A statistical survey of malignant skin tumors has been systematically conducted in cooperation with the Prognosis and Statistical Investigation Committee of the Japan Skin Cancer Society, the Malignant Melanoma Group funded by a Grant-in-Aid for Cancer Research from the Ministry of Health, Labour and Welfare, and the Institute for Analysis of Prognostic Factors in Skin Cancer. The aims of this nationwide questionnaire survey are to examine changes in the number of patients with malignant skin tumors over time in Japan and to investigate the epidemiology, prognostic factors, and disease-related survival rate over time by collecting detailed case report forms from selected hospitals. Patients with target tumors were reported from all over the country, and major symptoms were also included in the analysis. The survey is not only expected to produce longitudinal knowledge about the status of malignant skin tumors in Japan, but also to allow identification of prognostic factors and improvements in treatment. Thus, the survey may be useful for future improvement of the diagnosis and treatment of this disease. The present manuscript includes a thorough discussion of these issues with the focus on malignant melanoma. [Skin Cancer (Japan) 2005; 20: 234-248]

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A 78-year-old man had been treated under the diagnosis of chronic eczema by a local dermatologist. Skin eruptions became erythrodermic four months prior to the referral to our clinic. Seborrheic keratoses were reported to have appeared on his head, neck, chest, and back and increased rapidly in number. Laboratory examination revealed multiple colon cancers and lymphomatoid papuloses on his left thigh. The association of these three (erythroderma, multiple colon cancer, lymphomatoid papulosis) is found to be rare. [Skin Cancer (Japan) 2005; 20: 249-253]

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Mycosis fungoides (MF) classically described shows an indolent clinical course, however, numerous clinical and histological variants of the classical presentation have been reported. We report a 59-years-old female who developed erythroderma with ulcers during treatment for psoriasis vulgaris. The appearance of self-healing cutaneous small nodules was also followed during the clinical course. Histological and immunohistochemical examination from patch and nodule lesions revealed MF and lymphomatoid papulosis, respectively. Although partial remission was achieved with PUVA and IFN-γ treatment, the recurrence of erythematous plaques with ulcers rapidly progressed on the whole body. She died within 7 months of the follow-up period after the presentation of erythroderma. This case may be one of aggressive variants of MF, or categorized as unclassified cutaneous T-cell lymphomas presenting with ulcerative erythroderma. [Skin Cancer (Japan) 2005; 20: 254-258]

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A 75-year-old woman developed a subcutaneous nodule on the abdomen as well as an ulcer on the upper lip. She had a history of gastric B-cell lymphoma 10 years earlier and had received a combination of gastrectomy and chemotherapy. Remission had been maintained thereafter. Histological examination of the abdominal nodule demonstrated atypical B-cell proliferation in the dermis, indicating diffuse large B-cell lymphoma. Some of the tumor cells showed apoptosis by the Tunnel method. On the other hand, histology of the lip specimen demonstrated squamous cell carcinoma. Immunoglobulin heavy chain gene-rearrangement analyses by nested polymerase chain reaction did not detect B-cell monoclonalities in the paraffin-embedded tissues of the cutaneous and gastric lymphomas, respectively. However, we speculated that the cutaneous B-cell lymphoma might have been metastasis from gastric B-cell lymphoma, becauce cutaneous lymphoma developed in and near the operation scar, and it has been reported that primary cutaneous B-cell lymphoma is not frequent. [Skin Cancer (Japan) 2005; 20: 259-263]

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Sentinel lymph node biopsy is increasingly used for treating melanoma in Japan. When sentinel lymph nodes are positive, complete lymph node dissection is generally recommended. In the case of groin nodal metastases, it remains controversial whether the radical groin dissection (RGD) or superficial groin dissection (SGD) should be performed. Sixteen patients of cutaneous melanoma with regional lymph node micrometastases who underwent elective lymph node dissection or lymph node dissection after positive sentinel lymph nodes were examined. In five cases of elective radical groin dissection, iliac and obturator lymph node metastases were not observed. No significant differences of melanoma-specific survival rate were also observed between RGD and SGD. Therefore, SGD appears to be a good indication in the case of groin sentinel node micrometastases. [Skin Cancer (Japan) 2005; 20: 264-267]

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A 66-year-old man had noticed a subcutaneous mass in the right chest about 5 to 6 years previously, but left it untreated. However, since the mass had begun to grow, about one and a half years ago, he consulted a local doctor, and was referred to our department in August 2003 for further evaluation. At initial examination revealed a large, well-demarcated, freely movable, subcutaneous mass, 17×10×7cm, which was palpable from the right anterior shoulder region to the anterior chest. On the outside of this mass, a cauliflower-like tumor with a 15×13×7cm ulcer was protruding. A wide excision extending up to 2cm from the tumor borders was performed, followed by shoulder skin flap repair and free skin grafting. Histological examination of the totally resected specimen revealed a dermatofibrosarcoma protuberans (DFSP) lesion in which relatively uniform, spindle-shaped cells grew in a cart-wheel pattern, and a fibrosarcomatous lesion showing a herringbone pattern. Histochemicalstaining showed that the DFSP lesion was strongly positive for CD34, but the fibrosarcomatous lesion was weakly stained. Cytogenetic examination identified the COL1A1-PDGFB fusion gene. These findings led to a diagnosis of dermatofibrosarcoma protuberans with fibrosarcomatous change. [Skin Cancer (Japan) 2005; 20: 268-272]

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A 60-year-old man noticed a grayish brown pigmented macule on the right calcaneal region about 1 year before presentation to our clinic in March, 2003. A tumor developed from the macule and became ulcerated. We diagnosed it as malignant melanoma and the tumor was widely excised in May, 2003. Additional right inguinal region lymphadenectomy revealed no apparent metastasis. Thereafter, many intracranial metastatic lesions were found by brain MRI. Whole brain radiation therapy reduced the brain metastatic lesions remarkably. We further performed a gamma knife treatment for the remaining lesions. Brain MRI revealed that no recognized metastatic lesions were identified following three additional courses of DAC-Tam treatment.
It has been assumed that the multiple metastases of malignant melanoma in the brain were refractory. We propose that radiation therapy may be a recommendable therapy, because whole brain radiation therapy combined with gamma knife treatment was effective in the present case. [Skin Cancer (Japan) 2005; 20: 273-277]

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A 64-years-old man with anal canal adenocarcinoma with Pagetoid spread to the perianal region is reported. He had noticed a perianal skin lesion about 8 months previously. The lesion gradually expanded; therefore, he visited our hospital. Physical examination revealed irregular erythematous plaque around his anus. Biopsy revealed intradermal large atypical foam cells suggestive of Paget's disease. Another tumor was found in the anal canal. Biopsy revealed well-differentiated adenocarcinoma. Thus, the patient was diagnosed with Paget's disease combined with anal canal adenocarcinoma, and resection of the skin tumor and anal canal polypectomy were performed. One year later, due to local recurrence and right inguinal lymph node metastasis of the anal canal cancer, the patient underwent rectal resection, regional lymph node dissection and radiation therapy. Histologically, tumor cells were found continuously infiltrating from the mucous to dermal region. The immunohistochemical stain was positive for CK20 and negative for GCDFPI5 on both lesions. These findings proved the case to have intraepidermal spread of the anal canal adenocarcinoma instead of Paget's desease. [Skin Cancer (Japan) 2005; 20: 278-281]

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We reported a case of partial spontaneous regression of large Bowen's disease rising in a 74-year-old Japanese male. He had Bowen's disease on the left lower leg measuring 10×12cm, whose paracentral lesion showed spontaneous regression, which was considered to be associated with CD8⁺ T lymphocytes without amyloid deposition histopathologically. [Skin Cancer (Japan) 2005; 20: 282-285]

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A 77-year-old woman presented with a four-month history of a red tumor on the left cheek. The lesion had a dome-shaped and stemed tumor, 20×15mm in diameter. Histopathologically, the incisionally biopsied specimen showed the proliferation of clumping cells, basaloid cells and polygonal tumor cells with abundant clear, PAS-positive, diastase-sensitive cytoplasm. There were findings of actinic keratosis on the border of the tumor. No obvious metastatic lesion was found with chest and abdomen CT scan. One month after the biopsy, the nodule grew to 30×25mm in diameter. Surgical specimen showed disappearance of clear cells, prominence of eosinophilic tumor cells, and PAS-negative, and indicated the histopathological feature of well-differentiated SCC. We diagnosed this case as SCC occurred from actinic keratosis and that presented the feature of malignant trichilemmoma in progress. [Skin Cancer (Japan) 2005; 20: 286-290]

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Since July of 2000, we have tried to follow-up all patients with BCC after excision every 3 months for 2 years. Seventy-four patients with BCC were treated with excision during the course from July, 2000 to July, 2003 at the Department of Dermatology, Nagasaki University Hospital. Forty patients (54.9%) were followed at our hospital for 2 years. Eighteen patients (24.3%) did not complete the follow-up. The average age was 65.8 years in the former group and 71.7 years in the latter group. Two patients (2.7%) had a local recurrence; the intervals between excision and recurrence were 8 months and 45 months respectively. We concluded by recommending that patients, who have any high-risk factors for recurrence; recurrent BCCs, aggressive growth pattern and poorly defined borders, should be followed for 5 years in Japan. [Skin Cancer (Japan) 2005; 20: 291-295]

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We report a 49-year-old man with verrucous-keratotic malignant melanoma on his left forearm. The nodular tumor was uniformly black-colored, well circumscribed, and slightly elevated in a plateau with a warty and keratotic surface. Histopathologically, epitheloid atypical melanocytes, which formed irregular nests, were distributed from the upper part of the epidermis to the reticular dermis. The epidermis showed hyperkeratosis with papillomatous changes. The prominent clinical and histological verrucous features of this melanoma should be differentially diagnosed from other pigmented tumors such as seborrheic keratosis or basal cell carcinoma. Potential misdiagnosis of verrucous melanoma should be kept in mind. [Skin Cancer (Japan) 2005; 20: 296-300]

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We report a 62-year-old woman with eccrine porocarcinoma on perianal skin. The patient noticed a red tumor on perianal skin 10 months before the first consultation, and the tumor size had been increased. On the first consultation, elastic hard tumor with 3×4×2 cm in size was observed on the left perianal lesion, 1.5 cm away from the anus, and 2 lymph nodes were palpable in the left inguinal lesion. A diagnosis of eccrine porocarcinoma was made based on the histological findings of biopsy specimens from the tumor and left inguinal lymph node. The tumor and left inguinal lymph nodes were resected and adjuvant chemotherapy with 3 courses of FECOM (CBDCA, EPIR, MMC, 5-FU, and VCR) was given. Although recurrence of the tumor and the metastasis were not observed during the therapy, multiple coin lesions were found in the lung by computed tomography 3 months after cessation of FECOM. The 3 additional courses of FECOM did not change the number and the sizes of metastatic lung lesions for 2 months. However, weekly therapy of docetaxel was given for the metastasis in the right inguinal lymph node. The result was a condition in which, due to the number and sizes of the metastatic lung lesions but, the therapy could not prevent metastasis to the left pubic bone. [Skin Cancer (Japan) 2005; 20: 301-305]

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Case 1: An 86-year-old woman had a tumor on her left leg. One year after the tumor was excised, left inguinal lymph node metastasis appeared. Case 2: An 83-year-old man had a tumor on his right chest excised. Four years later, right axillary lymph node and right chest skin metastases developed. Case 3: A 78-year-old man was suffering from a tumor on his right waist for ten years and noticed a right inguinal lymph node swelling recently. Eight cases of EPC have been recorded in our department during the six years from 1999 to 2004. Among them, these three cases showed lymph node metastasis. In terms of treatment, radiation therapy was successful for the metastasis in case 1. Since chemotherapy is inefficient for EPC and many of the EPC patients are elderly people, radiation therapy may be a feasible choice for treatment. [Skin Cancer (Japan) 2005; 20: 306-310]

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Fifty-five patients with extramammary paget's disease treated at the Department of Dermatology, Kanazawa University of Medical Science between 1989 and 2004 are reviewed. The average age was 70.8 years old, and the male/female ratio was 2.9:1. Fifty patients had lesions on the genital area, 3 on the perianal region, 1 on the hypogastric region, and 4 on the axilla. Associated malignancies were detected in 12.7% of the cases. Twenty-seven cases had in situ regions. Lymph node metastases were found in 18% of the cases. Four patients showed local recurrence. Seven patients died from extramammary Paget's disease. The prognosis was related to the presence of nodules and the depth of invasion. [Skin Cancer (Japan) 2005; 20: 311-317]

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A 39-year-old female had noticed the presence of a subcutaneous tumor in the right lower abdomen for three to four years. She visited our department on Jul. 17th, 2003, complaining of the rapid growth of the tumor without symptoms such as spontaneous pain or pruritus. Two solid dome-shaped tumors were closely situated to each other, with diameters of 8 and 5 cm respectively, and with slightly red and brownish smooth surfaces. No palpitation was noticed.
Histopathological examination biopsied from the two tumors revealed proliferation of numerous spindle cells with striform pattern with many mitoses, positive with anti-bimentin antibody and anti-CD34 antibody stain, and negative with anti-C-kit, S-100, or NSE antibodies, which was diagnosed as fibrosarcoma. Total resection of the uterus was carried out due to the discovery of multiple tumors inside of it under MRI examination. However, hysteromyoma was diagnosed histopathologically, so that wide total resection of the fibrosarcoma was performed, including the fascia tightly attached to the tumors, 5 cm away from the borderline of the tumors with mesh-graft skin transplantation on Sept. 30th, 2003. Three cycles of chemotherapy consisting of adriamycin and iphoomid were tried, and metastatic tumors or recurrent lesions have not been found after the resection. [Skin Cancer (Japan) 2005; 20: 318-322]

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A 76-year-old woman had suffered from lymphedema of the right lower extremity for 10 years after undergoing a uterine cancer operation. She noticed multiple dark red nodules on the right lower extremity, and the number of nodules increased rapidly. Histological findings of the nodules revealed atypical tumor cells showing positive factor 8 associated antigen and CD34, resulting in the diagnosis of Stewart-Treves syndrome. She was treated with radiation, intravenous administration of inter-leukin-2, and docetaxel chemotherapy. The combination therapy of radiation and docetaxel showed a partial response. However, the nodules spread to the buttocks, lower left extremity and abdomen after three months. She died of respiratory failure one year after the first examination. In general, the prognosis of angiosarcoma, including Stewart-Treves syndrome, is poor. It is thus necessary to establish the standardization of therapy and to develop new drugs. [Skin Cancer (Japan) 2005; 20: 323-328]

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An 84-year-old woman had a two-month history of a dome-shaped tumor on the right lower jaw. Histopathological examination revealed cytokeratin20-positive tumor cells closely spaced in sheets in the dermis and subcutaneous fatty tissue. Ultrastructurally, dense-core granules, 100 nm in diameter, were identified in the cytoplasm. We diagnosed this tumor as Merkel cell carcinoma (MCC) and performed radiotherapy of 48Gy using 6MeV electrons. No evidence of recurrence or metastasis has been noted for 8 months. Several authors have reported surprising efficacy for radiotherapy as monotherapy for MCC. We reviewed 11 Japanese cases of MCC treated with radiotherapy alone. In 6 of 11 cases, no evidence of recurrence or metastasis was noted. The most common clinical features of these cases were tumor diameter of less than 3 cm and absence of lymph node metastasis at initial medical examination. [Skin Cancer (Japan) 2005; 20: 329-333]

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The patient was a 66-year-old woman who had developed systemic asymptomatic red papules for about 5 years before the initial consultation. Spontaneous recovery and recurrence of papules repeatedly occurred, and the individual papules disappeared spontaneously for about 10 days during the initial stage of the disorder. However, the interval of spontaneous recovery gradually continued several months thereafter. Pathohistological examinations demonstrated dense cellular infiltration, including large atypical CD30-positive cells, between the upper and middle layers of the dermis. Basedon the clinical course and biopsy findings, the patient was diagnosed as having lymphomatoid papulosis. Furthermore, immunostaining revealed that tumor cells fromthis patient were positive for fascin, indicating a risk to develop maligmant lymphoma. Therefore careful observation of clinical course is required in our patient. [Skin Cancer (Japan)2005; 20: 334-337]

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