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The Desmoid Tumor Research Foundation

October 2023

Unusual Presentation of an Uncommon Malignancy: A 74-Year-Old Woman with Aggressive Fibromatosis of the Large Intestine Presenting as a Liver Mass and the Therapeutic Management

Author Information

Christos Vallilas 1, Stavros P Papadakos 2, Theodoros Androutsakos 3, Alexandros Pergaris 4, Panagiotis Sarantis 1, Eirini A Apostolidi 3, John Griniatsos 5, Michalis V Karamouzis 1, Stavros Sougioultzis 3

1Molecular Oncology Unit, Department of Biological Chemistry, Medical School, National and Kapodistrian University of Athens, Athens, Greece. 2Department of Gastroenterology, General Hospital of Athens "Laiko" Medical School of National and Kapodistrian University of Athens, Athens, Greece. 3Department of Pathophysiology, "Laikon" General Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece. 4First Department of Pathology, Medical School, National and Kapodistrian University of Athens, Athens, Greece. 5First Department of Surgery, Laiko Hospital, National and Kapodistrian University of Athens, Medical School, Athens, Greece.

Publication

Am J Case Rep

Summary

BACKGROUND Desmoid tumors are a fibroblastic proliferation of soft tissues, with an extreme inclination for local dissemination and recurrence. Surgical excision is the usual treatment choice, with data regarding pharmaceutical treatment being scarce. CASE REPORT A 74-year-old female patient was admitted to "Laikon" General Hospital of Athens, Greece presenting with acute kidney injury secondary to diarrhea. The ultrasound, CT, and abdominal MRI performed showed a 12×6×10 cm tumorous liver lesion. Biopsy of the lesion revealed loosely organized, mesenchymal tissue with spindle cells, and myxoid stroma. Immunochemistry was positive for SMA and b-catenin. Right hemicolectomy was performed with tumor-free surgical margins (R0 resection) and tamoxifen was initiated. Six months after the last MRI (3 months after the use of tamoxifen), a follow-up MRI was performed. The tumor had increased to 14.2×11×12.3 cm, and at the next follow-up it had grown to 20.3×19 cm maximal dimensions; no new metastases were found. The patient received sorafenib and pazopanib. Our patient had PFS with sorafenib for more than 2 years and remained in a good performance status (ECOG 1). For Pazopanid, the median PFS for this treatment option was 6.5 months. CONCLUSIONS The results were good and show a promising method for the treatment of this rare but severe malignancy.

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