Removing a solid-cystic intraabdominal desmoid tumor at a gastro-pancreatic lesion

Background

Desmoid tumors (also called aggressive fibromatosis, deep musculoaponeurotic fibromatosis) are locally aggressive tumors with no potential for metastasis or dedifferentiation. Radiologically, they are usually non-specific, slow-growing solid masses that are difficult to distinguish from other soft tissue tumors, but they rarely show cystic lesions. Sporadic desmoid tumors can occur at any site in the body, and anatomic locations are classified into 3 regions: trunk/extremity, abdominal wall, and intraabdominal. Complete surgical resection is the only curative option, but intraabdominal desmoid tumors are often unresectable, because they often infiltrate the mesentery. Here, we report a case of an intraabdominal desmoid tumor that occurred at a gastro-pancreatic lesion with cystic features, and presents the successful laparoscopic resection of the tumor.

Case presentation

An intraabdominal cystic mass was incidentally detected between the stomach and the pancreas in a 52-year-old woman undergoing postoperative follow-up computed tomography (CT) for chondrosarcoma of a rib. Her family history was unremarkable. Blood counts and serum biochemistry results were within normal limits. An abdominal CT scan revealed a 20 × 18-mm well-defined cystic mass with a solid component (Fig. 1a,b). The tumor was adjacent to the stomach and the pancreas body, but there was no sign of invasive growth to the organs. MRI showed a cystic mass with high intensity on T2-weighted images and low intensity on T1-weighted images. The lesion showed no sign of diffusion restriction by diffusion-weighed MRI. The solid component of the cystic wall was gradually enhanced in the late phase. Subsequent endoscopic ultrasonography (EUS) showed an 18 × 13 mm round cystic mass, with a heterogeneous nodular lesion at the side of the serosa of the stomach (Fig. 1c). Esophago-gastric endoscopy showed no local lesion on the mucosal surface of the stomach (Fig. 1d). An intraabdominal tumor was suspected, but it was difficult to determine the specific diagnosis.

Preoperative images. a, b CT image showing a well-defined round, 20-mm cystic mass with a rim of soft tissue in the gastro-pancreatic region. The tumor is dense, adjacent to the gastric wall and pancreatic parenchyma. Slight enhancement of the solid component is seen after intravenous administration of contrast material. Arrows indicate the tumor. c Endoscopic ultrasound image showing a well-defined cystic mass. d Gastric endoscopy showed no abnormal findings of the gastric mucosa

Laparoscopic resection via 5 trocars was performed with the patient in the supine position. After the gastro-colic ligament was cut, the omental bursa was opened widely. The tumor was confirmed at the superior border of tghe pancreas body, and adhered to the posterior wall of the stomach (Fig. 2a). With careful dissection, the left gastric artery and vein were isolated and preserved. The tumor was cut from the stomach by wedge resection of the gastric wall using a linear stapler (Fig. 2b). Then, we performed a spleen-preserving distal pancreatectomy. The pancreas body-tail was carefully mobilized and separated from the splenic artery and vein by dissecting the small vessels. The pancreas was dissected at the right side of the tumor using a linear stapler (Fig. 2c). We performed a complete laparoscopic excision, and the spleen and splenic vessels were completely preserved (Fig. 2d). The operative time was 279 min, and intraoperative blood loss was 15 mL. The postoperative course was uneventful, and the patient was discharged healthy.

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Intraoperative findings. a The tumor was located in the gastro-pancreatic region and locally invaded the gastric wall and the pancreatic parenchyma. The arrow indicates the tumor. b Wedge resection of the stomach was performed using a linear stapler. c The pancreatic parenchyma was transected using a linear stapler. The arrow indicates the tumor. d The splenic artery (red tape) and splenic vein (blue tape) were preserved. The arrow indicates pancreatic stump

Macroscopic examination of the resected specimen revealed a capsulated cystic mass measuring approximately 20 mm with serous content. The whitish solid nodule containing the cystic lesion existed at the wall of the stomach (Fig. 3a). Microscopic examination demonstrated the distribution of spindle tumor cells, partly arranged in a fascicular fashion and accompanied by a collagenous stroma (Fig. 3b, c). Mitotic figures were rarely seen. The tumor cells were attached to the muscular layer of the gastric wall (Fig. 3b). A benign cystic lesion was also observed, but its origin was not determined. Immunohistochemically, the spindle cells were diffusely positive for β-catenin protein in the nucleus (Fig. 3d), but negative for theα-SMA, desmin, S-100, c-kit, and CD34 proteins. These findings were consistent with the diagnosis of intraabdominal desmoid tumor.

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Macroscopic and microscopic findings. a The cut surface of the excised solid-cystic mass showing light tan glistening tissue. The arrow indicates the tumor. b A lower power view shows that the tumor cells (asterisk) originated from the gastric wall and attached to the pancreatic parenchyma without invasion (hematoxylin and eosin staining; original magnification × 40). c Histopathological findings show uniform cord-like proliferation of spindle-shaped cells with minimal atypia, and the intercellular spaces are filled with thick bundles of collagen fibers (hematoxylin and eosin staining; original magnification × 100). d Immunohistochemically, the spindle cells are diffusely and strongly positive for nuclear β-catenin protein. (× 100)
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Author

Affiliations

  1. Department of Hepatobiliary-Pancreatic Surgery, National Hospital Organization Kyushu Cancer Center, 3-1-1 Notame, Minami-ku, Fukuoka, 811-1395, Japan
    • Keishi Sugimachi
    • , Tomohiro Iguchi
    •  & Yohei Mano
  2. Department of Gastroenterological Surgery, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan
    • Mitsuhiko Ohta
    • , Masahiko Ikebe
    • , Masaru Morita
    •  & Yasushi Toh
  3. Department of Hepato-Biliary-Pancreatology, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan
    • Terumasa Hisano
  4. Department of Orthopedic Surgery, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan
    • Ryohei Yokoyama
  5. Department of Pathology, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan
    • Kenichi Taguchi

Contributions

KS, TI, MO, YM and TH collected, analyzed and interpreted the patient data. RY and KT performed and interpreted the histological examination. KS, MI, MM and YT were responsible for study design, writing and scientific revision. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Keishi Sugimachi.

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