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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5186_Библиотеки_им_академика_М_И_Перельмана.pdf
nodules of histologically benign smooth muscle in the omentum and peritoneum, often
numberinginthe tensto hundreds.Thenodulesareusually small,firm, graytowhite,and
covertheperitonealsurfaces,clinicallysimulatingadisseminatedmalignancy.Thiscondition
typicallyoccursduringthereproductiveyears,andmanypatientsarepregnantatthetimeof
diagnosis.Despitethealarmingappearance,disseminatedperitonealleiomyomatosisis
usuallyassociatedwithanindolentclinicalcourse,andcanbetreatedconservativelywith
long-termfollow-up.
UterineTumorResemblingOvarianSexCord–StromalTumor
Mesenchymal neoplasms that resemble ovarian sex cord tumors are classified as uterine
tumors resembling ovarian sex cord tumors provided there is no recognizable endometrial
stromalcomponent(49).Mostarewell-circumscribed;cytologicatypiaisabsentandmitoses
are rare. These tumors are often immunoreactive for sex cord–stromal markers such as
inhibinand/orcalretinin;theyexpresskeratinand/ordesmininsomecases.Mosttumorsof
thisgrouphaveabenignclinicalcourse.
Figure6.52Uterinemyxoidleiomyosarcoma. Thisrarevariant of uterine leiomyosarcoma
may exhibit clinically aggressive behavior in the absence of significant mitotic activity (>2
mitotic figures per 50 high-power fields). The presence of cytologic atypia and tumor cell
necrosisdistinguishesthis infiltrative lesionfrommyxoidleiomyoma.(top, low power;bottom,
highpower)
EndometrialStromalTumors
Neoplasms in the endometrial stromal group resemble the stroma of normal proliferative
phaseendometrium. In the uterus,theyare divided into twomaincategories: endometrial
stromalnoduleandendometrialstromalsarcoma.Botharecomposedofamonomorphous
populationofovoidtospindledcellspossessingscantycytoplasmandsmall,bland,uniform
nucleiwithevenlydistributedchromatin.Thesecellsareembeddedinanabundantreticulin
framework that contains a highly characteristic, delicate, arborizing vasculature. Focal
hyaline thickening of the vessel walls and collagen bands may be present. Endometrial
stromal nodules are clinically benign, whereas endometrial stromal sarcomas may recur,
sometimesmanyyearsaftertheprimarytumorhasbeenremoved.Thesetumorsoftenharbor
JAZF1/SUZ12genefusions.
EndometrialStromalNodule
Endometrial stromal nodules are well circumscribed, usually small and intramural (Fig.
6.53).Infiltrationofthemyometriumoruterinevasculatureis absent.Because diagnosisis
basedoncompletecircumscriptionandabsenceoflymphovascularinvasion,the distinction
between stromal nodule and stromal sarcoma can usually be made only at the time of
hysterectomy.
Low-GradeEndometrialStromalSarcoma
Endometrial stromal sarcoma (low-grade) accounts for less than 20% of all uterine
sarcomas, occurs almost exclusively in adults, and has a peak incidence in the fifth
decade;morethanthree-quartersofwomenarepremenopausal.Thereisnoassociation
with previous irradiation, and nor do patients share the risk profile of patients with
endometrial carcinoma. Low-grade endometrial stromal sarcoma is an indolent neoplasm
with a protracted clinical course. At the time of clinical presentation, most tumors are
confined to the uterus. Extrauterine disease is associated with a higher risk for
recurrencebutiscompatiblewithlong-termsurvival.
Patientscometoclinicalattentionbecauseofamassthatmaybeassociatedwithabdominal
pain or uterine bleeding. When advanced, the uterus is asymmetrically enlarged by a
typicallyyellowortantumormassthatinfiltratesthesurroundingnormalmyometrium,often
extendingintotheendometrialcavityasapolypoidgrowth.Theneoplasmisdistinguished
from endometrial stromal nodule by (i) the presence of infiltrating margins or (ii)
vascular invasion (Fig. 6.54). In most cases, mitotic figures are difficult to find, but
occasionaltumorshaveinexcessof10mitoticfiguresper10high-powerfields.Althoughit
hasbeentraditionaltostratifylow-gradeendometrialstromalsarcomabasedonthemitotic
index, this classification has relatively little utility in diagnostic practice and patient
management (51). Low-grade endometrial stromal sarcoma should be distinguished
from “high-grade endometrial stromal sarcoma” and “undifferentiated uterine
sarcoma.”
Figure 6.53 Endometrial stromal nodule. Endometrial stromal tumors are composed of
small,uniformcells with scantcytoplasmsimilartostromal cellsinnormalproliferativephase
endometrium. When an endometrial stromal tumor is well circumscribed and there is no
lymphovascularinvasion,itisclinicallybenignandclassifiedasanendometrialstromalnodule.
Figure6.54Low-gradeendometrialstromalsarcoma. The presence ofinfiltrativemargins
andlymphovascularinvasiondistinguish low-grade endometrialstromalsarcomafrombenign
stromalnodule.(top,lowpower;bottom,highpower)
High-GradeEndometrialStromalSarcoma
Thetermhigh-gradeendometrialstromalsarcomahasbeenreintroducedtoidentifya
subset of uterine mesenchymal neoplasms that demonstrate histologic and
immunohistologic evidence of endometrial stromal cell differentiation, but possess
cellularity, cytologic atypia, and a mitotic index beyond that seen in the usual low-grade
endometrialstromalsarcoma(52).OnetypeharborsaYWHAE-NUTM2 genefusion andis
readilyrecognizableasmalignantbutdoesnotdemonstratethepleomorphismandanaplasia
seen in undifferentiated uterine sarcomas. Histologically, this tumor is characterized by a
high-grade round to epithelioid cell component that is strongly and diffusely cyclin D1positive, but CD10-negative. Expression of ER and PR is generally diminished in
comparisontotheusuallow-gradeendometrialstromalsarcoma.Anothertypeofhigh-grade
endometrialstromalsarcomaharborsZC3H7B-BCORgenefusion.Thislattertypeexhibitsa
myxoid matrix and uniformly cellular fascicles of spindle cells with a high mitotic index.
This tumor expresses CD10, cyclin D1, and BCOR. Both tumor types exhibit a more
aggressiveclinicalcoursethanlow-gradeendometrialstromalsarcoma(53).
UndifferentiatedUterineSarcoma
Undifferentiateduterinesarcomasaremuchlesscommonthanlow-gradeendometrial
stromalsarcomas.Theyareeasilyrecognizedascytologicallymalignant,andarecomposed
ofhighlycellular,oftenpleomorphic,undifferentiatedroundedtospindledcellswithahigh
mitotic index (Fig. 6.55). Most resemble the undifferentiated malignant stroma often
encounteredincarcinosarcomas.Undifferentiateduterinesarcomas,insharpcontrastto
endometrial stromal sarcomas, are aggressive neoplasms with a high incidence of
metastases.
OtherUterineMesenchymalTumors
Inflammatory myofibroblastic tumors and uterine perivascular epithelioid neoplasms have
uncertain clinical behavior, i.e., some are clinically benign while others, less commonly,
recur.
InflammatoryMyofibroblasticTumor
Inflammatory myofibroblastic tumors may arise in the uterus. These tumors are
composed of stellate spindle cells in a myxoid matrix with occasional ganglion-like cells.
Thereisoftenanadmixedlymphoplasmacellular,neutrophilic,oreosinophilicinflammatory
infiltrate.Thesetumorsmayexpresssmoothmuscleorendometrialstromalmarkers,butthey
are identified by the presence of expression of ALK on immunohistochemistry and ALK
fusionsonfluorescenceinsituhybridization(54).
UterinePerivascularEpithelioidNeoplasm
Uterine perivascular epithelioid neoplasm is characterized by spindled cells and cells
arranged in short fascicles or cell nests, prominent intrinsic vasculature ranging from
capillarynetwork tothick-walled, largecalibervessels,and clearto eosinophiliccells with
granularcytoplasm.Thestromamaybehyalinized. Most express HMB45. Although most
are sporadic, occasional cases, especially those that exhibit features of
lymphangioleiomyomatosis,maybeassociatedwithtuberoussclerosiscomplex(55).
MixedMüllerianNeoplasms
Mixed müllerian neoplasms are biphasic, epithelial–mesenchymal proliferations that
exhibita range of clinicalbehaviorsfrom benigntohighlymalignant. Adenofibroma,
adenomyoma, and atypical polypoid adenomyoma are the common biphasic epithelial–
mesenchymal lesions at the benign end of the spectrum, whereas adenosarcoma and
carcinosarcomarepresentthemalignantendofthespectrum(Table6.9).
Figure 6.55 Undifferentiated uterine sarcoma. Mesenchymal tumors showing marked
cellularity, cytologic atypia, and high mitotic index are classified as undifferentiated uterine
Table6.9MixedMüllerianNeoplasms
EpithelialElements
Mesenchymal
Elements Benign Malignant
Benign Adenomyoma
Adenofibroma
Atypicalpolypoidadenomyoma
Malignant Adenosarcoma
Endometrialstromalsarcomawith
glandularelements
Carcinosarcoma
Homologous
Heterologous
Adenofibroma
Adenofibromas are clinically benign with little risk for recurrence after they are
completely excised. They typically have broad, fibrotic to mildly cellular stroma, with
interveningcleft-likeepithelial-linedsurfacesmorphologicallysimilartophyllodestumorof
thebreast(Fig.6.56). Authoritiesdifferon the appropriatemitotic indexfor distinguishing
adenofibroma from adenosarcoma. Zaloudek and Norris (56) advocate a threshold of 4
mitotic figures per 10 high-power fields, whereas Clement and Scully (57) suggest a
threshold of 2 mitotic figures per 10 high-power fields. In most instances, the 4 mitotic
figuresper10high-powerfieldscriterionissufficienttodiagnoseadenosarcoma,buttumors
withparticularly cellular stroma or borderlinemitoticcounts are best regardedasbeingof
uncertain malignant potential, particularly if subepithelial condensation is present (see
Adenosarcomabelow).Manypathologistsbelievethatadenofibromaisnotadistinctentity;
insteaditisconsideredtorepresentoneendofamixedglandular-stromalspectruminwhich
adenosarcomawithsarcomatous overgrowthrepresentstheoppositeend.Uterine curettings
exhibiting features of adenofibroma may occasionally be associated with an intrauterine
tumormorecloselyaffiliatedtoadenosarcoma(andviceversa).
AtypicalPolypoidAdenomyoma
Atypical polypoid adenomyoma is a polypoid endometrial proliferation composed of
irregular glands set in a stroma composed of smooth muscle or, more commonly,smooth
muscle and fibrous tissue (Fig. 6.57). Morular or squamous metaplasia is present in most
casesandis oftenflorid.The endometrialsamplingstypicallyconsistoflargefragments or
chunks of tissue simulating carcinoma. The condition occurs in premenopausal or
perimenopausalwomen;aclinicalhistoryofinfertilityisnotuncommon.Theselesionscan
recur locally, but do not have metastatic potential. Reproductive conservation utilizing
proceduresshort ofhysterectomyis warrantedfor the conventionalAPA, providedthere is
regularfollow-up(58).
Figure 6.56 Adenofibroma of the uterus. Irregular, clefted glands are surrounded by
prominentpaucicellularandmitoticallyinactivestroma.
Figure6.57 Atypical polypoid adenomyoma. This polyp typically occurs in the lower uterine
segmentandiscomposedofcomplexendometrioidglandswithsquamousmetaplasiasetina
fibromuscularstroma.(top,lowpower;bottom,highpower)
Adenosarcoma
Adenosarcomais an uncommon, predominantly low-grade malignant biphasic tumor
thatiscomposedofbenignepithelialelementsandsarcomatousstroma.Mostpatientswith
uterinecorpusadenosarcomaarepostmenopausal.
Uterine corpus adenosarcoma typically presents as a polypoid growth that protrudes
throughthecervicalosorappearstoarisefromthecervixorloweruterinesegment;often
thereisahistoryofrecurrentpolyps,whichinretrospectmayrepresentearlyorsubtleforms
ofadenosarcoma.Microscopically,the tumorconsists ofuniformly distributed,oftencystic