Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy for Moderately and Poorly Differentiated Appendiceal Adenocarcinoma: Survival Outcomes and Patient Selection

Ann Surg Oncol. 2017 Sep;24(9):2646-2654. doi: 10.1245/s10434-017-5938-8. Epub 2017 Jul 10.


Background: Moderately and poorly differentiated adenocarcinoma of the appendix represents an aggressive histological variant with a high risk of recurrence and death.

Methods: Overall, 178 patients with moderately and poorly differentiated appendiceal adenocarcinoma were identified from a prospective database. Clinical, pathologic, and treatment factors were analyzed for outcomes.

Results: Diagnostic laparoscopy (DL) identified radiographic occult peritoneal metastasis in 25 (42%) patients. These patients had a significantly lower peritoneal carcinomatosis index (PCI) and improved overall survival (OS) compared with those with radiographic disease. Twenty-seven (41%) patients were excluded from cytoreductive surgery (CRS) because of findings on DL, while 116 (65%) patients underwent CRS and hyperthermic intraperitoneal chemotherapy (HIPEC), with a median disease-free survival (DFS) of 23 months. Mucinous histology (hazard ratio [HR] 0.52, p = 0.04) and PCI (HR 1.054, p = 0.02) were independent predictors of DFS. The median OS following CRS and HIPEC was 48 months. Mucinous histology (HR 0.352, p = 0.018), signet ring cells (HR 3.34, p = 0.02), positive peritoneal cytology (HR 0.081, p = 0.04), and PCI (HR 1.076, p = 0.004) were independently associated with OS. Eight-five (73.3%) patients received neoadjuvant chemotherapy, and 40 (47.1%) patients achieved a radiographic response; 36 (42.3%) had stable disease, while 9 (10.6%) had progressive disease. Stable or responsive disease was associated with improved median OS of 44 months, compared with 21 months for those with progressive disease (p = 0.011).

Conclusions: In selected patients, long-term survival can be obtained. Mucinous histology, absence of signet ring cells, negative peritoneal cytology, PCI ≤ 20, and response/stable disease after neoadjuvant chemotherapy are important selection criteria for CRS and HIPEC.

MeSH terms

  • Adenocarcinoma, Mucinous / diagnostic imaging
  • Adenocarcinoma, Mucinous / secondary
  • Adenocarcinoma, Mucinous / therapy*
  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
  • Appendiceal Neoplasms / diagnostic imaging
  • Appendiceal Neoplasms / pathology
  • Appendiceal Neoplasms / therapy*
  • Cell Differentiation
  • Chemotherapy, Adjuvant
  • Cytoreduction Surgical Procedures*
  • Disease Progression
  • Disease-Free Survival
  • Female
  • Humans
  • Hyperthermia, Induced*
  • Male
  • Middle Aged
  • Neoplasm Grading
  • Patient Selection
  • Peritoneal Neoplasms / diagnostic imaging
  • Peritoneal Neoplasms / secondary
  • Peritoneal Neoplasms / therapy*
  • Survival Rate