Uterine malignancy 2

Question 1

What are the common associations of type 1 endometrial tumours ?

Answer 1

• Endometrioid and mucinous phenotypes
• PTEN, KRAS, PIK3CA mutations
• Associated with atypical hyperplasia as precursor lesion
• Microsatellite instability
• Lynch syndrome associated
• Related to unopposed oestrogen throughout life e.g. no pregnancies or late menopause

Question 2

Breifly what is lynch syndrome ?

Answer 2

• iIs a cancer predisposition syndrome - causing high risk of colon, endometrial and ovarian cancer
• Due to the inheritance of a defective DNA mismatch repair gene. Autosomal dominant inheritance.

Question 3

What are the common associations/key points about type 2 endometrial tumours ?

Answer 3

• Serous and clear cell phenotypes
• TP53 mutation and overexpression
• Precursor lesion serous endometrial intraepithelial carcinoma
• Spreads along Fallopian tube mucosa and peritoneal surfaces so can present with extrauterine disease
• More aggressive than endometrioid/mucinous carcinoma
• Surgery usually more extensive and adjuvant chemo/radiotherapy used more frequently

Question 4

What are the characteristic features of serous cell carcinoma of the endometrium?

Answer 4

Characterised by a complex papillary (small projections) and/or glandular archietecture with diffuse, marked nuclear pleomorphism

Question 5

What is the characteristic features of clear cell carcinoma of the endometrium ?

Answer 5

Cells with clear cytoplasm and a compact-alveolar (nested) or acinar growth pattern

Question 6

What is the difference in the invasive progression of endometrioid and serous cell carcinomas ?

Answer 6

Endometrioid usually confined to the uterus at presentation

Serous cell may spread early to the peritoneal cavity

Question 7

What is the grading for endometrial carcinomas

Answer 7

Endometrioid carcinoma are primarily graded by their architecture

1. Grade 1 5% or less solid growth
2. Grade 2 6-50% solid growth
3. Grade 3 >50% solid growth

Serous carcinoma and clear cell carcinoma are not formally graded

Question 8

What is the staging of endometrial carcinomas

Answer 8

Stage I Tumour confined to the uterus:

• IA no or < 50% myometrial invasion
• IB Invasion equal to or > 50% of myometrium

II Tumour invades cervical stroma

III Local and or regional tumour spread:

• IIIA Tumour invades serosa of uterus and/or adnexae
• IIIB Vaginal and/or parametrial involvement
• IIIC Metastases to pelvic and/or para-aortic lymph nodes

IV Tumour invades bladder and or bowel mucosa (IVA) and/or distant metastases (IVB)

Question 9

What are the 3 main other types of endometrial carcinoma and what do they arise from

Answer 9

Endometrial stromal sarcoma:

• Tumour arising from endometrial stroma

Carcinosarcoma:

• Mixed tumour with malignant epithelial and tromal elements
• Poor prognosis

Leiomyosarcoma

Question 10

What are the key points about endometrial stromal sarcoma ?

Answer 10

Can be high or low grade

High grade associated with increased proliferative and atypica, also metastases most commonly to ovary or lung may be the first presentation for high grade

Question 11

What are the key points about carcinoma sarcoma ?

Answer 11

• Heterologous elements commonly seen in about 50% cases (rhabdomyosarcoma, chrondrosarcoma, osteosarcoma)
• The presence of a rhabdomyosarcomatous component has the worst prognosis

Question 12

What are some of the other abnormalities seen in the uterus ?

Answer 12

• Fibroids (leiomyoma)
• Leiomyosarcoma (rare)

Question 13

What is a leiomyosarcoma ?

Answer 13

• A malignant smooth muscle tumour commonly displaying a spindle cell morphology
• The most common uterine sarcoma
• Most occur in women >50 years
• Commonest symptoms abnormal vaginal bleeding, palpable pelvic mass and pelvic pain
• Poor prognosis even if confined to uterus at time of diagnosis
• Overall 5 year survival rates 15-25%