Desmoid-type fibromatosis mimicking cystic retroperitoneal mass: Case report and literature review

Kyu Chong Lee, Jongmee Lee, Baek Hui Kim, Kyeong Ah Kim, Cheol Min Park

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    9 Citations (Scopus)


    Background: Retroperitoneal desmoid-type fibromatosis (DF) is an uncommon mesenchymal neoplasm presenting as a firm mass with locally aggressive features. It usually manifests as a well-circumscribed or ill-defined, solid mass on cross-sectional imaging. Cystic changes of DF have been described in the literature in association with prolonged medical treatment or abscess formation. However, spontaneous cystic change israrely reported. Case presentation: Here we report the case of a 46-year-old patient with a DF mimicked a large cystic tumor in the retroperitoneum. Ultrasonography and computed tomography were performed in order to search for localizations and characteristics of the cystic tumor. Radiological findings showed an oval cystic mass with a relatively thick wall, measuring 18.3×12.3×21.5cm in the left upper abdomen. Laparoscopic spleen-preserving distal pancreatectomy was performed and histopathological examination by immunohistochemical study enabled us to diagnose a DF invading the pancreatic parenchyma. The patient remained asymptomatic during an 8-month follow up period. Conclusions: We report an extremely rare case of retroperitoneal DF with spontaneous cystic change. DF can manifest as a mainly cystic mass with a thick wall, as in our case, which makes the correct diagnosis difficult. DF should be included in the preoperative differential diagnosis of a cystic retroperitoneal mass, regardless of its rarity.

    Original languageEnglish
    Article number29
    JournalBMC Medical Imaging
    Issue number1
    Publication statusPublished - 2018 Sept 17

    Bibliographical note

    Funding Information:
    This study was supported by a Korea University Grant (K1132871).

    Publisher Copyright:
    © 2018 The Author(s).


    • Computed tomography
    • Desmoid tumor
    • Desmoid-type fibromatosis
    • Retroperitoneum
    • Ultrasonography

    ASJC Scopus subject areas

    • Radiology Nuclear Medicine and imaging


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