ReviewFrantz's tumor: Is mutilating surgery always justified in young patients?
Introduction
Solid pseudopapillary tumor of the pancreas (Frantz's tumor) is a rare lesion.
It is of low malignant potential, but can produce extensive local invasion.
The best treatment is a complete resection without breaking up the lesion and avoiding laparoscopic approach [1].
However, since the doubling time of this tumor appears to be slow (765 days) [2], the question arises as to whether mutilating resection sacrificing organs and/or blood vessels is warranted.
We decided to assess this question after observing high postoperative morbidity following treatment of a young girl in our department.
The purpose of this report is to describe our patient and a retrospective review of previous cases of incomplete resection for Frantz's tumor.
In order to evaluate long-term risk of this tumor after incomplete resection, we contacted all authors who published case reports describing incomplete resection of Frantz's tumor between 1985 and 2008 to request follow-up information. Incomplete resection was defined in two categories, i.e., resection microscopically incomplete (R1) and resection macroscopically incomplete (R2).
Section snippets
Methods
An 11-year-old girl was admitted to our hospital for vomiting, jaundice and abdominal mass.
Radiologic examination demonstrated a voluminous mass (12 cm) developing from the pancreas to contiguous organs.
A preoperative biopsy was performed and it was diagnosed as a Frantz's tumor (Fig. 1).
The girl was underwent multiple surgical interventions. Procedures included a cephalic duodeno-pancreatectomy extended to isthmus, partial resection of the superior mesenteric vein, right and transverse
Results
We contacted 14 authors (by email, letter, or fax) who reported cases of Frantz's tumor treated by incomplete resection.
One author described 3 cases, two described 2 cases and the other 11 authors described 1 case each.
The total number of cases was 18 including 10 R1 and 8 R2 resections [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16].
In one case the tumor broke up during resection [13].
Eleven authors responded including 2 who could not access the data, 1 who had lost
Discussion
Because of the indolent nature of Frantz's tumor and high morbidity following aggressive surgery [17], [18], the most appropriate surgical approach is still controversial.
According to this retrospective study, overall median survival after incomplete resection of Frantz's tumor reported in the literature was only 5.7 years.
There was no statistically significant difference between patients who underwent R1 resection (microscopically incomplete) or R2 resection (macroscopically incomplete). These
Conclusion
Despite the low malignant potential of Frantz's tumor, the results of our review of the literature show that, median survival in patients who undergo incomplete is only 5.7 years. This is unacceptable for young patients.
Complete surgical resection should be performed in patients presenting locally invasive solid-pseudopapillary tumor of the pancreas, even if it requires difficult, mutilating surgery.
Further study is needed.
Conflict of interest statement
None declared.
Acknowledgments
THANKS TO: M Brennan [13], J Cameron [16], F Carboni [4], K Horvath [6], T Nakagohri [10], LLPJ Ooi [5], J Podevin [12], LM Sclafani [13], M Siech [18], GB Thompson [15] for providing their follow-up data.
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