902 results match your criteria: "Atypical Fibroxanthoma"

A rapidly-growing friable nodule on the cheek.

Dermatol Online J

August 2024

Department of Dermatology, Stanford University School of Medicine, California, USA Department of Dermatology, The Permanente Medical Group, Sacramento, California, USA.

Atypical fibroxanthoma and pleomorphic dermal sarcoma are on a spectrum of cutaneous tumors that present as ulcerated lesions in older adults. We present an 84-year-old man with pleomorphic dermal sarcoma, initially presenting as a bleeding lesion of the cheek that progressed to an eroded nodule.

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Undifferentiated pleomorphic sarcoma (UPS) and related tumors are the most common type of soft tissue sarcoma. However, this spectrum of tumors has different etiologies with varying rates of metastasis and survival. Two dermal-based neoplasms in this class of pleomorphic sarcomas, atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS), are challenging to differentiate at initial biopsy but vary significantly in prognosis.

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We report the case of a Hispanic male whose conjunctival atypical fibroxanthoma (AFX) grew very large, with intraocular as well as extraocular muscle extensions, and was treated with exenteration. A 50-year-old male presented with a one-month history of foreign-body sensation in his left eye. The initial examination revealed an erythematous, vascularized, pedunculated lesion arising from the left eye conjunctiva.

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T-cell Clonality in Pleomorphic Dermal Sarcoma in Male Veterans: A Report of 2 Cases and a Review of the Literature.

Am J Dermatopathol

December 2024

Dermatopathology Section, Department of Pathology and Laboratory Medicine, VA Boston Healthcare System, West Roxbury, MA; and.

The standard treatment of choice for pleomorphic dermal sarcoma (PDS), a relatively uncommon soft tissue sarcoma and 1 morphologically similar to atypical fibroxanthoma, is wide local excision with close clinical follow-up. Studies regarding management of advanced/metastatic PDS with immune checkpoint inhibitors are limited as most STSs have historically been viewed as being immunologically inert. Contradicting this belief, in this report, we describe 2 cases of PDS with a robust host response.

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The incidence of atypical fibroxanthoma and pleomorphic dermal sarcoma in Denmark from 2002 to 2022.

Surg Oncol

December 2024

Department of Plastic Surgery and Burns Treatment, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. Electronic address:

Article Synopsis
  • * A total of 1,118 patients were examined, revealing that 84.5% had AFX, while 15.5% were diagnosed with PDS; the median age at diagnosis was around 77 years for both types.
  • * The incidence rate of AFX and PDS increased from 0.2 to 0.5 per 100,000 people over the study period, with PDS showing worse overall survival compared to AFX.
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Rare Intravascular Involvement of Pleomorphic Dermal Sarcoma Seen During Mohs Micrographic Surgery.

Perm J

December 2024

Department of Mohs Surgery, Barranca Medical Offices, Southern California Permanente Medical Group , Irvine, CA, USA.

Pleomorphic dermal sarcoma (PDS) and atypical fibroxanthoma (AFX) are rare mesenchymal tumors that share similar clinical, histological, and immunohistochemical characteristics. Careful histopathological examination of a biopsy specimen that includes subcutaneous fat remains the preferred way to differentiate between these tumors. AFX is limited to dermal invasion, whereas PDS demonstrates deeper invasion.

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Article Synopsis
  • * After removing the lesion, doctors found it contained features of both basal cell carcinoma and atypical fibroxanthoma.
  • * The final diagnosis confirmed the presence of both types of cancer in the lesion, marking it as a rare case of a cutaneous collision.
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Article Synopsis
  • A 68-year-old woman had a concerning eye lesion initially thought to be an atypical fibroxanthoma, but it was later diagnosed as spindle cell carcinoma after a biopsy.
  • Spindle cell carcinoma is a rare and aggressive type of squamous cell carcinoma, highlighting the need for careful surgical technique and comprehensive analysis for accurate diagnosis.
  • The case contributes to limited research on this type of cancer affecting the eye, aiming to improve future diagnostic and treatment guidelines as more data becomes available.
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Article Synopsis
  • Primary cutaneous sarcomatoid squamous cell carcinoma (PCSSCC) can be difficult to diagnose due to its similarities with other spindle cell tumors and atypical expressions of markers like smooth muscle actin (SMA).
  • A study of 16 cases showed that these tumors were high-grade and typically involved deeper skin layers, with most cases exhibiting atypical spindle and epithelioid cell patterns.
  • The research highlights the importance of using multiple epithelial markers for accurate diagnosis, while also noting that SMA expression can complicate the identification process.
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(1) Background: Rare skin cancers include epithelial, neuroendocrine, and hematopoietic neoplasias as well as cutaneous sarcomas. Ultraviolet (UV) radiation and sunburns are important drivers for the incidence of certain cutaneous sarcomas; however, the pathogenetic role of UV light is less clear in rare skin cancers compared to keratinocyte cancer and melanoma. In this study, we compared the degree of actinic elastosis (AE) as a surrogate for lifetime UV exposure among selected rare skin cancers (atypical fibroxanthoma [AFX], pleomorphic dermal sarcoma [PDS], dermatofibrosarcoma protuberans [DFSP], Kaposi sarcoma [KS], Merkel cell carcinoma [MCC], and leiomyosarcoma [LMS]) while taking into account relevant clinical variables (age, sex, and body site).

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Article Synopsis
  • * The exact cause of cCS is unknown; it may arise from a single cell capable of differentiating into both types of tissue or from the interaction of existing carcinoma and sarcoma cells.
  • * Histologically, cCS can show various types of epithelial and sarcomatous characteristics, necessitating further research to improve understanding of its development and treatment options.
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Article Synopsis
  • The study investigates TRPS1 immunoreactivity in various cutaneous mesenchymal neoplasms and tumors of uncertain differentiation, highlighting gaps in existing research.
  • Out of 135 analyzed cases, TRPS1 was found to be highly prevalent in dermatofibromas and atypical fibroxanthomas, while being less common in vascular tumors and absent in certain tumors like perineuriomas.
  • The findings indicate significant differences in TRPS1 expression levels between AFXs and other tumor types, although it shows limited effectiveness in differentiating between atypical fibroxanthomas and sarcomatoid squamous cell carcinoma.
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Article Synopsis
  • Pleomorphic dermal sarcomas are aggressive skin tumors that often lead to local recurrence and can spread, making early detection crucial.
  • Differentiating between atypical fibroxanthoma and pleomorphic dermal sarcoma requires a thorough examination of the entire tumor after removal.
  • Using Mohs micrographic surgery and conducting detailed histopathological evaluations can help improve diagnosis, risk assessment, and treatment options for better patient outcomes.
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Article Synopsis
  • - The study investigates superficial CD34-positive fibroblastic tumors (SCD34FT), a rare low-grade skin neoplasm that often expresses CADM3, in comparison to atypical fibrous histiocytomas (FH).
  • - Out of 40 examined cases of atypical FH, 55% of CD34-positive tumors were reclassified as SCD34FT due to their CADM3 positivity and frequent PRDM10 rearrangement.
  • - The findings suggest that distinguishing between SCD34FT and atypical FH using CADM3 immunohistochemistry is crucial, as many SCD34FT tumors exhibit morphologic features similar to atypical FH.
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Article Synopsis
  • - The rise of telemedicine due to COVID-19 highlights the need for clinicians to effectively use virtual care tools, particularly teledermatology.
  • - A case series of 3 patients at a Midwestern medical center shows how teledermatology helped quickly diagnose rare skin cancers like Merkel cell carcinoma and malignant melanoma.
  • - Each case outlines the initial virtual consultation and tracks the timeline to in-person biopsies, illustrating how teledermatology can improve healthcare for at-risk patients.
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Article Synopsis
  • An 88-year-old male had a large mass on his left eye's conjunctiva two months after having atypical fibroxanthoma (AFX) removed.
  • Imaging revealed that the tumor invaded surrounding tissues, leading to the complete surgical removal of the mass.
  • Following the surgery, he was diagnosed with large B-cell lymphoma in the throat but showed no AFX recurrence after seven months; the case is noted as the first of its kind involving AFX invading the orbit.
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Article Synopsis
  • Dermatofibroma (DF) is a benign tumor typically found on the limbs and trunk of young adults, characterized by a specific arrangement of fibroblast-like cells and various secondary elements, which can lead to misdiagnosis as other tumors like sarcomas.
  • A notable case was documented involving a 26-year-old woman whose DF was initially misdiagnosed as angiosarcoma after a biopsy showed atypical spindle cells; imaging indicated tumor and lymph node involvement.
  • The report underscores the need for careful evaluation of DF’s clinical and histological features to prevent misdiagnosis, and suggests that nanopore DNA sequencing may provide valuable diagnostic insights.
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Ex Vivo Confocal Laser Scanning Microscopy in Rare Skin Diseases.

Cancers (Basel)

April 2024

Department of Dermatology and Allergy, LMU University Hospital, LMU Munich, 80337 Munich, Germany.

Article Synopsis
  • Ex vivo confocal laser scanning microscopy (CLSM) has been primarily used for common skin diseases, with limited exploration of its effectiveness in rare skin tumors and inflammatory conditions.
  • The study involved imaging 50 tissue samples, including rare disorders, to evaluate diagnostic efficacy and involved three blinded investigators assessing the images for distinct features.
  • Results indicated that imaging expertise significantly improved diagnostic accuracy, with the trained dermatopathologists achieving up to 92% agreement with standard dermatohistopathology, showcasing CLSM's potential as a valuable tool for identifying rare skin disorders.
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Article Synopsis
  • - Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare tumors that require careful differentiation, as PDS can spread while AFX does not infiltrate beyond the dermis.
  • - A study involving 13 patients found that preoperative sonography accurately distinguished between AFX and PDS in all cases, even changing the diagnosis in 3 patients, which was confirmed post-surgery.
  • - Using ultrasound can enhance the clinical diagnosis, speed up treatment, and improve patient care, as AFX can be treated with micrographic surgery while PDS requires more extensive surgical approaches and lymph node evaluation.
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