Pseudomyxoma peritonei: a clinical case of this poorly understood condition
A 76-year-old woman presented to our emergency department (Hospital Professor Doutor Fernando Fonseca, Amadora, Portugal) with dyspnea, increased abdominal girth, and peripheral edema for 2 weeks. Her medical history included diabetes mellitus type 2, hypertension, and dyslipidemia. She was medicated with ramipril, furosemide, aspirin, simvastatin, rabeprazole, and lorazepam.\nThe physical examination revealed tachypnea, a blood pressure of 144/82 mmHg, a pulse rate of 83 beats per minute (bpm), and apyrexia. Expiratory time was increased, and there were rales at the base of the lungs. The cardiovascular system did not reveal any remarkable changes. There was collateral venous circulation on the abdomen wall, the wall was slightly depressible, and percussion revealed tympanic resonance in the center of the abdomen and dullness in both lumbar regions. There was no palpable organomegaly or lymphadenopathy. Peripheral edema was present.\nLaboratory investigations showed an absence of leukocytosis (8,400 leukocytes/μL) with neutrophilia (6,100 neutrophils/μL) and a C-reactive protein value of 6.21 mg/dL. The patient’s erythrocyte sedimentation rate was 88 mm, her carcinoembryonic antigen level was 177 ng/mL (0–3 ng/mL), her cancer antigen 15–3 level was 55 U/mL (0–30 U/mL), her cancer antigen 125 level was 57.5 U/mL (0–35 U/mL), and her cancer antigen 19.9 level was lower than 2.5 U/mL (0–37 U/mL).\nParacentesis was attempted but was unsuccessful. Abdomen ultrasonography and computed tomography showed images that were suggestive of pseudomyxoma and moderate, multiseptate ascites ().\nThe patient was submitted to laparotomy with right oophorectomy, omentectomy, and pseudomyxoma debulking (). At surgery, 6,200 g yellow-orange gelatinous material () was removed. The histological examination of biopsies (from small intestine, ovary, peritoneum, and epiploon) revealed mucinous neoplastic cells and diffuse intra-abdominal mucin, characteristics that allowed the diagnosis of PMP.\nThe morphological () and immunophenotypic (cytokeratin 7 focally positive [], cytokeratin 20 diffusedly positive [], and p16 negative []) patterns were compatible with colorectal/appendicular origin.\nIn the postoperative course, the patient had hemodynamic instability that reverted with fluid replacement and amines for a short period.\nShe was discharged in a satisfactory condition with follow-up at the oncology division. Because of her functional status, the patient has not received chemotherapy. The treatment is being based on symptomatic relief.\nTwo years and 8 months later, she has referred occasionally with postprandial fullness and moderate ascites. Her carcinoembryonic antigen and cancer antigen 125 levels are 133 ng/mL and 139 U/mL, respectively. The last image exam (abdomen ultrasonography) showed a small volume of perihepatic, perisplenic, and interloop fluid with peritoneal leaflets thickening. It was possible to identify a mass with 58 × 30 mm in the left parietocolic groove, indicative of peritoneal carcinomatosis (). Enlarged ganglia were also seen, the biggest of which was at the hepatic hilum, at 32 mm ().