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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5186_Библиотеки_им_академика_М_И_Перельмана.pdf
figuresper10high-powerfields;tumorswithahighmitoticindexmaybemoreaggressive.
LeydigCellTumorsandStromalLuteomas
Steroidcelltumors,nototherwisespecified,mustbedistinguishedfromLeydigcelltumors
and stromal luteomas, both of which tend to exhibit a benign clinical course. Leydig cell
tumors are recognized by the presence of Reinke crystals. They are either small and
typicallyhilarinlocation,orlargeandreplacingmostoftheovarianparenchyma.Stromal
luteomasaretypically small(lessthan3cm), well-circumscribedtumorsthatoccurwithin
theovarianstroma.Asizecriterionof1cmhasbeenimposedtodistinguishLeydigcell
tumors and stromal luteomas from benign, nonneoplastic ovarian steroid cell
proliferations.
GermCellTumors
These tumors are derived from the primordial germ cells of the ovary.Most are mature
cysticteratomasandareclinicallybenign.Theremaininggermcelltumorsaremalignant;
mostoccurinchildrenoradolescentgirls(74).
MatureCysticTeratoma
Matureteratomasaretypicallycystic,althoughsolidvariantsdooccur.Theyhaveawideage
range,occurringinfemalesfrom 2to80years(mean32years).Upto15%arebilateralat
presentation. Mature teratomas are one of the most common ovarian neoplasms,
accountingfor30–45%ofallovariantumors,andasmanyas60%ofallbenignovarian
tumors. Recurrences may appear in the residual ipsilateral ovary following cystectomy,
particularlywhenthetumorsaremultipleorruptured.Thepresenceofmatureglialimplants
intheperitoneum(grade0implants)doesnotadverselyaffectprognosis(61)(Fig.6.80).
Figure6.79 Steroidcelltumorofovary.Nestsofpolygonalcellswithcentralroundednuclei
mayexhibitfinelyvacuolated,eosinophilic,or,lesscommonly,opticallyclearcytoplasm.
Figure6.80 Matureteratoma.Cysticneoplasmcontainsteeth,hair,andsebaceousmaterial.
Monodermal mature teratomas are not uncommon in the ovary,and include struma ovarii
(thyroid),carcinoid,strumalcarcinoid,ependymoma,andprimitiveneuroectodermaltumor.
Thedevelopmentofasecondarysomaticcarcinomamayrarelyoccurinmatureteratomasin
postmenopausal women. Squamous carcinoma and adenocarcinoma, usually of intestinal
type,accountformostcasesofsecondarycarcinoma.Secondarysarcomasarelesscommon
andtendtooccurinyoungerpatients.
ImmatureTeratoma
Immature teratomas are distinguished from mature teratomas by the presence of variable
amounts of immature embryonal tissue, typically in the form of immature
neuroectodermal tissue (Fig. 6.81). Prognosis is dependent on the grade and stage of
disease.Tumorsaregradedonthebasisoftheamountofimmaturetissuepresent.Grading
hasbeentraditionallybasedonathree-tieredsystem,althoughatwo-tieredsystemmay
be more reproducible. Treatment of immature teratomas has evolved. Surgery alone is
considered curative in children and adolescent patients regardless of the grade.
Chemotherapyisusedforpatientswhorelapse.
Dysgerminoma
Dysgerminoma is identical to its testicular counterpart, the seminoma. The ovarian
tumorsareunilateralin80%ofpatients,large(meandiameter15cm),solid,andtanin
appearance;cystformationisseeninareasofinfarction.Mostoccurinthesecondand
thirddecades,although5%presentinchildrenlessthan5yearsofage.Thetumorsare
composedofadiffuseproliferationofroundedcellswithdiscretecellmembranesandcentral
nuclei, with one to four prominent nucleoli (Fig. 6.82). Lymphocytes and granulomas are
often present. Some tumors contain syncytiotrophoblastic cells, which may be associated
with elevated serum beta-human chorionic gonadotropin. The neoplastic cells express
placental alkaline phosphatase, CD117, and OCT 3-4. Calcifications should prompt
considerationforthepresenceofconcomitantgonadoblastoma(61).
Figure 6.81 Immature teratoma. Top: Teratomas are graded on the amount of immature
tissue, most commonly manifested by immature neural tissue, that is present. Bottom: In
contrast, the presence of mature glial tissue does not affect prognosis, even when it forms
nodulardepositsthroughouttheperitoneum(gliomatosis).
Figure6.82 Dysgerminoma.Topleft:Solid,paletanlobulatedgrowthpatternischaracteristic
of dysgerminoma. Top right and bottom: The tumor is composed of sheets of ovoid to
polygonal cells with clear cytoplasm, prominentcell borders, andcentral nuclei with multiple
smallnucleoli.Interspersedmaturelymphocytesarecharacteristic.
Figure 6.83 Yolksac tumor of ovary. Top: Microcystic reticular pattern of yolk sac tumor.
Bottom:ThesetumorsexpressAFP(left)andglypican-3,aswellasSALL4(right).
YolkSacTumor
Yolk sac tumor (endodermal sinus tumor) occurs in females from as young as 17
monthsto43years(mean20years).Thetumorisusuallyunilateral,solid,andcysticwith
areas of hemorrhage and necrosis. Areticularor tubulocystic pattern with Schiller–Duval
bodies is characteristic, but microcystic, macrocystic, solid, and glandular patterns can be
seen(Fig.6.83).Yolksactumorsareassociatedwithelevatedserumalphafetoprotein(AFP)
titers,andthetumorsexpressAFP,cytokeratin,glypican-3,andSALL4(77).
Gonadoblastoma
This tumor is composed of dysgerminoma cells admixed with sex-cord derivatives
resembling Sertoli or granulosa cells. Gonadoblastoma is typically diagnosed in
childrenoryoungadults.Mostarebilateral,butthismaynotbemacroscopicallyapparent.
Calcificationswithinhyalinizedbodiesofthesex-cordcomponentareseeninmorethan80%
of cases. Almost all gonadoblastomas are associated with an underlying gonadal
disorder,eitherpureormixeddysgenesis,withaYchromosomebeingdetected.
Figure6.84 Smallcellcarcinoma,hypercalcemictypeofovary.Sheetsofimmaturesmall
cells with high mitotic index are punctuated by follicle-like spaces containing eosinophilic
material.
EmbryonalCarcinoma
Pure embryonal carcinoma is rare in the ovary but may be admixed with other germ cell
tumors;thisappearstobeparticularlycommoningonadoblastomas.Embryonalcarcinomas
expresscytokeratin,CD117,OCT3-4,andCD30.
MiscellaneousOvarianTumors
Theovarygivesrisetoavarietyofotherbenignandmalignanttumorsthatdonoteasilysort
into one or another of the major ovarian tumor categories. Most of these tumors are
extremely rare and include such diverse entities as paraganglioma; myxoma; small cell
carcinoma, hypercalcemic type; microcystic stromal tumor, small cell carcinoma,
pulmonarytype;andlarge-cellneuroendocrinecarcinoma,amongothers(78).Onlysmall
cellcarcinoma,hypercalcemictype,occurswithsufficientfrequencytowarrantdiscussionin
thischapter.
SmallCellCarcinoma,HypercalcemicType
This is an uncommon, highly malignant tumor presenting in young women, often in
association with paraneoplastic hypercalcemia. The tumors are usually large and
unilateral,eveninthepresenceofadvancedstagedisease.Approximately50%oftumorsare
confinedtotheovaryatpresentation,andthesetumorsappeartohaveabetterprognosisthan
tumors with extraovarian spread. Small cell carcinoma, hypercalcemic type is typically
composedofsmall,undifferentiated,and mitoticallyactivecells(Fig.6.84),althoughlarge
cells may be present and, in some cases, form the predominant cell type. The tumor cells
growinsolidsheetspunctuatedbyvariablysizedfollicle-likespaces. Mucinousepithelium
maybeseeninasmanyas15%ofcases(67).ThesetumorsharbormutationsinSMARCA4.
Smallcellcarcinoma,hypercalcemictypeshouldnotbeconfusedwithsmallcellcarcinoma,
pulmonarytype.Thelattertumoroccursinpostmenopausalwomenandishistologicallyand
immunohistologicallysimilartosmallcellneuroendocrinecarcinomaofthelung.
SecondaryTumorsoftheOvary—Metastases
Tumors secondarily involving the ovary include carcinoma, lymphoma or leukemia,
melanoma,andsarcoma.The tubular gastrointestinal tract, particularly the colon, is the
most common source of metastatic carcinoma (Fig. 6.85), followed by the breast and
pancreatobiliarytract. Tumorsarisinginany site may secondarily spread to the ovary;the
relativefrequencyoftheprimarysitevariesindifferentcountries,dependingontherelative
incidence of various types of cancer, and on changing patterns in the treatment of these
cancers. The classic Krukenberg tumor refers to metastatic signet-ring carcinoma
involvingthe ovaries (Fig.6.86), which typically arisesinthe stomach, appendix, orlarge
bowel.Avarietyoffeatures maysuggestanovarianmetastasis, includingbilateraldisease,
surfacenodules,extensivelymphaticinvolvement,anddiametersmallerthan10cm.
NonneoplasticLesionsoftheOvary
Manynonneoplasticlesionsoftheovarymaymimicanovarianneoplasm.Mostoccurduring
thereproductiveyears.Someareassociatedwithinfertility.Theseincludecystsoffollicular
origin,massive ovarianedema,stromal hyperplasiaandhyperthecosis, endometriosis,
andavarietyofpregnancy-associatedchanges(79).