Correspondence Clinical Letter
Clinical Letter Pedunculated dermatofibrosarcoma protuberans: an unusual presentation
DOI: 10.1111/ddg.12416
the spindle cells extended radially to the superficial subcutaneous tissue (Figure 2a, e). Immunohistochemistry revealed that the spindle cells were diffusely positive for vimentin and CD34 (Figure 3a, b) and negative for CD68, Factor XIIIa, and S-100 protein. Based on the pathology and immunohistochemistry, we diagnosed the whole lesion as DFSP. The surgical margins were tumor-free, and 16 months after surgery, the patient remains free of disease.
Dear Editors,
Discussion
Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor with a low- to intermediate-grade of malignancy, characterized by an indolent, slow, but infiltrative growth, and a tendency for local recurrence. DFSP usually presents clinically as an indurated plaque or a raised slowly growing firm, nodular dermal-subcutaneous mass [1–3]. We recently encountered a pedunculated DFSP, an exceptional presentation that has only been reported once previously.
The most common clinical manifestation of DFSP is an asymptomatic indurated plaque. Although DFSP sometimes presents as a solid nodule or a tumor [4], it does not typically show a protuberant or nodular growth pattern until late in the course [5]. The growth pattern of DFSP can become deep or vertical in longstanding or recurrent tumors [3]. The present case had a unique pedunculated tumor, and the clinical features differed from those typical of DFSP. A pedunculated DFSP is extremely rare, and only one
History A 49-year-old man presented with a plaque in the left inguinal region. The plaque had been present for two years and had enlarged gradually; a firm nodule had appeared in the region over a period of roughly six months. Three months before presentation, a pedunculated soft mass had appeared in the plaque and this mass had also increased in size. Examination revealed a 10 × 8-cm slightly indurated plaque in the left inguinal region that contained a 3.5 × 2.5-cm firm, intradermal-subcutaneous nodule and a 3 × 3 × 2.5-cm brownish pedunculated soft mass (Figure 1a, b). Although the clinical findings were suggestive of DFSP, the pedunculated tumor itself was suggestive of a soft fibroma or a neurofibroma. Histological and immunohistochemical evaluation of the incisional biopsy taken from the intradermal nodule led to the diagnosis of DFSP. Under general anesthesia, a wide excision was performed with 3-cm margins and including the fascia lata. The surgical defect was covered by a split-thickness skin graft. Histologically, the surgical specimen included an exophytic pedunculated tumor and a dermal-subcutaneous tumor (Figure 2a). The pedunculated tumor was composed of spindle cells with wavy nuclei within a mucinous stroma (Figure 2b). These spindle cells were seen throughout the pedunculated lesion, but invasion of these cells was limited to the superficial subcutaneous tissue underneath the pedunculated tumor (Figure 2a, c). In contrast, the dermal-subcutaneous nodule was characterized mainly by nodular proliferation of fibroblast-like spindle cells arranged in a storiform pattern (Figure 2d). The deepest part of the nodule was located in the deep subcutaneous tissue. Between the two lesions,
1132
Figure 1 A 10 x 8-cm indurated plaque with a 3.5 x 2.5-cm firm nodule (white arrow) and a 3 x 3 x 2.5-cm brownish pedunculated soft mass (black arrow) (a). The stalk of the pedunculated mass (b).
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1212
Correspondence Clinical Letter
Figure 2 Histological findings of the dissected specimen (haematoxylin- eosin staining). Gross pathology of the entire lesion (original magnification x 1) (a). Most of the pedunculated lesion shows a less cellular zone consisting of spindle cells within a mucinous stroma (original magnification x 400) (b). The spindle cells extend around individual adipocytes (original magnification x 100) (c). The dermal-subcutaneous lesion is composed of fibroblast-like spindle cells arranged in a storiform pattern (original magnification x 400) (d). The spindle cells extend around individual adipocytes in the superficial layer of the subcutaneous tissue between the two tumors (original magnification x 100) (e).
Figure 3 An immunohistochemical study shows cytoplasmic positivity for CD34 (original magnification x 400). Spindle cells within a mucinous stroma in the pedunculated lesion (a). Spindle cells in the dermal-subcutaneous lesion (b).
previous case has been reported [6]. This was a solitary 1.2-cm pedunculated nodule on the thigh of a 26-year-old woman, which was clinically thought to represent either a neurofibroma or a skin tag. Histological examination of the biopsy specimen revealed proliferation of spindle cells with wavy nuclei associated with clefts in the stroma and areas of slightly increased mucin, resembling a neurofibroma. The authors interpreted these findings of subtle crowding of spindle cells as early DFSP, although three additional surgical pro-
cedures were required to eradicate the residual DFSP underneath the pedunculated lesion. In contrast, the pedunculated tumor in the present case developed late in the clinical course. The occurrence of such a lesion, with exophytic growth without deep invasion, at such a time in the clinical course is clearly exceptional. The histological findings of the pedunculated tumor were same as those of the case described above [6], suggesting that the histological findings of scant spindle cells with mucinous stroma
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1212
1133
Correspondence Clinical Letter
could indicate the exophytic growth of the tumor itself rather than early-stage DFSP. In addition, the soft nature of the pedunculated lesion in the present case, as in the previously reported case [6], is unusual for DFSP. The soft texture could be attributed to the histological characteristic of mucinous change in the stroma. The rare clinical features presented here made it difficult to diagnose the pedunculated tumor as DFSP. Furthermore, histologically, proliferation of spindle cells with wavy nuclei in a pedunculated tumor containing increased areas of mucinous stroma could be easily misdiagnosed as a neural tumor such as neurofibroma. Immunohistochemical study with CD34 is therefore essential for the diagnosis of a pedunculated lesion as DFSP.
Acknowledgement
Yasuhiro Nakamura Department of Skin Oncology/Dermatology Saitama Medical University International Medical Center 1397–1 Yamane, Hidaka Saitama 350–1298, Japan E-mail: [email protected]
References 1
2
This work was partly supported by National Cancer Center Research and Development Fund (26-A-4).
3
Conflict of interest None.
4
Yasuhiro Nakamura1, Michio Shimizu2, Yasuhiro Fujisawa3, Shijima Taguchi4, Yoshiyuki Nakamura3, Yukiko Teramoto1, Sayuri Sato1, Akifumi Yamamoto1 (1) Department of Skin Oncology/Dermatology, Saitama Medical University International Medical Center, Saitama, Japan (2) Department of Pathology, Saitama Medical University International Medical Center, Saitama, Japan (3) Department of Dermatology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan (4) Department of Dermatology, Mito Kyodo General Hospital, Mito, Japan
1134
Correspondence to
5
6
Zelger B, Sidoroff A, Stanzl U et al. Deep penetrating ermatofibroma versus dermatofibrosarcoma protuberans. d A clinicopathologic comparison. Am J Surg Pathol 1994; 18: 677–86. Sigel JE, Bergfeld WF, Goldblum JR. A morphologic study of dermatofibrosarcoma protuberans: expansion of a histologic profile. J Cutan Pathol 2000; 27: 159–63. Archontaki M, Korkolis DP, Arnogiannaki N et al. Dermatofibrosarcoma protuberans: a case series of 16 patients treated in a single institution with literature review. Anticancer Res 2010; 30: 3775–9. Oliveira-Soares R, Viana I, Vale E et al. Dermatofibrosarcoma protuberans: a clinicopathological study of 20 cases. J Eur Acad Dermatol Venereol 2002; 16: 441–6. Fiore M, Miceli R, Mussi C et al. Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol 2005; 23: 7669–75. Resnik KS, DiLeonardo M, Hunter CJ. Pedunculated presentation of dermatofibrosarcoma protuberans. J Am Acad Dermatol 2003; 49: 1139–41.
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1212
Clinical Letter Pedunculated dermatofibrosarcoma protuberans: an unusual presentation
DOI: 10.1111/ddg.12416
the spindle cells extended radially to the superficial subcutaneous tissue (Figure 2a, e). Immunohistochemistry revealed that the spindle cells were diffusely positive for vimentin and CD34 (Figure 3a, b) and negative for CD68, Factor XIIIa, and S-100 protein. Based on the pathology and immunohistochemistry, we diagnosed the whole lesion as DFSP. The surgical margins were tumor-free, and 16 months after surgery, the patient remains free of disease.
Dear Editors,
Discussion
Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor with a low- to intermediate-grade of malignancy, characterized by an indolent, slow, but infiltrative growth, and a tendency for local recurrence. DFSP usually presents clinically as an indurated plaque or a raised slowly growing firm, nodular dermal-subcutaneous mass [1–3]. We recently encountered a pedunculated DFSP, an exceptional presentation that has only been reported once previously.
The most common clinical manifestation of DFSP is an asymptomatic indurated plaque. Although DFSP sometimes presents as a solid nodule or a tumor [4], it does not typically show a protuberant or nodular growth pattern until late in the course [5]. The growth pattern of DFSP can become deep or vertical in longstanding or recurrent tumors [3]. The present case had a unique pedunculated tumor, and the clinical features differed from those typical of DFSP. A pedunculated DFSP is extremely rare, and only one
History A 49-year-old man presented with a plaque in the left inguinal region. The plaque had been present for two years and had enlarged gradually; a firm nodule had appeared in the region over a period of roughly six months. Three months before presentation, a pedunculated soft mass had appeared in the plaque and this mass had also increased in size. Examination revealed a 10 × 8-cm slightly indurated plaque in the left inguinal region that contained a 3.5 × 2.5-cm firm, intradermal-subcutaneous nodule and a 3 × 3 × 2.5-cm brownish pedunculated soft mass (Figure 1a, b). Although the clinical findings were suggestive of DFSP, the pedunculated tumor itself was suggestive of a soft fibroma or a neurofibroma. Histological and immunohistochemical evaluation of the incisional biopsy taken from the intradermal nodule led to the diagnosis of DFSP. Under general anesthesia, a wide excision was performed with 3-cm margins and including the fascia lata. The surgical defect was covered by a split-thickness skin graft. Histologically, the surgical specimen included an exophytic pedunculated tumor and a dermal-subcutaneous tumor (Figure 2a). The pedunculated tumor was composed of spindle cells with wavy nuclei within a mucinous stroma (Figure 2b). These spindle cells were seen throughout the pedunculated lesion, but invasion of these cells was limited to the superficial subcutaneous tissue underneath the pedunculated tumor (Figure 2a, c). In contrast, the dermal-subcutaneous nodule was characterized mainly by nodular proliferation of fibroblast-like spindle cells arranged in a storiform pattern (Figure 2d). The deepest part of the nodule was located in the deep subcutaneous tissue. Between the two lesions,
1132
Figure 1 A 10 x 8-cm indurated plaque with a 3.5 x 2.5-cm firm nodule (white arrow) and a 3 x 3 x 2.5-cm brownish pedunculated soft mass (black arrow) (a). The stalk of the pedunculated mass (b).
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1212
Correspondence Clinical Letter
Figure 2 Histological findings of the dissected specimen (haematoxylin- eosin staining). Gross pathology of the entire lesion (original magnification x 1) (a). Most of the pedunculated lesion shows a less cellular zone consisting of spindle cells within a mucinous stroma (original magnification x 400) (b). The spindle cells extend around individual adipocytes (original magnification x 100) (c). The dermal-subcutaneous lesion is composed of fibroblast-like spindle cells arranged in a storiform pattern (original magnification x 400) (d). The spindle cells extend around individual adipocytes in the superficial layer of the subcutaneous tissue between the two tumors (original magnification x 100) (e).
Figure 3 An immunohistochemical study shows cytoplasmic positivity for CD34 (original magnification x 400). Spindle cells within a mucinous stroma in the pedunculated lesion (a). Spindle cells in the dermal-subcutaneous lesion (b).
previous case has been reported [6]. This was a solitary 1.2-cm pedunculated nodule on the thigh of a 26-year-old woman, which was clinically thought to represent either a neurofibroma or a skin tag. Histological examination of the biopsy specimen revealed proliferation of spindle cells with wavy nuclei associated with clefts in the stroma and areas of slightly increased mucin, resembling a neurofibroma. The authors interpreted these findings of subtle crowding of spindle cells as early DFSP, although three additional surgical pro-
cedures were required to eradicate the residual DFSP underneath the pedunculated lesion. In contrast, the pedunculated tumor in the present case developed late in the clinical course. The occurrence of such a lesion, with exophytic growth without deep invasion, at such a time in the clinical course is clearly exceptional. The histological findings of the pedunculated tumor were same as those of the case described above [6], suggesting that the histological findings of scant spindle cells with mucinous stroma
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1212
1133
Correspondence Clinical Letter
could indicate the exophytic growth of the tumor itself rather than early-stage DFSP. In addition, the soft nature of the pedunculated lesion in the present case, as in the previously reported case [6], is unusual for DFSP. The soft texture could be attributed to the histological characteristic of mucinous change in the stroma. The rare clinical features presented here made it difficult to diagnose the pedunculated tumor as DFSP. Furthermore, histologically, proliferation of spindle cells with wavy nuclei in a pedunculated tumor containing increased areas of mucinous stroma could be easily misdiagnosed as a neural tumor such as neurofibroma. Immunohistochemical study with CD34 is therefore essential for the diagnosis of a pedunculated lesion as DFSP.
Acknowledgement
Yasuhiro Nakamura Department of Skin Oncology/Dermatology Saitama Medical University International Medical Center 1397–1 Yamane, Hidaka Saitama 350–1298, Japan E-mail: [email protected]
References 1
2
This work was partly supported by National Cancer Center Research and Development Fund (26-A-4).
3
Conflict of interest None.
4
Yasuhiro Nakamura1, Michio Shimizu2, Yasuhiro Fujisawa3, Shijima Taguchi4, Yoshiyuki Nakamura3, Yukiko Teramoto1, Sayuri Sato1, Akifumi Yamamoto1 (1) Department of Skin Oncology/Dermatology, Saitama Medical University International Medical Center, Saitama, Japan (2) Department of Pathology, Saitama Medical University International Medical Center, Saitama, Japan (3) Department of Dermatology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan (4) Department of Dermatology, Mito Kyodo General Hospital, Mito, Japan
1134
Correspondence to
5
6
Zelger B, Sidoroff A, Stanzl U et al. Deep penetrating ermatofibroma versus dermatofibrosarcoma protuberans. d A clinicopathologic comparison. Am J Surg Pathol 1994; 18: 677–86. Sigel JE, Bergfeld WF, Goldblum JR. A morphologic study of dermatofibrosarcoma protuberans: expansion of a histologic profile. J Cutan Pathol 2000; 27: 159–63. Archontaki M, Korkolis DP, Arnogiannaki N et al. Dermatofibrosarcoma protuberans: a case series of 16 patients treated in a single institution with literature review. Anticancer Res 2010; 30: 3775–9. Oliveira-Soares R, Viana I, Vale E et al. Dermatofibrosarcoma protuberans: a clinicopathological study of 20 cases. J Eur Acad Dermatol Venereol 2002; 16: 441–6. Fiore M, Miceli R, Mussi C et al. Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol 2005; 23: 7669–75. Resnik KS, DiLeonardo M, Hunter CJ. Pedunculated presentation of dermatofibrosarcoma protuberans. J Am Acad Dermatol 2003; 49: 1139–41.
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1212
