Peritoneal metastases from mucinous endocervical adenocarcinoma

Abstract

Pseudomyxoma peritonei is a clinical condition characterized by mucinous tumors and mucinous ascites that accumulates in large volume and over time will interrupt gastrointestinal function. Pseudomyxoma peritonei most commonly arises from a mucinous neoplasm of the appendix (Sugarbaker et al., 1996). Also, occasionally this syndrome may arise from the colon or rectum, gallbladder, small intestine, ovarian teratoma, lung, breast, pancreas, fallopian tube, and urachus (Sugarbaker, 2012a). Described here are three patients presenting with mucinous peritoneal metastases arising from an adenocarcinoma of the endocervix. This cause of this rare condition is unclear; it may be caused by retrograde menstruation. Evaluation of these patients for cytoreductive surgery (CRS) and hyperthermic perioperative chemotherapy (HIPEC) at a peritoneal surface oncology treatment center early in the natural history of the disease is recommended. Patient presentation 1 A 33 year old woman presented in May of 2011 with chief complaints of abdominal distention and pain. A past history of cone biopsy of the cervix in 2002 showed pre-cancerous cells with no invasive malignancy. CT of the chest, abdomen, and pelvis showed a large volume of intraabdominal fluid consistent with combined mucinous and serous ascites (Fig. 1). Laparoscopy was performed showing bilateral adnexal masses, copious mucinous fluid, and peritoneal metastases on the abdominal wall, the right hemidiaphragm, and greater omentum. Pathology showed mucinous adenocarcinoma and immunostains suggested a cervical origin with P16 and CK7 positive and CK20 negative. Fig. 1 Abdominal and pelvic CT on a patient with adenocarcinoma of the endocervix. Paracentesis resulted in the drainage of 10 l of ascites. Upper and lower gastrointestinal endoscopy was not revealing of a cancerous process. She underwent a 9-hour complete CRS with greater omentectomy, appendectomy, right upper quadrant peritonectomy, lesser omentectomy, pelvic peritonectomy, hysterectomy, and bilateral salpingo-oophorectomy (Sugarbaker, 2012b). Pathology showed in-situ and invasive mucinous adenocarcinoma of the endocervix with metastases to ovaries, fallopian tubes, omentum, and peritoneum. The appendix was normal except for overlying extracellular mucin. She was treated with HIPEC and early postoperative intraperitoneal chemotherapy (EPIC) (Sugarbaker, 2012b). The patient was treated with a single cycle using cisplatin (50 mg/m2) and doxorubicin (15 mg/m2) by intraperitoneal administration at 42 °C. Continuous infusion intravenous ifosfamide was given over the 90 min of HIPEC. Fifteen minutes prior to infusion 2-mercaptoethane sulfonate NA (MESNA) at 260 mg/m2 was given as a bolus. The MESNA was repeated at 4 and 8 h after initiation of the HIPEC. Early postoperative intraperitoneal chemotherapy (EPIC) with paclitaxel at 20 mg/m2 in one liter of 1.5% dextrose peritoneal dialysis solution was used postoperative days one through five (Sugarbaker, 2012b). Systemic chemotherapy was given over five months using paclitaxel at 135 mg/m2 administered over 24 h on day one plus cisplatin 50 mg/m2 administered on day two. Treatment was every 21 days for six cycles. She remains asymptomatic on 6 monthly CT follow-up at 3 years.