A 36-year-old Hispanic woman had an appendicitis-associated appendectomy at 6 years of age, and post-Cesarean section uterine atony-related total abdominal hysterectomy at age 26 years. Other medical, surgical, or family histories were unremarkable.
The patient was seen with a 6-month history of abdominal distention and discomfort. At physical examination, no abdominal masses or ascites were detected. A pelvic ultrasound (US) was performed, and an adnexal cystic mass of 19 × 16 × 12 cm with solid component was observed in the area of the left adnexa. There was no ascites or any other abnormalities observed in the pelvic cavity. Serum CA-125 was 4.13 U/ml, and routine preoperative laboratory tests were obtained and reported as normal, as was the chest x-ray. A CT scan was conducted as part of the preoperative evaluation; the scan confirmed the US findings and revealed solid areas in a cystic tumor (Figures 1a and 1b).
An exploratory laparotomy was performed with the diagnosis of a suspicious malignant adenexal mass. During the procedure, a tumor measuring 19 × 16 × 12 cm was found in the left retroperitoneal space, the tumor completely separated from the ovaries or other intrapelvic structures. Complete and intact removal of the lesion was obtained; this was sent for frozen section, and was subsequently reported as mucinous cystadenocarcinoma. At the time of the surgery, both ovaries and fallopian tubes were grossly normal, as were all intra-abdominal structures. The patient's postoperative course showed no complications or adverse events. Six courses of adjuvant chemotherapy were administered with carboplatinum at a dose of 6 area under the curve (AUC) and paclitaxel at 175 mgs/m2. CA-125 was obtained after the fifth chemotherapy course and was reported as 5.3 U/ml. A pelvic US was performed 8 months later, and a new pelvic mass measuring 7 × 6 × 6 cm at the same site was found. An exploratory laparotomy was conducted and a sigmoid resection was required due to a mass involving this. Infracolic omentectomy was also carried out due to multiple nodules. The surgery was considered optimal cytoreduction with no visible lesion remaining. The Pathology report stated mucinous cystadenocarcinoma.
Second-line chemotherapy was initiated with oral etoposide (50 mg daily for 21 days). After one cycle, disease progression was identified and the patient decided to stop chemotherapy. Tamoxifen (20 mg/day) was instituted as palliative management.
On gross examination, an ovoid, well-defined tumor with measurements of 19 × 16 × 12 cm with smooth grey surface was received. Internal surface comprised a multilocular mass with thin walls and mucinous material only, while a small area exhibited solid nodules in the wall (Figure 2). Microscopically, tumor walls were covered with a single line of mucinous cells with small basal nuclei and mucinous cytoplasm. In addition, ovarian-like stroma was identified in the wall. These epithelial areas showed transitions with intraepithelial carcinoma (Figure 3) and areas of borderline mucinous tumor; high-grade adenocarcinoma with dedifferentiation in desmoplastic stroma was identified. High-grade adenocarcinomatous component infiltrated the tumor capsule was seen.
The recurrent tumor's surgical specimen comprised a multiorgan pelvic resection with an ill-defined white mass with gross infiltration to muscle and fatty tissue of the pelvis wall, left ovary, fallopian tube, omentum, and in wall of the colon, without lesion in the mucosa. Microscopically, high-grade adenocarcinoma similar to high-grade areas of the previous lesion was identified, the ovary demonstrating direct infiltration from the abdominal mass.
The patient, a 21-year-old female with no remarkable previous medical or surgical history complained of diffuse abdominal discomfort 1 month prior to the patient's presentation at the Emergency Service due to acute abdominal pain and intestinal occlusion-compatible clinical data. Abdominal and pelvic US was performed, revealing a cystic mass with solid areas (Figure 4). An emergency laparotomy was performed; during the surgery, a retroperitoneal tumor measuring 26 × 18 × 16 cm was observed. The latter was totally removed, ascitic fluid was detected and drained, and cytology was reported as negative for malignant cells. The tumor was located near the pancreas, but was not attached to this organ or to any other intra-abdominal or pelvic structures. Ovaries, uterus, colon, and appendix were macroscopically normal, as were other intra-abdominal structures. Postoperative serum CA-125 was 105 U/ml (0–21), while CA-15-3 was 32.2 U/ml (0–53) and CA-19-9 was 5.4 U/ml (0–37).
Systemic adjuvant chemotherapy was proposed; nonetheless, the patient refused this treatment. Therefore, close follow-up was advised, and the patient has been seen at the Medical Oncology Service over the last 6 months with serial CA-125 serum levels at latest measurement of 13.3 U/ml. An abdominal CT was performed, and no evidence of disease was found.
Grossly, the tumor was a well-defined multilobular mass of 26 × 18 cm. Cut surface exhibited a multicystic tumor with thin walls and solid white areas. Microscopically, the lesion was a mucinous tumor with well-differentiated mucous glands with cribiform or papillary architecture, and cells had pseudo-stratified, large regular nuclei with mucinous cytoplasm, these areas demonstrating transition to cystic areas lined by a single line of mucinous cells without atypia. No capsular invasion was identified.