Article: Concurrent uveoretinitis and pineocytoma in a child suggests a causal relationship

Uveoretinitis was observed in a 9-year-old girl 6 months prior to the clinical appearance of a pineal tumour. Surgical removal was not successful but biopsy revealed a parenchymal neoplasm with differentiated pinealocytes and absent mitotic activity. Some of the tumour cells contained S-antigen, rhodopsin, and serotonin. Systemic glucocorticoid therapy followed by radiation therapy caused considerable reduction in size of the tumour and a complete normalisation of all eye symptoms. This report demonstrates for the first time that a pineocytoma can occur together with uveoretinitis in humans. The latter resembles the experimentally induced autoimmune uveoretinitis described in animals. It is speculated that the retinitis might reflect an autoimmune response to S-antigen present in some tumour cells of the pineocytoma.

Citing Articles

Molecular genetics of pineal region neoplasms.

Taylor M, Mainprize T, Squire J, Rutka J J Neurooncol. 2002; 54(3):219-38.

PMID: 11767289 DOI: 10.1023/a:1012713506549.

Neuroendocrine alterations in uveitis patients.

Wollmann H, Pleyer U, Friedel S, Zierhut M, Thiel H, Gupta D Graefes Arch Clin Exp Ophthalmol. 1994; 232(5):297-301.

PMID: 8045439 DOI: 10.1007/BF00194479.

Structural and ultrastructural characteristics of human pineal gland, and pineal parenchymal tumors.

Jouvet A, Fevre-Montange M, Besancon R, Derrington E, Saint-Pierre G, Belin M Acta Neuropathol. 1994; 88(4):334-48.

PMID: 7839826 DOI: 10.1007/BF00310377.

References

1.

Merryman C, Donoso L, Zhang X, Heber-Katz E, Gregerson D . Characterization of a new, potent, immunopathogenic epitope in S-antigen that elicits T cells expressing V beta 8 and V alpha 2-like genes. J Immunol. 1991; 146(1):75-80. View

2.

Korf H, Bruce J, Vistica B, Rollag M, STEIN B, Klein D . Immunoreactive S-antigen in cerebrospinal fluid: a marker of pineal parenchymal tumors?. J Neurosurg. 1989; 70(5):682-7. DOI: 10.3171/jns.1989.70.5.0682. View

3.

Korf H, Czerwionka M, Reiner J, Schachenmayr W, Schalken J, de Grip W . Immunocytochemical evidence of molecular photoreceptor markers in cerebellar medulloblastomas. Cancer. 1987; 60(8):1763-6. DOI: 10.1002/1097-0142(19871015)60:8<1763::aid-cncr2820600815>3.0.co;2-3. View

4.

Perentes E, Rubinstein L, Herman M, Donoso L . S-antigen immunoreactivity in human pineal glands and pineal parenchymal tumors. A monoclonal antibody study. Acta Neuropathol. 1986; 71(3-4):224-7. DOI: 10.1007/BF00688043. View

5.

Nussenblatt R, MITTAL K, Ryan S, Green W, MAUMENEE A . Birdshot retinochoroidopathy associated with HLA-A29 antigen and immune responsiveness to retinal S-antigen. Am J Ophthalmol. 1982; 94(2):147-58. DOI: 10.1016/0002-9394(82)90069-1. View

6.

STERNBERGER L, HARDY Jr P, CUCULIS J, MEYER H . The unlabeled antibody enzyme method of immunohistochemistry: preparation and properties of soluble antigen-antibody complex (horseradish peroxidase-antihorseradish peroxidase) and its use in identification of spirochetes. J Histochem Cytochem. 1970; 18(5):315-33. DOI: 10.1177/18.5.315. View

7.

Bienfang D, Kelly L, NICHOLSON D, Nussenblatt R . Ophthalmology. N Engl J Med. 1990; 323(14):956-67. DOI: 10.1056/NEJM199010043231405. View

8.

DiSclafani A, Hudgins R, Edwards M, Wara W, Wilson C, Levin V . Pineocytomas. Cancer. 1989; 63(2):302-4. DOI: 10.1002/1097-0142(19890115)63:2<302::aid-cncr2820630216>3.0.co;2-d. View

9.

Korf H, Foster R, Ekstrom P, Schalken J . Opsin-like immunoreaction in the retinae and pineal organs of four mammalian species. Cell Tissue Res. 1985; 242(3):645-8. DOI: 10.1007/BF00225432. View

10.

Korf H, Klein D, Zigler J, Gery I, Schachenmayr W . S-antigen-like immunoreactivity in a human pineocytoma. Acta Neuropathol. 1986; 69(1-2):165-7. DOI: 10.1007/BF00687054. View

11.

Korf H, Moller M, Gery I, Zigler J, Klein D . Immunocytochemical demonstration of retinal S-antigen in the pineal organ of four mammalian species. Cell Tissue Res. 1985; 239(1):81-5. DOI: 10.1007/BF00214906. View

12.

Cohen I, Young D . Autoimmunity, microbial immunity and the immunological homunculus. Immunol Today. 1991; 12(4):105-10. DOI: 10.1016/0167-5699(91)90093-9. View

Concurrent Uveoretinitis and Pineocytoma in a Child Suggests a Causal Relationship