However, there seems to be a convergence towards consensus with recent publication of classification systems, which remarkably improved on past efforts. Different workers proposed various classification systems and terminologies, but none of the proposed systems could fully reconcile the bland histology exhibited by the tumors with their capacity for disseminated peritoneal disease, PMP. Review of existing literature on appendiceal neoplasm portrayed some controversy which revolved around what should be the right taxonomy for mucinous neoplasms, a group of neoplasm that often showed a wide range of clinical behavior varying from indolent to aggressive. (b) It shows pushing invasion into the fibrotic wall and luminal mucin. (a) It shows a single layer of neoplastic mucin secreting epithelium resting on a fibrotic wall. Photomicrograph of low-grade appendiceal mucinous neoplasm. No perforation was seen, and no mucin deposits were present on the appendix or intestinal tissues. The cyst contained gelatinous mucin, and its wall measured 1 cm in thickness (Figure 1). The appendix was transformed into a firm whitish cystic mass measuring 8 cm × 7 cm × 7 cm. Pathology: Gross examination showed a right hemicolectomy tissue. She has remained in good health 2 years post-surgery. A right hemicolectomy and ileo-transverse 2-layer anastomosis was done with good outcome. The finding at laparotomy was a large caecal mass which was adherent to the uterus and bladder.
She was transferred to the general surgery unit where she underwent exploratory laparotomy. Computed tomography (CT) scan was requested for, but the patient could not afford it. The mass had a well-defined, relatively hyperechogenic thick margin and measured 15 × 7.17 × 10.5 cm. Transabdominal ultrasound scan showed a large, irregular shaped, complex heteroechoic mass, that abuts the right aspect of the urinary bladder and the uterus. A provisional diagnosis of right iliac fossa mass probably of ovarian origin was made prompting a review by a gynecologist. Abdominal palpation revealed a mobile, nontender, soft right lower abdominal mass. On physical examination, her vital signs were normal. The swelling was of gradual growth with intermittent dull ache that preceded the swelling by a few months. Case PresentationĪ 67-year-old African woman, 20 years postmenopausal, and on treatment for diabetes and hypertension, presented with a right lower abdominal swelling of five-month duration. We report a case of cystic sclerohyalinized LAMN mimicking an ovarian mass, and we review the literature for the underlying pathogenetic mechanism and clinical implication of this striking morphology. Gross pathology appearances have been described as dilated or cystic in most reported cases. In terms of clinical presentation and pathologic appearance, the majority of LAMN present as abdominal mass or pain with fewer numbers discovered incidentally during procedures for some other conditions. About 20% of patients with mucinous epithelial neoplasm of the appendix develop PMP. The clinical importance of LAMN lies in its ability to disseminate throughout the peritoneal cavity as pseudomyxoma peritonei (PMP), a condition that often runs an interminable clinical course requiring aggressive treatment. The term is used in the current classification schemes to denote mucinous appendiceal tumors with low-grade cytology in addition to any of the following: loss of the lamina propria and muscularis mucosae, fibrosis of the submucosa, pushing growth into the wall of the appendix, dissecting acellular mucin in the wall of the appendix, and cellular or acellular mucin outside of the appendix. Low-grade appendiceal mucinous neoplasm (LAMN) is a terminology originally introduced by Misdraji and colleagues. Primary epithelial neoplasm is present in about 0.6% of all appendectomies just as mucinous neoplasm is estimated to occur in 0.3% of all surgically resected appendices. Neoplasms of the vermiform appendix are rare, estimated to occur in 2% percent of all appendectomy tissues. We review the literature for the pathogenesis and clinical implication of sclerohyalinization in mucinous appendiceal tumors. We report a case of low-grade appendiceal mucinous neoplasm presenting as firm sclerotic cystic mass and was initially suspected to be an ovarian mass in a postmenopausal woman. It sometimes poses challenges in clinical, radiological, and pathological diagnosis, and it may be confused with gynecological conditions in females. Low-grade appendiceal mucinous neoplasm is a tumor of the appendix whose major clinical relevance derives from its inherent potential for peritoneal dissemination as pseudomyxoma peritonei.
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