# Small Hepatoid Variant of Solid Pseudopapillary Neoplasm of the Pancreas: A Unique Case With Long‐term Follow‐up

**Authors:** Hisakazu Matsumoto, Kosuke Minaga, Shotaro Ueno, Asuka Sone, Eiki Chikugo, Kohei Tonomura, Shogo Nakano, Yoshito Uenoyama, Kazuo Ono, Yukitaka Yamashita

PMC · DOI: 10.1002/deo2.70310 · DEN Open · 2026-03-03

## TL;DR

A rare small pancreatic tumor with liver-like features was found in a 37-year-old man and remained stable for 5 years without surgery.

## Contribution

First reported long-term follow-up of a nonoperatively managed hepatoid variant of solid pseudopapillary neoplasm.

## Key findings

- The 6 mm tumor showed no progression over 5 years of active surveillance.
- Immunohistochemistry confirmed hepatoid differentiation in the solid pseudopapillary neoplasm.
- Conservative management may be viable for small, asymptomatic hepatoid SPNs.

## Abstract

Hepatoid tumors are rare neoplasms that arise outside the liver but exhibit morphological and immunophenotypic features resembling hepatocellular carcinoma. Although hepatoid differentiation sometimes occurs in pancreatic ductal adenocarcinoma, its occurrence in solid pseudopapillary neoplasm (SPN) is exceedingly rare. Consequently, the clinicopathological characteristics and natural history of this variant remain poorly understood. We report a case of a 37‐year‐old asymptomatic male with a 6 mm solid pancreatic body lesion that was incidentally detected during routine abdominal ultrasonography. Multimodal imaging showed a well‐circumscribed solid mass showing early‐phase hyperenhancement relative to the surrounding pancreatic parenchyma. Tissue samples obtained via endoscopic ultrasonography contained polygonal epithelioid cells with abundant eosinophilic cytoplasm and a hepatoid appearance. In immunohistochemistry, HepPar‐1 and CD10 were diffusely positive, chromogranin A and synaptophysin were absent, and β‐catenin accumulated within the nucleus, all supporting a diagnosis of SPN with hepatoid differentiation. Although surgical resection was recommended, the patient declined and was subsequently managed with active surveillance. After 5 years of follow‐up, the lesion remained morphologically stable without clinical progression. To our knowledge, this case report is the first to describe the long‐term natural course of a pancreatic hepatoid SPN managed nonoperatively. The patient's maintained stability supports the indolent biological behavior of this rare variant. Thus, conservative management with close surveillance may be feasible for carefully selected patients with small, asymptomatic tumors. However, additional cases are needed to clarify optimal management strategies.

## Linked entities

- **Proteins:** MME (membrane metalloendopeptidase), ctnnb1.S (catenin beta 1 S homeolog)
- **Diseases:** hepatocellular carcinoma (MONDO:0007256), pancreatic ductal adenocarcinoma (MONDO:0005184)

## Full-text entities

- **Genes:** AFP (alpha fetoprotein) [NCBI Gene 174] {aka AFPD, FETA, HPAFP}, MME (membrane metalloendopeptidase) [NCBI Gene 4311] {aka CALLA, CD10, CMT2T, NEP, SCA43, SFE}, CTNNB1 (catenin beta 1) [NCBI Gene 1499] {aka CTNNB, EVR7, MRD19, NEDSDV, armadillo}, SYP (synaptophysin) [NCBI Gene 6855] {aka MRX96, MRXSYP, XLID96}, CHGA (chromogranin A) [NCBI Gene 1113] {aka CGA, PHE5, PHES}, KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, KRT7 (keratin 7) [NCBI Gene 3855] {aka CK7, K2C7, K7, SCL}, CEACAM3 (CEA cell adhesion molecule 3) [NCBI Gene 1084] {aka CD66D, CEA, CGM1, CGM1a, W264, W282}, IMP3 (IMP U3 small nucleolar ribonucleoprotein 3) [NCBI Gene 55272] {aka BRMS2, C15orf12, MRPS4}, NCAM1 (neural cell adhesion molecule 1) [NCBI Gene 4684] {aka CD56, MSK39, NCAM}, LEF1 (lymphoid enhancer binding factor 1) [NCBI Gene 51176] {aka ECTD1, ECTD17, LEF-1, TCF10, TCF1ALPHA, TCF7L3}, CD34 (CD34 molecule) [NCBI Gene 947], SLC4A1 (solute carrier family 4 member 1 (Diego blood group)) [NCBI Gene 6521] {aka AE1, BND3, CD233, CHC, DI, EMPB3}
- **Diseases:** hepatic lesions (MESH:D056486), node metastasis (MESH:D008207), HCC (MESH:D006528), pancreatic body lesion (MESH:D010182), necrosis (MESH:D009336), Pseudopapillary Neoplasm of the Pancreas (MESH:D010190), PDAC (MESH:D021441), pancreatic incidentalomas (MESH:D010195), cystic tumors (MESH:D018297), Hepatoid tumors (MESH:D009369)
- **Chemicals:** Hematoxylin (MESH:D006416), Papanicolaou (-)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

10 references — full list in the complete paper: https://tomesphere.com/paper/PMC12955827/full.md

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Source: https://tomesphere.com/paper/PMC12955827