Letters to the Editor

syringomatous carcinoma in an erythematous plaque and solid carcinoma-like features in a nodule.3 The fact that nodules with solid carcinoma-like histopathological features had appeared from an erythematous plaque leads us to hypothesize that the tumor cells differentiated into two directions of either tubular structures in an erythematous plaque or solid carcinoma-like features in a nodule. In accordance with this hypothesis, immunohistochemical staining shared a positive reaction with EMA in tumor cells of both syringomatous carcinoma and solid carcinoma-like features. The size of this tumor (24 cm 9 15 cm) is quite large compared with previous cases, and the location is relatively rare since most cases of syringomatous carcinoma occur on the face, axillae and scalp.5 In conclusion, we report a case that supports the concept of syringomatous carcinomas having several different histological expression patterns.

CONFLICT OF INTEREST:

The authors declare no financial

or other conflict of interest.

Departments of 1Dermatology, 2Pathology, Yokohama City Minato Red Cross Hospital, Yokohama, and 3Department of Diagnostic Pathology, Tokyo Medical University, Tokyo, Japan doi: 10.1111/1346-8138.12683

REFERENCES 1 Ohnishi T, Kaneko S, Egi M, Takizawa H, Watanabe S. Syringoid eccrine carcinoma: report of a case with immunohistochemical analysis of cytokeratin expression. Am J Dermatopathol 2002; 24: 409–413. 2 Washio K, Bito T, Ono R, Horikawa T, Nishigori C. Syringomatous carcinoma on the leg. J Dermatol 2012; 39: 1041–1043. 3 Requena L, Kiry H, Ackerman AB. Solid carcinoma. In: Neoplasms with apocrine differentiation, Philadelphia: Lippincott Raven, 1998: 833–855. 4 Petersson F, Skogvall I, Elmberger G. Sclerosing sweat duct-like carcinoma of the tongue - a case report and a review of the literature. Am J Dermatopathol 2009; 31: 691–694. 5 Abenoza P, Ackerman AB.Syringomatous carcinoma. In: Neoplasms with eccrine differentiation, Philadelphia: Lea&Febiger, 1990; 373– 412.

Sonoko NAKAZONO,1 Naoko TAKAYAMA,1 Kohei NOJIMA,1 Jiro KUMAGAI,2 Miki IZUMI,3 Takeshi NAMIKI1

Rare case of Langerhans cell sarcoma with cutaneous manifestation arising on the inguinal region Dear Editor, Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells (LC) with aggressive clinical behavior. We report an 87-year-old Japanese woman with LCS with inguinal involvement. After she noticed a tumor in the right inguinal region 3 years earlier she was evaluated in another hospital. Histopathological study returned a diagnosis of LCS of lymph node while she was treated palliatively because of her advanced age. The tumor enlarged, gradually affecting the overlying skin with profuse exudation, and she was referred to our hospital. Physical examination revealed a fist-sized, irregularly shaped tumor, with an underlying ulcer in the right inguinal region (Fig. 1a). Computed tomography showed a 94 mm 9 51 mm tumor close to the right inguinal vein with invasion of the sartorius and pectineal muscles (Fig. 1b). No distant metastasis was recognized. On histological examination, the tumor consisted of large polygonal or round mono- or multinuclear cells with extensive necrosis. Remarkable nuclear pleomorphism and prominent mitoses were seen (Fig. 1c). The tumor cells were positive for CD1a and S-100 protein (Fig. 1d, e). The Ki-67 index was 40% (Fig. 1f) which is relatively high compared with that reported in LC histiocytosis.1 Electron microscopy demonstrated Birbeck granules (Fig. 1g) and the

earlier diagnosis of LCS was confirmed. We removed the tumor and dissected the right inguinal lymph nodes because her lesions were localized to the inguinal area and persistent exudation was intractable. There was no evidence of local recurrence or distant metastasis 8 months later. Only 37 cases of LCS, first described in 1984,2 have been reported.3 LCS affects various tissues such as bone, lung, liver, spleen, lymph nodes and other soft tissues. Cutaneous involvement is extremely rare. Only 13 cases of LCS affecting the skin, including three cases with skin lesions alone, have been reported previously. LCS is highly aggressive with a poor prognosis and a high mortality rate.1 Of 37 reported patients, 18 (48.6%) died of LCS and 12 (32.4%) obtained complete remission (CR) by chemotherapy, radiotherapy, surgery or a combination of these treatments. While none of the 18 dead cases manifested skin lesions, all three cases with skin lesion alone obtained CR, suggesting that patients with skin involvement may have a comparatively better prognosis. As the number of cases with LCS involving the skin and regional lymph nodes is small, no standard treatment has been established. The documentation of more such cases is needed for new insights into the treatment and prognosis of LCS affecting the skin and lymph nodes.

Correspondence: Nozomi Jimura, M.D., Department of Dermatology, Field of Sensory Organology, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima 890-8520, Japan. Email: [email protected]

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Letters to the Editor

(a)

(b)

(d)

(e)

(f)

(c) (g)

Figure 1. (a) A fist-sized, irregularly shaped tumor with an underlying ulcer in the right inguinal region. (b) Computed tomography showing a 94 mm 9 51 mm tumor located close to the right inguinal vein invading into the sartorius and pectineal muscles. (c) The tumor consists of large polygonal and round mono- or multinuclear cells. Remarkable nuclear pleomorphism and prominent mitoses were seen. Note extensive necrosis (hematoxylin–eosin, original magnification 9400). Immunohistochemically, the tumor cells were positive for (d) CD1a and (e) S-100 protein (9400). (f) The Ki-67 index was 40% (9400). (g) Electron microscopy demonstrated the presence of typical (arrows) and anomalous (arrowhead) Birbeck granules.

CONFLICT OF INTEREST:

The authors declare no conflict

REFERENCES

of interest.

Nozomi JIMURA, Shigeto MATSUSHITA, Naoko BABA, Hidemichi KUBO, Koichiro TAKEDA, Tomoko FUKUSHIGE, Kazuyasu FUJII, Takuro KANEKURA Department of Dermatology, Field of Sensory Organology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan

1 Jaffe E, Harris N, Stein H, et al. Pathology and Genetics of Tumors of Haematopoietic and Lymphoid Tissues. Lyon: WHO Classification IARC Press, 2001; 273–283. 2 Wood C, Wood GS, Deneau DG, Oseroff A, Beckstead JH, Malin J. Malignant histiocytosis X: report of a rapidly fatal case in an elderly man. Cancer 1984; 54: 347–352. 3 Wang Y, Zhou X, Wang Z. Langerhans cell sarcoma in the cervical lymph node: a case report and literature review. Acta Haematol 2013; 129: 114–120.

doi: 10.1111/1346-8138.12692

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Rare case of Langerhans cell sarcoma with cutaneous manifestation arising on the inguinal region.

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