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Acta Dermatovenerol Croat 2020 Dec;28(7):210-214

Pedro Rodríguez-Jiménez, MD, Dermatology Department, Hospital Universitario de la Princesa, Madrid, Calle Diego de Leon 62, 28006 Madrid, Spain;

The body of literature supporting the use of Mohs micrographic surgery (MMS) in tumors outside the main indications (basal cell carcinoma, squamous cell carcinoma, dermatofibrosacroma protuberans, lentigo maligna) is constantly growing, but it is still based on case reports, case series, or at best institutional case series that focus on a single malignancy. Our aim in this review was to assess use of MMS in an array of rare tumors outside the usual indications. A review was performed using the MEDLINE database and the search engine ClinicalKey®. Read More

Int J Dermatol 2021 Apr 7. Epub 2021 Apr 7.

Oral and Maxillofacial Surgery Department, Consorci Corporació Sanitària Parc Taulí, Sabadell, Barcelona, Spain.

Am J Dermatopathol 2021 Mar 9. Epub 2021 Mar 9.

Department of Pathology, Veterans Affairs Palo Alto Health Care System, Palo Alto, CA; Department of Pathology, Stanford University School of Medicine, Stanford, CA; Department of Dermatology, Stanford University School of Medicine, Stanford, CA; Department of Pathology, Veterans Affairs Central California Health Care System, Fresno, CA; and Cytogenetics Laboratory, Department of Pathology, Stanford Health Care, Palo Alto, CA.

Abstract: Atypical fibroxanthoma (AFX) is a neoplasm that most commonly occurs on sun-damaged skin of the head and neck in elderly patients and that usually exhibits indolent clinical behavior with complete excision. The granular cell variant of AFX demonstrates overlapping histopathologic features with dermal non-neural granular cell tumor (NNGCT), which typically arises on the extremities of young to middle aged adults with rare reports of regional metastasis. A subset of NNGCT harbors ALK rearrangements and expresses ALK by immunohistochemistry. Read More

Int J Dermatol 2021 Mar 14. Epub 2021 Mar 14.

Dermatology and Mohs Micrographic and Reconstructive Surgery, Epiphany Dermatology, Dallas, TX, USA.

Cutaneous mesenchymal tumors (CMT) are rare tumors with wide clinicopathologic heterogeneity. Treatment of malignant cutaneous mesenchymal tumors traditionally includes wide local excision (WLE), though Mohs micrographic surgery (MMS) has been increasingly used. A PubMed literature review of articles from inception until September 2019 related to malignant CMT and surgical treatment with MMS or WLE was completed. Read More

J Cutan Pathol 2021 Mar 6. Epub 2021 Mar 6.

Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

Sarcomatoid dedifferentiated melanoma (SDDM) is a recently recognized subtype of melanoma that stains diffusely for CD10 and lacks the expression of the usual melanocytic markers including S100, SOX10, MITF, and Melan A. Advances in next-generation DNA sequencing technology have facilitated the increased recognition of this rare, aggressive spindle cell melanoma. Herein, a case of relatively early lesion of SDDM arising in association with melanoma in situ is highlighted. Read More

J Dermatol 2021 May 23;48(5):e215-e216. Epub 2021 Feb 23.

Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.

Otolaryngol Clin North Am 2021 Apr 16;54(2):369-378. Epub 2021 Feb 16.

Department of Otolaryngology-Head and Neck Surgery, Lewis Katz School of Medicine at Temple University, Fox Chase Cancer Center, 3440 North Broad Street, Philadelphia, PA 19140, USA.

Cutaneous sarcomas represent a rare group of tumors presenting in the head and neck. In this article, we discuss specific sarcoma tumor types and their presentation, pathogenesis, histologic findings, and management recommendations. Tumors to be reviewed include dermatofibrosarcoma protuberans, atypical fibroxanthoma, pleomorphic dermal sarcoma, cutaneous leiomyosarcoma, and angiosarcoma. Read More

Dermatopathology (Basel) 2021 Jan 12;8(1):25-28. Epub 2021 Jan 12.

Section of Pathology, Department of Emergency and Organ Transplantation, University of Bari, 70121 Bari, Italy.

Atypical fibroxanthoma-like amelanotic melanoma is a very rare variant of melanoma that can, if not correctly recognized and framed, lead to diagnostic errors that can potentially cause problems of extreme relevance to patients. Correct knowledge of this entity and the execution of adequate immunohistochemical investigations are the basic conditions for the correct management of this lesion. We report on a case of atypical fibroxanthoma-like amelanotic melanoma, which clinically simulated a fibrohistiocytic lesion, and which created differential diagnostic problems, and finally, we conduct a short review of the literature. Read More

Br J Oral Maxillofac Surg 2021 05 11;59(4):460-465. Epub 2020 Sep 11.

Oral & Maxillofacial Surgery Department, St. Richards Hospital, Chichester.

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm that shares histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumour necrosis, and vascular and perineural invasion, which confers a risk of local recurrence and metastases. To study the clinical and pathological spectrum more comprehensively, we retrieved 49 cases of pleomorphic dermal sarcoma from our departmental files. There was a strong male predominance (n=45). Read More

Am J Surg Pathol 2021 02;45(2):240-254

Institute of Pathology.

Dedifferentiated melanoma (DM) and undifferentiated melanoma (UM) is defined as a primary or metastatic melanoma showing transition between conventional and undifferentiated components (DM) or lacking histologic and immunophenotypic features of melanoma altogether (UM). The latter is impossible to verify as melanoma by conventional diagnostic tools alone. We herein describe our experience with 35 unpublished cases to expand on their morphologic, phenotypic, and genotypic spectrum, along with a review of 50 previously reported cases (total: 85) to establish the diagnostic criteria. Read More

J Cosmet Dermatol 2021 Jan 6. Epub 2021 Jan 6.

Department of Dermatology, Health Science University Ümraniye Training and Research Hospital, Istanbul, Turkey.

Background: Dermatofibroma, also known as cutaneous benign fibrous histiocytoma, is a common skin tumour.

Aim: The aim of this paper was to present a rare variant of dermatofibroma, atrophic dermatofibroma, emphasizing histopathological and dermoscopic features.

Patients/methods: A case of atrophic dermatofibroma in a female patient with the characteristic histopathological features and newly demonstrated dermoscopic findings is presented. Read More

Actas Dermosifiliogr 2021 May 7;112(5):392-405. Epub 2020 Dec 7.

Servicio de Anatomía Patológica, Complejo Hospitalario Universitario A Coruña, A Coruña, España.

The relation between atypical fibroxanthoma and pleomorphic dermal sarcoma has led to confusion and debate in the literature. Both tumors present on sun-exposed skin, typically on the head and neck, in patients of advanced age. Both are comprised of a variable mix of histiocytoid, spindle, epithelioid, and/or giant multinucleated cells with pleomorphic nuclei. Read More

Dermatol Online J 2020 Oct 15;26(10). Epub 2020 Oct 15.

Department of Dermatology, Indiana University School of Medicine, Indianapolis, IN.

Atypical fibroxanthomas are rare, superficial dermal tumors. Most cases are benign and only locally destructive with a low rate of metastasis. Lesions are most commonly found on sun-exposed sites of elderly light-skinned patients and present as asymptomatic nodules with irregular borders; ulcerations and friability are other key characteristics. Read More

J Hand Surg Am 2020 Oct 17. Epub 2020 Oct 17.

Department of Plastic and Reconstructive Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea.

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal tissue tumor that shares pathological features with atypical fibroxanthoma, but also exhibits tumor necrosis, invasion beyond the superficial subcutis, and vascular or perineural infiltration. In addition, PDS also has relatively high rates of local recurrence and metastasis and is usually encountered in elderly men, especially in the head and neck area. In this article, we report a rare case of PDS that infiltrated the fascial tissues in the forearm of a female patient. Read More

Eur J Dermatol 2020 Oct;30(5):545-553

Department of Dermatology, Instituto Valenciano de Oncología. Valencia, Spain.

Background: Relatively little is known about the true aggressive potential of pleomorphic dermal sarcoma (PDS) or optimal management strategies.

Objective: To describe the outcomes of 16 cases of PDS treated at our hospital (14 with modified Mohs micrographic surgery [M-MMS] and two with conventional surgery) and establish an adequate plan for management.

Materials & Methods: We reviewed 16 PDS cases treated at our hospital between October 2007 and June 2019 and compared our results with the available evidence. Read More

Am J Dermatopathol 2021 May;43(5):362-364

Bennett Surgery Center, Santa Monica, CA.

Abstract: Radiation can induce changes to skeletal muscle cells that may mimic and thus be confused with cells of atypical fibroxanthoma (AFX), pleomorphic dermal sarcoma, spindle cell squamous cell carcinoma, and other spindle soft-tissue tumors. An 80-year-old White man presented for Mohs micrographic surgery of an AFX on the left lateral neck. The medical history was notable for a tongue squamous cell carcinoma 9 years before that had been treated with wide local excision, left neck dissection, and radiation to the oral cavity and left neck. Read More

J Cutan Pathol 2021 Feb 16;48(2):247-254. Epub 2020 Oct 16.

Department of Research, Institute for Biomedical Research of A Coruña (INIBIC). University of A Coruña (UDC), A Coruña, Spain.

Introduction: Connexins are transmembrane channel proteins that interconnect adjacent cells and allow the exchange of signaling molecules between cells and the extracellular milieu. They have been investigated in many tumors to obtain information about tumor nature, behavior, and prognosis.

Methods: Herein, we present a study on the immunohistochemical expression of connexin (Cx) 43 in 16 cases of atypical fibroxanthoma (AFX). Read More

Am J Dermatopathol 2020 Dec;42(12):972-976

Department of Pathology, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain; and.

Atypical fibroxanthoma is a rare mesenchymal skin tumor of intermediate malignancy that typically occurs on sun-damaged skin of elderly patients. Histologically, it is composed of pleomorphic cells with hyperchromatic nuclei and abundant cytoplasm, commonly arranged in a spindle cell pattern. Different histologic variants have been described during the past years. Read More

Pathologica 2020 Jun;112(2):105-109

Department of Medical Sciences and Public Health, Unit of Pathology, University of Cagliari, Italy.

Atypical fibroxanthoma (AFX) has been considered as the non-infiltrating precursor lesion of pleomorphic dermal sarcoma (PDS), which shows an aggressive clinical behavior, because of its extensive invasion of the deeper skin layers. Although these two tumors may represent two stages of the same disease, it can be difficult to differentiate between them, because of their similar clinical and histological features . Furthermore, they must be distinguished from a spindled variant of squamous carcinoma, melanoma and leiomyosarcoma . Read More

Hippokratia 2019 Oct-Dec;23(4):181-185

Department of Radiology, AHEPA University Hospital, Thessaloniki, Greece.

Background: Pleomorphic dermal sarcoma is a potentially high-grade cutaneous spindle cell tumor that closely resembles atypical fibroxanthoma in the superficial, dermal aspects but with adverse pathological features. Chronic inflammation, as several autoimmune disorders are co-associated with chronic myelomonocytic leukemia.

Case Description: We report here an 84-year-old male patient with swelling lump on the upper third of the left arm. Read More

J Am Acad Dermatol 2020 12 15;83(6):e429-e430. Epub 2020 Jul 15.

Division of Dermatology, Department of Medicine, Washington University in St. Louis, St Louis, Missouri. Electronic address:

Dermatol Online J 2020 May 15;26(5). Epub 2020 May 15.

San Diego Family Dermatology, National City, CA Touro University California College of Osteopathic Medicine, Vallejo, CA Scripps Family Medicine Residency, Scripps Mercy Hospital Chula Vista, Chula Vista, CA Family Medicine Residency, Family Health Centers of San Diego, San Diego, CA.

Pleomorphic dermal sarcoma is a cutaneous soft tissue sarcoma that presents as a rapidly enlarging tumor, typically on a sun-exposed location of elderly individuals. The neoplasm shares many similar features - clinical, pathologic, immunohistochemical and genomic - with atypical fibroxanthoma. However, adverse histologic characteristics (deep subcutaneous invasion, tumor necrosis, lymphovascular invasion, and/or perineural invasion) differentiate pleomorphic dermal sarcoma from atypical fibroxanthoma and may account for the more aggressive biologic behavior of pleomorphic dermal sarcoma: local recurrence and metastases. Read More

Dermatol Ther 2020 11 15;33(6):e13962. Epub 2020 Jul 15.

Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Dresden, Germany.

Atypical fibroxanthoma (AFX) is a rare, low-grade dermal sarcoma. We analyzed our files from January 2001 to January 2020 for AFX. Clinical parameters, histopathology, treatment and outcome have been investigated. Read More

J Cutan Pathol 2021 Feb 28;48(2):207-210. Epub 2020 Jul 28.

Department of Anatomic Pathology, Azienda Ospedaliero-Universitaria di Modena, Modena, Italy.

Background: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) share clinical, pathological, immunohistochemical and molecular features, though PDS is associated with a more aggressive behavior.

Methods: We reviewed 71 tumors fulfilling criteria for AFX and PDS to further stratify their biological potential.

Results: Lesions were mainly located on the scalps of elderly men, and were often ulcerated. Read More

Hum Pathol 2020 08 12;102:60-69. Epub 2020 Jun 12.

Department of Pathology, University of Michigan, 2800 Plymouth Road, Ann Arbor, MI, 48109, USA; Department of Dermatology, University of Michigan, 1500 E. Medical Center Drive, Ann Arbor, MI, 48109, USA; Rogel Cancer Center, University of Michigan, 1500 E. Medical Center Drive Ann Arbor, MI, 48109, USA. Electronic address:

Sarcomas on photodamaged skin vary in prognosis and management, but can display overlapping microscopic and immunophenotypic features. Improved understanding of molecular alterations in these tumors may provide diagnostic and therapeutic insights. We characterized 111 cutaneous sarcomatoid malignancies and their counterparts, including primary cutaneous angiosarcoma (n = 7), atypical fibroxanthoma (AFX) (n = 21), pleomorphic dermal sarcoma (PDS) (n = 17), extracutaneous undifferentiated pleomorphic sarcoma (n = 8), cutaneous leiomyosarcoma (LMS) (n = 5), extracutaneous LMS (n = 9), sarcomatoid squamous cell carcinoma (spindle cell squamous cell carcinoma) (S-SCC) (n = 24), and conventional cutaneous squamous cell carcinoma (SCC) (n = 20), by next-generation sequencing (NGS) using the StrataNGS panel for copy number variations, mutations, and/or fusions in more than 60 cancer-related genes. Read More

J Am Acad Dermatol 2021 Mar 1;84(3):794-796. Epub 2020 Jun 1.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address:

Cureus 2020 Apr 23;12(4):e7801. Epub 2020 Apr 23.

Plastic, Reconstructive and Hand Surgery, Northwell Health/Donald and Barbara Zucker School of Medicine, Staten Island University Hospital, Staten Island, USA.

Malignant fibrous histiocytoma (MFH) is an undifferentiated high-grade pleomorphic sarcoma and is considered the most common primary soft tissue sarcoma in adults. MFH is known to arise in the trunk, extremities and retroperitoneum although it can arise anywhere in the body.MFH of the skin is uncommon and even less frequent is the involvement of the scalp, especially with skull invasion. Read More

Case Rep Dermatol 2020 Jan-Apr;12(1):70-75. Epub 2020 Apr 8.

Department of Pathology, Zealand University Hospital, Roskilde, Denmark.

Cutaneous spindle cell squamous cell carcinoma (SpSCC) is a rare and often aggressive subtype of squamous cell carcinoma (SCC), which usually appears in sun-exposed areas, in areas that have received prior ionizing radiation, or in immunosuppressed individuals. SpSCCs are histologically characterized by keratinocytes infiltrating the dermis as single cells with elongated nuclei rather than as cohesive nests or islands and, in contrast to conventional SCC, are lacking features of keratinization. Immunohistochemical studies are useful to distinguish SpSCC from other spindle cell neoplasms, such as spindle cell/desmoplastic melanoma, cutaneous leiomyosarcoma, and atypical fibroxanthoma. Read More

ANZ J Surg 2020 11 27;90(11):2322-2324. Epub 2020 Apr 27.

Division of Cancer Surgery, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.

Background: Pleomorphic dermal sarcoma (PDS) is a rare, poorly defined skin neoplasm with features similar to atypical fibroxanthoma, but with adverse histopathological characteristics indicating metastatic potential such as tumour necrosis, invasion beyond superficial subcutis or vascular and/or perineural infiltration. Optimal treatment for PDS is uncertain and reported outcomes vary due to the rarity of this diagnosis and uncertainty over histopathological categorization. The aim of this study was to review the clinical and histopathological features of PDS in a single Australian centre. Read More

Hum Pathol 2020 05 1;99:107-115. Epub 2020 Apr 1.

Department of Pathology, Ospedale Pediatrico Bambino Gesú, Rome, 00165, Italy.

Fibrous histiocytoma (FH) or dermatofibroma is a common cutaneous lesion mostly seen in adults and rare in the first two years of life. Two hundred sixty-seven patients younger than 18 years with a diagnosis of FH or dermatomyofibroma, a lesion with morphologic overlap with FH, were identified from the files of a single institution, with only 13 (4.8%) occurring in patients younger than 5 years. Read More