Kumiko Kimura

Metastatic Cutaneous Plasmacytoma: A Case Report Associated with IgA λ Multiple Myeloma and a Review of the Literature of Metastatic Cutaneous Plasmacytomas Associated with Multiple Myeloma and Primary Cutaneous Plasmacytomas

We present the case of a 67‐year‐old Japanese woman with immunoglobulin A lambda (IgA λ) multiple myeloma (MM). She had firm nodular cutaneous lesions on the trunk and scalp without adjacent bone involvement. The patient was diagnosed as having IgA λ MM of stage IIIA with 52% plasmacytosis in the bone marrow six months before the appearance of the cutaneous lesions. The abnormal plasma cells showed moderate to marked dysplasia in both the bone marrow and skin lesions. The abnormal plasma cells in the bone marrow exhibited abnormal karyotypes: 41, XX, der (1) t (1p; 1q), −4, −10, −14, −16, −17, 17p+, that differed from the “unfavorable” karyotype reported previously.

Penile hybrid verrucous-squamous carcinoma associated with a superficial inguinal lymph node metastasis.

A 62-year-old Japanese man who presented with penile carcinoma is reported. The initial exophytic neoplasm excised from the coronal sulcus and prepuce on the abdominal side of the penis was diagnosed histologically as verrucous carcinoma. Twenty-six months after the primary operation, an epithelial neoplasm recurred within the scar of the primary operation. The neoplasm histologically showed verrucous carcinoma and multiple invasive foci of conventional squamous cell carcinoma in the advancing edge of the tumor, as such representing a hybrid verrucous-squamous carcinoma. A lymph node metastasis in the left superficial inguinal lymph node occurred 4 months after the second operation. A total bilateral inguinal lymphadenectomy was performed and revealed no other lymph node metastases. The patient is alive without local recurrence or evidence of metastases in pelvic lymph nodes or visceral organs 2 years after the resection of the hybrid verrucous-squamous carcinoma. The initial verrucous carcinoma, recurrent hybrid verrucous-squamous carcinoma, and metastatic lymph node were negative for human papillomavirus DNA type 6, 11, 16, 18, and 33 sequences by dot blot hybridization of polymerase chain reaction products. The characteristics of hybrid verrucous-squamous carcinoma and importance of lymph node metastasis in penile carcinoma are discussed.

Ferguson smith type multiple keratoacanthomas and a keratoacanthoma centrifugum marginatum in a woman from Japan

We report a case of multiple keratoacanthomas on the sun-exposed skin of a 37-year-old woman from Japan. She had experienced 4 similar episodes of evolution and involution of multiple keratoacanthomas during a period of 10 years since she was 27 years old. She was given the diagnosis of Ferguson Smith type keratoacanthoma. This is the seventh Japanese case of Ferguson Smith type keratoacanthoma described in detail in the literature. In addition, the patient was found to have an annular, coral reef-like eruption on the front of her neck, which was diagnosed as a keratoacanthoma centrifugum marginatum. A combination of different variants of keratoacanthoma in 1 patient is uncommon, and only 2 patients with the same combination of lesions, as that seen in our patient, have been reported. Our patient was treated by a relatively low dose (0.5 mg/kg body weight) of etretinate. Both variants of keratoacanthoma showed good response to the treatment. Effectiveness of etretinate for treating keratoacanthoma centrifugum marginatum has only been reported in a few cases. Our patient had no relapse during a period of 15 months after cessation of etretinate treatment.

Neutrophil-rich anaplastic large cell lymphoma presenting in the skin.

A neutrophil-rich anaplastic large cell lymphoma (ALCL) presented in the skin of a 47-year-old Japanese woman. The solitary cutaneous lesion was an eroded, 10-mm, dome-shaped nodule involving the skin of her left upper arm. Histologically, it showed a proliferation of pleomorphic, anaplastic, large tumor cells with nuclei of various shapes, including embryo-shaped, Reed-Sternberg cell-like binucleated, and wreath-shaped multiple nuclei, in the dermis and subcutaneous tissues. There was an admixture of neutrophils ranging from about 30% to more than 50% per field. Immunophenotypically, the neoplastic cells were positive for CD30, CD4, leukocyte common antigen, anaplastic lymphoma kinase-1, epithelial membrane antigen, and granzyme B. She had no peripheral neutrophilia. The lesson was excised, and the site on the left upper arm was irradiated. Six and a half months after diagnosis, however, swelling of a left axillary lymph node appeared; it also showed a proliferation of anaplastic large tumor cells admixed with numerous neutrophils ranging from about 25% to more than 60% per field. Southern blot analysis of T-cell receptor gene rearrangement revealed a clonal band. The patient was treated with six courses of cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy with complete remission. Seventeen cases of neutrophil-rich ALCL arising in the skin, lymph node, muscle, testis, and skull bone were reviewed. This form may possibly be induced by trauma or irritation of conventional ALCL, although the true mechanism for the infiltration of neutrophils is still unclear.

Giant dermatofibroma: a rare variant of dermatofibroma preferentially developing on the lower limbs

A 20-year-old man presented to our outpatient clinic with a 3-year history of a slowly growing nodule on his right leg. Physical examination revealed a brown, firm nodule, 50 mm in diameter, which was immobile owing to the adhesion to the subcutaneous tissue (Fig. 1a). The surface of the nodule was moderately hyperkeratotic and partly covered with brown crusts. An initial incisional biopsy demonstrated an irregularly arranged dense proliferation of fibroblast-like cells throughout the dermis and superficial subcutaneous tissue. Magnetic resonance imaging demonstrated invasion close to the fascia of the anterior tibial muscle (Fig. 1b). A simple surgical excision was made for diagnosis and treatment. Histopathological examination revealed a relatively sparse proliferation of fibroblast-like tumour cells without nuclear atypia throughout the entire dermis (Fig. 2a). The overlying epidermis demonstrated moderate hyperkeratosis (Fig. 2b). The tumour cells were loosely arranged within the mature collagen bundles (Fig. 2c) and partially extended into the superficial subcutaneous tissue along the fibrous septa (Fig. 2d). Immunohistochemical examination demonstrated a lack of CD34 expression in the proliferating tumour cells. From the histopathological and immunohistochemical features, a diagnosis of giant dermatofibroma (DF) was made. DF is a benign cutaneous tumour, generally <20 mm in diameter. However, these tumours sometimes enlarge and may be misdiagnosed as malignant tumours, such as dermatofibrosarcoma protuberans (DFSP). Giant DF is a clinical variant of DF, designated by Danckaert and Karassik in 1975 and characterized by its unusually large size. Although there is no clear definition of the size for the diagnosis of giant DF, Requena et al. used a size larger than 35 mm for the diagnosis of giant DF in their study. In previous reports, the size of the tumour ranged from 35 to 120 mm, and almost all the lesions were located on the lower limbs. No recurrence after surgical excision has been reported to date. Distinguishing giant DF from malignant fibrous tumours, especially DFSP, may be difficult, owing to its unusual clinical appearance. Although histopathological examination reveals conventional features of DF in most cases, an incisional biopsy specimen from the tumour may demonstrate architectural features similar to DFSP. In order to distinguish between the two entities of giant DF and DFSP, several reports have suggested that immunohistochemical analysis of CD34 and factor XIIIa expression is useful. However, even these methods could fail to lead to a diagnosis because there may be staining variability among different cases. Focal CD34 reactivity may be demonstrated in some DF cases, whereas some DFSPs uncharacteristically express factor XIIIa, but not CD34. Recently, Cribier et al. reported that immunohistochemical staining of stromelysin 3 (ST3) may be useful for the distinction in such problematic cases. In their study, none of the 40 DFSP cases expressed ST3, while all 40 DF cases, including 10 giant DFs, expressed ST3. In our case, initial incisional biopsy was insufficient to permit a definite diagnosis to be made. Careful histopathological re-evaluation of the surgical specimens revealed that most of the tumour showed the typical histopathological features of DF. In addition, the negative result of immunohistochemical staining with antiCD34 supported the diagnosis of giant DF. We suggest that a thorough histopathological examination of large tissue samples from the tumour, together with immunohistochemical staining, is recommended for the correct diagnosis of giant DF. CP D

A novel A97P amino acid substitution in α‐galactosidase A leads to a classical Fabry disease with cardiac manifestations

Background Fabry disease results from a genetic deficiency of α‐galactosidase A (GLA) activity. Phenotype–genotype correlations in this condition have not as yet been fully elucidated.

Pedunculated Melanoma with Pulmonary and Bony Metastases

We report a patient with pedunculated melanoma. Rounded and epithelioid‐type melanoma cells had proliferated in the epidermis and massively into the deep dermis in the pedunculated nodule. At the base of the pedicle, lengtiginous‐type melanoma cells formed nests of various sizes in the epidermis extending ten or more rete ridges beyond the site of invasion. Thus, this case shows that a pedunculated melanoma is not necessarily a variant of nodular melanoma. Six years after the primary operation, late recurrence was detected in regional lymph nodes, and nine years after the initial operation, the patient was found to have three large metastatic nodules, up to 8.5 cm in longest diameter, in her lung and bone. Although she died six months after the metastasectomy, prompt surgical excision of the primary tumor and metastasectomy can provide longer survival.