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Table 5. Clinical outcomes in PACC
Age at Median With
Reference Data source Years n diagnosis tumor size metastasis OS Stage 4 OS Early stage
OS (median)
(median) (median)
(median) (cm) (%)
[142] NCDB 1985- 865 67 5.9 32.1 N/A 17.2%* NR-22.6m
2005
[145] SEER 1988- 672 56 53.1 47 22%* 72%*^
2003
[144] SEER 2004- 252 63.8 N/A 54.4 10 7 18,29^
2016
[143] German Cancer 2000- 233 66 N/A 33.9 22 6 34^
Registry Group 2019
[141] SI-Harvard 1996- 66 64 4.3 42 13 15 38
Hospitals 2019
[147] Korean Tumor 2003- 59 59.2 4.6 0 N/A N/A 78.8^
Registry 2018
System
[148] SI-West China 2006- 52 50.8 5.0 30.8 39 N/A 48^
Hospital 2016
Seo 2017 [146] SI-Asan 1997- 20 57 4.0 0 N/A N/A 75^
Medical Center 2015
Matos MI 1998- 17 59 5.3 23.5 19 N/A 61^
[159]
2009 2008
Multiple retrospective case series examining survival in PACC patients have been conducted. *indicates that 5-year overall survival percentage is
documented; ^indicates value is for resected patients only; SI: Single institution; MI: multi-institution; n: number of patient cases; OS: overall
survival (in months unless otherwise specified); N/A: not available; NR: not reached.

crystals are visualized [162,163] . Patients experiencing LHS usually have advanced metastatic disease. LHS in
PACC has been recently well-reviewed by Taskin et al. .
[164]

Levels of the PDAC serum tumor marker CA 19-9 are elevated in less than 30% of patients with PACC and
may be related to non-specific biliary duct irritation rather than tumor production in most cases .
[141]
Nevertheless, poor survival outcomes were observed in patients with tumors that do express CA 19-9 [146,147] .
Larger cohorts would be required to assess the prognostic value of this marker in PACC. Elevation of CEA
has been observed in about one-fifth of PACC patients , and elevated blood Alpha-fetoprotein (AFP)
[141]
levels in younger patients and should raise suspicion of PACC, especially when associated with a pancreatic
mass . Serum lipase might be used as a surrogate marker of tumor response in some patients, as PACC
[165]
frequently produces high levels of lipase, but this has not been well characterized in the literature.

Clinical management of PACC
There are currently no treatment guidelines for PACC. All treatment is extrapolated from standards of care
for PDAC as no prospective or randomized trials examining treatment paradigms have been reported.
Staging of PACC is as per PDAC staging [Table 1].

Resectable disease
Surgical resection is the treatment of choice for early-stage disease. Resection has been shown to result in a
dramatic improvement in cohort overall survival as compared to no resection in multiple retrospective case
series and population-based studies [141-144,166] . In fact, resection was the most predictive factor for overall
survival in some studies. In one study, 5-year survival for Stage I or II patients receiving resection was 42%
compared to 9% for those who did not get surgery . Negative margins are predictive of better long-term
[167]
survival in some studies.