopathological examination revealed a tumor composed of small atypical cells which showed a high nuclear/cytoplasm ratio, nuclear molding, and increased mitotic activity. Immunohistochemical examination revealed positive reactivity for Ki-67, synaptophysin, CD56, and negative reactivity for chromogranin, cytokeratin 20, and thyroid transcription factor-1.
CONCLUSIONS
Primary neuroendocrine carcinoma of the eyelid is extremely rare, but the tumor has high malignancy and readily metastasizes. Poorly differentiated neuroendocrine carcinoma should be considered in the differential diagnosis of a rapidly growing eyelid mass.

Keyword

Eyelid; Merkel cell carcinoma; Poorly differentiated neuroendocrine carcinoma; Small cell carcinoma; Small cell neuroendocrine carcinoma

MeSH Terms

Aged
Carcinoma, Merkel Cell
Carcinoma, Neuroendocrine*
Carcinoma, Small Cell
Diagnosis, Differential
Eyelids*
Frozen Sections
Fungi
Humans
Keratin-20
Male
Synaptophysin
Thyroid Gland
Keratin-20
Synaptophysin

Figure

• 

  Figure 1. Clinical photograph of the patient. (A) Clinical photograph demonstrates mass on the lateral side of right upper eyelid. (B) yellowish and papillary mass on the upper eyelid is noted. (C) well vascularized 1.2 × 1.2 cm sized mass on the right upper eyelid is noted.

• 

  Figure 2. Clinical photograph of the patient's surgery. (A) Intraoperative photography showing the marked tumor of the upper eyelid. (B) Full-thickness upper eyelid defect is shown after tumor excision under frozen-section control. (C) The tarsoconjunctival flap is made and advanced in the upper eyelid defect. (D) The flap and alloderm graft is sutured to the upper eyelid levator muscle, the medial tarsal remnant, and the lateral canthal tendon remnant. (E) Mobilized orbicularis muscle is shown. (F) A posterior auric-ular skin graft is sutured.

• 

  Figure 3. Histopathological examination of mass. (A) Low-power magnification of tumor shows nests of small round cells with increased nuclear to cytoplasmic ratio and hyperchromic nuclei (hematoxylin and eosin, ×100). (B) High-power magnification reveals molding of small round cells with increased nuclear to cytoplasmic ratio (hematoxylin and eosin, ×400). (C-E) Immunohistochemical stains show positivity for synaptophysin, CD56 and negativity for chromogranin, respectively (×100).

• 

  Figure 4. Head and neck magnetic resonance imaging demonstrating nodule in the right parotid gland. (A) T1-weighted axial image demonstrating 1.5 cm-sized mass on superficial lobe of the right parotid gland with low signal intensity. (B) T2-weighted axial image demonstrating high signal intensity mass on the right parotid gland. (C) Diffusion weighted image demonstrating diffusion restricted 1.5 cm-sized nodule in right parotid gland. T1WI = T1-weighted imaging; T2WI = T2-Weighted imaging; DWI = diffusion weighted imaging.

• 

  Figure 5. Clinical photographs of the patient. (A) At 1 year after surgery. (B) At 1 year after right superficial parotidectomy with facial nerve preservation, selective neck dissection, sternocleidomastoid muscle flap surgery.

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