Ann Hepatobiliary Pancreat Surg.  2021 May;25(2):276-282. 10.14701/ahbps.2021.25.2.276.

Desmoid type fibromatosis of the distal pancreas: A case report

• Park CG
• Lee YN
• Kim WY

• KMID: 2516252
• DOI: http://doi.org/10.14701/ahbps.2021.25.2.276

Abstract

A 23-year-old Korean female presented epigastric pain of two-months’ duration. She had a laparoscopic ovarian cyst excision 8 months previously. Clinical examination was normal. An abdominal computed tomogram (CT) demonstrated a 10-cm solid mass in the distal pancreas, with signs of splenic artery and vein occlusion, gastric and transverse colon invasion. Operative findings showed a mass involving distal pancreas, invasive to the posterior wall of the antrum of the stomach and transverse colon and 4th portion of the duodenum without lymph node involvement. The surgery consisted of a distal pancreatectomy, splenectomy and combined partial resection of the stomach, transverse colon and 4th portion of the duodenum. The immunohistochemistry and histopathological features were consistent with a confirmed diagnosis of intra-abdominal desmoid type fibromatosis (DTF). The prognosis of pancreatic DTF is not known and she showed no recurrence or distant metastasis during a 3 year follow-up. Herein we report a rare case with an isolated, sporadic, and non-trauma-related DTF, located at the pancreatic body and tail.

Keyword

Pancreatic desmoid type fibromatosis; Desmoid tumor; Aggressivefibromatosis

Figure

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  Fig. 1 Abdominal computed to-mogram (CT) showed a 7.7-cm solid mass in the distal pancreas, with signs of splenic artery and vein occlusion, duodenum ‘D’, gastric ‘G’ and transverse colon invasion ‘C’.

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  Fig. 2 A huge lobulated con-tour mass was found in the body and tail of the pancreas, showing low signal intensity on T1-weighted image (A) and slightly high T2-weighted image (B) with diffusion restriction (C) and delayed enhancement pattern (D).

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  Fig. 3 Endoscopic ultrasono-gram shows a huge soft tissue mass with no cystic portion but multiple echogenic foci in the distal pancreas.

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  Fig. 4 PET-CT showed heterogeneous hypermetabolic tumor in left upper abdomen.

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  Fig. 5 Gross specimen of the pancreatic body solid mass measuring 10 cm in size.

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  Fig. 6 H&E staining showed spindle cell mass in the pan-creatic gland (A: H&E ×40 mag-nification, B: H&E ×100 magni-fication).

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  Fig. 7 Immunohistochemical staining showed in strong positive beta-catenin.

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