In August 2011, a 61-year-old Japanese man visited our clinic complaining of a tumor on the scalp, lymphadenopathy of the neck with tenderness, and high fever. He had been aware of the tumor for 5 years, during which time it had not changed in size. The lymphadenopathy and high fever were resolved after administration of antibiotics for several days, and the tumor, 10 mm in diameter, was resected. A pathological study revealed a mixture of lobules of cells with abundant foamy cytoplasm and atypical basaloid cells, suggesting a diagnosis of sebaceous carcinoma. Wide resection with a 20-mm margin from the initial operation scar was carried out at Yokohama City University Hospital, 40 days later. Microscopy revealed no tumor cells in the specimen, and no recurrence has been observed for 15 months thereafter.[Skin Cancer (Japan) 2013 ; 28 : 13-15]

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A 99-year-old woman had noticed a pigmented spot beside her left nostril for more than ten years. In addition to two keratinized erythemas with crust and erosion on both dorsal sides of the nose, a black linear plaque 20× 2 mm was observed from the medial angle of the right eye to the cheek, along the wrinkle lines. Dermoscopic examination of the linear lesion showed a blue-gray globular pattern with hemorrhage. The lesion histologically formed nests in the dermis, continuous with the epidermis, and was composed of basaloid cells with a peripheral palisading arrangement. We diagnosed this case as linear basal cell carcinoma (linear BCC). The linear lesion was simply sutured and the dorsa of the nose were reconstructed with free skin grafts with a preserved subcutaneous vascular network. Linear BCC is an uncommon variant of BCC that most often occurs along the relaxed skin tension lines (RSTL), especially on the lower eyelid and the cheek.[Skin Cancer (Japan) 2013 ; 28 : 16-19]

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Angiosarcoma is treated with surgery, chemotherapy, radiotherapy, and/or immunotherapy with recombinant interleukin-2 (rIL-2). It is often difficult to find an appropriate treatment for angiosarcoma in elderly patients because of general states or complications of the patients. We experienced four cases of angiosarcoma which developed in patients who were 80 years old or older, and treated with weekly low-dose docetaxel (DTX) and radiotherapy (RT). The size of the tumors was reduced in all cases. The average survival period of the four patients was 17.1 months. Although no bone marrow suppression was observed, appetite loss was seen in three of the patients, and pleural fluid was seen in one. Low dose DTX + RT with sufficient care for their side effects may be effective for tumor treatment, and adequate to preserve the quality of life (QOL) of elderly patients with angiosarcoma.[Skin Cancer (Japan) 2013 ; 28 : 20-23]

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A 43-year-old woman presented with a slowly enlarging, asymptomatic mass in the posterior region of the neck. CT and MRI showed a well-circumscribed fatty mass. The tumor was completely removed surgically, and atypical lipomatous tumor/well differentiated liposarcoma was diagnosed based on histopathological examination. After an additional extensive resection, no recurrence was evident for 1.5 years.[Skin Cancer (Japan) 2013 ; 28 : 24-28]

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We report two cases of Merkel cell carcinoma : Case 1, a 58-year-old man who noticed a subcutaneous nodule on the left upper arm ; Case 2, a 79-year-old man who noticed a reddish elevated nodule on the left thigh. Histopathological examination of the nodules revealed that a nest of tumor cells with round nuclei and poor cytoplasm was localized in the dermis of the subcutaneous fatty tissue, but did not extend into the epidermis. Immunohistochemical examination revealed that the tumor cells were positive for CK20, chromogranin-A, et al. Histopathological and immunohistochemical examinations of the two cases were similar. The recently described MCPyV is reportedly present in Merkel cell carcinomas. We examined the presence of MCPyV.[Skin Cancer (Japan) 2013 ; 28 : 29-33]

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A 68-year-old woman was referred to our hospital in October 2011 for an umbilical nodule that had appeared 2 months earlier. A skin biopsy specimen taken from the lesion histologically showed atypical squamous cell invasion from the epidermis into the dermis, suggesting cutaneous squamous cell carcinoma. Magnetic resonance imaging (MRI) was performed before treatment, and revealed that she had a smooth tumor, approximately 10.5×13.5×17 centimeters at her right ovary. At that time, we diagnosed her as having a cutaneous squamous cell carcinoma and a benign ovarian tumor. However, during the operation, the ovarian tumor was diagnosed as being histologically malignant. Thus, the patient underwent wide-excision. Finally, we diagnosed this case as being a Sister Mary Joseph's nodule associated with mature cystic ovarian teratoma. She underwent additional chemotherapy combined with Paclitaxel and Carboplatin. During the follow up over the 18 subsequent months, there was no evidence of disease recurrence or metastasis. Although malignant transformation of a mature cystic ovarian teratoma is very rare, in such a case the tumor occasionally presents as a squamous cell carcinoma. Thus, a Sister Mary Joseph's nodule originating from mature cystic ovarian teratoma is indistinguishable from cutaneous squamous cell carcinoma. This article reports important information for dermatologists.[Skin Cancer (Japan) 2013 ; 28 : 34-38]

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We have experienced a large number of patients with Bowen's disease (BD) from 2004 to 2011 in the Nagasaki Municipal Hospital. The ratio of the number of BD cases to the total number of new outpatients was as much as 0.94%. No epidemic factors such as arsenic or tar in Nagasaki are present, and it is speculated that the episode of the atomic bomb disaster in 1945 is responsible for the cutaneous carcinogenesis. A detailed inquiry developed the realization that the survivors with BD tended to have plural BD and other skin cancers. Since the exposed patients with BD had no apparent high ratio of cancer compared with non-exposed patients with BD, there is a possibility of an internal exposure around the Nagasaki district. A genome abnormality and successive high risk of cancer induced by the effects of the atomic bomb explosion might have led to an increase in skin cancer over a long period of time.[Skin Cancer (Japan) 2013 ; 28 : 39-45]

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A case of squamous cell carcinoma arising in an epidermal cyst on the knee was reported. The patient was a 73-year-old male who had a thirty-year history of a mass on his right knee. During the period, sometimes foul-smelling discharge was seen from the lesion. A wide local excision with a split thickness skin grafting was performed. A histopathological examination demonstrated that the cystic lesion was composed of a benign squamous epithelium with a transition to squamous cell carcinoma. The postoperative course was uneventful, and no local recurrence or metastasis was found in 14 months after surgery.[Skin Cancer (Japan) 2013 ; 28 : 46-49]

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We report a case of verrucous carcinoma on the ear with dermal myiasis. A 66-year-old man noted a mass on the right ear five years ago. This time he appealed for the bleeding from a mass and visited a hospital. On examination, a tumor swelled like the shape of cauliflower was present on his right ear and several maggots existed in the tumor. A well-differentiated squamaous cell carcinoma was suspected by an incisional biopsy. We underwent a resection of the ear leaving part of earlobe. The final pathological findings were consistent with a verrucous carcinoma. Three years after the operation, he is well with no signs of recurrence.[Skin Cancer (Japan) 2013 ; 28 : 50-54]

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We report a case of verrucous carcinoma, an intractable cancer, which developed after an episode of diabetic foot ulcer in a young diabetic patient and which was resistant to conservative therapy. In this case, biopsy revealed features of pseudocarcinomatous hyperplasia ; however, the possibility of verrucous carcinoma was undeniable. Subsequently, tumor resection was performed using a step-ladder advancement plantar flap, and favorable recovery was achieved. Histopathological examination revealed epidermal papillary growth, dyskaryosis, multinucleated cells, and increased mitosis, which led to a diagnosis of verrucous carcinoma. There was no lymph node metastasis and at present, 13months after surgery, no signs of recurrence have been observed.[Skin Cancer (Japan) 2013 ; 28 : 55-58]

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An 81-year-old male presented with a nodule on the left nasal ala. He had notice the nodule one month before the first visit. A physical examination revealed a reddish nodule with central ulceration, measuring 17×14mm, on his left nasal ala. The lesion was histologically diagnosed as cutaneous leiomyosarcoma and was resected with a 1cm surgical margin including full-thickness of nasal ala. The defect was reconstructed using the scalping forehead flap combined with auricular cartilage graft. There has been neither recurrence nor metastasis for 2.5 years after surgery. The superficial leiomyosarcoma can be classified into cutaneous and subcutaneous types. It is important to distinguish these two types because the subcutaneous type frequently metastasizes. We reviewed the cases reported between 1963 and 2012 in Japanese literature.[Skin Cancer (Japan) 2013 ; 28 : 59-62]

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We evaluated the clinical manifestations, management, and prognostic characteristics of extramammary Paget's disease (EMPD). Sixty-nine patients with extramammary Paget's disease had been treated in our division between January 2000 and December 2011. The mean age at diagnosis was 72.4 years (range 49-97 years), and the male-to-female ratio was 2.5 : 1. Sixty-three of the sixty-nine patients had lesions in the genital area, four in the perianal area and two in the axillary area. Surgical resection was performed in sixty cases, radiation therapy was administered in five cases and chemotherapy was administered in nine cases. The 5-year survival rate was 100% in stage Ⅰ, Ⅱ, Ⅲ and 0% in stage Ⅳ. [Skin Cancer (Japan) 2013 ; 28 : 63-67]

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We report two cases of IVLBCL following a skin biopsy of senile hemangiomas which led to the diagnosis.
Patient 1 : An 82-year-old man presented with rapidly developing disturbed consciousness of several weeks duration. Serum levels of LDH and soluble IL-2 receptor were elevated. CT scan and MRI of his brain showed no abnormal findings. A physical examination revealed multiple erythemas on the abdomen and several senile hemangiomas on the chest.
Patient 2 : A 72-year-old woman was admitted for further examination of her liver and renal dysfunctions. She presented with disturbed consciousness with a duration of a few days. MRI of her brain showed high signal intensity lesions on T2-weighted images at the occipital area. Serum levels of LDH and soluble IL-2 receptor were elevated. We observed two senile hemangiomas on the neck, but no other skin lesions were noted. Biopsies from senile hemangiomas revealed capillary hemangiomas filled with atypical large B cells in both cases.
In a suspected case of IVLBCL, a skin biopsy of senile hemangioma may be beneficial to forming an early diagnosis.[Skin Cancer (Japan) 2013 ; 28 : 68-73]

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We report a case of a 69-year-old-woman with a malignant peripheral nerve sheath tumor in neurofibromatosis 1. Her abdominal neurofibroma was resected in 2003, and the recurrent neurofibroma was resected again in 2007. However, in 2009, it recurred again, and rapidly increased in size. She was referred to our hospital. We resected the tumor, and diagnosed it as low grade MPNST. We carried out radical resection on the rectus abdominal muscle with a 5 cm margin from the scar and reconstructed the skin defect by mesh graft. There has been no evidence of recurrence or metastasis 3 years post operatively. We think that the neurofibroma, which is a benign tumor, might have changed into MPNST, which is a malignant tumor, as a result of several surgeries in this case.[Skin Cancer (Japan) 2013 ; 28 : 74-78]

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An 84-year-old Japanese woman was referred to our hospital with a dark red ulcerated tumor on her right axilla and non-indurated annular erythematous lesions on her trunk.
Histological examination of the tumor showed anaplastic large lymphoid cells extensively infiltrated in the dermis and subcutaneous tissue. Numerous admixed eosinophils and histiocytes also were present. These anaplastic large lymphoid cells were positive for CD30, CD3, CD45RO, TIA-1, Granzyme B and negative for CD20, CD79, ALK, immunohistochemically. Biopsy specimens from non-indurated annular erythematous lesions showed perivascular lymphoid infiltration in the upper dermis. PET-CT revealed multiple positive lesions at bilateral axillary lymph nodes. A lymphadectomy was perfomed and anaplastic large CD30 positive lymphoid cells, eosinophils were revealed histologically.
She received eight cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) therapy and complete remission was achieved. The erythema annulare centrifugum lesions resolved following chemotherapy.[Skin Cancer (Japan) 2013 ; 28 : 79-83]

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A 56-year-old female who presented with vulvar pruritic erythema underwent wide excision and skin grafting after a diagnosis of vulvar Paget's disease. Four years later, local recurrence was identified by skin biopsy, cystoscopic biopsy and vaginal brushing smears. Paget's cells were present in the vulvar skin, the external urethral meatus, the bladder neck, the vagina and the uterine cervix. Secondary Paget's disease was excluded after positive results for cytokeratin 7 (CK7) and gross cystic disease fluid protein-15 (GCDFP-15), and a negative result for cytokeratin 20 (CK20) immunohistochemical staining.
We performed anterior pelvic exenteration : resection of the uterus, the vagina, the uterine adnexa, the urethra and the bladder, including a skin excision and the formation of an ileal conduit. A histopathological examination revealed that the Paget's cells extended horizontally to the left ureter in the bladder wall.[Skin Cancer (Japan) 2013 ; 28 : 84-88]

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An 80-year-old female patient underwent treatment for her nevus of Ota with external beta-ray 50-years ago. The irradiated areas became scarred and ulcerated a few years later. Being resistant to a variety of topical treatment, tumorous lesions had developed 2-3 years prior to her first visit. At our clinic, an irregularly shaped, verrucous hyperkeratotic mass of hard consistency was noticed from the left outer eye angle toward the upper-lateral direction. The mass was located on poikilodermic “chronic radiodermatitis” lesions. Pathology indicated characteristics of squamous cell carcinoma. The tumor was curatively and successfully excised. Statistically there have not been many cases of postirradiation skin cancer especially caused by external beta-ray. The reason for this was considered because there are few cases treated by external beta-ray. In addition, in future cases of postirradiation skin cancer, the possibility of an increase in the number of cases of basal cell carcinoma generated through long incubation periods in the use of low radiation dose was suggested.[Skin Cancer (Japan) 2013 ; 28 : 89-93]

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We report the effect of radiation therapy on squamous cell carcinoma of the skin as a first-line therapy. Ten patients who received radiation therapy as a first-line treatment for squamous cell carcinoma of the skin between 1999 and 2012 were retrospectively evaluated. The patients comprised 7 men and 3 women and had a mean age of 74.2 years. Seven patients had head and neck lesions and 4 patients had lower lip lesions. All the patients whose tumor size was under 2 cm achieved complete remission. Three patients whose tumor size was over 5 cm received chemoradiation therapy including cisplatin and 2 patients achieved complete remission. None of the patients had local recurrence after radiation during a follow-up period (4-80 months). Radiation therapy should be used if the size of the tumor is under 5 cm and it is difficult for dermatologic and plastic surgeons to excise the tumor and reconstruct the excised region functionally and cosmetically.[Skin Cancer (Japan) 2013 ; 28 : 94-97]

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A 19-year-old male patient first noticed asymptomatic erythema with petechiae covering the whole body at the age of 11, which expanded in size one year prior to his visit. Histological examination found infiltration of various cells, including small lymphocytes in the epidermis, and perivascular infiltration of small lymphocytes with an apparently notched nucleus, large, atypical lymphocytes and histiocytes in the dermis. Extravasation of erythrocytes from blood vessels was also found. Immunohistochemical analysis indicated a large proportion of CD3, CD4, and CLA-positive cells, as well as dissemination of CD30 and granzymeB-positive cells. Immunostaining was negative for TIA-1 and ALK, and monoclonal rearrangement of the T-cell receptor was not detected. The absence of Pautrier microabscesses and mycosis cells in the epidermis ruled out the diagnosis of mycosis fungoides and large cell transformation. The infiltrated cells were various and included small lymphocytes, large atypical cells, and histiocytes, while the immunohistochemistry was similarly diverse. Low positivity for CD30 ruled out the diagnosis of primary cutaneous anaplastic large cell lymphoma, while the clinical findings were not consistent with LyP. On the basis of these observations, we made the diagnosis of cutaneous T-cell lymphoma with petechiae.[Skin Cancer (Japan) 2013 ; 28 : 98-102]

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Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor which may recur locally and rarely causes distant metastasis. It is characterized by a specific rearrangement of chromosomes 17 and 22. We present 2 cases of metastatic DFSP who were treated with imatinib methylate. Case 1 : a 43-years-old woman who had a large DFSP on the abdomen underwent wide local excision.
Five years after surgery, multiple intraabdominal metastases occurred and she was started on daily oral imatinib mesylate at a dose of 400mg/body on an outpatient basis. Three months after administration of the treatment, an18% reduction of the tumor size was obtained. Case 2 : a 46-years-old man who had a large DFSP on the abdomen underwent wide local excision. Three years after surgery, lung and skin metastases occurred and were resected. Seven months after the second surgery, mediastinal metastasis subsequently appeared 7 months after the second surgery. He was started on daily oral imatinib mesylate at a dose of 400mg/body on an outpatient basis and the dose was increased to 800mg/body. Twenty months after the initiation of treatment, computed tomography showed that the metastatic lesion in the mediastinum had disappeared.[Skin Cancer (Japan) 2013 ; 28 : 103-107]

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