H Devalia, S Nagral, R Khemani, A Pandit
Department of Surgery, Seth GS Medical College, Parel, Mumbai.
Department of Surgery, Seth GS Medical College, Parel, Mumbai.
A middle aged male patient presented with gradual distension of the abdomen. Imaging modalities showed classical features of pseudomyxoma peritoneii which was confirmed by aspiration cytology. Details of the case are described and relevant literature is reviewed.
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Devalia H, Nagral S, Khemani R, Pandit A. Pseudomyxoma peritoneii. J Postgrad Med 1997;43:21-2
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Devalia H, Nagral S, Khemani R, Pandit A. Pseudomyxoma peritoneii. J Postgrad Med [serial online] 1997 [cited 2023 Jan 28 ];43:21-2
Available from: https://www.jpgmonline.com/text.asp?1997/43/1/21/418
Pseudomyxoma peritoneii (PMP) is a rare condition and has been defined as a clinical entity characterized by the presence of mucinous ascites from grade I cystadenocarcinoma, redistribution of tumour within the abdominal cavity to predictable anatomic sites and an origin usually from appendix or ovary. PMP arising from colon, breast, endometrium, pancreas, common bile duct carcinoma and urachus have also been reported. We report a case of PMP, which showed classical features on imaging and was confirmed by histology.
A forty-five years old male presented with gradual distension of abdomen for two months not accompanied by fever, abdominal pain or weight loss. There was history of occasional vomiting not related to meals. On examination, the patient had multiple ill-defined lumps palpable all over the abdomen. The abdomen was non-tender, dull to percussion and there was no fluid thrill. The liver and the spleen were not palpable. Blood investigations including haemoglobin, complete blood count and liver function tests were within normal limits. A plain film of the abdomen was normal. A Barium series of the small bowel was essentially normal except for extrinsic mass effects on the ileum. A Barium enema was normal. The appendix could not be visualised. Ultrasonography (USG) showed septated ascites with numerous ‘suspended echoes’ that did not move as the patient changed positions. A computerised tomography (CT) scan showed anteriorly situated septated ascites with scalloping of hepatic margin from extrinsic compression of liver [Figure:1]. Both the USG and CT scan findings were classical of PMP.
A fine needle aspiration cytology (FNAC) of the abdominal mass revealed plenty of mucin with low grade cystadenocarcinoma cells [Figure:2] confirming the diagnosis of PMP. Patient was offered surgery, which he refused. He has been lost to follow up.
PMP is a low grade carcinoma whose biology is characterized by a redistribution phenomenon with large amount of extracellular mucin. Although the distant spread like metastasis to lungs is rare, local invasion can occur at a superficial level on the surface.
The common presentation of PMP is pain in abdomen, distension of abdomen and/or a lump in abdomen. Fever, anorexia, nausea, vomiting and weight loss may be associated. Thus the clinical features are vague and in our country could be mistaken for abdominal tuberculosis. The site of tumour origin is most commonly the appendix or ovary. It has been suggested that PMP is an independent primary neoplasm that develops as a result of neoplastic field change that affects colonic type epithelium. Histologically they are mucin producing low grade cystadenomas or cystadenocarcinomas. The low grade of the carcinoma may account for indolent behaviour, lack of distant metastasis and frequent long-term survival associated with PMP. The common sites of tumour load are the greater omentum (Omental Cake), the undersurface of the right hemidiaphragm, the right retrohepatic space, the left abdominal gutter, at the ligament of Treitz and pelvic spaces. Peritoneal surfaces of bowels are spared. The tumour progresses by the production of mucous, exfoliation of tumour cells and redistribution of these cells around abdomen. FNAC, USG and CT scan abdomen are prime modalities of investigations for diagnosis of PMP. USG shows septated ascites with numerous ‘suspended echoes’ that do not move as the patient changes position. These were seen in our case. Combined with USG, paracentesis may accurately establish the diagnosis of gelatinous ascites. CT being the most valuable, shows anteriorly situated septated ascites, cystic lesions (10-45 Hounsfield units) with partial calcification, extrinsic compression of viscera without direct invasion. A specific sign of PMP is scalloping of hepatic margin from extrinsic compression of liver by fluid filled ascitic spaces containing gelatinous material.
The goal of therapy should be complete cytoreduction with the use of ‘peritonectomy’ procedures followed by early post-operative intraperitoneal chemotherapy. Aggressive and “repetitive debulking” surgery including appendectomy, bilateral oophorectomy and omentectomy at the initial procedure should be undertaken. Involvement of appendix is common and appendectomy is always indicated when PMP is encountered. Reoperation is associated with more difficult dissection, with higher postoperative morbidity compared to previous surgery. Additional therapy is indicated in those patients who present with peritoneal irritation and palpable abdominal mass as they are at maximum risk of recurrence. Intraperitoneal radioisotope instillation and intraperitoneal chemotherapy have been used. Intraperitoneal 5-Fluoro-uracil appears to be an effective drug. Systemic chemotherapy is rarely indicated.
Abdominal CT is an excellent technique to follow-up these patients for recurrence. An aggressive attitude should also be maintained when repeat laparotomies are done for debulking, relief of intestinal obstruction or fistulization because long-term survival is a rule in these patients.
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