Ocular non-teratoid medulloepithelioma with teratoid metastases in ipsilateral intraparotid lymph nodes

Abstract

Purpose:

To describe a rare presentation of a case of intraocular non-teratoid medulloepithelioma with teratoid metastases in ipsilateral intraparotid lymph nodes.

Case description:

A 9-year-old male child with previous history of ciliary body non-teratoid medulloepithelioma presented with a swelling in the right pre-auricular region for 1 month. Magnetic resonance imaging and positron emission tomography–computed tomography showed a right intraparotid mass with enlarged ipsilateral cervical lymph nodes. A core biopsy was taken from the lesion, which on microscopy showed a tumor composed of small round cells arranged in cords, tubules lined by multilayered cells, and in cribriform pattern. These cells were embedded in a hypocellular, loose myxoid matrix. Based on the histopathological characteristics and previous history, a diagnosis of medulloepithelioma metastastic to ipsilateral parotid gland was made. The patient underwent right total conservative parotidectomy and bilateral neck dissection. Histopathological examination revealed metastatic medulloepithelioma in five out of eight intraparotid lymph nodes, with extranodal extension into the adjacent parotid parenchyma. Foci of hyaline cartilage were identified within the tumor, leading to a diagnosis of metastatic teratoid medulloepithelioma.

Conclusion:

Intraparotid lymph node metastases from intraocular medulloepithelioma is a rare possibility and we recommend that the parotid should be evaluated in cases of intraocular medulloepithelioma at initial presentation as well as during the follow-up period. Also, metastasis should be considered in all pediatric patients with solitary mass lesions showing unconventional histology for a primary parotid neoplasm.

Keywords

Medulloepithelioma intraocular tumor teratoid parotid lymph node metastases small round cell tumor

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References

Saunders

Margo

CE.

Intraocular medulloepithelioma. Arch Pathol Lab Med 2012; 136: 212–216.

Kaliki

Shields

Eagle

et al. Ciliary body medulloepithelioma: analysis of 41 cases. Ophthalmology 2013; 120(12): 2552–2559.

Verdijk

RM.

On the classification and grading of medulloepithelioma of the eye. Ocul Oncol Pathol 2016; 2(3): 190–193.

Eagle

Jr Biswas

Edward

et al. Medulloepithelioma of the ciliary body. In: Grossniklaus

Eberhart

Kivela

(eds) WHO classification of tumours of the eye. 4th ed. Lyon: IARC, 2018, pp. 81–83.

Shields

Eagle

Jr Shields

et al. Congenital neoplasms of the nonpigmented ciliary epithelium (medulloepithelioma). Ophthalmology 1996; 103(12): 1998–2006.

Broughton

Zimmerman

LE.

A clinicopathologic and follow-up study of fifty-six intraocular medulloepitheliomas. Am J Ophthalmol 1978; 85: 407–418.

Tsai

SY.

Malignant ciliary body medulloepithelioma with brain and parotid metastasis. J Pediatr Ophthalmol Strabismus 2017; 54: e18–e22.

Singh

Gupta

Kakkar

et al. Fine needle aspiration cytology of metastatic ocular medulloepithelioma. Cytopathology 2011; 22(5): 343–345.

Hellman

Harocopos

Lin

LK.

Successful treatment of metastatic congenital intraocular medulloepithelioma with neoadjuvant chemotherapy, enucleation and superficial parotidectomy. Am J Ophthalmol Case Rep 2018; 11: 124–127.

10.

Viswanathan

Mukul

Qureshi

et al. Orbital medulloepitheliomas—with extensive local invasion and metastasis: a series of three cases with review of literature. Int J Pediatr Otorhinolaryngol 2008; 72(7): 971–975.