ABSTRACT

Tumors of the vulva 368 Epithelial tumors: malignant 368

Basal cell carcinoma 368 Sebaceous carcinoma 369 Squamous carinoma and vulval intraepithelial neoplasia (VIN) 369

Skin appendage tumors 374 Bartholin gland adenomas 374 Bartholin gland carcinoma 374 Bartholin gland nodular hyperplasia 374 Extramammary Paget’s disease 375

Melanocytic tumors 377 Malignant melanoma 377 Special site nevi 377

Mesenchymal tumors 377 Aggressive angiomyxoma 377 Angiomyofibroblastoma 378 Cellular angiofibroma 379 Sarcomas 380 Smooth muscle neoplasms 380

Miscellaneous tumors 381 Adenosis 381 Ectopic breast tissue and neoplasms 381 Unusual vulval tumors 382

Tumors of the vagina 382 Epithelial lesions, benign 382

Vaginal adenosis 382

Epithelial lesions, malignant 382 Squamous cell carcinoma and vaginal intraepithelial

neoplasia (VAIN) 383

Melanocytic lesions 383 Malignant melanoma 383 Melanosis 383

Mesenchymal tumors 383 Fibroepithelial stromal polyps of the lower female

genital tract 383 Sarcomas 385

Mixed epithelial and mesenchymal tumors 386 Mixed tumor of the vagina (spindle cell epithelioma) 386 Mullerian (mesonephric) papilloma 387

Secondary metastasis 387

Tumors of the uterine cervix 388 Epithelial tumors: glandular lesions, benign 388

Ectopic prostatic tissue 388 Endocervical tunnel clusters 388 Glandular hyperplasia, diffuse laminar 389 Glandular hyperplasia, lobular endocervical 389 Mesonephric remnants and mesonephric hyperplasia

of the uterine cervix 390 Microglandular hyperplasia of the cervix 391 Prolapsed Fallopian tube 392

Epithelial tumors: glandular lesions, malignant 393 Adenocarcinoma, common variants 393

Microinvasive (early invasive) 393 Mucinous endocervical adenocarcinoma 393 Mucinous intestinal adenocarcinoma 393

Colloid or signet ring cell carcinoma 393 Endometrioid adenocarcinoma 394

Adenocarcinoma, less common variants 394 Clear cell mesonephroid 394 Serous papillary adenocarcinoma 394 Villoglandular papillary adenocarcinoma 394 Minimal deviation adenocarcinoma 395

Adenocarcinoma, rare variants 396 Adenoid cystic carcinoma 396 Adenosquamous carcinoma 396 Basaloid carcinoma of the uterine cervix

(adenoid basal carcinoma) 396 Glassy cell carcinoma 398 Malignant mixed mullerian tumor

(carcinosarcoma/sarcomatoid carcinoma) 398 Mesonephric adenocarcinomas 398

Cervical intraepithelial glandular neoplasia (CGIN) 399

Epithelial tumors: squamous lesions, benign 402 Condyloma acuminatum 402

Epithelial tumors: squamous lesions, malignant 402 Cervical intraepithelial neoplasia (CIN) 402 Microinvasive squamous carcinoma (MICA) 405 Squamous carcinoma 406

Highly differentiated keratinizing squamous cell cancer of the cervix 408

Lymphoepithelial carcinoma 408 Papillary squamotransitional cell carcinoma 408 Rapidly prognesive carcinoma 409 Squamous carcinoma of the uterine cervix with

CINIII-like growth pattern 409

Mesenchymal tumors 409 Sarcomas 409

Miscellaneous tumors 410 Malignant lymphoma/leukemia and lymphoma-like lesion 410 Melanocytic lesions 410 Mixed epithelial and mesenchymal tumors 410

Adenomyomas 410 Mixed mullerian tumors, malignant (sarcomatoid

carcinoma/carcinosarcoma) 410 Mixed mullerian tumors, mullerian adenofibroma 410 Mixed mullerian tumors, mullerian adenosarcoma 410

Neuroendocrine tumors 410 Secondary tumors 411 Unusual primary tumors 411

Extrarenal Wilms’ tumor 411 Primitive neuroectodermal tumor 411 Teratoma 411 Yolk sac tumor 411

WHO classification of cervical tumors 411

Tumors of the uterine corpus 412 Epithelial lesions, benign 412

Endometrial hyperplasia 412 Endometrial hyperplasia, papillary 413 Endometrial metaplasia 414 Endometrial polyp 415

Epithelial lesions, malignant 416 Endometrial carcinoma 416

Endometrioid-type carcinoma 420 Adenocarcinoma with squamous differentiation 421

Variants of endometrioid adenocarcinoma Villoglandular adenocarcinoma 421 Secretory adenocarcinoma 421 Ciliated cell carcinoma 421 Endometrioid carcinoma with sertoliform

differentiation 421 Other endometrial carcinoma variants

Uterine serous papillary carcinoma 421 Clear cell carcinoma 421 Mucinous carcinoma 421 Pure squamous cell carcinoma 421 Undifferentiated carcinoma 421 Mixed type carcinoma 421

Rare types of endometrial carcinoma Endometrial small cell neuroendocrine carcinoma 421 Microglandular adenocarcinoma 421 Signet ring cell carcinoma 422 Transitional cell carcinoma 422 Glassy cell carcinoma 422 Mucinous adenocarcinoma of intestinal type 422 Lymphoepithelial-like carcinoma 422 Giant cell carcinoma of the endometrium 422 Endometrial adenocarcinoma with trophoblastic

differentiation 422 Verrucous carcinoma 422 Minimal deviation endometrioid adenocarcinoma 422 Adenocarcinomas arising from adenomyosis uteri 422 Metastatic tumors 422

Endometrial intraepithelial carcinoma 423

Mesenchymal tumors 424 Endometrial stromal tumors 424

Endometrial stromal nodule (ESN) 425 Endometrial stromal sarcoma (ESS; previously termed

low-grade endometrial stromal sarcoma) 425 Mixed endometrial stromal and smooth muscle tumors 429 Undifferentiated uterine sarcoma 431

Perivascular epithelioid cell tumor (‘PEComa’) 431 Smooth muscle tumors, leiomyomas 431

Classic leiomyomas 432 Cellular leiomyoma 432 Hemorrhagic cellular (apoplectic) leiomyoma 433 Epithelioid leiomyoma 433 Symplastic (bizarre) leiomyoma 436 Mitotically active leiomyoma 436 Myxoid leiomyomas 436 Lipoleiomyoma 436 Vascular leiomyoma 436 Leiomyoma with haematopoietic elements 436 Leiomyoma with sex cord-like pattern 436 Granular cell leiomyoma 436 Diffuse uterine leiomyomatosis 436 Intravenous leiomyomatosis 436 Benign metastasizing leiomyoma 436 Disseminated peritoneal leiomyomatosis 437 Perinodular hydropic leiomyoma 437 Multinodular hydropic leiomyoma 437 Infiltrative uterine leiomyomas 437 Cotyledonoid dissecting leiomyoma 437

Smooth muscle tumor of uncertain malignant potential (STUMP) 438

Smooth muscle tumors, leiomyosarcomas 438 ‘Classic’ leiomyosarcomas 438 Uterine epithelioid leiomyosarcoma 439 Dedifferentiated leiomyosarcoma 440 Myxoid leiomyosarcoma 440 Intravenous leiomyosarcomatosis 440 Osteoclast-like giant cells in smooth muscle tumors 440 Uterine leiomyosarcoma with a clear cell component 440 Uterine sarcoma with liposarcomatous differentiation 440

Miscellaneous tumors 441 Nodular histiocystic hyperplasia of the endometrium 441

Mixed epithelial and mesenchymal tumors 441 Adenomyomas 441 Adenomyosis 443 Mixed mullerian tumors, malignant (sarcomatoid

carcinoma/carcinosarcoma) 443 Mixed mullerian tumors, mullerian adenofibroma 445 Mixed mullerian tumors, mullerian adenosarcoma 445

Tamoxifen-related gynecological lesions 447 Tamoxifen-related polyps 447 Tamoxifen-related carcinomas 447 Tamoxifen-related sarcomas 448 Other tamoxifen-related lesions 448

Trophoblastic tumors and tumor-like lesions 448 Choriocarcinoma 448 Epithelioid trophoblastic tumor (ETT) 449 Exaggerated placental site (EPS) 451

Placental site nodule (PSN) 454 Placental site trophoblastic tumor (PSTT) 455

WHO classification of uterine corpus tumors 457

Tumors of the ovary 457 Epithelial-stromal tumors 457

Clear cell tumors 457 Benign and borderline clear cell tumors 457 Clear cell carcinoma 457

Endometrioid tumors 460 Benign endometrioid tumors 460 Proliferating (borderline) endometrioid tumors 460 Endometrioid carcinoma 460

Mucinous tumors, benign (mucinous cystadenoma) 464 Mucinous tumors, malignant (mucinous carcinoma) 464 Mucinous tumors, proliferating (borderline) 465 Seromucinous tumors 467

Atypical proliferative tumor 467 Atypical proliferative seromucinous tumor with

intraepithelial carcinoma 467 Atypical proliferative seromucinous tumor with

microinvasion 467 Invasive seromucinous carcinoma 467

Serous epithelial ovarian tumors, benign 469 Benign serous cyst 469 Benign serous adenofibroma 469 Benign papillary serous cystadenoma 469 Serous cystadenofibroma 470

Serous epithelial ovarian tumors, malignant 470 Serous epithelial ovarian tumors, proliferating (borderline) 471

Proliferating (borderline) serous tumors with a micropapillary pattern (so-called ‘micropapillary carcinoma’) 472

Proliferating (borderline) serous tumors with microinvasion 472

Extraovarian peritoneal lesions ‘implants’ associated with proliferating (borderline) serous tumors 472

Primary peritoneal proliferating (borderline) serous tumors 472

Proliferating (borderline) serous tumor arising in pelvic lymph nodes 472

Transitional cell tumors (Brenner tumors) 475 Benign transitional cell (Brenner) tumor 475 Proliferating transitional cell (Brenner) tumor 475 Malignant transitional cell tumors (including malignant

Brenner tumor) 475

Germ cell tumors 477 Choriocarcinoma, carcinoma 478 Dysgerminoma 478 Embryonal carcinoma 478 Polyembryoma (polyembryonic carcinoma) 479 Teratoma, immature 479 Teratoma, mature cystic teratoma (dermoid cyst) 481

Mature cystic teratoma 481 Mature cystic teratoma with secondary malignant

transformation 481 Teratoma, mature solid-type 483 Teratoma, monodermal, and highly specialized teratomas 484

Struma ovarii 484 Carcinoid tumor 484

Neuroectodermal tumors 485 Epidermoid cyst in the ovary 485

Yolk sac tumor (endodermal sinus tumor) 486

Mixed germ cell and sex cord-stromal tumors 488 Gonadoblastoma 488 Unclassified (gonadal anlage tumor) 488

Sex cord-stromal tumors 489 Granulosa-stromal tumors, adult granulosa cell tumor 489 Granulosa-stromal tumors, juvenile granulosa cell tumor 492 Gynandroblastoma 492 Hyperreactio luteinalis (multiple luteinized follicle cysts) 493 Massive ovarian edema 493 Ovarian fibromatosis 494 Sclerosing stromal tumor 494 Sertoli-stromal cell tumors, Sertoli-Leydig cell tumors

(androblastomas) 495 Sex cord tumor with annular tubules 498 Stromal hyperplasia and stromal hyperthecosis 499 Thecoma-fibroma (fibrothecoma) group of ovarian

tumors 499 Thecoma 500 Luteinized thecomas 500 Stromal tumors with minor sex cord element 500 Fibroma 500 Fibrothecoma 500 Classical malignant fibrothecomas 500

Unclassified sex cord-stromal tumors 501

Steroid (lipid) cell tumors 501 Leydig cell hyperplasia 502

Hilar Leydig cell hyperplasia 503 Stromal Leydig cell hyperplasia 503

Pregnancy luteoma 503

Tumors of the rete ovarii 504 Cysts of the rete ovarii 504 Adenomas of the rete ovarii 504 Adenomatous hyperplasia 504 Rete adenocarcinoma 504

Tumors of uncertain origin and miscellaneous tumors 504 Endometriosis 504

Deep endometriosis 505 Incisional endometriosis 505 Gastrointestinal endometriosis 505 Ovarian endometriotic cysts 505 Malignant transformation of endometriosis 505 Endometriosis-associated stromal sarcomas 505 Atypical endometriosis 505

Hepatoid ovarian carcinoma 506 Mural nodules in ovarian epithelial cystic tumors 506 Ovarian tumor of probable wolffian origin 507 Small cell carcinoma (hypercalcemic type) 508 Uterus-like ovarian mass 509

Secondary (metastatic) ovarian tumors 510 Metastatic breast carcinoma 510

Metastatic gastrointestinal carcinomas 510 Metastatic colonic adenocarcinoma 510 Krukenberg tumor 511 Metastatic carcinoma of the gallbladder, bile ducts

or pancreas 513

Basal cell carcinoma (BCC) of the vulva comprises 2-4% of all vulvar cancers. In general, vulvar BCCs tend to grow at

significant morbidity and occasional mortality if they are neglected or improperly treated. Genital BCC occurred in an older age group with no identifiable predisposing risk factors and did not show evidence of human papillomavirus (HPV) infection. Hematogeneous metastasis at presentation appears to result in rapidly progressive disease. The treatment of choice consists of surgical excision with tumor-free margins. Because of local

Metastasis from other parts of the female genital tract 513 Synchronous or metastatic endometrial or

ovarian carcinoma 513 Ovarian metastases from cervical carcinoma 513 Other metastatic tumors 514 Metastatic renal cell carcinoma 514 Metastatic transitional cell carcinoma of the ovary 514

WHO revised classification of ovarian tumors 511

Tumors of the Fallopian tube 516 Epithelial tumors 516

Adnexal tumor of probable wolffian origin 516 Carcinomas of the Fallopian tube 516 Epithelial metaplasia, hyperplasia and pseudocarcinomatous

lesions 516

Miscellaneous lesions 517 Adrenal rest 517 Salpingitis isthmica nodosa 517

Tumors of the peritoneum 517 Mesothelial tumors 517

Adenomatoid tumor 517 Inclusions, cysts and hyperplasia 518

Mesothelioma, deciduoid 519 Mesothelioma, diffuse malignant 519 Mesothelioma, multicystic 520 Mesothelioma, well-differentiated papillary 520

Metastatic tumors 520 Gliomatosis 520 Pseudomyxoma peritonei 521 Strumosis peritonei 522

Miscellaneous lesions 522 Disseminated peritoneal leiomyomatosis 522 Splenosis 522 Supernumerary ovary 522 Trophoblastic implants 522

Mullerianosis (mullerian tumor-like conditions) 523 Deciduosis 523 Endocervicosis 523 Endometrioid, clear cell and transitional cell changes 523 Endosalpingosis 523 Mullerian inclusion cysts 524

Primary peritoneal epithelial tumors 525 Mucinous tumors 525 Serous tumors 525

Tumors of the female genital tract form a heterogeneous group of epithelial, mesenchymal, sex-cord, germ cell, or mesothelial origin. They are either benign or malignant tumors. Of the most commonly encountered benign lesions, leiomyoma ranks number one, followed by endocervical or endometrial polyps, dermoid cyst or benign ovarian cysts. Amongst the malignant tumors of the female genital tract, endometrial carcinoma, squamous cell carcinoma of the cervix and its precursor lesions (CINI-III), and epithelial ovarian tumors are frequently seen in gynecological practice. Of the sex cord-stromal tumors, adult granulosa cell tumor and fibrothecoma are not uncommon, while malignant germ cell tumors are very rare.