Fallopian tubes & broad ligament

Broad ligament

Wolffian tumor / Female adnexal tumor of probable Wolffian origin


Editorial Board Member: Gulisa Turashvili, M.D., Ph.D.
Deputy Editor-in-Chief: Jennifer A. Bennett, M.D.
Elena Lucas, M.D.
Wenxin Zheng, M.D.

Last staff update: 29 August 2022 (update in progress)

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PubMed search: Female adnexal tumor of Wolffian origin

Elena Lucas, M.D.
Wenxin Zheng, M.D.
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Cite this page: Lucas E, Zheng W. Wolffian tumor / Female adnexal tumor of probable Wolffian origin. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/ovarytumorfatwo.html. Accessed December 9th, 2022.
Definition / general
  • Adnexal tumor arising from remnants of Wolffian (mesonephric) duct
Essential features
  • Adnexal tumor arising from mesonephric (Wolffian duct) remnants
  • Heterogeneous histologic appearance
  • Diagnosis based predominantly on the anatomical site and morphology
Terminology
  • Wolffian tumor
  • Female adnexal tumor of probable Wolffian origin (FATWO)
  • Wolffian adnexal tumor (WAT)
  • Wolffian adenoma
  • Retiform Wolffian adenoma
  • Mesonephric tumor, NOS
ICD coding
  • ICD-O: 9110/1 - mesonephric tumor, NOS
  • ICD-11: 2F33.Y & XH2WJ5 - benign neoplasm of other or unspecified female genital organs & Wolffian tumor
Epidemiology
Sites
Pathophysiology
  • Believed to originate from mesonephric (Wolffian) duct remnants
  • Arises along the trajectory of the Wolffian duct: from the ovarian hilum, along mesosalpinx, broad ligament, lateral aspects of the uterus and cervix, to the outer third of the vagina (Hum Pathol 1999;30:856)
Etiology
  • Unknown
Clinical features
  • Adnexal mass with nonspecific symptoms or an incidental finding
  • Complaints include lower abdominal pain / distention, pelvic mass, abnormal vaginal bleeding or ascites
  • Most tumors are hormonally inert; rare tumors are hormonally active, causing endometrial hyperplasia (Gynecol Oncol 1995;59:304)
  • Most tumors are unilateral; rarely bilateral
Diagnosis
  • Tumor location
  • Surgical specimen: gross and microscopic examination
Radiology description
  • Well defined, encapsulated, lobulated or ovoid mass, often separate from ovaries, tubes and uterus
  • May demonstrate solid and cystic areas
  • Low signal intensity rim around the tumor on T2 weighted MRI images; might be a characteristic feature (J Obstet Gynaecol Res 2016;42:1046)
Radiology images

Images hosted on other servers:
CT and MRI: large mass

CT and MRI: large mass

MRI: mass near ovary

MRI: mass near ovary

Prognostic factors
  • Low malignant potential; most tumors behave in a benign fashion
  • Recurrence or metastases occur in 11 - 20% of cases; median of 48 months to recurrence (range 13 - 96 months) (Gynecol Oncol 2002;86:225)
  • Adverse prognostic factors: large size (> 10 cm), capsular invasion and rupture with tumor implants (Histopathology 2005;46:716)
  • Aggressive behavior includes intra-abdominal spread, recurrence or distant metastasis, typically to the liver and lungs (Gynecol Oncol 2002;86:225)
  • High cellularity, nuclear pleomorphism and high mitotic activity are associated with worse prognosis but tumors with minimal nuclear atypia and low mitotic rate may recur; no single feature is predictive of malignant behavior (Tohoku J Exp Med 1997;181:371)
Case reports
Treatment
  • Hysterectomy and bilateral salpingo-oophorectomy; tumor debulking
  • Role of adjuvant chemotherapy or radiation therapy is unknown
  • For recurrent / metastatic KIT (CD117) expressing tumors, tyrosine kinase inhibitor Gleevac (STI571) is a treatment option (Int J Gynecol Cancer 2004;14:546)
Clinical images

Images hosted on other servers:
Adnexal mass

Adnexal mass

Gross description
  • Size from < 1 cm to 30 cm
  • Encapsulated mass, often nodular, lobulated appearance
  • Often centered within or hanging from broad ligament or fallopian tube by a pedicle
  • Solid or partially cystic; may have spongy appearance
  • Cut surface is rubbery, variegated, pale yellow, gray-white or tan
  • May be hemorrhagic or necrotic
  • References: Cancer 1973;31:671, Am J Surg Pathol 1983;7:125, Hum Pathol 1999;30:856
Gross images

AFIP images
Broad ligament tumor

Broad ligament tumor

Solid and cystic cut surface

Solid and cystic cut surface

Lobulated cut surface

Lobulated cut surface



Images hosted on other servers:
Large peritoneal mass

Large peritoneal mass

Mass arising from the fimbriae

Mass arising from the fimbriae

Frozen section description
  • May be mistaken for adenocarcinoma, sex cord stromal tumor or mesonephric carcinoma
  • If the tumor is extraovarian, the tumor location (e.g., broad ligament, mesosalpinx) may help to include FATWO in the differential diagnosis during intraoperative consultation
  • Variety of patterns may aid with a differential diagnosis, although this may be difficult to appreciate during frozen section due to limited sampling
Microscopic (histologic) description
  • Cribriform, tubular, cystic, tubulocystic, sieve-like, solid cords and diffuse growth patterns
  • Usually a single pattern predominates but several architectural patterns may coexist (Am J Surg Pathol 1983;7:125)
  • Solid sheets of spindle or polygonal cells may be the dominant morphology
  • Fibrous stroma varies from scant to large areas of hyalinization
  • Intraluminal, bright eosinophilic secretions are often present
  • Cells are columnar or cuboidal, may have scant clear cytoplasm, flat or hobnail appearance
  • Large cytoplasmic vacuoles and signet ring cell-like forms may be seen (Int J Surg Pathol 2008;16:222)
  • Uniform, round or oval nuclei with pale, evenly dispersed chromatin; may have nuclear grooves
  • Nuclear atypia is mild to moderate
  • Mitotic activity is typically low (up to 1/10 - 3/10 high power fields)
  • Malignant tumors may present as undifferentiated carcinoma, high cellularity, pleomorphic nuclei or show increased mitoses (up to 16/10 high power fields) (Am J Surg Pathol 1983;7:125)
Microscopic (histologic) images

Contributed by Elena Lucas, M.D. and Wenxin Zheng, M.D.
Nodular architecture

Nodular architecture

FATWO showing various patterns

FATWO showing various patterns

Tubular growth pattern

Tubular growth pattern

Tubules infiltrating stroma

Tubules infiltrating stroma

Tubular and solid pattern

Tubular and solid pattern


Resembling endometrioid carcinoma

Resembling endometrioid carcinoma

Tubules with eosinophilic secretions

Tubules with eosinophilic secretions

Solid growth pattern

Solid growth pattern

Malignant FATWO

Malignant FATWO


CD10

CD10

Inhibin

Inhibin

Calretinin

Calretinin

Negative stains
Electron microscopy description
Molecular / cytogenetics description
Sample pathology report
  • Right adnexal mass, right salpingo-oophorectomy:
    • Female adnexal tumor of probable Wolffian origin (FATWO), 6.0 cm (see synoptic report and comment)
    • Comment: FATWO (also known as Wolffian tumor) is a rare tumor of low malignant potential. These tumors are thought to arise from mesonephric remnants. Most tumors behave in a benign fashion, although some tumors exhibit aggressive behavior. Although features known to be associated with adverse prognosis (e.g., large size, capsular invasion, rupture, high cellularity, nuclear pleomorphism, high mitotic activity) are absent in this case, cases without these features may also recur. Clinical correlation and follow up are recommended.
Differential diagnosis
Board review style question #1
A 48 year old woman is diagnosed with a 6 cm adnexal mass arising from the broad ligament. She undergoes salpingo-oophorectomy. Microscopically, the tumor has a nodular appearance with low grade cells arranged in a sieve-like pattern and tightly packed tubules with eosinophilic secretions. No necrosis is seen. Mitotic activity is 1/10 high power fields. The tumor cells are negative for PAX8 and positive for CD10. Which of the following is likely to be true?

  1. Final diagnosis is based on ancillary studies
  2. Minimal nuclear atypia and low mitotic rate are predictive of benign behavior
  3. Patients often have virilizing symptoms
  4. Tumor likely originated from mesonephric embryological remnants
  5. Tumor originated from paramesonephric embryological remnants
Board review style answer #1
D. Tumor likely originated from mesonephric embryological remnants

Comment Here

Reference: Female adnexal tumor of probable Wolffian origin
Board review style question #2

This image is from a 50 year old woman with an encapsulated adnexal mass, found incidentally. Which of the following statements is true?

  1. Calretinin and inhibin immunostains help to differentiate it from sex cord stromal tumors
  2. CD117+ tumors harbor KIT mutation
  3. ER and PR immunostains help to differentiate it from endometrioid carcinoma
  4. FOXL2 mutation is present in the majority of tumors
  5. Tumor is typically positive for pancytokeratin and CD10
Board review style answer #2
E. Tumor is typically positive for pancytokeratin and CD10. This is a female adnexal tumor of probable Wolffian origin (FATWO), which is typically positive for pancytokeratin and CD10.

Comment Here

Reference: Female adnexal tumor of probable Wolffian origin
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