Primary Neuroendocrine Carcinoma of Ocular Adnexa

Abstract

We present our findings in a case of primary neuroendocrine carcinoma (NEC) of the lacrimal gland and a case of primary Merkel cell carcinoma (MCC) of the eyelid. An 86-year-old man noticed a swelling of the left upper eyelid three months earlier. We performed excision biopsy and histopathological examination indicated that he had a primary NEC of the left lacrimal gland. He underwent chemotherapy followed by excision including the clinically visible margins and 50 Gy radiotherapy of the surgical margins. He had neither recurrence nor metastasis for 6 months since the last radiotherapy. An 80-year-old man noticed a nodule in the right upper eyelid and was referred to our hospital because the size was increasing rapidly. A complete surgical excision of the margins of the tumor was performed with histopathological confirmation of negative margins. The final diagnosis was a primary MCC of the right upper eyelid. After surgery, he underwent 50 Gy radiotherapy on the neck to prevent metastasis. No recurrence or metastasis was found for two years. Although primary NEC of the ocular adnexa is extremely rare, the tumor has high malignancy and readily metastasizes. Thus, combined therapy including surgery, radiotherapy, and/or chemotherapy is needed for complete management of NEC.

Review of 268 lacrimal gland biopsies in an Australian cohort

“…Adenoidcystic carcinoma is the most common malignant epithelial tumour of the lacrimal gland and accounted for over half of the malignant epithelial lesions in our series. Primary neuroendocrine carcinoma of the lacrimal gland is exceedingly rare, and only one case has been reported previously . Our patient was a 93‐year‐old woman with a 3‐month history of left lacrimal gland swelling who subsequently underwent exenteration.…”

Small Cell Neuroendocrine Carcinoma Presenting as Recurrent Dacryocystitis: Case Report of a Rare Entity

“…5 There are reports of neuroendocrine carcinoma occurring as primary lesions of the orbit, ocular adnexa, and lacrimal gland, as well as the paranasal sinuses, with one previous reported entity of a primary lacrimal sac tumor. [5][6][7][8][9][10][11][12][13][14][15][16] Histopathological analysis of SCNECs may be hard to differentiate compared to other small round cell tumors including but not limited to metastatic lung primary small cell carcinoma, certain malignant lymphomas, melanomas, and most similarly Merkel cell carcinoma. 17 Regardless of the primary location of these tumors, their morphologic, ultrastructural, and immunohistochemical features are similar to pulmonary small cell carcinomas.…”

“…Though their rarity makes definitive patterns difficult, there have been at least 7 previously reported cases of sinonasal malignancies with extension into the orbit presenting with a myriad of symptoms (Table ). [5][6][7][8][9][10][11][12][13][14][15][16] It should be noted that none specifically presented as acute dacryocystitis. Theoretically, the DCR ostomy site could have allowed a local malignancy to easily enter the orbit in the patient.…”

“…5 There are reports of neuroendocrine carcinoma occurring as primary lesions of the orbit, ocular adnexa, and lacrimal gland, as well as the paranasal sinuses, with one previous reported entity of a primary lacrimal sac tumor. 5–16…”

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“…Regarding the morphological division of exocrine glandular epithelium, it is considered that the compound exocrine glands can also be classified according to the number of the lobules they form; thus, there are: (i) unilobulated glands, which contain a single lobule and their intralobular excretory ducts converge into a single terminal duct, opening at the surface (e.g., minor salivary glands) [27]; (ii) multilobulated glands, which contain several lobules and intralobular excretory ducts; the lobules are separated by conjunctive septa, containing interlobular excretory ducts, converging into a single terminal duct, opening at the surface (e.g., major salivary glands) [28]; (iii) "battalion" glands, multilobulated glands, in which each lobule has a terminal and independent excretory duct (e.g., lacrimal [29], mammary [30] and prostate [31] glands). The epithelia of salivary, mammary, and prostate glands share therefore many similarities, as they are composed of epithelial acinar and ductal cells, myoepithelial cells [32] and neuroendocrine cells [31,33,34]. Also, in these epithelial tissues, the myoepithelial cells are the key cellular participants in morphogenesis, maintenance, and repair [35], with similar mechanisms of self-renewing and differentiation (e.g., overexpression of stromal-derived factor-1alpha -SDF-1α or CXCL12α) [32].…”