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Case Reports
. 2022 Feb 11;14(2):e22139.
doi: 10.7759/cureus.22139. eCollection 2022 Feb.

Periampullary Carcinoma in a 13-Year-Old With Microsatellite Instability Treated Successfully With Pancreaticoduodenectomy

Sivaraman Kumarasamy  1 , Lileswar Kaman  1 , Cherring Tandup  1 , Uttam K Thakur  2 , Ajay Savlania  1
Affiliations
Case Reports

Periampullary Carcinoma in a 13-Year-Old With Microsatellite Instability Treated Successfully With Pancreaticoduodenectomy

Sivaraman Kumarasamy et al. Cureus. .

Abstract

Periampullary carcinoma in adolescents is very rare and may be associated with hereditary syndromes. Pancreaticoduodenectomy (PD) in adolescents is rarely performed. The experience and results of pancreaticoduodenectomy in adolescents are not well reported. Here, we report a case of periampullary carcinoma, duodenal origin, signet ring type with microsatellite instability (MSI), in a 13-year-old male for which pancreaticoduodenectomy was successfully done.

Keywords: cancer in young; microsatellite instability (msi); pancreaticoduodenectomy; periampullary carcinoma; whipple’s procedure.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Biphasic CECT of the abdomen showing dilated CBD in its entire course (arrow) with an abrupt cutoff in the distal end before joining the duodenum.
Figure 2
Figure 2. 18F-FDG PET-CT showing FDG avid soft tissue periampullary thickening measuring ~2.7 × 1.2 cm with a maximum standard unit value (SUVmax) of 6.9 extending into the D2–D3 junction with ill-defined fat planes with pancreas medially (arrow).
Figure 3
Figure 3. Cut specimen showing a 3.5 × 2.5 cm ulceroproliferative growth in the ampullary region in the D2 (arrow).
Figure 4
Figure 4. Microscopic examination on high-power magnification showing sheets of signet ring cells seen with clear cytoplasm filled with mucin and eccentric nuclei (arrow).
Figure 5
Figure 5. Immunohistochemistry markers.
A: MLH1. B: MSH2. C: MSH6. D: PMS2. Tumor cells are negative for MLH1 and PMS2, suggesting a deficiency of MMR proteins (A and D). MSH2 and MSH6 are retained in the tumor cells (B and C).
See this image and copyright information in PMC

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