Fibroxanthoma Research Articles

A Case of Multifocal Atypical Fibroxanthoma on the Leg

A Case of Multifocal Atypical Fibroxanthoma on the Leg

Atypical Fibroxanthoma With Regional Lymph Node Metastasis

Atypical fibroxanthoma (AFX) is a low-grade sarcoma usually occurring on sun-damaged skin of the head and neck in elderly patients. Metastatic disease has been reported very rarely. The potential aggressiveness of AFX is controversial. We describe herein a patient who developed metastatic disease in cervical lymph nodes. Our patient was an 87-year-old man with a 7-week history of a rapidly growing AFX presenting as a 1.5-cm sessile nodule on his right mandible. Two months following excision, the patient developed cervical lymphadenopathy. Histopathologic analysis of the cervical lymph nodes revealed spindle-cell tumors with histologic characteristics identical to those of the primary AFX, and the tumors were immunonegative for cytokeratin MNF-116 and S-100. In addition, we review and analyze cases from the literature and articles related to immunohistochemical stains used to diagnose AFX. Atypical fibroxanthoma is a diagnosis of exclusion, and only a small number of metastatic AFX cases have been reported. A review of the literature pertaining to immunohistochemical stains suggests the potential benefit of use of CD10, procollagen I, CD99, CD117, p63, and LN-2 in differentiating AFX from other spindle-cell tumors. The metastatic potential of AFX may not be fully appreciated, and clinicians should be reminded of its potential aggressive behavior.

Atypical fibroxanthoma – what is it, what it is not

Atypical fibroxanthoma – what is it, what it is not

Anti‐cytokeratin CAM 5.2 does not act as a surrogate of the cytokeratin 8/18 monoclonal antibody. Comment on: “Utility of p63 in the differential diagnosis of atypical fibroxanthoma and spindle cell squamous cell carcinoma”, in J Cutan Pathol 2009; 36: 543

Anti‐cytokeratin CAM 5.2 does not act as a surrogate of the cytokeratin 8/18 monoclonal antibody. Comment on: “Utility of p63 in the differential diagnosis of atypical fibroxanthoma and spindle cell squamous cell carcinoma”, in <i>J Cutan Pathol</i> 2009; 36: 543

Basal cell carcinoma and atypical fibroxanthoma: An unusual collision tumor

To the Editor: We report the case of an 85-year-old white female who presented to our dermatology department with a 2.5 cm × 1.8 cm asymptomatic erythematous plaque of 3 years' duration on the nose. The plaque had a translucent border and fine telangiectasias and was centred by a black/violaceous hemorrhagic nodule (Fig 1, A).

Atypical Fibroxanthoma: A Histological and Immunohistochemical Review of 171 Cases

The clinical and histological features of 171 atypical fibroxanthomas (AFX) from a single institution in Western Australia are outlined. This area experiences high levels of solar radiation, and all assessable biopsies showed solar elastosis. Patients were aged between 41 and 97 years (median age 74), with 76% of tumors occurring in men (male to female ratio approximately 3 to 1). Most tumors were small, with a median diameter of 10 mm and a range of 4-35 mm. Only 5% exceeded 20 mm in diameter. Most AFX were well-circumscribed dermal lesions, with limited invasion of subcutis in a minority. Histological variants identified included keloidal (n = 8), clear cell (n = 3), and granular cell (n = 3), plaque like (n = 4), and myxoid (n = 1). Bland cytological appearances (spindle cell nonpleomorphic AFX) were noted in 5 tumors, with osteoclast-like giant cells in 2. Features suggesting regression were present in 22 cases. Two cases recurred locally, none metastasized. No tumors expressed melanocytic or epithelial markers. Seventy-four percent of cases expressed smooth muscle actin, typically strongly and diffusely. No AFX stained with desmin. Only 1 of 50 cases was CD117 positive. In conclusion, AFX may show a wide range of histological appearances, and a panel of immunohistochemical markers is essential to make the correct diagnosis. Histological mimics, such as poorly differentiated squamous cell carcinoma, must be carefully excluded. Specific diagnosis is important because there seems to be a very low risk of recurrence or metastasis despite the frequently alarming histology.

Atypical fibroxanthoma of the scalp following hair transplantation in a 35-year-old male

Atypical fibroxanthoma (AFX) is an uncommon spindle cell neoplasm of the elderly. This case report presents an atypical case of AFX of the scalp 8 years after hair transplantation in a 35-year-old male patient. Possible synergistic effects of previous sun exposure radiation to the scalp, together with the thermal and radiation injury of carbon dioxide (CO(2)) laser, might explain the mechanisms of the development of AFX at such an early age. To the best of our knowledge, this case report is the first description in the medical literature of development of skin malignancy on a hair-transplanted scalp.

CD117 is Not a Useful Marker for Diagnosing Atypical Fibroxanthoma

CD117 is Not a Useful Marker for Diagnosing Atypical Fibroxanthoma

CD163 Expression Is Present in Cutaneous Histiocytomas but Not in Atypical Fibroxanthomas

CD163, a hemoglobin scavenger receptor, is expressed in monocytes and macrophages. Recent work has shown that this marker is specific for neoplasms of histiocytic differentiation. Our aim was to test the ability of CD163 to separate cutaneous histiocytomas from their morphologic mimics. We tested the expression of CD163 in 78 cases, including 19 xanthogranulomas, 16 atypical fibroxanthomas, 6 reticulohistiocytomas, 8 epithelioid cell histiocytomas, 9 cases of Langerhans cell histiocytosis, 10 xanthomas, and 10 intradermal Spitz nevi. CD163 expression was seen in all xanthogranulomas and reticulohistiocytomas, 4 epithelioid cell histiocytomas, 2 cases of Langerhans cell histiocytosis, and 8 xanthomas but was absent in atypical fibroxanthomas and Spitz nevi. CD163 is an excellent marker for confirming histiocytic differentiation and is useful in eliminating morphologic mimics such as Spitz nevi from the differential diagnosis. The lack of CD163 in atypical fibroxanthomas argues against a histiocytic origin for this tumor.

Keloidal and Other Collagen Patterns in Atypical Fibroxanthomas

Collagen deposition is observed in several cutaneous neoplasms and its derivation is variable. It can represent (1) a mesenchymal component of a biphasic tumor, (2) a desmoplastic host response to a neoplasm, or (3) a product of the tumor cells. The result is that collagenous (desmoplastic) variants of many cutaneous neoplasms are well recognized. In regard to atypical fibroxanthoma (AFX), conventional wisdom holds that intratumoral collagen is not commonly observed, apart from the rare sclerotic subtype of the neoplasm. This has not been our experience. The relatively frequent observation of keloidal collagen in AFXs and the dearth of information in the literature about this phenomenon prompted us to investigate the matter. All cases of AFX diagnosed at our institution within a 10-year period (1999-2008) were reviewed. The group included 64 males and 27 females with a mean age of 75 years. The majority of the cases (92%) were located on the head or neck. The conventional pleomorphic subtype of the tumor was encountered most frequently (67%). In 17 of the 91 cases (19%), prominent intratumoral keloidal collagen was present. This morphological observation poses interesting questions: (1) Is the collagen part of a host response to the neoplasm or is it a product of the tumor cells? (2) If the latter, does it cast light on the controversial histogenesis of AFX? (3) Does it signify an involutional stage of the tumor and imply a favorable prognosis? (4) Is it related to the "sclerotic" variant of the tumor? We discuss the above quandaries and emphasize the need to register this variant of AFX amongst other morphological subtypes of the tumor. The goal is to facilitate accuracy in diagnosis and to promote further investigation.

Squamous Cell Carcinoma With Osteoclast-Like Giant Cells: 2 Cases Misdiagnosed as Atypical Fibroxanthoma

Squamous Cell Carcinoma With Osteoclast-Like Giant Cells: 2 Cases Misdiagnosed as Atypical Fibroxanthoma

CD10‐positive myxofibrosarcomas: a pitfall in the differential diagnosis of atypical fibroxanthoma

CD10 is now commonly used to differentiate atypical fibroxanthoma (AFX) from melanoma, spindle cell and dedifferentiated variants of squamous cell carcinoma and leiomyosarcoma. However, we have encountered CD10-positive tumors that mimicked AFX but proved to be myxofibrosarcomas. The purpose of this study was to evaluate CD10 expression in a wide range of mesenchymal neoplasms that may involve the skin using tissue microarrays. Our results indicate that in addition to AFX, CD10 expression is common in myxofibrosarcomas, undifferentiated pleomorphic sarcomas, dermatofibromas and dermatofibrosarcoma protuberans. Myxofibrosarcomas commonly present in the skin and may be difficult to distinguish from AFX on small biopsies and CD10 positivity may confound the diagnostic difficulty.

CD10, p63 and CD99 expression in the differential diagnosis of atypical fibroxanthoma, spindle cell squamous cell carcinoma and desmoplastic melanoma

Atypical fibroxanthoma (AFX) is a pleomorphic spindle cell lesion of the skin; it is considered in the differential diagnosis with spindle cell malignant melanoma (MM) and sarcomatoid carcinoma/spindle cell squamous cell carcinoma (SCC). An optimum approach has yet to fully emerge with respect to the immunohistochemical discrimination of these lesions. Departmental archives from 1978 onwards were searched for clinicopathologically confirmed cases of AFX, MM and SCC. Immunostains for CD10, CD99 and p63 were performed in each case. Scored staining results were analyzed using Fisher's Exact Test. Twenty-seven of 31 cases of AFX were positive for CD10, as compared with 3 of 22 SCCs and 0 of 20 MMs. CD10 positivity was preferentially associated with the diagnosis of AFX (p < 0.001). p63 reactivity was observed in 15/22 cases of SCCs, 5/31 AFXs and 1/20 MMs. CD99 reactivity was observed in 3/31 cases of AFX, 2/22 SCCs and 3/20 MMs. CD10 positivity is relatively specific in this context for the diagnosis of AFX. Its utility is enhanced when only strong, diffuse membranocytoplasmic staining is considered as a positive result. In contrast to prior reports, p63 was not found to be highly sensitive for SCC. Similarly, CD99 showed no preferential staining of any single diagnostic group of lesions.

Primary and Metastatic Malignant Tumors of the Scalp

In recent years there have been a number of interesting advances in several topics relating to the diagnosis and treatment of cutaneous lesions with particular applicability to primary and metastatic malignancies of the scalp. In this article we provide a general update of advances in this field, and cover the more salient points relating to a variety of malignant tumors that have been reported to appear on the scalp as primary or metastatic lesions. A search and review of the literature on PubMed was made to identify and discuss relevant points relating to diagnosis and treatment of primary and metastatic tumors of the scalp. We describe the anatomy of the scalp, epidemiology of scalp tumors, theories of field cancerization and field therapy, photodynamic therapy, excisional surgical techniques and reconstruction, lymphoscintigraphy, chemoprevention, as well as details relating to atypical fibroxanthoma, Brooke-Spiegler syndrome, nevus sebaceus, cutaneous lymphoma, and metastatic disease. There is a very broad differential diagnosis for scalp nodules, which includes many different benign and malignant diseases, and treatment should be tailored accordingly. Given the potential for poor prognosis with some of the more aggressive malignancies that can be found in this anatomic area, the importance of a thorough physical examination cannot be emphasized enough, and early detection is critical to provide patients with the best chance for a favorable outcome.

Keloidal and granular cell change in a series of 171 atypical fibroxanthomas

Keloidal and granular cell change in a series of 171 atypical fibroxanthomas

Expression of Snail1 in the vimentin‐expressing squamous cell carcinoma mimicking atypical fibroxanthoma: possible involvement of an epithelial–mesenchymal transition

Expression of Snail1 in the vimentin‐expressing squamous cell carcinoma mimicking atypical fibroxanthoma: possible involvement of an epithelial–mesenchymal transition

Cutaneous Metastases of Malignant Melanoma: A Clinicopathologic Study of 192 Cases With Emphasis on the Morphologic Spectrum

Metastatic melanoma represents one of the most common types of cutaneous metastases. In up to 5% of patients, metastatic melanoma can be the first manifestation of the disease. For the most part, the histologic diagnosis of metastatic melanoma poses little diagnostic difficulty; however, some metastases may adopt unusual or unfamiliar appearances mimicking other benign and malignant conditions. We present a study of 192 cases of cutaneous metastatic melanomas with special emphasis on their spectrum of morphologic features. The patients were 115 men and 77 women, 23-91 years of age (mean 57 years). Most tumors were located on the proximal legs, scalp, and arms and ranged from 0.8 to 3.0 cm. One hundred ten cases showed the classic morphologic appearance of melanoma (well-circumscribed epithelioid dermal/subcutaneous nodule), 82 cases showed unusual histologic appearances that mimicked other benign and malignant neoplasms. In 16 patients (8.3%), there was no evidence of primary melanoma and the cutaneous metastasis was the only manifestation of the disease. The histologic diagnosis of cutaneous metastatic melanoma can pose difficulties for diagnosis, especially in the face of an unknown primary neoplasm. Unusual features observed in this series included examples of cutaneous metastatic melanoma that closely simulated metastatic carcinoma, dermatofibroma, leiomyosarcoma, angiosarcoma, nevoid melanoma, halo nevus, blue nevi, and atypical fibroxanthoma. Several cases also showed rhabdoid, balloon cell, and alveolar features. Immunohistochemical stains plus careful clinical history helped to establish the correct diagnosis. Our series illustrates that the differential diagnosis of cutaneous metastatic melanoma can be broad and difficult. To the best of our knowledge, this is the largest series of cutaneous metastatic melanomas reported in the literature.

Atypical fibroxanthoma presenting immunoreactivity against CD10 and CD99

Atypical fibroxanthoma presenting immunoreactivity against CD10 and CD99

Primary cutaneous myxoid spindle cell squamous cell carcinoma: a clinicopathologic study and review of the literature

Mucocutaneous squamous cell carcinoma (SCC) may rarely exhibit intracellular mucin production. Extracellular mucin production is an even rarer finding in SCC that is not well documented in the literature. Here, we report six cases of primary cutaneous and mucocutaneous SCC with prominent extracellular stromal mucin deposition and an epithelial spindle cell component. We propose the term 'yxoid spindle cell SCC' (MSC SCC) to describe the histologic characteristics of these six cases. We also propose a set of histologic and immunohistochemical findings for distinguishing MSC SCC from primary cutaneous and metastatic spindle cell neoplasms including other sarcomatoid carcinomas, myxoid sarcomas and the spindle cell variant of atypical fibroxanthoma (AFX). The criteria can also help discern MSC SCC from spindle cell melanomas, which may rarely show a prominent myxoid stroma. Given the small numbers of cases reported to date, the presence of prominent myxoid stroma in primary cutaneous spindle cell SCC has unknown prognostic significance at this time.

Atypical fibroxanthoma: a series of 25 cases

Atypical fibroxanthoma is a rare mesenchymal tumour of skin that develops on skin of elderly patients. We analysed our patients with atypical fibroxanthoma over the last 8 years. We analysed the pathology files of our hospital for the period 2001-2009. In all cases, histology and immunohistology were performed. Data on co-morbidities, treatment and outcome were obtained. We identified 25 patients (except two female patients, the rest were men) aged 52-95 years (mean: 79.5 years; standard deviation +/- 9.06 years). All tumours were localized in the head and neck region, except a single tumour on the shoulder. Fourteen patients had a cancer history, six had actinic keratoses or Bowen's disease (n = 1). Five patients had cardiac surgery or pacemaker, one each had a renal transplant, systemic sarcoidosis or non-Hodgkin's lymphoma. Medical history was positive for radiotherapy or chemotherapy in four patients. Histology showed a spindle-shaped dermal and subcutaneous tumour growth intermingled with multinucleated giant cells. The phenotype of tumour cells was vimentin-positive, but S100- and keratin-negative. Some tumours showed a focal expression of CD68. Complete microhistographic controlled surgery ('Mohs like') was possible in all cases followed by mesh-graft transplantation in three patients. Eighteen patients showed a complete remission. Four patients had a relapse within 2 years of follow-up treated by surgery. One patient is still under radiotherapy. All patients with a safety margin of 2 cm had no recurrence or relapse during follow-up. Micrographic controlled surgery with wide 2 cm safety margins is the treatment of choice. A regular follow-up for the next 5 years is recommended.