Malignant fibrous neoplasms of long bones: analysis of the surveillance, epidemiology, and end results database from 1973 to 2015.

BACKGROUND: Malignant fibrous neoplasms (MFN) of long bones are rare lesions. Moreover, the prognostic determinants of MFN of long bones have not been reported. This study aimed to present epidemiological data and analyse the prognostic factors for survival in patients with MFN. MATERIALS AND METHODS: The Surveillance, Epidemiology, and End Results (SEER) programme database was used to screen patients with malignant fibrous neoplasms (MFN) of long bones from 1973 to 2015, with attention to fibrosarcoma, fibromyxosarcoma, periosteal fibrosarcoma and malignant fibrous histiocytoma. The prognostic values of overall survival (OS) and cancer-specific survival (CSS) were assessed using the Cox proportional hazards regression model with univariate and multivariate analyses. The Kaplan-Meier method was used to obtain OS and CSS curves. RESULTS: A total of 237 cases were selected from the SEER database. Malignant fibrous histiocytoma was the most common form of lesion in long bones. Multivariate analysis revealed that independent predictors of OS included age, stage, tumour size and surgery. Age, stage, tumour size and surgery were also independent predictors of CSS. Additionally, the most significant prognostic factor was whether metastasis had occurred at the time of initial diagnosis. CONCLUSION: Among patients with MFN of long bones, age (> 60 years), tumour size (> 10 cm), distant stage, and non-surgical treatment are factors for poor survival.

Incidence rate and clinicopathological features of 62 atypical fibroxanthomas in a North-Western Spanish population.

BACKGROUND/OBJECTIVES: Atypical fibroxanthoma (AFX) is a mesenchymal neoplasm of unknown incidence. It has been determined that AFX is a tumour with low aggressiveness as long as it is properly diagnosed. Our objectives were to exclude pleomorphic dermal sarcomas or other skin tumours incorrectly diagnosed as AFX in our centre after applying strict diagnostic criteria and to assess the behaviour of appropriately diagnosed AFX. METHODS: We conducted an observational retrospective analysis of 73 patients diagnosed with AFX in our centre between 1998 and 2018. After selecting cases fulfilling AFX criteria, we made an analysis of predictive factors for local recurrence. Crude and sex-adjusted incidence rates were calculated. RESULTS: Out of 73 cases, 62 were eventually diagnosed as AFX. We examined for absence of tumour necrosis, lymphovascular or perineural invasion and infiltration of deep structures. Cytokeratin AE1-AE3, desmin and CD34 were negative in all cases. The remaining tumours were reclassified. The incidence of AFX in our health-care area was estimated at 0.59 cases every 100 000 inhabitants per year. In our series, 72.6% of the patients were men with mean age at diagnosis of 81 years. Average tumour diameter was 12 mm. The most common location was head and neck (96.8%). Only four local recurrences were detected over a mean of 47-month follow-up. CONCLUSIONS: We report a series of AFX in our health-care area. We verify its indolent course when it is properly diagnosed.

Atypical Fibroxanthoma: The Washington University Experience.

BACKGROUND: Atypical fibroxanthoma (AFX) is a rare dermal neoplasm typically occurring on sun-exposed skin in the elderly. As AFX remains a diagnosis of exclusion, updated characterization and treatment assessments are necessary to support informed diagnosis and management. OBJECTIVE: Characterization of contemporary AFX and surgical outcomes by Mohs micrographic surgery (MMS) and conventional local excision (LE). METHODS: Retrospective cohort analysis of all cases of AFX at our institution from January 2000 through July 2016. RESULTS: Among 75 cases with median age at diagnosis 73 years, most occurred on the head and neck (68) independent of age. Most treated cases (42) underwent MMS alone, with median tissue removal greater for LE (2.6 cm, 4.5 cm) than MMS (0.6 cm, 1.2 cm). Over a median 26 months of follow-up, 6 recurrences were observed among 50 cases, with metastases in 2 cases. Intent-to-treat recurrence rates were 3.4% for MMS and 25% for LE. One nonrecurrent and 2 recurrent cases received revised diagnoses after initial treatment, yielding a true recurrence rate of 8.5%. CONCLUSION: Despite diagnostic confounding by similar pathologies, surgical treatment of AFX remains effective. Tissue-sparing resection by MMS affords the potential for cosmetic and reconstructive advantage, without compromising recurrence compared with conventional excision.

Cellular Dermatofibroma: Clinicopathologic Review of 218 Cases of Cellular Dermatofibroma to Determine the Clinical Recurrence Rate.

BACKGROUND: Cellular dermatofibromas, a variant of dermatofibroma, are reported to recur at rates of 26% to 50%. OBJECTIVE: To determine whether there are distinct clinical or histological differences between cellular dermatofibromas that recur versus those that do not. To determine recurrence rates in a real-world clinical setting. MATERIALS AND METHODS: A retrospective analysis of the medical records and skin biopsies of cellular dermatofibroma in the University of Utah Health system between December 2011 and 2016. Clinical and dermatopathological features were evaluated to find distinct differences between the cellular dermatofibromas that recurred compared with those that did not. RESULTS: There were no significant differences in histology between the primary lesions in recurrent and nonrecurrent cases. One factor that seemed to be associated with a greater likelihood of recurrence was an initial lesion size greater than 1 cm. The authors' data suggest that if the margins are involved on initial biopsy, there is a 10% chance of recurrence. This percentage is far less than the 26% to 50% reported in the past literature. CONCLUSION: If a patient presents with a cellular dermatofibroma larger than 1 cm and positive margins at initial biopsy, a careful discussion should be had between the provider and patient about the low risk of local recurrence.

Asociación lupus eritematoso sistémico y esclerosis tuberosa, un caso / Coincidence of tuberous sclerosis and systemic lupus erythematosus-a case report

La esclerosis tuberosa (ET), también llamada enfermedad de Pringle Bourneville, es una facomatosis con posible afectación dérmica, neurológica, renal y pulmonar. Se caracteriza por el desarrollo de proliferaciones benignas en numerosos órganos, que dan lugar a las diferentes manifestaciones clínicas. Se asocia a la mutación de 2 genes: TSC1 (hamartina) y TSC2 (tuberina), con la alteración funcional del complejo diana de la rapamicina (mTOR). La activación de la señal mTOR ha sido descrita recientemente en el lupus eritematoso sistémico (LES), y su inhibición podría resultar beneficiosa en pacientes con nefritis lúpica. Presentamos el caso de una paciente que 30 años después del inicio de LES con afectación renal grave (glomerulonefritis tipo IV), resuelta con pulsos intravenosos de ciclofosfamida, comenzó con manifestaciones clínicas del complejo esclerosis tuberosa (CET). Consideramos de interés la coexistencia de estas 2 entidades, ya que solo hemos encontrado 2 casos similares en la literatura (AU)

Tuberous sclerosis, also called Bourneville Pringle disease, is a phakomatosis with potential dermal, nerve, kidney and lung damage. It is characterized by the development of benign proliferations in many organs, which result in different clinical manifestations. It is associated with the mutation of two genes: TSC1 (hamartin) and TSC2 (tuberin), with the change in the functionality of the complex target of rapamycin (mTOR). MTOR activation signal has been recently described in systemic lupus erythematosus (SLE) and its inhibition could be beneficial in patients with lupus nephritis. We report the case of a patient who began with clinical manifestations of tuberous sclerosis complex (TSC) 30 years after the onset of SLE with severe renal disease (tipe IV nephritis) who improved after treatment with iv pulses of cyclophosphamide. We found only two similar cases in the literature, and hence considered the coexistence of these two entities of great interest (AU)

Clinical and histological patterns of dermatofibroma without gross skin surface change: A comparative study with conventional dermatofibroma.

BACKGROUND: Dermatofibroma sometimes clinically presents as a nodular lesion without gross skin surface change. Clinicopathologic features of this variant of dermatofibroma have not been evaluated. AIMS: To assess clinicopathologic features of dermatofibroma presenting as a subcutaneous nodule. METHODS: This study reviewed the clinical and histological features of 42 cases of subcutaneous dermatofibromas and compared them with 95 cases of conventional dermatofibroma. RESULTS: Dermatofibroma without gross skin surface change was associated with a shorter pre-diagnosis duration than conventional dermatofibroma. Increase in size during the pre-diagnosis period was significantly more frequent in the conventional type. In addition, these dermatofibromas were more likely than the conventional type to occur in the head and neck region. Although tumor depth was deeper than in the conventional type, less than half of the dermatofibromas without gross skin surface change were found histologically to be "subcutaneous" or "deep-penetrating dermatofibroma". Subcutaneous extension was more frequent in these dermatofibromas while focal stromal hyalinization and hemosiderin deposits were more common in the conventional type. LIMITATIONS: This study is a retrospective, single center design. CONCLUSION: The present study suggests that dermatofibroma without gross skin surface change is a variant type with distinct clinical and histological features that distinguish them from conventional dermatofibroma.

Atypical fibroxanthoma: a series of 56 tumors and an unexplained uneven distribution of cases in southeast Germany.

BACKGROUND: Atypical fibroxanthoma is a rare mesenchymal tumor of the head and neck region. METHODS: We analyzed the files of 3 large dermatology hospitals from the years 2001 to 2013 in southeast Germany. RESULTS: We identified 53 patients (56 tumors) with a male predominance. The mean age was 78.0 years ± 8.3 years. Mohs surgery was performed in all cases resulting in complete remission in 45 patients. Five patients had a relapse within 2 years, and 4 developed metastases. None of the tumors with a safety margin of ≥2 cm relapsed. The majority of cases were found in the Dresden region. CONCLUSION: Atypical fibroxanthoma demonstrates an uneven geographic distribution in southeast Germany that demands further investigations. Mohs surgery with ≥2 cm safety margins is the treatment of choice. A regular follow-up is recommended.

Dermatosis en pacientes con sobrepeso y obesidad y su relación con la insulina / Skin Disorders in Overweight and Obese Patients and Their Relationship With Insulin

INTRODUCCIÓN: La prevalencia de obesidad se ha incrementado mundialmente en los últimos años. Existen estudios que describen las dermatosis que se asocian con la obesidad; sin embargo, existe poca evidencia de su asociación con los niveles de insulina. OBJETIVO: Describir las dermatosis presentes en pacientes con sobrepeso y obesidad y su asociación con los niveles de insulina. MATERIAL Y MÉTODOS: Se incluyeron pacientes de ambos sexos, mayores de 6 años, no diabéticos que acudieron a la consulta durante los meses de enero a abril de 2011. Todos los sujetos fueron valorados por un dermatólogo, se realizó exploración física, antropometría, historia médica, medicamentos y medición de glucosa e insulina de ayuno. Los pacientes se dividieron de acuerdo a sobrepeso y grado de obesidad y se compararon con Anova o Chi cuadrado, dependiendo del tipo de variable. Se realizó análisis de regresión para evaluar la independencia de las asociaciones. RESULTADOS: Fueron incluidos 109 pacientes (95 adultos y 13 niños; 83,5% mujeres), con edad media de 38 ± 14 años y un índice de masa corporal de 39,6 ± 8 kg/m2. Las dermatosis encontradas fueron: acantosis nigricans (97%), fibromas (77%), queratosis pilar (42%) e hiperqueratosis plantar (38%). Las que se asociaron de forma estadísticamente significativa con el grado de obesidad fueron acantosis nigricans (p = 0,003), fibromas (p = 0,001) e hiperqueratosis plantar. El grado de acantosis nigricans en el cuello, su topografía y el número de fibromas mostraron asociación significativa e independiente con los niveles de insulina. CONCLUSIONES: La acantosis y los fibromas deberían considerarse marcadores clínicos de hiperinsulinemia en población obesa y no diabética

INTRODUCTION: The prevalence of obesity has increased worldwide in recent years. Some authors have described skin conditions associated with obesity, but there is little evidence on the association between insulin levels and such disorders. OBJECTIVE: To describe the skin disorders present in overweight and obese patients and analyze their association with insulin levels. MATERIAL AND METHODS: The study included nondiabetic male and female patients over 6 years of age who were seen at our hospital between January and April 2011. All the patients were evaluated by a dermatologist, who performed a physical examination, including anthropometry, and reviewed their medical history and medication record; fasting blood glucose and insulin were also measured. The patients were grouped according to degree of overweight or obesity and the data were compared using analysis of variance or the χ2 test depending on the type of variable. The independence of the associations was assessed using regression analysis. RESULTS: In total, 109 patients (95 adults and 13 children, 83.5% female) were studied. The mean (SD) age was 38 (14) years and the mean body mass index was 39.6 ± 8 kg/m2. The skin conditions observed were acanthosis nigricans (AN) (in 97% of patients), skin tags (77%), keratosis pilaris (42%), and plantar hyperkeratosis (38%). Statistically significant associations were found between degree of obesity and AN (P = 0.003), skin tags (P = 0.001), and plantar hyperkeratosis. Number of skin tags, AN neck severity score, and AN distribution were significantly and independently associated with insulin levels. CONCLUSIONS: AN and skin tags should be considered clinical markers of hyperinsulinemia in nondiabetic, obese patients

Uncommon cutaneous neoplasms of the head and neck.

This article concentrates on the less-common cutaneous malignancies such as merkel cell, atypical fibroxanthoma, malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, microcystic adnexal carcinoma, and sebaceous carcinoma. The clinical and histopathologic descriptions of each, most current and emerging etiologies, diagnosis, staging, treatment, and prognosis are discussed.

Genetic skin diseases predisposing to basal cell carcinoma.

Basal cell carcinoma (BCC) is the commonest cancer in humans. Predisposing factors reflect common genetic variations and environmental influences in most cases. However, an underlying Mendelian disorder should be suspected in a specific subset of patients, namely those with multiple, early onset lesions. Some specific conditions, including Gorlin, Bazex-Dupré-Christol and Rombo syndromes, and Xeroderma Pigmentosum, show BCC as a prominent feature. In addition, BCC may represent a relatively common, although less specific, finding in many other genodermatoses. These include disorders of DNA replication/repair functions (Bloom, Werner, Rothmund-Thomson and Muir-Torre syndromes), genodermatoses affecting the folliculo-sebaceus unit (Brooke-Spiegler, Schöpf-Schulz-Passarge and Cowden syndromes), immune response (cartilage-hair hypoplasia and epidermodysplasia verruciformis) and melanin biosynthesis (oculocutaneous albinism and Hermansky-Pudlak syndrome), and some epidermal nevus syndromes. Further conditions occasionally associated with BCCs exist, but the significance of the association remains to be proven.

The significance of crystalline/chrysalis structures in the diagnosis of melanocytic and nonmelanocytic lesions.

BACKGROUND: Crystalline/chrysalis structures (CS) are white shiny streaks that can only be seen with polarized dermatoscopy. OBJECTIVES: We sought to estimate the prevalence and assess the clinical significance of CS in melanocytic and nonmelanocytic lesions. METHODS: This was a prospective observational study in which dermatoscopic assessment of lesions was recorded in consecutive patients examined during a 6-month period. In addition, a data set of biopsy-proven melanomas was retrospectively analyzed. RESULTS: In all, 11,225 lesions in 881 patients were prospectively examined. Retrospectively, 229 melanomas imaged with polarized dermatoscopy were analyzed. In the prospective data set, a median of 12.7 lesions (range, 1-54) were evaluated per patient. None of clinically diagnosed Clark nevi (n = 9750, 86.8%) demonstrated CS. Overall, CS were observed in 206 (1.8%) lesions, most commonly dermatofibromas and scars among nonbiopsied lesions. A total of 265 (2.4%) lesions were biopsied, including 20 melanomas and 36 nevi. Among biopsied malignant lesions, CS were most commonly observed in basal cell carcinoma (47.6%) and invasive melanomas (84.6%). Melanomas were more likely to have CS than biopsied nevi (odds ratio = 9.7, 95% confidence interval 2.7-34.1). In the retrospective data set, CS were more commonly observed among invasive melanomas (41%) compared with in situ melanomas (17%) (odds ratio = 3.4, 95% confidence interval 1.9-6.3, P < .001). The prevalence of CS correlated with increased melanoma thickness (P = .001). LIMITATIONS: Biopsied lesions represent a small percentage of the total number of lesions evaluated. CONCLUSION: Among biopsied malignant lesions, CS are most commonly observed in basal cell carcinoma and invasive melanomas and rarely seen in nevi. In melanoma, CS may reflect increased tumor thickness and progression.

Dermatofibroma y metástasis pulmonares. Tratamiento actual / Dermatofibroma Metastasizing to the Lung: Current Treatment

El dermatofibroma es una lesión cutánea común en la práctica dermatológica. La manifestación típica es una pápula de crecimiento lento, firme y solitaria. Desde el punto de vista histopatológico, se define en ocasiones como una reacción del tejido conectivo dérmico y en otras como una proliferación neoplásica benigna. En 1990 se publicó por primera vez la existencia de metástasis pulmonares de este tumor. Desde entonces se han recogido en todo el mundo 12 casos de similares características. Presentamos el caso de una mujer joven con un dermatofibroma recidivante en el hombro y metástasis pulmonares bilaterales. Analizamos qué características histológicas del tumor podrían hacer sospechar un comportamiento posterior inusualmente agresivo(AU)

Dermatofibromas are very common skin tumors. Their typical presentation is as a slow-growing, firm, solitary papule. They have been described histopathologically as a reaction of the connective tissue of the skin or as a benign neoplasm. Cases of these tumors metastasizing to the lung were first reported in 1990. Since then 12 cases with similar characteristics have been reported worldwide. We present the case of a young woman with a recurrent dermatofibroma on the shoulder that metastasized to both lungs. We discuss the histologic characteristics of this tumor that could raise suspicion of unusually aggressive behavior(AU)

Fibrohistiocitoma maligno «inflamatorio» de cordón espermático con infiltración sobre leioma inguinal. Presentación de 1 caso con revisión conceptual y de la literatura / Inflammatory malignant fibrous histiocytoma of the spermatic cord infiltrating an inguinal leiomyoma. Case report with conceptual and bibliographic reviews

Objetivo: Estudiar los fibrohistiocitomas malignos (FHM) paratesticulares desde el punto de vista clínico-histológico, inmunohistoquímico y su histogénesis. Métodos: A través de Medline y búsqueda no indexada de la literatura científica internacional hemos encontrado un total de 77 casos de FHM paratesticulares que globalmente plantean problemas diagnósticos, terminológicos y terapeúticos. Resultado: Incluímos el caso número 78 de FHM de cordón espermático que presenta unas especiales características (no descritas hasta la fecha) de fistulización cutánea e infiltración de un leiomioma inguinal, con confirmación histológica e inmunohistoquímica. Realizamos una revisión conceptual de este tipo de neoplasias incluyendo la valoración de las contradicciones y reclasificaciones que ha sufrido desde su primera descripción, lo que hace que sean subestimados y su prevalencia real en las series internacionales sea muy variable: del 7 al 37% de los sarcomas paratesticulares. También, el hecho de su baja incidencia ha condicionado una carencia de protocolos de diagnóstico y tratamiento. Conclusiones: Los tumores paratesticulares, aunque infrecuentes, tienen una alta tasa de malignidad. El concepto de FHM ha variado a lo largo de los últimos 15 años. Hoy, este término, es sinónimo de sarcoma pleomórfico indiferenciado y ha llegado a ser un diagnóstico de exclusión (Vimentina + / CD 68 + -ocasionalmente- sin diferencias ultraestructurales), reservándose el subtipo inflamatorio para aquellos que tienen un componente inflamatorio e histiocítico. Es imprescindible en esta localización la realización de una biopsia intraoperatoria, ya que incluso es difícil precisar preoperatoriamente, si una lesión es benigna o maligna. El diagnóstico diferencial tan solo se resuelve tras el estudio microscópico e inmunohistoquímico. Un leiomioma próximo a esta localización (inguinal) podría diagnosticarse y confundirse con un nódulo del FHM principal. Su inmunofenotipo nos permitió diagnosticarlo no solo como leiomioma (marcadores de actinas y desmina positivos), sino también observar la infiltración por el FHM (AU)

Objectives: To study paratesticular malignant fibrous histiocytomas (FHM) from the clinical, histological, immunohistochemical and histogenetic, point of view. Methods: Through Medline and not index-linked search of international scientific literature we have found a total of 77 cases of paratesticular FHM that globally create diagnostic, terminological and therapeutic problems. Results: We include the case number 78 of FHM of spermatic cord, that presents a few special characteristics (not described before) of cutaneous fistula and infiltration of an inguinal leiomyoma, with histological and immunohistochemical confirmation. We performed a conceptual review of this type of tumours, including the evaluation of contradictions and reclassifications that has undergone from its first description, which causes that they are underestimated and their real prevalence in international series is very variable: from 7 to 37 % of paratesticular sarcomas. Also, the fact of their low incidence has determined the lack of protocols for diagnosis and treatment. Conclusions: Paratesticular tumours, although infrequent, have a high rate of malignancy. The FHM concept has varied throughout the last 15 years. Today, this term is synonymous of pleomorphic undifferentiated sarcoma and has been an exclusion diagnosis (Vimentin [+] / CD 68 [+] -occasionally- without ultrastructural differences), reserving the inflammatory subtype for which they have an inflammatory and histiocytic component. The accomplishment of an intraoperatory biopsy is essential in this location, since it is even difficult to know preoperatively, if a lesion is benign or malignant. The differential diagnosis is only solved after microscopic and inmunohistochemical study. Leiomyoma next to this location (inguinal) could be diagnosed and confused with a nodule of the main FHM. Their immunophenotype allowed us to diagnose it not only as leiomyoma (myogenic markers were positive -non skeletal muscle-), but also to observe the infiltration by the FHM (AU)

Dermoscopy of dermatofibromas: a prospective morphological study of 412 cases.

OBJECTIVE: To describe the dermoscopic features, including vascular structures and patterns associated with dermatofibromas in a large series of cases. DESIGN: Digital dermoscopic images of the prospectively collected dermatofibromas were evaluated for the presence of multiple structures and patterns. SETTINGS: Dermatofibromas were collected in the Departments of Dermatology of the Hospital de Sant Pau i Santa Tecla, Tarragona, Spain, and Hospital de Sant Llatzer, Palma de Mallorca, Spain. PATIENTS: A total of 412 dermatofibromas (from 292 patients) with complete documentation were collected. MAIN OUTCOME MEASURES: Frequency and intraobserver and interobserver agreement of the dermoscopic structures and patterns in dermatofibromas. RESULTS: A total of 19 morphological dermoscopic structures were evaluated. Pigment network was observed in 71.8% (3% atypical pigment network), white scarlike patch in 57.0%, and a white network in 17.7%. Different vascular structures were observed in 49.5% (dotted vessels in 30.6%). Ten dermoscopic patterns were observed. The most common pattern seen in our series (34.7% of cases) was central white patch and peripheral pigment network, but in 65.3% of the cases, dermatofibromas presented different patterns including simulators of melanoma. CONCLUSION: The most common pattern associated with dermatofibroma is the classic dermoscopic pattern (pigment network and central white patch), but this tumor has a wide range of presentations.

Incidence patterns of soft tissue sarcomas, regardless of primary site, in the surveillance, epidemiology and end results program, 1978-2001: An analysis of 26,758 cases.

Soft tissue sarcomas (STS) are a heterogeneous group of uncommon tumors that show a broad range of differentiation that may reflect etiologic distinction. Routine tabulations of STS are not morphology-specific. Further, the lack of inclusion of sarcomas arising in all organs in most standard evaluations underestimates the true rates. We analyzed the 1978-2001 Surveillance, Epidemiology and End Results program incidence rates of STS regardless of primary site, except bones and joints, using the 2002 criteria of the WHO classification. There were 26,758 cases available for analysis. Leiomyosarcomas accounted for 23.9%, malignant fibrous histiocytomas 17.1%, liposarcomas 11.5%, dermatofibrosarcomas 10.5%, rhabdomyosarcomas 4.6% and angiosarcomas 4.1%. Almost half (47.9%) of the sarcomas arose in the soft tissues, 14.0% in the skin and 7.0% in the uterus. Overall, incidence rates were highest among black women (6.26/100,000 woman-years) and the lowest among white women (4.60/100,000). Age-adjusted rates increased at 1.2% and 0.8% per year among white males and females, respectively, both trends statistically significant, while rates among blacks declined slightly. About 40% of leiomyosarcomas among women were uterine in origin, with a black/white rate ratio of 1.7. This rate ratio increased to 2.0 when we accounted for the lower prevalence of intact uteri among black compared to white women. Total STS rates rose exponentially with age. Rates for both uterine leiomyosarcoma and dermatofibrosarcoma increased rapidly during the childbearing years, peaking at about age 40 and 50, respectively. Incidence patterns of STS varied markedly by histologic type, supporting the notion that these tumors may be etiologically distinct.

Fibroxantoma atípico. Estudio clinicopatológico de 10 casos / Atypical fibroxanthoma. Clinical/pathological study of 10 cases

Introducción. El fibroxantoma atípico (FXA) es un tumor poco frecuente de histogénesis incierta, considerado por la mayoría de los autores como la variante superficial del histiocitoma fibroso maligno (HFM). El objetivo principal de este trabajo es presentar las características clínicas e histológicas de una serie de 10 pacientes. Material y métodos. Los datos fueron recogidos retrospectivamente de la historia clínica informatizada. Se analizan variables clínicas (edad de aparición, tiempo hasta el diagnóstico, localización, patología acompañante, evolución), histológicas (patrón arquitectural, celularidad, ulceración, invasión vascular o perineural, afectación tejido celular subcutáneo, pleomorfismo, mitosis, infiltrado inflamatorio acompañante) e inmunohistoquímica. Casos clínicos. Las características clinicoepidemiológicas coinciden en general con las publicadas en la literatura especializada: edad avanzada de aparición, poco retraso hasta el diagnóstico, afectación de piel con daño actínico y buena evolución. Histológicamente todos los casos presentaban un predominio fusocelular, dispuesto en un patrón vagamente estoriforme junto con células gigantes y células poligonales eosinófilas. Discusión. El diagnóstico es siempre de exclusión, y deben diferenciarse mediante inmunohistoquímica de otros tumores fusocelulares como carcinomas epidermoides, melanomas, leiomiosarcomas o dermatofibrosarcoma protuberans entre otros. A pesar de ser un tumor poco frecuente es preciso conocerlo para evitar tratamientos agresivos e innecesarios

Introduction. Atypical fibroxanthoma (AFX) is a rare tumor of unknown histogenesis, considered by most authorities as a superficial form of malignant fibrous histiocytoma (MFH). The aim of this work is to report the clinicopathological features of 10 cases of AFX. Material and methods. Data were retrospectively collected of the computerized medical history. Clinical (age, onset-diagnosis time, location, accompanying pathology, outcome), histological (architectural pattern, cell type, ulceration, vascular or perineural invasion, subcutis involvement, pleomorphism, mitosis, inflammatory infiltrate) and immunohestochemical variable were analyzed. Cases report. Clinical and epidemiological features coincide with those previously reported: onset late in life, short time onset-diagnosis, involvement of skin with notable sun damage and a good outcome. Pathologically all the cases showed a spindle-cell prevalence arranged in a vaguely storiform pattern, along with both, multinucleated and eosinophilic cells. Discussion. The diagnosis of AXF is always of exclusion. Other spindle-cell tumors such as squamous cell carcinoma, malignant melanoma, leyomiosarcoma or dermatofibrosarcoma protuberans must be ruled out by immunohistochemical techniques. In spite of its rarity, the recognition of AFX is important in order to avoid inappropriately aggressive treatment