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Year : 2018  |  Volume : 11  |  Issue : 6  |  Page : 562-564  

Female adnexal tumor of Wolffian origin: Case report of a rare tumor

1 Department of Obstetrics and Gynecology, Dr. D Y Patil Medical College, Dr. D Y Patil Vidyapeeth, Pune, Maharashtra, India
2 Department of Pathology, Dr. D Y Patil Medical College, Dr. D Y Patil Vidyapeeth, Pune, Maharashtra, India

Date of Submission30-Apr-2018
Date of Acceptance03-Aug-2018
Date of Web Publication15-Nov-2018

Correspondence Address:
Himadri Bal
Department of Obstetrics and Gynecology, Dr. D Y Patil Medical College, Dr. D Y Patil Vidyapeeth, Pimpri, Pune, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_71_18

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Female adnexal tumor of Wolffian origin (FATWO) is a rare, poorly understood lesion characterized by variety of epithelial patterns and is known to occur in the broad ligament, mesosalpinx, fallopian tube, ovary, and pelvic peritoneum. About 80 cases of FATWO have been reported till date. A 70-year-old posthysterectomized woman reported with lump abdomen. She was provisionally diagnosed as malignant ovarian tumor. The patient was taken up for staging laparotomy. A solid mass arising from the left adnexa was removed along with the right-sided salpingo-oophorectomy and pelvic lymphadenectomy. Histopathological report was “benign Wolffian adnexal tumor.” FATWOs are rare tumors arising from the remnants of the mesonephric duct. Although most of them are benign, some have the potential for malignant behavior. Diagnosis is often based on exclusion of other neoplasms. Total abdominal hysterectomy and bilateral salpingo-oophorectomy are considered the recommended mode of therapy. The role of adjuvant radiation therapy or chemotherapy remains debatable.

Keywords: Adjuvant therapy, female adnexal tumor of Wolffian origin, immunohistochemistry, malignancy potential

How to cite this article:
Bal H, Agrawal SA, Kumar H, Shokeen V. Female adnexal tumor of Wolffian origin: Case report of a rare tumor. Med J DY Patil Vidyapeeth 2018;11:562-4

How to cite this URL:
Bal H, Agrawal SA, Kumar H, Shokeen V. Female adnexal tumor of Wolffian origin: Case report of a rare tumor. Med J DY Patil Vidyapeeth [serial online] 2018 [cited 2019 Apr 25];11:562-4. Available from: http://www.mjdrdypv.org/text.asp?2018/11/6/562/245434

  Introduction Top

The Wolffian ducts regress in the female during embryogenesis. Its remnants can be traced to the ovarian hilum, the broad ligament, and the lateral aspect of the uterus and vagina as the Gartner's duct, paroophoron, and epoophoron. Female adnexal tumor of Wolffian origin (FATWO) is presumed to arise from these relics of the Wolffian duct. These tumors are characterized by a variety of epithelial patterns and are known to occur in the broad ligament, mesosalpinx,  Fallopian tube More Details, or ovary. Preoperative diagnosis of FATWO is difficult because of the rarity of the disease and the paucity of available literature on this entity. There is no comprehensible recommendation regarding its preoperative workup and optimal treatment. Here, we report one such case of FATWO which we came across, rather stumbled upon incidentally because of the histopathological report.

  Case Report Top

A 70-year-old posthysterectomized woman reported with lump abdomen and dyspepsia accompanied by disturbed bowel and bladder habits for 1 year. The patient had undergone a vaginal hysterectomy for genital prolapse 25 years back.

On abdominal examination, there was a 26 cm × 18 cm hard abdominopelvic mass with irregular surface and restricted mobility. Vaginal examination corroborated abdominal findings. CT pelvis showed a heterogeneous mass lesion of size 20 cm × 17 cm × 10 cm in the pelvis and lower abdomen and reported as primary malignant neoplasia, most likely “ovarian mucinous cystadenocarcinoma.” Tumor markers were normal.

On staging laparotomy, a solid capsulated mass of size 18 cm × 12 cm × 8 cm with smooth bosselated surface was found arising from the left adnexa [Figure 1]. Peritoneal washing was sent for cytology. Abdominal touring did not reveal any suspicious area. The tumor was removed along with right-sided salpingo-oophorectomy, bilateral pelvic lymphadenectomy, and infracolic omentectomy. Paraaortic nodes were not enlarged.
Figure 1: Tumor mass at laparotomy

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The histopathology report of the mass was a surprise. It read “Sections show a neoplasm composed of epithelial cells growing in the form of hollow tubules and diffuse sheets. The tubules are lined by columnar/cuboidal cells. Few large cystic spaces are also seen lined by cuboidal cells [Figure 2]. Some foci show round to oval cells with abundant eosinophilic cytoplasm. Some mucin-secreting glands are also noted.” Final report – “Benign Wolffian Adnexal Tumour.” Immunohistochemistry was carried out, which was positive for cytokeratin 7, vimentin, and calretinin.
Figure 2: High-power view showing neoplasm composed of epithelial cells in the form of hollow tubules

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The patient had an uneventful recovery and discharged on postoperative day 10.

  Discussion and Review of Literature Top

Kariminejad and Scully in 1973 reported nine tumors which were morphologically different and located in the broad ligament. Since the broad ligament is also the site of Wolffian duct remnants, these tumors were named by them as FATWO.[1] In 2003, WHO rechristened these tumors as Wolffian adnexal tumor. About 80 cases have been reported in the literature so far.[2]

FATWOs are generally found in women in the age group of 18–81 years (mean 50 years).[3] Most tumors are unilateral, originating from the broad ligament and some from the ovary or paravaginal tissue. Clinically, these cases are asymptomatic or have nonspecific complaints. The tumor is incidentally detected as an abdominal lump or adnexal mass. Such vague, nonspecific clinical findings make the diagnosis of FATWO difficult or at times accidental, like it happened in our case.

MRI findings were described by Matsuki et al. as a slightly hyperintense mass with cystic degeneration in the adnexa, which was difficult to differentiate from a subserosal leiomyoma or an ovarian tumor.[4] Routine tumor markers for ovarian malignancy do not have much role in the diagnosis of FATWO. Ramirez et al. in their review of Wolffian tumors found normal preoperative Ca 125 level.[5]

Macroscopically, the tumors may be solid, firm, and sometimes spongy in feel. Devouassoux-Shisheboran et al. described four histological patterns: (i) diffuse solid nest of spindle cells; (ii) epitheloid tubular pattern of packed, winding, branching, and anastomosing tubules; (iii) cribriform-sieve-like pattern of epithelioid cells; and (iv) multicystic.[6] In the present case, hollow tubular pattern and diffuse sheets of cells along with cystic spaces were seen.

The main differential diagnosis of this entity is sex cord stromal tumors and endometrioid ovarian or fallopian tube adenocarcinoma. The role of immunohistochemistry in clinching the diagnosis of FATWO has also been studied. Positive immunoreaction to pan-cytokeratin, CAM 5.2, cytokeratin 7, and vimentin supports diagnosis of FATWO.[7]

FATWOs are generally regarded as benign. However, literature has shown recurrence when the tumor was treated with local resection. Therefore, FATWOs are now thought to have low malignancy potential. Out of the 80 cases detected so far, 21 have shown recurrence after treatment. Liver has been the most common site of recurrence. Criteria for malignancy, as suggested by Sivirdis et al., are size >10 cm, hypercellularity, capsular invasion, capsule rupture, and noticeable metastasis. However, the prognosis does not correlate with clinical presentation and cytology. Recurrence could occur in the absence of the aggressive histological character and even several years after the diagnosis.[8]

Treatment option remains ill-defined due to the rarity of the disease. According to Lesin et al. complete surgical resection with hysterectomy and bilateral adnexectomy is the most effective therapy.[9] Role of adjuvant radiotherapy or chemotherapy is controversial. Cisplatin, paclitaxel, and doxorubicin have all been tried in recurrent disease with insignificant response. Malignant FATWO has been reported to be potentially hormone sensitive because relapse after pregnancy has been reported. Cases treated postsurgery with molecular targeted therapy such as tyrosine kinase inhibitor have shown significant disease-free interval.[10] However, further studies are needed.

  Conclusion Top

FATWOs are rare tumors arising from the remnants of the mesonephric ducts. These tumors pose diagnostic difficulties due to paucity of specific clinical features and varied laboratory criteria. Diagnosis is often presumptive based on the exclusion of other neoplasms. Immunohistochemistry plays a significant role in the diagnosis. Although most of them are benign, some have malignant potential. Hence, surgery advocated in these cases is generally hysterectomy with bilateral salpingo-oophorectomy unless the patient is young and requiring preservation of fertility. The role of adjuvant therapy is debatable and is not routinely administered. After initial surgical treatment, patients should be appropriately followed up over a long-term period. Further research in these tumors is recommended.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kariminejad MH, Scully RE. Female adnexal tumor of probable Wolffian origin. A distinctive pathologic entity. Cancer 1973;31:671-7.  Back to cited text no. 1
Heatley MK. Is female adnexal tumour of probable Wolffian origin a benign lesion? A systematic review of the English literature. Pathology 2009;41:645-8.  Back to cited text no. 2
Türkçapar AF, Seçkin B, Güngör T, Sirvan L, Mollamahmutoğlu L. Diagnosis and management of female adnexal tumor of probable Wolffian origin (FATWO) arising from ovary: A case report. J Turk Ger Gynecol Assoc 2013;14:56-9.  Back to cited text no. 3
Matsuki M, Kaji Y, Matsuo M. Female adnexal tumour of probable Wolffian origin: MR findings. Br J Radiol 1999;72:911-3.  Back to cited text no. 4
Ramirez PT, Wolf JK, Malpica A, Deavers MT, Liu J, Broaddus R. Wolffian duct tumors: Case reports and review of the literature. Gynecol Oncol 2002;86:225-30.  Back to cited text no. 5
Devouassoux-Shisheboran M, Silver SA, Tavassoli FA. Wolffian adnexal tumor, so-called female adnexal tumor of probable Wolffian origin (FATWO): Immunohistochemical evidence in support of a Wolffian origin. Hum Pathol 1999;30:856-63.  Back to cited text no. 6
Yu N, Zhao D, Liu Y, Zhang T. Wolffian tumor: Report of five cases and review of literature. Int J Clin Exp Pathol 2017;10:3558-64.  Back to cited text no. 7
Deshimaru R, Fukunaga T, Sato T, Morinaga S, Takahashi M. A case of metastatic female adnexal tumor of probable Wolffian origin. Gynecol Oncol Rep 2014;10:22-4.  Back to cited text no. 8
Lesin J, Forko-Ilić J, Plavec A, Planinić P. Management of Wolffian duct tumor recurrence without chemotherapy. Arch Gynecol Obstet 2009;280:855-7.  Back to cited text no. 9
Steed H, Oza A, Chapman WB, Yaron M, De Petrillo D. Female adnexal tumor of probable Wolffian origin: A clinicopathological case report and a possible new treatment. Int J Gynecol Cancer 2004;14:546-50.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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