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CASE 60

CASE 60

By: Dr. Ahmad M. Aljefri M.B.B.S

HISTORY: Flank mass.

What are your findings?

What is the differential diagnosis?

What are the causes?

FINDINGS:

Axial T1, T2 fat suppressed, coronal HASTE and post contrast axial images demonstrate a subcutaneous mass in close proximity to the adjacent abdominal wall muscles. It exhibits iso-signal intensity to the adjacent skeletal muscles on T1, heterogenous and predominantly low signal on T2fs and HASTE images. On post contrast administration, there’s a little to no enhancement.

DIFFERENTIA DIAGNOSIS:

Fibromatosis, hematoma, calcified tumor.

DIAGNOSIS:

Fibromatosis.

PEARLS AND DISCUSSION:

Soft tissue fibromatosis is a wide range entity of fibroblastic and myofibroblastic proliferation that share the presence of spindle cells fibroblasts with little to no mitotic activity on histopathological analysis. In 2002, the World Health Organization (WHO) Committee for Classification of Soft Tissue Tumors classified these lesions, in accordance to their anatomical location, into superficial and deep ones. The superficial fibromatoses include palmar and plantar fibromatosis. The deep fibromatoses include desmoid type and abdominal wall fibromatosis. There are several types of fibromatosis primarily affecting children including fibromatosis colli, lipofibromatosis, calcifying aponeurotic fibroma, inclusion body fibromatosis, and myofibroma and myofibromatosis.

Deep fibromatosis includes intra-abdominal desmoid, abdominal wall desmoid and extra-abdominal desmoid tumors.  Intra-abdominal desmoid tumors can occur in the pelvis in form of pelvic fibromatosis or in the mesentery as a component of Gardner’s syndrome. Extra-abdominal desmoid and abdominal wall desmoid tumors are distinct clinical entities as the latter exhibits a predilection for young women occurring around or during a pregnancy and might regress after it. Extra-abdominal desmoid on the other hand occurs between puberty and the age of 40.  is known by several names including aggressive fibromatosis and well-differentiated fibrosarcoma.

Radiography is often non-specific and might show a silhouette of a soft tissue mass. Calcification is rare. Ultrasound examination of these lesions will demonstrate a characteristic acoustic shadowing. On magnetic resonance, the MR signal is variable and depends on the histological composition and different components of collagen and the cellular component. On T1 weighted images, the exhibit an iso-signal to skeletal muscles or slightly higher and on T2-weighted images these lesions demonstrate a low signal intensity lower than the muscles and there may be some areas of high signal intensity pertaining to the cellular component. The cellular component contributes to the avid enhancement on post contrast administration. It is notable that necrosis and hemorrhage are not salient features of these lesion. Invasion or displacement of the adjacent soft tissues are known growth pattern. Depending on the collagen component, if abundant, these lesions will be very hard by core needle image guided biopsy. Larger bore needles such as 12-14-gauge might be useful.

Fibromatosis is notoriously known for local recurrence with little to no metastasizing potential. Treatment options includes surgical excision, chemotherapy. NSAID are recently used to treat these lesions to halt their growth. Image guided procedures such as cryoablation demonstrated promising results while radiofrequency ablation has been reported used in recurrent fibromatosis.

FURTHER READING:

  1. Robbin, M. R., Murphey, M. D., Temple, H. T., Kransdorf, M. J., & Choi, J. J. (2001). Imaging of Musculoskeletal Fibromatosis. RadioGraphics, 21(3), 585–600. doi:10.1148/radiographics.21.3.g01ma21585

  2. Lee, J. C., Thomas, J. M., Phillips, S., Fisher, C., & Moskovic, E. (2006). Aggressive Fibromatosis: MRI Features with Pathologic Correlation. American Journal of Roentgenology, 186(1), 247–254. doi:10.2214/ajr.04.1674

  3. Lee, J. C., Thomas, J. M., Phillips, S., Fisher, C., & Moskovic, E. (2006). Aggressive Fibromatosis: MRI Features with Pathologic Correlation. American Journal of Roentgenology, 186(1), 247–254. doi:10.2214/ajr.04.1674

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