Uterine leiomyoma 子宫肌瘤 and sarcoma 子宫肉瘤

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2 Uterine leiomyoma 子宫肌瘤 and sarcoma 子宫肉瘤 Obstetrics and Gynecology Hospital of Fudan University Weiwei Feng, MD,Ph.D

3 Contents Uterine leiomyomas Myomas fibroids symptoms incidence diagnosis classification pathology The most common benign conditions of the uterus Differential diagnosis treatment degenerations

4 Incidence True incidence--- uncertain Clinically evident in 20%~30% of the women over 30 years old 69%~77% of women who underwent hysterectomy for non-cancerous condition were found have leiomyomas an exceedingly frequent event

5 Etiology Related to hormones ( estrogen and progesterone) Elevated ER expression in myomas Abnormal cytogenetics

6 Classification ( location) Intramural 60~70% Corpus ( 90%) Subserous 20% Submucous 10~15% Cervix ( 10%)

7 subserosal leiomyomas ( 浆膜下肌瘤 )

8 Multiple leiomyomas-intramural 肌壁间肌瘤

9 Submucosal leiomyoma 粘膜下肌瘤

10 Pathology- grossly examination : Pseudo capsule Margins : blunt, non-infiltrating, pushing cut surface: whorled, spiral patterns of fibers

11 Leiomyoma-- microscopic features Elongated smooth muscle cells and abundant reticulinsm. No nuclear atypia, mitotic figures are absent or sparse.

12 Degenerations ( 变性 ) Hyaline degeneration Red degeneration Cystic degeneration Benign degenerations Degeneration with calcification Sarcomatous degeneration malignant degeneration ( 0.4~0.8%)

13 Hyaline degeneration ( 透明样变 ) uniform, eosinophilic, ground-glass appearance

14 Red degeneration ( 红色变性 ) A deep pink or red, consistancy softer The ghosts of the muscle cells and their nuclear remain

15 Sarcomatous change( 肉瘤样变 ) 1. Margin not well defined, blurred, merging, irregular 2. Yellow, tan, or gray color 3. Loss of whorled pattern 4. Heterogeneity 5. Softer, less rubbery 6. Absence of a bulging surface

16 Symptoms 40~50% asymptomatic, discovered incidentally after routine examination

17 Menorrhagia ( 月经过多 ) menorrhagia Intramural myoma Submucous myoma anaemia Short of breath Palpitations weakness

18 Pelvic mass, compressive symptoms frequency/ retention Nephrohydrosis Ureter disrention constipation Discomfort urethral obstruction Cervical or lower segment Ureteral obstruction Cervical or broad ligment Recto-sigmoid compression Posterior Pelvic mass--- pressure Depending on location of the myoma

20 pain Text Red degeneration in here Torsion of pendunculated myoma Extruding of submucous myoma

21 Other symptoms discharge Infertility/ abortion lower abdominal discomfort

22 Myoma and infertility Leiomyomas are an infrequent primary cause of infertility 27% of women who received myomectomy had a history of infertility.

23 Myoma and pregnancy Pregnancy loss, abortion Increased cesearan section Post partum hemorrahage Red degeneration Growth of myomas Most patients have uncomplicated pregnancies and diliveries. No demonstatable change in size has been noted in 70~80%

24 Physical signs General examination signs related to amenia : pale, low Bp signs related to mass: palpable mass, asymmetric abdomen

25 myoma of corpus Asymmetric enlargement of uterus Distorted uterine contour Consistency firm or rubbery Hard or stony ( calcified) Soft ( cystic)

26 myoma of cervix Distortion and elongation of the cervical canal Retention of urine nephrohydrosis

27 Diagnostic methods History Physical signs Ultrasound Based on 1,2,3, diagnosis is not difficult

28 4 Cervical cytology 5 D&C To rule out cervical cancer and endometrial cancer

29 6 Hysterosalpingography 7 Hysteroscopy 8 laparoscopy 9 Other lab studies ( HCG, Hb)

30 Differential diagnosis 1 Pregnant uterus Ovarian tumor Uterine adenomyosis Endometrial cancer and Other diseases

31 Pregnant uterus and leiomyomas Pregnant uterus leiomyoma History amenorrhoea Regular period, menorrhagia Signs Ultrasoun d Symmetric enlarged uterus Sac or fetus in cavity Lab. test HCG + HCG - Usually distorted uterus Low-echoed mass

32 Ovarian tumor VS. leiomyoma Solid ovarian tumor VS. Subserous leiomyoma Ovarian cyst VS. Cystic /hyaline degenerative myoma

33 adenomyosis 腺肌病 VS. leiomyomas adenomyosis leiomyoma

34 Endometrial cancer / hyperplasia VS. submucous leiomyoma Age Irregular bleeding thickness of endometrium ultrasound D & C. hysteroscopy

35 Management

36 1. principle Question: What will you do before you recommend treatment to a patient with leiomyoma? factors should be taken into consideration age desire of childbearing symptoms location size malignant change

37 2. observation Observation with close follow-up primarily for small and asymptomatic leiomyomas; perimenopausal women

38 3. Medications Androgens Mifepristone ( Ru486) : 12.5mg P.O. progesterone receptor antagonist GnRH analogues short term use gesorelin ( 3.6mg q28d 6), leuprorelin: ( 3.75mg q28d 6)

39 GnRH analogues Effecacy : 40~60% decrease in uterine volume Side effecs: hypoestrogenism reversible bone loss and hot flashes GnRH agonists with estrogen add-back therapy Regrowth is experienced within a few months after stopping therapy. Cost

40 Preservation of fertility before attempting conception Indications of GnRH analogues Treatment of anemia to allow recovery of Hb before surgery, minimizing the need for blood transfusion Preoperative treatment of large leiomyomas to make vaginal hysterectomy, hysteroscopic resection, or laparoscopic surgery more feasible. Treatment of Women with contraindications to surgery, or personal or medical indications for delaying surgery

41 4. Surgery Asymptomatic leiomyomas do not usually require surgery Which patients need surgery? Which factors should be considered?

42 Abnormal uterine bleeding with resistant anemia, unresponsive to hormone management Indications for surgery Chronic pain with severe dysmenorrhea, dyspareunia, or lower abdominal pressure or pain Acute pain, as in torsion of a peduculated leiomyoma, or prolapsing submucosal fibroid. To be continued

43 Indications for surgery Markedly enlarged uterine size with compression symptoms ( Urinary symptoms or signs such as hydronephrosis after complete evaluation Infertility, with leiomyomas as the only abnormal finding Rapid enlargement of uterus during the premenopausal years or any increase in a postmenopausal women

44 surgical procedures Myomectomy Indications: 1. young patients who desire for childbearing 2. patients refuse the loss of uterus which they associate with the idea of femineity. recurrence risk: as high as 50%, and up to 1/3 requiring repeat surgery

45 Myomectomy Abdominal laparoscopic: vaginal: cervix or submucous myoma hystero-scopic : submucous myoma Advantages of laparoscopy: Minimizes incision, quicker recovery Disadvantages Risks of convertion to a laparotomy Immature suture technique: uterine rupture during pregnancy

46 hysterectomy indications: older and no requirement of uterine preservation concerns: reduction of ovary preservation sexual satisfaction Abdominal / laparoscopic / vaginal

47 video Laparoscopic myomectomy

48 Uterine sarcomas

49 General information Rare tumors of mesodermal origin 2~6% of uterine malignancies Poor prognosis ( death occurring within 1 to 2 years after diagnosis, except ESS)

50 Classification Whether mesodermal elements or epithelial elements exist at the same time Pure: only malignant mesodermal elements are present mixed: both malignant mesodermal and malignant epithelial elements present

51 Whether malignant mesodermal elements are normally present in uterus Homologous : tumor origins from smooth muscle and stroma Heterologous: malignant striated muscle and cartilage.

52 Three commonest uterine sarcomas Leiomyosarcoma (~45%) Endometrial stromal sarcoma (ESS), low grade and high grade (15~25%) Uetrine sarcomas MMMT(30~40%) Maligmant Mixed mesodermal tumor

53 leiomyosarcoma( 平滑肌肉瘤 ) Age: yr, Usually arise de novo from uterine smooth muscle, rarely arise in a preexisting leiomyoma Rapid enlargement of a fibroid is a possible sign of malignancy D&C are diagnostic only for ~10% of tumors that are submucosal. Diagnosis usually not made before surgery. Poor prognosis

54 leiomyosarcoma mitotic figures> 10/10HPF severe cytologic atypia coagulative tumor necrosis

55 Endometrial stromal sarcoma ESS, low grade ( 低级别子宫内膜间质肉瘤 ) Most ESS involve endometrium, infiltrate muscles, sometimes protrude from the OS. D&C lead to diagnosis (about half). The only uterine sarcoma related to Estrogen, ER, PR (+), response to hormone treatment Behavour : indolent, late recurrence and metastasis may occur. 5-yr survival >80%

56 ESS, low grade Origin: endometrial stroma cells, the cells is similar to proliferative phase With invasive margin and vascular features ESS with invasive border

57 ESS, high grade after WHO 2003 Undifferentiated endometrial sarcoma ( UES) 未分化子宫肉瘤 ) UES: behave aggressively, with 5-yr survival < 50%. UES with severe atypia

58 MMMT Malignant mesodermal mixed tumor or carcinosarcoma ( 癌肉瘤 ) In FIGO 2009, carcinosarcoma was regarded as type II endometrial carcinoma, because the prognosis is mainly determined by epithelial elements.

59 MMMT older age group, most patients being postmenopausal. enlarged or irregular uterus, and the tumor protrudes through the cervical OS like a polyp in approximately half the patients. Aggressive, grows rapidly, recurrence rate: high, 5 yr survival 11~35%.

60 MMMT adenosarcoma Carcinosarcoma 2009 EC

61 Pattern of spread Directly spread (to myometrium, pelvic structures) pelvic vessels, lymphatics,

62 Symptoms and signs Uterine Bleeding ( 75%~95%) Pelvic pain (33%) Pelvic mass ---Enlarged uterus ( 15%~50%) Prolapsed necrotic tissue through cervical os

63 staging Old staging system: UICC New staging systems ( FIGO 2009) Three different staging systems for 1. leiomyosarcoma 2. ESS and adenosarcoma 3. carcinosarcoma

64 Staging FIGO 2009 leiomyosarcoma I Tumor limited to uterus IA<5CM IB 5CM II Tumor grows outside of uterus but not outside the pelvis IIA tumor is growing into adnexa IIB tumor is growing to the tissue of pelvis other than adnexa III tumor grows into tissue of abdomen ( not just intruding into abdomen) IIIA in one place IIIB in 2 or more places IIIC tumor has spread to pelvic/ parpaotic lymphnodes IV The tumorr has spread to the urinary bladder or the rectum, and/or to distant organs, such as the bones or lung IVA spread to bladder or the rectum IVB distant metastasis

65 Staging FIGO 2009 ESS and adenosarcoma I Tumor limited to uterus IA limited to endometrium IB <1/2 myometrium IC 1/2 myometrium II Tumor grows outside of uterus but not outside the pelvis IIA tumor is growing into adnexa IIB tumor is growing to the tissue of pelvis other than adnexa III tumor grows into tissue of abdomen ( not just intruding into abdomen) IIIA in one place IIIB in 2 or more places IIIC tumor has spread to pelvic/ parpaotic lymphnodes IV The tumor has spread to the urinary bladder or the rectum, and/or to distant organs, such as the bones or lung IVA spread to bladder or the rectum IVB distant metastasis

66 Staging FIGO 2009 for carcinosarcoma Staging for endometrial cancer

67 Treatment 1. Surgery: only treatment of proven curative value typically : hysterectomy + bilateral oorphorectomy

68 Surgical staging: hysterectomy/ radical hysterectomy+ pelvic and or para-aortic lymphnectomy +omentatectomy+ peritoneal washing cytoreductive surgery for advanced stage ( III or IV) patients

69 2. Adjunvant therapy: Chemotherapy +/- radiotherapy Radiotherapy improves tumor control in the pelvis without influencing final outcome chemotherapy : response rate (~20%) Drugs: doxorubicin, cisplatin, ifosfamide, palitaxel

70 3. Hormone therapy ( only used in ESS, low grade) progesterone, letrozol GnRH antagonist

71 Prognosis prognositic factors stage is the most important prognostic variable. Cell type, grade, metastasis, and treatment leiomyosarcoma : 5-year survival: 30%~40%. If the leiomyosarcoma arises in a benign fibroid, the prognosis is improved.

72 MMMT: recurrence rate 53%, poor prognosis, 5-yr survival 11~35%. ESS, low grade. 5-yr survival >80%. are indolent tumors with a tendency to late recurrence. UES are highly aggressive with 5-yr survival less than 40%.

73 Case discussion A 29 year old woman complains heavy bleeding during period for 1 year. She has regular period. Physical examination shows pale and short of breath. Pelvic examination revealed enlarged uterus with a size of twomonth pregnancy. A 65/55/50 mm low-echoes mass with clear margin was seen by ultrasound. In addition, a 23/20/19mm low echoes mass protrudes from uterus cavity. Lab test: Hb: 80g/L.

74 Questions What s the diagnosis? ( give the evidence) Which diseases should be excluded? What is the suitable treatment? Does this treatment affect fertility?

3/25/2019. Rare uterine cancers ~3% Leiomyosarcoma Carcinosarcoma (MMMT) Endometrial Stromal Sarcomas Aggressive tumors High Mortality Rates

3/25/2019. Rare uterine cancers ~3% Leiomyosarcoma Carcinosarcoma (MMMT) Endometrial Stromal Sarcomas Aggressive tumors High Mortality Rates J. Anthony Rakowski D.O., F.A.C.O.O.G. MSU SCS Board Review Coarse Rare uterine cancers ~3% Leiomyosarcoma Carcinosarcoma (MMMT) Endometrial Stromal Sarcomas Aggressive tumors High Mortality Rates Signs