Mammogram on mediolateral oblique projection showing a well-defined mass with irregular margins, bilobulated density without any calcifications. 

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... and fifth decades of life. The tumor grows slowly over years and rarely metastasizes; however, local recurrence is frequent. The most common sites for metastasis are lymph nodes and the lungs. The treatment of the tumor is resection with wide margins (1,2). Here we present radiologic findings of DFSP with un- usual presentation located in the deep skin over the breast, which was excised successfully and without local recurrence during a 36-month follow-up period. A 36-year-old female with a palpable right breast mass located in the upper inner quadrant and change in skin color without a significant history was admitted to surgery department. She claimed that the mass had first appeared two years before and was slowly growing. She had no history of recent trauma or surgical operation. On inspection, a well circumscribed, plaque type lesion was ob- served, along with a violet-purple change in color of the skin over the right breast (Fig. 1). The patient reported neither systemic disease or known illness nor continuous drug usage. Physical examination was unremarkable except for a well-defined non-tender mass on the right breast. The left breast and bilateral axillaries were normal. The patient was referred for radiologic examination to exclude a primary breast tumor. Bilateral breast ultrasound (US) and color Doppler ultrasound (CDUS) was performed with a Toshiba, Powervision 6000 SSA-370A (Tokyo, Japan) with 6-11 MHz high frequency linear transducer.US demonstrated a 3.5x1.5 cm sized solid, hyperechogenic lesion with sharp margins in the subcutaneous fat tissue, without marked vascularity on CDUS (Fig. 2). The left breast and both axillaries were unremarkable. The right mammography (HFXPlus-Fischer Imaging, Denver, USA) on mediolateral oblique (MLO) projection showed a well-defined bilobulated density with irregular margins and no calcifications (Fig. 3). Based on the mammography and sonography findings, the mass was considered suspect of ma- lignancy and breast magnetic resonance imaging (MRI) study was planned for further examination. A contrast-enhanced breast MRI was performed using a 1.5 tesla MR unit (Somatom Vision Plus, Siemens, Erlangen, Germany) at our institution. Unfortunately, the mass could not be demonstrated on either T1, T2 or postcontrast subtracted images (Fig. 4). The patient was referred to surgery department and wide excision of the mass was performed. On pathologic evaluation, spindle cells were seen to be arranged in a storiform pattern, with minimal pleomorphism (Fig. 5). Immunohistochemical stain with CD34 was positive (Fig. 6) and definitive histologic diagnosis was DFSP. On follow-up, 36 months after surgical treatment, the patient continued to be symptom free, with no signs of tumor recurrence. Dermatofibrosarcoma protuberans is a rare fi- brous tumor of the soft tissue, commonly arising from dermis and subcutaneous tissue, which was first described by Darier and Ferran in 1924 (3). It ac- counts for 0.1% of all malignant tumors and 1% of all soft tissue sarcomas. The tumor is mostly located on the trunk skin (50%-60%) and is more common in men than women. The majority of these tumors are less than 5 cm in diameter. It can be seen at any age but it is much more common between ages 20 and 50, however, there are few cases reported in early childhood. DFSP has an indolent growth pattern and its symptoms are mostly long lasting, spanning over months and years (1,2,4). DFSP poses a diagnostic challenge as the clinical symptoms and the radiologic signs are nonspecific. The tumor usually causes a red-purple change of color on the overlying skin on inspection, and if it presents with a red and irregular bordered lesion, it can mimic a hemangioma. When DFSP is located in the breast, it can be mistaken for primary breast tumor and the accurate diagnosis is difficult to reach. Usually after clinical evaluation, US is the first choice for imaging and CDUS is very helpful for vascular situation but neither of the modalities is specific for diagnosis. Shin et al . report that a diagnosis of DFSP should be considered if US reveals an oval mass in the subcutaneous tissue that is abutting against the skin and has a focal lobulated margin with hypoechogenicity or an irregular margin with mixed echogenicity (5). Although an echogenic macrolobulated oval mass located over the breast lying in the deep dermis and the subcutaneous tissue without marked vasculariza- tion was demonstrated, an exact diagnosis could not be made in the present study. Unfortunately, mammography was also found to be nonspecific, with macrolobulated density on MLO projection. Consid- ering mammography and sonography findings, a primary breast tumor could not be excluded and the present case was classified as BI-RADS category 4. Computed tomography is not indicated unless bony invasion or pulmonary metastasis is suspected in some occasional cases. Although MRI is also nonspecific for the exact diagnosis of DFSP, it is useful for identifying the extent and location of the mass, es- pecially in large recurrent tumors. In addition, the areas of hemorrhage within the tumor may be demonstrated by use of MRI and can suggest the diagnosis (6,7). Another current imaging modality, multivoxel proton [1H]) MR spectroscopy (MRS), is accepted as an adjunctive method to breast MRI on differential diagnosis of benign versus malignant tumors. A recent case study of DSFP located on the breast, reported by Ivanovic et al ., did not show significant cholin peak on [1H] MRS (8). In the present case, the mass could not be demonstrated on either T1 or T2 weighted images, and there was no significant enhancement on postcontrast subtracted images on breast MRI examination. It is considered that the discrimination failed both on pre- and post-contrast images as the mass was probably carrying similar signal characteristics of the adjacent subcutaneous fat tissue in which the lesion developed. Complete surgical resection is accepted as the op- timal treatment for primary or recurrent DFSP. Studies have shown that resection with wide margins is essential and recurrence rates after local resection are reduced while the excision margins are widened. The combination of adjuvant radiotherapy before or after the surgery is a treatment option, particularly in those who cannot undergo wide surgical excision for several reasons. In addition, there are some success- ful clinical reports on imatinib, a tyrosine kinase in- hibitor, which can induce regression in patients with unresectable or metastatic DFSP (1,2,4). Proper follow-up seems critical since the most significant characteristic of the tumor is recurrence, and local recurrence generally occurs in the first 3 years after surgery. Sonographic evaluation and re-biopsy is advisable if suspicion occurs. In addition, mammography might be helpful in the diagnostic work- up in breast located tumors for periodic control of recurrence. In conclusion, DFSP is a rare soft tissue tumor of cutaneous origin that mostly occurs on the trunk and might be rarely seen on the breast skin and can be confused with primary breast tumors. We believe that the importance of the present case is to upgrade the awareness of this soft tissue tumor while keep- ing in mind the differential diagnosis of other breast tumors. 1. Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibrosarcoma protuberans. Cancer 2004;101:2503-8. 2. McArthur G. Dermatofibrosarcoma protuberans: recent clinical progress. Ann Surg Oncol 2007;14:2876-86. 3. Darier J, Ferrand M. Dermatofibromes progressifs et recidivants ou fibrosarcomes de la peau. Ann Dermatol Syphiligr 1924;5:542-62. 4. Sanmartín O, Llombart B, López-Guerrero JA, Ser- ra C, Requena C, Guillén C. Dermatofibrosarcoma protuberans. Actas Dermosifiliogr 2007;98:77-87. 5. Shin YR, Kim JY, Sung MS, Jung JH. Sonographic findings of dermatofibrosarcoma protuberans with pathologic correlation. J Ultrasound Med 2008;27:269-74. 6. Torreggiani WC, Al-Ismail K, Munk PL, Nicolaou S, O’Connell JX, Knowling MA. Dermatofibrosarcoma protuberans: MR imaging features. A JR Am J Roentgenol 2002;178:989-93. 7. Beaman FD, Kransdorf MJ, Andrews TR, Murphey MD, Arcara LK, Keeling JH. Superficial soft-tissue masses: analysis, diagnosis, and differential consi- derations. Radiographics 2007;27:509-23. 8. Djilas-Ivanovic D, Prvulovic N, Bogdanovic-Stoja- novic D, Vicko F, Sveljo O, Ivkovic-Kapicl T. Derma- tofibrosarcoma protuberans of the breast: mam- mographic, ultrasound, MRI and MRS features. Arch Gynecol Obstet 2009;280:827-30. Systemic scleroderma (SS) is a systemic disorder of the connective tissue, of obscure etiology and characterized by fibrosis of the skin, visceral organs and vascular walls. Clinically, it is a heterogeneous disorder characterized by high collagen deposition into the connective tissue, microcirculation abnormalities, and immune system impairment (1). It may be localized to the skin and show a mild clinical course, or may involve visceral organs, kidneys, lungs, heart and gastrointestinal organs, assuming a malignant course (1). The pathophysiology of SS is complex. En- vironmental, genetic, vascular and immune factors, fibroblasts and matrix substances seem to be impli- cated in the pathogenesis of the disease, thus SS is classified in autoimmune diseases, or generally in connective tissue disorders (2). Immune fibroblast stimulation occurs due to some unknown reason, which is followed by an enhanced collagen production, vascular endothelial lesions and fibrosis (2). Raynaud’s phenomenon is pronounced in SS, found in 90%-98% of cases (1,2); however, ...