immature ovarian teratoma

4
Immature ovarian teratoma Factfile immature teratoma of the ovary rare variant of ovarian germ cell tumor < 3% of all teratomas, < 1% of all ovarian malignacies ~20% of all germ cell tumors ~10% of the cases contralateral benign (cystic) teratoma 90% of the patients < 30 years of age, mean age 19 yrs Serum markers might be elevated (esp. AFP and HCG) 6 – 35 cm in diameter (average 18cm) CF: predominantly solid, fleshy, sometimes cystic Tasks for the handling and reporting of immature ovarian teratomas Exclusion of benign solid teratoma Determining the grade of the tumor Grading based on the extent of immature tissue within the teratoma In the past tailored on immature neuroepithelium (because of this tissue is most recogniseable and increases the reproducibility) 3-tiered grading system Grading in immature teratoma (Gonzales-Crussi) Grade 1 Occasional foci of immature tissue: <1 one low power field (x40) in a given slide no adjuvant chemotherapy required Grade 2 1-4 low power fields per slide adjuvant chemotherapy required Grade 3 > 4 low power fields in a given slide adjuvant chemotherapy required Tumor grade is the crucial feature for determining the prognosis and the necessity of adjuvant treatment sometimes a two tired grading system (low versus high grade) was suggested (O`Connor & Norris 1994)

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Page 1: Immature Ovarian Teratoma

Immature ovarian teratoma Factfile immature teratoma of the ovary

bull rare variant of ovarian germ cell tumor ndash lt 3 of all teratomas ndash lt 1 of all ovarian malignacies ndash ~20 of all germ cell tumors

bull ~10 of the cases contralateral benign (cystic) teratoma bull 90 of the patients lt 30 years of age mean age 19 yrs bull Serum markers might be elevated (esp AFP and HCG) bull 6 ndash 35 cm in diameter (average 18cm) bull CF predominantly solid fleshy sometimes cystic

Tasks for the handling and reporting of immature ovarian teratomas

bull Exclusion of benign solid teratoma

bull Determining the grade of the tumor ndash Grading based on the extent of immature tissue within the

teratoma ndash In the past tailored on immature neuroepithelium (because of

this tissue is most recogniseable and increases the reproducibility)

ndash 3-tiered grading system Grading in immature teratoma (Gonzales-Crussi)

bull Grade 1 ndash Occasional foci of immature tissue lt1 one low power field

(x40) in a given slide no adjuvant chemotherapy required bull Grade 2

ndash 1-4 low power fields per slide adjuvant chemotherapy required

bull Grade 3 ndash gt 4 low power fields in a given slide adjuvant

chemotherapy required Tumor grade is the crucial feature for determining the prognosis

and the necessity of adjuvant treatment sometimes a two tired grading system (low versus high grade)

was suggested (O`Connor amp Norris 1994)

some suthors suggest that surgical resection alone might be curative reserving poylchemotherapy (bleomycin etoposide and cisplatin ie BEP scheme) for cases with relapse (Cushing et al 1999)

to ensure adequate diagnosis and adequate grading of the lesion adequate sampling is required one block per 1-2cm of largest tumor dimension Immature Tissue within Teratoma

bull Immature ectodermal tissue ndash Mainly neuroectodermal rosettes and tubules

bull Immature mesenchyme ndash Loose myxoid stroma with focal differentiation into ndash Immature cartilage ndash Immature fat ndash Osteoid rhabdomyoblasts

bull Immature endodermal tissue ndash Hepatic tissue ndash Intestinal type epithel with basal vacuolisation ndash Embryonic renal tissue with Wilms tumor

it has been proposed that high-grade immature teratomas are characterised by the presence of immature neuro-epithelial structures and low-grade tumors by the presence of somite organogenesis (Cho et al 2005)

theoretically any of the immature elements might be quantitated for grading purpose but immature neuroepithelium is the easiest to assess (Norris et al 1976)

Differential diagnoses of immature ovarian teratomas

bull mature teratoma with foci of immature neuroepithel tissue bull occasionally mature teratomas might contain minute foci of

immature tissue (ie lt21mmsup2) bull a study of 360 ovarian teratomas that contain immature

tissue included 350 immature teratomas and only ten mature cystic teratomas with microfoci of immature tissue

bull during a follow up of 11 months to 7 years none of the latter cases recurred

bull this study suggests that the dx of immature teratoma is not appropriate when only minute foci of immature tissue is

present in an otherwise mature cystic teratoma (Yanai-Inbar amp Scully 1987))

bull but adequate sampling is required

bull monodermal teratoma with malignant somatic type tumros teratoma exclusively or predominantly composed of a single type tissue derived from 1 embryonal layer and adult type malignant tumor

bull malignant cell type in malignant transformation is directly related to the predominant mature cell type

bull eg struma ovarii can develop into a papillary thyroid carcinoma in lt5

bull in bi- and triphasic mature teratoma squamous cell carcinoma is the most common type of malignancy (05-2 and typically occurs in post-menopausal women (Hackethal et al 2008)

bull treatment of malignant transformation of mature teratoma is based on the cell type of the tumor

bull MMMT admixture of primitive appearing tissues without

organisation into coordinated tissue units with recognisable embryonalfetal structures

Schedule for the differential diagnoses of immature ovarian teratomas

WHO 2003 IJGP 19876203

Germ cell tumors with teratomatous features

Monodermal T withmalignant somatic

type tumors

Dermoid cyst withfoci of neoroepith

tissue (lt21 mmsup2) benign

Mature T with fociof immaturity

Immature Teratoma

Ependymoma benign

PNET maligne

Glioblastoma maligne

Adequate sampling is required

Grading important

References Cho NH Kim YT Lee JH Song C Cho SW Cho SH Chi JG Diagnostic challenge of fetal ontogeny and its application on the ovarian teratomasInt J Gynecol Pathol 2005 Apr24(2)173-82

Cushing B Giller R Ablin A Cohen L Cullen J Hawkins E Heifetz SA Krailo M Lauer SJ Marina N Rao PV Rescorla F Vinocur CD Weetman RM Castleberry RP Surgical resection alone is effective treatment for ovarian immature teratoma in children and adolescents a report of the pediatric oncology group and the childrens cancer group Am J Obstet Gynecol 1999 Aug181(2)353-8

Deodhar KK Suryawanshi P Shah M Rekhi B Chinoy RF Immature teratoma of the ovary a clinicopathological study of 28 cases Indian J Pathol Microbiol 2011 Oct-Dec54(4)730-5

Hackethal A Brueggmann D Bohlmann MK Franke FE Tinneberg HR Muumlnstedt K Squamous-cell carcinoma in mature cystic teratoma of the ovary systematic review and analysis of published data Lancet Oncol 2008 Dec9(12)1173-80

Heifetz SA Cushing B Giller R Shuster JJ Stolar CJ Vinocur CD Hawkins EP Immature teratomas in children pathologic considerations a report from the combined Pediatric Oncology GroupChildrens Cancer Group Am J Surg Pathol 1998 Sep22(9)1115-24

McCluggage WG Ovarian neoplasms composed of small round cells a review Adv Anat Pathol 2004 Nov11(6)288-96

Norris HJ Zirkin HJ Benson WL Immature (malignant) teratoma of the ovary a clinical and pathologic study of 58 cases Cancer 1976 May37(5)2359-72

OConnor DM Norris HJ The influence of grade on the outcome of stage I ovarian immature (malignant) teratomas and the reproducibility of grading Int J Gynecol Pathol 1994 Oct13(4)283-9

Rezk Y Sheinfeld J Chi DS Prolonged survival following salvage surgery for chemorefractory ovarian immature teratoma a case report and review of the literature Gynecol Oncol 2005 Mar96(3)883-7

Saba L Guerriero S Sulcis R Virgilio B Melis G Mallarini G Mature and immature ovarian teratomas CT US and MR imaging characteristics Eur J Radiol 2009 Dec72(3)454-63

Vicus D Beiner ME Clarke B Klachook S Le LW Laframboise S Mackay H Ovarian immature teratoma treatment and outcome in a single institutional cohort Gynecol Oncol 2011 Oct123(1)50-3

Yanai-Inbar I Scully RE Relation of ovarian dermoid cysts and immature teratomas an analysis of 350 cases of immature teratoma and 10 cases of dermoid cyst with microscopic foci of immature tissue Int J Gynecol Pathol 19876(3)203-12

Page 2: Immature Ovarian Teratoma

some suthors suggest that surgical resection alone might be curative reserving poylchemotherapy (bleomycin etoposide and cisplatin ie BEP scheme) for cases with relapse (Cushing et al 1999)

to ensure adequate diagnosis and adequate grading of the lesion adequate sampling is required one block per 1-2cm of largest tumor dimension Immature Tissue within Teratoma

bull Immature ectodermal tissue ndash Mainly neuroectodermal rosettes and tubules

bull Immature mesenchyme ndash Loose myxoid stroma with focal differentiation into ndash Immature cartilage ndash Immature fat ndash Osteoid rhabdomyoblasts

bull Immature endodermal tissue ndash Hepatic tissue ndash Intestinal type epithel with basal vacuolisation ndash Embryonic renal tissue with Wilms tumor

it has been proposed that high-grade immature teratomas are characterised by the presence of immature neuro-epithelial structures and low-grade tumors by the presence of somite organogenesis (Cho et al 2005)

theoretically any of the immature elements might be quantitated for grading purpose but immature neuroepithelium is the easiest to assess (Norris et al 1976)

Differential diagnoses of immature ovarian teratomas

bull mature teratoma with foci of immature neuroepithel tissue bull occasionally mature teratomas might contain minute foci of

immature tissue (ie lt21mmsup2) bull a study of 360 ovarian teratomas that contain immature

tissue included 350 immature teratomas and only ten mature cystic teratomas with microfoci of immature tissue

bull during a follow up of 11 months to 7 years none of the latter cases recurred

bull this study suggests that the dx of immature teratoma is not appropriate when only minute foci of immature tissue is

present in an otherwise mature cystic teratoma (Yanai-Inbar amp Scully 1987))

bull but adequate sampling is required

bull monodermal teratoma with malignant somatic type tumros teratoma exclusively or predominantly composed of a single type tissue derived from 1 embryonal layer and adult type malignant tumor

bull malignant cell type in malignant transformation is directly related to the predominant mature cell type

bull eg struma ovarii can develop into a papillary thyroid carcinoma in lt5

bull in bi- and triphasic mature teratoma squamous cell carcinoma is the most common type of malignancy (05-2 and typically occurs in post-menopausal women (Hackethal et al 2008)

bull treatment of malignant transformation of mature teratoma is based on the cell type of the tumor

bull MMMT admixture of primitive appearing tissues without

organisation into coordinated tissue units with recognisable embryonalfetal structures

Schedule for the differential diagnoses of immature ovarian teratomas

WHO 2003 IJGP 19876203

Germ cell tumors with teratomatous features

Monodermal T withmalignant somatic

type tumors

Dermoid cyst withfoci of neoroepith

tissue (lt21 mmsup2) benign

Mature T with fociof immaturity

Immature Teratoma

Ependymoma benign

PNET maligne

Glioblastoma maligne

Adequate sampling is required

Grading important

References Cho NH Kim YT Lee JH Song C Cho SW Cho SH Chi JG Diagnostic challenge of fetal ontogeny and its application on the ovarian teratomasInt J Gynecol Pathol 2005 Apr24(2)173-82

Cushing B Giller R Ablin A Cohen L Cullen J Hawkins E Heifetz SA Krailo M Lauer SJ Marina N Rao PV Rescorla F Vinocur CD Weetman RM Castleberry RP Surgical resection alone is effective treatment for ovarian immature teratoma in children and adolescents a report of the pediatric oncology group and the childrens cancer group Am J Obstet Gynecol 1999 Aug181(2)353-8

Deodhar KK Suryawanshi P Shah M Rekhi B Chinoy RF Immature teratoma of the ovary a clinicopathological study of 28 cases Indian J Pathol Microbiol 2011 Oct-Dec54(4)730-5

Hackethal A Brueggmann D Bohlmann MK Franke FE Tinneberg HR Muumlnstedt K Squamous-cell carcinoma in mature cystic teratoma of the ovary systematic review and analysis of published data Lancet Oncol 2008 Dec9(12)1173-80

Heifetz SA Cushing B Giller R Shuster JJ Stolar CJ Vinocur CD Hawkins EP Immature teratomas in children pathologic considerations a report from the combined Pediatric Oncology GroupChildrens Cancer Group Am J Surg Pathol 1998 Sep22(9)1115-24

McCluggage WG Ovarian neoplasms composed of small round cells a review Adv Anat Pathol 2004 Nov11(6)288-96

Norris HJ Zirkin HJ Benson WL Immature (malignant) teratoma of the ovary a clinical and pathologic study of 58 cases Cancer 1976 May37(5)2359-72

OConnor DM Norris HJ The influence of grade on the outcome of stage I ovarian immature (malignant) teratomas and the reproducibility of grading Int J Gynecol Pathol 1994 Oct13(4)283-9

Rezk Y Sheinfeld J Chi DS Prolonged survival following salvage surgery for chemorefractory ovarian immature teratoma a case report and review of the literature Gynecol Oncol 2005 Mar96(3)883-7

Saba L Guerriero S Sulcis R Virgilio B Melis G Mallarini G Mature and immature ovarian teratomas CT US and MR imaging characteristics Eur J Radiol 2009 Dec72(3)454-63

Vicus D Beiner ME Clarke B Klachook S Le LW Laframboise S Mackay H Ovarian immature teratoma treatment and outcome in a single institutional cohort Gynecol Oncol 2011 Oct123(1)50-3

Yanai-Inbar I Scully RE Relation of ovarian dermoid cysts and immature teratomas an analysis of 350 cases of immature teratoma and 10 cases of dermoid cyst with microscopic foci of immature tissue Int J Gynecol Pathol 19876(3)203-12

Page 3: Immature Ovarian Teratoma

present in an otherwise mature cystic teratoma (Yanai-Inbar amp Scully 1987))

bull but adequate sampling is required

bull monodermal teratoma with malignant somatic type tumros teratoma exclusively or predominantly composed of a single type tissue derived from 1 embryonal layer and adult type malignant tumor

bull malignant cell type in malignant transformation is directly related to the predominant mature cell type

bull eg struma ovarii can develop into a papillary thyroid carcinoma in lt5

bull in bi- and triphasic mature teratoma squamous cell carcinoma is the most common type of malignancy (05-2 and typically occurs in post-menopausal women (Hackethal et al 2008)

bull treatment of malignant transformation of mature teratoma is based on the cell type of the tumor

bull MMMT admixture of primitive appearing tissues without

organisation into coordinated tissue units with recognisable embryonalfetal structures

Schedule for the differential diagnoses of immature ovarian teratomas

WHO 2003 IJGP 19876203

Germ cell tumors with teratomatous features

Monodermal T withmalignant somatic

type tumors

Dermoid cyst withfoci of neoroepith

tissue (lt21 mmsup2) benign

Mature T with fociof immaturity

Immature Teratoma

Ependymoma benign

PNET maligne

Glioblastoma maligne

Adequate sampling is required

Grading important

References Cho NH Kim YT Lee JH Song C Cho SW Cho SH Chi JG Diagnostic challenge of fetal ontogeny and its application on the ovarian teratomasInt J Gynecol Pathol 2005 Apr24(2)173-82

Cushing B Giller R Ablin A Cohen L Cullen J Hawkins E Heifetz SA Krailo M Lauer SJ Marina N Rao PV Rescorla F Vinocur CD Weetman RM Castleberry RP Surgical resection alone is effective treatment for ovarian immature teratoma in children and adolescents a report of the pediatric oncology group and the childrens cancer group Am J Obstet Gynecol 1999 Aug181(2)353-8

Deodhar KK Suryawanshi P Shah M Rekhi B Chinoy RF Immature teratoma of the ovary a clinicopathological study of 28 cases Indian J Pathol Microbiol 2011 Oct-Dec54(4)730-5

Hackethal A Brueggmann D Bohlmann MK Franke FE Tinneberg HR Muumlnstedt K Squamous-cell carcinoma in mature cystic teratoma of the ovary systematic review and analysis of published data Lancet Oncol 2008 Dec9(12)1173-80

Heifetz SA Cushing B Giller R Shuster JJ Stolar CJ Vinocur CD Hawkins EP Immature teratomas in children pathologic considerations a report from the combined Pediatric Oncology GroupChildrens Cancer Group Am J Surg Pathol 1998 Sep22(9)1115-24

McCluggage WG Ovarian neoplasms composed of small round cells a review Adv Anat Pathol 2004 Nov11(6)288-96

Norris HJ Zirkin HJ Benson WL Immature (malignant) teratoma of the ovary a clinical and pathologic study of 58 cases Cancer 1976 May37(5)2359-72

OConnor DM Norris HJ The influence of grade on the outcome of stage I ovarian immature (malignant) teratomas and the reproducibility of grading Int J Gynecol Pathol 1994 Oct13(4)283-9

Rezk Y Sheinfeld J Chi DS Prolonged survival following salvage surgery for chemorefractory ovarian immature teratoma a case report and review of the literature Gynecol Oncol 2005 Mar96(3)883-7

Saba L Guerriero S Sulcis R Virgilio B Melis G Mallarini G Mature and immature ovarian teratomas CT US and MR imaging characteristics Eur J Radiol 2009 Dec72(3)454-63

Vicus D Beiner ME Clarke B Klachook S Le LW Laframboise S Mackay H Ovarian immature teratoma treatment and outcome in a single institutional cohort Gynecol Oncol 2011 Oct123(1)50-3

Yanai-Inbar I Scully RE Relation of ovarian dermoid cysts and immature teratomas an analysis of 350 cases of immature teratoma and 10 cases of dermoid cyst with microscopic foci of immature tissue Int J Gynecol Pathol 19876(3)203-12

Page 4: Immature Ovarian Teratoma

References Cho NH Kim YT Lee JH Song C Cho SW Cho SH Chi JG Diagnostic challenge of fetal ontogeny and its application on the ovarian teratomasInt J Gynecol Pathol 2005 Apr24(2)173-82

Cushing B Giller R Ablin A Cohen L Cullen J Hawkins E Heifetz SA Krailo M Lauer SJ Marina N Rao PV Rescorla F Vinocur CD Weetman RM Castleberry RP Surgical resection alone is effective treatment for ovarian immature teratoma in children and adolescents a report of the pediatric oncology group and the childrens cancer group Am J Obstet Gynecol 1999 Aug181(2)353-8

Deodhar KK Suryawanshi P Shah M Rekhi B Chinoy RF Immature teratoma of the ovary a clinicopathological study of 28 cases Indian J Pathol Microbiol 2011 Oct-Dec54(4)730-5

Hackethal A Brueggmann D Bohlmann MK Franke FE Tinneberg HR Muumlnstedt K Squamous-cell carcinoma in mature cystic teratoma of the ovary systematic review and analysis of published data Lancet Oncol 2008 Dec9(12)1173-80

Heifetz SA Cushing B Giller R Shuster JJ Stolar CJ Vinocur CD Hawkins EP Immature teratomas in children pathologic considerations a report from the combined Pediatric Oncology GroupChildrens Cancer Group Am J Surg Pathol 1998 Sep22(9)1115-24

McCluggage WG Ovarian neoplasms composed of small round cells a review Adv Anat Pathol 2004 Nov11(6)288-96

Norris HJ Zirkin HJ Benson WL Immature (malignant) teratoma of the ovary a clinical and pathologic study of 58 cases Cancer 1976 May37(5)2359-72

OConnor DM Norris HJ The influence of grade on the outcome of stage I ovarian immature (malignant) teratomas and the reproducibility of grading Int J Gynecol Pathol 1994 Oct13(4)283-9

Rezk Y Sheinfeld J Chi DS Prolonged survival following salvage surgery for chemorefractory ovarian immature teratoma a case report and review of the literature Gynecol Oncol 2005 Mar96(3)883-7

Saba L Guerriero S Sulcis R Virgilio B Melis G Mallarini G Mature and immature ovarian teratomas CT US and MR imaging characteristics Eur J Radiol 2009 Dec72(3)454-63

Vicus D Beiner ME Clarke B Klachook S Le LW Laframboise S Mackay H Ovarian immature teratoma treatment and outcome in a single institutional cohort Gynecol Oncol 2011 Oct123(1)50-3

Yanai-Inbar I Scully RE Relation of ovarian dermoid cysts and immature teratomas an analysis of 350 cases of immature teratoma and 10 cases of dermoid cyst with microscopic foci of immature tissue Int J Gynecol Pathol 19876(3)203-12