대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Desmoid tumor (Aggressive fibromatosis)

Radiologic Findings

Fig 1. Chest PA shows a left retrocardiac paravertebral mass-like opacity.
Fig 2-4. The chest CT images demonstrate an enhancing infiltrative left paravertebral soft-tissue lesion with obliteration of the fat plane from the aorta.
Fig 5. Axial fused FDG PET/CT image reveals an FDG-avid focus at the corresponding left paravertebral soft-tissue lesion.

Brief Review

Mediastinal mass excision with en bloc partial resection of parietal pleura and descending thoracic aorta and patch repair of the aorta was performed. The final pathological diagnosis was aggressive fibromatosis (desmoid).
Desmoid tumors (DFs; aggressive fibromatosis, deep musculoaponeurotic fibromatosis) are locally aggressive tumors with no known potential for metastasis or dedifferentiation. DFs are rare; The estimated incidence in the general population is 2-4 per million population per year. Most desmoids arise sporadically, although 5-15 percent are associated with familial adenomatous polyposis.
On CT, DF appears as a soft-tissue mass, either well-marginated, as most commonly seen in abdominal-wall tumors, or with ill-defined infiltrative margins, as seen in extra-abdominal or mesenteric tumors. DF shows variable attenuation, similar to or slightly higher than that demonstrated by skeletal muscle, with hyper- and hypoattenuation, probably reflecting collagen and myxoid elements, respectively. Enhancement is variable, with the majority of the masses demonstrating mild-to-moderate enhancement.
DF is not typically very metabolically active and often demonstrates standardized uptake values (SUVmax) of less than or equal to 4.8. However, increased uptake may occur in more cellular areas.
DFs have an unpredictable clinical course, and close observation is the preferred strategy for stable, asymptomatic primary or recurrent desmoids. Surgical resection has been the cornerstone of treatment for DF. The overall surgical strategy should also use function-preserving surgical approaches to minimize major morbidity (functional and/or cosmetic).

References

1. Marta Braschi-Amirfarzan, Abhishek R. Keraliya, Katherine M. Krajewski. Et al. Role of Imaging in Management of Desmoid-type Fibromatosis: A Primer for Radiologists. RadioGraphics 2016 36:3, 767-782.
2. Rosa, F., Martinetti, C., Piscopo, F. et al. Multimodality imaging features of desmoid tumors: a head-to-toe spectrum. Insights Imaging 11, 103 (2020).

Keywords

Thoracic Desmoid Tumor (Fibromatosis) ,