CASE REPORT Year : 2023  |  Volume : 11  |  Issue : 1  |  Page : 93-97

Endometrioid adenocarcinoma within an endometriotic cyst in the mesosalpinx: A case report and review of the literature

Nouf Faisal Alharbi1, Mohammed Mubarak Hajla2, Tarek Elsharkawy3, Ashraf Alharbi4, Randa Abdelsayed5, Fathia Ebrahim Aljama5
1 Department of Pathology, General Directorate of Health Affairs, Dammam, Eastern Province, Kingdom of Saudi Arabia
2 Department of Pathology, Dammam Health Network, Dammam, Eastern Province, Kingdom of Saudi Arabia
3 Department of Pathology, Faculty of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Eastern Province, Kingdom of Saudi Arabia
4 Department of Internal Medicine, King Faisal Hospital, Makkah, Kingdom of Saudi Arabia
5 Department of Obstetrics and Gynecology, Faculty of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Eastern Province, Kingdom of Saudi Arabia

Date of Submission23-May-2022Date of Decision16-Aug-2022Date of Acceptance14-Dec-2022Date of Web Publication14-Jan-2023

Correspondence Address:
Nouf Faisal Alharbi
Department of Pathology, General Directorate of Health Affairs, Eastern Province, 28th Street, Ghirnatah, Dammam 32245
Kingdom of Saudi Arabia

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/sjmms.sjmms_249_22

Endometriosis-related neoplasms may arise within endometriotic cysts of organs of the female genital tract or other organ systems. Herein, we present a case of endometrioid adenocarcinoma arising within an endometriotic cyst in the mesosalpinx. A 27-year-old single female presented to the clinic with a history of right hypochondrial pain. Pelvic MRI revealed a bulky uterus with three intramural uterine fibroids and a complex cyst in the mesosalpinx. The excised cyst showed predominantly confluent growth of relatively well-defined glands resembling proliferative-phase endometrium in a background of endometriosis. The tumor was diagnosed as endometrioid adenocarcinoma within an endometriotic cyst, pathologic stage pT1a. She was referred to the oncology team, where a positron emission tomography scan showed unremarkable results. Although these neoplasms have been reported in various locations within the female genital tract, endometrioid adenocarcinoma arising from endometriosis in the mesosalpinx is seldom reported.

Keywords: Cyst, endometriosis, female reproductive system, endometrioid adenocarcinoma, mesosalpinx, neoplasms, ovarian epithelial cancer

How to cite this article:
Alharbi NF, Hajla MM, Elsharkawy T, Alharbi A, Abdelsayed R, Aljama FE. Endometrioid adenocarcinoma within an endometriotic cyst in the mesosalpinx: A case report and review of the literature. Saudi J Med Med Sci 2023;11:93-7
How to cite this URL:
Alharbi NF, Hajla MM, Elsharkawy T, Alharbi A, Abdelsayed R, Aljama FE. Endometrioid adenocarcinoma within an endometriotic cyst in the mesosalpinx: A case report and review of the literature. Saudi J Med Med Sci [serial online] 2023 [cited 2023 Jan 15];11:93-7. Available from: https://www.sjmms.net/text.asp?2023/11/1/93/367766   Introduction 

Malignant transformation of endometriosis is well-documented in the literature. The incidence is reported to be up to 1% in women.[1] Hormonal changes associated with menopause may affect the malignant transformation of endometriosis.[2],[3] Endometrioid and clear cell carcinomas are the most common types reported among others.[4],[5],[6],[7] Endometriosis-related neoplasms arise within endometriotic cysts of organs of the female genital tract and other organ systems. The most common location is the ovary,[1] with the pelvic peritoneum, the rectovaginal septum, and the intestinal wall being much less common.[7],[8] The uterine cervix, a rare location for endometrioid adenocarcinoma (EAC) arising from endometriosis to present in, has been reported.[9] Primary EAC in the mesosalpinx has only been reported once.[10]

Herein, we present a very rare case of EAC arising within an endometriotic cyst of the mesosalpinx in a young woman.

  Case Presentation 

A 27-year-old single female presented to the outpatient clinic with a history of right hypochondrial pain for 1 month. She underwent a pelvic ultrasound and computed tomography (CT) scan of the abdomen and pelvis, which revealed a complex right adnexal mass. She was diagnosed with a right adnexal mass. Menarche was at 12 years of age. Her menses were regular every 28 days for five days with normal flow. She had a medical history of kidney stones. However, she had no past surgical history. She had a family history of breast cancer (paternal aunt), tongue cancer (paternal uncle) and lymphoma (maternal aunt). She had no known family history of uterine, ovarian, or colon cancer.

On presentation, the patient was stable, average built. Her vital signs and general physical examination were unremarkable. The abdomen was soft, lax, with no tenderness or palpable pelvic or abdominal mass. Blood investigations revealed hemoglobin of 12.5 mg/dl. Tumor markers cancer antigen 125, alpha-feto protein, carcinoembryonic antigen, carbohydrate antigen 19-9 were within normal limits. Beta-human chorionic gonadotropin was negative. Transabdominal ultrasound exhibited a bulky uterus with three intramural uterine fibroids. The largest one was located in the fundus, measured 4 × 3 cm. The second largest, located anteriorly, measured 3 × 3 cm. A posteriorly located third fibroid measured 2.5 × 2 cm. A clear cyst was identified on the right adnexa, measuring 8 × 6 cm, along with a solitary vascular nodule measuring 2 × 2 cm. The left ovary was unremarkable.

Pelvic magnetic resonance imaging revealed a complex cystic lesion measuring 8.9 × 6.2 cm in the right mesosalpinx with high T2-weighted image (T2 WI), low T1-weighted image (T1 WI) signal intensity, with nondependent papillary projection. A solid mural nodule was seen along the left anterolateral wall adjacent to the uterus. The mural nodule measured 2.3 × 1.8 cm. A focus of blooming was identified along the wall, representing calcification. Post-contrast, a peripheral rim enhancement was seen along the cyst and solid enhancement of the mural nodule. The mural nodule showed diffusion restriction. There was neither evidence of local invasion nor was there a fatty component. The left ovary was unremarkable. The uterus was pushed slightly to the left side by the mass effect of the right adnexal cystic mass. Three uterine lesions were found, suggestive of fibroids. They shared a similar signal intensity of low T2 WI, iso-intense T1 WI and heterogeneous enhancement less than that of the uterus. The largest of them was a subserosal pedunculated mass arising from the uterine fundus measuring 4 × 3.2 × 3.5 cm. The second-largest fibroid was seen in the anterior uterine corpus in the myometrial component, causing a contour bulge of the uterus anteriorly measuring 2.6 × 2.2 × 2.6 cm in its right-to-left, anteroposterior and cornu to cornu dimension, respectively. A small fibroid was identified in the myometrium of the posterior uterine corpus with a subserosal component measuring 1 × 1 × 0.8 cm. The uterus otherwise showed normal zonal anatomy with normal endometrial thickness. The junctional zone measured 0.8 cm. The vagina, urethra, urinary bladder and rectum were unremarkable. Small volume inguinal and external iliac lymph nodes were seen. However, no size-significant lymph nodes were detected. A small amount of physiologic fluid was seen in the pelvis; however, there was no ascites. The imaged bowel loops were not dilated. There were no aggressive enhancing bone lesion. The impression was that of a large complex right ovarian cystic mass concerning for neoplasm, likely serous tumor, warranting surgical excision to exclude malignancy. Other differential diagnosis could not be excluded radiologically such as para-ovarian cyst (mesonephric or para-mesonephric) and pelvic wall peritoneal cyst [Figure 1]a, [Figure 1]b, [Figure 1]c.

Figure 1: (a) MRI pelvis coronal view illustrate right adnexal mass (M) and uterus (U). (b) MRI pelvis sagittal view demonstrating cystic adnexal mass (M) with papillary projecting solid mural nodule (N). (c) MRI pelvis axial view demonstrate relationship between adnexal cyst (M), uterus (U) and uterine leiomyoma (L). MRI: Magnetic resonance imaging

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Exploration of the pelvic cavity through mini-laparotomy showed minimal peritoneal fluid sent for cytologic examination. The left ovary and  Fallopian tube More Details were normal. The right ovary was within normal limits. However, a mesosalpinx (para-ovarian) cyst overstretched the right fallopian tube that was otherwise normal with a free fimbrial end [Figure 2]a. The cyst measured 9 × 6 cm. It had a smooth, regular surface with a thin wall and was attached to the surrounding structures by loose connective tissue. No pelvic adhesions were identified. The cyst was delivered intact and easily separated. It was excised with the preservation of the right ovary and right fallopian tube [Figure 2]b. The pelvic peritoneal surface was smooth. The uterus was bulky with three fibroids. Myomectomy was done [Figure 2]c.

Figure 2: (a) The cyst before removal (M). The right ovary (O) is seen on the left side of the cyst. The fallopian tube is shown (F). (b) The right ovary and fallopian tube after removal of the cyst (c) The cyst (left) alongside three uterine leiomyomas (d) The cyst wall showing endometrial glands and stroma (H and E, ×200) (e) Higher magnification of the cyst wall showing endometrial glands and stroma (H and E, ×400)

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Grossly, two specimens were received. The first were three uterine leiomyomata ranging in size from 2.7 to 4 cm. The second specimen was an intact cyst that measured 10.5 × 8 × 5.5 cm. The outer serosal lining was smooth and glistening with focal areas of congestion. There were no adhesions. Upon opening the specimen, the cyst was filled with yellowish serous fluid. The inner wall was lined by a yellowish gelatinous-like material. Focal yellow streaks were lining the inner wall. Firm yellowish nodules were attached to the inner wall, the largest of which measured 0.4 × 0.3 cm. A soft tan, polypoid mass measuring 2.5 × 2 × 1.5 cm was identified. The wall thickness of the cyst was 0.2 cm. Microscopically, endometrial stroma and glands were seen in the cyst wall embedded in a dense fibrous stroma [Figure 2]d and [Figure 2]e. Features of fresh hemorrhage and hemosiderin-laden macrophages were evident. The polypoid mass that was attached to the inner wall of the cyst showed predominantly back-to-back (confluent) growth of relatively well-defined glands, resembling proliferative-phase endometrium [Figure 3]a and [Figure 3]b. These glands had irregular borders and were embedded in a hyalinized stroma along with foamy histiocytes. Squamous morules were identified throughout the tumor (shown in [Figure 3]c). The nuclei of the glands had preserved polarity and mild nuclear enlargement and hyperchromasia. Immunohistochemically, the tumor stained positive for cytokeratin 7, paired-box gene 8, epithelial membrane antigen, and progesterone receptor. The glandular epithelium stained positive for estrogen receptor and vimentin [Figure 3]d and [Figure 3]e. Tumor protein p53 stain showed wild-type positivity. The tumor was negative for Wilms tumor protein (WT-1) and Napsin A. Immunohistochemical stains (IHC) for mismatch repair proteins (MMR) MLH1, MSH2, MSH6, and PMS2 revealed intact nuclear expression (MMR-proficient). The tumor was diagnosed as EAC within the endometriotic cyst, grade 1 (Fédération Internationale de Gynécologie et d'Obstétrique [FIGO] grading system).[11] The largest tumor dimension was 2.5 cm. The pathologic staging was pT1a (ovary, fallopian tubes and peritoneum, American Joint Committee on Cancer [AJCC] manual, 8th Ed).[12]

Figure 3: (a) The tumor showed confluent growth of relatively well-defined glands in a hyalinized stromal background (H and E, ×40) (b) Hyalinized stromal background (H and E, ×200) (c) Squamous morules were evident (H and E, ×200) (d) The glandular epithelium in the tumor expressed ER (ER IHC, ×200) (e) The glandular epithelium in the tumor was positive for Vimentin (Vimentin IHC, ×200)

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Postoperatively, the patient did well and was discharged in a satisfactory condition. The patient was referred to the oncology team. A positron emission tomography scan was done, which showed unremarkable results. At the time of reporting this case, the patient had been on periodic surveillance, with no recurrence for one year since the cystectomy.

  Discussion 

The first reported case of a malignant epithelial tumor of the broad ligament was in 1952,[13] and since then, 17 cases have been reported.[14] Subtypes of malignant tumors included serous carcinoma, transitional cell carcinoma, clear cell carcinoma, and EAC. Only 5 cases of EAC have been reported in the literature. The first case was reported in 1979 by Clark et al.[10] Aslani and Scully reported three EAC cases of four primary carcinomas of the broad ligament.[15] The most recent case was by Vaysse et al. in 2008.[16] Two out of the five reported EAC cases had synchronous endometrial EAC. Both cases were in Aslani's case series. Gardner et al.[17] have proposed criteria for the diagnosis of primary paratubal carcinoma: a primary location within or on the surface of the broad ligament and complete separation of the tumor from the uterus and the ipsilateral fallopian tube and ovary. The case presented here matches the criteria.

The pathogenesis of extraovarian EAC is still debatable. The role of endometriosis in the pathogenesis has been documented in the literature.[4],[18],[19] Rarely, the malignant transformation of endometriosis occurs, and this has been reported under the influence of estrogen replacement therapy;[20] however, the present patient had not received this therapy.

The evident squamous morules along with the well-differentiated glandular morphology favor the diagnosis of EAC. The adjacent endometriotic glands suggest transformation to EAC. The present case showed hyalinized stroma akin to that of clear cell carcinoma. However, it was negative for napsin A IHC. The well-differentiated glands along with the relative monomorphic appearance of the nuclei and the negative WT-1 IHC preclude the diagnosis of serous carcinoma.

Although there was no significant family history for MMR-deficient carcinomas, the patient's young age warranted the use of IHC for mismatch repair proteins (MMR) MLH1, MSH2, MSH6 and PMS2.

Due to their proximity to the ovary, paratubal carcinomas management has been suggested to follow that for ovarian carcinomas. However, treatment recommendations are yet clear because of the limited experience in managing such cases. For young women wanting to preserve their fertility, conservative management such as unilateral salpingo-oophorectomy could be considered.

  Conclusion 

This case is a stage pT1a well-differentiated primary EAC within an endometriotic cyst of the mesosalpinx under close follow-up. The cyst was excised with the preservation of the right ovary and right fallopian tube. Treatment recommendations are not yet clear in the literature owing to the limited experience in managing paratubal carcinomas. To the best of our knowledge, this is the second reported case in the same location.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the Journal. The patient understands that name and initials will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Peer review

This article was peer-reviewed by two independent and anonymous reviewers.

Financial support and sponsorship

None.

Conflicts of interest

There are no conflicts of interest.

 

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