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Background: Dermatofibrosarcoma protuberans (DFSP) is a superficial soft tissue sarcoma, and surgical excision is the first-line treatment. The aim of this systematic review is to provide an update about the current indications and clinical results regarding the use of postoperative radiotherapy in DSFP, considering both adjuvant and salvage setting. Methods: We conducted a systematic literature review using the main scientific database, including Cochrane library, Scopus, and PubMed, for any relevant article about the topic, and we considered all available papers without any time restriction. Results: Twenty-two papers, published between 1989 and 2023, were retrieved and considered eligible for inclusion in this review. Regarding the fractionation schedules, most authors reported using standard fractionation (2 Gy/die) with a wide total dose ranging from 50 to 70 Gy. The local control after postoperative radiotherapy was excellent (75–100%), with a median follow-up time of 69 months. Conclusions: After the primary surgical management of DFSP, postoperative radiotherapy may either be considered as adjuvant treatment (presence of risk factors, i.e., close margins, recurrent tumours, aggressive histological subtypes) or as salvage treatment (positive margins) and should be assessed within the frame of multidisciplinary evaluation.

Fibroblastic connective tissue nevus (FCTN) is a rare, benign, and recently described dermal mesenchymal lesion characterized by CD34-positive spindle cells. We present a case of FCTN on the upper back of a 9-month-old boy who was diagnosed with a benign lipoma by ultrasound.

Fibroblastic connective tissue nevus (FCTN) is a rare, benign, and recently described dermal mesenchymal lesion characterized by CD34-positive spindle cells. We present a case of FCTN on the upper back of a 9-month-old boy who was diagnosed with a benign lipoma by ultrasound.

Objective: To teach how to perform a comprehensive assessment and nursing care plan for a skin tumor (Dermatofibrosarcoma Protuberans) according to the AREA and DREOM model. Methods: Clinical review of the skin tumor under study and pedagogical presentation of a case. Holistic assessment using Marjory Gordon's 11 Functional Patterns, elaboration of the care plan according to NANDA-NOC-NIC taxonomy, application of the AREA model in the selection of diagnoses and DREOM model in the identification of outcomes and interventions. Different validated scales were used to support the objective assessment. Results: Dermatofibrosarcoma Protuberans is a cutaneous sarcoma of low incidence, but with a high rate of recurrence. This tumor has an important capacity for local infiltration that can affect the subcutaneous and integumentary tissue, so the recommended treatment is complete excision or resection. The care of the postoperative surgical wound after tumor excision, its sequelae, as well as the biopsychosocial approach, are topics of study that the literature does not address in depth. In this case, 10 NANDA-I diagnoses were initially identified, prioritizing 3 of them according to the AREA model, in relation to problems of mobility, self-perception and tissue injury. The care plan assesses 8 NOC outcomes and applies 13 NIC interventions, according to the DREOM model. Conclusions: The prognosis of Dermatofibrosarcoma Protuberans after surgical treatment is good; however, there are cases with lymph node involvement or distant metastases that impair the patient's quality of life due to severe sequelae. The application of the AREA and DREOM models is a standardized methodology that allows us to identify the best nursing action guidelines (care), through a daily reality in patients with skin tumors who, after an aggressive surgical intervention, present multiple problems of biopsychosocial affectation. We believe that the results obtained can be valuable information that can se, Objetivo: Enseñar a realizar una valoración integral y plan de cuidadosde enfermería ante un tumor cutáneo (Dermatofibrosarcoma Protuberans) según el modelo AREA y DREOM. Métodos: Revisión clínica del tumor cutáneo a estudio y exposición pedagógica de un caso. Valoración holística mediante los 11 Patrones Funcionales de Marjory Gordon, elaboración del plan de cuidados según taxonomía NANDA-NOC-NIC, aplicación del modelo AREA en la selección de diagnósticos y modelo DREOM en la identificación de resultados e intervenciones. Se utilizaron diferentes escalas validadas para apoyar la valoración objetiva. Resultados: El Dermatofibrosarcoma Protuberans es un sarcoma cutáneo de baja incidencia, pero con alto índice de recidivas. Este tumor tiene una importante capacidad de infiltración local que puede afectar al tejido subcutáneo y tegumentario, por lo que el tratamiento recomendado es la extirpación completa o resección. Los cuidados de la herida quirúrgica postoperatoria tras la exéresis del tumor, sus secuelas, así como el abordaje biopsicosocial, son temas de estudio que la literatura no aborda en profundidad. En el caso planteado se identificaron inicialmente 10 diagnósticos NANDA-I, priorizando 3 de ellos según el modelo AREA, en relación con los problemas de movilidad, autopercepción y lesión tisular. El plan de cuidados valora 8 resultados NOC y aplica 13 intervenciones NIC, según el modelo DREOM. Conclusiones: El pronóstico del Dermatofibrosarcoma Protuberans tras el tratamiento quirúrgico es bueno; sin embargo, hay casos con afectación ganglionar o metástasis a distancia que merman la calidad de vida del paciente por las graves secuelas. La aplicación de los modelos AREA y DREOM es una metodología estandarizada que permite identificar las mejores pautas de actuación enfermera (cuidados), mediante una realidad cotidiana en pacientes sometidos a tumores cutáneos que, tras una intervención quirúrgica agresiva, presentan múltiples problemas de afectación a nivel biop

Fibroblastic connective tissue nevus (FCTN) is a rare, benign, and recently described dermal mesenchymal lesion characterized by CD34-positive spindle cells. We present a case of FCTN on the upper back of a 9-month-old boy who was diagnosed with a benign lipoma by ultrasound.

Fibroblastic connective tissue nevus (FCTN) is a rare, benign, and recently described dermal mesenchymal lesion characterized by CD34-positive spindle cells. We present a case of FCTN on the upper back of a 9-month-old boy who was diagnosed with a benign lipoma by ultrasound.

Fibroblastic connective tissue nevus (FCTN) is a rare, benign, and recently described dermal mesenchymal lesion characterized by CD34-positive spindle cells. We present a case of FCTN on the upper back of a 9-month-old boy who was diagnosed with a benign lipoma by ultrasound.

The thoracoabdominal flap is a rotation flap, and is well known for reconstruction of defects following resections for breast cancer, but the flap is not well known for reconstructing defects following resections of soft-tissue sarcomas involving the chest wall. Here we present two patients with superficial chest wall sarcomas consisting of a dermatofibrosarcoma protuberans in a 42-year-old man and a recurrent myxofibrosarcoma in a 76-year-old man. The tumors were resected with the surrounding tissue. The defect was reconstructed with a thoracoabdominal flap elevated from the ipsilateral thorax (medially-based flap). Neither case developed necrosis of the flap or reduced shoulder range of motion. The chest wall presents few options for a donor vessel. The thoracoabdominal flap has an axial blood supply and does not require a microsurgical procedure. A thoracoabdominal flap is a suitable reconstruction option for a defect after the resection of a superficial soft-tissue sarcoma in the chest wall.

We present an 81-year-old man who presented for evaluation of an indurated plaque with an exophytic, pearly, skin-red-brown colored nodule with central ulceration on his chest that evolved over the course of several months and was initially suspected to be basal cell carcinoma. Biopsy demonstrated histological features of dermal spindle cell proliferation in a storiform fashion with CD34 positivity confirming a diagnosis of dermatofibrosarcoma protuberans (DFSP). Dermatofibrosarcoma protuberans are rare, slowly progressive soft tissue sarcomas. The rate of DFSP is greatest among African Americans (8.3/1,000,000), occurring nearly twice as frequently when compared to Caucasians. Aside from race/ethnicity, age, and skin trauma, no specific risk factors are associated with DFSP. Complete excision is curative. Given its pearly skin colored appearance, papular/nodular/atrophic morphology variants, and tendency to form indurated plaques, DFSP may be mistaken for nodular and morpheaform basal cell carcinoma subtypes, as well as a variety of other conditions. This case highlights the importance of maintaining DFSP on the differential diagnosis of slowly progressive nodules and indurated plaques, especially in African Americans.

We present an 81-year-old man who presented for evaluation of an indurated plaque with an exophytic, pearly, skin-red-brown colored nodule with central ulceration on his chest that evolved over the course of several months and was initially suspected to be basal cell carcinoma. Biopsy demonstrated histological features of dermal spindle cell proliferation in a storiform fashion with CD34 positivity confirming a diagnosis of dermatofibrosarcoma protuberans (DFSP). Dermatofibrosarcoma protuberans are rare, slowly progressive soft tissue sarcomas. The rate of DFSP is greatest among African Americans (8.3/1,000,000), occurring nearly twice as frequently when compared to Caucasians. Aside from race/ethnicity, age, and skin trauma, no specific risk factors are associated with DFSP. Complete excision is curative. Given its pearly skin colored appearance, papular/nodular/atrophic morphology variants, and tendency to form indurated plaques, DFSP may be mistaken for nodular and morpheaform basal cell carcinoma subtypes, as well as a variety of other conditions. This case highlights the importance of maintaining DFSP on the differential diagnosis of slowly progressive nodules and indurated plaques, especially in African Americans.

[Resumen] Introducción: El Dermatofibrosarcoma Protuberans es un sarcoma cutáneo de baja incidencia, lento crecimiento, indoloro y escasa tendencia a la metástasis, pero con alto índice de recidivas. Este tumor tiene una importante capacidad de infiltración local que puede afectar al tejido subcutáneo y tegumentario, por lo que el tratamiento recomendado es la extirpación completa o resección. El pronóstico tras el tratamiento quirúrgico es bueno; sin embargo, hay casos con afectación ganglionar o metástasis a distancia que merman la calidad de vida del paciente y pueden provocar la muerte. Los cuidados de la herida quirúrgica postoperatoria tras la exéresis del tumor, sus secuelas, así como el abordaje biopsicosocial, son temas de estudio que la literatura no aborda en profundidad. Objetivo: Realizar una valoración integral y plan de cuidados postoperatorio de una paciente intervenida por un Dermatofibrosarcoma Protuberans en pierna, con resultado de injerto cutáneo y linfedema iatrogénico, desde atención primaria. Metodología: Caso clínico. Valoración mediante los 11 Patrones Funcionales de Marjory Gordon, elaboración del plan de cuidados según taxonomía NANDA-NOC-NIC, aplicación del modelo AREA en la selección de diagnósticos y modelo DREOM en la identificación de resultados e intervenciones. Complementariamente se utilizaron diferentes escalas validadas para apoyar la valoración objetiva. Resultados: Se identificaron inicialmente 10 diagnósticos NANDA, priorizando 3 de ellos en relación con los problemas de movilidad, autopercepción y lesión tisular. El plan de cuidados incluye 8 resultados NOC y 13 intervenciones NIC. Actualmente está resuelto satisfactoriamente el diagnóstico del deterioro de la integridad tisular. Respecto al deterioro de la movilidad física y al trastorno de la imagen corporal, las acciones llevadas a cabo han mejorado el estado de la paciente, pero no se alcanza la resolución plena. Conclusiones: El caso clínico nos ha permitido identificar, [Resumo] Introdución: O Dermatofibrosarcoma Protuberans é un sarcoma cutáneo de baixa incidencia, lento crecemento, indoloro e escasa tendencia á metástase, pero con alto índice de recidivas. Este tumor ten unha importante capacidade de infiltración local que pode afectar ao tecido subcutáneo e tegumentario, polo que o tratamento recomendado é a extirpación completa ou resección. O prognóstico tras o tratamento cirúrxico é bo; con todo, hai casos con afectación ganglionar ou metástase a distancia que minguan a calidade de vida do paciente e poden provocar a morte. Os coidados da ferida cirúrxica postoperatoria tras a exéresis do tumor, as súas secuelas, así como a abordaxe biopsicosocial, son temas de estudo que a literatura non aborda en profundidade. Obxectivo: Realizar unha valoración integral e plan de coidados postoperatorio dunha paciente intervida por un Dermatofibrosarcoma Protuberans en perna, con resultado de enxerto cutáneo e linfedema iatroxénico, desde atención primaria. Metodoloxía: Caso clínico. Valoración mediante os 11 Patróns Funcionais de Marjory Gordon, elaboración do plan de coidados segundo taxonomía NANDA-NOC-NIC, aplicación do modelo AREA na selección de diagnósticos e modelo DREOM na identificación de resultados e intervencións. Complementariamente utilizáronse diferentes escalas validadas para apoiar a valoración obxectiva. Resultados: Identificáronse inicialmente 10 diagnósticos NANDA, priorizando 3 deles en relación cos problemas de mobilidade, autopercepción e lesión tisular. O plan de coidados inclúe 8 resultados NOC e 13 intervencións NIC. Actualmente atópase resolto satisfactoriamente o diagnóstico da deterioración da integridade tisular. Respecto á deterioración da mobilidade física e ao trastorno da imaxe corporal, as accións levadas a cabo melloraron o estado da paciente, pero non se alcanza a resolución plena. Conclusións: O caso clínico permitiunos identificar as pautas de actuación enfermeira (coidados), mediante unha realidade, [Abstract] Introduction: Dermatofibrosarcoma Protuberans is a skin sarcoma with low incidence, slow growth, painless and little tendency to metastasis, but with a high rate of recurrence. This tumor has a significant local infiltration capacity that can affect the subcutaneous and tegumentary tissue, so the recommended treatment is complete removal or resection. The prognosis after surgical treatment is good; however, there are cases withnodal involvement or distant metastases that impair the patient’s quality of life and can lead to death. Post-operative surgical wound care after tumor exeresis, its sequelae, as well as the biopsychosocial approach, are subjects of study that the literature does not address in depth. Objective: To perform a comprehensive assessment and post-operative care plan of a patient undergoing surgery for Dermatofibrosarcoma Protuberans in the leg, resulting in skin grafting and iatrogenic lymphedema, from primary care. Methodology: Clinical case. Assessment using Marjory Gordon's 11 Functional Patterns, elaboration of the care plan according to NANDA-NOC-NIC taxonomy, application of the AREA model in the selection of diagnoses and DREOM model in the identification of results and interventions. Complementary to this, different validated scales were used to support the objective assessment. Results: 10 NANDA diagnoses were originally identified, prioritizing 3 of them in relation to mobility problems, self-perception and tissue injury. The care plan includes 8 NOC outcomes and 13 NIC interventions. The diagnosis of impaired tissue integrity is now satisfactorily resolved. Regarding the deterioration of physical mobility and body image disorder, the actions taken have improved the patient's condition, but full resolution is not achieved. Conclusions: The clinical case has allowed us to identify the guidelines for nursing action (care), using a daily reality in patients with skin tumours who, after an aggressive surgical intervention, are cared

Mohs micrographic surgery (MMS) is a breakthrough surgical technique that has changed the management of neoplasms in dermatology. Through continued practice and evolution, MMS now can successfully treat a variety of rare non-melanocytic cutaneous malignancies for which achieving remission and the optimal aesthetic result after surgery was previously challenging. Mohs micrographic surgery has utility for conditions besides melanoma. Herein, we discuss this versatility of Mohs micrographic surgery. Specifically, Mohs micrographic surgery can be successfully used for cases such as dermatofibrosarcoma protuberans, atypical fibroxanthomas, extramammary Paget disease, Merkel cell carcinoma, sebaceous carcinoma, and microcystic adnexal carcinoma.

Mohs micrographic surgery (MMS) is a breakthrough surgical technique that has changed the management of neoplasms in dermatology. Through continued practice and evolution, MMS now can successfully treat a variety of rare non-melanocytic cutaneous malignancies for which achieving remission and the optimal aesthetic result after surgery was previously challenging. Mohs micrographic surgery has utility for conditions besides melanoma. Herein, we discuss this versatility of Mohs micrographic surgery. Specifically, Mohs micrographic surgery can be successfully used for cases such as dermatofibrosarcoma protuberans, atypical fibroxanthomas, extramammary Paget disease, Merkel cell carcinoma, sebaceous carcinoma, and microcystic adnexal carcinoma.

[Resumen] Introducción: El Dermatofibrosarcoma Protuberans es un sarcoma cutáneo de baja incidencia, lento crecimiento, indoloro y escasa tendencia a la metástasis, pero con alto índice de recidivas. Este tumor tiene una importante capacidad de infiltración local que puede afectar al tejido subcutáneo y tegumentario, por lo que el tratamiento recomendado es la extirpación completa o resección. El pronóstico tras el tratamiento quirúrgico es bueno; sin embargo, hay casos con afectación ganglionar o metástasis a distancia que merman la calidad de vida del paciente y pueden provocar la muerte. Los cuidados de la herida quirúrgica postoperatoria tras la exéresis del tumor, sus secuelas, así como el abordaje biopsicosocial, son temas de estudio que la literatura no aborda en profundidad. Objetivo: Realizar una valoración integral y plan de cuidados postoperatorio de una paciente intervenida por un Dermatofibrosarcoma Protuberans en pierna, con resultado de injerto cutáneo y linfedema iatrogénico, desde atención primaria. Metodología: Caso clínico. Valoración mediante los 11 Patrones Funcionales de Marjory Gordon, elaboración del plan de cuidados según taxonomía NANDA-NOC-NIC, aplicación del modelo AREA en la selección de diagnósticos y modelo DREOM en la identificación de resultados e intervenciones. Complementariamente se utilizaron diferentes escalas validadas para apoyar la valoración objetiva. Resultados: Se identificaron inicialmente 10 diagnósticos NANDA, priorizando 3 de ellos en relación con los problemas de movilidad, autopercepción y lesión tisular. El plan de cuidados incluye 8 resultados NOC y 13 intervenciones NIC. Actualmente está resuelto satisfactoriamente el diagnóstico del deterioro de la integridad tisular. Respecto al deterioro de la movilidad física y al trastorno de la imagen corporal, las acciones llevadas a cabo han mejorado el estado de la paciente, pero no se alcanza la resolución plena. Conclusiones: El caso clínico nos ha permitido identificar, [Resumo] Introdución: O Dermatofibrosarcoma Protuberans é un sarcoma cutáneo de baixa incidencia, lento crecemento, indoloro e escasa tendencia á metástase, pero con alto índice de recidivas. Este tumor ten unha importante capacidade de infiltración local que pode afectar ao tecido subcutáneo e tegumentario, polo que o tratamento recomendado é a extirpación completa ou resección. O prognóstico tras o tratamento cirúrxico é bo; con todo, hai casos con afectación ganglionar ou metástase a distancia que minguan a calidade de vida do paciente e poden provocar a morte. Os coidados da ferida cirúrxica postoperatoria tras a exéresis do tumor, as súas secuelas, así como a abordaxe biopsicosocial, son temas de estudo que a literatura non aborda en profundidade. Obxectivo: Realizar unha valoración integral e plan de coidados postoperatorio dunha paciente intervida por un Dermatofibrosarcoma Protuberans en perna, con resultado de enxerto cutáneo e linfedema iatroxénico, desde atención primaria. Metodoloxía: Caso clínico. Valoración mediante os 11 Patróns Funcionais de Marjory Gordon, elaboración do plan de coidados segundo taxonomía NANDA-NOC-NIC, aplicación do modelo AREA na selección de diagnósticos e modelo DREOM na identificación de resultados e intervencións. Complementariamente utilizáronse diferentes escalas validadas para apoiar a valoración obxectiva. Resultados: Identificáronse inicialmente 10 diagnósticos NANDA, priorizando 3 deles en relación cos problemas de mobilidade, autopercepción e lesión tisular. O plan de coidados inclúe 8 resultados NOC e 13 intervencións NIC. Actualmente atópase resolto satisfactoriamente o diagnóstico da deterioración da integridade tisular. Respecto á deterioración da mobilidade física e ao trastorno da imaxe corporal, as accións levadas a cabo melloraron o estado da paciente, pero non se alcanza a resolución plena. Conclusións: O caso clínico permitiunos identificar as pautas de actuación enfermeira (coidados), mediante unha realidade, [Abstract] Introduction: Dermatofibrosarcoma Protuberans is a skin sarcoma with low incidence, slow growth, painless and little tendency to metastasis, but with a high rate of recurrence. This tumor has a significant local infiltration capacity that can affect the subcutaneous and tegumentary tissue, so the recommended treatment is complete removal or resection. The prognosis after surgical treatment is good; however, there are cases withnodal involvement or distant metastases that impair the patient’s quality of life and can lead to death. Post-operative surgical wound care after tumor exeresis, its sequelae, as well as the biopsychosocial approach, are subjects of study that the literature does not address in depth. Objective: To perform a comprehensive assessment and post-operative care plan of a patient undergoing surgery for Dermatofibrosarcoma Protuberans in the leg, resulting in skin grafting and iatrogenic lymphedema, from primary care. Methodology: Clinical case. Assessment using Marjory Gordon's 11 Functional Patterns, elaboration of the care plan according to NANDA-NOC-NIC taxonomy, application of the AREA model in the selection of diagnoses and DREOM model in the identification of results and interventions. Complementary to this, different validated scales were used to support the objective assessment. Results: 10 NANDA diagnoses were originally identified, prioritizing 3 of them in relation to mobility problems, self-perception and tissue injury. The care plan includes 8 NOC outcomes and 13 NIC interventions. The diagnosis of impaired tissue integrity is now satisfactorily resolved. Regarding the deterioration of physical mobility and body image disorder, the actions taken have improved the patient's condition, but full resolution is not achieved. Conclusions: The clinical case has allowed us to identify the guidelines for nursing action (care), using a daily reality in patients with skin tumours who, after an aggressive surgical intervention, are cared

Fibrous hamartoma of infancy (FHI) is a rare benign soft tissue tumor with a triphasic organoid histologic appearance. The authors present a case of a 21-month-old healthy girl with a slowly growing flesh-colored subcutaneous plaque 12cm in size on the lower back, with overlying hypertrichosis. A punch biopsy revealed a proliferation of spindle cells infiltrating the dermis and hypodermis organized in a dense storiform pattern with a strong diffuse positivity for CD34. The diagnosis of congenital dermatofibrosarcoma protuberans (DFSP) was considered and an excision was performed. Histopathologic analysis showed an extensive poorly demarcated mass infiltrating the dermis and hypodermis, composed of different components: a monomorphous fibroblastic/myofibroblastic component, a mature adipose component, and an immature mesenchymal basophilic component. The clinical aspects with the histologic and immunohistochemical features led to the diagnosis of giant fibrous hamartoma of infancy. In our case the strong diffuse CD34 positivity was a diagnostic pitfall leading to an incorrect hypothesis of congenital DFSP. The characteristic triphasic histology of FHI was missed owing to the small size of the punch biopsy. This article highlights the importance of being aware of the CD34+ dermal mesenchymal tumor differential diagnosis and the necessity of appropriate size biopsies to avoid sampling error.

Fibrous hamartoma of infancy (FHI) is a rare benign soft tissue tumor with a triphasic organoid histologic appearance. The authors present a case of a 21-month-old healthy girl with a slowly growing flesh-colored subcutaneous plaque 12cm in size on the lower back, with overlying hypertrichosis. A punch biopsy revealed a proliferation of spindle cells infiltrating the dermis and hypodermis organized in a dense storiform pattern with a strong diffuse positivity for CD34. The diagnosis of congenital dermatofibrosarcoma protuberans (DFSP) was considered and an excision was performed. Histopathologic analysis showed an extensive poorly demarcated mass infiltrating the dermis and hypodermis, composed of different components: a monomorphous fibroblastic/myofibroblastic component, a mature adipose component, and an immature mesenchymal basophilic component. The clinical aspects with the histologic and immunohistochemical features led to the diagnosis of giant fibrous hamartoma of infancy. In our case the strong diffuse CD34 positivity was a diagnostic pitfall leading to an incorrect hypothesis of congenital DFSP. The characteristic triphasic histology of FHI was missed owing to the small size of the punch biopsy. This article highlights the importance of being aware of the CD34+ dermal mesenchymal tumor differential diagnosis and the necessity of appropriate size biopsies to avoid sampling error.

Dermatofibrosarcoma protuberans (DFSP) is a skin fibroblastic tumour that is locally aggressive, with a tendency for local recurrence, but rarely metastasizes. A unique collaboration of multi-disciplinary experts from the European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization of Research and Treatment of Cancer (EORTC) was formed to make recommendations on DFSP diagnosis and treatment, based on systematic literature reviews and the experts' experience. Diagnosis is suspected clinically and confirmed by pathology. Analysis by fluorescence in situ hybridisation (FISH) or multiplex reverse transcriptase-polymerase chain reaction (RT-PCR) to detect specific chromosomal translocations and fusion gene transcripts is useful to confirm a difficult DFSP diagnosis. Treatment is mainly surgical, with the aim to achieve complete resection of the tumour. In order to reduce the recurrence rate, the treatment of choice of DFSP seems to be Mohs' micrographic surgery (MMS) and related variants. In hospitals where only standard histopathological procedures are available, standard excision with lateral safety margin of 3cm is advisable. Imatinib (Glivec®) is approved in Europe for the treatment of inoperable primary tumours, locally inoperable recurrent disease, and metastatic DFSP. Imatinib has also been given to patients with extensive, difficult-to-operate tumours for preoperative reduction of tumour size, but the usefulness of this attitude should be confirmed by clinical trials. Therapeutic decisions for patients with fibrosarcomatous DFSP should be primarily made by an interdisciplinary oncology team ('tumour board').

Dermatofibrosarcoma protuberans (DFSP) is a skin fibroblastic tumour that is locally aggressive, with a tendency for local recurrence, but rarely metastasizes. A unique collaboration of multi-disciplinary experts from the European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization of Research and Treatment of Cancer (EORTC) was formed to make recommendations on DFSP diagnosis and treatment, based on systematic literature reviews and the experts' experience. Diagnosis is suspected clinically and confirmed by pathology. Analysis by fluorescence in situ hybridisation (FISH) or multiplex reverse transcriptase-polymerase chain reaction (RT-PCR) to detect specific chromosomal translocations and fusion gene transcripts is useful to confirm a difficult DFSP diagnosis. Treatment is mainly surgical, with the aim to achieve complete resection of the tumour. In order to reduce the recurrence rate, the treatment of choice of DFSP seems to be Mohs' micrographic surgery (MMS) and related variants. In hospitals where only standard histopathological procedures are available, standard excision with lateral safety margin of 3 cm is advisable. Imatinib (Glivec®) is approved in Europe for the treatment of inoperable primary tumours, locally inoperable recurrent disease, and metastatic DFSP. Imatinib has also been given to patients with extensive, difficult-to-operate tumours for preoperative reduction of tumour size, but the usefulness of this attitude should be confirmed by clinical trials. Therapeutic decisions for patients with fibrosarcomatous DFSP should be primarily made by an interdisciplinary oncology team ('tumour board')., SCOPUS: ar.j, info:eu-repo/semantics/published

A 92-year-old woman was referred for the assesment of an asymptomatic subcutaneous tumor that developed after an accidental fall. The mass clinically and radiologically simulated a subcutaneous hematoma. Finally, the histological study was consistent with subcutaneous dermatofibrosarcoma protuberans.

A 92-year-old woman was referred for the assesment of an asymptomatic subcutaneous tumor that developed after an accidental fall. The mass clinically and radiologically simulated a subcutaneous hematoma. Finally, the histological study was consistent with subcutaneous dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DFSP), a rare medium grade sarcoma, occasionally occurs in childhood and is even more rarely present at birth. In children, the clinical appearance may be mistaken for a vascular malformation and so delayed diagnosis is not uncommon. Dermatofibrosarcoma protuberans is locally invasive and notorious for its high recurrence rate even after attempted wide local excision owing to extensive subclinical and asymmetrical extensions. In adult DFSP, Mohs Micrographic Surgery (MMS) is the treatment of choice because it offers a higher clearance rate compared to wide local excision. However, MMS may result in extended operating times owing to tissue processing and multiple stages. In children, this means a prolonged period under general anesthetic, which may be undesirable. We describe an interesting case of a 4- year-old girl diagnosed with DFSP. She underwent a modified MMS procedure in which she had two short general anesthetics. The advantage of MMS technique in which the full peripheral and deep margin of the specimen was examined.

Dermatofibrosarcoma protuberans (DFSP), a rare medium grade sarcoma, occasionally occurs in childhood and is even more rarely present at birth. In children, the clinical appearance may be mistaken for a vascular malformation and so delayed diagnosis is not uncommon. Dermatofibrosarcoma protuberans is locally invasive and notorious for its high recurrence rate even after attempted wide local excision owing to extensive subclinical and asymmetrical extensions. In adult DFSP, Mohs Micrographic Surgery (MMS) is the treatment of choice because it offers a higher clearance rate compared to wide local excision. However, MMS may result in extended operating times owing to tissue processing and multiple stages. In children, this means a prolonged period under general anesthetic, which may be undesirable. We describe an interesting case of a 4- year-old girl diagnosed with DFSP. She underwent a modified MMS procedure in which she had two short general anesthetics. The advantage of MMS technique in which the full peripheral and deep margin of the specimen was examined.

Fibroblast growth factors (FGFs) and their receptors (FGFRs) control a wide range of biological functions ; however, their involvement in the pathogenesis of dermatofibroma (DF) and dermatofibrosarcoma protuberans (DFSP) is currently unknown. In this study, we first confirmed the histological diagnosis by detecting fusion COL1A1-PDGFB transcripts in DFSP, and examined the expression of all FGFRs (FGFR1-4), some of their ligands (FGF1, 2, 9), and forkhead box N1 (FOXN1) as a downstream target of FGFR3 in DF and DFSP by immunohistochemical analysis. Although we failed to detect the expression of FGF1 and FGF9 as specific ligands for FGFR3 in DF, overexpression of FGFR3 and FOXN1 was observed in the epidermal regions of DF, suggesting that the epidermal regions of DF were similar to seborrhoeic keratosis both in terms of histological features and the activation of FGFR3/FOXN1. In addition, strong expression of FGF2 and FGFR4 was observed in the tumor lesions of DF. Expression patterns of FGFR3/FOXN1 and FGF2/FGFR4 in DF were in contrast with those of DFSP. The activation of FGFR signaling pathways may be not only relevant to the pathogenesis of DF, but also very useful in the differential diagnosis of DF and DFSP.

Dermatofibrosarcoma protuberans is a soft-tissue tumor that may recur locally and rarely causes metastases to vital organs. Dermatofibrosarcoma protuberans has specific chromosomal abnormalities involving the platelet-derived growth factor beta-chain locus that may render these tumors responsive to targeted therapy with the tyrosine kinase inhibitor imatinib mesylate. A patient with locally recurrent and metastatic dermatofibrosarcoma protuberans who had already undergone surgery 22 times was initially treated with imatinib mesylate 400 mg/day. The treatment dose was increased after 7 days to 400 mg twice daily. The patient was followed up for response and toxicity by physical examination and imaging studies, comprising computed tomography and fluorodeoxyglucose positron emission tomography. Clinical response could be demonstrated after the first month of treatment, and subsequent computed tomography and positron emission tomography documented a response to imatinib mesylate therapy. Our patient is now in sustained remission with minimal toxicity. We conclude that antitumor activity of metastatic dermatofibrosarcoma protuberans can be obtained with imatinib mesylate treatment with minimal side-effects., Case Reports, Journal Article, info:eu-repo/semantics/published

Dermatofibrosarcoma protuberans is a soft-tissue tumor that may recur locally and rarely causes metastases to vital organs. Dermatofibrosarcoma protuberans has specific chromosomal abnormalities involving the platelet-derived growth factor beta-chain locus that may render these tumors responsive to targeted therapy with the tyrosine kinase inhibitor imatinib mesylate. A patient with locally recurrent and metastatic dermatofibrosarcoma protuberans who had already undergone surgery 22 times was initially treated with imatinib mesylate 400 mg/day. The treatment dose was increased after 7 days to 400 mg twice daily. The patient was followed up for response and toxicity by physical examination and imaging studies, comprising computed tomography and fluorodeoxyglucose positron emission tomography. Clinical response could be demonstrated after the first month of treatment, and subsequent computed tomography and positron emission tomography documented a response to imatinib mesylate therapy. Our patient is now in sustained remission with minimal toxicity. We conclude that antitumor activity of metastatic dermatofibrosarcoma protuberans can be obtained with imatinib mesylate treatment with minimal side-effects., Case Reports, Journal Article, info:eu-repo/semantics/published

The clinicopathologic and immunohistochemical features of 28 dermatofibrosarcoma protuberans (DFSP), giant cell fibroblastomas (GCFs), and hybrid lesions occurring in children are presented, including molecular data for seven of them. There were 19 pure adult-type DFSP (9 male and 10 female patients aged between a few days [neonate] and 13 years, median 7 years), 5 pure GCF (all males aged from 2 to 8 years, median 4 years), and 4 hybrid tumors (all males aged from 1 to 4 years, median 2.5 years). Tumor locations in pure adult-type DFSP included the trunk (6) and lower (11) and upper (2) limbs. Pure GCFs were observed on the trunk (4) and knee (1), and hybrid lesions on the trunk (2) and lower (1) and upper (1) extremities. Tumor size (n = 20) ranged from 0.6 to 5 cm (median 2 cm). Histologically, pure DFSP presented as monotonous and infiltrative, low-grade, dermal/hypodermal storiform spindle cell proliferations, sparing adnexal structures. GCF showed a dense fibrous to myxoid matrix containing slender wavy spindle cells and multinucleated giant stromal cells often lining angiectoid spaces. Hybrid lesions showed varying combinations of DFSP and GCF areas. Mitotic activity ranged from 1 to 3 mitoses per 10 high power fields. All tumors were diffusely positive for vimentin and CD34 but negative for smooth muscle actin, desmin, epithelial membrane antigen, and cytokeratins; one pure adult-type DFSP was also S-100 protein positive; <1% of nuclei were Ki67 (Mib-1) positive. One karyotyped adult-type DFSP showed an unbalanced t(17;22) (q22;q13) translocation. Multiplex RT-PCR analysis and sequencing of PCR products in seven cases showed COL1A1-PDGFB gene fusion transcripts in two pure DFSP, two pure GCFs, and one hybrid lesion. Results were uncertain in one pure GCF; one adult-type DFSP was negative. Treatment procedures were known for 27 patients, consisting of 16 wide excisions and 11 marginal excisions. Follow-up information on 15 widely excised tumors (median 24 mon, SCOPUS: ar.j, info:eu-repo/semantics/published

The clinicopathologic and immunohistochemical features of 28 dermatofibrosarcoma protuberans (DFSP), giant cell fibroblastomas (GCFs), and hybrid lesions occurring in children are presented, including molecular data for seven of them. There were 19 pure adult-type DFSP (9 male and 10 female patients aged between a few days [neonate] and 13 years, median 7 years), 5 pure GCF (all males aged from 2 to 8 years, median 4 years), and 4 hybrid tumors (all males aged from 1 to 4 years, median 2.5 years). Tumor locations in pure adult-type DFSP included the trunk (6) and lower (11) and upper (2) limbs. Pure GCFs were observed on the trunk (4) and knee (1), and hybrid lesions on the trunk (2) and lower (1) and upper (1) extremities. Tumor size (n = 20) ranged from 0.6 to 5 cm (median 2 cm). Histologically, pure DFSP presented as monotonous and infiltrative, low-grade, dermal/hypodermal storiform spindle cell proliferations, sparing adnexal structures. GCF showed a dense fibrous to myxoid matrix containing slender wavy spindle cells and multinucleated giant stromal cells often lining angiectoid spaces. Hybrid lesions showed varying combinations of DFSP and GCF areas. Mitotic activity ranged from 1 to 3 mitoses per 10 high power fields. All tumors were diffusely positive for vimentin and CD34 but negative for smooth muscle actin, desmin, epithelial membrane antigen, and cytokeratins; one pure adult-type DFSP was also S-100 protein positive; <1% of nuclei were Ki67 (Mib-1) positive. One karyotyped adult-type DFSP showed an unbalanced t(17;22) (q22;q13) translocation. Multiplex RT-PCR analysis and sequencing of PCR products in seven cases showed COL1A1-PDGFB gene fusion transcripts in two pure DFSP, two pure GCFs, and one hybrid lesion. Results were uncertain in one pure GCF; one adult-type DFSP was negative. Treatment procedures were known for 27 patients, consisting of 16 wide excisions and 11 marginal excisions. Follow-up information on 15 widely excised tumors (median 24 mon, SCOPUS: ar.j, info:eu-repo/semantics/published

Dermatofibrosarcoma protuberans is a rare mesenchimal tumour mainly characterized by its local aggressiveness and high reported recurrence rates. Classically, wide excisional surgery with at least 3 cm of free tissue margins is recommended as treatment. We present a series of 18 patients with variable gross free tissue margins but confirmed histological free margins in all cases whatever the surgical excision really was. Only one patient presented a local recurrence (5.5%) of a very large (14 cm) sarcomatous variant of dermatofibrosarcoma protuberans. Mean follow-up in this series was 52.1 months. Those results emphasize that histological rather than theoretical «golden rules» are mandatory to locally control this aggressive neoplasm., SCOPUS: ar.j, info:eu-repo/semantics/published

Dermatofibrosarcoma protuberans is a rare mesenchimal tumour mainly characterized by its local aggressiveness and high reported recurrence rates. Classically, wide excisional surgery with at least 3 cm of free tissue margins is recommended as treatment. We present a series of 18 patients with variable gross free tissue margins but confirmed histological free margins in all cases whatever the surgical excision really was. Only one patient presented a local recurrence (5.5%) of a very large (14 cm) sarcomatous variant of dermatofibrosarcoma protuberans. Mean follow-up in this series was 52.1 months. Those results emphasize that histological rather than theoretical «golden rules» are mandatory to locally control this aggressive neoplasm., SCOPUS: ar.j, info:eu-repo/semantics/published

Dermatofibrosarcoma protuberans is an uncommon malignant mesenchymal tumor which is characterized by local aggressivity and recurrence. About 50 cases have been reported in the pediatric population. In the case presented, the lesion as a mass 5 mm in diameter, with normal overlying skin and no pain. The diagnosis was made by histology obtained on an incisional biopsy. Treatment was by wide local excision., SCOPUS: ar.j, info:eu-repo/semantics/published

Dermatofibrosarcoma protuberans is an uncommon malignant mesenchymal tumor which is characterized by local aggressivity and recurrence. About 50 cases have been reported in the pediatric population. In the case presented, the lesion as a mass 5 mm in diameter, with normal overlying skin and no pain. The diagnosis was made by histology obtained on an incisional biopsy. Treatment was by wide local excision., SCOPUS: ar.j, info:eu-repo/semantics/published

Dermatofibrosarcoma protuberans is a slow-growing but locally aggressive malignant neoplasm of the skin. The preferential locations are on the trunk and proximal extremities but it can occur in the head and neck region in about 15% of the cases. This is a report of such a case and a review of the literature of head and neck cases attempting to analyse the profile of patients and surgical prognosis factors. The recurrence rate is important (20% to 55%) and dependent on tile free tissue margins. Those margins are at least 3 cm in all directions; this is difficult to assess in the head and neck region because of both functional and cosmetic aspects. Distant metastases are rare but related to a long recurrence history. This behaviour gives the impression that prognosis is poorer for dermatofibrosarcoma in the head and neck than within other locations., SCOPUS: ar.j, info:eu-repo/semantics/published

Dermatofibrosarcoma protuberans is a slow-growing but locally aggressive malignant neoplasm of the skin. The preferential locations are on the trunk and proximal extremities but it can occur in the head and neck region in about 15% of the cases. This is a report of such a case and a review of the literature of head and neck cases attempting to analyse the profile of patients and surgical prognosis factors. The recurrence rate is important (20% to 55%) and dependent on tile free tissue margins. Those margins are at least 3 cm in all directions; this is difficult to assess in the head and neck region because of both functional and cosmetic aspects. Distant metastases are rare but related to a long recurrence history. This behaviour gives the impression that prognosis is poorer for dermatofibrosarcoma in the head and neck than within other locations., SCOPUS: ar.j, info:eu-repo/semantics/published