MULLERIAN PAPILLOMA ENTITY RECOGNITION FAILURE 04/04/2016 OUT OF SIGHT, OUT OF MIND: LESSER KNOWN LESIONS OF THE VULVOVAGINAL TRACT

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1 OUT OF SIGHT, OUT OF MIND: LESSER KNOWN LESIONS OF THE VULVOVAGINAL TRACT 23 rd ANNUAL SEMINAR IN PATHOLOGY 30 APRIL 2016 W. Dwayne Lawrence MD MSc (Path.) Chief of Pathology and Laboratory Medicine Women & Infants Hospital of Rhode Island Professor and Vice Chair Department of Pathology and Laboratory Medicine Warren Alpert Medical School of Brown University Providence, RI ENTITY RECOGNITION FAILURE IF YOU ARE UNAWARE OF THE ENTITY, YOU ARE UNLIKELY TO MAKE THE DIAGNOSIS COROLLARY: IF THE TRUE DIAGNOSIS IS NOT IN YOUR DIFFERENTIAL DIAGNOSIS, YOU ARE UNLIKELY TO MAKE THE DIAGNOSIS This message is brought to you by the good people at MULLERIAN PAPILLOMA 1

2 Differential Diagnosis for Vaginal Bleeding in the Child Trauma Sexual abuse Vulvovaginitis Irritation Lichen sclerosus Sexually transmitted diseases (eg., condylomata) Endocrine/hormonal Precocious puberty Exogenous estrogen Foreign body Anatomic lesions Urethral prolapse Neoplasia (eg, hemangioma, granulosa cell tumor, embryonal rhabdomyosarcoma, adenocarcinoma, yolk sac tumor, mullerian papilloma) Vaginal Polyp in a Newborn Eur J Pediatr (2008) 167: MULLERIAN PAPILLOMA: A MIMIC OF SARCOMA BOTRYOIDES SARCOMA BOTRYOIDES MULLERIAN PAPILLOMA SARCOMA BOTRYOIDES SARCOMA BOTRYOIDES MULLERIAN PAPILLOMA MULLERIAN PAPILLOMA SARC BOT 2

3 PSEUDOSARCOMA (WITH OR WITHOUT PREGNANCY) PSEUDOSARCS OF PREGNANCY ATYPICAL MITS RADIATION TYPE FIBROBLASTS ADENOSARCOMATOID CELLULLAR PSEUDOSARCOMATOUS FEP OF PREGNANCY 3

4 NEVER MAKE ANY DIAGNOSIS OF MALIGNANCY IN A PREGNANT WOMAN OR ONE WHO MIGHT BE PREGNANT WITHOUT AT LEAST TALKING TO THE CLINICIAN DON T BE A COWBOY! CALL THE ATTENDING! MAKING A DIAGNOSIS OF MALIGNANCY IN PREGNANCY POST OP SPINDLE CELL NODULE 4

5 POSTOP SPINDLE CELL NODULE BLADDER TYPICAL POST OP SPINDLE CELL LESION? EPISIOTOMY SITE BUSIER POSTOP SPINDLE CELL NODULE 5

6 ATYPICAL POST OP SPINDLE CELL LESION? RECURRENCE? PROLAPSED FALLOPIAN TUBE FALLOPIAN TUBE PROLAPSE POST VAGINAL HYSTERECTOMY: A MIMIC OF PAPILLARY CARCINOMA PROLAPSED TUBE 6

7 ENTERIC NEOPLASMS OF THE INTROITUS AND HYMENAL AREA Types of Adenocarcinoma Presenting in a Vaginal/Periurethral Location I. Tumors of vaginal origin A. Mullerian origin (often associated with DES) Clear cell adenocarcinoma Endometrioid adenocarcinoma Endocervical-type adenocarcinoma (mucinous carcinoma of endocervical type) Intestinal-type adenocarcinoma (mucinous carcinoma of intestinal type) B. Mesonephric adenocarcinoma II. Tumors of urethral origin Clear cell adenocarcinoma Mucinous adenocarcinoma Intestinal-type histology Endocervical-type histology III. Metastatic adenocarcinoma to vagina/urethra INTROITAL/HYMENAL GI DIFFERENTIATION 7

8 TVA CA INITIAL BX ALCIAN BLUE RESECTION CHROMO Tjalma WAA, Colpaert CGA. Primary vaginal adenocarcinoma of intestinal type arising from a tubulovillous adenoma. Int J Gynecol Cancer 2006;16: MIXED TUMOR OF THE VAGINA MIXED TUMOR OF THE VAGINA MIXED TUMOR Am J Surg Pathol May;17(5): Spindle cell epithelioma, the so-called mixed tumor of the vagina. A clinicopathologic, immunohistochemical, and ultrastructural analysis of 28 cases 8

9 VAGINAL MIXED TUMOR aka SUPERFICIAL CERVICOVAGINAL MYOFIBROBLASTOMA aka SPINDLE CELL EPITHELIOMA VULVAR ANGIOMYOFIBROBLASTOMA VLV LM MIMICKING AGG ANGIOMYXOMA 9

10 AGGRESSIVE ANGIOMYXOMA STALK INTERNAL AMFB VS AGG AGMYX AMFB AA MYX VULVAR VESTIBULAR PAPILLOMATOSIS 10

11 VULVAR VESTIBULAR PAPILLOMATOSIS 34 YOF VULVAR VESTIBULAR PAPILLOMATOSIS 24 YOF VAGINAL CONDYLOMA 11

12 PAGETS DISEASE ARISING FROM A LOCO-REGIONAL CANCER (COLONIC) PAGETS DZ FROM COLON CA CDX2 TOKER vs PAGET vs BOWEN DR. WHO? DR.BOWEN PAGETOID DYSKERATOSIS DR. TOKER DR. PAGET 12

13 PAGETOID VIN 3 PIGMENTED LESIONS OF THE VULVA AND VAGINA ASYMMETRICALLY PIGMENTED PATCH ON THE VULVO-PERINEAL AREA:? MALIGNANT MELANOMA MICRO NEXT SLIDE Pigmented Extra-mammary Paget s Disease. Acta Derm Venereol 2011; 91:

14 DENDRITIC CELLS FILLED WITH MELANIN MELANOCYTES WITH DENDRITIC PROCESSES PAGETS CELLS FONTANA-MASSON CAM 5.2 HMB-45 Two cases of vulval pigmented extramammary Paget's disease: histochemical and immunohistochemical studies. British Journal of Dermatology 2000; 142: ATYPICAL GENITAL NEVI THE SET UP REQ FORM 14

15 CAVEATS ABOUT PREGNANCY AND HORMONE RELATED LESIONS GENITAL NEVI CAN BE ATYPICAL REALLY ATYPICAL ATYPICAL GENITAL NEVI IN YOUNG WOMEN 28 YOF RIGHT LABIUM MINUS 15

16 VULVOVAGINAL MELANOSIS VULVAR MELANOSIS VULVOVAGINAL MUCOSAL LENTIGINOSIS vs VULVOVAGINAL MELANOMA 42 YOF 61 YOF PERINEUM 16

17 VULVOVAGINAL MELANOMA VULVOVAGINAL MUCOSAL MELANOSIS (L) VULVAR MELANOMA (R) 29 YOF 43 YOF THE MANY FACES OF PIGMENTED VIN 17

18 PIGMENTED HIGH GRADE VIN VIN 3 VIN 3 37 YOF DEEPER MELANIN PIGMENT APPEARS BLUE AS IN A BLUE NEVUS BLUE NEVUS SUPERFICIAL MELANIN PIGMENT APPEARS DARK BROWN VAGINA THE MANY COLORS OF VULVAR VIN OTHER SQUAMOUS LESIONS OF THE VULVA 18

19 BOWENOID PAPULOSIS: A TERM WHOSE TIME HAS COME AND GONE BOWENOID PAPULOSIS JUST ANOTHER PERINEAL FIBROEPITHELIAL POLYP? VULVAR WHITE LESION 19

20 INVERTED FOLLICULAR KERATOSIS VULVAR SCC MIMICKING ANGIOSARCOMA 82 YOF WITH PRIOR VLV CA AND RADIATION RX: NEW TUMOR OF VLV 20

21 82 YOF WITH PRIOR VLV CA AND RADIATION RX: NEW TUMOR VLV AE1/AE3 p63 A 43 YOF WITH A 3.5 CM VULVAR MASS: VULVAR SARCOMATOID SQUAMOUS CELL CA REFERENCE: SQUAMOUS CELL CARCINOMA OF THE VULVA WITH SARCOMATOID FEATURES. CHOI DS et al. GYNECOLOGIC ONCOLOGY, 2006 SIMPLEX VIN 3 (d VIN 3) AND INVASIVE SQUAMOUS CARCINOMA 21

22 SIMPLEX VIN 3 HART W USCAP SIMPLEX VIN 3 p53 + SIMPLEX VIN 3 22

23 SIMPLEX (DIFFERENTIATED) VIN 3 SIMPLEX ATYPICAL HYPERPLASIA? SIMPLEX VIN 3 WITH EARLY INVASION 23

24 NON-VIN ASSOCIATED EARLY INVASION SQUAMOUS HYPERPLASIA WITH HYPERKERATOSIS NON-SIMPLEX VIN SIVC NON-SIMPLEX VIN SIVC 24

25 SIMPLEX VIN MOST COMMON PRECURSOR TO INVASIVE WD SCC SIMPLEX (DIFFERENTIATED) VIN 3? SQUAMOUS HYPERPLASIA WHAT IS THE DIAGNOSIS? CAN YOU TRUST ANY WHITE LESION OF THE LOWER FEMALE GENITAL TRACT? 25