Angiosarcoma frequently occurs on the scalp and face in elderly patients and tends to recur locally and metastasize early despite multimodality therapy. Generally, angiosarcomas have been treated with surgical excision, radiation therapy, chemotherapy and biotherapy. But the overall prognosis is poor. This report is a retrospective study of 260 patients with angiosarcoma, based on a study conducted by The Japanese Association of Dermatologic Surgery. We investigate prognostic factors and optimal treatment.
We consider that advanced age and formation of ulcers is associated with a poor prognosis. We suggest that surgical excision to histological negative margins and chemotherapy improve the prospect for survival.[Skin Cancer (Japan) 2009 ; 24 : 350-362]

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Twenty patients with angiosarcoma of the scalp who had been treated at our hospital between 1988 and 2008 were reviewed. The average age was 70.8 years old (range : 30-90 years), and the male/female ratio was 14 : 6. The median survival period was 21.4 months. Fourteen patients had developed distant metastasis, ten had developed lung metastasis, and six had developed cervical lymph node metastasis. Eleven patients underwent surgery and sixteen patients underwent IL-2 therapy and radiotherapy. Nine patients underwent chemotherapy ; we currently use Docetaxel chemotherapy as an adjuvant therapy after surgical excision. We suggest that local recurrence is not associated with survival time, and taxoid chemotherapy is useful for the control of distant metastasis.[Skin Cancer (Japan) 2009 ; 24 : 363-368]

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We analyzed 18 patients with angiosarcoma treated at Okayama University Hospital in the past 15 years. The first 4 cases received a surgical excision as an initial treatment for the primary tumour, but some of these cases experienced local or distant recurrence possibly related to the wound healing process after surgical treatment. Other cases received radiotherapy as a key treatment thereafter. Most cases underwent systemic therapy such as administration of IL-2 or, recently, taxane in combination with topical therapy. Four cases treated with radiotherapy for primary lesions survived more than 3 years, but none with surgical treatments. Patients with long-term survival seemed to have better response to radiotherapy. However, we could not conclude the superiority of radiotherapy because of the paucity of cases with the disease. A prospective, multi-institutional, comparative clinical study to determine the adequate treatment would be needed. In addition, PET/CT was effective for the detection of metastatic lesions.[Skin Cancer (Japan) 2009 ; 24 : 369-376]

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Seventy-one patients with angiosarcoma were treated in our department during a 21-year period between 1987 and 2008. Sixty of them had angiosarcoma of the scalp, and the total number of follow-up patients was fifty-five. Of the 55 patients, 19 until 2000 were treated mainly with IL-2/LAK immunotherapy, and 36 after 2001 were treated according to our stage-distinct remedy strategy established in 2003. The basic therapeutic plan of our strategy is complete radiotherapy followed by continuous “taxoid”-chemotherapy for six months at least to prevent recurrence. As a result, our strategy indicated a better prognosis than immunotherapy only. However, lung-metastasis is still a main cause of death. Intra-pleural chemo-therapy introduced by us makes it possible to mitigate a poor prognosis.[Skin Cancer (Japan) 2009 ; 24 : 377-384]

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Angiosarcoma is a rare, highly aggressive tumor. It is a malignant vascular tumor that compromises only approximately 1% of all skin malignancies in Japan. The overall prognosis is poor, with reported 5-year survival rates of approximately 10%. Given the rarity of the tumor, relatively little is known concerning its features, natural history, or optimal treatment. Although surgical resection remains the cornerstone of therapy, because of the pattern of diffuse, clinically undetectable spread, the disease is sometimes difficult to resect completely. Adjuvant radiation therapy has resulted in a significant benefit in local control. Effective systemic treatment options are limited for patients with angiosarcoma. Two taxanes (paclitaxel and docetaxel) demonstrated good efficacy in patients with advanced angiosarcoma. We also reviewed the efficacy of MAID (Mesna/Ifosfamide/Doxorubicin/Dacarbazine) combination chemotherapy in them. Future clinical research should be explored by therapeutic centers for establishing a treatment policy for this rare tumor with a very poor prognosis.[Skin Cancer (Japan) 2009 ; 24 : 385-391]

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An artificial dermis can be temporarily applied after excision of skin cancer. The most favorable indications for this method are as follows : 1) tumors with unclear margins ; 2) tumors, in which the depth or invasion level cannot be determined with intraoperative histopathological diagnosis ; 3) tumors for which the degree of malignancy is difficult to determine. In addition, “4) deep defects after removal of tumors” can also be an indication for an artificial dermis. The advantage of this method is that reconstruction can be implemented after a definitive diagnosis is made in cases when the degree of malignancy cannot be definitely determined with biopsy, or when the width of excision margin cannot be determined before surgery. Therefore, even when there is a residual tumor after excision or the safety margin is not sufficient, the skin graft or flap will not be wasted at the time of additional excision. In addition, this method can provide sufficient time for consulting with patients and their families on the reconstruction method after a definitive pathological diagnosis has been made, and it is also beneficial for obtaining informed consent. On the other hand, the disadvantage is that two surgeries are required, and this leads to a prolonged treatment period.[Skin Cancer (Japan) 2009 ; 24 : 392-396]

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Radical surgery is necessary for the surgical treatment of malignant skin tumors. However, depending on the patient's background, the type of cancer and its location, local skin flap surgery is often required as the reconstruction method since it takes into consideration aesthetic and functional aspects after surgery. Nonetheless, in malignant melanomas, skin grafting is preferred as a reconstruction method because of issues such as [#1] the detection of local recurrence, [#2] lymph flow from the primary tumor, and [#3] in-transit metastases. Regarding [#1], thin skin grafts are used in order to facilitate the early detection of local recurrences. Therefore, in such cases, we are careful never to take grafts from donor regions within the lymphatic basin. Concerning [#2], it is a fact that the conditions of the creation of local skin flaps will inevitably bring forth limitations. Skin flap creation might even have an impact on postoperative aftercare. Also in regard to [#3], the reasons include the fact that local skin flaps are not suitable for the early detection of in-transit metastases or for their treatment by subtotal integumentectomy.[Skin Cancer (Japan) 2009 ; 24 : 397-402]

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In reconstruction following the resection of malignant skin tumors, the optimal treatment plan must be selected taking into account all factors with regard to both the human factor and the tumor factor. But, in the reconstructive factor, the principle of the defect coverage is, “The best tissue is the same tissue.” The coverage achieved by using the neighboring tissue is the best. Such a principle is especially suitable for the scalp area, except where we cannot locate a donor for the hairy region. Scalping flap is aggressively used for the reconstruction of the defects of the scalp after excision of malignant skin tumors, taking into consideration the human, tumor and reconstruction factors. Our scalping flap is a V-Y advancement and island fashioned flap that has a vascular network of superficial temporal, posterior auricle and occipital vessels. It is possible for this method to be used for reconstruction of large-sized defects on all areas in the scalp. We consider that our procedure is the first choice for reconstruction after excision of a malignant skin tumor in the scalp.[Skin Cancer (Japan) 2009 ; 24 : 403-408]

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In periocular reconstruction after cancer resection, three aspects should be considered : 1) the oncological aspect, 2) the functional aspect, 3) the aesthetic aspect. A one-stage operation is oncologically better, because it allows early adjuvant therapy. The flap is desirably harvested from a nearby area to match the color and texture. However, in the case of a highly malignant tumor, the flaps containing lymphatic vessels between the tumor and the lymph nodes should be avoided. We think transferring functional orbicularis oris muscle to the upper lid margin enables the eye to close more securely.
We reconstructed the periocular region after resection of highly malignant tumors with a one-stage operation, namely, median forehead flap, orbicularis oris bi-pedicle flap, or free radial forearm flap, etc. In case of the free flap, we think replacing the free flap skin by skin graft harvested from a nearby area such as the subclavicular area allows the color and texture to match better. We used standard methods after resection of low and moderate malignant tumors, namely, switch flap from the lower eyelid, lateral orbital flap, etc. These flaps are aesthetically better than median forehead flaps. There was no local recurrence in 27 cases after a mean follow-up period of 5 years.[Skin Cancer (Japan) 2009 ; 24 : 409-415]

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A retrospective investigation was performed of the patients who underwent resection of malignant skin tumors of the nose at our hospital during the past 5 years. Among 23 patients with BCC of the nose who underwent reconstruction with either a skin flap or skin graft, 17 patients received a local skin flap and 6 patients received a skin graft, showing the predominance of local flaps. With respect to the relationship between tumor size and the method of reconstruction, local skin flaps were selected for the tumors with a mean longer axis of 7.8mm, and skin grafts were selected for those with a mean longer axis of 12.6mm. Thus, skin grafts were selected for longer tumors, but a statistically significant difference was not observed. The mean age of the patients who received local skin flaps was 67.4years, whereas the mean age of those who received skin grafts was 77.0years. Although reconstruction with a skin graft was more frequent among relatively older patients, there was also no statistically significant difference compared with younger patients.[Skin Cancer (Japan) 2009 ; 24 : 416-422]

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We analysed 55 patients who underwent perineal reconstruction after malignant tumor resection in the department of plastic and reconstructive surgery in the Tokushima University Hospital. This group comprised 19 male and 36 female patients. There were 18 patients with squamous cell carcinoma, 25 with extramammary Paget's disease, 2 with malignant melanoma, 1 with basal cell carcinoma and 9 with other tumors. The defects were reconstructed with skin graft in 12 cases and with flap transplantation in 43 cases. The transplanted flaps consisted of gluteal fold flap (internal pudendal artery perforator flap) in 31 cases, rectus abdominis flap (musculocutaneous flap) in 6, gluteus maximus muscle (perforator) flap in 3, and scrotum flap in 3. We had no recurrent case, and only one case who underwent flap revison.[Skin Cancer (Japan) 2009 ; 24 : 423-426]

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Skin cancer can develop in any region of the body ; thus, an aggressive approach is required regarding both resection and reconstruction. This report presents an interdisciplinary team approach for treating patients with complex advanced skin maligancy.
A series of eight patients with a cutaneous malignancy underwent free flap reconstruction. Three patients developed lesions on the scalp, three were on the face, one on the hand, and one on the lower extremity.
The lesions were resected with a sufficient margin, and reconstruction was carried out by a surgical team of dermatologists, plastic surgeons, neurosurgeons, head and neck surgeons and orthopedic surgeons.
Each patient was offered additional treatment after surgery by the interdisciplinary team depending upon their individual needs. This interdisciplinary team approach provided the optimal treatment for these patients with complex advanced skin malignancy, because each specialist addressed a specific need of the patient.[Skin Cancer (Japan) 2009 ; 24 : 427-434]

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We performed photodynamic therapy (PDT) on 30 patients with solitary actinic keratosis (AK), and reported the treatment results. We added 10 cases and report the results. The method of PDT consisted of the application of 20% ALA for 4 hrs, after which exposure to an excimer-dye laser at 630 nm was performed at a dose of 50J/cm² 3 times at weekly intervals. Therapeutic effects were assessed 1 week after PDT and atypical cells were observed in 4 patients. During a follow-up of 12 months, 3 patients relapsed. Furthermore, AK lesions were divided into 2 groups according to diameter (1cm), and the histological severity was classified according to the classification of Röwert et al. (mild : atypical keratinocytes found in basal layers of the epidermis ; moderate : extending to the lower two-thirds of the epidermis, severe : more than two-thirds of the epidermis). We finally classified them into two groups : group A (mild & moderate) and group B (severe) and compared the treatment results in each group.[Skin Cancer (Japan) 2009 ; 24 : 435-438]

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Photodynamic therapy (PDT) is useful for the treatment of superficial skin cancer. Since 2003, we adopted the light emitting diode (LED) as a light source, with which we treated 38 cases of actinic keratoses. Thirty-three cases out of 35 Japanese cases responded well to our protocol of PDT with an LED light source. However, we found 9 recurrent cases of AK ; therefore, we performed surgical treatment. PDT therapy for AK is effective, but the standard protocol (first dose, durations of follow-up, interval of each PDT etc.) has not been established. Accumulation of cases and future clinical observation should explore and establish the standard protocol of PDT for AK.[Skin Cancer (Japan) 2009 ; 24 : 439-441]

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Photodynamic therapy with 5-aminolevulinic acid (ALA-PDT) is a non-invasive, safe, cosmetic outcome, and effective treatment for superficial skin cancers. Although common basal cell carcinoma (BCC) in Japanese populations is a melanotic lesion, ALA-PDT is effective in the treatment of certain non-melanoma skin cancers. We report cases of BCC treated with ALA-PDT using a light emitting diode (LED). Between April 2008 and March 2009, at the Department of Dermatology, Kyorin University School of Medicine, 3 superficial BCCs on the faces and 2 nodular BCCs on the backs of 5 patients were treated with 3 to 8 ALA-PDT sessions. Finally complete response was detected in 3/3 superficial BCCs, and 2/2 nodular BCCs. ALA-PDT is an effective treatment not only for superficial BCC but also for nodular BCC.[Skin Cancer (Japan) 2009 ; 24 : 442-449]

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Photodynamic diagnosis (PDD) is a non-invasive method to diagnose the possibility of cancer (or precancer). In dermatology, ALA (5-aminolevulinic acid)-induced protoporphyrin IX (PpIX) is used for PDD as well as for photodynamic therapy (PDT). The fluorescence of induced porphyrins is effective in detecting and delineating neoplastic skin areas, especially in patients with multiple lesions of actinic keratosis. However, in some patients with extramammary Paget's disease on extragenital areas or with squamous cell carcinoma on the earlobe, the detection of the area using PDD was not always compatible with the histopathological investigation. In the extragenital areas or the earlobe, the area determined by PDD may not be accurate.[Skin Cancer (Japan) 2009 ; 24 : 450-454]

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Surgical resection continues to be the basic treatment for skin cancer, but with an increase in the number of elderly patients and patients with complications, the number of cases that are not ideal candidates for surgery has also been increasing. We have adopted topical photodynamic therapy (PDT) using 5-aminolevulinic acid, and topical immunotherapy with imiquimod cream as two types of noninvasive treatment that can be performed on such patients. It is necessary to be careful when selecting candidates, however. PDT and topical imiquimod treatment are, nevertheless, considered to be useful treatments for patients with extramammary Paget's disease.[Skin Cancer (Japan) 2009 ; 24 : 455-462]

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The diagnostic chart for dermoscopy consists of a 3-point checklist as a screening technique and a two-step procedure for the classification of pigmented lesions. If we use this diagnostic chart, we can conduct melanoma and basal cell carcinoma screening ; we have only to check the list, and save the effort of writing the findings in difficult technical words, and then we can follow the diagnostic algorithm correctly. The three-point checklist was intended for use by non-experts as a screening technique. Its sensitivity for detecting malignancy by non-experts was about 90%. The two-step procedure distinguishes the difference, at the first step, between melanocytic and non-melanocytic lesions and diagnoses non-melanotic lesions as seborrheic keratosis, basal cell carcinoma, angioma, and angiokeratoma. At the second step, it differentiates benign nevi from malignant melanomas by pattern analysis. The use of the diagnostic chart for dermoscopy leads to clarification of the diagnostic evidence, and it then becomes easy to plan the following treatment. We should try to train diagnosticians in the accurate use of dermoscopy.[Skin Cancer (Japan) 2009 ; 24 : 463-471]

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Ultrasound sonography, plain X-ray, computed tomography, and magnetic resonance imaging are useful for the diagnosis of subcutaneous disorders, especially soft tissue tumors. Among them, high-frequency ultrasound probe sonography (15-30MHz) is the most useful, because it can show not only subcutaneous lesions but also dermal and epidermal lesions. In this chapter, the basic principle of ultrasound sonogpraphy and the typical and characteristic findings of common cutaneous and subcutaneous skin tumors are summed up.[Skin Cancer (Japan) 2009 ; 24 : 472-480]

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Magnetic Resonance Imaging (MRI) uses a powerful field to align the nuclear magnetization of hydrogen atoms in the water in the body. MR microscopy using a microscopic surface coil is useful for cutaneous and subcutaneous lesions with higher resolution and contrast. MRI signals would change with different tissue biochemical components, and MR microscopy using a microscopic surface coil is useful. The intensity could be affected by a paramagnetic substance, such as melanin or by a tenacious fluid such as mucin deposition. The contrast of MRI would be further changed by weighting T1 or T2 component. The tissue water or a benign tumor would be low in T1-weighted imaging (T1WI), high in T2-weighted imaging (T2WI), while fat tissues or tumors demonstrate high signals both in T1WI and T2WI. Paramagnetic substances or viscous fluid would be high in T1WI, low in T2WI. The low signal images both in T1WI and T2WI could be fibrosis or a malignant tumor.[Skin Cancer (Japan) 2009 ; 24 : 481-488]

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Evaluation of lymph nodes (LNs) is a critical factor for the staging procedures of cutaneous lymphomas (CLs). To choose an LN, which is most possibly involved by CL, for an LN biopsy, we need to assess the LNs using diagnostic imaging in addition to physical examination. PET/CT can screen the whole body, and evaluate CL lesions spatially and qualitatively at the same time. Not only visualized information but also numerical data (standardized uptake value : SUV) can be provided. SUV tends to correlate with the degree of nodal involvement by CLs to some extent. On the other hand, ultrasound is a non-invasive and handy examination that excels in qualitative assessment. Findings that are suspicious of nodal involvement include oval to round shape, diffusely low-echoic cortex, enhanced posterior and posterior marginal echo, and irregularly increased blood supply. PET/CT evaluation, combined with ultrasound, could provide more accurate information for the LN staging of CLs.[Skin Cancer (Japan) 2009 ; 24 : 489-496]

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Malignant melanoma becomes almost incurable as soon as the first distant metastasis appears. The relative lack of active treatment that can overcome metastatic melanoma can explain such a bad prognosis. Although many chemotherapeutic agents have been tested both alone and in combination, very few have yet shown any activity that would significantly improve the poor prognosis of this disease. Both DAC-Tam regimen (a combination of dacarbazine, nimustine, cisplatin, and tamoxifen) and single agent dacarbazine are used for advanced melanoma as systemic chemotherapy in Japan. Although the former regimen achieves a better response rate, neither provides any survival benefit. Dacarbazine has therefore been considered the reference drug for about forty years. In this article, guidelines to evaluate the response to treatment in solid tumors, a grading system for the adverse effects of cancer treatment, and the details of cancer treatment using dacarbazine are described.[Skin Cancer (Japan) 2009 ; 24 : 497-503]

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There is no standard regimen of anticancer chemotherapy for non-melanoma skin cancers, and there has been only weak supportive evidence such as results of small case studies or case reports for chemotherapy of squamous cell carcinoma of the skin and extramammary Paget's disease. Chemotherapy for squamous cell carcinoma has often been used as neoadjuvant chemotherapy for patients with distant metasases and advanced primary lesions. A combination of cisplatin and doxorubicin is used as first-line therapy and CPT-11 is used as second-line therapy in such cases. Recently, for advanced extramammary Paget's disease, oncologists have been using a combination of 5-fluorouracil and cisplatin or docetaxel as a single agent. Large-scale clinical trials of chemotherapy, possibly multi-center studies, and development of new drugs for non-melanoma skin cancers are expected in the future.[Skin Cancer (Japan) 2009 ; 24 : 504-509]

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Cutaneous T-cell lymphomas (CTCLs) in advanced stages and adult T-cell leukemia-lymphoma (ATL) are fatal malignancies. Because therapeutic trials using different combination chemotherapies to improve the poor prognosis of these diseases have been unsatisfactory, an alternative therapeutic approach, hematopoietic stem cell transplantation (HSCT), has been tried. Although autologous HSCT after high-dose chemotherapy has yielded disappointing results, a better therapeutic outcome was obtained with allogeneic HSCT. As allogeneic HSCT induce GVHD by eliciting cellular immune responses of donors against recipient cells, graft-versus-lymphoma effects play a beneficial role.[Skin Cancer (Japan) 2009 ; 24 : 510-515]

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Almost 1000 Children are newly diagnosed with leukemia every year in Japan. Today, childhood leukemia is considered to be a “Curable disease”. Development of chemo-therapeutic strategies combined with supportive therapy have made this improvement of prognosis possible, and the efforts to make even better prognoses are still going on.
In this article, the development and the present status of the therapeutic approach to childhood leukemia are discussed.[Skin Cancer (Japan) 2009 ; 24 : 516-522]

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Metastatic malignant melanoma of the genitourinary tract is rare. We report a case of metastatic malignant melanoma of the bladder. A 79-year-old man had a blackish nodule on his sacrum area. The lesion tended gradually to become enlarged ; he came to our hospital with complaints of hematuria and feelings of dizziness in February 2008. Transurethral resection of three bladder tumors was performed. Histological examination revealed malignant melanoma. Moreover, the tumor of his sacrum area had undergone simple excision, and histological examination revealed malignant melanoma. We diagnosed primary malignant melanoma on his sacrum area, and metastatic malignant melanoma of his bladder mucosa. MRI had already revealed multiple brain metastases and he died 2 months after his first medical examination.[Skin Cancer (Japan) 2009 ; 24 : 541-545]

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