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OtherVulvarLesions
BartholinCyst
Bartholinductcystisthemostcommon cysticgrowthinthevulva.Itoccursposteriorlyin
the vulvar vestibule. Excision is usually reserved for those lesions that fail to respond to
conservativemanagement.SomeinvestigatorsrecommendexcisionofBartholinglandcysts
toexcludeadenocarcinomawhencystsorabscessesoccurinpatientsmorethan40yearsof
age. The cyst lining can be variable: endocervical-like mucinous, squamous, and
transitionalepitheliumisoftenpresent.Cellularatypia,cellularstratification,andmitotic
figuresareconcerningfeatures,andshouldwarrantfullpathologicexaminationoftheentire
cystwalltoexcludecarcinoma.
BartholinGlandAdenocarcinoma
Anuncommontumor,thisneoplasm affectswomen50years ofageandolder.Theclinical
impression is usually that of a Bartholin duct cyst. Histologic types include
adenocarcinoma,squamouscellcarcinoma,adenoidcysticcarcinoma,andhybridtypes.
AtransitionzoneadjacenttotheBartholinglandconfirmsthediagnosis.Approximately20%
ofpatientshaveipsilateralgroinlymphnodemetastasesatinitialdiagnosis.
AdenoidCysticCarcinoma
Adenoidcysticcarcinomaisaclinicallyaggressiveneoplasm.Itiscomposedofcribriform
nestscontainingeosinophilichyaline materialwithintheglandlumens,resemblingadenoid
cysticcarcinomaofthesalivarygland.Likesalivaryglandadenoidcysticcarcinomas,pure
adenoidcysticcarcinomasarisinginthelowergenitaltracthavebeenfoundtoharborNFIB
generearrangements(35).
FibroepithelialStromalPolyp
Fibroepithelialstromalpolypsoccuronthevulvabutaremostcommonlyseeninthevagina
(36).Theymaybesingleormultiple.Fibroepithelialstromalpolypsprobablyrepresenta
reactivelesionratherthanatrueneoplasm.
HidradenomaPapilliferum
Ararebenignglandularneoplasm,papillaryhidradenomausuallyoccursintheregionofthe
intralabial sulcus in adult women. The tumor is well circumscribed and composed of
complex, branching papillae lined by a double layer of outer myoepithelial and inner
epithelialcells(37).
ExtramammaryPagetDisease
Pagetdiseaseofthe vulvaisanintraepithelialneoplasmcharacterizedbylarge,roundcells
with abundant, pale cytoplasm, often forming small intracytoplasmic lumina or glandlike
structures (38). Vulvar Paget disease may be primary or secondary, and presents
clinically as a red, eczematous lesion. Postmenopausal women are most commonly
affected. Approximately 90% are primary and noninvasive (cutaneous), whereas 10% are
associated with an underlying invasive carcinoma (cutaneous, anorectal, urothelial). All
forms of Paget disease are immunoreactive for cytokeratin and epithelial membrane
antigen. Classic primary cutaneous vulvar Paget disease expresses CK7 and HER2 (Fig.
6.33),whereassecondaryPaget’softenexhibitsanimmunophenotypeoftheprimarysiteof
origin.
MesenchymalTumors
A variety of specialized genital stromal neoplasms occurs in the vulvovaginal region (and
occasionally in the cervix) of reproductive-aged women (38). Most are small, hormonally
responsive,andclinicallyindolent.Themostcommonspecializedgenitalstromaltumorsare
angiomyofibroblastoma, cellular angiofibroma, superficial angiomyxoma, and
superficial myofibroblastoma (39). Often mistaken clinically for a Bartholin gland cyst,
thesespecializedgenitalstromallesionsmayrecurlocallyifincompletelyexcised,butthey
arenotassociatedwithaggressiveclinicalbehavior.
Prepubertal vulvar fibroma is unlikely to be confused with any of the typical vulvar
mesenchymallesionsbecauseofitspredilectionforprepubertalfemales.Thislesion,which
most commonly involves the labia majora, presents as a unilateral or rarely, bilateral, ill
defined, and painless subcutaneous vulvar mass with microscopic features that suggest a
hamartomatousprocess.
Other mesenchymal neoplasms that behave in a clinically benign fashion include
lipoma, neurofibroma, schwannoma, granular cell tumor, glomus tumor, and
hemangioma.
Dermatofibrosarcomaprotuberansisalow-gradecutaneoustumorwithahighriskfor
recurrenceifincompletelyexcised.High-gradesarcomasthatmostcommonlyoccurinthe
vulva include rhabdomyosarcoma, proximal epithelioid sarcoma, alveolar soft part
sarcoma, peripheral primitive neuroectodermal tumor, and postradiation
angiosarcoma.
Rarely, smooth muscle tumors may involve the vulvar region. Criteria for malignancy
differfromthoseforuterinesmooth muscletumorsandarebasedonsize (>5cm), mitotic
index(>5mitoticfiguresper10high-powerfields),infiltrativemargins,andcellularatypia.
AggressiveAngiomyxoma
Aggressive angiomyxoma occurs in the deep vulvar and inguinal soft tissue and is
characterizedbyapaucicellularspindlecellproliferation,separatedbyloosemyxoidstroma
(Fig. 6.34). Mitotic activity is low. Despite the bland histologic appearance, it is an
infiltrativetumorthatmaylocallyinvadedeeppelvicstructuresifincompletelyexcised(37).
Aggressive angiomyxoma occurs most commonly during the third to fifth decades. The
clinicalimpressionfrequentlyincludesBartholinglandcystorhernia;theextentofdiseaseis
oftenunderestimated.
MelanocyticTumors
Malignantmelanomaaccountsfor lessthan10%ofallvulvarmalignanciesandconsistsof
threetypes:mucosaloracrallentiginous,nodular,andsuperficialspreading.Upto25%
of tumors are unclassified. Vulvar melanoma occurs more commonly in elderly, white
women,andtypicallypresentsasanodularmassthatmaybepigmented;satellitelesionsare
common.MelanomasexpressS-100protein,HMB-45,andMelanA(Fig.6.35).Clarklevels
andBreslowthicknessshouldbereportedforallvulvarmelanomas.
Figure 6.33 Paget disease of vulva. Top: Paget cells are confined to the intraepidermal
compartment,formingsmallgland-likestructures.Bottom:ThePagetcellsexpresscytokeratin
7.
Benign,atypical,anddysplasticnevioccurinthevulva.Benignnevimay becongenital or
acquired. Atypical vulvar nevi (atypical melanocytic nevi of the genital type) occur
primarilyinyoung,reproductive-agedwomenandare characterizedbyatypical,superficial
melanocytes,and variably sized junctional melanocytic nests. They are distinguished from
melanomas on the basis of small size, circumscription, absence of pagetoid spread,
significantcytologicatypia,andmitoticactivityinthedeeperdermalmelanocytes.Atypical
vulvarnevimayappearmoreatypicalduringpregnancy.Theyarenotassociatedwith
dysplasticnevielsewhere.
Figure 6.34 Aggressive angiomyxoma of vulva. Paucicellular spindle cell proliferation
separatedbyloose myxoid stroma.Despitetheblandappearance,thisisaninfiltrativetumor
withapropensityforrecurrenceifincompletelyexcised.
Dysplasticnevioccurpredominantlyinyoungerwomenandexhibitan irregularborder;
microscopically, clusters of atypical spindled and epithelioid nevus cells with prominent
nucleoliandnuclearpleomorphismareseen.Unlikeatypicalvulvarnevi,dysplasticvulvar
nevimaybeassociatedwithdysplasticnevielsewhereonthetrunkandextremities.
UncommonNeoplasms
Other tumors that occur in the vulva include cutaneous adnexal tumors, tumors that arise
fromspecializedanogenitalmammary-likeglands,andtumorsofminorvestibularandSkene
glandorigin(37).
UterineCorpus
EndometrialNeoplasms
Theendometrial morphologicchanges withwhich thegynecologiconcologist isconcerned
are limited, and chiefly involve endometrial carcinoma and its precursors. This section
focusesontheseproliferations,andthosethatfigureintheirdifferentialdiagnosis.
In discussing endometrial nonsecretory proliferations, it is helpful to realize that the
endometriumcangiverisetoavarietyofepithelialphenotypesthataremorecommonly
encountered in other parts of the müllerian-derived system: the ovary, the fallopian
tubes, and the endocervix. The term metaplasia is used for benign epithelial
proliferations of this type, and special variant carcinoma is used for the malignant
patterns. Endometrial epithelial proliferations, whether benign or malignant, typically
featuremixturesofthesedifferentiatedepithelialtypes.Thecarcinomaprecursorsfeaturethis
mixed epithelial phenotype in varying degrees, hence the full designation
hyperplasia/metaplasia,whichistobeunderstoodwhenhyperplasiaisusedunmodified.
EndometrialHyperplasia/Metaplasia
The term endometrial hyperplasia denotes a proliferating endometrium featuring
glandulararchitectural abnormalities that result in glandularcrowding and take the
form of either cystic dilatation of glands (simple hyperplasia) or glandular budding
(complexhyperplasia). The current WHO taxonomy stratifies hyperplastic endometria on
thebasisoftheircytologicfeaturesintoatypicalendometrialhyperplasiaandnonatypical
endometrialhyperplasia,thelattertermimplyingthatsignificantcytologicatypiaisabsent.
Theriskposedby hyperplasiaforthesubsequent developmentofendometrialcarcinomais
roughlycorrelated withthe degree ofcytologic atypiapresent.The assessmentof atypia is
subjecttoobserverdisagreement(Table6.6;Figs.6.36and6.37).
Figure 6.35 Vulvar melanoma. Top: Nests of epithelioid cells extensively replace normal
vulvartissue.Bottom:StrongexpressionofS100protein.
DifferentialDiagnosis
AtrophicorWeaklyProliferativeEndometriumwiththeArchitectureof
Hyperplasia
Whenacomplexhyperplasia is the last unshed endometrium of a postmenopausal woman
andthe epitheliumsubsequently becomesatrophic inthewakeofestrogen withdrawal,the
patternoftenmimicshyperplasia.Confusionisavoidedwhenitisnotedthattheepitheliumis
atrophicandnotproliferating.
Well-DifferentiatedAdenocarcinoma
Thisisthechiefdifferentialdiagnosticconsiderationandexhibitsoneofthehighestlevelsof
expertdisagreementingynecologicpathology.Thedifferentialdiagnosisisdiscussedbelow.
Table6.6FeaturesofEndometrialHyperplasias
HyperplasiaWithoutAtypia AtypicalHyperplasia
a
Histology Increasednumberofroundglands,which
maybecysticallydilated(“Swiss
cheese”).
Theglandsarecloselypackedandhave
irregularcontours;littlestromaremains
betweenglands.Nocytologicatypia.
a
Cytologicatypia(nuclearpleomorphism,
lossofpolarity,prominentnucleoli).
Architecturemaybeeithersimpleor
(morecommonly)complex.
Clinical Perimenopausalandpostmenopausal
women
Postmenopausal
Malignant
potential
5–10% ≥30%
a
Mostatypicalhyperplasiaarecomplex.
EndometrialCarcinoma
HistologicTypes
Carcinomasmaybeclassifiedintermsoftheirdifferentiatedhistopathologicfeatures(Table
6.7). The endometrium gives rise to a variety of differentiated carcinomas, but more than
80%areglandularneoplasmsthatresembletheepitheliumfoundinendometrialhyperplasia.
Squamous or squamoid (morular) differentiation is commonly encountered in this
endometrioid or usual adenocarcinoma. The term endometrioid is used to denote this
histologic pattern, and to distinguish it from endometrial, which is the generic term for
carcinomasthat originatedanatomically intheendometrium. Othermüllerian-differentiated
types (e.g., serous, clear cell, mucinous) make up the remainder of the endometrial
carcinomas.
Endometrial carcinomas may be grouped with an eye to the hormonal (and associated
epidemiologic) background in which they arise: hyperestrogenic settings (type I) or
hypoestrogenicsettings(typeII).
Patients in the first group (type I) tend to be between 40 and 60 years of age, although
carcinomacan developin youngerwomen, including,rarely,those intheir 20s.Theymay
have a history of chronic anovulation or estrogen hormone-replacement therapy, and
the carcinomas are usually well-differentiated,stage I, non-myoinvasive tumors associated
withendometrial hyperplasia/metaplasia. Thelatter may befoundeither concurrently orin
previousendometrialsamplings.MostofthetumorsareER-positiveandPR-positiveand
p53-negativeandexpresslowlevelsoftheproliferationantigenKi-67.Patientsinthisfirst
grouphaveaveryfavorableprognosisafterhysterectomy.
Figure6.36 Simpleendometrialhyperplasiawithoutatypia.Anincreasednumberofround
glandsisseen,someofwhicharedilated.Thereisnocytologicatypia.
Figure6.37 Complexatypicalhyperplasia.Crowded,irregularglandsshowlittleintervening
stroma. The glands show rounded, pleomorphic nuclei with prominent nucleoli. (top, low
power;bottom,highpower)
Table6.7ClassificationofEndometrialCarcinoma
EndometrioidCarcinoma(Usual)
a. Withsecretoryfeatures
b. Withvilloglandularfeatures
c. Withsquamousfeatures(includesadenoacanthomaandadenosquamouscarcinomas—seetext)
OtherCarcinomas
a. Serous
b. Clearcell
c. Puremucinous
d. Puresquamouscell
e. Mixed
f. Undifferentiated
g. Dedifferentiated
h. Neuroendocrine