Concurrent Benign Teratoid Medulloepithelioma and Pineoblastoma

Abstract

Teratoid medulloepithelioma of the ciliary body is a rare tumor usually occurring in young children. Primary tumors of the pineal gland are also quite rare and have a similar age distribution. We report the case of a 2-year-old girl who had a leukocoria of the right eye. Cataract surgery was performed; however, the patient developed a total retinal detachment refractory to treatment. She eventually developed early changes of phthisis bulbi and underwent an evisceration. Histopathologic examination revealed a benign teratoid medulloepithelioma containing islands of hyaline cartilage and rhabdomyoblasts. During the course of her evaluation, a pineoblastoma was discovered and surgically excised. While the association of pineal tumors with retinoblastomas has been well established, no such association has been reported for medulloepitheliomas. To our knowledge, this is the first recorded case of a benign teratoid medulloepithelioma occurring concurrently with a pineoblastoma.

An advanced case of pediatric ciliary body medulloepithelioma with detailed literature review

“…A total of 65 studies involving 97 children with medulloepithelioma of the CB were considered eligible for inclusion in this review and we added our present case, with a total of 98 cases summarized in Table 1 . [ 2 , 4 , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] , [24] , [25] , [26] , [27] , [28] , [29] , [30] , [31] , [32] , [33] , [34] , [35] , [36] , [37] , [38] , [39] , [40] , [41] , [42] , [43] , [44] , [45] , [46] , [47] , [48] , [49] , [50] , [51] , [52] , [53] , [54] , [55] , [56] , [57] , [58] , [59] , [60] , [61] , [62] , [63] , [64] , [65] , [66] , [67] , [68] , [69] , [70] , [71] ] The other 3 cases series that were not included in the analysis are summarized in Table 2 for further information […”

“…Recurrence or necessity of enucleation after iridocyclectomy were reported in 9 (9.1 %), [ 4 , 12 , 18 , 29 , 30 , 34 , 36 , 44 , 50 ] regional metastasis to the neck in 2 (2 %) [ 37 , 52 ], and/or parotid in 5 (5 %) cases [ 41 , 61 , 65 , 68 , 72 ]. CB medulloepithelioma associated with other tumors such as pleuropulmonary blastoma or pineoblastoma (5/5 %) [ 8 , 13 , 23 , 51 , 58 , 61 ] were also reported.…”

“…Intracranial involvement can occur even with adjuvant chemotherapy or radiotherapy [ 8 , 63 , 76 ]. Additionally, medulloepithelioma can be associated to pleuropulmonary blastoma, which was observed in 1 % of patients included in an international Pleuropulmonary Blastoma Registry and presented in 5 % of our review of the literature [ 13 , 23 , 51 , 58 , 61 ]. Although the proportion of histologically malignant medulloepitheliomas is high, death was observed in 10 % to 12 % of cases [ 5 , 34 ].…”