Pub Date : 2024-06-06DOI: 10.1097/dad.0000000000002743
Nada Shaker, H. Blankenship, Vaishali Masatkar, Shuo Niu, Omar P. Sangueza
Primary cutaneous gamma/delta T-cell lymphoma (PCGD-TCL) is a rare yet highly aggressive subtype of primary cutaneous lymphoma. Characterized by its challenging diagnosis and poor prognosis, PCGD-TCL presents unique clinical and histopathological features that distinguish it from other primary cutaneous lymphoma subtypes. Here, we report the case of a 75-year-old man who initially presented with multiple erythematous indurated plaques over his back and bilateral lower extremities. The initial biopsy suggested primary cutaneous T-cell lymphoma (PCTCL) with a CD30-negative phenotype. However, within a 2-month interval, the disease progressed rapidly, manifesting as extensive skin involvement across the chest and upper extremities. A repeat skin biopsy was performed, revealing dermal atypical lymphocytes without epidermotropism. Immunohistochemical analysis demonstrated positivity for CD3, CD5, and CD4, as well as T-cell receptor delta (TCR delta) expression, along with the loss of CD8 and CD30 expression. These findings were consistent with a diagnosis of PCGD-TCL. Despite therapeutic interventions, including systemic treatments, the patient's condition deteriorated rapidly, ultimately leading to his demise within a month of receiving the PCGD-TCL diagnosis. This case highlights the diagnostic complexities associated with PCGD-TCL, emphasizing the importance of careful histopathological examination and immunophenotypic characterization. Given its aggressive nature and propensity for rapid dissemination, early recognition of PCGD-TCL is paramount for initiating appropriate therapeutic interventions. However, effective treatment options for PCGD-TCL remain limited, and the disease typically carries an unfavorable prognosis. Further research is needed to elucidate the underlying molecular mechanisms driving the pathogenesis of PCGD-TCL, to identify novel therapeutic targets, and to improve patient outcomes. In addition, increased awareness among clinicians and pathologists regarding the clinical presentation and diagnostic criteria of PCGD-TCL is crucial for facilitating timely diagnosis and management of this challenging malignancy.
{"title":"Navigating Diagnostic and Therapeutic Challenges in Primary Cutaneous Gamma/Delta T-Cell Lymphoma: A Case Study of Fatal Outcomes Within Two Months","authors":"Nada Shaker, H. Blankenship, Vaishali Masatkar, Shuo Niu, Omar P. Sangueza","doi":"10.1097/dad.0000000000002743","DOIUrl":"https://doi.org/10.1097/dad.0000000000002743","url":null,"abstract":"\u0000 Primary cutaneous gamma/delta T-cell lymphoma (PCGD-TCL) is a rare yet highly aggressive subtype of primary cutaneous lymphoma. Characterized by its challenging diagnosis and poor prognosis, PCGD-TCL presents unique clinical and histopathological features that distinguish it from other primary cutaneous lymphoma subtypes. Here, we report the case of a 75-year-old man who initially presented with multiple erythematous indurated plaques over his back and bilateral lower extremities. The initial biopsy suggested primary cutaneous T-cell lymphoma (PCTCL) with a CD30-negative phenotype. However, within a 2-month interval, the disease progressed rapidly, manifesting as extensive skin involvement across the chest and upper extremities. A repeat skin biopsy was performed, revealing dermal atypical lymphocytes without epidermotropism. Immunohistochemical analysis demonstrated positivity for CD3, CD5, and CD4, as well as T-cell receptor delta (TCR delta) expression, along with the loss of CD8 and CD30 expression. These findings were consistent with a diagnosis of PCGD-TCL. Despite therapeutic interventions, including systemic treatments, the patient's condition deteriorated rapidly, ultimately leading to his demise within a month of receiving the PCGD-TCL diagnosis. This case highlights the diagnostic complexities associated with PCGD-TCL, emphasizing the importance of careful histopathological examination and immunophenotypic characterization. Given its aggressive nature and propensity for rapid dissemination, early recognition of PCGD-TCL is paramount for initiating appropriate therapeutic interventions. However, effective treatment options for PCGD-TCL remain limited, and the disease typically carries an unfavorable prognosis. Further research is needed to elucidate the underlying molecular mechanisms driving the pathogenesis of PCGD-TCL, to identify novel therapeutic targets, and to improve patient outcomes. In addition, increased awareness among clinicians and pathologists regarding the clinical presentation and diagnostic criteria of PCGD-TCL is crucial for facilitating timely diagnosis and management of this challenging malignancy.","PeriodicalId":501833,"journal":{"name":"The American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141377429","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-06DOI: 10.1097/dad.0000000000002738
Carmelo Urso
Melanocytic nevi can show a great number of morphological, cytoarchitectural, and stromal variations. Some of these histopathological patterns, infrequent or unusual, can occasionally produce diagnostic doubts or problems. A 22-year-old female patient presented a poorly pigmented cutaneous polypoid lesion of the scalp. Histopathological examination showed an intradermal melanocytic nevus composed of aggregates, nests, and cords of benign melanocytes, with collagenous stroma and large lipomatous areas. In the lipidized portion of the lesion, nevus cells, arranged in clusters, nests, and cords, were intimately associated with mature-appearing adipocytes, CD34-positive spindle cells, Alcian Blue–positive fibromyxoid stroma, and eosinophilic collagen bundles, findings resembling those typically seen in spindle cell lipoma. Spindle cell lipomatous metaplasia, rarely observed in some benign nonmelanocytic skin lesions, can be considered an additional unusual, not previously described, stromal variation occurring in melanocytic nevi.
{"title":"Melanocytic Nevus With Spindle Cell Lipomatous Metaplasia","authors":"Carmelo Urso","doi":"10.1097/dad.0000000000002738","DOIUrl":"https://doi.org/10.1097/dad.0000000000002738","url":null,"abstract":"\u0000 Melanocytic nevi can show a great number of morphological, cytoarchitectural, and stromal variations. Some of these histopathological patterns, infrequent or unusual, can occasionally produce diagnostic doubts or problems. A 22-year-old female patient presented a poorly pigmented cutaneous polypoid lesion of the scalp. Histopathological examination showed an intradermal melanocytic nevus composed of aggregates, nests, and cords of benign melanocytes, with collagenous stroma and large lipomatous areas. In the lipidized portion of the lesion, nevus cells, arranged in clusters, nests, and cords, were intimately associated with mature-appearing adipocytes, CD34-positive spindle cells, Alcian Blue–positive fibromyxoid stroma, and eosinophilic collagen bundles, findings resembling those typically seen in spindle cell lipoma. Spindle cell lipomatous metaplasia, rarely observed in some benign nonmelanocytic skin lesions, can be considered an additional unusual, not previously described, stromal variation occurring in melanocytic nevi.","PeriodicalId":501833,"journal":{"name":"The American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141377476","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-06DOI: 10.1097/dad.0000000000002745
Oluwaseyi Adeuyan, Emily R. Gordon, Brigit A. Lapolla, Celine M. Schreidah, V. Jobanputra, Alejandro A. Gru, L. J. Geskin
Among liposarcomas, well-differentiated liposarcoma and dedifferentiated liposarcoma are the most common. The majority of these tumors are found in deep retroperitoneum or extremities. When found outside the retroperitoneum, these adipose-derived tumors are known as atypical lipomatous tumors (ALT). Superficial ALT are particularly rare; thus, little is known about their clinical presentation, genomic status, and management. Here, we present the case of a 54-year-old man with an intermittently bothersome, slowly growing mass on his left upper back for over 2 years, which was incidentally diagnosed as ALT. This patient's ALT, however, showed a profound degree of pleomorphism with MDM2 and control centromere 12 (CEP12) coamplification and negative CD34 and S100 and RB1 expression, unlike most other ALT described in the literature. This case report details the diagnostic workup and histopathological findings for adipose tumors and summarizes the different subtypes, including atypical spindle cell/pleomorphic lipomatous tumor, pleomorphic liposarcoma, and spindle cell/pleomorphic lipoma, with brief discussion on management.
在脂肪肉瘤中，分化良好的脂肪肉瘤和脱分化脂肪肉瘤最为常见。这些肿瘤大多发生在腹膜后深部或四肢。在腹膜后以外的部位发现的脂肪源性肿瘤被称为非典型脂肪瘤（ALT）。浅表性脂肪瘤尤其罕见，因此人们对其临床表现、基因组状态和治疗方法知之甚少。在此，我们介绍了一例 54 岁男性的病例，他的左上背部有一个间歇性的、缓慢生长的肿块，已经持续了两年多，被偶然诊断为 ALT。然而，与文献中描述的大多数其他 ALT 不同的是，该患者的 ALT 表现出高度多形性，伴有 MDM2 和对照中心粒 12（CEP12）共扩增，CD34、S100 和 RB1 表达阴性。本病例报告详细介绍了脂肪瘤的诊断方法和组织病理学结果，总结了不同的亚型，包括非典型纺锤形细胞/多形性脂肪瘤、多形性脂肪肉瘤和纺锤形细胞/多形性脂肪瘤，并简要讨论了处理方法。
{"title":"Superficial Atypical Lipomatous Tumor With Pleomorphic Features: Case Report and Discussion of the Literature","authors":"Oluwaseyi Adeuyan, Emily R. Gordon, Brigit A. Lapolla, Celine M. Schreidah, V. Jobanputra, Alejandro A. Gru, L. J. Geskin","doi":"10.1097/dad.0000000000002745","DOIUrl":"https://doi.org/10.1097/dad.0000000000002745","url":null,"abstract":"\u0000 Among liposarcomas, well-differentiated liposarcoma and dedifferentiated liposarcoma are the most common. The majority of these tumors are found in deep retroperitoneum or extremities. When found outside the retroperitoneum, these adipose-derived tumors are known as atypical lipomatous tumors (ALT). Superficial ALT are particularly rare; thus, little is known about their clinical presentation, genomic status, and management. Here, we present the case of a 54-year-old man with an intermittently bothersome, slowly growing mass on his left upper back for over 2 years, which was incidentally diagnosed as ALT. This patient's ALT, however, showed a profound degree of pleomorphism with MDM2 and control centromere 12 (CEP12) coamplification and negative CD34 and S100 and RB1 expression, unlike most other ALT described in the literature. This case report details the diagnostic workup and histopathological findings for adipose tumors and summarizes the different subtypes, including atypical spindle cell/pleomorphic lipomatous tumor, pleomorphic liposarcoma, and spindle cell/pleomorphic lipoma, with brief discussion on management.","PeriodicalId":501833,"journal":{"name":"The American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141380104","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-01DOI: 10.1097/dad.0000000000002651
Margarita Muñoz de Toro, Vaishali Masatkar, H. Blankenship, Christine Ahn, O. Sangüeza
{"title":"Chronic Painful Vegetative Plaques on the Buttocks: Answer","authors":"Margarita Muñoz de Toro, Vaishali Masatkar, H. Blankenship, Christine Ahn, O. Sangüeza","doi":"10.1097/dad.0000000000002651","DOIUrl":"https://doi.org/10.1097/dad.0000000000002651","url":null,"abstract":"","PeriodicalId":501833,"journal":{"name":"The American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141229856","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-01DOI: 10.1097/dad.0000000000002678
Michael Tang, Brett C. Neill, Cary Chisholm, Stanislav N. Tolkachjov
{"title":"A Firm, Subcutaneous Nodule on the Temple of an African American Adolescent Boy: Challenge","authors":"Michael Tang, Brett C. Neill, Cary Chisholm, Stanislav N. Tolkachjov","doi":"10.1097/dad.0000000000002678","DOIUrl":"https://doi.org/10.1097/dad.0000000000002678","url":null,"abstract":"","PeriodicalId":501833,"journal":{"name":"The American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141233084","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-01DOI: 10.1097/dad.0000000000002614
Amanda A Onalaja-Underwood, T. Pincelli, O. Sokumbi
{"title":"Indurated Subcutaneous Thigh Nodules: Challenge","authors":"Amanda A Onalaja-Underwood, T. Pincelli, O. Sokumbi","doi":"10.1097/dad.0000000000002614","DOIUrl":"https://doi.org/10.1097/dad.0000000000002614","url":null,"abstract":"","PeriodicalId":501833,"journal":{"name":"The American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141231570","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-01DOI: 10.1097/dad.0000000000002615
Amanda A Onalaja-Underwood, T. Pincelli, O. Sokumbi
{"title":"Indurated Subcutaneous Thigh Nodules: Answer","authors":"Amanda A Onalaja-Underwood, T. Pincelli, O. Sokumbi","doi":"10.1097/dad.0000000000002615","DOIUrl":"https://doi.org/10.1097/dad.0000000000002615","url":null,"abstract":"","PeriodicalId":501833,"journal":{"name":"The American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141232080","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-01DOI: 10.1097/dad.0000000000002650
Margarita Muñoz de Toro, Vaishali Masatkar, H. Blankenship, Christine Ahn, O. Sangüeza
{"title":"Chronic Painful Vegetative Plaques on the Buttocks: Challenge","authors":"Margarita Muñoz de Toro, Vaishali Masatkar, H. Blankenship, Christine Ahn, O. Sangüeza","doi":"10.1097/dad.0000000000002650","DOIUrl":"https://doi.org/10.1097/dad.0000000000002650","url":null,"abstract":"","PeriodicalId":501833,"journal":{"name":"The American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141232538","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-01DOI: 10.1097/dad.0000000000002644
Nada Shaker, Omar P. Sangueza
{"title":"An Isolated Presentation of Multiple Flesh-Colored Papules on the Dorsal Hands and Forearms: A Diagnostic Challenge","authors":"Nada Shaker, Omar P. Sangueza","doi":"10.1097/dad.0000000000002644","DOIUrl":"https://doi.org/10.1097/dad.0000000000002644","url":null,"abstract":"","PeriodicalId":501833,"journal":{"name":"The American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141230205","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-01DOI: 10.1097/dad.0000000000002728
R. Abadi, M. Kurban, O. Abbas
{"title":"Painless Symmetric Thickening of Proximal Interphalangeal Joints in an Adolescent Girl: Answer","authors":"R. Abadi, M. Kurban, O. Abbas","doi":"10.1097/dad.0000000000002728","DOIUrl":"https://doi.org/10.1097/dad.0000000000002728","url":null,"abstract":"","PeriodicalId":501833,"journal":{"name":"The American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141228844","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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