Case Report: A 7 year-old boy with Xeroderma Pigmentosum (XP) and who presents a recurrent conjunctival atypical fibroxanthoma after two surgeries. This is the third procedure and the patient is treated with a surgical excision of the tumour and cryotherapy at the surgical bed. Due to the risk of recurrence, topical Mitomycin C 0,02% was added at post-operative care achieving a good clinical outcome. Read More
Atypical fibroxanthoma (AFX) is a rare spindle cell neoplasm predominantly found on the head and neck of elderly individuals with sun-damaged skin, with no evidence-based guidelines for their management. A systematic retrospective review of the literature focusing on treatment modality found a recurrence and metastasis rate of 8.0% (5. Read More
Clin Lab Med 2017 Sep 15;37(3):603-631. Epub 2017 Jun 15.
Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Mayo Clinic, Hilton 11, 200 First Street Southwest, Rochester, MN 55905, USA. Electronic address:
Fibrohistiocytic tumors are a diverse group of reactive and neoplastic lesions including xanthoma, fibrous histiocytoma and its variants, solitary xanthogranuloma, dermatofibrosarcoma protuberans, and atypical fibroxanthoma. This article reviews some of the more commonly encountered fibrohistiocytic tumors with an emphasis on clinical presentation, macroscopic and histologic characteristics, molecular/cytogenetic findings where applicable, and differential diagnoses. Read More
Opinion Statement: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) tumors share many clinical, etiologic, and histologic features and likely represent components of a tumor spectrum. In dermatologic oncology, differentiating between AFX and PDS is pivotal as tumors with histological features consistent with PDS are more likely to behave in a clinically aggressive manner. Importantly, the term "pleomorphic dermal sarcoma" (PDS) is a more appropriate designation than "undifferentiated pleomorphic sarcoma" (UPS) for describing deeper, more aggressive, histologically high-grade cutaneous tumors that otherwise resemble AFX. Read More
Atypical fibroxanthoma represents a low-grade sarcoma that usually presents in the elderly population on sun-damaged areas of the head and neck regions. This neoplasm is characterized on histology by atypical pleomorphic, epithelioid to spindled cells, arranged in a haphazard pattern, set within a background of solar elastosis. In this case report, we present a unique case of the rare variant of clear-cell atypical fibroxanthoma arising in an unusual place, specifically the lower extremities. Read More
Cutaneous sarcomas comprise a heterogeneous group of mesenchymal spindle cell tumors of the dermis and subcutis, one of the best-known entities being dermatofibrosarcoma protuberans. Other sarcomas addressed in this review include atypical fibroxanthoma, cutaneous undifferentiated pleomorphic sarcoma, leiomyosarcoma, liposarcoma, and angiosarcoma. With the exception of dermatofibrosarcoma protuberans, which has its peak incidence in middle-aged adults, cutaneous sarcomas usually occur in elderly individuals starting in the sixth or seventh decade of life. Read More
Purpose: To evaluate the suitability of treating atypical fibroxanthoma (AFX), an uncommon skin malignancy, with electronic brachytherapy.
Material And Methods: From Feb 2013 to Sep 2014, we were referred a total of 8 cases of AFX in 7 patients, all involving the scalp. All of them were treated with electronic brachytherapy 50 Kev radiations (Xoft Axxent(®), Fremont, California). Read More
Atypical fibroxanthoma (AFX) represents a rare mesenchymal neoplasm arising predominantly in the head and neck area of elderly patients. Clinically, the neoplasm is characterized by a rapid and exophytic growth with frequent ulceration of the epidermis. Histopathologically, AFX represents a well-circumscribed, dermal-based neoplasm composed of a variable admixture of large histiocytoid cells, enlarged spindled and epithelioid tumor cells, and multinucleated tumor giant cells with bizarre and pleomorphic nuclei. Read More
Squamous cell carcinoma (SCC) is the second most cutaneous malignancy after basal cell carcinoma (BCC) with increasing incidence. In the view its nodular manifestation; it can appear similar to nodular BCC, atypical fibroxanthoma, sclerosing liposarcoma and desmoplastic melanoma. Prognostic and therapeutic implications are different in all these conditions and thus their distinction becomes important. Read More
Since the initial description of the granular cell tumor in 1926, numerous other neoplasms, both benign and malignant, have been described to exhibit granular cell change. In most cases, diagnosis remains straightforward via recognition of retained histopathological morphology of the archetypal tumor, despite the presence of focal granular appearance. However, tumors with granular cell differentiation can present a diagnostic challenge either by mimicking alternative diagnoses, or by failing to exhibit architectural clues of the primary entity, thus requiring an immunohistochemical work-up. Read More
Dermatofibroma-like granular cell tumour (GCT) is a rare entity, with only two cases having been described so far. We report another case in a 62-year-old woman, discuss histopathological features, and review other tumours in which granular changes have been observed. Our tumour was composed predominantly of oval-to-spindle granular cells with prominent nucleoli, arranged in short fascicles and storiform pattern, infiltrating around collagen bundles. Read More
Atypical fibroxanthoma (AFX) is a histologic mimicker of a variety of spindle cell neoplasms, and careful microscopic and immunohistochemical evaluation is critical in establishing the correct diagnosis. Here we report the histologic and immunohistochemical work up of a 1 cm nodule involving the left dorsal hand of a 66-year-old patient. Light microscopy revealed fascicles of spindled and pleomorphic cells within the dermis showing increased mitotic activity occurring in the background of sun-damaged skin. Read More
*Division of Dermatology, Department of Medicine, Vanderbilt University, Nashville, Tennessee; †Department of Dermatology, University of Oklahoma, Oklahoma City, Oklahoma; ‡Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut.
Background: Atypical fibroxanthoma (AFX) is a rare cutaneous spindled cell neoplasm. For both diagnostic and therapeutic purposes, it is important to distinguish AFX from other poorly differentiated tumors, including undifferentiated pleomorphic sarcoma (UPS).
Objective: The authors aimed to identify the clinical, histologic, and immunohistochemical expression of LN2, ezrin, and CD10 in AFX and UPS tumors. Read More
Learning Objectives: After studying this article, the participant should be able to: 1. Identify clinical features of nonmelanoma skin cancer; 2. Distinguish low-risk versus high-risk basal cell carcinoma and squamous cell carcinoma; 3. Read More
*Department of Dermatology, Mayo Clinic, Rochester, Minnesota;†Division of Biomedical Statistics and Informatics, Mayo Clinic Rochester, Minnesota;‡Division of Dermatologic Surgery, Mayo Clinic, Rochester, Minnesota.
Background: The incidence of rare cutaneous malignancies is unknown. Current estimates of rare cutaneous malignancy incidences are based on broad epidemiologic data or single institution experiences, not population-based data.
Objective: To determine the incidence of several rare nonmelanoma skin cancers. Read More
Dermatofibromas are most frequently encountered in women on the lower extremities, often after minor trauma. A recurrent lesion of the right lower eyelid developed in a 64-year-old woman. It harbored "monster cells" that were large, with either multiple nuclei or a single, large, convoluted, and hyperchromatic nucleus. Read More
JAAD Case Rep 2016 Nov 5;2(6):445-447. Epub 2016 Dec 5.
Melanoma and Cutaneous Oncology Program, Huntsman Cancer Institute, University of Utah, Salt Lake City, Utah; Intermountain Health Care, Salt Lake City, Utah.
This review considers neoplastic lesions that originate in the skin, and which have the potential to imitate hematopoietic proliferations at a histological level. They include lymphoepithelioma-like carcinoma, Merkel cell carcinoma, benign lymphadenoma, pseudolymphomatous angiosarcoma, lymphadenoid dermatofibroma, lymphomatoid atypical fibroxanthoma, histiocytoid (epithelioid) hemangioma, and inflamed melanocytic lesions. The clinical and pathological features of those tumors are considered. Read More
Background: Recurrence rates after Mohs micrographic surgery (MMS) for rare cutaneous tumours are poorly defined.
Objective: To investigate the recurrence rate after MMS for rare cutaneous tumours at a university centre.
Methods & Materials: Retrospective review of all rare cutaneous tumours treated with MMS at a large university centre between January 2008 and December 2012. Read More
Aims: The diagnosis of undifferentiated pleomorphic sarcoma (UPS) may be challenging, as other lesions with undifferentiated spindle cell morphology must be excluded, including melanoma. Microphthalmia-associated transcription factor (MiTF) stains naevi and epithelioid melanomas, as well as some mesenchymal neoplasms. The aim of this study was to evaluate the prevalence of MiTF and melanocytic markers in UPS and a subset of atypical fibroxanthoma (AFX). Read More
Calogero Pagliarello, Francesca Peccerillo, Alfredo Zucchi, Rocco Giuseppe Tortorella, Roberto Ricci, Ignazio Stanganelli, Claudio Feliciani, Sergio Di Nuzzo, Section of Dermatology, Department of Clinical and Experimental Medicine, University of Parma, 43100 Parma, Italy.
Atypical fibroxanthoma (AFX) is a spindle cell neoplasm with low metastatic potential but high tendency to recur after surgery. Because of the rarity of this lesion and its aspecific clinical features, AFX could be easily misdiagnosed and undertreated by many clinicians who encounter them. Dermoscopy represents a valuable tool for easily assessing skin lesions, even though histological examination is required for final diagnosis. Read More
Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3. Read More
Introduction: The development of cutaneous neoplasms at immunization sites following vaccination is uncommon, and only few have been reported in the literature worldwide. We report an unusual case of an ulcerated giant dermatofibroma that developed as a chronic nonhealing plaque in the immunization scar of a young boy after vaccination.
Case Report: A 13-month-old Chinese boy presented with an unusual skin reaction on the vaccination site at the right anterolateral thigh following a routine intramuscular injection of '5-in-1' (diphtheria, tetanus, pertussis, polio and Haemophilus influenzae B) vaccine at 4 months of age. Read More
Academic Teaching Hospital Dresden-Friedrichstadt, Department of Dermatology and Allergology; Institute of Pathology "Georg Schmorl"; Department of Anesthesiology and Intensive Care Medicine, Emergency Medicine and Pain Management, Dresden, Germany.
Cellular fibrous dermatofibroma is a rare variant of dermatofibroma/histiocytoma. We present a 61 years old female with a slow-growing, firm tumor on the sole of her right foot. The tumor was removed by slow Mohs surgery within 2 cm negative margin. Read More
Objective: To investigate the clinicopathologic features, diagnosis and differential diagnosis of extranodal Rosai-Dorfman disease(RDD)of the breast.
Methods: Twelve cases of extranodal RDD of the breast were analyzed using hematoxylin-eosin stain and immunohistochemical staining.The morphological features and immunophenotype were observed by light microscopy, accompanied with a review of the literature. Read More
Keloidal atypical fibroxanthoma (KAF) has recently been categorized as a variant of atypical fibroxanthoma. This paper will emphasize the importance of including KAF in both clinical and histological differential diagnosis of benign and malignant lesions which exhibit keloidal collagen and will also review the current literature on epidemiology, pathogenesis, histology, immunochemistry and treatments. Read More
Background: Patients with xeroderma pigmentosum (XP) are strongly predisposed to the development of numerous cutaneous cancers. However, the extent of ocular pathology in these patients has not been adequately studied.
Methods: We conducted a retrospective study of tumors involving the ocular surface and ocular adnexa from 6 XP patients. Read More
Background: Atypical fibroxanthoma (AFX) is a pleomorphic tumor that most commonly presents in the elderly and arises on sun-exposed skin.
Methods: We report a case of a 24-year-old white male with xeroderma pigmentosum (XP), who developed an AFX of the conjunctiva, and review the literature.
Results: The patient presented with a rapidly growing conjunctival mass which was surgically excised and found to be AFX. Read More
Sebaceous carcinoma is a rare but serious malignancy that may be difficult to diagnose when poorly differentiated. Other epithelial tumors with clear cell change may mimic sebaceous carcinoma. Few useful or specific immunohistochemical markers for sebaceous differentiation are available. Read More
Background: Expression of p16 is frequently evaluated in melanocytic lesions. Expression of p16 in cutaneous histiocytic, fibrohistiocytic and undifferentiated lesions has not been well characterized.
Methods: We evaluated p16 expression in a cohort of histiocytic (reticulohistiocytoma, Langerhans cell histiocytosis, xanthogranuloma, Rosai Dorfman disease and xanthoma), fibrohistiocytic (dermatofibroma, epithelioid fibrous histiocytoma and dermatofibrosarcoma protuberans) and undifferentiated (atypical fibroxanthoma and pleomorphic undifferentiated sarcoma) lesions. Read More
Dermatofibromas are benign fibrohistiocytic tumors that involve the dermis. They have often a polymorphous clinical aspect, being frequently confused with other lesions varying from vascular tumors to melanoma. An important tool in diagnosis is represented by dermoscopy, which facilitates the recognition of dermatofibromas' characteristic structures such as central white patch and peripheral pigment network. Read More
Xeroderma pigmentosum (XP) is a rare, autosomal recessive disease involving a defect in DNA repair leading to the premature development of numerous aggressive cutaneous malignancies. Although atypical fibroxanthoma (AFX) is a neoplasm typically found in the setting of extensive sun exposure or therapeutic radiation, AFXs are rarely associated with children with XP. We report the case of a 13-year-old Guatemalan girl with the XP type C variant who developed one of the largest AFXs reported on a child's finger. Read More
Atypical fibroxanthoma is a malignant skin tumor with histologic features similar to those of undifferentiated pleomorphic sarcoma, but lacking its more aggressive behavior. The tumor is composed of pleomorphic cells with hyperchromatic nuclei and abundant cytoplasm, commonly arranged in a spindle cell pattern. Recent genetic studies have identified similarities between atypical fibroxanthoma and undifferentiated pleomorphic sarcoma, such as the presence of 9p and 13q deletions in both tumors, favoring a common histogenesis. Read More
Departments of *Dermatology, and †Pathology, Hospital del Mar, Parc de Salut Mar, Barcelona, Spain; and ‡Inflammatory and Cardiovascular Disorders Program, IMIM (Institut Hospital del Mar d'Investigacions Mèdiques), Barcelona, Spain.
Atypical fibroxanthoma (AFX) is considered a fibroblastic or myofibroblastic neoplasm probably corresponding to a superficial variant of undifferentiated pleomorphic sarcoma (UPS). However, an epithelial origin has also been postulated. An immunohistochemical study of the epithelial to mesenchymal transition (EMT) phenomenon was performed in a series of 19 AFX and 4 UPS to discern an epithelial origin. Read More
Atypical fibroxanthoma is considered to be a low-grade sarcoma, characterized by a proliferation of bizarre spindled cells. A case of a rare variant of this tumor, a clear-cell atypical fibroxanthoma, presenting with rapid growth on a 63-year-old female, is reported. The differential diagnosis of a clear cell proliferation and a review of the immunohistochemistry markers used in the diagnosis of atypical fibroxanthoma are discussed. Read More
Atypical fibroxanthoma (AFX) is a group of cutaneous tumors characterized by a population of fusiform, epithelioid and pleomorphic cells. Clinically, AFX is commonly found on the head and neck of older adults as a solitary ulcerated nodule. Clear cell atypical fibroxanthoma is a very rare variant of AFX, with only 13 cases reported to date. Read More
Background: Until now, almost nothing is known about the tumorigenesis of atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS). Our hypothesis is that AFX is the non-infiltrating precursor lesion of PDS.
Materials And Methods: We performed the world-wide most comprehensive immunohistochemical and mutational analysis in well-defined AFX (n=5) and PDS (n=5). Read More
Atypical fibroxanthoma (AFX) is an uncommon spindle cell tumor with intermediate or borderline malignant potential. Clinically, it may be misdiagnosed as a squamous cell carcinoma (SCC) or malignant melanoma. Solar irradiation has been implicated in its pathogenesis. Read More
*Department of Pathology, Hospital Universitario de Fuenlabrada, Madrid, Spain; †Department of Pathology, Hospital Universitario Fundación Alcorcón, Madrid, Spain; ‡Department of Pathology, Hospital Universitario de Getafe, Madrid, Spain; §Department of Pathology, Hospital Universitario 12 de Octubre, Madrid, Spain; ¶Department of Dermatology, Hospital Universitario de Fuenlabrada, Madrid, Spain; ‖Department of Pathology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain; **Department of Pathology, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain; and ††Department of Pathology, Hospital Universitario Infanta Sofía de San Sebastián de los Reyes, Madrid, Spain.
Atypical fibroxanthoma (AFX) is an uncommon dermal-based neoplasm arising on the sun-damaged skin of elderly people. Clear cell AFX is a rare variant with only 12 cases reported until the present date, all of them as case reports, except for 1 small series of 3 cases. The authors report 6 new cases and review the literature with special emphasis on the differential diagnosis. Read More
*Dermatopathology Division, Pathlab Bay of Plenty, Tauranga, New Zealand †Skin at Da Vinci Clinic, Tauranga, New Zealand ‡Clinipath Pathology, West Perth, Australia.
Prof. Uwe Wollina, MD, Department of Dermatology and Allergology, Dresden-Friedrichstadt Academic Teaching Hospital, Friedrichstrasse 41, 01067 Dresden, Germany;
The face is a common localization for skin malignancies. Mohs surgery or delayed Mohs surgery are considered the gold standard of treatment despite new drug developments. We analyzed our patient files over a three-year period. Read More
Objectives: Tumors and tumor-like lesions (TLL) of the paratesticular region are rare. Very few studies depicting the spectrum of lesions of this location are available in the literature. Malignant neoplasms arising in the paratesticular region represent only 7. Read More