American Journal of Dermatopathology最新文献

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Comparison of the IHC Expression of p16, p53, and MIB-1 in Extragenital Skin Bowenoid Lesions With High- and Low- Chronic Sun Damage.
IF 1.1 4区 医学
American Journal of Dermatopathology Pub Date : 2025-02-06 DOI: 10.1097/DAD.0000000000002939
Pedro Gil-Pallares, Maria E Gil-Pallares, Alba Navarro-Bielsa, Olalla Figueroa-Silva, Laura Taboada-Paz, José M Suárez-Peñaranda
{"title":"Comparison of the IHC Expression of p16, p53, and MIB-1 in Extragenital Skin Bowenoid Lesions With High- and Low- Chronic Sun Damage.","authors":"Pedro Gil-Pallares, Maria E Gil-Pallares, Alba Navarro-Bielsa, Olalla Figueroa-Silva, Laura Taboada-Paz, José M Suárez-Peñaranda","doi":"10.1097/DAD.0000000000002939","DOIUrl":"10.1097/DAD.0000000000002939","url":null,"abstract":"<p><strong>Background: </strong>Bowen disease and bowenoid actinic keratosis are difficult to distinguish due to overlapping histopathology. This study evaluates the role of p16, p53, and MIB-1 staining patterns in differentiating high-chronic sun damage (H-CSD) and low-chronic sun damage bowenoid lesions.</p><p><strong>Methods: </strong>Sixty extragenital in situ squamous cell carcinomas were included. Lesions with elastosis were considered H-CSD. P16, p53, and MIB-1 staining patterns were classified as block, gradient, or focal.</p><p><strong>Results: </strong>Seventy-two percent of lesions were H-CSD. Full-thickness dysplasia was observed in all lesions, and basal layer involvement in 97%. P16 staining was positive in 80%, matching areas of higher atypia, with block pattern more frequent in H-CSD (58% vs. 47%, P = 0.047). P53 was positive in 47%, with block pattern more common in H-CSD (40% vs. 18%, P = 0.02). MIB-1 was positive in all cases. P16 and MIB-1 patterns coincided in 75%, independently of sun exposure.</p><p><strong>Conclusions: </strong>P16 and p53 expression may be less frequent in bowenoid lesions than previously described. Histopathological features like basal layer and adnexal or follicular involvement may not differentiate H-CSD from low-chronic sun damage lesions or Bowen disease from bowenoid actinic keratosis. Variations in p16, p53, and MIB-1 staining could indicate different dysplasia pathways, although further studies are needed to clarify their prognostic significance.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143257261","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}
引用次数: 0
Rhabdomyosarcoma Harboring NRAS or HRAS Mutation Arising in Giant Congenital Melanocytic Nevus: Report of 2 Cases.
IF 1.1 4区 医学
American Journal of Dermatopathology Pub Date : 2025-02-06 DOI: 10.1097/DAD.0000000000002934
Irina Kletskaya, Dmitry Konovalov, Alexander Druy, Ruslan Abasov, Anastasia Salomatina, Margarita Zaitseva, Guzel Kazaryan, Nadezhda Pishchayeva, Irena Belousova, Dmitry V Kazakov
{"title":"Rhabdomyosarcoma Harboring NRAS or HRAS Mutation Arising in Giant Congenital Melanocytic Nevus: Report of 2 Cases.","authors":"Irina Kletskaya, Dmitry Konovalov, Alexander Druy, Ruslan Abasov, Anastasia Salomatina, Margarita Zaitseva, Guzel Kazaryan, Nadezhda Pishchayeva, Irena Belousova, Dmitry V Kazakov","doi":"10.1097/DAD.0000000000002934","DOIUrl":"10.1097/DAD.0000000000002934","url":null,"abstract":"<p><strong>Abstract: </strong>Nonmelanoma malignancies associated with congenital melanocytic nevi (CMN) are extremely rare, with only 12 reported cases of rhabdomyosarcoma (RMS) to date. We present 2 additional cases of RMS arising in giant CMN, with immunohistochemical and molecular biologic investigations. The first case was a 32-year-old woman with a personal history of melanoma in giant CMN who, after successful treatment and long remission, presented with a new 1-cm nodule within the CMN. Microscopically, the atypical areas exhibited a round cell/alveolar morphology with immunoreactivity for desmin and myogenin, and lacked PAX3/7::FOXO1 fusions typical for alveolar RMS on a reverse transcription polymerase chain reaction analysis. An identical NRAS p.Q61R mutation with comparable variant allele frequency (32% and 44%) was identified in both the nevus and the RMS tissue by next-generation sequencing. The second patient was a 5-year-old girl with a rapidly growing, bleeding, ulcerated 3 × 4 cm interscapular mass within a giant CMN that histologically seemed as a proliferation of pleomorphic spindle, polygonal and epithelioid cells with marked pleomorphism immunoreactive for myogenin, muscle-specific actin, and smooth muscle actin. Next-generation sequencing yielded an HRAS p.Q61R mutation with limited variant allele frequency (7%) in the RMS component, while ATRX p.Q2193* variant was detected in the nevus. Our study is apparently the first report of NRAS and HRAS mutations in tumors with RMS phenotype arisen in CMN.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143257308","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}
引用次数: 0
Clinicopathologic Features of PRAME-Positive Common Melanocytic Nevi: A Case-Control Study.
IF 1.1 4区 医学
American Journal of Dermatopathology Pub Date : 2025-02-06 DOI: 10.1097/DAD.0000000000002932
Ronan J Knittel, Nathan T Harvey, Nima Mesbah Ardakani, Blake O'Brien, Stephen Lee, Benjamin A Wood
{"title":"Clinicopathologic Features of PRAME-Positive Common Melanocytic Nevi: A Case-Control Study.","authors":"Ronan J Knittel, Nathan T Harvey, Nima Mesbah Ardakani, Blake O'Brien, Stephen Lee, Benjamin A Wood","doi":"10.1097/DAD.0000000000002932","DOIUrl":"10.1097/DAD.0000000000002932","url":null,"abstract":"<p><strong>Abstract: </strong>Preferentially Expressed Antigen in Melanoma (PRAME) immunohistochemistry has been found to be relatively sensitive and specific for the diagnosis of melanoma, although the detection of PRAME positivity in some melanocytic nevi is a significant limitation. This study was designed to investigate the features of common melanocytic nevi showing PRAME staining, encountered in routine community practice. We reviewed all pathology reports on common melanocytic nevi seen in routine practice for a 1-month period and found that 7.1% of nevi stained with PRAME were considered positive by the original reporting pathologist. A total of 41 PRAME-positive nevi (representing 4.7% of nevi) and a control group of 43 PRAME-negative nevi collected during the same period were identified. The histologic features were reviewed, and the diagnosis and PRAME staining were recorded in a masked fashion by 3 dermatopathologists. Our results suggest that caution is warranted in the interpretation of PRAME in the assessment of small lentiginous melanocytic nevi with a low level of suspicion for melanoma, because these are not infrequently PRAME positive. We found a statistically significant association between the presence of solar elastosis and lentiginous growth pattern, and PRAME status ( P < 0.01). When comparing the original diagnosis with the reviewer's diagnosis, the original diagnosis was severely atypical in 54% of PRAME-positive cases, while only 7% were considered to show severe atypia on review masked to PRAME status ( P < 0.001). There was no such discrepancy among PRAME-negative cases (9% vs. 19%, considered severely atypical). This finding provides evidence of a \"PRAME bias\" in the interpretation of melanocytic lesions with no or mild atypia, whereby such lesions are classified as severely atypical when PRAME is positive, likely with the intention of prompting re-excision.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143257173","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}
引用次数: 0
Relapsing Cutaneous Metastatic Breast Cancer: A Clinical Conundrum. 复发性皮肤转移性乳腺癌:临床难题。
IF 1.1 4区 医学
American Journal of Dermatopathology Pub Date : 2025-02-01 Epub Date: 2024-10-15 DOI: 10.1097/DAD.0000000000002855
Yashika Doshi, Nelry Gonsalves, Bela J Shah
{"title":"Relapsing Cutaneous Metastatic Breast Cancer: A Clinical Conundrum.","authors":"Yashika Doshi, Nelry Gonsalves, Bela J Shah","doi":"10.1097/DAD.0000000000002855","DOIUrl":"10.1097/DAD.0000000000002855","url":null,"abstract":"","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":"162-164"},"PeriodicalIF":1.1,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142480044","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}
引用次数: 0
A Case of Leukemia Cutis (Acute Myeloid Leukemia) With Epidermotropism. 表皮白血病(急性髓性白血病)伴嗜表皮性1例。
IF 1.1 4区 医学
American Journal of Dermatopathology Pub Date : 2025-02-01 Epub Date: 2024-12-31 DOI: 10.1097/DAD.0000000000002917
Brenna M Aran, Juanita Duran, Alejandro A Gru
{"title":"A Case of Leukemia Cutis (Acute Myeloid Leukemia) With Epidermotropism.","authors":"Brenna M Aran, Juanita Duran, Alejandro A Gru","doi":"10.1097/DAD.0000000000002917","DOIUrl":"10.1097/DAD.0000000000002917","url":null,"abstract":"<p><strong>Abstract: </strong>Acute myeloid leukemia is a cancer involving uncontrolled proliferation of hematopoietic cells. Cutaneous involvement is referred to as leukemia cutis (LC). The histopathologic presentation of LC is variable, and may present with perivascular, periadnexal, dermal, or subcutaneous infiltrate. Epidermotropism is notably absent. We report an unusual case of acute myeloid LC with epidermotropism in a 68-year-old man. A punch biopsy revealed a mononuclear myeloblast infiltrate involving the dermis and subcutaneous tissue. The epidermis was focally acanthotic, with several vesicles and atypical epidermotropic cells. Mitotic figures and apoptotic cells were present. Immunohistochemistry showed the blasts to be positive for CD56, CD123, and lysozyme, and weakly positive for CD4 and CD163. Negative immunostaining included CD3, CD20, CD34, TdT, and CD117. Epstein-Barr virus in situ hybridization was negative. A bone marrow biopsy revealed the same myeloblast population with identical phenotype to the skin. The blasts were negative for CD34, CD117, CD3, CD19, CD163, CD68, CD61, myeloperoxidase, pankeratin, E-cadherin, CD4, and transcription factor 4. A diagnosis of acute myeloid leukemia with monocytic differentiation and leukemia cutis was established. These findings suggest that the differential diagnosis for conditions with epidermotropism may be even broader than previously thought.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":"141-144"},"PeriodicalIF":1.1,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142924000","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}
引用次数: 0
Postautologous Hematopoietic Stem Cell Transplantation Disseminated Hypopigmented Keratosis. 自体造血干细胞移植后弥散性低色素角化病。
IF 1.1 4区 医学
American Journal of Dermatopathology Pub Date : 2025-02-01 Epub Date: 2024-12-10 DOI: 10.1097/DAD.0000000000002895
Supasan Sripodok, Nan-Lin Wu, Yu-Hung Wu
{"title":"Postautologous Hematopoietic Stem Cell Transplantation Disseminated Hypopigmented Keratosis.","authors":"Supasan Sripodok, Nan-Lin Wu, Yu-Hung Wu","doi":"10.1097/DAD.0000000000002895","DOIUrl":"10.1097/DAD.0000000000002895","url":null,"abstract":"<p><strong>Abstract: </strong>Disseminated hypopigmented keratosis is a rare distinct clinical entity. This report describes the case of a 19-year-old male patient who developed disseminated hypopigmented keratosis 2 months after autologous hematopoietic stem cell transplantation for pineal choriocarcinoma. The patient displayed numerous tiny whitish or depigmented macules and papules on the chest, back, posterior neck, shoulders, arms, and thighs, closely resembling lichen nitidus. Microscopically, the lesion was orthokeratotic, acanthotic, and flat-bottomed without cytological atypia. Dermal inflammation was minimal. Fontana-Masson, Melan-A, and sex-determining region Y-box transcription factor 10 staining revealed a significant decrease in both basal melanin content and the number of melanocytes. The patient was initially administered a topical agent comprising hydrocortisone and urea, which exhibited minimal improvement. This case report suggests that disseminated hypopigmented keratosis may be a rare cutaneous manifestation of autologous hematopoietic stem cell transplantation. Additional research is necessary to gain a more comprehensive understanding of the pathogenesis and the clinical course of this disease.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":"138-140"},"PeriodicalIF":1.1,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142808325","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}
引用次数: 0
Quantifying Mast Cell and Eosinophil Cellular Density in Skin Biopsy Tissue From Adults With Maculopapular Cutaneous Mastocytosis as Compared With Urticaria and Normal Skin: A Retrospective Histopathologic Study. 与荨麻疹和正常皮肤相比,量化成人大疱性皮肤肥大细胞增多症皮肤活检组织中肥大细胞和嗜酸性粒细胞的密度:一项回顾性组织病理学研究。
IF 1.1 4区 医学
American Journal of Dermatopathology Pub Date : 2025-02-01 Epub Date: 2024-11-05 DOI: 10.1097/DAD.0000000000002878
Anne L King, Carmen M Montagnon, Austin Todd, Shruti Agrawal, Carilyn N Wieland, Julia S Lehman, Emma F Johnson
{"title":"Quantifying Mast Cell and Eosinophil Cellular Density in Skin Biopsy Tissue From Adults With Maculopapular Cutaneous Mastocytosis as Compared With Urticaria and Normal Skin: A Retrospective Histopathologic Study.","authors":"Anne L King, Carmen M Montagnon, Austin Todd, Shruti Agrawal, Carilyn N Wieland, Julia S Lehman, Emma F Johnson","doi":"10.1097/DAD.0000000000002878","DOIUrl":"10.1097/DAD.0000000000002878","url":null,"abstract":"<p><strong>Background: </strong>Maculopapular cutaneous mastocytosis (MPCM) is a rare disorder characterized by a pathologic accumulation of mast cells in the skin, which may or may not be accompanied by systemic mastocytosis. Diagnosis of MPCM on skin biopsy can be challenging because the findings may be subtle. Although mast cell density in MPCM has been reported, data informing a proposed cutoff for diagnosis and diagnostic criteria are limited.</p><p><strong>Methods: </strong>We identified adult patients diagnosed with MPCM and urticarial tissue reaction/chronic urticaria on skin biopsy and compared the mast cell and eosinophil counts per 1 mm 2 in 10 cases each of MPCM, chronic urticaria, and normal skin from routine biopsies. All slides were stained with CD117, and CD117-positive mast cells were counted per 1 mm 2 using digital microscopy. Eosinophils were counted on hematoxylin and eosin-stained slides per 1 mm 2 using digital microscopy.</p><p><strong>Results: </strong>The median number of mast cells per 1 mm 2 was significantly higher in MPCM than in cases of urticaria and normal skin/control tissue (177.3 vs. 26.8 vs. 47.8 mast cell per mm 2 , respectively; P ≤ 0.001). The calculated \"cut point\" for mastocytosis versus chronic urticaria and normal skin was 66 mast cells per 1 mm 2 , whereas the value for controls versus urticaria was 37 mast cells per 1 mm 2 . Eosinophils had similar density in MPCM and urticaria, and their presence was significant in the differentiation of MPCM and urticaria from normal tissue.</p><p><strong>Conclusions: </strong>This study adds to the literature by providing objective mast cell density data to distinguish challenging cases of cutaneous mastocytosis from urticarial reactions and normal skin. Future studies could explore the development of computer-aided estimations of cellular density with more extensive comparison with other inflammatory conditions to translate our findings more readily into clinical practice.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":"105-109"},"PeriodicalIF":1.1,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142584973","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}
引用次数: 0
Primary Cutaneous Methotrexate-Associated T-Cell Lymphoproliferative Disorder in the Setting of Autoimmune Disease: A Case Series and Review of the Literature.
IF 1.1 4区 医学
American Journal of Dermatopathology Pub Date : 2025-02-01 DOI: 10.1097/DAD.0000000000002905
Sarah Nocco, Cynthia Magro
{"title":"Primary Cutaneous Methotrexate-Associated T-Cell Lymphoproliferative Disorder in the Setting of Autoimmune Disease: A Case Series and Review of the Literature.","authors":"Sarah Nocco, Cynthia Magro","doi":"10.1097/DAD.0000000000002905","DOIUrl":"10.1097/DAD.0000000000002905","url":null,"abstract":"<p><strong>Abstract: </strong>Methotrexate (MTX), an antimetabolite targeting certain autoimmune conditions and various hematologic malignancies, has been associated with iatrogenic lymphoproliferative disease (LPD) primarily of B-cell lineage. Less commonly are T-cell neoplasms where primary skin involvement is considered rare. Three cases were encountered in the medical practice of one of the authors. The patients ranged in age from 38 years to 99 years (2 women and 1 man) with 2 having rheumatoid arthritis and 1 having ankylosing spondylitis. All 3 patients received MTX. The cases included subcutaneous peripheral T-cell lymphoma not otherwise specified (NOS) (1 patient), mycosis fungoides (1 patient), and a primary aggressive epidermotropic cytotoxic T-cell lymphoma (1 patient) that proved to be fatal. One patient had spontaneous regression following MTX withdrawal; she later developed a recurrence while off MTX. Two patients died, 1 of unrelated causes and 1 of lymphoma. Seven previously reported cases included subcutaneous panniculitis-like T-cell lymphoma (2 cases), primary cutaneous CD4+ LPD (2 cases), peripheral T-cell lymphoma (NOS) (1 case), anaplastic large cell lymphoma (1 case), and peripheral T-cell lymphoma localized to fat (1 case). Regression without recurrence occurred in 6 of the 7 patients with MTX withdrawal. The patients were on the MTX for an average of 4 years and had a median age of 61 years with a slight dominance of men over women. Three of the 7 cases showed Epstein-Barr encoding region (EBER) positivity while the 3 cases reported in this series were negative. MTX-associated T-cell LPD involves older patients on long-term MTX where EBER positivity is more frequent than extracutaneous MTX-associated T-cell LPD. A spectrum of classic forms of CTCL is seen with subcutaneous involvement representing a significant percentage of cases. Regression with MTX withdrawal occurs although not in every case.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":"47 2","pages":"145-152"},"PeriodicalIF":1.1,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11776889/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143034723","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}
引用次数: 0
Pigmented Syringomatous Carcinoma/Sweat Gland Carcinoma of the Vulva With Melanocytic Colonization: An Uncommon Presentation of a Rare Sweat Gland Neoplasm.
IF 1.1 4区 医学
American Journal of Dermatopathology Pub Date : 2025-02-01 Epub Date: 2024-11-21 DOI: 10.1097/DAD.0000000000002887
Nada Shaker, Sama Alazawi, Omar Sangueza
{"title":"Pigmented Syringomatous Carcinoma/Sweat Gland Carcinoma of the Vulva With Melanocytic Colonization: An Uncommon Presentation of a Rare Sweat Gland Neoplasm.","authors":"Nada Shaker, Sama Alazawi, Omar Sangueza","doi":"10.1097/DAD.0000000000002887","DOIUrl":"https://doi.org/10.1097/DAD.0000000000002887","url":null,"abstract":"<p><strong>Abstract: </strong>Primary vulvar carcinomas are rare and constitute a diverse group of neoplasms. These primary tumors are typically classified based on their presumed tissue of origin or histological characteristics. Among these, carcinomas of sweat gland origin are particularly significant. They closely resemble similar malignancies in nonvulvar skin, including various cutaneous adnexal-type cancers such as apocrine and eccrine adenocarcinomas. Syringomatous carcinoma of the vulva is a rare malignant sweat gland neoplasm known for its infiltrative growth and tendency for local recurrence. Typically, these malignancies manifest as nonulcerated nodules or plaques, primarily in the head and neck region. The occurrence of syringomatous carcinoma in the vulvar region is exceptionally rare. Herein, we present a unique case of a 35-year-old woman with a dark mole measuring 1.5 × 1.0 cm on the vulva. Complete excision was performed to exhibit an infiltrative haphazard proliferation of elongated ductules and tubules, displaying significant cytologic atypia characterized by irregular nuclear contours and variably prominent nucleoli. Extensive melanocytic pigment deposition and stromal fibrosis were also observed. Immunohistochemical staining demonstrated positive expression of epithelial markers, including keratins (AE1/AE3) and epithelial membrane antigen, supporting the diagnosis of syringomatous carcinoma. CK7 and carcinoembryonic antigen were negative, whereas SOX10 and pan melanin highlighted admixed, cytologically bland melanocytes within the epidermis and neoplastic nests. This case represents a highly unusual presentation of syringomatous carcinoma associated with melanocyte colonization. Due to limited data on the optimal management strategies, a multidisciplinary approach involving gynecologic oncologists, dermatopathologists, and radiation oncologists is essential for treatment decisions. Long-term follow-up is crucial, considering the potential for local recurrence and metastatic spread, emphasizing the importance of comprehensive clinical management for favorable patient outcomes of this rare malignancy.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":"47 2","pages":"119-121"},"PeriodicalIF":1.1,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143034722","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}
引用次数: 0
Subcutaneous Sweet Syndrome With Vasculitis Features: Case Report and Review. 具有血管炎特征的皮下甜综合征:病例报告与回顾
IF 1.1 4区 医学
American Journal of Dermatopathology Pub Date : 2025-02-01 Epub Date: 2024-10-15 DOI: 10.1097/DAD.0000000000002845
Jia Tang, Bing Lv
{"title":"Subcutaneous Sweet Syndrome With Vasculitis Features: Case Report and Review.","authors":"Jia Tang, Bing Lv","doi":"10.1097/DAD.0000000000002845","DOIUrl":"10.1097/DAD.0000000000002845","url":null,"abstract":"<p><strong>Background: </strong>Subcutaneous Sweet Syndrome (SSS) is a rare variant of Sweet Syndrome characterized by neutrophilic infiltration of subcutaneous adipose tissue without vasculitis. The presence of vasculitis in SSS is uncommon and poses diagnostic challenges.</p><p><strong>Case presentation: </strong>A 38-year-old female presented with a one-year history of recurrent painful erythematous nodules on her limbs and face. Physical examination revealed asymmetrical erythematous patches and tender subcutaneous nodules with central necrotic eschars on the lower limbs. Laboratory tests were unremarkable except for a mildly elevated erythrocyte sedimentation rate. Histopathological analysis showed significant neutrophilic infiltration within the adipose lobules and vascular walls, along with extravasation of red blood cells, indicating vasculitis. The patient responded promptly to systemic corticosteroids; however, symptoms recurred upon tapering, necessitating ongoing steroid therapy.</p><p><strong>Discussion: </strong>This case underscores the rare occurrence of vasculitis in SSS, expanding the histopathological spectrum of the disease. Literature review suggests that vasculitis in SSS may result from neutrophil-mediated vascular damage rather than immune complex deposition. The recurrent symptoms upon steroid tapering highlight the therapeutic challenges in managing SSS with vasculitis.</p><p><strong>Conclusion: </strong>Recognition of vasculitis in SSS is crucial for accurate diagnosis and effective management. Further research is warranted to elucidate the pathogenesis and develop targeted treatment strategies for SSS with vasculitis.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":"133-137"},"PeriodicalIF":1.1,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142480046","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}
引用次数: 0
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