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J Cutan Pathol 2022 May 13. Epub 2022 May 13.

Department of Pathology, Duke University Hospital.

Cutaneous inflammatory myofibroblastic tumors (IMT) constitute a rare entity, generating a diagnostic pitfall when diagnosing spindle cell proliferation within the dermis. Raising awareness of this tumor among dermatopathologists remains vital in differentiating it from common cutaneous tumors such as fibrous histiocytoma, atypical fibroxanthoma, melanoma, poorly differentiated carcinoma, and other more aggressive tumors. Accurate diagnosis of IMT aid in ensuring appropriate management and follow-up for patients while preventing unnecessary harm and overtreatment. Read More

Actas Dermosifiliogr 2022 May 6. Epub 2022 May 6.

Servicio de Dermatología, Fundación Instituto Valenciano de Oncología, Valencia, España.

Am J Dermatopathol 2022 May 3. Epub 2022 May 3.

Department of Pathology, Pathology Associates of Central Illinois, Springfield Memorial Hospital, Springfield, IL.

Am J Dermatopathol 2022 Apr 27. Epub 2022 Apr 27.

Department of Pathology, Michigan Medicine, University of Michigan, Ann Arbor, MI; and.

Abstract: A 58-year-old man presented with a lesion on the nose suspicious for basal cell carcinoma. An initial biopsy specimen reviewed at an outside institution showed a cytologically atypical spindle cell proliferation that lacked expression of cytokeratins or melanocytic markers. The resulting differential diagnosis included atypical fibroxanthoma and pleomorphic dermal sarcoma. Read More

Dermatol Online J 2022 Jan 15;28(1). Epub 2022 Jan 15.

Rutgers Robert Wood Johnson Medical School, Piscataway, New Jersey, USA Dermatology Service, VA New York Harbor Healthcare System, Brooklyn, New York, USA.

Pleomorphic dermal sarcoma (PDS) was recognized in the 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone as a clinical entity with adverse histopathologic features compared to the more superficial and less aggressive atypical fibroxanthoma (AFX). Although the gold standard treatment of AFX is Mohs micrographic surgery (MMS), the optimal treatment for PDS has yet to be determined. We report the case of a 71-year-old man with a PDS with perineural invasion on the scalp, with no recurrence one-year post-treatment with MMS and adjuvant radiation therapy. Read More

Front Oncol 2022 14;12:873771. Epub 2022 Apr 14.

Department of Dermatology, University Hospital Cologne, Cologne, Germany.

Background: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are increasingly common sarcomas of the skin with a genetic UV signature. Immunosuppression is a known risk factor for developing other UV-induced skin cancers such as cutaneous squamous cell carcinoma (cSCC), basal cell carcinoma (BCC), and Merkel cell carcinoma with increased mortality. In case reports or small case series of AFX/PDS patients, immunosuppression has been hypothesized as a risk factor for the development of distant metastases. Read More

J Surg Oncol 2022 Apr 19. Epub 2022 Apr 19.

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

Background: Risk factors for local atypical fibroxanthoma (AFX) recurrence and progression to pleomorphic dermal sarcoma (PDS) have not previously been identified.

Objective: To identify risk factors and provide follow-up suggestions for local AFX recurrence and progression to PDS.

Methods And Materials: A literature search was performed in the PubMed, EMBASE, and Cochrane databases. Read More

Actas Dermosifiliogr 2022 Feb 24. Epub 2022 Feb 24.

Servicio de Dermatología, Fundación Instituto Valenciano de Oncología, Valencia, España.

Cancers (Basel) 2022 Mar 26;14(7). Epub 2022 Mar 26.

Department of Plastic Surgery and Breast Surgery, Zealand University Hospital (SUH) Roskilde, University of Copenhagen, 4000 Roskilde, Denmark.

Primary dermal sarcomas (PDS) belong to a highly clinically, genetically and pathologically heterogeneous group of rare malignant mesenchymal tumours primarily involving the dermis or the subcutaneous tissue. The tumours are classified according to the mesenchymal tissue from which they originate: dermal connective tissue, smooth muscle or vessels. Clinically, PDS may mimic benign soft tissue lesions such as dermatofibromas, hypertrophic scarring, etc. Read More

Ear Nose Throat J 2022 Apr 7:1455613221084072. Epub 2022 Apr 7.

Department of Otorhinolaryngology, 91577Jeju National University College of Medicine, Jeju-do, Korea.

Atypical fibroxanthoma (AFX) is a dermal spindle-cell cutaneous malignancy, which is a relatively benign type of skin cancer that occurs in the elderly with sun-damaged skin. This is a case study of a rapidly enlarged left auricular mass lesion in an elderly patient who was diagnosed with AFX based on histopathological and immunohistochemical findings, and then treated by complete excision to prevent recurrence or metastasis. There was no recurrence during the 1-year follow-up. Read More

J Clin Pathol 2022 Mar 22. Epub 2022 Mar 22.

Department of Anatomic Pathology, Kyushu University, Fukuoka, Japan

Aims: Dermatofibroma/fibrous histiocytoma (DF/FH) is a common cutaneous mesenchymal neoplasm exhibiting benign biological behaviour. However, the immunohistochemical utility of erythroblast transformation-specific-related gene (ERG) for diagnosing DF remains unknown. The authors reviewed the immunohistochemical status of ERG in different subtypes of DF and in its differential diagnoses. Read More

J Cutan Pathol 2022 Mar 17. Epub 2022 Mar 17.

Department of Dermatology, Saint Louis University School of Medicine, St. Louis, Missouri, USA.

Atypical fibroxanthoma (AFX) with osteoclast-like giant cells is a rare entity. We present the case of an elderly woman who presented with a pink-purple dome-shaped nodule with central hyperkeratotic crust. Biopsy revealed a cellular, dermal-based tumor comprised of spindle, oval, and osteoclast-like giant cells with pleomorphism. Read More

Ann Diagn Pathol 2022 Feb 10;58:151915. Epub 2022 Feb 10.

Department of Pathology, Division of Laboratories, Pharmacy and Biomedical Genetics, University Medical Center Utrecht, Heidelberglaan 100, Utrecht 3584 CX, the Netherlands.

Background: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare cutaneous neoplasms forming a spectrum. Case reports with recurrences and metastasis have been published despite the current view that AFX is benign. The aim of this study was to identify clinical and histopathological features that predict tumor recurrence. Read More

J Dtsch Dermatol Ges 2022 Feb 31;20(2):235-243. Epub 2022 Jan 31.

Department of Dermatology, University Hospital, Johannes Gutenberg University Mainz, Germany.

Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare cutaneous neoplasms representing histomorphological, genetic as well as epigenetic variants of a disease spectrum. Both tumors typically manifest as nonspecific, often ulcerated, skin- to flesh-colored nodules in chronically sun-damaged skin of elderly male patients. AFX is a rather well demarcated, often rapidly growing tumor. Read More

Histopathology 2022 Jan;80(1):135-149

Division of Pathology, Cancer Research UK Edinburgh Centre, Edinburgh, UK.

Primary cutaneous and mucosal melanoma shows a wide histological spectrum. The correct diagnosis depends upon the demonstration of melanocytic differentiation by recognition of an associated in-situ component or immunohistochemical evidence of a melanocytic phenotype using conventional melanocytic markers, such as S-100, SOX10, Melan-A and HMB-45. Exceptionally, melanomas lose their melanocytic phenotype, at least focally, and show differentiation towards other lineages. Read More

Clin Exp Dermatol 2022 May 22;47(5):833-849. Epub 2022 Feb 22.

Department of Dermatology, Manor Hospital, Walsall Healthcare NHS Trust, Walsall, UK.

Mohs micrographic surgery (MMS) is a precise and effective method commonly used to treat high-risk basal cell carcinoma and squamous cell carcinoma on the head and neck. Although the majority of evidence for MMS relates to keratinocyte cancers, there is published evidence for other types of skin cancer. This review aims to discuss the evidence for using MMS to treat six different types of skin cancer [malignant melanoma, lentigo maligna, dermatofibrosarcoma protuberans, atypical fibroxanthoma (AFX), microcystic adnexal carcinoma and pleomorphic dermal sarcoma (PDS)] particularly in the context of survival rates and cancer recurrence. Read More

Cureus 2021 Dec 15;13(12):e20426. Epub 2021 Dec 15.

Patholgy, Aberdeen Royal Infirmary, Aberdeen, GBR.

The finding of a pigmented lesion within another distinct lesion is rare but not unheard of. Here, we describe the presence of an atypical fibroxanthoma within a melanoma in a 72-year-old female referred to the plastics surgery department with a pigmented lesion on her left knee. It was excised in view of clinical suspicion of melanoma. Read More

J Dermatol 2022 Jan 16;49(1):e44-e45. Epub 2021 Nov 16.

Department of Dermatology, Kansai Medical University Medical Center, Osaka, Japan.

Cancer Rep (Hoboken) 2021 Nov 11:e1583. Epub 2021 Nov 11.

Department of Dermatology, University College London Hospitals NHS Foundation Trust, London, UK.

Background: Pleomorphic dermal sarcoma (PDS) describes rare dermal-based malignant tumours that are morphologically similar to atypical fibroxanthoma (AFX). PDS may be differentiated from AFX by the presence of one or more of the following histologic features: subcutaneous invasion, tumour necrosis, lymphovascular invasion (LVI), and/or perineural infiltration (PNI).

Aims: To further define the clinicopathological features, surgical management, and outcomes of PDS primary tumours. Read More

J Neurol Surg B Skull Base 2021 Dec 4;82(6):689-694. Epub 2021 Feb 4.

Department of Plastic Surgery, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom.

 Cutaneous malignancies are on the rise, associated with an increased number in scalp cancers that require wide local excision (WLE) to ensure clearance; the inelastic nature of the scalp poses a particular challenge when dealing with such large defects.  A series of 68 cases with large scalp defects following WLE for the clearance of squamous cell carcinoma, atypical fibroxanthoma, dermatofibrosarcoma protuberans, and melanoma skin cancers are presented. These cases were treated in one center under local anesthesia and underwent extended scalp flaps to close the resulting defect primarily without the use of skin grafts for the flap donor site on the scalp. Read More

J Am Acad Dermatol 2021 Sep 26. Epub 2021 Sep 26.

Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois; Department of Surgery, Feinberg School of Medicine, Northwestern University, Chicago, Illinois; Department of Otoloaryngology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois. Electronic address:

Background: Mohs micrographic surgery or wide local excision is the treatment of choice for fibrohistiocytic tumors with metastatic potential, including atypical fibroxanthoma (AFX) and cutaneous undifferentiated pleomorphic sarcoma (cUPS). Since margin clearance is the strongest predictor of clinical recurrence, improved recommendations for appropriate surgical margins help delineate uniform excision margins when intraoperative margin assessment is not available.

Objective: To determine appropriate surgical wide local excision margins for AFX and cUPS. Read More

Int J Surg Pathol 2022 May 20;30(3):313-316. Epub 2021 Sep 20.

16582Department of Pathology, University Hospital Virgen Macarena, Sevilla, Spain.

Numerous cells with very large and irregular nuclei ("monster" cells) have not hitherto been reported in desmoplastic melanoma (DM). Their prognostic significance in melanomas is a matter of debate, although some authors have associated them with more aggressive tumor behavior. We report a mixed DM on the scalp of an 88-year-old woman imitating an atypical fibroxanthoma. Read More

BMJ Case Rep 2021 Aug 23;14(8). Epub 2021 Aug 23.

Plastic Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Atypical fibroxanthoma (AFX) is a rare cutaneous ulcerative lesion with a good prognosis. Diagnosis is difficult and is usually made by exclusion from pleomorphic dermal sarcoma and squamous cell carcinoma. Definitive treatment varies from Mohs micrographic surgery to wide local excision. Read More

Acta Med Litu 2021 29;28(1):165-169. Epub 2021 Apr 29.

1 Pulmonology Department Sismanogleio Hospital, Athens, Greece.

Atypical fibroxanthoma is an infrequent, low-grade superficial cutaneous neoplasm, usually presenting as a nodule or plaque of red color. It is considered as a superficial variant of pleomorphic dermal sarcoma. Although atypical fibroxanthoma has similar histologic features to pleomorphic dermal sarcoma, it has less aggressive behavior. Read More

Rare Tumors 2021 30;13:20363613211026483. Epub 2021 Jun 30.

Department of Pathology and Cytology, Clinical Hospital Center Rijeka, Rijeka, Croatia.

Malignant mesenchymal tumors of oropharyngeal mucosa are rare. Those with fibroblastic and histiocytic differentiation in the skin are called atypical fibroxanthoma (AFX) and in the soft tissue undifferentiated pleomorphic sarcoma (UPS). Here we present a case of an older patient with a history of multiple basal cell carcinomas and recently with a rapidly growing polypoid lesion in the mucosa of posterior oropharyngeal wall with AFX/UPS morphology. Read More

J Cutan Pathol 2021 Dec 29;48(12):1493-1496. Epub 2021 Jul 29.

Ackerman Academy of Dermatopathology, New York, New York, USA.

Melanoma may mimic a variety of skin lesions clinically and histopathologically, and presents diagnostic challenges. In this article, we describe a case of melanoma in an 89-year-old man with a very rare histopathologic presentation, namely the presence of pleomorphic and multinucleated giant cells with abundant cytoplasm, highly resembling an atypical fibroxanthoma. The differential diagnoses in conjunction with the findings in immunohistochemical study are also discussed. Read More

Pol Merkur Lekarski 2021 Jun;49(291):221-223

Institute of Hematology and Transfusiology Medicine, Warsaw, Poland: Department of General, Oncological and Metabolic Surgery.

Atypical fibrous histiocytoma (AFH) is an uncommon variant of benign skin neoplasm, fibrous histiocytoma. Despite having pseudosarcomatous histological features, atypical fibrous histiocytoma is characterized by a benign clinical course.

Aim: The aim of the study was to present the case of local recurrence of atypical fibrous histiocytoma in scar after the primary excision. Read More

BMJ Case Rep 2021 Jul 2;14(7). Epub 2021 Jul 2.

Surgery, Saint Barnabas Medical Center, Livingston, New Jersey, USA.

An 82-year-old man presented with a right scalp lesion which had been increasing in size. The patient's medical history was significant for a heart transplant 25 years before, and he was on chronic immunosuppression. Biopsy of the lesion showed atypical fibroxanthoma. Read More

Genes (Basel) 2021 06 25;12(7). Epub 2021 Jun 25.

Dermatology Department, University Hospital Zurich, 8091 Zurich, Switzerland.

Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare tumors developing in chronically sun-exposed skin. Clinicopathological features are similar, but they differ in prognosis, while PDS has a more aggressive course with a higher risk for local recurrence and metastases. In current clinical practice, they are diagnosed by exclusion using immunohistochemistry. Read More

JAAD Case Rep 2021 Jul 27;13:100-102. Epub 2021 May 27.

Department of Dermatology, HCA Healthcare/USF Morsani College of Medicine GME, Largo Medical Center, Largo, Florida.