From Imaging to Pathology :
How to assess Benignity in Rare Ovarian Tumors?

Corinne Balleyguier, Radiology

Pierre Duvill...
How to assess Benignity in Rare Ovarian
Tumors?
—  Imaging ??
—  Nearly never…
—  Excepted for some functional ovarian ...
Functional Ovarian Lesions
Functional ovarian
cyst: YES
—  Ultrasound may

assess the content
of a liquid cyst

—  Harmonic imaging

may be useful ...
Color Döppler?
—  Color Döppler is not

accurate:
—  In 30 % of cases, arterial

flow is found in cystic
wall
—  Usuall...
Haemorrhagic functional
cyst: YES

Internal content with thin
walls
No blood flow inside thin
walls
The cyst must disappea...
Endometrioma: YES
—  Ultrasound is usually

enough
—  Typical features:
—  Thin echoes,

homogeneous blood
cyst
Complex endometrioma: US/MRI
—  Rarely, clots may

simulate papillary
projections
—  Additional MRI is useful
T2	


T1	


Endometrioma
Hyperintense T1w
Hyper / Hypointensity T2w
Hyperintense T1+ FS

T1 + Fat Sat
And Rare Ovarian Tumors ??
What are Rare Ovarian Tumors ?

Germ cell
ovarian tumors

Immature teratoma

Monodermal teratoma
(struma ovarii)
Carcinoid...
Stromal Tumors

Fibroma
Thecoma
Fibrosarcoma
Stromal T with minor
sex-cord
differenciation
Sclerosing stromal T

Sex cord
...
To play in defence
or
what are the findings for benign?
Pure solid ovarian tumor on
ultrasound
Ovarian Fibroma
—  Diagnosis of ovarian fibroma may nearly be assess on

imaging : US + MRI
—  Ultrasound findings:
—  ...
Ovarian Fibroma
Ovarian Fibroma : MRI
—  MRI is the best imaging

examination to assess fibrous
content
—  Hypointense signal : T1w and
...
Ovarian Fibroma
Serous Borderline Cystadenoma
Benign

Malignant
Bénin

Borderline

Thomassin-Naggara, I. et al. Radiology
2008;0:248107112...
Right Fibroma
Left Fibrothecoma
Stromal Ovarian Tumors
(Fibroma, Thecoma)
—  Unilateral tumors nearly always benign
—  Solid content, white/yellow colou...
Homogenous, echoic Cyst on
Ultrasound
Echoic, homogenous cyst

Mature
Teratoma
Teratoma : Germ Cell Tumour
— Ultrasound features can vary according

internal content : cyst, fat, calcification.
— Ult...
Cystic Mature
Teratoma
MRI may assess fatty content
— Fat signal characterization:
— Hyperintense signal : T1w
— Hyperintense signal : T2w
— ...
T1w

T2w

Cystic
mature
teratoma
T1 FS
Benign Teratoma : Pathology
—  Polydermal teratoma:
—  Solid (15-20 % of solid teratoma)
—  Cystic (dermoïd cyst)
—  2...
Benign Teratoma
—  Monodermal dedicated
—  Struma Ovarii
—  Ovarian carcinoïd T
—  Insulet carcinoïds
—  Trabecular c...
Attacking Game or How to find Malignant
Features …
Most common rare malignant
Ovarian Tumors…
Immature Teratoma
Granulosa cell Tumor
Immature Teratoma?
—  Immature teratoma is rare
—  Difficult diagnosis on imaging
—  Imaging :
—  Heterogenous interna...
Immature Teratoma
Pathologist : Referee
Immature Teratoma
—  Clinical features :
—  3% of ovarian teratoma (nearly 20% of primary

malignant germ cell tumors an...
Immature Teratoma
—  Microscopic features:
— 

— 

— 

Polymorphous lesions including variable amount of
mainly neurec...
Immature Teratoma
—  Treatment and prognosis:
—  According tumor stage and tumor grade.
—  Conservative surgery if poss...
Pitfall: Growing Teratoma
—  Multifocal peritoneal

extension of an immature
teratoma after complete
response with CT :
—...
Granulosa Cell Tumor
—  Rare ovarian T (0.6-3% of all ovarian T)
—  5% of malignant lesions

—  Mesenchymatous and sex-...
Adult Granulosa Cell T
35 yo
15 yo
Juvenile Granulosa Cell T
Pathologist : Referee
Adult Granulosa Cell Tumors
—  Clinical features :
—  6% of malignant ovarian tumors
—  Usually diagnosed after menopau...
Juvenile Granulosa Cell Tumors
—  Clinics:
—  5% of granulosa cell tumors and 97% are

diagnosed before 30 years
—  Hor...
Granulosa Cell Tumors
—  Prognostic factors :
—  Stage at diagnosis

—  Evolution and treatment:
—  Malignant lesions ...
Across the Line…
Very rare Ovarian Tumors…
Does Imaging help to diagnose a
rare malignant Ovarian Tumor?
—  Common features to detect :
—  Size > 10 cm
—  Haemorr...
Endodermal sinus Tumor : Yolk sac T
—  Rare malignant germ cell tumor (< 1 % malignant T)
—  20-30 yo

—  Variable feat...
Yolk Sac Tumor
—  Clinics:
—  20% of malignant germ cell ovarian tumors (medium

age 16-19 yo) (10% before10 yo)
—  Fas...
Yolk sac Tumor
—  Macroscopic features:
—  Large tumor (15 cm), unilateral, sometimes

peritoneal carcinomatosis.
—  Ex...
Choriocarcinoma
—  < 1 % malignant T
—  15 yo
—  Imaging features :
—  Necrosis
—  Hypervascularization
—  Periphera...
Conclusion
—  To assess benignity in rare ovarian

tumors …….
—  With pathology: YES, usually
—  With surgery…
—  Imag...
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
Urology gynecology anapath et imagerie c balleyguier
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Urology gynecology anapath et imagerie c balleyguier

  1. 1. From Imaging to Pathology : How to assess Benignity in Rare Ovarian Tumors? Corinne Balleyguier, Radiology Pierre Duvillard, Pathology Gustave Roussy, Villejuif
  2. 2. How to assess Benignity in Rare Ovarian Tumors? —  Imaging ?? —  Nearly never… —  Excepted for some functional ovarian lesions —  Simple functional cyst —  Haemorraghic functional cyst —  .. —  Some common benign ovarian lesions —  Endometrioma —  Ovarian fibroma —  Pathology?? —  Nearly ever… —  To avoid for functional lesions …
  3. 3. Functional Ovarian Lesions
  4. 4. Functional ovarian cyst: YES —  Ultrasound may assess the content of a liquid cyst —  Harmonic imaging may be useful to better define cystic content
  5. 5. Color Döppler? —  Color Döppler is not accurate: —  In 30 % of cases, arterial flow is found in cystic wall —  Usually with a low resistive index —  Be careful to misunderstanding with a malignant lesion !!!
  6. 6. Haemorrhagic functional cyst: YES Internal content with thin walls No blood flow inside thin walls The cyst must disappeared on short time follow-up at D6 NO MRI !!
  7. 7. Endometrioma: YES —  Ultrasound is usually enough —  Typical features: —  Thin echoes, homogeneous blood cyst
  8. 8. Complex endometrioma: US/MRI —  Rarely, clots may simulate papillary projections —  Additional MRI is useful
  9. 9. T2 T1 Endometrioma Hyperintense T1w Hyper / Hypointensity T2w Hyperintense T1+ FS T1 + Fat Sat
  10. 10. And Rare Ovarian Tumors ??
  11. 11. What are Rare Ovarian Tumors ? Germ cell ovarian tumors Immature teratoma Monodermal teratoma (struma ovarii) Carcinoid T Neuroectodermic T Non teratomatous T (Dysgerminoma) Yolk sac T Embryonnary carcinoma Non gravidic choriocarcinoma Polyembryoma Ovarian tumor classification OMS 2003 Mixed germ cell Tumeurs
  12. 12. Stromal Tumors Fibroma Thecoma Fibrosarcoma Stromal T with minor sex-cord differenciation Sclerosing stromal T Sex cord ovarian T Granulosa stroma cell T Sertoli stromal cell T Sex cord of mixed or unclassified cell type Gynandroblastoma Indifferenciated sex cord T Ovarian tumor classification OMS 2003 Steroïd T Stromal Luteoma Leydig cell T Steroid cell T
  13. 13. To play in defence or what are the findings for benign?
  14. 14. Pure solid ovarian tumor on ultrasound
  15. 15. Ovarian Fibroma —  Diagnosis of ovarian fibroma may nearly be assess on imaging : US + MRI —  Ultrasound findings: —  Solid ovarian mass —  Homogeneous content —  Arterial flow —  Ultrasound may be doubtful in case of old ovarian fibroma: —  Heterogenous —  Posterior attenuation —  Low blood flow —  èMRI
  16. 16. Ovarian Fibroma
  17. 17. Ovarian Fibroma : MRI —  MRI is the best imaging examination to assess fibrous content —  Hypointense signal : T1w and T2w —  Moderate to high intense uptake —  Enhancing curves lesion / myometrium may be useful —  Fibrothecoma : —  Hypointense signal : T2w —  Intense uptake after injection
  18. 18. Ovarian Fibroma
  19. 19. Serous Borderline Cystadenoma Benign Malignant Bénin Borderline Thomassin-Naggara, I. et al. Radiology 2008;0:2481071120
  20. 20. Right Fibroma Left Fibrothecoma
  21. 21. Stromal Ovarian Tumors (Fibroma, Thecoma) —  Unilateral tumors nearly always benign —  Solid content, white/yellow colour, homogenous —  Medium size : 6 cm —  Histologically, architecture is fasciculated without any atypia or mitoses —  Good prognosis allows conservative surgery, especially for young women
  22. 22. Homogenous, echoic Cyst on Ultrasound
  23. 23. Echoic, homogenous cyst Mature Teratoma
  24. 24. Teratoma : Germ Cell Tumour — Ultrasound features can vary according internal content : cyst, fat, calcification. — Ultrasound diagnosis may be difficult : — A dermoïd cyst may mimic intestinal loops
  25. 25. Cystic Mature Teratoma
  26. 26. MRI may assess fatty content — Fat signal characterization: — Hyperintense signal : T1w — Hyperintense signal : T2w — Hypointense signal: T1w with fat suppression
  27. 27. T1w T2w Cystic mature teratoma T1 FS
  28. 28. Benign Teratoma : Pathology —  Polydermal teratoma: —  Solid (15-20 % of solid teratoma) —  Cystic (dermoïd cyst) —  27-44% of all ovarian tumors —  Non specific clinical symptoms —  Diameter usually < 15 cm —  Bilateral in15% of cases, sometime multiple —  External wall smooth, half-solid, half-cystic with pilosebaceous tissue —  Very heterogenous on pathology including components of the 3 primitive mature tissues.
  29. 29. Benign Teratoma —  Monodermal dedicated —  Struma Ovarii —  Ovarian carcinoïd T —  Insulet carcinoïds —  Trabecular carcinoïds —  Mucinous carcinoïds (globlet cell carcinoïds) —  Strumal carcinoïds
  30. 30. Attacking Game or How to find Malignant Features …
  31. 31. Most common rare malignant Ovarian Tumors… Immature Teratoma Granulosa cell Tumor
  32. 32. Immature Teratoma? —  Immature teratoma is rare —  Difficult diagnosis on imaging —  Imaging : —  Heterogenous internal content —  Haemorrhagic content (MRI++) —  Heterogenous, intense uptake —  Large size —  Multiple irregular calcifications —  Tissue thickening within Rokitansky’s protuberance
  33. 33. Immature Teratoma
  34. 34. Pathologist : Referee
  35. 35. Immature Teratoma —  Clinical features : —  3% of ovarian teratoma (nearly 20% of primary malignant germ cell tumors and 10-20% of malignant ovarian tumors occuring before 20 yo). —  Usually revealed with an abdominal mass and abdominal pain. —  Extra ovarian lesions in 33% of cases (peritoneal implants). —  Large tumors (18 cm medium size), usually unilateral with rupture or break of capsula in 50% of cases.
  36. 36. Immature Teratoma —  Microscopic features: —  —  —  Polymorphous lesions including variable amount of mainly neurectodermal immature tissue. Other immature content are possible : ◆  Embryologic epithelial tissue (endodermal, ectodermal..). ◆  Immature mesenchymatous tissue (cartilage, striated muscle…). ◆  Liver, renal, vitellin tissue... Mature tissue may be associated.
  37. 37. Immature Teratoma —  Treatment and prognosis: —  According tumor stage and tumor grade. —  Conservative surgery if possible + chemotherapy (BEP) for tumors grade 2 and 3 and stage II and III. —  Clinical complete response is obtained in nearly all cases after CT (77% of 5 years remission). —  Residual peritoneal lesions may be detected (fibrous nodule). —  Growing teratoma syndroma is exceptionnal.
  38. 38. Pitfall: Growing Teratoma —  Multifocal peritoneal extension of an immature teratoma after complete response with CT : —  Benign lesion —  Surgery may be complete to remove all peritoneal implants —  Nimkin K, Pediatr Radiol. 2004 Mar;34(3):259-62.
  39. 39. Granulosa Cell Tumor —  Rare ovarian T (0.6-3% of all ovarian T) —  5% of malignant lesions —  Mesenchymatous and sex-cord like tumors —  Two types: —  Adult (AGT) —  Juvenile (JGT) : malignancy risk is higher —  Medium size : 10 cm —  Hormonal clinical symptoms —  Partly cystic, haemorrhagic content
  40. 40. Adult Granulosa Cell T 35 yo
  41. 41. 15 yo Juvenile Granulosa Cell T
  42. 42. Pathologist : Referee
  43. 43. Adult Granulosa Cell Tumors —  Clinical features : —  6% of malignant ovarian tumors —  Usually diagnosed after menopause —  Associated to hyperestrogenic features in 75% of cases —  Diagnosed at stage 1 in 90% of cases —  Unilateral in 85% of cases, variable size, heterogenous with capsule rupture in 10 -12% of cases
  44. 44. Juvenile Granulosa Cell Tumors —  Clinics: —  5% of granulosa cell tumors and 97% are diagnosed before 30 years —  Hormonal features in a child —  Unilateral in 98% for stage I cases —  Macroscopic features are similar to those of Adult form tumors
  45. 45. Granulosa Cell Tumors —  Prognostic factors : —  Stage at diagnosis —  Evolution and treatment: —  Malignant lesions with low progression, late recurrence in adult (20% at 5 years) —  Death rate : 12.5% —  Surgical treatment : —  Conservative for juvenile form stage IA
  46. 46. Across the Line… Very rare Ovarian Tumors…
  47. 47. Does Imaging help to diagnose a rare malignant Ovarian Tumor? —  Common features to detect : —  Size > 10 cm —  Haemorrhagic content : ultrasound, MRI (++) —  Irregular calcifications —  Central necrosis: MRI (hyperT2, hypoT1, peripheral heterogenous enhancement) —  Jung SE, Radiographics. 2002 —  Ascitis —  Peritoneal carcinomatosis —  Lymph node
  48. 48. Endodermal sinus Tumor : Yolk sac T —  Rare malignant germ cell tumor (< 1 % malignant T) —  20-30 yo —  Variable feature (cystic to solid) —  Imaging features: —  Haemorrhagic content —  Hyperintense uptake —  Heterogenous
  49. 49. Yolk Sac Tumor —  Clinics: —  20% of malignant germ cell ovarian tumors (medium age 16-19 yo) (10% before10 yo) —  Fast growing tumors : abdominal mass, pelvic pain. Emergency for surgery : risk of ovarian torsion or tumor rupture. —  α-foetoprotein synthesis (high level (>1000 ng/ml).
  50. 50. Yolk sac Tumor —  Macroscopic features: —  Large tumor (15 cm), unilateral, sometimes peritoneal carcinomatosis. —  External wall smooth, rupture in 25% of cases. —  Solid and cystic, bloody content, necrosis —  Other germ cell tumor may be associated (15% of dermoïd cyst). —  Prognostic factors —  Clinical stage : 5 years survival 70-90% for stage 1 —  30 à 50% for other stage. —  Residual lesions after surgery —  Liver lesions.
  51. 51. Choriocarcinoma —  < 1 % malignant T —  15 yo —  Imaging features : —  Necrosis —  Hypervascularization —  Peripheral calcifications —  Biology : —  βHCG serous elevation with a non gravid uterus —  Treatment and prognosis: —  Very aggressive tumors with peritoneal extension —  Same treatment as other malignant germ cell tumors.
  52. 52. Conclusion —  To assess benignity in rare ovarian tumors ……. —  With pathology: YES, usually —  With surgery… —  Imaging : NO —  Exception : —  Functional lesions —  Fibrous tumors —  Mature teratoma : +/—  Imaging as an adjunct tool : —  Necrosis, calcifications, blood

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