336:-fetoprotein levels are milder than those found in patients with hepatoblastomas. Infantile hepatic hemangioendothelioma is strongly suggested by the presence of a vascular lesion on imaging studies. A complex, heterogeneous mass is often seen on ultrasonograms; a complex tumor that lacks central enhancement can be seen on CT scans; and the vascular nature of the lesion along with dilation of the aorta proximal to the origin of the celiac artery and a decrease in the diameter distally, indicating significant shunting, is seen on angiograms. Because most hemangioendotheliomas in infants sponanteously involute and regress within the first 12 to 18 months of life, asymptomatic lesions are generally managed conservatively. Infants who have severe anemia and/or thrombocytopenia can be given blood products; for those who have cardiac failure, diuretics and digoxin are often given. To stop further growth and to speed regression of lesions in infants with more significant clinical sequelae, treatment with corticosteroids or interferon-Ξ±-2a is administered. To slow the growth of tumors that are rapidly enlarging, chemotherapy and radiation therapy have been used. Surgical resection, partial hepatectomy, and embolization of afferent vessels should be considered for severe cases.
364:
67:
344:
40:
384:
318:
phases: an initial rapid growth phase, which is followed by a period of spontaneous involution (usually within the first 12 to 18 months of life). Detection of the hemangioendothelioma within the first 6 months of life is attributed to the initial rapid growth during this time; however, the tumor has been detected with fetal ultrasonography. Histopathologically, there are 2 types of hepatic hemangioendotheliomas:
231:(also known as "Infantile kaposiform hemangioendothelioma") is an uncommon vascular tumor, first described by Niedt, Greco, et al. (Hemangioma with Kaposi's sarcoma-like features: report of two cases.(Niedt GW, Greco MA, Wieczorek R, Blanc WA, Knowles DM 2nd. that affects infants and young children, with rare cases having also been reported in adults. Pediatr Pathol. 1989;9(5):567-75.)
411:
have all been used to treat these masses, although studies on survival have yet to be conducted to delineate various treatment regimens. Kaposiform hemangioendothelioma might respond to chemotherapy or antiangiogenic therapies. Recently propanolol and steroids have been shown to be very effective in
205:, and commonly presents in a child or young adult who develops blue nodules of firm consistency on a distal extremity. These tumors were reclassified by Dr. Weiss in 1996 as "spindle cell hemangioma", rather than hemangioendothelioma, due to the excellent prognosis observed in a group of 78 patients.
317:
because of extensive arteriovenous shunting within the lesion. It is the third most common liver tumor in children, the most common benign vascular tumor of the liver in infancy, and the most common symptomatic liver tumor during the first 6 months of life. These hemangioendotheliomas have 2 growth
331:
In children, distinguishing between a primary malignant liver tumor (hepatoblastoma) and a benign primary hepatic lesion (hemangioendothelioma) is crucial. The absence of urinary catecholamines supports the diagnosis of hemangioendothelioma. In patients with hemangioendotheliomas, elevations in
363:
237:, also known as "Dabska tumor", "papillary intralymphatic angioendothelioma" (PILA), "Dabska-type hemangioendothelioma", "hobnail hemangioendothelioma", and "malignant endovascular papillary angioendothelioma", is a rare
343:
1095:
866:
Yamada A, Uematsu K, Yasoshima H, Sakurai K, Hori K, Ohya M, Ohkubo E, Ogasawara H (Feb 1998). "Endovascular papillary angioendothelioma (Dabska tumor) in an elderly woman".
321:
Type I: Hemagioendotheliomas of this type have multiple vascular channels that are formed by an immature endothelial lining with stromal separation from bile ductules.
185:
in 2003. These tumors are so named because their histologic appearance resembles a proliferation of epithelioid cells, with polygonal shape and eosinophilic cytoplasm.
1764:
260:
tumor classification, there is uncertainty as to whether EPA is a distinct entity or a heterogenous group of tumours. The lesion usually presents as a slow-growing
589:
Perkins P, Weiss SW (October 1996). "Spindle cell hemangioendothelioma. An analysis of 78 cases with reassessment of its pathogenesis and biologic behavior".
1153:
1666:
1586:
1201:
383:
793:
Argani P, Athanasian E (Sep 1997). "Malignant endovascular papillary angioendothelioma (Dabska tumor) arising within a deep intramuscular hemangioma".
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Treatment is varied and depends on the site and extent of tumor involvement, sites of metastasis, and specific individual factors. Surgical
1619:
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Type II: These hemangioendotheliomas have an appearance that is more disorganized and hypercellular, and there are no bile ductules.
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1370:
227:
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1601:
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823:
McCarthy EF, Lietman S, Argani P, Frassica FJ (Feb 1999). "Endovascular papillary angioendothelioma (Dabska tumor) of bone".
1606:
1534:
1629:
631:"Strong ERG Positivity in Papillary Intralymphatic Angioendothelioma of the Testis of a 24-Year-Old Male: A Case Report"
1878:
1873:
1791:
1392:
1146:
1139:
909:
Patterson K, Chandra RS (Jul 1985). "Malignant endovascular papillary angioendothelioma. Cutaneous borderline tumor".
544:"Spindle cell hemangioendothelioma. A low-grade angiosarcoma resembling a cavernous hemangioma and Kaposi's sarcoma"
1463:
744:"Dabska tumor (endovascular papillary angioendothelioma) of testis: a case report with brief review of literature"
257:
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1025:
Verma, Saurav; Dhamija, Ekta; Barwad, Adarsh; Kumar, Venkatesan S.; Rastogi, Sameer (30 July 2020).
66:
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1206:
891:
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721:
571:
454:
429:
Billings SD, Folpe AL, Weiss SW (January 2003). "Epithelioid sarcoma-like hemangioendothelioma".
253:
80:
969:
309:
is a rare benign vascular tumour arising from mesenchymal tissue and is usually located in the
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of the head, neck, or extremity, of infants or young children. However, EPA has involved the
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1027:"A case report of Kaposiform haemangioendothelioma; response with propranolol and steroids"
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992:
314:
181:
of intermediate malignancy that was first described by Steven
Billings, Andrew Folpe, and
136:
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100:
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of a kaposiform hemangioendothelioma with "glomeruloid" nodules of endothelial cells.
895:
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182:
96:
696:
Fukunaga M (Oct 1998). "Endovascular papillary angioendothelioma (Dabska tumor)".
1108:
349:
Low power photomicrograph of an endovascular papillary angioendothelioma showing
1752:
1636:
370:
128:
1119:
1043:
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300:
124:
92:
47:
296:
17:
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666:
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103:
that commonly present with an enlarging mass and most commonly involve the
936:
Roos J, Pfiffner R, Stallmach T, Stuckmann G, Marincek B, Willi U (2003).
922:
887:
836:
806:
717:
647:
610:
567:
350:
954:
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typically occurring in adults, although it has been described in infancy.
60:
1087:
215:, first described in 1994, presenting as a slow-growing exophytic mass,
1843:
1710:
144:
1804:
1131:
354:
292:
281:
277:
216:
132:
543:
115:, although many other body sites have been reported, including the
310:
261:
249:
108:
104:
986:
What's Your
Diagnosis? Newborn with abdominal mass and distention
517:
Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007).
288:
265:
211:(also known as a "Hobnail hemangioendothelioma") is a low-grade
140:
120:
116:
1523:
1135:
389:
Characteristic budding, hobnail-like endothelial cells visible.
295:, and has been found in adults. Some reports indicate a good
280:
tissue as a neoplastic transformation of a larger existing
685:
Schwartz, RA. Dabska Tumor. Medscape, updated: Apr 5, 2013
201:) is a vascular tumor that was first described in 1986 by
369:
High power view showing a vascular tumor with cuboidal
91:
They have been described as masses that fall between a
484:
James, William; Berger, Timothy; Elston, Dirk (2005).
1479:
Limb CM + congenital non-progressive limb overgrowth
1077:
1836:
1773:
1557:
1456:
1439:
1413:
1391:
1357:
1294:
1272:
1265:
1256:
1231:
1215:
1178:
1169:
1081:
486:
54:
32:
980:
978:
1765:Intravascular papillary endothelial hyperplasia
1283:Cutaneous and/or mucosal CM ("port-wine" stain)
1015:. Condultant for Pediatricians. 2010;9:168-173.
995:. Consultant for Pediatricians. 2010;9:168-173.
818:
816:
512:
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506:
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1535:
1147:
629:Schultheis AM, Sandmann M, Steurer S (2013).
480:
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476:
474:
472:
470:
468:
8:
1186:Infantile hemangioma / Hemangioma of infancy
624:
622:
620:
168:Hemangioendotheliomas may be classified as:
1381:Familial intraosseous vascular malformation
1004:Banks T, Podraza J. What's Your Diagnosis?
742:Bhatia A, Nada R, Kumar Y, Menon P (2006).
1542:
1528:
1520:
1269:
1262:
1175:
1154:
1140:
1132:
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1006:Newborn with abdominal mass and distention
680:
678:
676:
65:
38:
29:
1052:
1042:
953:
769:
759:
656:
646:
1338:βAcquiredβ progressive lymphatic anomaly
737:
735:
235:Endovascular papillary angioendothelioma
421:
339:
1823:Multifocal lymphangioendotheliomatosis
7:
1620:Proliferating angioendotheliomatosis
1449:Capillary-arteriovenous malformation
313:. It often presents in infancy with
155:Possibly Bartonella spp bacteraemia
880:10.1111/j.1440-1827.1998.tb03887.x
710:10.1111/j.1440-1827.1998.tb03847.x
373:lining the vessels. Few entrapped
25:
1797:Acquired progressive lymphangioma
1502:Bannayan-Riley-Ruvalcaba syndrome
412:kaposiform hemangioendothelioma.
199:Spindle-cell hemangioendothelioma
1244:Epithelioid hemangioendothelioma
1216:Locally aggressive or borderline
938:"Infantile hemangioendothelioma"
603:10.1097/00000478-199610000-00004
560:10.1097/00000478-198608000-00001
443:10.1097/00000478-200301000-00006
382:
362:
342:
174:Epithelioid hemangioendothelioma
1572:Blue rubber bleb nevus syndrome
1487:Macrocephaly - CM (M-CM / MCAP)
1457:Associated with other anomalies
1378:Cerebral cavernous malformation
1371:Blue rubber bleb nevus syndrome
1223:Kaposiform hemangioendothelioma
228:Kaposiform hemangioendothelioma
1321:Generalized lymphatic anomaly
542:Weiss SW, Enzinger FM (1986).
307:Infantile hemangioendothelioma
189:Composite hemangioendothelioma
1:
1446:Lymphatic-venous malformation
1384:Verrucous venous malformation
209:Retiform hemangioendothelioma
1667:Immunosuppression-associated
1630:Infantile hemangiopericytoma
1324:Kaposiform lymphangiomatosis
1300:Incorrect name: Lymphangioma
1296:Lymphatic malformations (LM)
83:of intermediate malignancy.
1792:Lymphangioma circumscriptum
1471:Servelle-Martorell syndrome
1393:Arteriovenous malformations
1367:Familial VM cutaneo-mucosal
256:. Although included in the
252:have been described in the
1895:
1490:Microcephaly - CM (MICCAP)
1464:Klippel-Trenaunay syndrome
1044:10.1186/s13569-020-00134-8
1031:Clinical Sarcoma Research
519:Dermatology: 2-Volume Set
258:World Health Organization
46:
37:
1811:Lymphangioleiomyomatosis
1652:African lymphadenopathic
1551:Tumours of blood vessels
1375:Glomuvenous malformation
488:. (10th ed.). Saunders.
248:. Approximately 30 such
1273:Capillary malformations
1198:Spindle-cell hemangioma
377:are also noted (arrow).
1738:Universal angiomatosis
1704:Targeted hemosiderotic
1587:Endovascular papillary
1258:Vascular malformations
1202:Epithelioid hemangioma
761:10.1186/1746-1596-1-12
113:musculoskeletal system
1475:Sturge-Weber syndrome
1468:Parkes Weber Syndrome
1415:Arteriovenous fistula
1347:Nonne-Milroy syndrome
1190:Congenital hemangioma
837:10.1007/s002560050482
303:is occasionally seen.
77:Hemangioendotheliomas
1577:Hemangioendothelioma
1358:Venous malformations
1331:Gorham-Stout disease
984:Banks T, Podraza J.
955:10.1148/rg.236035024
521:. St. Louis: Mosby.
375:seminiferous tubules
285:cavernous hemangioma
270:subcutaneous tissues
33:Hemangioendothelioma
1307:Common (cystic) LM
911:Arch Pathol Lab Med
795:Arch Pathol Lab Med
648:10.1155/2013/531479
591:Am. J. Surg. Pathol
431:Am. J. Surg. Pathol
1879:Vascular neoplasia
1874:Vascular anomalies
1760:Pyogenic granuloma
1625:Hemangiopericytoma
1342:Primary lymphedema
1207:Pyogenic granuloma
1163:Vascular anomalies
1011:2010-07-02 at the
991:2010-07-02 at the
254:medical literature
246:lymphatic channels
203:Sharon Weiss, M.D.
87:Signs and symptoms
81:vascular neoplasms
1861:
1860:
1828:Lymphangiomatosis
1787:lymphangiosarcoma
1647:African cutaneous
1517:
1516:
1513:
1512:
1483:Maffucci syndrome
1435:
1434:
1252:
1251:
1129:
1128:
528:978-1-4160-2999-1
74:
73:
27:Medical condition
16:(Redirected from
1886:
1674:Hemangioblastoma
1642:Kaposi's sarcoma
1544:
1537:
1530:
1521:
1498:Proteus syndrome
1301:
1270:
1263:
1176:
1156:
1149:
1142:
1133:
1079:
1067:
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1056:
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1016:
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996:
982:
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739:
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682:
671:
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626:
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597:(10): 1196β204.
586:
580:
579:
548:Am J Surg Pathol
539:
533:
532:
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497:
482:
463:
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426:
386:
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79:are a family of
70:
69:
42:
30:
21:
1894:
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1889:
1888:
1887:
1885:
1884:
1883:
1864:
1863:
1862:
1857:
1832:
1769:
1657:AIDS-associated
1567:Hemangiosarcoma
1553:
1548:
1518:
1509:
1494:CLOVES syndrome
1452:
1431:
1409:
1387:
1353:
1335:Channel type LM
1316:Mixed cystic LM
1299:
1298:
1290:
1248:
1227:
1211:
1171:Vascular tumors
1165:
1160:
1130:
1125:
1124:
1090:
1076:
1071:
1070:
1024:
1023:
1019:
1013:Wayback Machine
1003:
999:
993:Wayback Machine
983:
976:
935:
934:
930:
908:
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903:
865:
864:
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825:Skeletal Radiol
822:
821:
814:
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791:
787:
741:
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733:
695:
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683:
674:
635:Case Rep Pathol
628:
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483:
466:
428:
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367:
358:
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335:
315:cardiac failure
191:is a low-grade
177:is an uncommon
166:
161:
153:
137:retroperitoneum
101:vascular tumors
89:
64:
28:
23:
22:
15:
12:
11:
5:
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1856:
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1818:Cystic hygroma
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1508:
1507:
1506:CLAPO syndrome
1504:
1499:
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1488:
1485:
1480:
1477:
1472:
1469:
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1460:
1458:
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1350:
1349:
1339:
1336:
1333:
1327:
1326:
1325:
1319:
1318:
1317:
1314:
1313:Microcystic LM
1311:
1310:Macrocystic LM
1304:
1302:
1292:
1291:
1289:
1288:
1285:
1280:
1276:
1274:
1267:
1260:
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1226:
1225:
1219:
1217:
1213:
1212:
1210:
1209:
1204:
1199:
1196:
1194:Tufted angioma
1191:
1188:
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1173:
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1144:
1136:
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1111:
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1083:
1082:Classification
1075:
1074:External links
1072:
1069:
1068:
1017:
997:
974:
948:(6): 1649β55.
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901:
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688:
672:
616:
581:
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498:
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164:Classification
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1743:Angiokeratoma
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1500:
1497:
1495:
1492:
1489:
1486:
1484:
1481:
1478:
1476:
1473:
1470:
1467:
1465:
1462:
1461:
1459:
1455:
1448:
1445:
1444:
1442:
1438:
1427:
1424:
1421:
1420:
1418:
1416:
1412:
1405:
1402:
1399:
1398:
1396:
1394:
1390:
1383:
1380:
1377:
1374:
1372:
1369:
1366:
1363:
1362:
1360:
1356:
1348:
1345:
1344:
1343:
1340:
1337:
1334:
1332:
1328:
1323:
1322:
1320:
1315:
1312:
1309:
1308:
1306:
1305:
1303:
1297:
1293:
1287:Reticulate CM
1286:
1284:
1281:
1279:Nevus simplex
1278:
1277:
1275:
1271:
1268:
1264:
1261:
1259:
1255:
1245:
1242:
1240:
1237:
1236:
1234:
1230:
1224:
1221:
1220:
1218:
1214:
1208:
1205:
1203:
1200:
1197:
1195:
1192:
1189:
1187:
1184:
1183:
1181:
1177:
1174:
1172:
1168:
1164:
1157:
1152:
1150:
1145:
1143:
1138:
1137:
1134:
1121:
1117:
1116:
1112:
1110:
1106:
1105:
1101:
1098:
1097:
1093:
1092:
1089:
1084:
1080:
1073:
1064:
1060:
1055:
1050:
1045:
1040:
1036:
1032:
1028:
1021:
1018:
1014:
1010:
1007:
1001:
998:
994:
990:
987:
981:
979:
975:
972:
971:
965:
961:
956:
951:
947:
943:
942:Radiographics
939:
932:
929:
924:
920:
916:
912:
905:
902:
897:
893:
889:
885:
881:
877:
873:
869:
862:
859:
854:
850:
846:
842:
838:
834:
830:
826:
819:
817:
813:
808:
804:
800:
796:
789:
786:
781:
777:
772:
767:
762:
757:
753:
749:
745:
738:
736:
732:
727:
723:
719:
715:
711:
707:
704:(10): 840β1.
703:
699:
692:
689:
686:
681:
679:
677:
673:
668:
664:
659:
654:
649:
644:
640:
636:
632:
625:
623:
621:
617:
612:
608:
604:
600:
596:
592:
585:
582:
577:
573:
569:
565:
561:
557:
554:(8): 521β30.
553:
549:
545:
538:
535:
530:
524:
520:
513:
511:
509:
507:
505:
503:
499:
495:
494:0-7216-2921-0
491:
487:
481:
479:
477:
475:
473:
471:
469:
465:
460:
456:
452:
448:
444:
440:
436:
432:
425:
422:
415:
413:
410:
406:
402:
394:
385:
380:
376:
372:
365:
360:
356:
352:
345:
340:
330:
329:
323:
320:
319:
316:
312:
308:
305:
302:
298:
294:
290:
286:
283:
279:
275:
271:
267:
263:
259:
255:
251:
247:
243:
240:
236:
233:
230:
229:
225:
222:
218:
214:
210:
207:
204:
200:
197:
194:
190:
187:
184:
180:
176:
175:
171:
170:
169:
163:
158:
156:
150:
148:
146:
142:
138:
134:
130:
126:
122:
118:
114:
110:
106:
102:
98:
94:
86:
84:
82:
78:
68:
62:
59:
57:
53:
49:
45:
41:
36:
31:
19:
1853:Angiofibroma
1848:angiosarcoma
1803:
1783:Lymphangioma
1699:Microvenular
1613:Spindle cell
1576:
1559:Blood vessel
1239:Angiosarcoma
1113:
1102:
1094:
1034:
1030:
1020:
1000:
968:
945:
941:
931:
917:(7): 671β3.
914:
910:
904:
874:(2): 164β7.
871:
867:
861:
831:(2): 100β3.
828:
824:
801:(9): 992β5.
798:
794:
788:
751:
748:Diagn Pathol
747:
701:
697:
691:
638:
634:
594:
590:
584:
551:
547:
537:
518:
485:
437:(1): 48β57.
434:
430:
424:
409:chemotherapy
405:radiotherapy
398:
306:
242:angiosarcoma
234:
226:
221:subcutaneous
213:angiosarcoma
208:
198:
193:angiosarcoma
188:
183:Sharon Weiss
172:
167:
154:
99:. They are
97:angiosarcoma
90:
76:
75:
18:Dabska tumor
1753:Angiolipoma
1721:Seriginosum
1694:Glomeruloid
1637:Venous lake
1592:Epithelioid
1099:: 9130-9133
868:Pathol. Int
698:Pathol. Int
371:endothelium
219:plaque, or
129:lymph nodes
1868:Categories
1748:of Mibelli
1679:Hemangioma
1597:Kaposiform
1115:DiseasesDB
641:: 531479.
416:References
355:hyalinized
301:metastasis
125:intestines
93:hemangioma
48:Micrograph
1775:Lymphatic
1689:Cavernous
1684:Capillary
1602:Infantile
1582:Composite
1428:In CM-AVM
1406:In CM-AVM
1364:Common VM
1232:Malignant
1037:(1): 12.
970:Full text
401:resection
395:Treatment
297:prognosis
239:low-grade
159:Diagnosis
56:Specialty
1607:Retiform
1440:Combined
1422:Sporadic
1400:Sporadic
1063:32765826
1009:Archived
989:Archived
964:14615570
896:24778725
853:23809159
845:10197456
780:16859564
726:38650082
667:23607024
576:31168575
459:28749740
451:12502927
351:papillae
274:testicle
61:Oncology
1844:Angioma
1711:Angioma
1662:Classic
1109:D006390
1054:7394668
923:3839367
888:9589482
807:9302935
771:1555613
718:9788271
658:3626176
611:8827025
568:3740350
276:, deep
264:of the
223:nodule.
145:stomach
1837:Either
1805:PEComa
1731:Tufted
1726:Spider
1716:Cherry
1425:In HHT
1403:In HHT
1329:LM in
1266:Simple
1179:Benign
1061:
1051:
962:
921:
894:
886:
851:
843:
805:
778:
768:
754:: 12.
724:
716:
665:
655:
609:
574:
566:
525:
492:
457:
449:
407:, and
293:spleen
282:benign
278:muscle
250:tumors
217:dermal
133:pleura
111:, and
63:
1120:33472
1096:ICD-O
892:S2CID
849:S2CID
722:S2CID
572:S2CID
455:S2CID
357:cores
353:with
311:liver
262:tumor
151:Cause
109:liver
105:lungs
1104:MeSH
1059:PMID
960:PMID
919:PMID
884:PMID
841:PMID
803:PMID
776:PMID
714:PMID
663:PMID
639:2013
607:PMID
564:PMID
523:ISBN
490:ISBN
447:PMID
299:but
291:and
289:bone
268:and
266:skin
141:heel
121:neck
119:and
117:head
95:and
1049:PMC
1039:doi
950:doi
915:109
876:doi
833:doi
799:121
766:PMC
756:doi
706:doi
653:PMC
643:doi
599:doi
556:doi
439:doi
244:of
1870::
1118::
1107::
1057:.
1047:.
1035:10
1033:.
1029:.
977:^
958:.
946:23
944:.
940:.
913:.
890:.
882:.
872:48
870:.
847:.
839:.
829:28
827:.
815:^
797:.
774:.
764:.
750:.
746:.
734:^
720:.
712:.
702:48
700:.
675:^
661:.
651:.
637:.
633:.
619:^
605:.
595:20
593:.
570:.
562:.
552:10
550:.
546:.
501:^
467:^
453:.
445:.
435:27
433:.
403:,
287:,
147:.
143:,
139:,
135:,
131:,
127:,
123:,
107:,
1846:/
1785:/
1609:)
1543:e
1536:t
1529:v
1155:e
1148:t
1141:v
1088:D
1065:.
1041::
966:.
952::
925:.
898:.
878::
855:.
835::
809:.
782:.
758::
752:1
728:.
708::
669:.
645::
613:.
601::
578:.
558::
531:.
496:.
461:.
441::
334:1
332:Ξ±
20:)
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