BACKGROUND Eccrine porocarcinoma (EPC) was first described in 1963 as an epidermotropic eccrine carcinoma. Fifty years later, its etiology remains poorly understood. The infrequent nature of this disease merits further inquiry into its etiology, presentation, and standards of management. Furthermore, the propensity for metastasis, which may be as high as 31% on presentation, increases the importance of investigating this rare disease. CASE REPORT The patient was a 63-year-old mechanic who presented with the lesion as a chronic wound following a chemical exposure. The lesion involved the ulnar aspect of his right palm and had concern for extension to the underlying tendons. He underwent a wide excision extending from the wrist to the proximal interphalangeal joint, preserving the ulnar neurovascular bundle. The hand was reconstructed with an anterolateral thigh fascia perforator flap and a skin graft. He had an excellent functional and cosmetic recovery. Unfortunately, he developed metastases to the lymph nodes, necessitating an axillary lymphadenectomy followed by adjuvant chemoradiation using concurrent cisplatin and docetaxel with radiation for 6 weeks. Follow-up at 18 months found no recurrence. CONCLUSIONS Cases of EPC presenting in the fingers have been managed with amputation of the involved phalanges; however, in addition to obtaining complete excision with negative margins, surgeons who deal with tumors of the hand must also consider the goals of limb preservation, functional preservation, and functional reconstruction. Options for reconstruction following excision include primary closure, dermal regeneration templates, skin grafts, flaps, and free-tissue transfer, depending on what tissue types are needed.
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http://dx.doi.org/10.12659/AJCR.925231 | DOI Listing |
J Low Genit Tract Dis
January 2025
Saint Louis University School of Medicine, St. Louis, MO.
Objective: Authors characterized all published adult cases of cutaneous, intertriginous Langerhans cell histiocytosis (LCH) to bring this clinical presentation to the attention of clinicians. We emphasize the morphology, histopathology, immunohistochemical profiles, and genetic mutations associated with these cases.
Materials And Methods: A systematic review of the National Center for Biotechnology Information's PubMed was conducted, utilizing the following specific key words to identify all adult LCH patients with cutaneous intertriginous involvement: "Intertriginous Langerhans," "Vulvar Langerhans," "Genital Langerhans," "Perineal Langerhans," "Perianal Langerhans," "Intergluteal Langerhans," "Inguinal Langerhans," "Axillary Langerhans," and "Inframammary Langerhans.
N Engl J Med
January 2025
From the Department of Medicine, Northwestern Memorial Hospital, Chicago (C.W.Y.); and the Departments of Medicine (J.S.G., A.J.Y.), Radiology (B.G.G.), and Pathology (B.M.H.), Massachusetts General Hospital, the Departments of Medicine (J.S.G., R.H.F., A.J.Y.), Radiology (B.G.G.), and Pathology (B.M.H.), Harvard Medical School, and the Department of Medicine, Brigham and Women's Hospital (R.H.F.) - all in Boston.
N Engl J Med
January 2025
Assistance Publique-Hôpitaux de Paris-Sorbonne Université, Paris, France
Genet Test Mol Biomarkers
January 2025
Department of Biology, University of Sistan and Baluchestan, Zahedan, Iran.
Fanconi anemia (FA) is a rare genetic disorder that affects multiple systems in the body and is the most prevalent congenital syndrome, leading to bone marrow failure. Twenty-two genes have been identified as contributors to the disease. Significant advancements have been made in the past 2 decades in understanding the genetic and pathophysiological processes involved.
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