We report a 54-year-old male who presented with a hemorrhaged black & red nodule in his left toe. We clinically diagnosed this tumor as malignant melanoma. The inguinal lymph nodes were already palpable, but his popliteal lymph node was not palpable. Before his operation, computed tomography surprisingly revealed metastases to the iliac lymph node, groin lymph nodes and popliteal lymph nodes. Preoperative lymph scintigraphy detected the exact location of the sentinel lymph node in the inguinal region but not in the popliteal region. Therefore we think that metastasis to the popliteal lymph node was caused by partial lymphatic obstruction in his groin. It is very rare for melanoma to metastasize from the toe to the popliteal lymph node. Toe amputation and lymph node dissection in the pelvic, inguinal and popliteal regions were performed. His treatment was followed by adjuvant chemotherapy with DAC-Tam-feron. There has been no evidence of tumor recurrence or metastasis one year after surgery.[Skin Cancer (Japan) 2011 ; 26 : 129-133]

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An 85-year-old man presented with a one-month history of generalized skin rash associated with continuous low-grade fever and sweating at night. A skin biopsy specimen showed a nonspecific finding of perivascular dermatitis. Laboratory findings revealed mild anemia, polyclonal hypergammaglobulinemia, and elevated levels of C-reactive protein, erythrocyte sedimentation rate and soluble interleukin-2 receptor. No serological abnormalities were found for HTLV-1 or EBV. Computed tomography findings showed generalized lymphadenopathy and mild splenomegaly. Histological findings of the inguinal lymph node showed destructive proliferation of CD4 and CD45RO positive atypical medium-sized lymphocytes associated with angiogenesis. Southern blot analysis detected a clonal rearrangement of T-cell receptor Cβ1 genes. Bone marrow aspiration revealed infiltration of lymphocytes with the same phenotype as those of the tumor cells in the lymph node. Based on these findings, a diagnosis of angioimmunoblastic T-cell lymphoma with bone marrow involvement (stage IV) was made.[Skin Cancer (Japan) 2011 ; 26 : 134-138]

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We report a 44-year-old man with brain, lung and retroperitoneum metastasis from malignant melanoma treated with dacarbazine. The primary lesion was not detected. He received stereotactic radiosurgery for the brain metastasis, followed by single-dose dacarbazine (250mg/m²/day × 5 days). After 3 courses, a 55.8% reduction in lung metastasis was observed, without serious adverse reaction. After 6 courses, the lung metastasis was removed surgically. Four months after surgical resection, new metastatic lesions were detected in the mediastinum, pancreas, and mesentery. He was treated with stereotactic radiosurgery for increased brain metastasis, followed by one single course of dacarbazine (250mg/m²/day × 5 days) and 2 single courses of cisplatin (100mg/m²/day × 1 day). He died 20 months after the first consultation with us. The combination therapy composed of single dacarbazine, stereotactic radiosurgery and surgery might have contributed to durable survival without serious adverse effect in this case.[Skin Cancer (Japan) 2011 ; 26 : 139-142]

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We report a case of head angiosarcoma in a 44-year-old Japanese male who presented with a parietal mass, and also a cervical mass during the same period. We performed a skin biopsy on the head mass, and diagnosed it as angiosarcoma. We extracted the malignant skin tumor, and covered the defect with a full thickness skin graft. However, the resection stump tested positive for cancer cells in histopathological examination ; we therefore added radiotherapy to his treatment. We diagnosed the right parotid neoplasm as metastasis of angiosarcoma, by needle biopsy. Three months later, the patient died postoperatively due to respiratory insufficiency caused by alveolar haemorrhage. Werner syndrome was diagnosed on the basis of the patient's consanguineous parents, a history of binocular cataracts, white hair and eyebrows indicating premature aging, a characteristic bird-like facial appearance, and elevated levels of hyaluronic acid following blood tests.[Skin Cancer (Japan) 2011 ; 26 : 143-147]

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We present 3 female patients with vulvar malignant melanoma. Case 1 : A 68-year-old woman noticed a small black nodule on the left labia majora one month before the first consultation. The tumor (tumor thickness 2.1 mm, pT3aN0M0, stage IIA) was removed with bilateral inguinal lymph node dissection. Five courses of chemotherapy (DAV-feron) were performed after surgical therapy. There has been no evidence of tumor recurrence or metastasis for 3 years. Case 2 : A 62-year-old woman noticed a tumor at the perineum one year before the first consultation. The tumor was resected with a 5 mm margin (tumor thickness 12.4 mm), and right inguinal lymph node biopsy was carried out because she rejected the radical operation. The histopathology of the inguinal lymph node showed metastatic melanoma (pT4bN3M0, stage IIIC). She died 8 months later due to a metastatic liver tumor. Case 3 : A 32-year-old woman noticed a tumor at the vulva two years before the first consultation. The tumor was excised with a 10 mm margin (tumor thickness 8 mm), and the sentinel lymph node biopsy showed metastatic melanoma in the inguinal lymph node. Further excision and right inguinal lymph node dissection were performed (pT4bN1bM0, stage IIIC). She rejected chemotherapy because she hoped to become pregnant in the future. Since local recurrence and metastasis to the left inguinal lymph node and lung were observed after one and a half years, chemotherapy (DAC-Tam) was performed. There is a poor prognosis for patients with vulvar malignant melanoma because women hesitate to consult doctors, resulting in missing the chance for early treatment. The effects of expanded operation and chemotherapy on vulvar malignant melanoma have been controversial. It is necessary to accumulate more data regarding therapies for vulvar malignant melanoma, although treatment at present follows the guideline for malignant melanoma.[Skin Cancer (Japan) 2011 ; 26 : 148-152]

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A 69-year-old man presented with a 1-year history of a slowly growing solitary nodule on his left cheek. A dermatological examination revealed a reddish, elastic firm, dome-shaped nodule, measuring 23×20 mm. Histologic examination revealed nests of tumor cells in the dermis. The tumor cells showed pale, oval to polygonal cell types with mitoses and necrosis. Immunohistochemically, the tumor cells were positive for chromogranin A, gross cystic disease fluid protein 15, estrogen receptor, progesterone receptor, and negative for vimentin, CAM5.2, cytokeratin (CK)7, CK20, CEA and S-100. The tumor cells were partially positive for mucicarmine, suggesting mucin-production. No distant or lymph node metastasis was found in positron emission tomography-computed tomography (PET-CT) and enhanced CT. The diagnosis of neuroendocrine sweat gland carcinoma was made. Wide resection and sentinel node biopsy (SNB) were performed. SNB revealed micrometastases in the cervical lymph node. We therefore performed lymphadenectomy, and added irradiation at a total dose of 50Gy. No recurrence or metastasis of the tumor has been noticed for 6 months after lymphadenectomy.[Skin Cancer (Japan) 2011 ; 26 : 153-156]

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An 81-year-old man noticed a small nodule on his left thigh five years ago. Since the tumor had increased in size during the past several months, he visited a clinic. Complete surgical excision was performed on a diagnosis of squamous cell carcinoma. The patient was referred to our hospital for further treatment.
Erythema and swelling were noted on his groin, and limb. PET-CT scan revealed metastasis to the para-aortic and groin lymph nodes. Histological examination of the left inguinal lymph nodes and erythema confirmed the tumor invasion. We finally diagnosed his condition as MPTC (pT2N1M1: stage IV). We selected radiation as symptomatic therapy. It was temporarily effective, but multiple metastases appeared on the whole left leg and he died within 3 months of the occurrence of the skin eruption.[Skin Cancer (Japan) 2011 ; 26 : 157-160]

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A 46-year-old woman presented with a skin tumor on her right temporal scalp ; it was resected at another clinic and was diagnosed as squamous cell carcinoma three years ago. This time, the patient noticed a reddish nodule on her right buttock one month before her first visit to our department. We performed an incisional biopsy, and the histological diagnosis was squamous cell carcinoma. Although general examinations such as MRI, CT, Ga-scintigraphy were performed, no distant metastases were identified. We resected the tumor on a fascia with a 3 cm margin under general anesthesia. After confirming the margin to be negative by quick pathological diagnosis, a split thickness skin graft was placed on the wound. There has been no evidence of recurrence or metastasis of the tumor one year after operation. We compared the tumor of the head with that of the buttock histopahologically. It was thought that these were different manifestations.[Skin Cancer (Japan) 2011 ; 26 : 161-164]

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Sebaceous nevus (SN) is known to be associated with several types of secondary malignant tumors. Among secondary malignant tumors, Basal Cell Carcinoma is thought to be the most frequent malignant tumor occurring on SN. Squamous cell carcinoma (KA type SCC) is thought to be rarer. We report a case of KA type SCC arising in SN.
A 53-year-old Japanese woman presented with a 6×5 cm ulcerated giant tumor on her scalp with a duration of four years, on which she had a skin-colored plaque since her childhood. Histological examination revealed KA type SCC arising from SN.
The tumor was resected above the myofascial, and skin grafting was performed.
After the operation, severe local infection of pseudomonas aeruginosa occurred. Wide debridement was performed, and the defect was reconstructed with a free flap from the latissimus dorsi muscle.
There has been no evidence of recurrence one year after surgery.[Skin Cancer (Japan) 2011 ; 26 : 165-169]

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We report a case of extraocular sebaceous carcinoma with basal cell carcinoma-like structures. An 85-year-old woman presented with an erosive lesion of her left cheek. Physical examination showed an erosive, crusted erythematous plaque with 11×12mm in diameter. A histopathological examination showed two distinctive patterns. One pattern was tumor nests comprising clear and vacuolated cells. These cells proliferated from epidermis to reticular dermis. The other pattern was tumor nests composed of small rounded basophilic cells with palisading architecture embedded in fibrous tissues. Immunohistochemical analyses revealed that tumor cells with sebaceous differentiation were positive for CAM5.2, CK19, CEA, EMA and Adipophilin, and weakly positive for Ber-EP4, but not CK1, and that tumor cells in basal cell carcinoma structures were positive for Ber-EP4, CAM5.2 and CK19, but not CK1, CEA, EMA and Adipophilin. Diagnosis of sebaceous carcinoma was made. A local excision with a1 cm margin was performed. The patient has been free from recurrence and metastasis.[Skin Cancer (Japan) 2011 ; 26 : 170-173]

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A 66-year-old woman noticed a pink nodule in her lower left thigh. After resection in April 2009, an enlarged left inguinal lymph node and left lower thigh edema were found. The patient was referred to our hospital in August. Histopathologically, the primary lesion was diagnosed as Merkel cell carcinoma accompanied by Bowen's disease in the surrounding area. Consecutive lymph node enlargement in the left inguinal area to the para-aortic area, and left hydronephrosis were found in CT imaging. Chemotherapy was initiated in accordance with the treatment of small cell lung cancer : one course of IP therapy (CDDP+CPT-11). four courses of EP therapy (CDDP+ETP) and one course of EC therapy (CBDCA+ETP). The metastatic lesion was significantly reduced and partial response could be maintained, though it only lasted a short time. She died of the disease in June 2010.[Skin Cancer (Japan) 2011 ; 26 : 174-178]

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A 74-year-old woman with metatstasized skin and lymph node lesions of Merkel cell carcinoma (MCC) was treated with chemotherapy using three different regimens. CDBDA+ETP, the first line chemotherapy for small cell lung cancer (SCLC), was administered first, and she showed stable disease in the CDBDA+ETP regimen. After 3 courses of CDBDA+ETP regimen, metastatic lesions regrew. We therefore switched to a cyclophosphamide, doxorubicin, and vincristine (CAV) regimen. She could not achieve a complete response, but achieved partial response in the CAV regimen. In our case, metastatic MCC was resistant to the chemotherapy using CPT 11 alone. Effective treatment options are limited for patients with metastatic MCC. The standard regimens of chemotherapy for MCC have not been established ; therefore, we had temporarily adopted regimens for SCLC, histological and cell biological features which are similar to MCC. Future clinical research should explore the most appropriate regimen for metastatic MCC.[Skin Cancer (Japan) 2011 ; 26 : 179-183]

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A 65-year-old male had a skin colored nodule on the nasal tip, and scattered black papules and patches on the head and neck, which he had noticed a year ago. A diagnosis of multiple basal cell carcinomas (BCCs) was made by skin biopsy in our hospital. The nasal tumor was excised completely, and the nasal tip was reconstructed with a nasolabial flap. The other BCCs were resected at the same time. He was also found to have some pits on the palms, and we suspected him of suffering from the nevoid basal cell carcinoma syndrome (NBCC). Calcified falx cerebri and a meningioma in the celebellum were identified by Computed Tomography and Magnetic Resonance Imaging. We finally diagnosed him as having NBCC. When a person with multiple BCCs and palmoplantar pits is diagnosed with NBCC, we should excise the BCCs immediately before others grow.[Skin Cancer (Japan) 2011 ; 26 : 184-190]

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Between January 1995 and December 2008, 133 new patients with cutaneous lymphomas were seen at the dermatology clinic of Okayama University Hospital. All of the patients were re-classified according to the revised WHO classification. The incidence rates were analyzed and the chances of survival were estimated. Of 133, 106 (79.7%) had primary cutaneous lymphomas (PCLs).
Compared with several reports from western countries, “mature T-cell and NK-cell neoplasms” were frequent in this study (87% vs. 77 or 72%). The 106 PCLs consisted of 56 (52.8%) with mycosis fungoides/Sezary syndrome (MF/SS), similar to rates in western countries. Six (5.7%) with adult T-cell leukemia/lymphoma (ATLL) were observed, rarely reported from western countries. No primary cutaneous follicle center lymphoma (pcFCL) was observed during this period.
The estimated survival of patients with MF was favorable (5-year disease specific survival rate : 5-y DSS 90.6%), but that of the patients with primary cutaneous anaplastic large cell lymphoma (C-ALCL) was extremely less favorable than previously reported (5-yDSS 47.4%).[Skin Cancer (Japan) 2011 ; 26 : 191-196]

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A 45-year-old man had a slightly elevated lesion on the occipital site from birth. He visited the Department of Neurosurgery on January 14th in 2009 because it had gradually increased in size. A head CT scan did not reveal invasion to the bone and the brain ; therefore, he was sent to the Department of Dermatology for consultation. A skin biopsy confirmed the suspected diagnosis of syringocystadenoma papilliferum ; we then resected the tumor. Pathological examination showed two different lesions in the dermis, which were not connected with the epidermis. One lesion had many nests of tumor cells with atypical nuclei which had increased, and the other included large or small lumens. The former consisted of the sweat gland carcinoma showing Alcianblue, D-PAS (+), CK7 (+), CEA (+), and sebaceous carcinoma showing CK7 (+), adiphophilin (+). And as we observed organoid nevus around the tumor, it was suggested that this tumor had developed from the organoid nevus and had become differentiated to the sweat gland and sebaceous glands.[Skin Cancer (Japan) 2011 ; 26 : 197-200]

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In the treatment of cutaneous angiosarcoma of the scalp, a combination therapy with radiation and anticancer taxoid pharmacological agent after limited or no surgery is replacing the use of a wide local resection. We report two cases : an 88-year-old Japanese female with dementia and an 85-year-old Japanese female with dementia and hemi-palsy after cerebral infarction. Histopathologically, both patients were given diagnoses of cutaneous angiosarcoma of the scalp. The tumors were each treated with radiation therapy and showed complete remission. However, after the primary treatment with radiation, case 1 demonstrated recurrence in the left parotid gland. She received a treatment with docetaxel hydrate but could not continue to have the treatment because of myelosuppression, and died 25 months after the first examination. Case 2 was administered docetaxel hydrate as an ambulant treatment. However, she could not undergo the full treatment course and a local recurrence developed. Therefore, she was hospitalized to receive the standard dose, but a metastasis to the lung was detected and she died 18 months after the first examination. We consider it important to combine radiation with anticancer drugs for cutaneous angiosarcoma. However, patients more than 80 years old often cannot tolerate the full course of anticancer drug therapy because of latent immunodeficiency, underlying medical conditions or background social factors. We emphasize that an evaluation of age and latent immunodeficiency is important in order to establish a standard treatment for cutaneous angiosarcoma of the scalp.[Skin Cancer (Japan) 2011 ; 26 : 201-205]

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The patient was a 60-year-old Japanese male with a 3-year history of erythematous and brown plaque on the body with alopecia on the scalp. The biopsy specimens revealed lymphocytic infiltrates in the epidermis and hair follicles, and part of the hair follicle was destroyed. Immunohistochemical stains showed that most infiltrating lymphoid cells expressed CD3⁺, CD4⁺, CD8⁺ and lacked CD7 expression, and a southern blot analysis for the T-cell receptorβ-chain Cβ1 revealed clonal rearrangement. The diagnosis was mycosis fungoides, stage IB (T2 N0 M0). This case showed similarity to folliculotropic mycosis fungoides (FMF) because of its marked folliculotropism and rapid progress.[Skin Cancer (Japan) 2011 ; 26 : 206-209]

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A 78-year-old man was referred to our department via the Department of Surgery at our hospital due to a recurrent thyroid tumor. He had undergone surgery for thyroid cancer at another hospital 15 years earlier, and resection of a local recurrence 12 years ago. He had received a total of 85 Gy of radiation for two other local tumor recurrences, 11 and 5 years ago respectively. He had been referred to the Department of Surgery due to a subsequent increase in tumor size as well as an increase in bleeding from 1 year ago.
A hemorrhagic and malodorous mass was observed on the right side of the anterior neck region on initial examination. As the tumor was highly hemorrhagic, embolization of the right superior thyroid artery was performed four times, and Mohs chemosurgery was performed to stop the bleeding from the tumor surface and to reduce the tumor size. Mohs chemosurgery reduced the amount of bleeding from the tumor region as well as the tumor size, results which improved the patient's quality of life (QOL).[Skin Cancer (Japan) 2011 ; 26 : 210-214]

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An 80-year-old woman presented with a red tumor on her left thigh which enlarged in the last three months. Histologically, the tumor cells had scant cytoplasm and round nuclei and expressed CK20, chromogranin and synaptophysin. The tumor was diagnosed as Merkel cell carcinoma. But after the biopsy the tumor began decreasing in size and regressed. Wide resection of the primary lesion was performed and sentinel node biopsy disclosed no residence of tumor of the primary lesion and no lymph node metastasis.[Skin Cancer (Japan) 2011 ; 26 : 215-218]

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A 42-year-old male visited our clinic asking for the treatment of a lesion histopathologically diagnosed as cutaneous malignant melanoma, arising on a pigmented plaque on his back and presenting since his childhood. The tumor thickness of the lesion was 4.5 mm and its TNM classification was stage IIC (T4bN0M0). Wide excision of the lesion and sentinel node biopsy were performed, and no metastasis was noted in the sentinel lymph node. After five courses of DAV-feron regimen had been administered, three million units of beta-interferon were administered intralesionally, monthly. Multiple liver metastases were noted by abdominal computed tomography examination one year and three months after wide resection of the lesion. Partial remission for four months was obtained after two courses of transarterial chemoembolization using cisplatin and gelatin sponge. A thirteen-month survival was achieved since the appearance of multiple liver metastases.[Skin Cancer (Japan) 2011 ; 26 : 219-222]

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We report a case of giant vascular eccrine spirademona which is a rare variant of eccrine spiradenoma. A 69-year-old man presented with a history of a small painful tumor on his right upper arm, which had developed 30 years ago.
On physical examination, a 25 mm sized, firm, smooth shaped subcutaneous nodule was seen.
A biopsy specimen of the lesion stained with H&E showed two tumor masses in the dermis ; the sizes were 20 mm and 3 mm in diameter. Histopathologic examination revealed clearly delimited cords, showing two types of cells : cells with large pale nuclei in the center and basaloid cells with small, dark nuclei at the periphery, and prominent blood-filled vessels.
These histological findings were diagnostic of giant vascular eccrine spiradenoma, a rare variant of eccrine spiradenoma.
Immunohistochemical findings have suggested differentiation toward the intradermal duct, secretory portion, and myoepithelial cells.[Skin Cancer (Japan) 2011 ; 26 : 223-227]

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We report a case of Malignant Peripheral Nerve Sheath Tumor (MPNST) with chest wall reconstruction. The patient was a 76-year-old man who had a subcutaneous tumor around the left hypochondrium. We performed massive resection and reconstruction of the chest wall by using a Marlex-mesh, latissimus dorsi muscle flap and a mesh skin graft. This tumor was histopathologically S-100 protein positive. The tumor was diagnosed as an MPNST. No recurrence or distant metastases were noted after 2 years' follow-up.[Skin Cancer (Japan) 2011 ; 26 : 228-232]

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