Teratoma pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Pathology classification of individual teratomas
Teratomas commonly are classified using the Gonzalez-Crussi grading system: 0 or mature (benign); 1 or immature, probably benign; 2 or immature, possibly malignant (cancerous); and 3 or frankly malignant. See also cancer staging. Teratomas are also classified by their content: a solid teratoma contains only tissues (perhaps including more complex structures); a cystic teratoma contain only pockets of fluid or semi-fluid such as cerebrospinal fluid, sebum, or fat; a mixed teratoma contains both solid and cystic parts. Cystic teratomas usually are grade 0 and, conversely, grade 0 teratomas usually are cystic.
Grade 0, 1 and 2 pure teratomas have the potential to become malignant (grade 3), and malignant pure teratomas have the potential to metastasize. These rare forms of teratoma with malignant transformation may contain elements of somatic (non germ cell) malignancy such as leukemia, carcinoma or sarcoma.[1] A teratoma may contain elements of other germ cell tumors, in which case it is not a pure teratoma but rather is a mixed germ cell tumor and is malignant. In infants and young children, these elements usually are endodermal sinus tumor, followed by choriocarcinoma. Finally, a teratoma can be pure and not malignant yet highly aggressive: this is exemplified by growing teratoma syndrome, in which chemotherapy eliminates the malignant elements of a mixed tumor, leaving pure teratoma which paradoxically begins to grow very rapidly.
"Benign" teratoma may prove to be malignant
A "benign" grade 0 (mature) teratoma nonetheless has a non-zero risk of malignancy. Recurrence with malignant endodermal sinus tumor has been reported in cases of formerly benign mature teratoma,[2] even in fetiform teratoma and fetus in fetu.[3][4] A grade 1 immature teratoma that appears to be benign (e.g., because AFP is not elevated) has a much higher risk of malignancy, and requires adequate follow-up.[5][6][7][8]
Teratoma with malignant transformation
A teratoma with malignant transformation or TMT is a very rare form of teratoma that may contain elements of somatic (non germ cell) malignant tumors such as leukemia, carcinoma or sarcoma.[1] Of 641 children with pure teratoma, 9 developed TMT[9]: 5 carcinoma, 2 glioma, and 2 embryonal (here, these last are classified among germ cell tumors).
Extraspinal ependymoma
Extraspinal ependymoma, usually considered to be a glioma (a type of non-germ cell tumor), may be an unusual form of mature teratoma.[10]
References
- ↑ 1.0 1.1 Harms D, Zahn S, Göbel U, Schneider DT (2006). "Pathology and molecular biology of teratomas in childhood and adolescence". Klinische Pädiatrie. 218 (6): 296–302. doi:10.1055/s-2006-942271. PMID 17080330.
- ↑ Ohno Y, Kanematsu T (1998). "An endodermal sinus tumor arising from a mature cystic teratoma in the retroperitoneum in a child: is a mature teratoma a premalignant condition?". Hum. Pathol. 29 (10): 1167–9. PMID 9781660.
- ↑ Chen YH, Chang CH, Chen KC, Diau GY, Loh IW, Chu CC (2007). "Malignant transformation of a well-organized sacrococcygeal fetiform teratoma in a newborn male". J. Formos. Med. Assoc. 106 (5): 400–2. PMID 17561476. (publisher offers free full text PDF to registered users)
- ↑ Hopkins KL, Dickson PK, Ball TI, Ricketts RR, O'Shea PA, Abramowsky CR (1997). "Fetus-in-fetu with malignant recurrence". J. Pediatr. Surg. 32 (10): 1476–9. PMID 9349774.
- ↑ Muscatello L, Giudice M, Feltri M (2005). "Malignant cervical teratoma: report of a case in a newborn". European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery. 262 (11): 899–904. doi:10.1007/s00405-005-0917-2. PMID 15895292.
- ↑ Ukiyama E, Endo M, Yoshida F, Tezuka T, Kudo K, Sato S, Akatsuka S, Hata J (2005). "Recurrent yolk sac tumor following resection of a neonatal immature gastric teratoma". Pediatr. Surg. Int. 21 (7): 585–8. doi:10.1007/s00383-005-1404-y. PMID 15928937.
- ↑ Bilik R, Shandling B, Pope M, Thorner P, Weitzman S, Ein SH (1993). "Malignant benign neonatal sacrococcygeal teratoma". J. Pediatr. Surg. 28 (9): 1158–60. PMID 7508500.
- ↑ Hawkins E, Issacs H, Cushing B, Rogers P (1993). "Occult malignancy in neonatal sacrococcygeal teratomas. A report from a Combined Pediatric Oncology Group and Children's Cancer Group study". The American journal of pediatric hematology/oncology. 15 (4): 406–9. PMID 7692755.
- ↑ Biskup W, Calaminus G, Schneider DT, Leuschner I, Göbel U (2006). "Teratoma with malignant transformation: experiences of the cooperative GPOH protocols MAKEI 83/86/89/96". Klinische Pädiatrie. 218 (6): 303–8. doi:10.1055/s-2006-942272. PMID 17080331.
- ↑ Aktuğ T, Hakgüder G, Sarioğlu S, Akgür FM, Olguner M, Pabuçcuoğlu U. (2000) Sacrococcygeal extraspinal ependymomas: the role of coccygectomy. J Pediatr Surg. 35(3):515-518. PubMed