An 86-year-old woman noted a subcutaneous mass in the left axilla 2 months before presentation. A biopsy of an enlarged axillary lymph node showed proliferation of CK20-positive basophilic small tumor cells. Computed tomography of the chest and abdomen detected multiple lymph nodes in the left axilla alone ; other than in the left axilla, no lesions were observed on fluorodeoxyglucose-positron emission tomography. We diagnosed lymph node metastasis in Merkel cell carcinoma without known cutaneous primary. The patient underwent axillary lymph node dissection and received postoperative radiation therapy. As of 13 months after surgery, no recurrence or metastasis has occurred.[Skin Cancer (Japan) 2017 ; 32 : 245-248]

Bexarotene is a synthetic retinoic acid agent, which selectively activates retinoid X receptors. It was approved in Japan in April 2016 as a treatment option for cutaneous T cell lymphoma. Hyperlipidemia and hypothyroidism are adverse effects associated with its use and titrating the induction dose is difficult. We administrated the drug to 6 patients diagnosed with cutaneous T cell lymphoma. The clinical effects observed were : 3 patients showed a partial response, 2 showed progression, and 1 showed no change in disease status. We discussed the clinical and the possible adverse effects associated with the use of this agent.[Skin Cancer (Japan) 2017 ; 32 : 249-255]

A 78-year-old woman was diagnosed with mycosis fungoides (MF) stage IB (T2bN0M0B0). Her disease state had been maintained for 4 years with the use of narrow-band ultraviolet B therapy ; however, she was observed to have developed erythroderma. Topical psoralen and ultraviolet A (PUVA) therapy failed to control her disease adequately, and concomitant administration of interferon (IFN) gamma-1a was initiated with the topical PUVA therapy. Her skin disease improved significantly after 4 weeks of therapy, and the improvement was maintained over 5 months. Erythematous plaques showed a tendency to spread over her body to involve 40% of her body surface area (BSA) after 27 weeks of the combination therapy. She was thereafter managed with 3 cycles of low-dose etoposide, following which the areas of erythematous plaques were reduced and involved only 2% of her BSA. Combination therapy using topical PUVA, IFN gamma-1a, and low-dose etoposide was effective to treat erythrodermic stage MF. The present case suggests that combination therapy might be a useful treatment option for advanced-stage MF.[Skin Cancer (Japan) 2017 ; 32 : 256-260]

We retrospectively investigated the post-surgical recurrence of melanoma treated at Niigata Cancer Center Hospital between 1989 and 2011. The recurrence rate was 36% (stage I, 14% ; stage II, 34% ; stage III, 66%) and the median recurrence-free survival time was 12 months. The sites of initial recurrence were local (8%), in-transit (27%), regional lymph nodes (35%), and distant organs (30%). Initial recurrences were detected by patients (42%) and doctors (53%). There are no definite follow-up recommendations for melanoma thus far. However, recently approved new agents, including immune checkpoint inhibitors and molecular-targeted agents, have proven to be effective for patients exhibiting low tumor burden and few metastatic organ sites. We need to reconsider the treatment strategy for melanoma, including post-surgical follow-up schedules to facilitate the earliest possible detection of recurrences.[Skin Cancer (Japan) 2017 ; 32 : 261-265]

To evaluate the significance of sentinel lymph node biopsy (SNB) in patients with cutaneous squamous cell carcinoma (cSCC), we retrospectively studied 110 patients who had been pathologically diagnosed with cSCC from April 2006 to December 2011. SNB was performed in 35 patients, 3 of whom showed positive results for metastasis. SNB showed 100% sensitivity in identifying sentinel lymph nodes (SNs), with 8.6% positive results and 2.9% false-negative results.

Compared to patients who did not undergo SNB, those who underwent SNB were younger and had larger tumors. The five-year survival rate of the SNB group was 100%, which was significantly higher than that of the non-SNB group.[Skin Cancer (Japan) 2017 ; 32 : 266-271]

Herein, we report a rare case of quadruple extramammary Paget's disease (EMPD) affecting four parts of the body (the genitals, both axillae, and the umbilical region). Four lesions were present in the genital area (on the suprapubic area, scrotum, penile shaft, and glans penis). An 88-year-old Japanese man presented with a 10-year history of a skin rash on the right inguinal area. A physical examination revealed a large tumor with a papillomatous appearance (size : 17×14 cm) in the suprapubic region, a slightly elevated plaque (size : 6.5×4.5 cm) on the scrotum, an erythematous tumor (size : 5×5 cm) on the penile shaft, and depigmentation on the glans penis. Furthermore, erythematous patches were observed on both axillae and the umbilicus. Biopsy examinations revealed Paget's cells in all lesions, except in the glans penis lesion. Histological examinations of the excised lesions did not reveal any invasive findings. After 1 year and 4 months, an erythematous tumor developed on the glans penis, where depigmentation had been previously observed, and he was diagnosed with EMPD. The patient subsequently developed rectal carcinoma 4 years later, and died from respiratory failure after the operation.[Skin Cancer (Japan) 2017 ; 32 : 272-278]

A 72-year-old Japanese man with neurofibromatosis type 1 presented with a subcutaneous nodule on the right inguinal region two months ago. Histopathological findings showed increased numbers of atypical S100 protein-positive spindle cells. Positron emission tomography-computed tomography revealed 18F-fluorodeoxyglucose (FDG) accumulation in the right inguinal lymph nodes. The patient was referred to us under the tentative diagnosis of a malignant peripheral nerve sheath tumor. Physical examination revealed not only numerous lesions of neurofibroma and café-au-lait spots on his trunk and extremities, but also an ulcerative lesion with black and depigmented macules on his right big toe. The patient underwent surgical resection and total lymph node dissection. Based on the histopathological findings, the patient was diagnosed with metastatic malignant melanoma.[Skin Cancer (Japan) 2017 ; 32 : 279-284]

A 48－year－old man presented with a tumor measuring 8 × 8 mm on the right forehead, which he noted 4 months before visiting our department. Histopathological findings indicated a cystic tumor. The tumor cells had abundant eosinophilic cytoplasm and prominent round vesicular nuclei. On immunohistochemical analysis, the cells were positive for S－100 protein, Melan－A, and HMB－45. The findings were diagnostic of rhabdoid melanoma. No other lesions were found on additional examinations, including positron emission tomography－computed tomography. To our knowledge, this is the 13th case of primary cutaneous rhabdoid melanoma reported to date.[Skin Cancer (Japan) 2017 ; 32 : 285－289]

The English in this document has been checked by a least two professional editors, both native speakers of English. For a certificate, please see :

http://www.textcheck.com/certificate/bqD5nc

A 77-year-old woman presented with a one-year history of a slowly growing pale pink nodule on her left shoulder. Histopathological examination showed a well-defined basophilic nodular area in the dermis. The nodular area was composed of spindle-shaped and round cells with moderate cellular atypia, which had some mitoses and distinct nucleoli in addition to anisokaryosis. Immunohistochemical staining revealed that these cells were strongly positive for S100 proteins, partially positive for HMB-45, and negative for Melan-A. The lesion affected only the dermis, and there was no primary focus other than the shoulder. Therefore, a diagnosis of primary dermal melanoma was established. Additional wide excision with sentinel node biopsy revealed no remaining tumor cells or sentinel node metastasis. No recurrence or metastasis was observed at one-year follow-up. Primary dermal melanoma cannot be completely distinguished from metastatic melanoma. Therefore, long-term follow-up is required despite its good prognosis.[Skin Cancer (Japan) 2017 ; 32 : 290-294]

A retrospective study assessing the clinical and pathological characteristics of 48 cases of metastatic skin tumors, including 4 cases with Sister Mary Joseph nodules (SMJNs), was performed on patients who visited our hospital over the last 17 years. Among the 48 cases studied, the pancreas (5 cases) was the second frequent primary site after the lungs (11 cases). In addition, 3 of the 5 metastatic pancreatic cancer cases presented clinical manifestations of SMJNs. In the present study, 3 (6.3%) of the 48 cases showed skin involvement as an initial clinical sign of visceral cancer, whereas 2 (50%) of 4 cases showed initial clinical signs of pancreatic cancer. Since there are few reported cases of SMJNs manifestations, its incidence rate is not rare compared with that of other visceral origins if the body’s surface area is considered. However, detecting SMJNs may play an important clinical role in recognizing the presence of silent visceral cancers including those of pancreatic origin.[Skin Cancer (Japan) 2017 ; 32 : 295-301]