Non-Peutz–Jeghers syndrome-associated ovarian sex cord tumor with annular tubules treated by radiotherapy: a case report and literature review

Li, Cheng; Aishajiang, Reyida; Teng, Yongliang; Xu, Tongge; Ding, Lijuan; Dong, Lihua

doi:10.1177/0300060521996563

Cited by 8 publications

(12 citation statements)

References 13 publications

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“…Therefore, serum progesterone and estradiol levels testing can be used for the diagnosis and the detection of recurrence. In fact, estradiol and progesterone concentrations drop dramatically after surgery, demonstrating the correlation between the upturn in concentrations and the onset of the disease [ 2 , 9 ]. The main treatment is surgery, and fertility-sparing surgery is safety recommended at reproductive age.…”

Section: Discussionmentioning

confidence: 99%

Exceptional lymph node recurrence of an unusual ovarian tumor 16 years later: a case report

Sakhri,

Slimane,

Bouaziz

et al. 2024

J Med Case Reports

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Background Sex cord-stromal tumors with annular tubules are a rare tumor accounting for less than 1% of all ovarian malignancies. However, they are characterized by very late recurrence, which can be as late as 30 years after diagnosis and treatment. Case presentation A 16-year-old female Caucasian patient was treated in our department for a stage IA ovarian sex cord-stromal tumors with annular tubules. She underwent a left salpingo-oophorectomy and ipsilateral pelvic node biopsy with no adjuvant treatment. She was seen for amenorrhea after being lost to follow up for 16 years. The diagnosis of recurrence was made by radiology and the elevation of serum inhibin B level. The patient underwent resection of the tumor, left segmental colectomy, and paraaortic lymphadenectomy because the mass was massively adherent to the left mesocolon. Histology confirmed the diagnosis with no metastatic lymph nodes. No adjuvant therapy was indicated. The patient was lost to follow-up again for 4 years and re-presented for amenorrhea. Serum inhibin B level was high. A second recurrence was suggested, and the patient underwent a laparoscopic surgery. We performed left pelvic and paraaortic lymphadenectomy, and 3 months after surgery the patient was pregnant. Conclusion Sex cord-stromal tumors with annular tubules is a slow-growing ovarian tumor with a high potential for recurrence and metastasis. Surgery is the mainstay of treatment. Due to the rarity of these tumors, they are often unsuspected and thus incompletely staged before primary surgery; the diagnosis is made by histological examination. The prognosis of these patients is unknown, and they require long-term follow-up.

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Section: Discussionmentioning

confidence: 99%

Exceptional lymph node recurrence of an unusual ovarian tumor 16 years later: a case report

Sakhri,

Slimane,

Bouaziz

et al. 2024

J Med Case Reports

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“…9 The first choice for simple SCTAT is surgical resection. [10][11][12][13] In this case, due to the diagnosis of SCTAT combined with AGCT, uterine and bilateral appendages were resected and lymph node dissection was performed. In previous literature, patients with SCTAT combined with AGCT were also treated with uterine and bilateral appendage resection and lymph node dissection.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Peutz–Jeghers-Associated Sex Cord Tumor With Annular Tubules Combined With Unilateral Adult Granulosa Cell Tumor: A Case Report

Feng

Zhang

2023

Int J Surg Pathol

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Sex cord tumor with annular tubules (SCTATs) is a rare sex cord stromal tumor in the ovary. SCTAT combined with adult granulosa cell tumor (AGCT) is even rarer. Here, we report a unique case of ovarian tumors with mixed AGCT and SCTAT components. Due to the unusual coexistence, molecular testing was separately performed on each ovary. Both SCTAT and AGCT components were found to have STK11 germline mutation. Furthermore, the AGCT component had an additional FOXL2 somatic mutation. Based on medical history and molecular testing we conclude that the ovarian tumors were associated with Peutz–Jeghers syndrome (PJS). Thus, we present the first report of bilateral PJS-associated SCTAT combined with unilateral AGCT.

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“…And it was maybe not really related to the hermaphroditism. Due to the scarcity of cases, there is no established standard treatment regimen for SCTAT, and the initial treatment is surgery [ 14 ]. Qian et al [ 15 ] reported that SCTAT had a recurrence rate of 46.2%.…”

Section: Discussionmentioning

confidence: 99%

True hermaphroditism with sex cord tumor with annular tubules (SCTAT): a rare case report and review of the literature

Shi

Tang

et al. 2022

BMC Women's Health

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Background True hermaphroditism is a rare condition. It is defined as the presence of both testicular and ovarian tissues in the same individual. Sex cord tumour with annular tubules (SCTAT) is a rare stromal tumour of the sex cord that occurs mostly in the ovaries. Case presentation A 16-year-old girl presented to the gynaecology department with primary amenorrhea. Gynaecological examination revealed an enlarged clitoris that looked like a small penis. The chromosome karyotype was chimaera. The postoperative pathology confirmed true hermaphroditism with SCTAT. The patient underwent hormonal replacement after an operation and had no evidence of recurrence for 6 months. Conclusion Cases of true hermaphroditism with SCTAT are extremely rare conditions. Surgery and hormonal replacement are important for improving the prognosis of such patients.

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Non-Peutz–Jeghers syndrome-associated ovarian sex cord tumor with annular tubules treated by radiotherapy: a case report and literature review

Cited by 8 publications

References 13 publications

Exceptional lymph node recurrence of an unusual ovarian tumor 16 years later: a case report

Exceptional lymph node recurrence of an unusual ovarian tumor 16 years later: a case report

Bilateral Peutz–Jeghers-Associated Sex Cord Tumor With Annular Tubules Combined With Unilateral Adult Granulosa Cell Tumor: A Case Report

True hermaphroditism with sex cord tumor with annular tubules (SCTAT): a rare case report and review of the literature

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