Merkel cell carcinoma: A systematic review of the demographic and clinical characteristics of 847 cases in Japan.

Merkel cell carcinoma (MCC) is a rare but aggressive neuroendocrine carcinoma of the skin associated with Merkel cell polyomavirus and immunosuppression. Although MCC incidence is rising worldwide, MCC has not been sufficiently investigated in Japan. This study aimed to determine MCC demographics in Japan, including incidence, age, sex, location, spontaneous regression, and pure/combined MCC. Using PubMed and Igaku Chuo Zasshi, 847 MCC cases between 1985 and 2015 were extracted, and the main epidemiological characteristics were described. The mean age of all patients was 77.5 years. Regarding the characterized lesions, 63.0% were located on the head and neck, 5.2% on the trunk, 12.6% on the upper limb, 15.1% on the lower limb, 3.5% on the buttocks, and 0.6% on the genitals. Histopathological information regarding the presence of other malignancies could be retrieved in 611 cases, and a coexisting malignancy, mainly squamous cell carcinoma and Bowen's disease, was present in 14.2%. Subcutaneous MCC was observed in 31 patients with a male : female ratio of 1.07 (16 men/15 women). Nodal lesions with unknown primary tumor location were described in 19 patients with a male : female ratio of 0.9 (nine men/10 women) and a mean age of 77.7 years. Of 640 evaluable cases, spontaneous regression developed in 9.1%. Among those 58 patients, the male : female ratio was 1:2.1 in 56 evaluable cases (18 men/38 women). Merkel cell polyomavirus was assessed in 180 patients, and the virus was detected in 31.1% and not detected in 68.9% of the patients. MCC is a rare disease in Japan, with incidence rates and male : female ratios differing from those in the USA and European countries. Besides, this study reveals the high frequency of subcutaneous MCC and MCC with divergent differentiation patterns and spontaneous regression in Japan compared to other countries.

Usefulness of ulceration and hyperkeratosis as clinical predictors of Merkel cell polyomavirus-negative and combined Merkel cell carcinoma: A retrospective study.

Merkel cell carcinoma is a rare neuroendocrine carcinoma of the skin that is associated with Merkel cell polyomavirus (MCPyV). The clinical appearance and demographic characteristics of this tumor have been described using the mnemonic AEIOU: asymptomatic, expanding rapidly, immune suppression, older than 50 years, and ultraviolet-exposed fair skin. In addition, MCC can be categorized based on morphology as pure MCC or combined MCC that exhibits neuroendocrine and other phenotypic elements. There is limited information regarding the clinical characteristics and prognosis of combined MCC. This retrospective study aimed to identify factors, such as ulceration or hyperkeratosis, that could predict MCPyV status and morphological variants. Twenty patients with MCC were divided into groups based on MCPyV status and morphology: MCPyV-positive or MCPyV-negative MCC and pure or combined MCC. The patients' MCPyV status was immunohistochemically determined using the CM2B4 antibody to the MCPyV large T-antigen. The patients' clinicopathological characteristics were evaluated to identify predictors of MCPyV-negative MCC and combined MCC. The presence of ulceration/hyperkeratosis predicted the presence of MCPyV-negative MCC (80% of cases) and combined MCC (50% of cases). None of the 10 patients with MCPyV-positive MCC had ulceration/hyperkeratosis. The clinical presence of ulceration/hyperkeratosis may help guide the diagnosis of MCPyV-negative MCC and combined MCC.

Atrophic dermatofibrosarcoma protuberans with diffuse eosinophilic infiltrate.

Atrophic dermatofibrosarcoma protuberans (atrophic DFSP) is a variant of dermatofibrosarcoma protuberans (DFSP), and is clinically characterized by depressed lesions. We report a patient with a typical atrophic DFSP lesion with marked eosinophilic infiltration. The patient was a 55-year-old woman with a dark-red, depressed lesion in the epigastric region. Histopathological examination of the lesion showed proliferation of fibroblast-like cells in a storiform pattern in the dermis and subcutaneous tissue. Immunohistochemical staining of tumor cells was positive for CD34. The lesion was histopathologically typical of DFSP, but no elevated lesion was clinically observed. Thus, a diagnosis of atrophic DFSP was made. Moreover, this tumor tissue exhibited marked eosinophilic infiltration. To our knowledge, they are no reports of eosinophilic infiltration in DFSP tissue. Therefore, this seems to be an extremely rare case of DFSP.

Disseminated Ochroconis gallopavum infection in a chronic lymphocytic leukemia: a case report and review of the literature on hematological malignancies.

Disseminated fungal infection is an important cause of morbidity and mortality among patients with hematological malignancies. Ochroconis gallopavum is a dematiaceous and thermotolerant fungus that causes opportunistic infections in immunocompromised hosts. About only 30 cases of this organism infection have been reported worldwide. We report a disseminated Ochroconis gallopavum infection in a B-cell chronic lymphocytic leukemia patient. In spite of intensive anti-fungal treatment, no improvement in the clinical condition was observed and the patient died 4 months after diagnosis of the infection. Ochroconis gallopavum infection is a potentially fatal disease in hematological malignancies.

Cutaneous sporotrichosis with refractory and reinfectious lesions in a healthy female.

We report a 71-year-old Japanese healthy female with an unusual clinical course of sporotrichosis; she developed infectious lesions on the face and the left upper limb at different periods. The initial lesion appeared on her right cheek and nose in 1992. Histopathologic and fungal examinations comfirmed the diagnosis of sporotrichosis. She was treated with oral potassium iodide and/or itraconazole and topical heat therapy. She had three recurrences and her facial plaque, which was resistant to several conventional treatments, was eventually excised and then grafted in 1996. After the surgical procedure, oral medication was continued for two months. In 2001, new lesions appeared on her left hand and forearm, which were anatomically different from the former lesions. Based on histopathologic and mycological findings, we confirmed reinfection with sporotrichosis. She was again treated with potassium iodide. The cutaneous lesions completely resolved after 26 weeks without surgical treatment.