Rhabdomyosarcoma Requiring Ovarian Transposition Release for Recurrent Severe Ovulation Pain Following Laparoscopic Ovarian Transposition: A Case Report.

The patient was referred to our hospital at the age of 4 years with complaints of genital bleeding and a vaginal mass. Biopsy of the cervix revealed an increase in short, spindle‐shaped, and round tumor cells with mitotic figures in the subepithelial stroma, leading to a diagnosis of RS. Magnetic resonance imaging (MRI) revealed a 5‐cm tumor originating in the cervix that filled the vagina. Chemotherapy was administered, resulting in remission. The chemotherapy regimen consisted of vincristine (46.5 mg/m 2 ), cyclophosphamide (8.4 mg/m 2 ), and actinomycin‐D (1750 mg/m 2 ) combined with vincristine (1.5 mg/m 2 ) and irinotecan (50 mg/m 2 ). At the age of 5 years, the cancer recurred, and transvaginal resection was performed. Pathological examination of the resected tissue revealed positive margins, indicating a high probability of residual cancer. A multidisciplinary team recommended pelvic proton therapy (PPT). To preserve ovarian function, laparoscopic OT was performed at the age of 6 years. Figure  1 presents the intraoperative images obtained during the initial surgery and schematic illustrations. Under general anesthesia, a 10‐mm umbilical camera port and a 5‐mm trocar were inserted in the suprapubic region, with two additional trocars placed in the bilateral lower abdomen at the same level. The bilateral ovarian ligaments and isthmus of the fallopian tubes were resected, and the bilateral mesovarium and peritoneum surrounding the infundibular pelvic ligaments were incised bilaterally to enhance ovarian mobility (Figure  1a ). Both ovaries were grasped using atraumatic forceps and mobilized to the iliac crest level, ensuring that the ovarian blood supply remained intact and was not kinked or compromised. The transpositioned ovaries and fallopian tubes were sutured to the peritoneum outside the lower paraperitoneal groove, adjacent to the descending colon, near the level of the iliac fossa, using nonabsorbable sutures (Figure  1b,c ). Hemoclips were attached to the pelvis of both ovaries as radiological markers. Figure  2 presents the pelvic radiograph and radiation simulation images obtained after laparoscopic OT. The clips affixed to both ovaries were confirmed to be positioned outside the pelvic cavity (Figure  2a ). Figure  2b illustrates the radiation simulation plan, highlighting the transposed ovaries and their optimal placement relative to the intended radiation field. Proton beam therapy was initiated after OT, with a total dose of 50 Gy. The calculated median (range) radiation dose to the ovaries was 1.4 (0.4–2.4) Gy, accounting for 2.3% of the total irradiation dose. The estimated radiation dose to the uterus was 41.4 Gy. Six years after OT, the patient (now aged 11 years and 8 months) presented to the emergency department on 3 consecutive days with complaints of intermittent, severe right lower abdominal pain that impaired ambulation. Although the patient experienced vomiting, blood tests revealed no signs of inflammation, thereby excluding enteritis as the likely diagnosis. Tenderness with muscular guarding was noted near the area where the right adnexa was fixed. Both ultrasonography and contrast‐enhanced computed tomography (CT) revealed no abnormal findings, and the cause of pain remained unclear (Figure  3 ). Table  1 presents a summary of the pain symptoms of the patient (including the frequency of pain, emergency room visits, and hormone levels), timing of abdominal ultrasound, CT, and abdominal MRI. At 1 month after the initial onset of abdominal pain, the luteinizing hormone (LH), follicle‐stimulating hormone (FSH), and estradiol (E2) levels were 2.1 mIU/mL, 2.1 mIU/mL, and 83 pg/mL, respectively. The decrease in FSH levels and increase in E2 levels were suggestive of ovulation onset. Figure  3 presents the T2 MRI scan recorded during the outpatient consultation at the age of 11 years and 11 months, which revealed that the right ovary was larger than the left ovary owing to the presence of a corpus luteum in the right ovary. Over the next 3 months, the patient experienced intermittent lower abdominal pain once a month. At 12 years of age, the patient visited the emergency room because of persistent pain lasting for 3 consecutive days. In the following month, the patient was transported by ambulance with similar symptoms. Blood tests showed post‐ovulatory findings (LH: 3.7 mIU/mL; FSH: 2.8 mIU/mL; E2: 23.1 pg/mL; and progesterone: 2.2 pg/mL). However, the intensity of the pain was disproportionate to that of typical ovulation pain, which complicated the definitive diagnosis. Low‐dose estrogen–progestin (LEP) is an option for treating ovulation pain. Owing to the young age of the patient, we were cautious regarding prescribing LEP without a definitive diagnosis. On the last visit to the emergency department, the pain was extremely severe and analgesics were ineffective, resulting in urgent diagnostic and therapeutic laparoscopic surgery. Intraoperative images and illustrations from the first surgery. (a) The ovarian ligament and isthmus of the fallopian tube were resected at the points indicated by double‐headed arrows. (b) Position of the right ovary: The right ovary and fallopian tube were sutured to the peritoneum outside the subperitoneal groove using nonabsorbable sutures as far from the pelvis as possible. Clips were attached to the upper and lower ends of the ovary. (c) Position of the left ovary: The left ovary and fallopian tube were sutured to the peritoneum above the iliac fossa using nonabsorbable sutures. Pelvic radiograph and radiation simulation images after laparoscopic ovarian transposition. (A) Pelvic radiograph following laparoscopic ovarian transposition. The clips attached to both ovaries (a, b) are located outside the pelvis. (B) Radiation simulation of the transposed ovaries (c, d) and their optimal positioning relative to the planned radiation field. Abdominal ultrasound, contrast‐enhanced CT, and MRI were performed prior to emergent ovarian transposition release. (a) Abdominal ultrasound findings at the initial visit to the emergency room for abdominal pain (11 years, and 8 months). (a) Right ovary. (b) Left ovary. (b) Contrast‐enhanced CT scan at the initial emergency visit for abdominal pain (11 years and 8 months). No abnormal findings, including ovarian torsion, were noted. (c) T2 MRI scans were performed during outpatient consultation at the age of 11 years and 11 months. A corpus luteum was identified in the right ovary (→), which appeared larger than the left ovary. CT, computed tomography; MRI, magnetic resonance imaging. Clinical course, hormonal blood test, and imaging results leading to emergent ovarian transposition release. Figure  4 shows intraoperative images. The uterus appeared enlarged compared with that during the first surgery (Figure  4c ), and no signs of ovarian torsion were observed. The right adnexa appeared more suspended ventrally on the abdominal wall than the left (Figure  4a,b ), suggesting that ovulatory stimulation and the increased weight of the ovary contributed to the peritoneal irritation symptoms. The adhesions around the infundibular pelvic ligament were carefully dissected, and the right ovary was repositioned closer to the uterus (Figure  4d ). Intraoperative images of ovarian transposition release surgery. (a) Left adnexa. (b) Right adnexa: The right adnexa is suspended more ventrally on the abdominal wall than on the left side. (c) The uterus appeared larger than it was during the first surgery. (d) The right ovary was positioned close to the uterus. The left infundibular pelvic ligament strongly adhered to the sigmoid colon, preventing the left ovary from being lowered into the pelvis; instead, it was positioned in the iliac fossa. The patient's postoperative course was uneventful, with no recurrence of abdominal pain.

This study was exempted from ethical approval as the Institutional Review Board does not require approval for case reports.

To the best of our knowledge, this is the first case report to document an OT release procedure performed to relieve the onset of ovulation pain following laparoscopic OT in childhood. In this case report, the patient initially achieved remission following chemotherapy, but later experienced disease recurrence, necessitating pelvic radiation therapy. The chemotherapy regimen included cyclophosphamide, which led to ovarian failure. A cumulative cyclophosphamide dose of 3010 mg/body (4703 mg/m 2 ) was administered. According to the 2013 American Society of Clinical Oncology guidelines [ 5 ], the risk of ovarian failure is considered low with a cyclophosphamide dose of < 8000 mg/m 2 . Accordingly, the risk of ovarian failure in this patient was determined to be low, and anti‐Müllerian hormone (AMH) levels were not measured. However, in similar cases in the future, measuring AMH would be advisable to assess ovarian function prior to OT [ 6 ]. In cases of pelvic irradiation with 50 Gy, ovarian cryopreservation or OT is necessary to preserve ovarian function. While ovarian cryopreservation does not allow for the secretion of female hormones until the ovaries are re‐implanted, OT enables the secretion of endogenous hormones, beginning with the onset of ovulation, thereby promoting natural puberty. Therefore, OT was selected for this patient. Additionally, the FertiPROTEKT network guidelines recommend combining OT with ovarian tissue cryopreservation in women undergoing pelvic radiotherapy to preserve both endocrine function and fertility, considering factors such as patient age, cancer type, and urgency of treatment [ 7 ]. In the present case, ovarian tissue cryopreservation should have been considered during OT or OT release. Therefore, in the future, a combined approach involving simultaneous OT and ovarian tissue cryopreservation should be considered as a comprehensive fertility preservation strategy. Two types of approaches can be used for OT [ 7 ]. In the medial approach, one or both ovaries were fixed behind the uterus, leaving the ovarian ligaments and mesosalpinges intact. In the lateral approach, the utero‐ovarian ligaments are resected, and the ovaries are mobilized on the infundibulopelvic ligaments. The fallopian tubes may either remain intact to preserve the possibility of spontaneous pregnancy or be separated from the uterus and transposed alongside the ovaries. In our patient, the medial OT was not selected because of the need for uterine irradiation. Additionally, the ovaries were transposed along with the fallopian tubes to preserve their function, considering the possibility of a future uterine transplant. Medial OT is recommended for patients with nongynecological cancers. It is especially suitable for patients with Hodgkin lymphoma because the radiation field primarily targets the lateral and anterior pelvic compartments, making the posterior midline space a relatively safer location for ovarian repositioning. Moreover, this approach preserves the fallopian tubes, therefore enabling spontaneous conception. A systematic review and meta‐analysis involving 323 women revealed that 71 (22%) patients underwent medial OT, while 252 (78%) patients underwent lateral OT (127 with non‐gynecologic tumors and 125 with gynecologic tumors) [ 8 ]. The medial approach was predominantly used in patients with non‐gynecologic cancers such as Hodgkin lymphoma. The comparison between medial and lateral OT did not show significant differences in terms of preserving endocrine function (odds ratio [OR], 0.65; p  = 0.120). However, the medial approach demonstrated favorable reproductive outcomes. The pregnancy (49.2%) and live birth (45%) rates following medial OT were significantly higher than those following lateral OT (6.5% and 13.4%, respectively). These differences were statistically significant (OR, 7.04; p  = 0.001; OR, 5.29; p  = 0.003, respectively). Therefore, for non‐gynecological cancers, the medial approach is recommended [ 8 ]. A major difference between children and adults is that they continue to grow. Therefore, the possibility that postoperative growth and ovulation onset may affect the long‐term outcomes of surgery must be considered. In this case, the onset of ovulation was challenging to determine because of amenorrhea caused by post‐irradiation endometrial dysfunction. Additionally, the pain was severe and unresponsive to analgesics, which is uncommon in ovarian pain. Ovarian function is typically assessed based on the patient's symptoms and serum FSH levels. It is generally considered preserved if the serum FSH levels are  50 pg/mL, and ultrasonography shows the presence of follicles [ 9 ]. Additionally, AMH measurement is recommended as a marker for evaluating ovarian reserve following chemotherapy [ 6 ]. In this case, the presence of the corpus luteum was confirmed by MRI, and gross findings of the ovary and hormone levels indicated that ovarian function was preserved through OT. The clinical findings suggest that the uncontrollable abdominal pain during ovulation was due to the weight of the enlarged ovary and ovulatory stimulation, which exerted mechanical strain on the abdominal wall and triggered peritoneal irritation. Although LEP therapy is the standard first‐line treatment for ovulation‐related pain, it was not initiated in this case because a definitive diagnosis could not be established during outpatient follow‐up, and the young age of the patient warranted a cautious approach. The diagnosis was confirmed during emergency laparoscopic surgery, which led to prompt resolution of symptoms. Lambert et al. reported on 16 prepubertal patients who underwent temporary OT surgery [ 10 ]. Their median (range) age at surgery was 3 (range: 2–9) years. In this approach, the ovarian ligament and fallopian tubes were not resected or separated but were moved above the iliac crest. The sutures were tied outside the body of the patient and affixed to the anterior abdominal wall using nondissolving sutures. Following irradiation, the sutures were untied in all patients, and the ovaries returned to their normal position within the pelvis. No intraoperative or postoperative complications were noted. In children, this method is particularly suitable for short‐term radiation therapy (8 days), such as brachytherapy for urinary tract RS and giant cell tumors [ 10 ]. However, if long‐term radiation therapy is required (5–6 weeks), peritoneal adhesions may prevent the ovaries from returning to their normal position in the pelvis [ 3 ]. This method was not used in this case as the patient required 6 weeks of radiotherapy. The safety and efficacy of OT for gonadal function preservation in adults have been the focus of several studies. The most common complications include torsion of the ovarian vessels, chronic ovarian pain, tubal infarction, and development of benign ovarian cysts [ 3 , 4 ]. In a large meta‐analysis, 5% to 16% of patients with gynecological cancer developed ovarian cysts after OT and were treated conservatively or surgically [ 11 ]. Moris et al. reported that after performing OT surgery in 107 patients with cervical cancer (average age, 33 years), benign ovarian cysts were found in 22 patients (23%) [ 9 ]. These cysts were diagnosed via ultrasonography in patients presenting with abdominal pain, and treatment with oral contraceptive pills effectively resolved the issue in 19 patients. Surgical intervention (laparoscopy in one patient) was required to treat persistent cysts in three other patients (one with a luteal cyst and two with benign mucinous and serous cysts). Of the 107 patients, three (2.8%) experienced chronic abdominal pain in the region of the displaced ovary, although no cysts were detected on radiography. One patient required surgery for mechanical intestinal obstruction caused by the formation of an adhesion band between the ovarian membrane and the displaced right ovary [ 9 ]. In this case, pain was observed once a month on a cyclical basis. Surgical findings revealed a corpus luteum; however, no ovarian cysts, torsion, or adhesions were observed. The right adnexa were suspended more ventrally on the abdominal wall than on the left. Therefore, the cause of the pain was considered to be the combined effect of peritoneal stimulation due to ovulation and increased ovarian weight during luteal formation. Arian et al. described a laparoscopic OT technique that uses a unique feature known as the ovarian tunnel, which maintains the retroperitoneal position of the ovarian blood vessels, potentially preserving the ovarian blood supply [ 12 ]. This method positions the ovary more dorsally, making it a technique worth considering. Data on whether the timing of OT administration before or after puberty affects ovarian function preservation are limited. Valduga et al. investigated 32 patients with cancer who underwent OT before the age of 26 years [ 13 ]. Following treatment, the pregnancy rate was 60% (6/10), while the rate of spontaneous menstrual cycle recovery was 74% (17/23) [ 13 ]. The authors emphasized that the effectiveness of OT in restoring ovarian function does not significantly differ when performed before puberty or during puberty [ 13 ]. The risk of acute ovarian failure increases with higher radiation doses to the ovaries and decreases with increasing age at the time of treatment. According to previous studies, when radiation is administered at birth, doses of 20.3, 18.4, 16.5, and 14.3 Gy are associated with an elevated risk of acute ovarian failure within 5, 10, 20, and 30 years, respectively [ 14 ]. Considering that the current patient was scheduled to receive 50 Gy of pelvic radiation at the age of 5 years, the risk of acute ovarian dysfunction was considered high, justifying the indication for OT. In a separate case series involving 16 prepubertal patients who underwent temporary OT prior to brachytherapy (median age at surgery: 3 years; range: 2–9 years), the median radiation dose delivered to the ovaries was 1.4 (range: 0.4–2.4) Gy [ 10 ]. The success rate of ovarian translocation surgery in pediatric populations is difficult to determine due to the limited number of well‐conducted studies; however, it is estimated to be between 60% and 90% [ 3 , 11 , 13 , 15 ]. A meta‐analysis of 29 studies (1160 patients) confirmed that OT is a safe and effective fertility‐preserving option in cervical cancer. OT preserved ovarian function in 91%–93% of patients undergoing surgery with or without brachytherapy, with low cyst and metastasis rates. Preservation dropped to 61% with external beam radiotherapy [ 15 ]. In this case, the calculated median radiation doses delivered to the ovary were 1.4 Gy on the right side and 1.3 Gy on the left side (range: 0.4–2.4 Gy). Given this radiation dose, the preservation of both ovaries remains a feasible outcome. In conclusion, patients should be informed that OT release may be required because of increased pain during ovulation as a postoperative complication. Dorsal fixation of the ovary may help prevent peritoneal irritation during ovulation.

This study was conducted in accordance with the ethical guidelines of the Declaration of Helsinki. The patient provided informed consent before inclusion in this case report.

In Japan, approximately 2500 children of 0–14 years of age are diagnosed with childhood cancer annually [ 1 ]. The age‐standardized incidence rate is 166.6 per million‐person years in children of 0–14 years of age [ 1 ]. Rhabdomyosarcoma (RS) is the most common soft tissue sarcoma in children. Although the incidence of RS remains unknown, approximately 102 patients with soft tissue and other extraosseous sarcomas are reported annually in Japan [ 1 ]. For a tumor developing in the uterus or vagina, a tailored approach combining surgery, radiation therapy, and chemotherapy is considered to preserve fertility [ 2 ]. Ovarian transposition (OT) is a surgical procedure performed to preserve ovarian function, particularly in patients undergoing pelvic or abdominal radiation therapy [ 3 ]. This procedure relocates the ovaries outside the radiation field to minimize damage. Common complications include injury and torsion of the ovarian blood vessels, chronic ovarian pain, benign ovarian cysts, and endometriosis [ 4 ]. However, OT release due to worsening ovulation pain has not been previously reported. Here, we report a case of laparoscopic OT release for recurrent severe lower abdominal pain that occurred 6 years after the initial laparoscopic OT.

The authors declare no conflicts of interest.