A Patient with Pseudomyxoma Peritonei

Abstract

Purpose: Pseudomyxoma peritonei is a condition characterized by diffuse collections of gelatinous material in the abdomen and pelvis. We report a patient with pseudomyxoma peritonei. Methods: A 62 year old African American lady presented to the ER with abdominal pain and vomiting. She denied fever, diarrhea, hematochezia, recent travel or sick contacts. Past medical history was significant for hysterectomy with bilateral salpingo-oophorectomy. Family history was significant for sister having breast cancer. Physical exam revealed a distended, non-tender abdomen. Labs revealed a normal CBC and biochemical profile. CT scan of abdomen/pelvis showed large amount of ascites with mass effect and omental caking. 1 ml of gelatinous liquid was obtained by CT guided paracentesis and it was negative for malignant cells. EGD and colonoscopy were found to be normal. Patient was lost to follow-up for 1.5 years and came back for increasing abdominal girth and palpable masses in the left upper and right lower quadrants. CT scan showed an increase in ascites and omental caking with mass effect on liver and spleen leading to scalloped appearance suggestive of pseudomyxoma peritonei. Ultrasound guided FNA of peritoneal mass showed atypical cells in mucinous background suspicious for mucinous carcinoma. Exploratory laparotomy revealed septate gelatinous masses with matted bowel loops. Biopsies were reported as metastatic mucinous adenocarcinoma with nuclear features borderline for malignancy. Immunohistochemistry was consistent with an appendiceal primary. Results: Patient was diagnosed with pseudomyxoma peritonei and was advised debulking surgery. Conclusion: Treatment of pseudomyxoma peritonei is surgical debulking with addition of heated intraperitoneal chemotherapy for malignancies. Prognosis for low-grade adenomatous mucinous epithelium with abundant extracellular mucin is significantly better than for peritoneal mucinous carcinoma.Figure: CT scan showing scalloped liverFigure. Histopathology