Language：Japanese   Publisher：公益社団法人 日本皮膚科学会  

DOI： 10.14924/dermatol.133.2519

CiNii Research

Language：English   Publisher：日本ヒト細胞学会  

新たに樹立された原発性乳房外Paget病(EMPD)細胞株KS-EMPD-1について述べた。当院で原発性EMPDと診断された86歳の日本人男性の左鼠径部腫瘍を外科切除し、腫瘍組織を細切後培養してKS-EMPD-1細胞を樹立した。KS-EMPD-1細胞は1年以上維持することが可能で、倍加時間は31.2±0.471時間であった。KS-EMPD-1細胞は持続的な増殖、スフェロイド形成及び浸潤性を示し、ショートタンデムリピート解析、全エクソームシーケンシング及び免疫組織染色により元の腫瘍と同じであった。ウェスタンブロッティングにて、KS-EMPD-1細胞はHER2、NECTIN4及びTROP2を発現していた。また、抗癌剤感受性試験においてKS-EMPD-1細胞はドセタキセルとパクリタキセルに対して高い感受性を示した。

Language：English   Publisher：Human Cell  

Extramammary Paget’s disease (EMPD) is a rare skin cancer that mainly occurs in apocrine sweat gland-rich areas in elderly people. The prognosis of metastatic EMPD is unfavorable because of the lack of fully effective systemic therapies. However, the difficulty in establishing a model of EMPD has hampered basic research for exploring its pathogenesis and optimal treatments. Here, we established for the first time an EMPD cell line (named KS-EMPD-1) from a primary tumor on the left inguinal region of an 86-year-old Japanese male. The cells were successfully maintained for more than 1 year, with a doubling time of 31.2 ± 0.471 h. KS-EMPD-1 exhibited constant growth, spheroid formation, and invasiveness, and was confirmed to be identical to the original tumor by short tandem repeat analyses, whole exome sequencing, and immunohistochemistry (CK7+CK20−GCDFP15+). Western blotting of the cells revealed the protein expression of HER2, NECTIN4, and TROP2, which have recently attracted attention as potential therapeutic targets for EMPD. KS-EMPD-1 was highly sensitive to docetaxel and paclitaxel on chemosensitivity test. The KS-EMPD-1 cell line is a promising resource for basic and preclinical research on EMPD to better define the tumor characteristics and treatment strategy of this rare cancer.

DOI： 10.1007/s13577-023-00951-1

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PubMed

Language：English   Publisher：Journal of Clinical Pathology  

Aims Dermatofibroma/fibrous histiocytoma (DF/FH) is a common cutaneous mesenchymal neoplasm exhibiting benign biological behaviour. However, the immunohistochemical utility of erythroblast transformation-specific-related gene (ERG) for diagnosing DF remains unknown. The authors reviewed the immunohistochemical status of ERG in different subtypes of DF and in its differential diagnoses. Methods Overall, 97 cases of ordinary DF/FH, 6 cases of aneurysmal FH, 10 cases of cellular FH, 5 cases of angiomatoid FH, 2 cases of epithelioid FH, 64 cases of dermatofibrosarcoma protuberans (DFSP) and 52 cases of fibrous scar were retrieved. As the other histological types of cutaneous neoplasms, 6 cases of myxofibrosarcoma, 4 cases of undifferentiated pleomorphic sarcoma, 11 cases of atypical fibroxanthoma, 19 cases of malignant melanoma, 20 cases of nevocellular nevus, 20 cases of neurofibroma, 19 cases of schwannoma, 8 cases of angioleiomyoma and 1 case of pilar leiomyoma were included. Results Immunohistochemical positivity for ERG was demonstrated in 87 of 97 cases (89.6%) of ordinary DF/FH, 7 of 10 cases (70%) of cellular FH, 3 of 6 cases (50%) of aneurysmal FH, 1 of 5 cases (20%) of angiomatoid FH and 1 of 52 cases (0.1%) of fibrous scar. All cases of DFSP, epithelioid FH and other types of cutaneous neoplasms included in the current investigation were negative for ERG. The intensity of ERG immunohistochemical staining in spindle-shaped cells appeared weaker than that in endothelial cells. Conclusions DF/FH was frequently positive for ERG immunostaining. ERG immunostaining may thus be useful to distinguish DF/FH from DFSP.

DOI： 10.1136/jclinpath-2022-208158

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PubMed

Language：English   Publisher：Virchows Archiv  

Angiosarcoma is a malignant vascular endothelial neoplasm with various histological patterns. Despite its highly malignant potential, histological prognostic prediction has not been adopted for angiosarcoma. This study aimed to establish a method of predicting the prognosis of primary angiosarcoma. Formalin-fixed, paraffin-embedded samples from 104 primary angiosarcomas were prepared. All the cases were reviewed based on histological examinations with H&E staining. Because the French Fédération Nationale des Centres de Lutte Contre Le Cancer system (FNCLCC) is not adopted for angiosarcoma, we experimentally established a modified version of FNCLCC. Immunohistochemical staining for ERG, CD31, CD34, D2-40, HHV-8, p16, C-MYC, and p53 was performed. Fluorescence in situ hybridization (FISH) was performed for 31 cases to assay c-MYC gene amplification. Multivariate analysis revealed that age (> 70 years old) (p = 0.0011), non-cutaneous angiosarcoma (p = 0.0265), metastasis on diagnosis (p < 0.0001), size ≥ 5 cm (p = 0.0388), no taxane chemotherapy (p = 0.0388), strong nuclear atypia (p = 0.0087), and the presence of luminal structure in ≥ 50% of the tumor volume (p = 0.0009) were independent poor prognostic factors. Among angiosarcomas with luminal formation, mFNCLCC scores were significantly correlated with a poorer prognosis. The overexpression of p16 was associated with less luminal formation (p = 0.0192). Immunohistochemical analysis of C-MYC showed a moderate level of concordance with FISH (Kappa value = 0.45). This study suggested that luminal formation and nuclear atypia may be poor histological prognostic factors of angiosarcoma and that mFNCLCC would be useful for predicting the prognosis of angiosarcoma with luminal formation.

DOI： 10.1007/s00428-023-03572-z

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PubMed

Language：Japanese   Publisher：Western Division of Japanese Dermatological Association  

DOI： 10.2336/nishinihonhifu.85.124

Scopus

CiNii Research

Language：Japanese   Publisher：日本皮膚科学会-西部支部  

61歳,女性。明らかな外傷歴はなかったが,初診の約4年前に右足底に褐色斑が出現した。褐色斑は徐々に増大したため,前医を受診し,生検でBowen病と診断された。切除を勧められたが放置していた。当院を紹介され受診した時,右足底の土踏まず部に7×6mmの褐色結節を認めた。前医での生検標本では,表皮全層に異型ケラチノサイトが増殖しており,個細胞角化やclumping cellを認めた。3mmマージンで全切除し,全層植皮術を行った。パラフィン包埋切片よりDNAを抽出し,ヒト乳頭腫ウイルス(human papillomavirus:HPV)の検索を行ったところ,粘膜/粘膜・皮膚型,ハイリスク型であるHPV31型のDNAが検出された。足底発症のBowen病は極めて稀であるが,しばしば粘膜/粘膜・皮膚型のHPVが検出された症例が報告されている。しかし,HPV31型が検出された足底Bowen病は,調べ得た限り国内外での報告はこれまでになく,自験例が第1例目となる。足底の黒褐色病変を認めたときは,Bowen病も鑑別の一つとして考慮し対応すべきであり,外陰部・手指だけでなく,足底のBowen病もHPVが発症に関与している可能性の高い病変であると考えられる。(著者抄録)

Web of Science

Publisher：協和企画  

DOI： 10.24733/pd.0000002993

CiNii Research

Language：Japanese   Publisher：(株)協和企画  

＜文献概要＞症例のポイント　・ステロイド外用薬や光線療法に抵抗性で,デュピルマブ投与後も痒みが改善しなかったアトピー性皮膚炎が,デルゴシチニブ外用により痒みが速やかに改善した例を経験した.・デルゴシチニブはJAKファミリーのキナーゼすべてを阻害することで,デュピルマブが直接は抑えられないIL-31やTSLP由来の痒みも抑えることができる.

Language：Japanese   Publisher：Western Division of Japanese Dermatological Association  

DOI： 10.2336/nishinihonhifu.84.7

CiNii Research

Language：English   Publisher：Journal of Clinical Medicine  

Sebaceous carcinoma and sweat gland carcinoma (malignant tumors with apocrine and eccrine differentiation) are rare malignant skin adnexal tumors that differentiate toward sebaceous gland and eccrine and apocrine glands, respectively. Owing to the rarity of these carcinomas, standard treatments for advanced disease have not been established. Because the prognosis of patients with systemic metastasis is poor, a new treatment for these diseases is eagerly desired. Trophoblast cell surface antigen 2 (TROP2) and sacituzumab govitecan, an antibody–drug conjugate of TROP2, have attracted attention in the treatment of various solid tumors. In the current study, we immunohistochemically investigated TROP2 expression in 14 sebaceous carcinoma and 18 sweat gland carcinoma samples and found strong and relatively homogeneous TROP2 staining in both cancer types. The mean Histoscore, a semi-quantitative scoring ranging from 0 (negative) to 300, was 265.5 in sebaceous carcinoma and 260.0 in sweat gland carcinoma. These observations directly suggest that both sebaceous carcinoma and sweat gland carcinoma could be potentially treated with TROP2-targeted antibody–drug conjugates such as sacituzumab govitecan.

DOI： 10.3390/jcm11030607

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PubMed

Language：Japanese  

Language：Japanese  

Language：English