The patient was an Asian female infant with a family history of nephroblastoma (father) and retinoblastoma (sibling). Her perinatal status was normal, and her medical history was unremarkable. The patient’s mother accidentally observed vaginal bleeding in her 7-month-old daughter. Her previous doctor prescribed antibiotics; however, no improvement was observed. The patient was referred to a tertiary hospital where she was diagnosed with a vaginal tumor. She was then referred to our hospital at 9 months of age (day 0).
Ultrasonography, enhanced computed tomography (CT), magnetic resonance imaging (MRI) (Fig. 1), and laboratory tests (including tumor markers) were performed. Distant or lymph node metastases were not detected. AFP was the sole abnormal tumor marker. The patient was clinically diagnosed with a YST arising from the vagina. A small incisional biopsy through the vaginal opening was performed on day 19 under general anesthesia, and the tumor was pathologically confirmed to be a YST with TNM staging T2bN0M0 and Children’s Oncology Group (COG) staging III.
After her parents provided informed consent, she was treated with six courses of neoadjuvant JEB regimen, which included etoposide, bleomycin, and carboplatin from day 27. The preference of carboplatin over cisplatin was determined by her parents who wanted to minimize renal toxicities after the family history of pediatric cancers including kidney tumor.
Four months after the primary chemotherapy, MRI revealed a shrunken, localized residual tumor. Her AFP declined logarithmically to 9.6 ng/mL on day 173 after completion of six cycles of JEB (cumulative agents per square: carboplatin 3600 mg, etoposide 2160 mg, and bleomycin 90 mg) (Fig. 2). Endoscopic evaluation and incisional biopsy were performed on day 156 revealing a wide-based yellowish tumor on the posterior vaginal wall. The pathology of the sample revealed approximately 10% viable tumor. After obtaining informed consent, anti-tumor treatment was completed, and the patient was followed under close observation with an expectation that the residual tumor undergoes differentiation and achieves remission.
Nevertheless, there was an elevation in the AFP level after a month of observation (day 191) suggesting that the first-line chemotherapy was insufficient. Therefore, surgical intervention was recommended. Another incisional biopsy was performed on day 198, which confirmed the tumor viability. The ifosfamide, carboplatin, and etoposide (ICE) regimen was initiated as the second-line chemotherapy on day 201. After two cycles of ICE, the AFP declined to 8.7 ng/mL on day 250. CT and MRI revealed a tumor on the posterior wall, adjacent to the cervix (Fig. 3). There was neither distant nor lymph nodes metastasis, and the anterior wall of the rectum was free from direct tumor invasion. Since the tumor was chemotherapy-resistant, we decided on complete resection of the tumor after obtaining informed consent on day 261.
Surgery began with vaginal evaluation using an Olympus GIF-PG260 endoscope (Olympus Corp., Tokyo, Japan). We confirmed that the tumor was confined to the posterior vaginal wall (Fig. 4a). The edge of the tumor was approximately 1 cm away from the cervix, and it was approximately 3 cm long and 3 cm wide, positioned from 3 o’clock on the left wall to 7 o’clock on the right wall. The patient was then fixed in the lithotomy position to secure the working space for intraoperative endoscopic investigation. A 5-mm cam port was inserted, and the intra-abdominal cavity was investigated. Laparoscopy confirmed the absence of dissemination of the tumor, nodules on the omentum, and swollen lymph nodes. A small amount of ascites was detected, but the cytology was negative. The top of the uterus was ligated to the abdominal wall to secure the space behind it. Laparoscopy provided good visualization of the area posterior to the vagina, and separation between the vagina and the anterior wall of the rectum was performed (Fig. 5a–d). During the laparoscopic procedure, the extent of the tumor was endoscopically confirmed, and marking sutures were placed on both proximal and distal ends of the tumor, penetrating through the vaginal wall to the pelvic cavity (Fig. 4b). Thus, the marker was identified laparoscopically, and the extent of the tumor was confirmed. No direct invasion of the tumor into the rectum was observed. The lateral side of the vagina was separated to mobilize the posterior half of the vagina.
The patient was then placed in the jackknife position, and a 5-cm posterior sagittal incision was made (Fig. 6a); this approach is similar to posterior sagittal anorectoplasty (PSARP) for the correction of an imperforated anus . The rectum was identified and taped after dissection through the subcutaneous and muscular layers (Fig. 6b). It was easy to separate the lateral side of the rectum because most of the separations had already been performed laparoscopically. The vagina and markers were easily identified by pushing the rectum aside, and the tumor was resected with direct visualization of the markers, vaginal wall, and tumor (Fig. 6c). After complete resection of the tumor from the vagina, we reconstructed the vagina with intermittent sutures on the posterior wall (Fig. 6d). A 14-Fr soft plastic tube was inserted to secure the cavity. The rectum was placed back, the dissected muscle and subcutaneous tissues were sutured in layers, and the incision was closed.
Pathological examination of the resected tumor confirmed complete resection of the tumor with viable tumor cells without invasion of the vertical or horizontal margins. After recovery from surgery, the patient received the third and fourth cycles of the ICE regimen as adjuvant therapy. AFP levels expectedly dropped and remained within the normal range (Fig. 2).
At each step of the treatment, the tumor board was held to discuss how to manage the patient including the regimen of chemotherapy and the surgical strategy. No complications of definitive surgery other than an episode of urinary tract infection, possibly due to temporary urinary incontinence, were observed.
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