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Giant desmoid fibromatosis of the pancreas

  • Jeng-Chang Chen
    Correspondence
    Corresponding author. Department of Surgery, Chang Gung Children's Hospital, 5, Fu-Shin Street, Kweishan, Taoyuan 333, Taiwan. Fax: +886 3 3288957.
    Affiliations
    Department of Surgery, Chang Gung Children's Hospital, College of Medicine, Chang Gung University, Taoyuan 333, Taiwan
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  • Shih-Chiang Huang
    Affiliations
    Department of Anatomical Pathology, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, Taoyuan 333, Taiwan
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Open AccessPublished:October 26, 2022DOI:https://doi.org/10.1016/j.pedneo.2022.08.004
      A 15-year-old girl had abdominal distension and appetite loss with episodic heartburn and vomiting for approximately 2 months, but gained 3 kg of weight. Physical examination revealed a huge and hard abdominal mass. Tumor markers including α-fetoprotein, β-human chorionic gonadotropin, carcinoembryonic antigen, CA125, and CA19-9 were all normal. Computed tomography disclosed a gigantic solid mass, occupying the whole abdominopelvic cavity (Fig. 1). Notably, the pancreas was only identifiable by its head and contiguous splenic vein. However, this tumor neither involved other intraabdominal vital structures nor caused paraaortic lymphadenopathy. Core needle biopsy showed hypocellular collagenized spindle-cell neoplasm without nuclear hyperchromasia and atypia, suggestive of desmoid fibromatosis (DF). This tumor was extirpated surgically (Fig. 2A), measuring 36 × 33 × 17 cm and weighing 13.4 kg. Histologically (Fig. 2B–D), it infiltrated the normal pancreas and expressed nuclear β-catenin.
      • Bhattacharya B.
      • Dilworth H.P.
      • Iacobuzio-Donahue C.
      • et al.
      Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions.
      Postoperatively, colonoscopy found no polyps. So far, she has been tumor-free for 12 months.
      Figure 1
      Figure 1Computed tomography (CT) of the abdomen, axial (left panels), sagittal (middle panels), and coronal (right panels) views, disclosed an exaggeratedly huge solid tumor, which had comparable CT density to the pancreas and kidneys, and slight contrast enhancement (left panels). The pancreas had missing body and tail, leaving the identifiable head (asterisk) and splenic vein (solid arrow). There was neither paraaortic lymphadenopathy nor radiographic evidence of tumor abutment or encasement of major vessels, including superior mesenteric vessels (open arrowheads), inferior vena cava (v), aorta (a), celiac trunk (open arrow), and hepatic portal vessels (solid arrowhead).
      Figure 2
      Figure 2(A) Cut surface of the resected DF showed a glistening trabecular appearance, and appeared whitish gray. There was no hemorrhage and necrosis. (B) Hematoxylin and eosin staining of specimen sections disclosed hypocellular neoplasm along with normal pancreatic tissues (open arrowheads) in abundant collagenous stroma. (C) In a magnified view, spindle-shaped tumor cells, arranged in well-defined fascicles, exhibited bland nuclear morphology as evidenced by the absence of nuclear hyperchromasia and atypia. (D) Immunohistochemical staining demonstrated the nuclear localization of β-catenin (open arrows) in spindle-shaped tumor cells. The nuclear counterstaining also showed fine chromatin and inconspicuous nucleoli of the tumor cell nuclei.
      DF is characterized by its clonally fibroblastic proliferation and locally infiltrative and aggressive growth,
      Desmoid Tumor Working Group
      The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients.
      leading to a tendency of local recurrence following resection despite its inability to metastasize. Thus, it has a variable and often unpredictable clinical course. Intraabdominal DF was relatively rare but frequently occurred in familial adenomatous polyposis.
      • Church J.
      • Xhaja X.
      • LaGuardia L.
      • O'Malley M.
      • Burke C.
      • Kalady M.
      Desmoids and genotype in familial adenomatous polyposis.
      Patients tended to be asymptomatic until signs of DF mass effects or local invasion.
      • Xu B.
      • Zhu L.H.
      • Wu J.G.
      • Wang X.F.
      • Matro E.
      • Ni J.J.
      Pancreatic solid cystic desmoid tumor: case report and literature review.
      Generally, active surveillance is the initial approach to DF. However, surgery remains the first-line treatment in the case of the limited surgical morbidities anticipated for symptomatic DF that grows rapidly,
      Desmoid Tumor Working Group
      The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients.
      ,
      • Orbach D.
      • Brennan B.
      • Bisogno G.
      • et al.
      The EpSSG NRSTS 2005 treatment protocol for desmoid-type fibromatosis in children: an international prospective case series.
      as with this patient whose DF expanded on such an unprecedented scale but was considered operable based on radiographic studies. The DF therapeutic algorithm makes no distinction between adults
      Desmoid Tumor Working Group
      The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients.
      and children.
      • Orbach D.
      • Brennan B.
      • Bisogno G.
      • et al.
      The EpSSG NRSTS 2005 treatment protocol for desmoid-type fibromatosis in children: an international prospective case series.

      Funding

      This work was supported by CMRPG3M0191 from Chang Gung Medical Foundation , Taiwan.

      Patient consent

      Obtained.

      Author contributions

      J-C.C treated the patient, wrote the manuscript and constructed the figures. S–C. H took the photographs of surgical specimens and tumor histology.

      Declaration of competing interest

      The author discloses no conflicts.

      References

        • Bhattacharya B.
        • Dilworth H.P.
        • Iacobuzio-Donahue C.
        • et al.
        Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions.
        Am J Surg Pathol. 2005; 29: 653-659
        • Desmoid Tumor Working Group
        The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients.
        Eur J Cancer. 2020; 127: 96-107
        • Church J.
        • Xhaja X.
        • LaGuardia L.
        • O'Malley M.
        • Burke C.
        • Kalady M.
        Desmoids and genotype in familial adenomatous polyposis.
        Dis Colon Rectum. 2015; 58: 444-448
        • Xu B.
        • Zhu L.H.
        • Wu J.G.
        • Wang X.F.
        • Matro E.
        • Ni J.J.
        Pancreatic solid cystic desmoid tumor: case report and literature review.
        World J Gastroenterol. 2013; 19: 8793-8798
        • Orbach D.
        • Brennan B.
        • Bisogno G.
        • et al.
        The EpSSG NRSTS 2005 treatment protocol for desmoid-type fibromatosis in children: an international prospective case series.
        Lancet Child Adolesc Health. 2017; 1: 284-292