Terminology Estimate the risk of malignancy in adnexal masses - Overview

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1 Understanding the IOTA (International Ovarian Tumor Analysis) terminology & Classification Using the IOTA simple rules to estimate the risk of malignancy in women with adnexal masses Elisabeth Epstein, Associate Professor Southern University Hospital, Karolinska Insitute Stockholm, Sweden Estimate the risk of malignancy in adnexal masses - Overview IOTA terminology & classification Predicting risk of malignancy using Pattern recognition Simple rules Case examples Type of lesion Terminology Classification according to Granberg -89 Risk of maligancy, IOTA trial n=1066, 2005 Cyst contence unilocular multiocular unilocularsolid multilocularsolid solid Unilocular cyst Multilocular cyst Unilocular-solid cyst 0.3% 10% 33% Sonolucent hemorragic mixed ground glass Low level Defintions Multilocular-solid cyst Solid 40% 62% Papillary projection Incomplete septa Aucoustic shadowing Assessment of vascularization Unilocular cyst no septa or solid components Unilocular solid with solid component > 3mm Pyosalpinx with cogwheel papilations >3mm Irregularities < 3mm in height, should not be regarded as papillary projection Incomplete septae Should not regarded as real septae White ball should not be classified as solid component Sludge/amorphous material should not be regarded as papillary projection Solid component < 80% Papillation > 3 mm in height 1

2 Multilocular cyst purely cystic lesion, with at least one septae Solid solid component comprise >80% of the lesion Assessment of cyst wall if any irregularity is seen: classify as irregular Incomplete septa Typical finding in hydrosalpinx Smooth/regular Smooth/regular Irregular: papillary projections Irregular: Sludge is also regarded as an irregularity Papillary projection solid protrusion > 3mm in height Diffrentiate solid components from papillary projections Papillary projections But, all solid components Irregular papillary projections Smooth papillary projection Seen in decidualized endometrioma Are solid components Are not papillary projections 2

3 Differentiate: amorphous wall deposits from solid papilary projections Cystic Content Amorphous wall deposits - endometrioma White ball - dermoid Papilary projection suspect malignancy/ BOT BOT with papilla Sonolucent Anechoic Low level Echogenic ground glass hemorragic mixed Acoustic shadows Colour score Acoustic shadow in fibroma Acoustic shadow In dermoid Acoustic shadowing from papillations in cystadenoma Colour score 1 No bloodflow Colour score 2 Minimal bloodflow Colour score 3 Colour score 4 Moderate bloodflow Marked/high bloodflow Prediction of malignancy using the IOTA terminology Subjective assessment using pattern recognition Simple rules (Mathematical models) LR1 (12 variables) LR2 (6 variables) Adnex model (multimodal) Simple Rules RM (risk predicition model9 Subjective assessment using Pattern recognition in discriminating benign from malignant lesions Absence of solid components and irregularities suggests benignity. Solid vascularized components and irregularities suggest malignancy. 3

4 Accuracy of ultrasound in the diagnosis of malignancy using saubjective pattern recognition sens spec Malignancy vs Benignity 88-96%* 90-96%* Correct specific diagnosis over all 40% in benign tumours 68%* Expert ultrasound examiners were uncertain in 8% of cases Valentin L et al, 2007: (n=1066): Example of difficult tumours: Fibroma Pedunculated/intraligamental Fibroid Struma ovarii Borderline Cystadeno(fibr)oma Serous BOT Serous cystadenoma Mucinous BOT Mucinous cystadenoma Valentin et al 1999, Timmerman et al 1999, Timmerman 2006 Fibroma Struma Ovarii Intraligamental fibroid Difficult masses 1: With papillary projections Difficult masses 2: Solid ovarian masses Malignancy Regular echogenicity shadowing, and sparse vascularization supports benign diagnosis. Irregular echogenicity/outline, no shadowing suggest malignancy Fibroma -benign Benign < 3 papillary projections Papillary max diameter < 7 mm No papillary flow Shadowing Malignant /Borderline > 4 papillary projections Papillary diameter > 7 mm Papillary flow Malignant granulosa cell tumor Cancer Benign Brenner tumor Difficult masses 2: Solid pelvic masses can also be extraovarian pathology Difficult masses 3: Multilocular cysts with a large number of locules Pedunculated sarcoma Tubal cancer Intraligamental fibroids Mescenterial fibromatosis Important to look for the ovary! Neurofibromatosis Mucinous cystadenoma Size <10 cm Suggests benign diagnosis Mucinous intestinal borderline Techa lutein cyst functional; pregnancy/molar/trofoblastic disease 4

5 Benign or malignant? Adnexal masses: Case 1 Woman 43 years old. Benign? Malignant? Diagnosis? Adnexal masses. Case 2 Adnexal masses: Case 3 Woman 63 years old, episodes with abundant fluor. Benign? Malignant? Diagnosis? Incidentally detected bilateral lesion, woman 80 years old. Diagnosis? Management? Adnexal lesions: Case 4 Adnexal masses: Case 5 Woman 29 years old, incidental finding. 5cm lesion. Diagnosis? Management? Woman 32 years, Pregnant GW 18+ abdominal pain Differential diagnosis? 5

6 IOTA Simple Rules - Classifies 80% of all lesions Predicition of malignancy in adnexal masses using IOTA models: Simple rules Ovarian cancer Mucinous cystadenoma Dermoid Simple Rules Accuracy very high in masses that apply to the rules (80%) sensitivity 91% specificity 96% Simple rules works well also in the hands of less experienced examiners (Alcazar 2013), (Sayasneh 2013) Work better than RMI both in the hands of experienced and less experienced examiners (Ameye 2012, Sayasneh 2013) IOTA Simple Rules is one of the best approaches to preoperatively classify adnexal masses as benign or malignant (Kaijser 2014) Correct application of the Simple Rules requires knowledge and proper use of the ultrasound features. (Timmerman 2000) Recomended in the national Swedish guidelines in favor of RMI especially in pre-mp women Benign (B) - features B2 Solid component < 7mm B3 Acoustic shadowing B4 Multilocular smooth, < 10 cm B5No bloodflow Malignant (M) - features Simple Rules - interpretetion Benign (B) features Malignt (M) features M1 Irregular solid lesion M2 Ascites M3 > 4 papillary projections B1 Unilocular B2 Largest solid component diameter <7 mm B3 Presence of acoustic shadows B4 Smooth multilocular tumour with largest diameter <100 mm B5 No blood flow M1 Irregular solid tumour M2 Presence of ascites M3 At least four papillary structures M4 Irregular multilocular solid tumour with largest diameter 100mm M5 Very strong blood flow (colour score 4) At least one B-feature, No M-features = probably benign At least one M-feature, No B-feature= probably malignant Both M and B features or neither B nor M features = inconclusive M4 Multilocular solid, > 10 cm M5 Strong blood flow 6

7 What to do with the inconclusive cases? Treat all inconclusive cases as malignant Send all inconclusive cases for expert US assessment (SR + SA, sens 91% spec 91%) Simple rules: Case examples Case example 1: Case example (1) Simple Rules tick box Woman, 32 years old, Largest diameter 12cm, Examined in non-oncology centre: Ultrasound features predictivefor a malignant tumor (M-features) M1 Irregular solid tumor M2 Presence of ascites M3 At least four papillary structures M4 Irregular multilocular solid tumor with largest diameter 100 mm Features predictive for a benign tumor (B-features) B2 Presence of solid components where the largest solid component has a largest diameter < 7 mm B3 Presence of acoustic shadows B4 Smooth multilocular tumor with largest diameter < 100 mm M5 Very strong blood flow (color score 4) B5 No blood flow (color score 1) No features present Case 1 management & findings Refer to oncology center? Refer for expert US examination? Operate in regional centre? Expert US assessment: Impossible to say if it is a mucinous cystadenoma or mucinos intestinal borderline, Probablility of invasive malignancy low Case example (2) Patient seen in oncological center Histology: Mucinous Cystadenoma 7

8 Case example (2) Simple Rules tick box Ultrasound features predictivefor a malignant tumor (M-features) M1 Irregular solid tumor M2 Presence of ascites Features predictive for a benign tumor (B-features) B2 Presence of solid components where the largest solid component has a largest diameter < 7 mm Case 2 management & findings Operate in regional centre? Follow-up if no symptoms or high co-morbidity? Expert US assessment: Papillation with shadowing and without blood flow support benign diagnosis M3 At least four papillary structures M4 Irregular multilocular solid tumor with largest diameter 100 mm B3 Presence of acoustic shadows B4 Smooth multilocular tumor with largest diameter < 100 mm Histology: Benign cystadenofibroma M5 Very strong blood flow (color score 4) B5 No blood flow (color score 1) Case example 3 Case example (3) Simple Rules tick box Age 74, no ascites, maximum diameter 67mm, max size of solid component 49mm, examined in oncology centre Ultrasound features predictivefor a malignant tumor (M-features) M1 Irregular solid tumor M2 Presence of ascites M3 At least four papillary structures M4 Irregular multilocular solid tumor with largest diameter 100 mm Features predictive for a benign tumor (B-features) B2 Presence of solid components where the largest solid component has a largest diameter < 7 mm B3 Presence of acoustic shadows B4 Smooth multilocular tumor with largest diameter < 100 mm M5 Very strong blood flow (color score 4) B5 No blood flow (color score 1) Case 3 management & findings High risk of ovarian cancer Should undergo surgery in oncology centre Preoerative assessment of tumor extension indicated (CT, PET-CT; Ultrasound) to assess if optimal debulking can be done. If not* consider tru-cut biopsy to establish diagnosis and to select Chemo. Histology: Stage III ovarian cancer * Pulmonary metastasis, deep liver metastasis, carcinosis on small intestine/ LN metastasis above renal arteries Bulky tumor in lever hilum Case example 4 Woman 32 years old. Unilateral lesion max diameter 97mm, seen in oncolony unit 8

9 Case example (4) Simple Rules tick box Ultrasound features predictivefor a malignant tumor (M-features) M1 Irregular solid tumor M2 Presence of ascites M3 At least four papillary structures M4 Irregular multilocular solid tumor with largest diameter 100 mm Features predictive for a benign tumor (B-features) B2 Presence of solid components where the largest solid component has a largest diameter < 7 mm B3 Presence of acoustic shadows B4 Smooth multilocular tumor with largest diameter < 100 mm Case 4 managment and findings Expert US assessment: Features resembelling teratoma. Struma Ovarii? Malignancy can not be ruled out. Advisable to perform ooforectomy Pre surgical tumor markers & CT M5 Very strong blood flow (color score 4) B5 No blood flow (color score 1) Case example (5) Case example (2) Simple Rules tick box Patient seen in oncological center, age 65 Smooth solid mass right ovary, measuring 68x68x65 mm Free fluid in the pouch of Douglas, but no ascites present Ultrasound features predictivefor a malignant tumor (M-features) M1 Irregular solid tumor M2 Presence of ascites M3 At least four papillary structures M4 Irregular multilocular solid tumor with largest diameter 100 mm Features predictive for a benign tumor (B-features) B2 Presence of solid components where the largest solid component has a largest diameter < 7 mm B3 Presence of acoustic shadows B4 Smooth multilocular tumor with largest diameter < 100 mm M5 Very strong blood flow (color score 4) B5 No blood flow (color score 1) Case example (5) Expert US assessment: fibroid? CT, tumor markers prior to surgery Advice Experienced examiners: pattern recogntion does the job. Moderate experienced examiners: Simple rules and Simple Rules RM help you in a large proportion of cases. Consider refering inconclusive cases for expert US assessment It is crucial to understand the terms and defintions to be able to use the models! 9

10 IOTA collaboration website Educational material IOTA Terminology Easy descriptors Simple rules IOTA models software LR2, simple rules ADNEX model IOTA online lectures How to make a report on an adnexal mass? Describe lesion(s) according to IOTA classification Unilocular, multilocular, etc.. Colourscore (1-4) Mobility? Assess probability of malignancy Certainly bening, probably benign, inconclusive, probably malignant, certainly malignant Try to give a specific diagnosis Fibroma, endometrioma, borderline tumor, hydrosalpinx, peritonela cyst, etc. Give advise on managment Thank you for your attention! 10