Low-grade appendiceal mucinous neoplasm

Case contributed by Mohammad Taghi Niknejad
Diagnosis almost certain

Presentation

Right lower quadrant pain for more than one month. An enlarged appendix was reported in the recent ultrasonography evaluation.

Patient Data

Age: 75 years
Gender: Male

The appendix is enlarged with a thick and irregular wall and focal eccentric enhancing soft tissue component, more prominent within the proximal third. The dilated appendix bulged into the cecum. Perirectal fat is intact, and no regional enlarged lymph node is identified.

A few subcentimeter simple cortical cysts are seen in the kidneys.

A small fat-containing umbilical hernia is present.

The prostate gland is enlarged, and its central portion protrudes cephalad into the urinary bladder base.

Case Discussion

The findings on CT suggest a neoplastic appendiceal mucocele, and the patient underwent an appendectomy and proximal partial colectomy. The histopathology and IHC evaluation confirmed a low-grade appendiceal mucinous neoplasm, a rare mucinous tumor of the appendix.

An appendiceal mucocele with wall irregularity, internal enhancing soft tissue component, and pseudomyxoma peritonei suggest malignancy.

Differentiating between the low-grade and high-grade mucinous neoplasms of the appendix can be difficult by imaging. The size, density and wall thickness of these mucoceles can be similar between the two groups. Furthermore, calcification, internal septations, peri appendiceal fat stranding and intraperitoneal free fluid are nonspecific. Differentiation is based on histopathology.

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