Lei My o Sarcoma

8/10/2019 Lei My o Sarcoma

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Endometrial carcinoma

Endometrial carcinomas begin in the lining of the uterus (endometrium). They arethe most common type of uterine cancer, accounting for more than 95% of all

uterine cancers.

Endometrioid carcinoma is the most common type of endometrial cancer,

accounting for 75%!% of all endometrial carcinomas. Endometrioidcarcinomas form in the glands in the endometrium. "ariants of endometrioid

carcinoma include#

o $ith suamous differentiation

adenocanthoma made up of malignant glandular cells and

benign suamous cells

adenosuamous made up of malignant glandular cells and

malignant suamous cells

o &illoglandular

o secretory

o ciliated cell

mucinous adenocarcinoma

serous adenocarcinoma (also called papillary serous carcinoma)

clear cell adenocarcinoma

mi'ed carcinoma

suamous cell carcinoma

transitional cell carcinoma

small cell carcinoma

undifferentiated carcinoma

terine sarcoma


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The >ynecologic 1ncology >roup(>1>" uses a classification scheme foruterine sarcomas that divides them into five categories=

i#ed homologous mullerian sarcoma

i#ed heterologous mullerian sarcoma

Leiomyosarcoma

?ndometrial stromal sarcoma

1ther

igure # /ut surface of this leiomyosarcoma sho$ing...

;omologous refers to similarity to endometrial stroma or myometrium,while heterologous indicates similarity to other cell types, including fat,

muscle, etc. alignant mi#ed mullerian tumors, now calledcarcinosarcomas, arise from endometrial adenocarcinoma, but resemblesarcoma on histology.

The typical gross appearance is a large (8$&cm", poorly circumscribedmass with a soft, fleshy consistency and a variegated cut surface that isgrey-yellow to pin0, with foci of hemorrhage and necrosis. $The histologicclassification of uterine sarcomas is based upon homology to normal celltypes and include UL! (analogous to myometrium", stromal sarcoma(analogous to endometrial stroma", and other heterologous cell types

(i.e., osteosarcoma, liposarcoma". !ee 6igures ) and .

igure 0# /ut surface of this leiomyoma $ith infarction...
http://www.gog.org/http://www.gog.org/


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icroscopically, most UL! are overtly malignant,with hypercellularity, coagulative tumorcell necrosis,abundant mitoses(8$& to )& mitotic figures (mf" per $&high power fields (hpf"", atypical mitoses, cytologic atypia, and infiltrativeborders. itotic rate is the most important determinant of malignancy, but

is modified by the presence of necrosis and cytologic atypia. Thediagnosis of UL! may be made in the presence of tumor necrosis andany mitoses. In the absence of tumor necrosis, the diagnosis can be madewith moderate to severe cytologic atypia and a mitotic inde#greater than$&mf@$&hpf. 9ithout tumor necrosis and significant atypia, a high mitoticinde# is compatible with a benign clinical course, however, data islimited.$!ee 2efinitions !idebar. !ee 6igures , *, and Table ).

igure 1# Tumor necrosis consists of ghosts (no nuclei) of tumor...

1ne study loo0ed at the e#pressionof particular mar0ers that are ofinterest in gynecological cancers (p*,?pidermal >rowth 6actor,and Alatelet 2erived >rowth 6actor" in tissue samples from patients whohad UL! or benign leiomyomas. Their data demonstrated significant andmolecular differences between benign and malignant smooth muscletumors of the uterus. The study also suggested a prognosticinterrelationship between e#pression of p* and stage in UL!.$*

subset of smooth muscle tumors will not be easily classified based onthe criteria and are designated as smooth muscle tumors of uncertainmalignant potential (!TUA". The literature is unresolved on whetherspecial studies such as proliferation inde# or stains for p* add to thediscriminating power of the basic criteria of mitoses, necrosis andcytologic atypia in determining the malignant potential of !TUA lesions.In practice, however, these stains are uncommonly used, and definitivediagnosis of sarcoma is never reported based on these stains alone. $',$+

igure 5# 2itosis (center of slide)....

Limited data has allowed some tumors, formerly classified as !TUA, intothe leiomyoma category and should be distinguished from theirsarcomatous counterparts.
http://en.wikipedia.org/wiki/Mitosishttp://en.wikipedia.org/wiki/Gene_expressionhttp://en.wikipedia.org/wiki/P53http://en.wikipedia.org/wiki/Epidermal_Growth_Factorhttp://en.wikipedia.org/wiki/Platelet-derived_growth_factorhttp://en.wikipedia.org/wiki/Mitosishttp://en.wikipedia.org/wiki/Gene_expressionhttp://en.wikipedia.org/wiki/P53http://en.wikipedia.org/wiki/Epidermal_Growth_Factorhttp://en.wikipedia.org/wiki/Platelet-derived_growth_factor


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Tumors now in the leiomyoma category include= mitotically active, cellular,epithelioid, my#oid, atypical (pleiomorphic, biarre, or symplastic"tumors. itotically active leiomyomas can occur in pre-menopausalwomen and have the typical macroscopic and histologic appearance of aleiomyoma with the e#ception that they have 8 *mf@hpf. Cellular

leiomyomas tend to have hypercellularity and can suggest the diagnosisof UL!, but they lac0 tumor cell necrosis, cytologic atypia and mitoticfigures. ?pithelioid leiomyomas are yellow or grey and may contain visibleareas of hemorrhage and necrosis, and tend to be solitary and softer thanthe usual leiomyoma. y#oid leiomyomas have my#oid materialseparating the tumor cells. They are soft and translucent withcircumscribed margins with neither cytologic atypia nor mitotic figures.typical leiomyomas lac0 all the other components with the e#ception ofatypia and have little recurrence potential.$!ee 6igure '.

Table : !iagnostic Criteria for L"#$Adapted from %%& '() Guidelines1*

#tandard smooth muscle

!ifferentiation+pithelioid differentiation

"y,oiddifferentiation

(istology

Cigar-shaped spindled cellswith scanty to abundant

eosinophilic cytoplasm

Bounded cells with centralnuclei, and clear to eosinophilic

cytoplasm

!pindle shaped cellsset within an

abundant my#oidmatri#

Criteriafor L"#

ny coagulative tumor cellnecrosisIn the absence of

tumor cell necrosis, thediagnosis re/uired diffuse,

moderate to severecytological atypia and a

mitotic inde# of 8$&mf@$&hpf. If the mitoticinde# is D $&mg@$&hpf, thechance of recurrence is low(less than )-%". In theabsence of coagulativetumor cell necrosis andsignificant atypia, a high

mitotic inde# is compatible

with a benign clinicalcourse. mf@hpf E mitoticfigures@high power fields

ny coagulative tumor cellnecrosis In the absence of tumor

cell necrosis, the diagnosisre/uires diffuse, moderate to

severe cytological atypia and amitotic inde# of 8*mf@$&hpf.

ny coagulativetumor cell necrosisIn the absence of

tumor cell necrosis,the diagnosis

re/uires diffuse,moderate to severecytological atypia

and a mitotic inde#of 8*mf@$&hpf.

igure 3# typia is seen...


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Unli0e smooth muscle tumors at other sites, uterine smooth muscletumors are generally not graded. Bather, clinical behavior is defined bythe designation to categories of UL!, leiomyoma, or !TUA. Thedistinction is important since grading UL! based on criteria at otherbody sites is misleading.

!iagnostic +-aluationAatients with abnormal uterine bleeding or a suspicious uterine lesionshould undergo endometrial sampling. Imaging studies and@or clinicalfindings are not specific for UL! versus other uterine tumors. Ultrasounde#amination, magnetic resonance imaging (BI", or computedtomography (CT" do not reliably distinguish between sarcoma,leiomyoma, endometrial cancer, lymphoma, intravenous leiomyomatosis,or adenomyosis.$4

BIs and 2iagnosis

The utility of BI for diagnosis is being addressed in case reports. Contrast resolution insoft tissues (better than ultrasonography" and lac0 of ioniing radiation show greatpromise as an imaging tool to evaluated L!. The findings of atypical degeneration withirregular contours should bring L! into the differential when evaluating leiomyomas (orother pelvic masses".$5-)$1ne study loo0ed at patients (including nine patients withpathologically proven L! and three with !TUA" in order to study the magneticresonance characteristics of non-benign uterine smooth muscle tumors. dditionally,

they analyed twelve cases of benign leiomyomas in which the gynecologists hadsuspected L!. !ie, location, signal intensity, and contrast enhancement of the tumors

were studied on an individual basis. 9ith some e#ceptions, the authors concluded thatmore than *&% of high signal on T)-weighted images and the presence of any smallhigh-signal areas on T$-weighted images with un-enhanced poc0ets were consideredBI suggestive for !TUA! and L!.))

#taging!taging is based on surgical, not clinical findings. ?#tensive local growthis a hallmar0 of UL! and spread of these tumors occur by local,lymphatic, and hematogenous routes (see 6igure )". etastasisfre/uently involves the lung. If the diagnosis of UL! is 0nown

preoperatively, chest imaging is necessary to evaluate for metastaticdisease.

!urgical staging for UL! is the same as for endometrial carcinoma(see6igure +". The surgery includes peritoneal washings for cytology,e#trafascial total abdominal hysterectomy, bilateral salpingo-oophorectomy, removal of enlarged lymph nodes, and biopsy or anysuspicious areas. !ome oncologists recommend omentectomy and pelvicand paraaortic lymph node sampling. !ee 6igure +.

igure : The International ederation of Gynecology
http://en.wikipedia.org/wiki/Endometrial_carcinomahttp://en.wikipedia.org/wiki/Endometrial_carcinoma


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and )bstetrics 0IG) #taging of UL"#

#tage I Tumor confined to corpus uteri

I Tumor limited to the endometriumI3 Tumor invades up to or less than *&% of the myometrium

IC Tumor invades more than *&% of the myometrium

#tage II Tumor in-ades cer-i, but does not e,tend beyond uterusII ?ndocervical glandular involvement only

II3 Cervical stroma invasion

#tage III Local and/or regional spreadIII Tumor involves uterine serosa and@or adne#a (direct e#tension or metastasis"

III3 7aginal involvement (direct e#tension or metastasis"IIIC etastasis to the pelvic and@or para-aortic lymph nodes

#tage I2

I7 Tumor invades the bladder mucosa and@or bowel mucosaI73 2istant metastasis(e#cluding metastasis to vagina, pelvic serosa, or adne#a. Including

metastasis to intra-abdominal lymph nodes other than para-aortic, and@or inguinal lymph nodes"

L"# should be grouped 3ith regard to the degree of differentiation as follo3s:>$ * percent or less of a nons/uamous or nonmorular solid growth

>) ' percent to *& percent of a nons/uamous or nonmorular solid growth> ore than *&% of a nons/uamous or nonmorular solid growth

The importance of lymph node dissection is controversial.)Though theinvolvement of lymph nodes is of prognosticsignificance, lymphadenectomyhas not been shown to be therapeutic.1utcomes have been comparable among similarly staged patients who didor did not undergo lymphadenectomy.)3ased on these results, mostperform lymph node dissection only in patients with clinically suspiciousnodes. Aatients with UL! confined to the uterus have a low ris0 of occultnodal disease ().%".)9hen UL! is diagnosed postoperatively, re-e#ploration for surgical staging is probably unnecessary since this ris0 ofmetastasis to lymph nodes and beyond is minimal.

1varian conservation may be an option for premenopausal women whowish to retain ovarian function. Two studies have suggested that retentionof the ovaries may not adversely affect prognosis in women with !tage IUL!.)Informed consent as to the uncertainty of outcome with

conservative surgery and close follow-up is clearly needed.
http://www.figo.org/http://en.wikipedia.org/wiki/Lymphadenectomyhttp://www.figo.org/http://en.wikipedia.org/wiki/Lymphadenectomy

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