A 44-year-old nulliparous Chinese female without personal or family history of disease was admitted to the Hubei cancer hospital in October 2021, who presented with weight loss, anorexia and hiccups for 3 months. On physical examination, an approximate 10cm, painless, mobile, suprapubic and right-pelvic mass was revealed. On inspection, occult blood was detected in stool, while preoperatively electronic gastroscopy and colonoscopy revealed no tumor lesion. The level of serum CA125 elevated to 187.6 umol/ml. Serum sex hormone and other tumor markers including AFP, CA199, CEA, β-HCG and inhibin were within normal range. Preoperative PET-CT, as shown in Figure 1, demonstrated a 7.8 x6.1x 6.3cm, solid and right-pelvic mass with SUVmax of 11.22, without clear boundary to adjacent intestine or right adnexa. Cervical cytology endocervical curettage was normal. The mass was diagnosed for ovarian tumor initially, followed by laparotomy.
The primary laparotomy revealed a 8 x7 x6cm, solid mass arising from distal ileum, separated from the right adnexa, and adherent to the adjacent mesentery and small intestine, resulting in intestinal stricture. The uterus, adnexa, omentum and peritoneum appeared grossly normal. 63cm small intestine with mesentery and the mass was resected merely in toto, and primary ileal mesenchymal tumor with whole intestinal layer invasion was suggested by the intraoperative frozen section. Secondary laparotomy was performed 3 weeks later, following postoperative pathologic confirmation of ileal GCT. No visible lesion was explored in abdominopelvic cavity, except several 1-2cm solid nodules in Douglas space. This surgery without tumor residue involved total hysterosalpingo-oophorectomy, omentectomy and pelvic floor peritonectomy. No evidence of tumor was identified in adnexa, uterus or omentum in microscopy, but only GCT metastasis was detected in Douglas space peritoneal nodules. Primary GCT of ileum was diagnosed by intraoperative exploration and postoperative pathology. Four cycles of postoperative intravenous-chemotherapy with paclitaxel and cisplatin was carried out. The patient remained only one month of disease free when metastatic tumor developed in liver.
Postoperative pathology revealed the primary GCT of ileum. Para-cancerous normal mucosa glands of ileum shown in Figure 2a, suggesting the location of GCT in ileum. Microfollicular containing eosinophilic material, gland-like arrangement with pyknotic nuclei and resembling Call–Exner bodies were noted in tumor tissue in Figure 2b,c. The nuclei of the majority of tumor cells were formed of pale chromatin with nuclear grooves shown in Figure 2d. Sparse reticulin staining around perivascular area was shown in Figure 3a. Immunohistochemistry (IHC) results: CD56 (+), CD99 (+), Inhibin (+), Ki-67 (+, Li: 10%), Vimentin (+), CK7(-), EMA (-), Desmin (-), and the positive IHC were shown in Figure 3b,c,d,e,f. Bilateral adnexa were entirely submitted for pathologic review using 3- to 5-mm sections. No GCT cell was identified.