Rib and pericardium invaded huge abdominal mass in young woman: A case report with literature review

Medicine (Baltimore). 2022 Sep 2;101(35):e30371. doi: 10.1097/MD.0000000000030371.

Abstract

Rationale: Desmoid fibromatosis is a rare benign tumor, but due to its rarity and diverse clinical course, treatment guidelines have not been established. However, since a good prognosis can be expected, an accurate diagnosis and appropriate treatment are required. We describe a rare case of desmoid fibromatosis on young female that presented as huge abdominal mass.

Patients concerns: A 28-year-old female with left upper abdominal pain 1 month ago was referred.

Diagnoses: Abdominal computed tomography and magnetic resonance imaging revealed a heterogeneous soft tissue mass approximately 29 × 17 cm in size in the left abdomen with abdominal wall invasion and pathological fracture in costochondral junction of the left 8th to 10th ribs.

Interventions: Surgical resection was performed.

Outcomes: 33 × 23 × 6 cm sized tumorous mass showed proliferation of bland fibromatosis and myofibroblast with nuclear β-catenin expression on pathological examination. Desmoid fibromatosis arising from intra-abdominal soft tissue with ribs and pericardium invasion was diagnosed.

Lessons: The mainstay of treatment of symptomatic desmoid fibromatosis is surgical resection, and in the case of abdominal tumor, it can be more dangerous when it invades adjacent organ. We report a case that required additionally multidisciplinary approach for surgery and postoperative treatment of huge abdominal desmoid tumor which infiltrate bone and pericardium beyond abdominal cavity.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Female
  • Fibroma*
  • Fibromatosis, Aggressive* / diagnostic imaging
  • Fibromatosis, Aggressive* / surgery
  • Humans
  • Pericardium / pathology
  • Ribs / pathology
  • beta Catenin

Substances

  • beta Catenin