Aggressive Pseudomyxoma Peritonei: A Case Report with an Unusual Clinical Presentation

Abstract

Introduction. Pseudomyxoma peritonei (PMP) is an uncommon surgical entity. We report a case of aggressive disease with an unusual clinical presentation and we analyze current data on diagnosis and management of PMP. Case Presentation. A 71-year-old male patient presented with intermittent diarrhea and loss of appetite during the last two months, without any other classic symptoms of PMP. The clinical examination was misleading due to patient's obesity. The radiological evaluation revealed ascites of the abdomen and possible mucocele of the appendix, whereas the laboratory exams showed high values of specific tumour markers. The patient underwent an exploratory laparotomy for definite diagnosis. Biopsies and immunohistochemical examination confirmed the diagnosis of an aggressive and extended peritoneal mucinous carcinomatosis (PMCA). The patient was programmed for adjuvant systematic chemotherapy, which was not completed due to progression of the disease. Conclusions. Progressed PMP can present with unspecific symptoms that mislead diagnosis. Cytoreductive surgery in combination with systematic chemotherapy could be appropriate for aggressive PMCA, even with an unfavourable prognosis.

Pseudomyxoma Peritonei: A Rare Case Report with Review of Literature

“…Our case presented in malignant form. Physiologic principles involved include accumulation and peritoneal absorption of mucinous cells [2]. The myxomatous appearance is attributed to the associated fibroblastic and vascular proliferation that is probably incited by mucin.…”

“…It has variable clinical presentations like abdominal distension, ovarian tumors or appendicitis like syndrome [2]. It was first described by Werth in 1884.…”

A Case of Pseudomyxoma Peritonei in a 69 Years Old Woman and Review of Literature

“…Some studies say also KRAS (p53) may be involved in the oncogenesis [2]. About histopathologic classification of PMP there is a debate [3].…”

“…In the other hand, PMCA is characterized by peritoneal lesions presenting more abundant mucinous epithelium with the architectural and cytologic features of carcinoma (irregular cells-rapidly dividing), with or without an associated primary mucinous adenocarcinoma. The majority of cases are described in appendix and in ovarian but there are described other side as colon, rectum, stomach, gallbladder, biliary tract and so on [2,4]. Signs and Symptoms of PMP are very specific and may include abdominal pain, pelvic pain, bloating, abdominal distension, alteration of intestinal motility and weight loss.…”

“…This disease is most commonly caused by an appendiceal tumor (mucocele) or by mucinous tumors of the ovary. The overall incidence is 1-2 per million per year, it is more common in women than in men (Male: Female ratio: 9:11) and median age is about 50 years with a range of 20-25 years [2].…”