Korea-Taiwan-Japan Joint Meeting for
Gynecological Pathology

Mini-lecture
Female Adnexal Tumor of Probable
Wolffian Origin (FATWO) in Taiwan:
A Small Case Series and Literature
Review

Presenter: Yeh-Han Wang M.D.
Department of Anatomic Pathology,
Taipei Institute of Pathology, Taiwan

Outlines• Introduction & Literature Review• Case Series Morphology
 Immunohistochemical profiles
 Genetic profiles• Take Home Message

Introduction: Female Adnexal Tumorof Probable Wolffian Origin (FATWO)• Rare extraovarian, extratubal tumors

firstly reported by Kariminejad and Scullyin a 9-case series in 1973.

• Currently “an epithelial tumor of Wolffian(mesonephric) origin.”• Most of the cases are benign or indolent

tumors confined within adnexa.

• Low grade malignant potential oraggressive behaviors in a minor (10%)
cases• Recurrence / Metastasis

Location & Gross Features• Often in broad ligament, mesosalpinx,ovarian hilus and pelvis.

Location & Gross Features• Often in broad ligament, mesosalpinx,

ovarian hilus and pelvis.

• Encapsulated, solid ovoid masses

with nodular, or lobulated appearance.

Three Pattern in the Original
Article• Solid or diffuse arrangement• Closely packed, winding, branching,

and anastomosing tubules• A sieve-like pattern with hollow

tubules varying in size and shapewith occasional cyst formation

Three Pattern in the Original
ArticleCase SeriesCase Series in Taiwan• An inter-institutional review• Four cases (1 benign, 3 malignant) retrieved• All arising from adnexa of middle to old age

women (50~63 y/o)

• Size varies from <1 cm to 16 cm• 3 malignant cases
 Radical surgery and chemotherapy
 One with local recurrence Follow-up interval: 12~51 months
 All alive with or without diseases

Case Series in Taiwan
Case AgeprimarysiteSurgery

ChemoInterval ofF/U

salpingooophorectomy

N

Loss of F/UCurrent
status

1

60

right
fallopian
tube

2

50

right
adnexaStaging surgeryY

12 months

alive

3

61

left ovary Staging surgeryY

42 months

alive

463

left ovary Staging surgery

Y

51 months

alive, recuronceMicroscopic
finding in fourcases

Case 1Sieve-like pattern

Case 2
Solid and sievelike patternCase 3
Solid and tubularpatternCase 4Spindle cellmorphologySummary in morphology• Sieve-like and tubular pattern are mostcharacteristic.

• Solid or spindle cell morphology would

cause difficulty in diagnosis

 Try to find minor sieve-like or tubularcomponents• Malignant cases defined by nuclear

atypia, infiltrative growth pattern andfrequent mitoses
 The nuclear grading is not so “high grade” tofit in typical high grade carcinoma or sarcoma

How to Make Differential
Diagnosis?
• Gross examination

 Primary site?
 Ovary? Tube? Others?

• Microscopic examination

 Diffuse pattern
 Tubular pattern Sieve-like pattern Spindle cell morphology

R’t tumor

R’t OV+tube

How to Making Differential
Diagnosis?
• Diffuse pattern Un-/Poorly-differentiated carcinoma?
 Neuroendocrine carcinoma?

• Tubular pattern Endometrioid adenocarcinoma?
 Granulosa cell tumor?
 Sertoli (-leydig) cell tumor?

• Sieve-like pattern Endometrioid adenocarcinoma?
 Granulosa cell tumor?
 Adenomatoid tumor/Mesothelioma?

• Spindle cell morphology

 Adenomatoid tumor/Mesothelioma?
 Fibroma/Thecoma?
 Sarcoma? (Leiomyosarcoma? Endometrial stromal sarcoma?)Immunohistochemicalprofiles

CK1234

EMA

1234

Not performed

Inhibin1234

Calretinin1234

CD 101234

Not available,also positive

Are General Markers Helpful?
Case

123

4

CK++++EMA--

Focal +/Inhibin--

Weak +-

Calretinin++--CD10

Focal +Focal +++Int J Gynecol Pathol Vol 35, No. 2 March 2016

12PAX8

Summary in IHC profiles• Markers with diffuse expression
 Pan-CK / Calretinin / Vimentin• Markers with variable expression
 Inhibin / Hormonal marker (ER/PR) / CD10
• Marker for differential diagnosis
 Inhibin / Pax-8 / CD10

A Possible Panel• Pan-CK / Calretinin / Inhibin / CD10 /Pax-8

Pathology International 2006; 56:95-100

24CD117

Genetic Profiles• CD117 (c-kit) expression: not necessarily

relating to genetic mutation on c-kit and
PDGFR gene (Harada, et al. Pathology International 2006; 56: 95-100)• NGS analysis revealing FATWO agenetically heterogeneous tumor (Cossu, et al. Int JGynecol Pathol 2017 (Epub ahead)) Three cases, three pathways: CTNNB1 + MET /PIK3CA / BRAF + CDKN2A

Take Home Message• FATWOs are rare and possibly

misdiagnosed.

 Identification of characteristic Sieve-like andtubular pattern would be helpful in diagnosis• Using an IHC panel would be also helpful

 Pan-CK / Calretinin / Inhibin / CD10 / Pax-8• Heterogeneity in genetics• No histological criterion predicting clinicalbehavior, and no standard treatment
medication

Acknowledgement• Dr. Chiung-Ru Lai (TaipeiVeterans General Hospital)• Dr. Tsui-Lien Mao (NationalTaiwan University Hospital)• Dr. Yee-Jee Jan and Dr. Chih-Ying Wu (Taichung VeteranGeneral Hospital)• Dr. Wan-Lin Shen and Dr. Sheng-

Tsung Chang (Chi-Mei Hospital).

Thank you for yourattention