Sarcomas Flashcards
What are the main types of uterine sarcoma
Leimyosarcoma
Endometrial stromal tumor (high grade, low grade and undifferentiated)
Adenosarcomas
Carcinosarcoma
How should sarcomas be followed up?
Follow‐up should be determined by risk of recurrence. As metastasis to the lungs is common, efforts must be made to rule these out remembering that early lesions tend to be asymptomatic but resectable.
Low‐grade sarcoma patients may be followed for local relapse every 4–6 months for the first 3–5 years, then yearly.
High‐grade tumors can be followed‐up every 3–4 months for the first 2–3 years, twice a year for the next 2–3 years, and then annually
What are the common sites of mets?
Lung
Abdomen
Pelvis
Pelvic and para aortic nodes
Pre op imaging for sarcomas for dx?
Pre op imaging with USS or PET is not capable for differentiating benign and malignant smooth muscle masses
Diffusion weighted MRI for tumor location and characterisation is suggested but yet to be validated
Risk factors for sarcomas
Tamoxifen (risk increase X3) ,
pelvic radiation
hereditary conditions
Age (carcinosarcomas and adenosarcomas)
What features of a leiomyoma may historically mimic sarcoma? (and confuse dx? )
Mitotically active cellular leiomyoma Haemorrhagic / hormone induced changes Myxoid epithelioid Massive lymphoid infiltration
What are the features of STUMP - smooth muscle tumors of uncertain malignant potential
Tumor cell necrosis in a typical leiomyoma
Necrosis of uncertain type
Marked diffuse atypia
Necrosis difficult to classify
Diffuse of focal atypia, borderline mitotic counts
Leiomyosarcomas
How common
What age?
1/800 fibroids
1-2% uterine malignancies
40 yo +
Leiomyosarcomas
How do they present?
AUB 56%
Palpable mass 54%
Pain 22%
Suspect in fibroid growth in post menopausal woman
Leiomyosarcomas
Pathological features?
10cm +
Soft, bulging, fleshy, Necrotic, haemorrhagic, no whorled appearance
Dx – hypercellularity, severe nuclear atypia, high mitotic rate
Other suggestive features Peri or post menopausal Extrauterine extension 10cm + Infiltrating border necrosis Atypical mitotic figures Dx can be difficult
Leiomyosarcomas
Immunohistochemistry
+ for desmin, h‐caldesmon, smooth muscle actin, and histone deacetylase 8 (HDCA8)
often immunoreactive for CD10
40% E P and androgen receptors
Overexpression p16
Ki67 is higher
germline mutations in fumarate hydratase are believed to be at increased risk
Leiomyosarcomas
Treatment ?
TAH + debulking of the tumor
Removal of the ovaries and lymph node dissection remain controversial as metastases to these organs rare
Chemo for advanced or recurrent disease
Lymph node metastases have been identified in 6.6% and 11% in two series of patients with leiomyosarcoma who underwent lymphadenectomy
Leiomyosarcomas
Prognosis?
poor
Recurrence 50-70%
40% first recurrence in the lung
5 year survival 25%
STUMP prognosis ?
Favorable prognosis
FU is recommended
Endometrial Stromal tumors High grade (HG) Low grade (LG) Undifferentiated (UD)
Presentation
(LG) 40-55 yo 50% premenopausal At risk MHT/tamoxifen/ PCOS AUB/mass (HG) 28 – 67 yup AUB, mass
(UD) post menopausal with PMB 60% present with high grade disease
Endometrial Stromal tumors High grade (HG) Low grade (LG) Undifferentiated (UD)
Pathological features
(LG) well differentiated endometrial stromal cells exhibiting mild atypia + Invade LVspaces
(HG) Intracavity polypoid or mural mass, mean 7.5cm, necrotic with haemorrhages
High grade round cells and low grade spindle cells
High mitotic activity
(UD) myometrial invasion, severe nuclear pleomorphisms, high mitotic activity and tumor cell necrosis
Endometrial Stromal tumors High grade (HG) Low grade (LG) Undifferentiated (UD)
Immunohistochemistry
(LG) CD 10 + SM actin and desmin 30% Negative h-caldesom and HDAC8 E, P ,A receptors +ve Recurrent translocation Ch 7 and 17 resulting in a fusion JAZF1 and SUZ12 (can be seen on FISH or PCR)
(HG) CD 10 neg E and P receptor negative Cyclin D1 immunoreactivity + C KIT + DOG1 neg YWHAE-FAM22 genetic fusion
(UD) CD10 +
E+P +/-
Endometrial Stromal tumors High grade (HG) Low grade (LG) Undifferentiated (UD)
Treatment
(LG) TAH BSO – hormone sensitive and if retaining ovaries much higher recurrence risk
LND doesn’t have a role
RT or hormonal tx eg progestational agents or aromastase inhibitors. MRT discouraged
(HG) as above
(UD) TAH BSO + Adjutant Tx chemo or RT
Endometrial Stromal tumors High grade (HG) Low grade (LG) Undifferentiated (UD)
prognosis
(LG) good prognosis but late recurrences so long term FU is needed 30% have recurrence
(HG) intermediate prognosis
Tx recurrences with RT and chemo
(UD) Very poor prognosis
Adenosarcomas
What is it?
Müllerian adenosarcoma is a mixed tumor of low malignant potential that shows an intimate admixture of benign glandular epithelium and low‐grade sarcoma, usually of endometrial stromal type
Adenosarcomas pathology (macro and micro)
Adenosarcomas with sarcomatous overgrowth tend to be larger with a fleshy, hemorrhagic, and necrotic cut surface. They invade the myometrium more often than conventional adenosarcomas.
polypoid tumors of approximately 5–6 cm in maximum diameter (range, 1–20 cm) that typically fill and distend the uterine cavity.
Hypercellular periglandular cuffs
Adenosarcomas
Immunohistochemistry
p53 + Ki-67 are stronger in adenosarcomas with sarcomatous overgrowth then in typical forms
CD10 + PR are higher in typical adenosarcomas
Adenosarcomas
Who gets them?
Postmenopausal women (average 58 years) but also in adolescents and young adults (30%)
Adenosarcomas
prognosis
Pretty good prognosis comparatively
Vaginal or pelvic recurrence occurs in approximately 25%–30% of cases at 5 years and is associated almost exclusively with myometrial invasion and sarcomatous overgrowth
Otherwise better prognosis
