185:". Up to 5% of patients with hereditary retinoblastoma are at risk of developing trilateral retinoblastoma. This tumor combination is more aggressive than an isolated pineoblastoma. Prognosis of patients with trilateral retinoblastoma is dismal, only a few patients have survived more than 5 years after diagnosis; all survivors were diagnosed with small tumors in a subclinical stage. Recent advances in (high-dose) chemotherapy treatment regimens and early detection have improved survival of patients with trilateral retinoblastoma to up to 50%.
63:
221:
99:
313:
Pineoblastomas are very aggressive tumors. 5-year survival for patients with pineoblastomas is around 58%. Prognosis for patients under 5 years old is lower, between 15 and 40%. Disseminated disease at diagnosis is also associated with worse outcomes. When pineoblastomas occur with retinoblastomas,
129:
Pineoblastomas typically occur at very young ages. One study found the average age of presentation to be 4.3 years, with peaks at age 3 and 8. Another cites cases to more commonly occur in patients under 2 years of age. Rates of occurrence for males and females are similar, but may be slightly more
240:
and peripheral calcification of the pineal gland. On MRI, pineoblastomas again appear as masses with heterogenous enhancement. They often appear hypo- to isointense on T1 and slightly hyperintense on T2-weighted images. Some areas of necrosis or hemorrhage may be seen as well. PET-CT has also been
296:
Initial treatment for pineoblastoma often includes a shunting procedure to redirect accumulated cerebrospinal fluid secondary to obstructive hydrocephalus. This shunt can help manage increased intracranial pressure and relieve some symptoms. Surgery to remove the tumor is associated with better
211:
Due to the aggressive nature of the disease, tumor spread at the time of diagnosis is common. Pineoblastomas often invades locally, with spread to the head and spine seen in 25–41% of patients. While CNS spread is relatively common, these tumors rarely cause distant metastases.
146:
secretion. Several tumors can occur in the area of the pineal gland, with the most aggressive being pineoblastoma. Pineoblastomas arise from embryonal cells in the pineal gland and are rapidly growing. They are considered grade 4 tumors, meaning they are
883:
247:
Biopsy is required for diagnosis. Pineoblastomas appear as high grade, highly cellular, small blue cells histologically. Features of aggressive malignancies can be seen, like high nucleus-to-cytoplasm ration, poorly differentiated cells,
130:
common in females. One study found incidence of pineoblastoma to be increased in black patients compared to white patients by around 71%. This difference was most apparent in patients aged 5 to 9 years old.
372:
297:
outcomes, however, this is not always possible due to the proximity of the pineal gland to neurovascular structures. Complete tumor resection is only seen in about 30% of cases. Following surgery,
256:, or neuroblastic, and Flexner-Wintersteiner, or retinoblastic, rosettes can also be seen. In contrast to other masses of the pineal gland, pineocytomatous rosettes are not present.
805:
Amato-Watkins AC, Lammie A, Hayhurst C, Leach P (2016-01-02). "Pineal parenchymal tumours of intermediate differentiation - An evidence-based review of a new pathological entity".
301:
to the brain and spinal cord can increase survival. However, radiation can only safely be used in patients over 3 years old due to the risk of significant neurological impairment.
895:
317:
Complete gross tumor resection is associated with improved prognosis, but is difficult and rare to achieve. Radiation therapy after surgery is also linked to improved survival.
942:
613:
Schultz KA, Stewart DR, Kamihara J, Bauer AJ, Merideth MA, Stratton P, et al. (1993). "DICER1 Tumor
Predisposition". In Adam MP, Everman DB, Mirzaa GM, Pagon RA (eds.).
688:
de Jong MC, Kors WA, de Graaf P, Castelijns JA, Moll AC, Kivelä T (December 2015). "The
Incidence of Trilateral Retinoblastoma: A Systematic Review and Meta-Analysis".
155:. Due to the pineal gland's location at the center of the brain and the rapidly growing nature of this disease, obstruction of CNS fluid is a common symptom.
733:
Kivelä T (June 1999). "Trilateral retinoblastoma: a meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma".
1045:
343:
935:
196:
The most common symptoms to occur with pineoblastoma are headache, behavior changes, and cognitive disturbances. These masses also often cause
422:
Sin-Chan P, Li BK, Ho B, Fonseca A, Huang A (July 2018). "Molecular
Classification and Management of Rare Pediatric Embryonal Brain Tumors".
1276:
1271:
1266:
928:
121:. Pineoblastoma can present at any age, but is most common in young children. They account for 0.001% of all primary CNS neoplasms.
188:
Additionally, various mutations or deletions in chromosomes 1, 9, 13, 16 and 22 have been associated with pineoblastoma incidence.
1125:
972:
118:
646:"Radiologic-pathologic correlation. Bilateral retinoblastoma with coexistent pinealoblastoma (trilateral retinoblastoma)"
1297:
1258:
253:
241:
used in diagnosis, and shows increased uptake of fludeoxyglucose with pineoblastomas compared to other pineal masses.
233:
1115:
1092:
182:
1168:
1108:
1087:
951:
903:
1078:
550:
261:
162:
dysregulation has been found to be associated with many cases of pineoblastoma, specifically, mutations in
1163:
305:
treatment can also be used, either before or after surgery; its optimal use is still under investigation.
205:
1220:
201:
578:
Blessing MM, Alexandrescu S (June 2020). "Embryonal Tumors of the
Central Nervous System: An Update".
1302:
1210:
1040:
257:
237:
62:
1215:
1103:
244:
Diagnosis also requires CSF sampling via lumbar puncture to assess for cytology and tumor markers.
92:
Just under half of all pineal gland tumors, which make up fewer than 1% of all primary brain tumors
1023:
269:
71:
768:
645:
174:
cause a tumor predisposition syndrome, and should be considered in patients with pineoblastoma.
1202:
1050:
1031:
967:
822:
750:
715:
667:
620:
595:
532:
483:
439:
298:
171:
51:
1180:
1120:
1065:
814:
742:
705:
697:
657:
587:
522:
514:
475:
466:
Tamrazi B, Nelson M, Blüml S (February 2017). "Pineal Region Masses in
Pediatric Patients".
431:
1243:
1175:
1133:
999:
501:
Greppin K, Cioffi G, Waite KA, Ostrom QT, Landi D, Takaoka K, et al. (April 2022).
314:
the prognosis is typically worse, and these patients require more aggressive treatment.
1158:
1073:
662:
527:
502:
178:
220:
1291:
1192:
1185:
1150:
1138:
277:
273:
249:
197:
840:
339:
181:. When retinoblastoma patients present with pineoblastoma this is characterized as "
1230:
1096:
1035:
302:
139:
114:
818:
769:"Pineoblastoma - About the Disease - Genetic and Rare Diseases Information Center"
908:
228:
Several imaging methods can be used to diagnose pineoblastoma. Initially, urgent
1248:
1055:
981:
377:
142:
is a small organ in the center of the brain that is responsible for controlling
746:
614:
591:
17:
1008:
995:
990:
977:
920:
701:
479:
435:
281:
152:
148:
1238:
986:
143:
826:
754:
719:
624:
599:
536:
487:
443:
98:
671:
518:
959:
159:
56:
875:
710:
229:
177:
Pineoblastoma may occur in patients with hereditary uni- or bilateral
1013:
1004:
887:
285:
167:
76:
Nausea, vomiting, headache, double vision, problems with eye movement
644:
Provenzale JM, Weber AL, Klintworth GK, McLendon RE (January 1995).
219:
163:
110:
97:
265:
924:
503:"Epidemiology of pineoblastoma in the United States, 2000-2017"
236:. CT will show large, multilobulated masses with heterogenous
865:
551:"Pineal Region Tumors Diagnosis and Treatment - NCI"
1257:
1229:
1201:
1149:
1064:
1022:
958:
869:
619:. Seattle (WA): University of Washington, Seattle.
88:
80:
70:
50:
40:
35:
117:. A pineoblastoma is a supratentorial midline
936:
158:The exact cause of pineoblastoma is unknown.
8:
943:
929:
921:
866:
61:
32:
709:
661:
526:
650:AJNR. American Journal of Neuroradiology
334:
332:
330:
204:. This can result in vision changes and
84:Childhood, between the ages of 20 and 40
326:
800:
798:
796:
794:
792:
790:
788:
683:
681:
468:Neuroimaging Clinics of North America
417:
415:
344:St. Jude Children's Research Hospital
7:
573:
571:
461:
459:
457:
455:
453:
413:
411:
409:
407:
405:
403:
401:
399:
397:
395:
366:
364:
362:
360:
27:Cancerous tumor of the pineal gland
25:
690:American Journal of Ophthalmology
272:marker positivity. This includes
1041:ACTH-secreting pituitary adenoma
973:Pancreatic neuroendocrine tumor
807:British Journal of Neurosurgery
202:increased intracranial pressure
119:primitive neuroectodermal tumor
1046:GH-secreting pituitary adenoma
102:Pineoblastoma on brain imaging
1:
819:10.3109/02688697.2015.1096912
232:are recommended, followed by
735:Journal of Clinical Oncology
373:"Brain Tumor: Pineoblastoma"
952:Tumours of endocrine glands
288:marker, positive staining.
1319:
747:10.1200/JCO.1999.17.6.1829
592:10.1016/j.path.2020.01.003
580:Surgical Pathology Clinics
224:Histology of pineoblastoma
773:rarediseases.info.nih.gov
702:10.1016/j.ajo.2015.09.009
480:10.1016/j.nic.2016.08.002
436:10.1007/s11912-018-0717-7
198:obstructive hydrocephalus
183:trilateral retinoblastoma
1169:Adrenocortical carcinoma
424:Current Oncology Reports
1126:Squamous-cell carcinoma
507:Neuro-Oncology Practice
284:, a specific pineal or
1164:Adrenocortical adenoma
225:
103:
260:staining will reveal
250:high mitotic activity
223:
101:
1211:Parathyroid neoplasm
258:Immunohistochemistry
238:contrast enhancement
1298:Endocrine neoplasia
1104:Parafollicular cell
696:(6): 1116–1126.e5.
519:10.1093/nop/npac009
371:Mayo Clinic Staff.
226:
172:germline mutations
104:
1285:
1284:
1051:Craniopharyngioma
1032:Pituitary adenoma
968:Pancreatic cancer
918:
917:
299:radiation therapy
192:Clinical features
96:
95:
30:Medical condition
16:(Redirected from
1310:
1181:Pheochromocytoma
945:
938:
931:
922:
867:
855:
854:
852:
851:
837:
831:
830:
802:
783:
782:
780:
779:
765:
759:
758:
741:(6): 1829–1837.
730:
724:
723:
713:
685:
676:
675:
665:
641:
635:
634:
632:
631:
610:
604:
603:
575:
566:
565:
563:
562:
547:
541:
540:
530:
498:
492:
491:
463:
448:
447:
419:
390:
389:
387:
385:
368:
355:
354:
352:
350:
336:
252:, and necrosis.
66:
65:
33:
21:
1318:
1317:
1313:
1312:
1311:
1309:
1308:
1307:
1288:
1287:
1286:
1281:
1253:
1244:Pinealoblastoma
1225:
1197:
1145:
1134:Thyroid adenoma
1079:epithelial-cell
1060:
1018:
1000:Somatostatinoma
954:
949:
919:
914:
913:
878:
864:
859:
858:
849:
847:
841:"Pineoblastoma"
839:
838:
834:
804:
803:
786:
777:
775:
767:
766:
762:
732:
731:
727:
687:
686:
679:
643:
642:
638:
629:
627:
612:
611:
607:
577:
576:
569:
560:
558:
549:
548:
544:
500:
499:
495:
465:
464:
451:
421:
420:
393:
383:
381:
370:
369:
358:
348:
346:
340:"Pineoblastoma"
338:
337:
328:
323:
311:
294:
218:
194:
136:
134:Pathophysiology
127:
109:is a malignant
60:
31:
28:
23:
22:
18:Pinealoblastoma
15:
12:
11:
5:
1316:
1314:
1306:
1305:
1300:
1290:
1289:
1283:
1282:
1280:
1279:
1274:
1269:
1263:
1261:
1255:
1254:
1252:
1251:
1246:
1241:
1235:
1233:
1227:
1226:
1224:
1223:
1218:
1213:
1207:
1205:
1199:
1198:
1196:
1195:
1190:
1189:
1188:
1183:
1173:
1172:
1171:
1166:
1155:
1153:
1147:
1146:
1144:
1143:
1142:
1141:
1136:
1128:
1123:
1118:
1113:
1112:
1111:
1101:
1100:
1099:
1090:
1074:Thyroid cancer
1070:
1068:
1062:
1061:
1059:
1058:
1053:
1048:
1043:
1038:
1028:
1026:
1020:
1019:
1017:
1016:
1011:
1002:
993:
984:
975:
970:
964:
962:
956:
955:
950:
948:
947:
940:
933:
925:
916:
915:
912:
911:
900:
892:
879:
874:
873:
871:
870:Classification
863:
862:External links
860:
857:
856:
845:www.stjude.org
832:
784:
760:
725:
677:
656:(1): 157–165.
636:
605:
586:(2): 235–247.
567:
555:www.cancer.gov
542:
513:(2): 149–157.
493:
449:
391:
356:
325:
324:
322:
319:
310:
307:
293:
290:
217:
214:
193:
190:
179:retinoblastoma
170:genes. DICER1
135:
132:
126:
123:
94:
93:
90:
86:
85:
82:
78:
77:
74:
68:
67:
54:
48:
47:
42:
38:
37:
29:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
1315:
1304:
1301:
1299:
1296:
1295:
1293:
1278:
1275:
1273:
1270:
1268:
1265:
1264:
1262:
1260:
1256:
1250:
1247:
1245:
1242:
1240:
1237:
1236:
1234:
1232:
1228:
1222:
1219:
1217:
1214:
1212:
1209:
1208:
1206:
1204:
1200:
1194:
1193:Paraganglioma
1191:
1187:
1186:Neuroblastoma
1184:
1182:
1179:
1178:
1177:
1174:
1170:
1167:
1165:
1162:
1161:
1160:
1157:
1156:
1154:
1152:
1151:Adrenal tumor
1148:
1140:
1139:Struma ovarii
1137:
1135:
1132:
1131:
1129:
1127:
1124:
1122:
1119:
1117:
1114:
1110:
1107:
1106:
1105:
1102:
1098:
1094:
1091:
1089:
1086:
1085:
1084:
1081:
1080:
1076:(malignant):
1075:
1072:
1071:
1069:
1067:
1063:
1057:
1054:
1052:
1049:
1047:
1044:
1042:
1039:
1037:
1033:
1030:
1029:
1027:
1025:
1021:
1015:
1012:
1010:
1006:
1003:
1001:
997:
994:
992:
988:
985:
983:
979:
976:
974:
971:
969:
966:
965:
963:
961:
957:
953:
946:
941:
939:
934:
932:
927:
926:
923:
910:
906:
905:
901:
898:
897:
893:
890:
889:
885:
881:
880:
877:
872:
868:
861:
846:
842:
836:
833:
828:
824:
820:
816:
812:
808:
801:
799:
797:
795:
793:
791:
789:
785:
774:
770:
764:
761:
756:
752:
748:
744:
740:
736:
729:
726:
721:
717:
712:
707:
703:
699:
695:
691:
684:
682:
678:
673:
669:
664:
659:
655:
651:
647:
640:
637:
626:
622:
618:
617:
609:
606:
601:
597:
593:
589:
585:
581:
574:
572:
568:
556:
552:
546:
543:
538:
534:
529:
524:
520:
516:
512:
508:
504:
497:
494:
489:
485:
481:
477:
473:
469:
462:
460:
458:
456:
454:
450:
445:
441:
437:
433:
429:
425:
418:
416:
414:
412:
410:
408:
406:
404:
402:
400:
398:
396:
392:
380:
379:
374:
367:
365:
363:
361:
357:
345:
341:
335:
333:
331:
327:
320:
318:
315:
308:
306:
304:
300:
291:
289:
287:
283:
280:protein, and
279:
278:neurofilament
275:
274:synaptophysin
271:
270:photoreceptor
267:
263:
259:
255:
251:
245:
242:
239:
235:
231:
222:
215:
213:
209:
207:
203:
200:, leading to
199:
191:
189:
186:
184:
180:
175:
173:
169:
165:
161:
156:
154:
150:
145:
141:
133:
131:
124:
122:
120:
116:
112:
108:
107:Pineoblastoma
100:
91:
87:
83:
79:
75:
73:
69:
64:
58:
55:
53:
49:
46:
45:Pineoblastoma
43:
39:
36:Pineoblastoma
34:
19:
1231:Pineal gland
1097:Hurthle cell
1082:
1077:
1036:Prolactinoma
902:
894:
882:
848:. Retrieved
844:
835:
813:(1): 11–15.
810:
806:
776:. Retrieved
772:
763:
738:
734:
728:
711:10138/223832
693:
689:
653:
649:
639:
628:. Retrieved
616:GeneReviews®
615:
608:
583:
579:
559:. Retrieved
557:. 2018-09-17
554:
545:
510:
506:
496:
474:(1): 85–97.
471:
467:
427:
423:
382:. Retrieved
376:
347:. Retrieved
316:
312:
303:Chemotherapy
295:
254:Homer Wright
246:
243:
227:
210:
195:
187:
176:
157:
140:pineal gland
137:
128:
125:Epidemiology
115:pineal gland
106:
105:
44:
1303:Brain tumor
1249:Pineocytoma
1203:Parathyroid
1056:Pituicytoma
982:Glucagonoma
378:Mayo Clinic
153:metastasize
81:Usual onset
41:Other names
1292:Categories
1116:Anaplastic
1093:Follicular
1009:Gastrinoma
991:Insulinoma
850:2022-10-05
778:2022-10-06
630:2022-10-11
561:2022-10-11
321:References
234:MR imaging
208:syndrome.
206:Parinaud's
1239:Pinealoma
1221:Carcinoma
1109:Medullary
1088:Papillary
1083:carcinoma
1024:Pituitary
430:(9): 69.
309:Prognosis
292:Treatment
216:Diagnosis
149:malignant
144:melatonin
89:Frequency
52:Specialty
1121:Lymphoma
960:Pancreas
899:: 9362/3
827:26571134
755:10561222
720:26374932
625:24761742
600:32389264
537:35371520
488:27889025
444:29995179
384:18 April
262:neuronal
160:MicroRNA
151:and may
72:Symptoms
57:Oncology
1216:Adenoma
1176:Medulla
1130:Benign
1066:Thyroid
909:D010871
891:: C75.3
672:7900586
663:8337708
528:8965073
286:retinal
113:of the
1159:Cortex
1014:VIPoma
825:
753:
718:
670:
660:
623:
598:
535:
525:
486:
442:
349:Mar 8,
268:, and
168:DROSHA
164:DICER1
59:
896:ICD-O
266:glial
111:tumor
904:MeSH
823:PMID
751:PMID
716:PMID
668:PMID
621:PMID
596:PMID
533:PMID
484:PMID
440:PMID
386:2022
351:2023
166:and
138:The
1259:MEN
884:ICD
815:doi
743:doi
706:hdl
698:doi
694:160
658:PMC
588:doi
523:PMC
515:doi
476:doi
432:doi
282:CRX
230:CTs
1294::
1277:2B
1272:2A
1034::
1007::
998::
989::
980::
907::
888:10
843:.
821:.
811:30
809:.
787:^
771:.
749:.
739:17
737:.
714:.
704:.
692:.
680:^
666:.
654:16
652:.
648:.
594:.
584:13
582:.
570:^
553:.
531:.
521:.
509:.
505:.
482:.
472:27
470:.
452:^
438:.
428:20
426:.
394:^
375:.
359:^
342:.
329:^
276:,
264:,
1267:1
1095:/
1005:G
996:δ
987:β
978:α
944:e
937:t
930:v
886:-
876:D
853:.
829:.
817::
781:.
757:.
745::
722:.
708::
700::
674:.
633:.
602:.
590::
564:.
539:.
517::
511:9
490:.
478::
446:.
434::
388:.
353:.
20:)
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
