Standardized therapeutic approaches to mycosis fungoides/Sézary syndrome have been demonstrated by a newly launched guideline for cutaneous lymphomas in Japan. However, we cannot treat all the patients, even at the same stage of the disease, with uniform regimens because of the differences in disease activities and progression. Since clinical responses to treatments may vary in each case, the choice and dosage of alternative regimens have been determined by doctors' own experiences. Nowadays, therapeutic options are limited because as compared with Western countries, only a few regimens have been approved officially in Japan. Therefore, we are obliged to select a feasible option among the limited regimens. In the present lecture, we show practical choices of treatments for each stage of mycosis fungoides and Sézary syndrome, and refer to a possible usage of molecular targeting agents.[Skin Cancer (Japan) 2010 ; 25 : 250-262]

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Cancer patients daily encounter various problems in their family lives, jobs, and finances, in addition to the cancer itself. In considering the problems of cancer patients, psychosocial and living distress should be focused on in addition to the disease.
Cancer influences not only patients but also people around them, and its burden affects the disease course as well as social and economic aspects, influencing various daily living activities.
Bereavement is one of the most striking events in human experience, and is associated with excess risk of mortality and morbidity.
For intervention of family members, multifaceted approaches including the physical, psychological, and social aspects, are necessary.[Skin Cancer (Japan) 2010 ; 25 : 263-273]

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I explained my process of histopathological diagnosis of soft tissue tumors of the skin. First, methods and issues in differentiation between epithelial and non-epithelial neoplasms were discussed ; then, those between benign and malignant tumors were also explained. Next, I mentioned the process of histopathological diagnosis of soft tissue tumors of the skin. For this purpose, I make diagnoses of differentiation of neoplastic cells from histo-patholigical and immunohisto-chemical similarities with normal tissue, at the beginning. Then, I search the characteristic structures, or morphological characteristics of neoplastic cells. Next, I discussed important findings in diagnosing dermatofibrosarcoma protuberans (DFSP) and those in differentiating DFSP from dermatofibroma or neurofibroma. I also presented the important points in differentiating undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma) from other malignant soft tissue neoplasms with pleomorphic cell features.[Skin Cancer (Japan) 2010 ; 25 : 274-281]

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Angiosarcoma (AS) is an aggressive malignancy with poor prognosis, and a 5-year survival rate of approximately 10%.
However, the guideline for the optimal treatment has not yet been well established.
The group study for AS of the Japanese Association of Dermatologic Surgery suggested that the 1-to 2-cm surgical margin group receives a significant overall survival benefit.
Moreover, the pathological margin-free group also shows a significantly improved overall survival rate.
In this study, the chemotherapy group was also observed to have experienced a significantly improved overall survival rate.
Therefore the optimal treatment is the resection of the tumor, preferably with negative margin. In addition, the initial chemotherapy may inhibit metastases.
The realization of complete resection thus plays an important role in the improvement of the AS prognosis.
We hope to prepare promptly a TNM staging manual, and to establish the appropriate adjuvant therapy for AS patients.[Skin Cancer (Japan) 2010 ; 25 : 282-287]

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We commonly treat cutaneous malignancies with metastases including the lymph nodes and distant metastases, and also cutaneous metastases from visceral malignancies. For patients having cutaneous malignancy with distant metastases, systemic therapy, such as anti-cancer chemotherapy, is administered, in principle. However, anti-cancer chemotherapy alone may not be fully effective in this population, and especially in patients with malignant melanoma ; sometimes an excellent clinical outcome can be potentially achieved by combined chemotherapy and topical treatment, including resection of the metastases, in appropriately selected patients. In addition, emergency surgery may be needed for bleeding associated with cerebral metastases or intestinal obstruction due to intestinal metastases. Meanwhile, for patients with cutaneous metastases from visceral malignancies, there has been little debate on the efficacy of topical treatment, including resection. We therefore examined the surgical indications for metastases, mainly in patients with malignant melanoma or cutaneous metastases from visceral malignancies.[Skin Cancer (Japan) 2010 ; 25 : 288-292]

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It is difficult for the surgeon to decide whether or not to use surgical or non-surgical treatment for actinic keratosis (AK) and basal cell carcinoma (BCC) on the nose. The principle of the treatment is based on surgical treatment, but sometimes we have to consider non-surgical treatments for various reasons. For AK, cryosurgery, topical 5-FU, CO2 laser, electrodesiccation, chemical peeling, photodynamic therapy (PDT) are used. For BCC, radiotherapy is used. The appropriate treatment to use is based on a case by case decision.[Skin Cancer (Japan) 2010 ; 25 : 293-299]

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Bowenoid papulosis (BP) is a disease characterized by multiple brown-to-black papules on the genitalia, and by histological changes similar to Bowen's disease. Human papillomavirus (HPV) has been reported to induce BP. On the other hand, verrucous carcinoma (VC) is a distinct variant of squamous cell carcinoma (SCC), which is also assumed to be associated with HPV infection. According to the anatomical localization of the tumor, four types can be distinguished : (1) VC of the oral cavity, known as Ackerman carcinoma or oral florid papillomatosis ; (2) VC of the anogenital area, also known as giant condyloma of Buschke-Loewenstein ; (3) VC occurring predominantly on the sole, also known as epithelioma cuniculatum ; and (4) VC occurring in other sites. In this article, the etiological role of HPV is discussed in the development of BP and each type of VC.[Skin Cancer (Japan) 2010 ; 25 : 300-307]

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Clinical and pathological characteristics of invasive Paget's disease with lymph nodes or distant metastases are reviewed from the author's past experience, as follows. Three autopsied cases are briefly presented. The presence of hematogenous spread and tumor thrombi are shown with pathology images. Several different patterns of lymphatic flow cause bilateral inguinal mets or hemilateral mets depending on the location of the primary tumor. Chemotherapy and radiation therapy, though palliative, prolong the survival duration. Some groups of patients rapidly develop invasive nodules and mets unexpectedly. Those cases that are diagnosed as in situ disease pathologically may develop mets later.[Skin Cancer (Japan) 2010 ; 25 : 308-316]

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There is no standard regimen of chemotherapy for advanced extramammary Paget's disease. Until now, a combination chemotherapy of 5-fluorouracil (5-FU) and cisplatin has been mainly used for this disease. Recently, however, some authors report that docetaxel is also effective. Therefore, we weighed the efficacy of docetaxel chemotherapy and the combination chemotherapy including 5-FU or TS-1, a prodrug of 5-FU. As a result, the response and disease control rates were 30.8% and 77.0% in docetaxel and 20.0% and 40.0% in 5-FU or TS-1. From this result, docetaxel may be more effective for advanced extramammary Paget's disease than 5-FU or TS-1. Larger clinical trials of chemotherapy are needed in the future.[Skin Cancer (Japan) 2010 ; 25 : 317-323]

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A 25-year-old woman presented with a small black speckle on her back which she had since birth. She noted a dark red tumor on the black speckle with surrounding scattered black nodules in March 2008. She had an excisional biopsy of the dark red tumor in August 2008. The tumor size was 7×5×5 mm and the shape was polypoid. Histologically, atypical melanocytes were forming various sizes of nests infiltrating into the dermis. At the adjacent area of the malignant lesion, nevus cells were observed closely. Whereas the lesion being suspected of malignant melanoma revealed 15% positive for MIB-1, the lesion being suspected of pigmented nevus revealed 5% positive for MIB-1. We considered this case as malignant melanoma in association with pigmented nevus. Following, extended resection and sentinel lymph node biopsy were performed in August 2008. The resected sentinel lymph node showed a micrometastasis.[Skin Cancer (Japan) 2010 ; 25 : 324-327]

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A 66-year-old man visited our hospital complaining of an asymptomatic dome-like tumor in the left back. Skin biopsy from the lesion revealed a neuroendocrine tumor which was positive for cytokeratin stain. The FDG-PET and CT detected uptake in the left back and left axillary yet not in the lung ; therefore, Merkel cell carcinoma (MCC) and metastasis to the left axillary lymphnode were diagnosed. Wide local excision and radical axillary dissection were performed, followed by radiation therapy with a total dose of 50 Gy in 25 fractions. Usually MCC occurs in those parts of the human body which are often exposed to sunlight. MCC of the trunk is relatively rare, as the trunk occupies only 10% of the primary lesion of MCC. In addition, in our case, a thyroid tumor existed concurrently. Although patients with MCC have been reported to contract subsequent primary tumors, none was associated with a thyroid tumor.[Skin Cancer (Japan) 2010 ; 25 : 328-330]

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The aesthetic expectations of nasal repair include symmetry, natural contour, excellent color and texture match, and a final product that remains inconspicuous to the casual observer. Reconstruction based on the aesthetic subunit principle has yielded good aesthetic outcomes in patients.
A retrospective investigation was performed of the patients who underwent resection of malignant skin tumors of the nose at our institutes during the past 8 years.
Based on nasal aesthetic sub-unit, 32 patients were divided into 4 groups according to their nasal defects. They were nasal dorsum, tip, alar lobule, and two or two above nasal subunit defects groups.
We tended to use a front nasal flap to reconstruct the nasal tip, a skin graft to reconstruct the dorsum, and a nasolabial flap to reconstruct an ala and a sidewall. A combination of flaps was used when the defect involved more than one unit.
In general, the surgeon should approach each patient as a distinct individual with a unique defect and thus tailor each reconstruction to fit the patient's needs and expectations.
Candidates for reconstruction should be selected on the basis of nasal structure and the local flap or skin graft feature.[Skin Cancer (Japan) 2010 ; 25 : 331-338]

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We report a case of Myxofibrosarcoma. A 51-year-old female patient visited our Department complaining of a soft tumor behind her left ear. An exisional specimen revealed spindle cells with storiform pattern, pleomorphism and atypia which had penetrated the dermis and the subcutaneous layer. Immunohistochemically, these tumor cells were positive for Vimentin and CD34. The tumor was diagnosed as a low-grade Myxofibrosarcoma. No recurrence or distant metastases were noted after 6 months follow-up.[Skin Cancer (Japan) 2010 ; 25 : 339-342]

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We report a case of a 60-year-old woman with a cutaneous primitive neuroectodermal tumor (PNET) on her left knee. The patient noticed a subcutaneous nodule on her left knee since April 2009, and that it had been gradually increasing in size for 4 months. Hematoxylin and eosin staining of the specimen showed that the tumor was composed of uniform small round cells with atypical nuclei. The tumor cells showed positive reactions with CD99, vimentin, CD56, and synaptophysin. Chromosomal fluorescence in situ hybridization (FISH) from the tumor cells showed translocation of chromosome No. 22 q12. Therefore, the tumor was diagnosed as PNET. In Japan, only 9 cases including our case have been reported.[Skin Cancer (Japan) 2010 ; 25 : 343-347]

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We evaluated the efficacy and toxicity of combination chemotherapy with CDDP, DTIC, IFN-β on 10 patients with metastatic melanoma. The regimen was CDDP 100mg/m² on day 1, DTIC 150mg/m² on days 1-3 and IFN-β 600×10⁴IU on days 4-8. The response rate was 30%. The severe toxicities were myelosuppression, nausea/vomiting.[Skin Cancer (Japan) 2010 ; 25 : 348-352]

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A 66-year-old man presented with a subcutaneous mass in the back in 2007. The mass was surgically resected and diagnosed as a metastatic tumor ; however, the primary lesion remained unknown. One year later, the tumor metastasized in the axillary and clavicular lymph nodes in addition to the back and chest subcutaneous tissue. After chemotherapy, wide resection and lymph node dissection were performed, followed by adjuvant chemotherapy together with radiotherapy in the orthopedics department of our hospital. The metastatic tumors were histopathologically S-100 protein positive, but negative for HMB-45 and MART-1, and were then diagnosed as a malignant peripheral nerve sheath tumor. In 2009, he had metastases in the adrenal glands, which were resected. Subsequently, he presented with multiple metastases in the skin and bone, and was sent to us for consultation. Since the serum level of 5-S-CD was elevated, tumors were reexamined immunohistochemically. Based on positivity for both MITF and high molecular weight-melanoma-associated antigen, we diagnosed this case as malignant melanoma.[Skin Cancer (Japan) 2010 ; 25 : 353-357]

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We report a case of squamous cell carcinoma arising in an epidermal cyst. The patient was a 78-year-old man who had a ten-year history of a mass on his left buttock. The lesion had gradually enlarged during the previous years. An excisional biopsy was performed. Histpathologically, there was a cystic lesion composed of benign squamous epithelia with a transition to squamous cell carcinoma. The patient is alive without local recurrence or distant metastasis at six months postoperatively. Malignant transformation of an epidermal cyst is uncommon.[Skin Cancer (Japan) 2010 ; 25 : 358-362]

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We report a case of mucinous carcinoma of the skin on the face. A 76-year-old man presented with a growing tumor in the left bucca which had begun four years earlier. On examination, a 22×24mm multilobular elastic hard tumor was present on the left cheek. This lesion showed capillary dilation on the surface, and a blister-like appearance in part. Under local anesthesia, the tumor was excised. Histological examination showed that the tumor was located in the dermis and subcutaneous fat with abundant mucin, which was separated by a fibrous septum. The mass of atypical tumor cells floated in the background of Alcian blue-positive mucin. Tumor cells displayed nuclear atypia and PAS staining positive cytoplasm. Immunohistochemically, tumor cells showed positive for EMA and CK7, slightly positive for CEA but negative for CK20. On gastro-intenstinal fiberscopy and CT scans of the body, we found no internal neoplasm. There is no evidence of recurrence or metastasis one year after the surgery.[Skin Cancer (Japan) 2010 ; 25 : 363-366]

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A 76-year-old Japanese woman presented with an erythematous lesion on the vulva which had been noticed about 2 years earlier and grown rapidly during the last 6 months. Physical examination revealed a nodule accompanied by erythematous plaque from the left large pudendal lip to the anus, and some swollen bilateral inguinal lymph nodes. Biopsy confirmed the diagnosis of subcutaneous invasion of extramammary Paget's disease. Computed tomography showed metastasis of bilateral inguinal and para-aortic lymph nodes. She was treated with local excision of the primary lesion, followed by weekly docetaxel and trastuzumab, as immunohistochemical examination revealed strongly HER2 positive. After 7 courses, lymph node metastasis disappeared with few adverse effects. Eight weeks after chemotherapy, the complete response had continued. The weekly docetaxel-trastuzumab combination chemotherapy can be a very effective and well-tolerated therapy for patients with HER-2-positive advanced extramammary Paget's disease.[Skin Cancer (Japan) 2010 ; 25 : 367-371]

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An 81-year-old woman was scalded with tempura oil on the left forearm about 1 year ago and formed an ulcer. We continued local drug treatment with a nearby doctor, but the blistering and ulceration gradually spread. Because she had been admitted to our hospital's orthopedics department for a fracture, she had consulted our department. Physical examination revealed a slightly circular, crimson granulated aspect with clear boundaries, with a diameter of 2cm on the left forearm. We took it to be surplus granulation resulting from the scald treatment in our department and treated it, but it didn't epithelize. So we performed a skin biopsy and diagnosed the neoplasm as Bowen's disease. We removed it surgically with a border of 1cm, and performed pedicle flap plastic surgery. There was no evidence of recurrence for half a year.[Skin Cancer (Japan) 2010 ; 25 : 372-375]

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