Imaging features of dermatofibrosarcoma protuberans with pathologic correlation and assessments for recurrence potential

Congress:

ECR 2013

Poster Number:

C-2231

Type:

Educational Exhibit

Keywords:

Pathology, Metastases, Cancer, Surgery, Diagnostic procedure, MR, CT, Soft tissues / Skin, Musculoskeletal soft tissue

Authors:

H. Goto, Y. Iwama, M. Kunisada, M. Fujii, C. Nisigori, K. Sugimura; Kobe city/JP

DOI:

10.1594/ecr2013/C-2231

DOI-Link:

https://dx.doi.org/10.1594/ecr2013/C-2231

Table 1: Table.1 Clinical characteristics of patients with DFSP

Table 2: Table.2 Association between local recurrence after wide excision and imaging...

Fig. 2: Figure 2 Sagittal MRI of the femur Patient with classical DFSP with a nodular...

Fig. 3: Figure 3 Axial MRI of the chest Patient with classical DFSP with infiltration...

Learning objectives

To comprehensively review the typical radiological appearance of dermatofibrosarcoma protuberans (DFSP), elucidate pathologic correlations in order to aid understanding of the imaging appearances encountered, and discuss DFSP imaging characteristics of DFSP that may reflect a high potential for local recurrence.

Background

Introduction Dermatofibrosarcoma protuberans Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing, fibrohistiocytic neoplasm with intermediate- to low-grade malignancy that is most commonly found in young to middle-aged adults. It is seen on many body surfaces: mainly on the trunk (42-72%), followed by the extremities (16-30%), and less commonly in the head and neck (10-16%).[1] Cytogenetic analysis of DFSP shows ring chromosomes or translocations that result in fusion of 17q22 and 22q13, [2,3]the location of the collagen type 1 alpha 1 gene and the platelet-derived growth factor...

Imaging findings OR Procedure details

Patients and methods This study included all patients with DFSP seen in the Departments of Dermatology, Plastic Surgery, and Orthopedics at Kobe University Hospital between April 2005 and April 2011. Clinical records were obtained from medical records. The patient age range was from 12 to 73 years, and the average age was 42 years. A total of 10 men and three women were included. All cases were diagnosed at Kobe University in the Department of Anatomical Pathology using conventional histologic analysis with hematoxylin and eosin...

Conclusion

No relationship was found between imaging appearance and local DFSP recurrence in the present series of 13 patients. Identifying DFSP during imaging and diagnosis along with performing a wide local resection are important for successful management of DFSP.

References

references 1.Chang CK, Jacobs IA, Salti GI. Outcomes of surgery for dermatofibrosarcoma protuberans. Eur J Surg Oncol.2004;30:341-5. 2. Altman DA, Nickoloff BJ, Fivenson DP. Differential expression of factor XIIIa and CD34 in cutaneous mesenchymal tumors. J Cutan Pathol.1993;20:154-8. 3.Abenoza P, Lillemoe T. CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. Am J Dermatopathol.1993;15:429-34. 4.Simon MP, Pedeutour F, Sirvent N, et.al. Deregulation of the platelet-derived growth factor B-chain gene via fusion with collagen gene COL1A1 in dermatofibrosarcoma protuberans and giant-cell fibroblastoma....

Personal Information

Hajimu Goto, MD Radiology Department, Kobe University School of Medicine 7-5-2 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan