Journal of Cutaneous Pathology最新文献

{"title":"Novel Histopathologic Features of Extramammary Paget's Disease in Metastatic Primary Cutaneous Apocrine Carcinoma to the Esophagus.","authors":"Dre Barnachea, Maki Yamamoto, Rupali Banker, Dong Ren, Bonnie Lee","doi":"10.1111/cup.14863","DOIUrl":"https://doi.org/10.1111/cup.14863","url":null,"abstract":"<p><p>Primary cutaneous apocrine carcinoma (PCAC) is an exceptionally rare cutaneous malignancy originating from apocrine glands, occurring most commonly in the axilla and anogenital regions. It typically follows a slow-growing clinical course, although aggressive behavior has been documented in select cases. While local recurrence and regional metastasis are not uncommon in PCAC, instances of distant metastases are rare, with only a handful of cases reported, including involvement of the liver, bone, and lung. We present a unique case of PCAC with pagetoid features arising in the groin and metastasizing to the esophagus, a highly unusual presentation not well described in the existing literature. PCAC manifesting as extramammary Paget's disease (EMPD), characterized by single malignant epithelial cells scattered throughout the epidermis, represents a rare and diagnostically challenging variant. Its ability to mimic metastatic carcinomas from various organs necessitates thorough clinical and pathological correlation for accurate diagnosis. This case report aims to illuminate the potentially aggressive behavior of PCAC and emphasizes the need for long-term surveillance and awareness of its less typical presentations.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145000620","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Carcinosarcoma Arising in an Eccrine Spiradenoma and Presenting With Metastasis: Case Report and Literature Review.","authors":"Lisa M Marinelli, Jared M Orrock, Andrew L Folpe","doi":"10.1111/cup.14867","DOIUrl":"https://doi.org/10.1111/cup.14867","url":null,"abstract":"<p><p>Eccrine spiradenomas are benign sweat gland neoplasms that rarely undergo malignant transformation. Carcinosarcoma arising from an eccrine spiradenoma is exceptionally rare. A 41-year-old male presented with a rapidly growing neck/shoulder mass, progressive numbness, spasticity, and weakness. Further workup additionally revealed an epidural mass with spinal cord compression. Both masses were excised and predominantly showed morphologic features of high-grade osteosarcoma, with overtly malignant spindled cells producing lace-like osteoid. However, a single section from the upper back mass contained a roughly 2 mm focus of conventional eccrine spiradenoma, with an adjacent small focus having features of a poorly differentiated non-small cell carcinoma. The final diagnosis was that of a high-grade carcinosarcoma with heterologous osteosarcomatous differentiation, arising from a pre-existing eccrine spiradenoma, with metastasis to the T4-5 epidural region. The patient experienced rapid regrowth of the spinal mass and underwent radiotherapy but had unresectable metastatic disease at 2 months follow-up. We describe what is to our knowledge only the 21st example of carcinosarcoma arising from eccrine spiradenoma, mimicking metastatic osteosarcoma. Awareness of this very rare entity, careful sampling, close microscopic examination, and, in selected cases, ancillary immunohistochemistry are the keys to making this challenging diagnosis.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144955785","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cutaneous FUS::TFCP2-Rearranged Rhabdomyosarcoma Initially Misdiagnosed as ALK-Rearranged Mesenchymal Neoplasm: A Case Report.","authors":"Daigo Shiraishi, Kenji Murata, Junya Shimizu, Yasutaka Murahashi, Taro Sugawara, Shintaro Sugita, Makoto Emori, Atsushi Teramoto","doi":"10.1111/cup.14869","DOIUrl":"10.1111/cup.14869","url":null,"abstract":"<p><p>FUS::TFCP2-rearranged rhabdomyosarcoma is a recently identified malignant neoplasm characterized by immunohistochemical evidence of the co-expression of rhabdomyoblastic markers and ALK. Herein, we report a case of cutaneous spindle cell/sclerosing rhabdomyosarcoma with FUS::TFCP2 fusion that was initially interpreted as an ALK-rearranged mesenchymal neoplasm in a 43-year-old male due to negative desmin expression, a rhabdomyoblastic marker. RNA sequencing was performed to detect ALK fusion counterparts; however, no ALK counterpart fusion was observed, and FUS::TFCP2 fusion was detected. Myogenin was negative, but MyoD1 was positive. Detection of FUS signals using FISH led to the diagnosis of FUS::TFCP2-rearranged rhabdomyosarcoma. In cases of ALK positivity and spindle cell or epithelioid cell morphology, FUS::TFCP2-rearranged rhabdomyosarcoma should be considered in the differential diagnosis using staining for rhabdomyoblastic markers other than desmin.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144955832","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Eccrine Coil Elastosis: A New Dermatopathological Feature Associated With Prediabetes and Diabetes","authors":"Carlos Monteagudo,&nbsp;Miguel Martínez-Rodríguez,&nbsp;Enrique García-Gómez,&nbsp;Silvia Pérez-Debén,&nbsp;Liria Terrádez,&nbsp;Esther Álvarez,&nbsp;José María Martín","doi":"10.1111/cup.14864","DOIUrl":"10.1111/cup.14864","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The prevalence of diabetes mellitus continues to rise. Cutaneous histological manifestations of diabetes include microangiopathy and atherosclerosis. No morphological alterations of sweat glands have been reported. However, eccrine coil elastosis has been observed in basal cell carcinoma biopsies of a patient with metabolic syndrome, a condition that may provoke elastin glycosylation and degradation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We conducted a retrospective study analyzing the associations between eccrine coil elastosis and well-established cardiovascular and metabolic risk factors in 245 patients with skin biopsies. The type (stretching, thickening, waving, multilamellation and fragmentation) and extent of eccrine coil elastosis were assessed. In doubtful cases, confirmation was achieved through lysozyme immunostaining.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The presence of waving, multilamellation, and/or thickening in at least one eccrine coil was independently associated with diabetes or prediabetes. Associations were also found with cardiovascular complications, age, and actinic damage, but not with hypertension, dyslipidemia, smoking, or overweight.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Although eccrine coil elastosis is not restricted to patients with prediabetes and diabetes, it is much more prevalent and appears earlier in patients with these two conditions. Detecting it in skin biopsies obtained for other reasons in patients with an unknown glycemic status may serve as a potential histologic clue warranting further metabolic evaluation.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"52 11","pages":"720-727"},"PeriodicalIF":1.1,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cup.14864","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144955787","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dermatofibrosarcoma Protuberans With a Novel FBN1::PDGFD Fusion: Expanding the Molecular Spectrum.","authors":"Ken-Ichi Yoshida, Yoji Kukita, Satoshi Takenaka, Toshinari Yagi, Keiichiro Honma, Shigeki Kakunaga","doi":"10.1111/cup.14868","DOIUrl":"https://doi.org/10.1111/cup.14868","url":null,"abstract":"","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144955824","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Histopathologic and Immunophenotypic Properties of Primary Cutaneous Large Cell Neuroendocrine Carcinomas","authors":"Myles R. McCrary,&nbsp;Riechelle Garcia,&nbsp;Janice Jiang,&nbsp;Michael B. Morgan","doi":"10.1111/cup.14853","DOIUrl":"10.1111/cup.14853","url":null,"abstract":"<div>\u0000 \u0000 <p>Cutaneous large cell neuroendocrine carcinoma (LCNEC) is a rare and poorly understood malignancy. Here we describe the clinicopathological characteristics of six cutaneous LCNEC case. A retrospective chart and slide review of PCLCNEC cases at a large commercial dermatopathology practice from January 2017 to May 2025 was performed. Patient characteristics, histopathologic features, and immunohistochemical profiles, including Merkel cell polyoma virus large T antigen/MCPyV antigenicity, were summarized. Demographically, PCLCNEC occurred in elderly white patients, presenting as a rapidly progressing nodule in sun-exposed skin. Histologically, the tumors were characterized by predominantly infiltrative growth patterns with varying levels of neuroendocrine architectural and cytologic features, including rosette and trabeculae formation, organoid nesting, and peripheral palisading. All were positive for CK7 and neuroendocrine markers and negative for MCPyV, CK20, and TTF1 expression by immunohistochemistry. Metastasis was identified in one patient, while the remaining five patients had no evidence of disease following wide local excision with a median follow-up period of 25 months. This series contributes valuable insights into the characterization and diagnosis of primary cutaneous LCNEC. In conjunction with previously reported data, we suggest that the morphology and immunophenotype support further inquiry into its potential distinction as a unique cutaneous entity.</p>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"52 11","pages":"696-703"},"PeriodicalIF":1.1,"publicationDate":"2025-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144955800","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"CRTC1::MAML2-Positive Hidradenoma With Sebaceous Differentiation","authors":"Megumi Aoki,&nbsp;Keisuke Goto,&nbsp;Kazuyasu Fujii,&nbsp;Mitsuharu Nomoto,&nbsp;Masamichi Goto,&nbsp;Manami Kajiwara,&nbsp;Toshitaka Nagao,&nbsp;Shigeto Matsushita","doi":"10.1111/cup.14862","DOIUrl":"10.1111/cup.14862","url":null,"abstract":"<div>\u0000 \u0000 <p>Hidradenoma can exhibit several cell types, including clear cells, polyhedral eosinophilic cells, squamoid cells, mucinous cells, oxyphilic (oncocytic) cells, and transitional (intermediate) cells. However, sebocytes have not yet been described in hidradenoma. Here, we present a case of <i>CRTC1::MAML2</i>-positive hidradenoma with sebaceous differentiation. In addition, this tumor had two cell-type layers that were morphologically similar to apocrine glandular cells and myoepithelial cells throughout the lesion. A 66-year-old male presented with a 20 mm nodule on his upper lip with a 3-year history. Histopathological examination revealed a multinodular tumor adherent to the overlying epidermis. No surrounding salivary glands were seen. The tumor consisted of eosinophilic ductoglandular cells surrounded by basaloid cells at the outer periphery. Numerous sebocytes were scattered throughout the tumor. Severe nuclear atypia or mitotic figures were not observed. Immunohistochemical examination showed no expression of α-smooth muscle actin, calponin, and S100 protein in the tumor cells. Adipophilin highlighted scattered sebocytic tumor cells. Immunoexpression of MLH1, PMS2, MSH2, and MSH6 was preserved. <i>MAML2</i> break-apart fluorescence in situ hybridization revealed frequent split signals. Sanger sequencing revealed a <i>CRTC1</i>(e1)<i>::MAML2</i>(e2) fusion. Based on this report, hidradenoma should be included in the differential diagnosis of cutaneous adnexal tumors with sebaceous differentiation other than sebaceous adnexal tumors. In addition, sebocytes should be added to the list of tumor cell types in hidradenoma.</p>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"52 11","pages":"690-695"},"PeriodicalIF":1.1,"publicationDate":"2025-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144955878","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Reaction to Cutaneous Filler Elleva Poly-l-Lactic Acid: Report of Two Cases","authors":"Pedro Carneiro Marinho,&nbsp;Maria Belen Mendoza Macias,&nbsp;Joaquim Mesquita Filho,&nbsp;Luciana França,&nbsp;Maria Auxiliadora Jeunon Sousa,&nbsp;Thiago Jeunon de Sousa Vargas","doi":"10.1111/cup.14857","DOIUrl":"10.1111/cup.14857","url":null,"abstract":"<div>\u0000 \u0000 <p>The global demand for aesthetic procedures has been steadily increasing, driving the development of new techniques and materials to meet this need. However, these procedures are not without risks, including the formation of subcutaneous nodules following injectable treatments. Histopathology plays a crucial role in identifying skin fillers based on their distinct morphological characteristics. In this article, we present two cases of subcutaneous nodules associated with the application of Elleva poly-<span>l</span>-lactic acid, providing a detailed histopathological analysis.</p>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"52 11","pages":"686-689"},"PeriodicalIF":1.1,"publicationDate":"2025-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144955820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnostic Utility of PRAME in Rare Melanoma Mimics: A Comparative Analysis Including GNET, MMNST, Epithelioid MPNST, and MITF-Rearranged Melanocytic Tumors","authors":"Ahmed Shah,&nbsp;Jeremiah F. Molligan,&nbsp;Carina A. Dehner,&nbsp;Jorge Torres-Mora","doi":"10.1111/cup.14849","DOIUrl":"10.1111/cup.14849","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Differentiating metastatic melanoma from histologic mimics such as malignant gastrointestinal neuroectodermal tumor (GNET), malignant melanotic nerve sheath tumor (MMNST), and epithelioid malignant peripheral nerve sheath tumor (EMPNST) poses significant diagnostic challenges due to overlapping morphology and immunophenotypes. PRAME is a novel immunohistochemical marker increasingly used to distinguish melanoma from its mimics, but remains underexplored in these rare tumor types.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>PRAME immunohistochemistry was performed on four GNETs, seven MMNSTs, 10 EMPNSTs, 16 metastatic melanomas (including eight undifferentiated melanomas), and two MITF-rearranged melanocytic tumors. PRAME expression was scored from 0 to 4+ based on the percentage of tumor nuclei showing moderate to strong staining.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>All GNETs, MMNSTs, and MITF-rearranged tumors were PRAME-negative. EMPNSTs showed variable expression: six were negative (0–1), one equivocal (2+), and three positive (3–4+). Fifteen of 16 melanomas were PRAME-positive. PRAME scores differed significantly among tumor types (<i>p</i> = 1.99 × 10⁻⁵). PRAME demonstrated high sensitivity and specificity for distinguishing metastatic melanoma from primary mimickers including GNET and MMNST, but low specificity in EMPNST (71.4%).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>PRAME reliably distinguishes metastatic melanoma from GNET and MMNST, supporting its use in this differential. However, its reduced specificity in EMPNST limits its standalone diagnostic value in this context, emphasizing the need for a multimodal approach.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"52 11","pages":"710-719"},"PeriodicalIF":1.1,"publicationDate":"2025-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144873431","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cutaneous Acanthamoebiasis: Two Cases Highlighting Diverse Histopathologic Findings","authors":"Nadia Siddiqui,&nbsp;Bicong Wu,&nbsp;Lindsay Gunnell,&nbsp;Evan George,&nbsp;Oliver Chang","doi":"10.1111/cup.14854","DOIUrl":"10.1111/cup.14854","url":null,"abstract":"<div>\u0000 \u0000 <p><i>Acanthamoeba</i> is a free-living ameba, known to most commonly cause amebic keratitis in contact lens users and granulomatous amebic encephalitis in immunocompromised patients. Cutaneous Acanthamoebiasis as a single-organ manifestation is less common. We report two cases seen in our department. Case one is a 23-year-old hospitalized patient with systemic lupus erythematosus and pancytopenia who acutely developed nodules on the extremities. Biopsy showed lymphohistiocytic inflammation and focal coagulative necrosis in the subcutaneous fat. Acanthamoeba trophozoites and cysts were apparent in H&amp;E-stained sections and highlighted by PAS and GMS stains. PCR studies were positive for <i>Acanthamoeba</i> species. Case two is an 81-year-old with diffuse large B-cell lymphoma on idelalisib, who presented with a three-week history of ulcerating nodules on the extremities. Biopsy from the left arm exhibited a mixed deep dermal infiltrate with neutrophils and histiocytes while biopsy from the right arm exhibited granulomatous inflammation. Acanthamoeba trophozoites and cysts were apparent in H&amp;E and PAS-stained sections, confirmed to be <i>Acanthamoeba</i> by PCR. Cutaneous Acanthamoebiasis should be considered in biopsies of cutaneous nodules from immunocompromised patients. The histopathologic inflammatory changes are variable and non-specific, while the organisms are easily overlooked due to their resemblance to histiocytes. Familiarity with these features is essential for accurate, timely diagnosis.</p>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"52 11","pages":"681-685"},"PeriodicalIF":1.1,"publicationDate":"2025-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144873430","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}