Goblet cell carcinoma of the appendix: Case review

Leigh Cassels-Gibson, Ultrasound, Leicester Royal Infirmary, University Hospitals of Leicester

50 year old male presented for urinary tract ultrasound.  Symptoms included dysuria, frequency and lower abdominal pain, persisting for several months.  No known infective cause.  Ultrasound requested to rule out bladder stones.  No previous relevant imaging.

Ultrasound:  Indenting on the external bladder wall from the right iliac fossa, is an avascular, hypoechoic mass (9cm x 3cm x 4cm), which contains several hyperechoic, shadowing foci.

CT:  Focal thick walled, enhancing collection related to superior aspect of the urinary bladder. A contiguous, enhancing soft tissue mass tethers and involves urinary bladder, terminal ileum, distal ileum and appendix in a stellate configuration. No fistula identified. Appearances of primary appendiceal pathology. In view of the enhancing soft tissue mass, surgical excision was advised.

Histology: A diffusely infiltrative Goblet Cell Carcinoma (GCC) of the Appendix.

Goblet cells are glandular cells which excrete mucins to protect associated mucous membranes. GCC occurs when there is excessive proliferation of both goblet and neuroendocrine cells.

Goblet cell carcinoma accounts for 5% of neoplasms of the appendix, with an annual incidence of 0.12 per million. GCCs are rare and share characteristics with adenocarcinoma and carcinoid tumours.  They almost exclusively involve the appendix.

Symptoms often present in 5th & 6th decade with no gender preference.  Clinical presentations are varied; most commonly acute appendicitis, pain and mass.  Other symptoms include bowel obstruction, intussusception and gastro-intestinal bleeding.

Imaging findings include ill-defined nodular thickening of the appendix (most commonly at the tip) with a diffuse pattern of infiltration.  The majority of GCCs are >2cm and demonstrate longitudinal growth patterns.  In 3% of patients with GCC, findings are incidental.

Treatment requires en-bloc, possibly followed by chemotherapy, systemic or hyperthermic intraperitoneal chemotherapy (HIPEC) for recurrent peritoneal disease.

View Poster Here