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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5186_Библиотеки_им_академика_М_И_Перельмана.pdf
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- •Contributors
- •Preface
- •Contents
- •Sporadic
- •Hereditary
- •Oncogenes
- •Oncogenes
- •Necrosis
- •Autophagy
- •Apoptosis
- •Angiogenesis
- •Biomarkers
- •Immunotherapy
- •Cytokines
- •Excretion
- •Antimetabolites
- •Fractionation
- •Hyperthermia
- •Brachytherapy
- •Palliation
- •Cervix
- •Vagina
- •Melanoma
- •Vulva
- •Adenofibroma
- •Adenosarcoma
- •Carcinosarcoma
- •Ovary
- •Choriocarcinoma
- •Incidence
- •Prevalence
- •Validity
- •Sensitivity
- •Specificity
- •Cervix

Figure 6.70 Pseudomyxoma peritonei. Cytologically low-grade mucinous epithelium is
presentwithinpoolsofmucinintheovarianstroma (top),peritoneum(middle),andwithinthe
appendix(bottom)inthiscondition.Mostcasesareassociatedwithanappendicealmucinous
neoplasm;rarely,thisconditionisencounteredinmucinousovariantumorsarisinginamature
teratoma.
Figure6.71 Endometrioidadenocarcinomaofovary.Ovarianendometrioidcarcinomahas
similar morphology to endometrial endometrioid carcinoma, including squamous cell
differentiation.
BorderlineTransitionalCell(Brenner)Tumor—TransitionalCellTumorofLow
MalignantPotential
Borderlinetumorsaretypicallyunilateral,solid,andcysticandusuallylarger(10to25
cm) than benign Brenner tumors. Microscopically, they feature coarse papillary fronds
linedbymultilayereduroepitheliumthatresembleslow-gradepapillaryurothelialcarcinoma
oftheurinarytract.Despitetheirepithelialproliferation,thesetumorsareclinicallybenign.
MalignantBrennerTumor
Malignant transitional cell tumors with benign or atypical proliferating transitional
elements are designated as malignant Brenner tumors. These tumors show nuclear
pleomorphism,hyperchromasia,numerousmitoticfigures,anddestructivestromalinvasion.
UndifferentiatedEpithelialTumors

Undifferentiatedcarcinomaslack histologicfeatures ofaspecific mülleriancell type.They
areinvariablyhighgrade.Becauseundifferentiatedareasarecommoninhigh-gradeovarian
carcinomas that contain specific features of serous, clear cell, or other differentiation
elsewhere,pureundifferentiatedcarcinomasareinfrequent(60).
MixedSurfaceEpithelial–StromalTumors
Mixedovarianepithelialtumorshavetwoormoredifferentiatedhistologiccelltypes,eachof
whichaccountforatleast10%ofthetumor.
SexCord–StromalTumors
Sexcord–stromaltumorsdemonstrateovarian,testicular,oramixtureofovarianand
testicularcelldifferentiation(74).Manyofthetumorsofthissubtypevariablyexpress
inhibin, a feature that is often used in confirming the presence of sex cord–stromal
differentiation.
AdultGranulosaCellTumors
Adultgranulosacelltumoristhemostcommonsex cord–stromaltumor inthe ovary.This
tumoroccursinfemalesoverawideagerange(mean52years)butismorecommoninlate
reproductiveyearsthaninthepediatricagegroup.Patientsoftenpresentwithestrogenic
symptoms(61).
Adultgranulosacelltumorsareunilateralandsolid,solidandcystic,orpredominantlycystic.
Microscopically, theyarecharacterized by a proliferation ofovoid,predominantly uniform
cells with an open chromatin pattern and nuclear grooves (Fig. 6.74). Mitotic figures are
present, but typically fewer in number. A variety of patterns can be observed, including
trabecular,insular,diffuse,andmicrofollicular,featuring characteristicCall–Exnerbodies
(smallroundspacesfilledwitheosinophilicmaterial formed by the surrounding granulosa
cells).Macrofollicles are present in most adultgranulosacell tumors. Adult granulosa cell
tumorisaneoplasmoflowmalignantpotential;recurrencesmayoccurmanyyearsafter
initial diagnosis. The most important prognostic feature is the stage of disease. Adult
granulosacelltumorisassociatedwithasomaticmutationinFOXL2(75).

Figure6.72Clearcelladenocarcinomaof ovary.Glands are lined by polygonal cells with
clear cytoplasm and enlarged, hyperchromatic, and pleomorphic nuclei. (top, low power;
bottom,highpower)

Figure 6.73 Brenner tumor of ovary. Nests of transitional epithelium are set in fibrous
stroma.Stromalcalcificationsmayimpartagrittytexture.
JuvenileGranulosaCellTumors
Ninety-sevenpercentofjuvenilegranulosacelltumorsoccurinfemalesyoungerthan30
years.Patientsoftenpresentwithisosexualpseudoprecocityormenstrualirregularities.
Most are unilateral and low stage, with a macroscopic appearance similar to the adult
granulosacelltumor.Theyaredistinguishedfromtheadultvariantbythepresenceoflarger,
more irregular follicles and rounded, more atypical nuclei that are euchromatic or
hyperchromatic and nongrooved (Fig. 6.75). Mitotic figures are often numerous. Most
juvenilegranulosacelltumorsareclinicallybenign,butapproximately10%willdevelop
recurrences,typicallywithinthefirst5years(74).
Sertoli–LeydigCellTumors
Sertoli–Leydig cell tumors occurmost commonly in women in their mid-20s but can
occur in females as young as 2 and as old as 75 years. Approximately one-third of
patientspresentwithvirilization;estrogenicmanifestationsarelessfrequent.Almostone-
halfofpatientsexhibitnoendocrinologicmanifestations(76).
Sertoli–Leydigcelltumorsaretypicallyunilateralandlowstage,but10%mayhaveovarian

surface involvement. Less than 5% exhibit extraovarian spreadat diagnosis. Most are
solid or solid and cystic, and pale yellow or tan in color. The characteristic features are
tubules or cords of Sertoli cells, with interspersed nests of Leydig cells enmeshed in
primitive gonadal stroma (Fig. 6.76). Rarely, a Sertoli-only cell tumor can be seen.
Approximately20% haveheterologouselements, whichmaybe epithelialormesenchymal
and include mucinous, cartilaginous, neuroendocrinologic (carcinoid tumor), or skeletal
muscular(rhabdomyosarcoma) differentiation. Retiformelements resembling rete testis are
seen in 15% of cases. The tumors are graded on the basis of the degree of Sertoli tubule
formationandtheextentofprimitivestroma.Well-differentiatedtumorshaveamitoticindex
oflessthan5mitoticfiguresper10high-powerfields,whereaspoorlydifferentiatedtumors
have a mitotic index greater than 10 mitoses per 10 high-power fields, and intermediate
tumorshaveanintermediatemitoticindex.IntermediateandpoorlydifferentiatedSertoli–
LeydigcelltumorsmaybeassociatedwithDICER1syndrome.
Sex-CordTumorwithAnnularTubules
ArarevariantofSertolicelltumor,thesex-cordtumorwithannulartubules,isdistinguished
by the presence of simple or complex annular tubules composed of Sertoli cells arranged
antipodallyaroundhyalinematerial(Fig.6.77).Tumorsareunilateral,and oftenassociated
withhormonalmanifestations.Upto25%areclinicallymalignant(61).One-thirdoccursin
patientswith Peutz–Jeghers syndrome,when they are clinically benign, bilateral, small,
andoftenincidentalfindings.
Gynandroblastoma
Whensexcord–stromaltumorscontainminorcomponentsofothertypesofsexcord–stromal
tumor,thetumorisusuallydesignatedbythemajorcomponent.However,whenatumoris
composed of an admixture of well-differentiated Sertoli cell tubules and granulosa cell
elements,and thesecond cellpopulation makesup atleast10%ofthe tumor,thetumoris
classified as a gynandroblastoma, and the relative contribution and subtypes are reported.
Mostsuchtumorsarebenign.

Figure 6.74 Adult granulosa cell tumor. Top: Ribbons of cells with coffee-bean nuclei
surround a macrofollicle. The mitotic index is usually low in these tumors. Bottom:
MicrofollicularpatternwithCall-Exnerbodies.

FibromaandThecoma
This group of stromal tumors is composed of spindle or oval cells, with scant cytoplasm
(fibroma)ormoreabundant,pale,lipid-richcytoplasm(thecoma),associated withvarying
degrees of collagen. Estrogenic manifestations are generally absent in fibromas but
occurinasmanyas60%ofpatientswiththecomas.Tumorsinthisgrouptendtooccurin
middleage(fibroma)oraftermenopause(thecoma).Mostareunilateral,solid,orsolidand
microcystic, and they vary from gray or white (fibroma) to bright yellow (thecoma).
Microscopically, the tumors are composed of cells arranged in fascicles or in a storiform
pattern;calcificationandhyalineplaquesmaybeseen(Fig.6.78).Patientswithnevoidbasal
cell carcinoma syndrome develop ovarian fibromas at a younger age, and the tumors are
bilateral,multinodular,andcalcified.Almostallfibromasandthecomasarebenign.Some
fibromaspresentwithascitesand apleural effusion(Meigssyndrome),which resolves
onremovalofthetumor.
Figure6.75Juvenilegranulosacelltumor. Macrofollicles in this tumor are surrounded by
cellswithmorehyperchromaticandoftenmoremitoticallyactivenucleithanthoseintheadult
type.

Figure6.76 Sertoli–Leydigcelltumor.SertolitubuleswithinterspersedLeydigcellsformthis
well-differentiatedtumor.

Figure6.77 Sex-cordtumorwithannulartubules.Prominenthyalinebodiesaresurrounded
by a proliferation of complex annular tubules. This tumor may be associated with Peutz–
Jegherssyndromeandistypicallyincidentalandclinicallybenigninthatsetting.Thosetumors
that are not associated with the syndrome may recur and demonstrate clinically aggressive
behavior.

Figure 6.78 Fibroma–thecoma of ovary. Spindle-shaped cells are dispersed in a variably
fibrousstroma.
SclerosingStromalCellTumors
These stromal tumors occur in young women and are rarely associated with endocrine
manifestations. They are unilateral and clinically benign. Sclerosing stromal tumors are
distinguishedby thepresence ofalternating,relativelyhypercellularand hypocellularareas
of stromal proliferation, arranged in a pseudolobular pattern. An extensive, thin-walled
vascularpatternisoftenpresent.
SteroidCellTumors,NotOtherwiseSpecified
Steroidcelltumorstendtooccurinyoung,reproductive-agedwomen,and25%occurbefore
theageof30years.Mostareconfinedtooneovaryatdiagnosis,butasmanyas20%have
extraovarian spread, and 30% are clinically malignant. Endocrine manifestations, when
present, tend to be androgenic, although estrogenic, progestogenic, and Cushingoid
manifestationsmaybeseen(74).Most are solidand pale yellow ororange, with thecolor
dependingonthesteroidcontent.Microscopically,thetumorsarecomposedofsolidnestsof
uniform,round,orpolygonalcells,withdistinctcellborders,andcentralnucleithatcontain
small, but distinct nucleoli (Fig. 6.79). The cytoplasm may be finely vacuolated, or
eosinophilic and granular.Most tumors are mitotically inactive with fewer than 2 mitotic
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