Letters to the Editor
(3/50 high-power fields) are associated with increased rate of metastasis (25% of cases) and a poor 5-year survival (59%).2 EHE mainly occur at muscles of extremities, soft tissues and visceral organs such as the liver and lung. Primary cutaneous EHE is extremely rare and EHE of breast soft tissue has been
(a)
(b)
(c)
(d)
reported only once previously.3 To our knowledge, this is the first cutaneous EHE appearing on the areola (Fig. 1). Tumor cells in EHE have abundant eosinophilic cytoplasm and show an infiltrative growth pattern. Intracytoplasmic vacuole is the most prominent feature of EHE.3 The mass can ulcerate sometimes.4 From its histological features, EHE must be differentiated from epithelioid hemangioma and epithelioid hemangiosarcoma. Clinically, when the lesion is on the breast, common neoplasm on the breast such as intraductal adenocarcinoma should be considered as well. EHE can be ruled out from them by a wide range of vascular antigens that are not seen in breast carcinomas. Complete surgical excision is the treatment of choice. Due to its indistinct clinical features and rarity, EHE is prone to be overlooked. EHE should be considered as a differential diagnosis of cutaneous mesenchymal tumor and attention to its recurrence is vital for both clinician and patient.
CONFLICT OF INTEREST:
None declared.
Song Youn PARK,1 Joshua K. LEE,3 Seongmoon JO,2 Chang-Hun HUH,1 Kwang-Hyun CHO,2 Jung-Im NA1 1
(e)
(f)
Department of Dermatology, Seoul National University Bundang Hospital, Seongnam-si, 2Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea, and 3College of Medicine, Emory University, Atlanta, GA, USA doi: 10.1111/1346-8138.12318
REFERENCES
Figure 1. (a) Ulcerative plaque on the areola at presentation. (b) Recurred lesion. (c) Infiltrative pattern endothelial cell proliferation among mucinous material (hyaline or myxoid stroma). Intracytoplasmic vacuolization is seen (hematoxylin–eosin [HE], original magnification 9200). (d–f) Immunohistochemistry of the patient. Positive for CD31, CD34 and Factor VIII (HE, 9100).
1 Phillip H, McKee EC, Granter SR. Pathology of the Skin, With Clinical Correlations, 3rd edn. Elsevier Mosby, Philadelphia, PA 2005. 2 Deyrup AT, Tighiouart M, Montag AG, Weiss SW. Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases. Am J Surg Pathol 2008; 32: 924–927. 3 Insabato L, Di Vizio D, Terracciano LM, Pettinato G. Epithelioid haemangioendothelioma of the breast. Breast 1999; 8: 295–297. 4 Grezard P, Balme B, Ceruse P, Bailly C, Dujardin T, Perrot H. Ulcerated cutaneous epithelioid hemangioendothelioma. Eur J Dermatol 1999; 9: 487–490.
Myxofibrosarcoma arising in a burn scar Dear Editor, The term “Marjolin ulcer” has been used to describe the malignant changes in burn scars. The most common malignant tumor is a squamous cell carcinoma, followed by basal cell carcinoma and malignant melanoma.1 Myxofibrosarcoma, also known as myxoid malignant fibrous histiocytoma, is a
mesenchymal malignant tumor of the soft tissue commonly occurring late in adult life.2 Myxofibrosarcoma in a burn scar is regarded as a rare condition. A 64-year-old female patient visited our department complaining of a skin lesion on her right shoulder. She had a history of a flame burn 7 years prior without any other past medical
Correspondence: Mi Ryung Roh, M.D., Ph.D., Department of Dermatology, Yonsei University College of Medicine, 211 Eonjuro, Gangnam-gu, Seoul 135-720, Korea. Email: [email protected]
© 2013 Japanese Dermatological Association
113
Letters to the Editor
(a)
(b)
(c)
(d)
Figure 1. (a) Various-sized erythematous nodules with erythematous indurated plaques on the right shoulder. (Inset) Enlarged photograph for lesion. (b) The specimen was composed of a diffuse myxoid hypocellular lesion and partial, highly cellular, pleomorphic lesions (hematoxylin–eosin [HE], original magnification 940). (Inset) The tumor cells, which showed a high degree of nuclear atypia, formed a storiform pattern with scattered bizarre epithelioid cells and giant cells (HE, 9400). (c) Immunohistochemical staining positive for CD68. (d) Immunohistochemical staining negative for cytokeratin.
history. On initial examination of her right shoulder, a 2 cm 9 3 cm mass consisting of various-sized erythematous nodules and indurated plaques was observed (Fig. 1a). Histopathologically, the mass was composed of mainly myxoid hypocellular lesions with partial highly cellular and some curvilinear blood vessels, and pleomorphic lesions. The apparent spindle tumor cells, which showed a high degree of nuclear atypia, formed a storiform pattern with scattered bizarre epithelioid cells and giant cells (Fig. 1b). Immunohistochemical staining revealed approximately 80% positive reactivity to CD68 in tumor cells and negative reactivity for cytokeratin (Fig. 1c,d). The tumor was diagnosed as a myxofibrosarcoma arising from a burn scar. Myxofibrosarcoma has been described as a myxoid variant of the malignant fibrous histiocytoma (MFH). Histopathologically, there is hyaluronic acid-rich myxoid stroma and proliferation of spindle or stellate cells in a haphazard arrangement. In immunohistochemical stains, tumor cells react positively with CD68, a1-antitrypsin and vimentin.3 Marjolin ulcer has been used to describe all kinds of malignant tumors arising from burn scars. Approximately 2% of
114
chronic burn scars undergo malignant changes.1 Ewing’s postulates require: (i) evidence of a burn scar; (ii) tumor within the boundaries of the scar; (iii) no previous tumor in the specific location; (iv) tumor histology must be compatible with the cell types found in the skin and the scar; and (v) there is an adequate interval between the burn injury and the tumor development.1 Cutaneous MFH arising from a burn scar is a rare event, with only 10 cases reported in the published work. In previously reported cases, the latent period between injury and tumor detection was 20–71 years. Most cases showed a storiform, pleomorphic pattern histopathologically.3,4 Our case was reported as a myxoid malignant fibrous histiocytoma, a subtype of MFH in a burn scar, which has never been reported before. Squamous cell carcinoma, the most common malignant tumor arising from burn scars, originates from epithelial cells and undergoes dermal invasion by atypical keratinocytes. However, myxofibrosarcoma has different findings consisting of hyaluronic acid-rich myxomatous stroma and proliferation of spindle or stellate cells in the dermis and subcutaneous tissues.4 The latent period
© 2013 Japanese Dermatological Association
Letters to the Editor
for tumor development from a burn scar was 7 years in our case, which is an earlier period than that reported in other cases. Herein, we present a case of myxofibrosarcoma that arose from a burn scar and review the published work of previous reports of cutaneous MFH in burn scars.
CONFLICTS OF INTEREST::
None declared.
Hannah HONG,1 Dong In KEUM,1 Ji Hye LEE,2 Mi Ryung ROH2 1
REFERENCES 1 Kowal-Vern A, Criswell BK. Burn scar neoplasms: a literature review and statistical analysis. Burns 2005; 31: 403–413. 2 Clarke LE, Zhang PJ, Crawford GH, Elenitsas R. Myxofibrosarcoma in the skin. J Cutan Pathol 2008; 35: 935–940. 3 Park CH, Kim YM, Lee SY, Park YL, Lee JS, Whang KU. A case of malignant fibrous histiocytoma developing in a burn scar. Korean J Dermatol 2008; 46: 1449–1452. 4 Ozercan IH, Okur MI, Coskun F, Yildirim AM. Malignant fibrous histiocytoma and squamous carcinoma derived from a burn scar. Acta Chir Belg 2004; 104: 745–747.
Department of Dermatology, Yonsei University Wonju College of Medicine, Wonju, and 2Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea doi: 10.1111/1346-8138.12338
© 2013 Japanese Dermatological Association
115
(3/50 high-power fields) are associated with increased rate of metastasis (25% of cases) and a poor 5-year survival (59%).2 EHE mainly occur at muscles of extremities, soft tissues and visceral organs such as the liver and lung. Primary cutaneous EHE is extremely rare and EHE of breast soft tissue has been
(a)
(b)
(c)
(d)
reported only once previously.3 To our knowledge, this is the first cutaneous EHE appearing on the areola (Fig. 1). Tumor cells in EHE have abundant eosinophilic cytoplasm and show an infiltrative growth pattern. Intracytoplasmic vacuole is the most prominent feature of EHE.3 The mass can ulcerate sometimes.4 From its histological features, EHE must be differentiated from epithelioid hemangioma and epithelioid hemangiosarcoma. Clinically, when the lesion is on the breast, common neoplasm on the breast such as intraductal adenocarcinoma should be considered as well. EHE can be ruled out from them by a wide range of vascular antigens that are not seen in breast carcinomas. Complete surgical excision is the treatment of choice. Due to its indistinct clinical features and rarity, EHE is prone to be overlooked. EHE should be considered as a differential diagnosis of cutaneous mesenchymal tumor and attention to its recurrence is vital for both clinician and patient.
CONFLICT OF INTEREST:
None declared.
Song Youn PARK,1 Joshua K. LEE,3 Seongmoon JO,2 Chang-Hun HUH,1 Kwang-Hyun CHO,2 Jung-Im NA1 1
(e)
(f)
Department of Dermatology, Seoul National University Bundang Hospital, Seongnam-si, 2Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea, and 3College of Medicine, Emory University, Atlanta, GA, USA doi: 10.1111/1346-8138.12318
REFERENCES
Figure 1. (a) Ulcerative plaque on the areola at presentation. (b) Recurred lesion. (c) Infiltrative pattern endothelial cell proliferation among mucinous material (hyaline or myxoid stroma). Intracytoplasmic vacuolization is seen (hematoxylin–eosin [HE], original magnification 9200). (d–f) Immunohistochemistry of the patient. Positive for CD31, CD34 and Factor VIII (HE, 9100).
1 Phillip H, McKee EC, Granter SR. Pathology of the Skin, With Clinical Correlations, 3rd edn. Elsevier Mosby, Philadelphia, PA 2005. 2 Deyrup AT, Tighiouart M, Montag AG, Weiss SW. Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases. Am J Surg Pathol 2008; 32: 924–927. 3 Insabato L, Di Vizio D, Terracciano LM, Pettinato G. Epithelioid haemangioendothelioma of the breast. Breast 1999; 8: 295–297. 4 Grezard P, Balme B, Ceruse P, Bailly C, Dujardin T, Perrot H. Ulcerated cutaneous epithelioid hemangioendothelioma. Eur J Dermatol 1999; 9: 487–490.
Myxofibrosarcoma arising in a burn scar Dear Editor, The term “Marjolin ulcer” has been used to describe the malignant changes in burn scars. The most common malignant tumor is a squamous cell carcinoma, followed by basal cell carcinoma and malignant melanoma.1 Myxofibrosarcoma, also known as myxoid malignant fibrous histiocytoma, is a
mesenchymal malignant tumor of the soft tissue commonly occurring late in adult life.2 Myxofibrosarcoma in a burn scar is regarded as a rare condition. A 64-year-old female patient visited our department complaining of a skin lesion on her right shoulder. She had a history of a flame burn 7 years prior without any other past medical
Correspondence: Mi Ryung Roh, M.D., Ph.D., Department of Dermatology, Yonsei University College of Medicine, 211 Eonjuro, Gangnam-gu, Seoul 135-720, Korea. Email: [email protected]
© 2013 Japanese Dermatological Association
113
Letters to the Editor
(a)
(b)
(c)
(d)
Figure 1. (a) Various-sized erythematous nodules with erythematous indurated plaques on the right shoulder. (Inset) Enlarged photograph for lesion. (b) The specimen was composed of a diffuse myxoid hypocellular lesion and partial, highly cellular, pleomorphic lesions (hematoxylin–eosin [HE], original magnification 940). (Inset) The tumor cells, which showed a high degree of nuclear atypia, formed a storiform pattern with scattered bizarre epithelioid cells and giant cells (HE, 9400). (c) Immunohistochemical staining positive for CD68. (d) Immunohistochemical staining negative for cytokeratin.
history. On initial examination of her right shoulder, a 2 cm 9 3 cm mass consisting of various-sized erythematous nodules and indurated plaques was observed (Fig. 1a). Histopathologically, the mass was composed of mainly myxoid hypocellular lesions with partial highly cellular and some curvilinear blood vessels, and pleomorphic lesions. The apparent spindle tumor cells, which showed a high degree of nuclear atypia, formed a storiform pattern with scattered bizarre epithelioid cells and giant cells (Fig. 1b). Immunohistochemical staining revealed approximately 80% positive reactivity to CD68 in tumor cells and negative reactivity for cytokeratin (Fig. 1c,d). The tumor was diagnosed as a myxofibrosarcoma arising from a burn scar. Myxofibrosarcoma has been described as a myxoid variant of the malignant fibrous histiocytoma (MFH). Histopathologically, there is hyaluronic acid-rich myxoid stroma and proliferation of spindle or stellate cells in a haphazard arrangement. In immunohistochemical stains, tumor cells react positively with CD68, a1-antitrypsin and vimentin.3 Marjolin ulcer has been used to describe all kinds of malignant tumors arising from burn scars. Approximately 2% of
114
chronic burn scars undergo malignant changes.1 Ewing’s postulates require: (i) evidence of a burn scar; (ii) tumor within the boundaries of the scar; (iii) no previous tumor in the specific location; (iv) tumor histology must be compatible with the cell types found in the skin and the scar; and (v) there is an adequate interval between the burn injury and the tumor development.1 Cutaneous MFH arising from a burn scar is a rare event, with only 10 cases reported in the published work. In previously reported cases, the latent period between injury and tumor detection was 20–71 years. Most cases showed a storiform, pleomorphic pattern histopathologically.3,4 Our case was reported as a myxoid malignant fibrous histiocytoma, a subtype of MFH in a burn scar, which has never been reported before. Squamous cell carcinoma, the most common malignant tumor arising from burn scars, originates from epithelial cells and undergoes dermal invasion by atypical keratinocytes. However, myxofibrosarcoma has different findings consisting of hyaluronic acid-rich myxomatous stroma and proliferation of spindle or stellate cells in the dermis and subcutaneous tissues.4 The latent period
© 2013 Japanese Dermatological Association
Letters to the Editor
for tumor development from a burn scar was 7 years in our case, which is an earlier period than that reported in other cases. Herein, we present a case of myxofibrosarcoma that arose from a burn scar and review the published work of previous reports of cutaneous MFH in burn scars.
CONFLICTS OF INTEREST::
None declared.
Hannah HONG,1 Dong In KEUM,1 Ji Hye LEE,2 Mi Ryung ROH2 1
REFERENCES 1 Kowal-Vern A, Criswell BK. Burn scar neoplasms: a literature review and statistical analysis. Burns 2005; 31: 403–413. 2 Clarke LE, Zhang PJ, Crawford GH, Elenitsas R. Myxofibrosarcoma in the skin. J Cutan Pathol 2008; 35: 935–940. 3 Park CH, Kim YM, Lee SY, Park YL, Lee JS, Whang KU. A case of malignant fibrous histiocytoma developing in a burn scar. Korean J Dermatol 2008; 46: 1449–1452. 4 Ozercan IH, Okur MI, Coskun F, Yildirim AM. Malignant fibrous histiocytoma and squamous carcinoma derived from a burn scar. Acta Chir Belg 2004; 104: 745–747.
Department of Dermatology, Yonsei University Wonju College of Medicine, Wonju, and 2Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea doi: 10.1111/1346-8138.12338
© 2013 Japanese Dermatological Association
115
