Spontaneous Regression Of A Sporadic Intra-abdominal Located Desmoid-type Fibromatosis
Kloeze, J., & van Veen, M. (2019). Spontaneous regression of a sporadic intra-abdominal located desmoid-type fibromatosis. Journal of surgical case reports, 2019(2), rjz037. https://doi.org/10.1093/jscr/rjz037
View Original Source →Abstract
BACKGROUND: Desmoid-type fibromatosis (DTF) is a rare benign proliferation of myofibroblasts with an unpredictable disease course. Treatment of intra-abdominal located DTF is difficult because of the close relationship with vital organs. CASE PRESENTATION: A healthy young male presents with an asymptomatic palpable mass in the lower right abdominal quadrant. A computed tomography shows a 10 × 7 cm2 pear-shaped mass, and pathological examination revealed DTF. A watchful waiting approach was initiated, as the patient was asymptomatic and surgery would imply a significant amount of intestinal resection. After a follow-up of 2 years, the tumor has regressed spontaneously and the patient is still without symptoms. CONCLUSIONS: DTF is a difficult to treat condition where individualized management is appropriate. An asymptomatic patient could be treated with a watchful waiting approach, even with intra-abdominal location. Thereby sparing unnecessary morbidity as the tumor can be stable for many years or even regress spontaneously.
Case Details
Disease Location
Ureter
Personal Characteristics
24-year-old male
Clinical Characteristics
Referred under the suspicion of an inguinal hernia. He was seen at the emergency department with a palpable mass in the lower right abdominal quadrant, which he first noticed 5 days earlier. Sonography revealed a mass of 10.4 × 6.6 cm2. A computed tomography was done for further analysis, and showed an intra-abdominal located pear-shaped mass of 9.5 × 7.1 × 9.1 cm3 along the right ureter and surrounded by small intestines, without invasion of abdominal structures. A sonography-guided biopsy was performed, and pathological examination confirmed desmoid-type fibromatosis (dtf) with positive beta-catenin nuclear enhancement
Remission Characteristics
Sonography after three months showed a reduction in size to 9.4 × 5.4 cm2. 1 year after diagnosis, an MRI showed regression of the tumor with a small resi- due of 2.2 × 1.2 × 1.9 cm2 along the right ureter and a minimal hydronephrosis of the right kidney without loss of renal function. A second MRI 6 months later and a third MRI 1 year later showed persisted regression without hydronephrosis
Treatment & Mechanisms
Clinical Treatment
Biopsy