JEADV
LETTER TO THE EDITOR
Diagnosis of dermatofibrosarcoma protuberans and assessment of pre-surgical margins using highdefinition optical coherence tomography imaging Editor Dermatofibrosarcoma protuberans (DFSP) is a slow-growing, fibrohistiocytic tumour that corresponds to 0.1–1% of all skin malignant neoplasms. Its indolent course and lack of early clinical clues account for frequent delays in diagnosis.1 Recognition of six recently described dermoscopic features could raise the suspicion for DFSP, avoiding diagnostic pitfalls and improving patient outcome.2 DFSP has a high recurrence rate after incomplete surgical excision. Therefore, a 2–4 cm wide local excision (WLE) or Mohs micrographic surgery is considered the treatment of choice.3 Imaging studies can provide additional information concerning disease extent. Magnetic resonance imaging and Doppler ultrasonography have been used with variable results.4,5 Several non-invasive imaging techniques, including high-definition optical coherence tomography (HD-OCT), have emerged in recent years aiming for higher accuracy of in vivo diagnosis. HD-OCT is capable of capturing not only slice but also en face images in real-time and fast three-dimensional acquisition, enabling visualization of individual cells.6 Hence, it represents an ideal tool for diagnostic purposes in skin cancer, and also for monitoring surgical and non-surgical treatment.7,8 As an example of the potential of HD-OCT, revealing new cytomorphological details in DFSP, we report a 50-year-old man who presented with a 3-year history of an asymptomatic plaque located on his left thigh that slowly enlarged becoming nodular. The physical examination revealed an endurated plaque, centred
(a)
by a firm, mobile, pink nodule and 12 mm in diameter (Fig. 1a). Dermoscopy disclosed peripheral, unfocused arborizing vessels and structureless light brown areas; few coiled vessels and shiny white streaks were also observed (Fig. 1b). A biopsy specimen was taken and the histopathological evaluation showed a dermal proliferation of multiple monomorphous spindle cells with a dense, storiform arrangement. No fibrosarcomatous change was seen. The cells stained positive for CD34 supporting the diagnosis of DFSP. Further examination by HD-OCT was performed, both in the lesion and adjacent normal skin overlying the expected 2 cm wide surgical margin. Tumour evaluation using the slice mode (Fig. 2a) enabled the observation of a thickened dermis and a loss of the dermal–epidermal junction (DEJ). En face mode revealed a disarray of dermal layering, replacement of normal collagen bundles by less-reflective elongated parallel cells with clear cytoplasm, and widened vessels seen as pleomorphic dark structures (Fig. 2b). In contrast, examination of adjacent skin showed normal epidermal and dermal architectural features. Rings of bright cells surrounding dark dermal papillae were seen at the DEJ. The refractile nature of the collagen explained the high reflectivity seen in the normal dermis, where blood vessels were also clearly differentiated from surrounding structures (Fig. 2c–d). WLE with 2 cm margin was then performed. The differential diagnosis of DFSP is wide, including dermatofibroma, keloid and morpheaform basal cell carcinoma, which are more commonly seen in daily practice.1 Our case provided unique morphological features of DFSP using HD-OCT, supporting its potential as an adjuvant to clinical diagnosis. Thickening of the dermis, loss of collagen bundles and densely packed less reflective parallel cells correlated well to the dermal storiform spindle cells typically seen in histopathology. DFSP extent is difficult to assess intra-operatively with traditional surgical excision. Three-dimensional resection including skin, subcutaneous tissue and underlying fascia is then advised. HD-OCT offers a fast and simple in vivo skin examination, with increased
(b)
Figure 1 (a) Firm pink nodule over an ill-defined, endurated plaque on the left thigh. (b) Dermoscopy showed multiple peripheral unfocused vessels, few coiled vessels, structureless light brown areas and shiny white streaks.
JEADV 2015
© 2015 European Academy of Dermatology and Venereology
Letter to the Editor
2
(a)
(c)
(b)
(d)
Figure 2 High definition-optical coherence tomography examination revealed (a) Nodular lesion (slice mode): attenuation of overlying epidermis, effacement of dermal–epidermal junction, and thickened less-refractile dermis. (b) Nodular lesion (en face mode): densely packed elongated cells with pale cytoplasm (yellow circles) were seen around pleomorphic vessels in the dermis (red arrows); few remaining high-refractile collagen bundles were also observed (black arrow). (c) Adjacent normal skin at 2 cm margin (slice mode): normal threelayered epidermal structure and dermal architecture, with a blurred dermal–epidermal junction. (d) Adjacent normal skin at 2 cm margin (en face mode): high-refractile parallel collagen bundles (yellow circle) surrounding round vessels seen as dark structures (red arrows) in the dermis.
axial and lateral resolution, reaching the reticular dermis. In our case, normal skin features in HD-OCT imaging were seen at 2 cm margin, which was later supported by histopathological confirmation of clear margins. Therefore, recognition of DFSP and normal skin presentation using HD-OCT can provide a reliable and easy way to assess pre-surgical margins in DFSP, decreasing relapsing rates, and also allowing for smaller excisions as a sparing tissue method.9 A. Oliveira,1,* E. Arzberger,2 I. Zalaudek,2 R. Hofmann-Wellenhof2 Department of Dermatology, Hospital de Curry Cabral – Centro Hospitalar de Lisboa Central, Lisboa, Portugal, 2Department of Dermatology, Medical University of Graz, Graz, Austria *Correspondence: A. Oliveira. E-mail: [email protected]
1
References 1 Bogucki B, Neuhaus I, Hurst EA. Dermatofibrosarcoma protuberans: a review of the literature. Dermatol Surg 2012; 38: 537–551. 2 Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol 2014; 71: 781–786.
JEADV 2015
3 Bernard J, Poulalhon N, Argenziano G, Debarbieux S, Dalle S, Thomas L. Dermoscopy of dermatofibrosarcoma protuberans: a study of 15 cases. Br J Dermatol 2013; 169: 85–90. 4 Torreggiani W, Al-Ismail K, Munk P, Nicolaou S, O’Connell JX, Knowling MA. Dermatofibrosarcoma protuberans: MR imaging features. AJR Am J Roentgenol 2002; 178: 989–993. 5 Thornton W, Reid J, Papay F, Vidimos A. Childhood dermatofibrosarcoma protuberans: role of preoperative imaging. J Am Acad Dermatol 2005; 53: 76–83. 6 Alawi SA, Kuck M, Wahrlich C et al. Optical coherence tomography for presurgical margin assessment of non-melanoma skin cancer – a practical approach. Exp Dermatol 2013; 22: 547–551. 7 Maier T, Kulichova D, Ruzicka T, Kunte C, Berking C. Ex vivo high-definition optical coherence tomography of basal cell carcinoma compared to frozen-section histology in micrographic surgery: a pilot study. J Eur Acad Dermatol Venereol 2014; 28: 80–85. 8 Wang KX, Meekings A, Fluhr JW et al. Optical coherence tomographybased optimization of Mohs micrographic surgery of basal cell carcinoma: a pilot study. Dermatol Surg 2013; 39: 627–633. 9 Boone M, Jemec GBE, Del Marmol V. High-definition optical coherence tomography enables visualization of individual cells in healthy skin: comparison to reflectance confocal microscopy. Exp Dermatol 2012; 21: 740– 744. DOI: 10.1111/jdv.13007
© 2015 European Academy of Dermatology and Venereology
LETTER TO THE EDITOR
Diagnosis of dermatofibrosarcoma protuberans and assessment of pre-surgical margins using highdefinition optical coherence tomography imaging Editor Dermatofibrosarcoma protuberans (DFSP) is a slow-growing, fibrohistiocytic tumour that corresponds to 0.1–1% of all skin malignant neoplasms. Its indolent course and lack of early clinical clues account for frequent delays in diagnosis.1 Recognition of six recently described dermoscopic features could raise the suspicion for DFSP, avoiding diagnostic pitfalls and improving patient outcome.2 DFSP has a high recurrence rate after incomplete surgical excision. Therefore, a 2–4 cm wide local excision (WLE) or Mohs micrographic surgery is considered the treatment of choice.3 Imaging studies can provide additional information concerning disease extent. Magnetic resonance imaging and Doppler ultrasonography have been used with variable results.4,5 Several non-invasive imaging techniques, including high-definition optical coherence tomography (HD-OCT), have emerged in recent years aiming for higher accuracy of in vivo diagnosis. HD-OCT is capable of capturing not only slice but also en face images in real-time and fast three-dimensional acquisition, enabling visualization of individual cells.6 Hence, it represents an ideal tool for diagnostic purposes in skin cancer, and also for monitoring surgical and non-surgical treatment.7,8 As an example of the potential of HD-OCT, revealing new cytomorphological details in DFSP, we report a 50-year-old man who presented with a 3-year history of an asymptomatic plaque located on his left thigh that slowly enlarged becoming nodular. The physical examination revealed an endurated plaque, centred
(a)
by a firm, mobile, pink nodule and 12 mm in diameter (Fig. 1a). Dermoscopy disclosed peripheral, unfocused arborizing vessels and structureless light brown areas; few coiled vessels and shiny white streaks were also observed (Fig. 1b). A biopsy specimen was taken and the histopathological evaluation showed a dermal proliferation of multiple monomorphous spindle cells with a dense, storiform arrangement. No fibrosarcomatous change was seen. The cells stained positive for CD34 supporting the diagnosis of DFSP. Further examination by HD-OCT was performed, both in the lesion and adjacent normal skin overlying the expected 2 cm wide surgical margin. Tumour evaluation using the slice mode (Fig. 2a) enabled the observation of a thickened dermis and a loss of the dermal–epidermal junction (DEJ). En face mode revealed a disarray of dermal layering, replacement of normal collagen bundles by less-reflective elongated parallel cells with clear cytoplasm, and widened vessels seen as pleomorphic dark structures (Fig. 2b). In contrast, examination of adjacent skin showed normal epidermal and dermal architectural features. Rings of bright cells surrounding dark dermal papillae were seen at the DEJ. The refractile nature of the collagen explained the high reflectivity seen in the normal dermis, where blood vessels were also clearly differentiated from surrounding structures (Fig. 2c–d). WLE with 2 cm margin was then performed. The differential diagnosis of DFSP is wide, including dermatofibroma, keloid and morpheaform basal cell carcinoma, which are more commonly seen in daily practice.1 Our case provided unique morphological features of DFSP using HD-OCT, supporting its potential as an adjuvant to clinical diagnosis. Thickening of the dermis, loss of collagen bundles and densely packed less reflective parallel cells correlated well to the dermal storiform spindle cells typically seen in histopathology. DFSP extent is difficult to assess intra-operatively with traditional surgical excision. Three-dimensional resection including skin, subcutaneous tissue and underlying fascia is then advised. HD-OCT offers a fast and simple in vivo skin examination, with increased
(b)
Figure 1 (a) Firm pink nodule over an ill-defined, endurated plaque on the left thigh. (b) Dermoscopy showed multiple peripheral unfocused vessels, few coiled vessels, structureless light brown areas and shiny white streaks.
JEADV 2015
© 2015 European Academy of Dermatology and Venereology
Letter to the Editor
2
(a)
(c)
(b)
(d)
Figure 2 High definition-optical coherence tomography examination revealed (a) Nodular lesion (slice mode): attenuation of overlying epidermis, effacement of dermal–epidermal junction, and thickened less-refractile dermis. (b) Nodular lesion (en face mode): densely packed elongated cells with pale cytoplasm (yellow circles) were seen around pleomorphic vessels in the dermis (red arrows); few remaining high-refractile collagen bundles were also observed (black arrow). (c) Adjacent normal skin at 2 cm margin (slice mode): normal threelayered epidermal structure and dermal architecture, with a blurred dermal–epidermal junction. (d) Adjacent normal skin at 2 cm margin (en face mode): high-refractile parallel collagen bundles (yellow circle) surrounding round vessels seen as dark structures (red arrows) in the dermis.
axial and lateral resolution, reaching the reticular dermis. In our case, normal skin features in HD-OCT imaging were seen at 2 cm margin, which was later supported by histopathological confirmation of clear margins. Therefore, recognition of DFSP and normal skin presentation using HD-OCT can provide a reliable and easy way to assess pre-surgical margins in DFSP, decreasing relapsing rates, and also allowing for smaller excisions as a sparing tissue method.9 A. Oliveira,1,* E. Arzberger,2 I. Zalaudek,2 R. Hofmann-Wellenhof2 Department of Dermatology, Hospital de Curry Cabral – Centro Hospitalar de Lisboa Central, Lisboa, Portugal, 2Department of Dermatology, Medical University of Graz, Graz, Austria *Correspondence: A. Oliveira. E-mail: [email protected]
1
References 1 Bogucki B, Neuhaus I, Hurst EA. Dermatofibrosarcoma protuberans: a review of the literature. Dermatol Surg 2012; 38: 537–551. 2 Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol 2014; 71: 781–786.
JEADV 2015
3 Bernard J, Poulalhon N, Argenziano G, Debarbieux S, Dalle S, Thomas L. Dermoscopy of dermatofibrosarcoma protuberans: a study of 15 cases. Br J Dermatol 2013; 169: 85–90. 4 Torreggiani W, Al-Ismail K, Munk P, Nicolaou S, O’Connell JX, Knowling MA. Dermatofibrosarcoma protuberans: MR imaging features. AJR Am J Roentgenol 2002; 178: 989–993. 5 Thornton W, Reid J, Papay F, Vidimos A. Childhood dermatofibrosarcoma protuberans: role of preoperative imaging. J Am Acad Dermatol 2005; 53: 76–83. 6 Alawi SA, Kuck M, Wahrlich C et al. Optical coherence tomography for presurgical margin assessment of non-melanoma skin cancer – a practical approach. Exp Dermatol 2013; 22: 547–551. 7 Maier T, Kulichova D, Ruzicka T, Kunte C, Berking C. Ex vivo high-definition optical coherence tomography of basal cell carcinoma compared to frozen-section histology in micrographic surgery: a pilot study. J Eur Acad Dermatol Venereol 2014; 28: 80–85. 8 Wang KX, Meekings A, Fluhr JW et al. Optical coherence tomographybased optimization of Mohs micrographic surgery of basal cell carcinoma: a pilot study. Dermatol Surg 2013; 39: 627–633. 9 Boone M, Jemec GBE, Del Marmol V. High-definition optical coherence tomography enables visualization of individual cells in healthy skin: comparison to reflectance confocal microscopy. Exp Dermatol 2012; 21: 740– 744. DOI: 10.1111/jdv.13007
© 2015 European Academy of Dermatology and Venereology
