Cervical Infantile Fibrosarcoma: a rare cause of paediatric parapharyngeal neck mass

Soft tissue tumors are not uncommon in childhood and comprise entities that range from common to very rare malignancies. Infantile fibrosarcoma (IFS) is a rare pediatric malignancy mainly seen in the first two years of life. The data about the incidence of infantile fibrosarcoma occurring in the neck in the Indian subcontinent is scarce. To the best of our knowledge, only one case of infant cervical IFS has been reported previously in the Indian subcontinent. We present another case of an eight-year-old male patient with a rapidly growing mass on the left side of the neck. He was successfully treated with a combined modality of surgery and chemotherapy with a good outcome. Among the soft tissue tumors of childhood, IFS is a rare entity. It has a good prognosis and lesser chance of distant metastasis as compared to adult fibrosarcoma. Though surgical excision is the mainstay of treatment, chemotherapy also has a significant role in the treatment of primary tumor and metastasis. We discuss the stated case to bring to the notice this uncommon cause, which can be considered as a differential diagnosis of upper cervical swellings. A better understanding of this entity would help in early diagnosis and aggressive treatment, reducing the overall morbidity and mortality.

Ameloblastic Fibrosarcoma: A Case Report and Literature Review

Here is described a case of ameloblastic fibrosarcoma (AFS) affecting the posterior mandible of a woman who was treated surgically and recovered without signs of recurrence or metastasis after 12 years of follow-up. Tumor sections were immunostained for cell cycle, epithelial and mesenchymal markers. Immunohistochemical analysis evidenced high Ki-67 positivity in stromal cells (mean of 20.9 cells/High power field). Epithelial cells displayed strong positivity for p53, p63 and cytokeratin 19. In addition to the case report, a systematic review of current knowledge is presented on the AFS’s clinical-demographic features and prognostic factors. Based on the review, 88/99 cases were diagnosed as AFS, 9/99 as ameloblastic fibro-odontosarcoma and 2/99 as ameloblastic fibrodentinosarcoma. All these lesions displayed very similar clinical-demographic and prognostic features. Moreover, the review provided evidence that first treatment, regional metastasis, distant metastasis and local recurrence were significant prognostic values for malignant odontogenic mesenchymal lesions. Based on the findings, segregation among ameloblastic fibrosarcoma, ameloblastic fibrodentinosarcoma and ameloblastic fibro-odontosarcoma seems illogical, considering all these lesions have similar predilections and outcomes.

Resumo Aqui é descrito um caso de fibrossarcoma ameloblástico afetando região posterior da mandíbula de uma mulher. Após o tratamento, a paciente ficou livre da doença durante os 12 anos de acompanhamento. Foi realizado imunohistoquimica para marcadores epiteliais, mesenquimais e do ciclo celular. Além disso, uma revisão sistemática de literatura também foi realizada, na tentativa de descobrir as características clínico-demográficas e fatores prognósticos da lesão. 88/99 casos foram diagnosticados como fibrossarcoma ameloblastico, 9/99 como fibro-odontosarcoma ameloblastico e 2/99 como fibrodentinosarcoma ameloblastico. Todas estas lesões exibem características clínico-demográficas e prognósticos muito semelhantes. Além disso, esta revisão forneceu evidências de que primeiro tratamento, metástases regionais, metástases à distância e recorrência local são valores prognósticos significativos para lesões odontogênicas mesenquimais malignas. A análise imunohistoquímica demonstrou elevada marcação positiva em células do estroma para Ki-67 (média de 20,9 células /HPF). As células epiteliais exibiram forte marcação para p53, p63 e citoqueratina 19. A segregação entre fibrosarcoma ameloblastico, fibrodentinosarcoma ameloblastico e fibro-odontosarcoma ameloblastico é ilógica, uma vez que todas essas lesões têm predileções e resultados semelhantes.

Dermatofibrossarcoma protuberante diagnosticado erroneamente como queloide e tratado com acetonido de triancinolona / Dermatofibrosarcoma protuberans misdiagnosed as keloid and treated with triamcinolone acetonide

INTRODUÇÃO: Dermatofibrossarcoma protuberante é um tumor de pele raro e de malignidade intermediária, com baixo potencial metastático, mas altas taxas de recorrência após tratamento cirúrgico. Por apresentar eventual semelhança clínica com cicatrizes hipertróficas e queloides, o diagnóstico correto mostra-se fundamental para o sucesso do tratamento. O objetivo do presente trabalho é fazer um alerta e relatar quatro casos de dermatofibrossarcoma protuberante erroneamente diagnosticados como queloide e tratados alhures com infiltração de acetonido de triancinolona. MÉTODO: Entre novembro de 1983 e janeiro de 2008, foram atendidos quatro pacientes com dermatofibrossarcoma protuberante que tinham sido submetidos alhures a infiltrações intralesionais de acetonido de triancinolona, em virtude de diagnóstico errôneo de queloide. Nos quatro casos, foram realizadas excisões cirúrgicas radicais, com remoção de 3 cm de tecido sadio nas margens laterais, incluindo-se, na margem profunda, uma estrutura anatômica não infiltrada pelo tumor. Os pacientes receberam avaliação médica periódica em longo prazo. RESULTADOS: Os pacientes foram acompanhados por uma média de 159 meses. Três pacientes (75%) permaneceram vivos, sem sinais de doença em atividade. Um paciente (25%) faleceu devido à doença, após tentativa de remover o avançado tumor recorrente, por meio de extensa cirurgia craniofacial. A recidiva ocorreu sete anos após a operação radical. CONCLUSÃO: Dermatofibrossarcoma protuberante deve ser considerado no diagnóstico diferencial dos queloides. A infiltração intralesional de acetonido de triancinolona só deverá ser realizada após diagnóstico de certeza, que pode demandar exame anatomopatológico prévio. Um exame clínico cuidadoso e o conhecimento da lesão favorecem um diagnóstico preciso e, portanto, um tratamento adequado.

INTRODUCTION: Dermatofibrosarcoma protuberans is a rare skin tumor with intermediate malignancy, low metastatic potential, and high recurrence rates after surgical treatment. Owing to a possible clinical resemblance with hypertrophic scars and keloids, the correct diagnosis is fundamental for treatment success. The objective of the present work is to report on four cases of dermatofibrosarcoma protuberans misdiagnosed as keloid and treated elsewhere with infiltration of triamcinolone acetonide. METHOD: Between November 1983 and January 2008, four patients with dermatofibrosarcoma protuberans who had undergone intralesional infiltration with triamcinolone acetonide elsewhere were treated because of an erroneous diagnosis of keloid. Radical surgical excision was performed, and 3 cm of healthy tissue was removed from the side margins, including the deep margin, an anatomical structure not infiltrated by the tumor. The patients underwent long-term periodic medical evaluations. RESULTS: The patients were followed-up for an average of 159 months. Three patients (75%) are still alive without signs of disease at the time of this report. One patient (25%) died of the disease after an attempt to remove the advanced recurrent tumor using extensive craniofacial surgery. Recurrence occurred 7 years after the radical operation. CONCLUSION: Dermatofibrosarcoma protuberans must be considered in the differential diagnosis of keloids. Intralesional infiltration with triamcinolone acetonide should only be performed after diagnostic confirmation , which may require pathological examination. A careful clinical examination and knowledge of the lesion favor a precise diagnosis and an appropriate treatment.

Mixofibrosarcoma de bajo grado: reporte de caso / Low grade mixofibrosarcoma: case report

El mixofibrosarcoma es un subtipo de sarcoma que comúnmente se presenta en las extremidades de personas ancianas. La presentación clínica no es característica y el aspecto histológico es altamente heterogéneo, lo que frecuentemente retrasa el diagnóstico o conduce a uno equivocado. Técnicas de histoquímica e inmunohistoquímica son mandatorias para establecer el diagnóstico de MFS. Presentamos el caso de un hombre de 57 años para ilustrar lo poco sugerente de este diagnóstico dada la presentación clínica. El manejo de este tumor es con cirugía, eventualmente radioterapia y seguimiento estricto.

Myxofibrosarcoma is a subtype of sarcoma commonly found in the extremities of elderly people. The clinical presentation is not characteristic and the histological aspect is highly heterogeneous, which often delays the diagnosis or leads to the wrong one. Histochemistry and immunohistochemistry techniques are required to establish the diagnosis of SFM. We present the case of a 57-year-old man to illustrate the unimpressive nature of this diagnosis given the clinical presentation. The management of this tumor is with surgery, eventually radiotherapy and strict follow-up.

Estigma e discriminação: experiências de mulheres HIV positivo nos bairros populares de Maputo, Moçambique / Stigma and discrimination: the experiences of HIV-positive women in poor neighborhoods of Maputo, Mozambique / Estigma y discriminación: las experiencias de las mujeres VIH positivas en los barrios populares de Maputo, Mozambique

A epidemia de HIV/AIDS é um sério problema de saúde pública em Moçambique, que convive com altas taxas de prevalência do HIV. O impacto da epidemia é agravado pelo forte estigma que atinge as pessoas soropositivas. O objetivo deste estudo foi investigar, com base em uma perspectiva socioantropológica, a experiência de mulheres HIV positivo nos bairros populares de Maputo e como lidam com o estigma e a discriminação. Foram realizadas entrevistas semiestruturadas com dez mulheres HIV positivo, residentes nos bairros populares de Maputo. Os resultados mostram como a desigualdade de gênero atua de forma importante na construção da vulnerabilidade das mulheres ao HIV, assim como em sua estigmatização e discriminação. No enfrentamento do estigma, as mulheres procuram preservar o sigilo do diagnóstico buscando apoio na reunião em grupos de pares HIV positivo. É fundamental que se implementem políticas públicas voltadas para o empoderamento das mulheres e redução do estigma associado ao HIV/AIDS.

The HIV/AIDS epidemic is a serious public health problem in Mozambique. The country has high prevalence rates, and the epidemic's impact is aggravated by the stigma affecting HIV-positive persons. This study takes a socio-anthropological perspective to analyze the experience of HIV-positive women in poor neighborhoods of Maputo and the ways they cope with stigma and discrimination. Semi-structured interviews were conducted with 10 HIV-positive women. The results show how gender inequalities increase women's vulnerability to HIV and contribute to their stigmatization and discrimination. In dealing with stigma, women try to keep their diagnosis confidential, seeking support in group meetings with others living with HIV. Public policies should focus on women's empowerment and the reduction of HIV/AIDS-related stigma.

El VIH/SIDA es un problema de salud pública grave en Mozambique, que convive con altas tasas de prevalencia del VIH. El impacto de la epidemia se ve agravada por el fuerte estigma que afecta a las personas con VIH. El objetivo de este estudio fue investigar, desde una perspectiva antropológica, la experiencia de las mujeres VIH positivas en los barrios populares de Maputo y cómo enfrentan el estigma y la discriminación. Se realizaron entrevistas semi-estructuradas con 10 mujeres VIH positivas que viven en barrios pobres de Maputo. Los resultados muestran cómo la desigualdad de género juega un papel importante en la construcción de la vulnerabilidad de las mujeres frente al VIH, así como en la estigmatización y discriminación. Para hacer frente el estigma, las mujeres buscan preservar la confidencialidad del diagnóstico y buscar apoyo en la reunión de grupos de pares con VIH. Es imprescindible implementar políticas públicas enfocadas al empoderamiento de las mujeres y a la reducción del estigma asociado con el VIH/SIDA.

Retalho Duoescapular: uma nova opção na cirurgia reparadora do ombro / Duoscapular flap: a new option in shoulder reconstructive surgery

INTRODUÇÃO: O Dermatofibrossarcoma do Ombro é patologia incomum e seu tratamento demanda extensas ressecções. O sistema escapular é fonte de retalhos bastante utilizados nesta região. MÉTODO: Realizado estudo longitudinal, prospectivo, através da condução de um caso de Dermatofibrossarcoma Protuberans em ombro direito, submetido a ressecção e reconstrução local com Retalho Duoescapular, obtido através da associação dos retalhos escapular e paraescapular. RESULTADOS: Paciente evoluiu sem intercorrências no pós-operatório, não sendo observadas complicações sistêmicas e locais, e limitações funcionais. CONCLUSÃO: O Retalho Duoescapular é nova e relevante opção para reconstrução de feridas extensas, com exposição de estruturas nobres no ombro. Permite fechamento primário da área doadora, sem acrescentar morbidade ao procedimento.

INTRODUCTION: Shoulder cutaneous fibrosarcoma is an unusual pathology that requires extensive resections. The scapula is a source of flaps widely used in this region. METHOD: A longitudinal and prospective study was carried out in a patient with protuberans cutaneous fibrosarcoma on the right shoulder who underwent resection followed by local reconstruction with a Duoscapular Flap (a combination of scapular and parascapular flaps). RESULTS: The patient had no postoperative complications, as systemic and local complications as well as functional limitations were not observed. CONCLUSION: Duoscapular Flap placement is a novel procedure and a relevant choice for the reconstruction of extensive wounds exposing noble structures in the shoulder. It allows the primary closure of the donor area without increasing the morbidity of the procedure.

Flape cutâneo em padrão axial auricular caudal para correção de defeito extenso após extirpação de fibrossarcoma facial em felino / Use of a caudal auricular axial pattern flap for correction of large defect after facial fribrosarcoma extirpation in a cat

Um felino que apresentava massa de grandes dimensões, localizada no lábio superior direito, foi submetido à cirurgia para exérese do aumento de volume mantendo-se margem de tecido sadio. O defeito facial foi reconstituído com um flape cutâneo em padrão axial auricular caudal. A ferida apresentou cicatrização em primeira intenção em sua maior extensão, com adequados resultados cosmético e funcional. A avaliação histopatológica revelou se tratar de fibrossarcoma, o qual não apresentou recidivas locais pelo período mínimo de 21 meses de pós-operatório.

A cat presented for clinical evaluation with a large mass on the superior lip was submitted to surgery to remove the tumor with the conservation of healthy tissue around it. The facial defect was reconstructed with a cutaneous flap in the caudal auricular axial pattern. The wound presented first intention healing on the major extension, with adequate functional and cosmetic results. According to the histopathological findings, it was a fibrosarcoma, which did not show any sign of relapse in the next 21 months after the surgical intervention.

Pediatric gliosarcoma with fibrosarcomatous differentiation: Report of a rare case.

Gliosarcoma is a rare variant of glioblastoma with a biphasic pattern showing glial and mesenchymal differentiation. It is seen in adults during their fifth to sixth decades of life and is extremely rare in children. We report a case of primary gliosarcoma with fibrosarcomatous differentiation in an 11-year-old boy presenting with headache and vomiting. Imaging showed a contrast-enhancing isodense space-occupying lesion with areas of calcification in the right temporoparietal cortex. A total excision was done and, on histopathologic examination, a differential diagnostic consideration of gliosarcoma and teratoma with malignant transformation was made. After immunohistochemical analysis, a final diagnosis of gliosarcoma with fibrosarcomatous differentiation was then made. Primary gliosarcoma is a very rare tumor in children with a poor prognosis.

Fibrosarcoma of the oral cavity.

Fibrosarcoma has been defined as a malignant tumor of the fibroblasts that shows no other evidence of cellular differentiation and is capable of recurrence and metastasis. Fibrosarcomas are rare but may occur anywhere in the body, most commonly in the retroperitoneum, thigh, knee and distal extremities. Fibrosarcoma is uncommon in the head and neck region and constitutes about 1% of all the malignancies affecting the human race. Of all the fibrosarcomas occurring in humans, only 0.05% occurs in the head and neck region. Of this, almost 23% is seen in the oral cavity. Fibosarcomas generally have a poor prognosis and the overall survival rate is 20-35% over a period of 5 years.

Aggressive fibromatosis versus low grade fibrosarcoma - a diagnostic dilemma

The fibromatosis are a group of lesions that can infiltrate widely, replacing muscle, fat and bone with fibrous tissue of varying cellularity. They do not develop distant metastases, however, locally they show an aggressive and infiltrative behaviour. The major challenge in the diagnosis lies in not over or under diagnose of fibrosarcoma and an underdiagnosis of reactive fibrosis. But this histologic differentiation is necessary owing to different treatment strategies

Dermatofibrosarcoma protuberans: comunicación de un caso y revisión de la literatura / Dermatofibrosarcoma protuberans: case report and literature review

El dermatofibrosarcoma protuberans (DFSP) es un tumor raro, de origen fibrohistiocítico y malignidad intermedia, caracterizado por su crecimiento local progresivo y su alta tasa de recidivas locales con bajo potencial metastásico. Afecta principalmente a adultos entres los 20 y 50 años, pero también se presentan casos en la niñez. Se localiza más frecuentemente en el tronco y raíz de miembros y menos del 5% de los casos afecta cuero cabelludo. Se presenta el caso de un varón de 20 años con un dermatofibrosarcoma protuberans en región frontoparietal izquierda con compromiso del cuero cabelludo, al que se le realizó tratamiento cirugía micrográfica de Mohs y se realiza una revisión de la literatura.

Effects of Toxoplasma gondii and Toxocara canis Antigens on WEHI-164 Fibrosarcoma Growth in a Mouse Model

Cancer is the main cause of death in developed countries. However, in underdeveloped countries infections and parasitic diseases are the main causes of death. There are raising scientific evidences indicating that parasitic infections induce antitumor activity against certain types of cancers. In this study, the effects of Toxoplasma gondii and Toxocara canis egg antigens in comparison with Bacillus Calmette Guerin (BCG) (known to have anticancer distinctive) on WEHI-164 fibosarcoma transplanted to BALB/c mice was investigated. Groups of 6 male BALB/c mice injected with T. gondii antigen, BCG, or T. canis egg antigen as case groups and alum alone as control groups. All mice were then challenged with WEHI-164 fibrosarcoma cells. The mice were examined for growth of the solid tumor and the tumor sizes were measured every other day up to 4 wk. The mean tumor area in T. gondii, BCG, or alum alone injected mice in 4 different days of measurements was 25 mm2, 23 mm2, and 186 mm2 respectively. Also the mean tumor area in T. canis injected mice in 4 different days was 25.5 mm2 compared to the control group (alum treated) which was 155 mm2. T. gondii parasites and T. canis egg antigens induced inhibition of the tumor growth in the fibrosarcoma mouse model. We need further study to clarify the mechanisms of anti-cancer effects.

Low Grade Fibromyxoid Sarcoma in Thigh

A low grade fibromyxoid sarcoma is a rare soft tissue tumor that has a tendency to develop in the deep soft tissue of young adults and the potential for local recurrence or distant metastasis. There have been several case reports and sporadic reports in the literature. However, only 1 case has been reported in Korea but without a follow-up result. We describe a 54-year-old female patient with a low-grade fibromyxoid sarcoma of the thigh that had been growing slowly for 34 years. A marginal resection of this tumor was performed. Currently, the patient is doing well without evidence of local recurrence or distant metastasis at 5 years after surgery.

A rare case of juvenile fibrosarcoma of eyelid.

Juvenile fibrosarcoma is not an uncommon tumor in children; however, eyelid involvement is extremely rare. Very few cases have been reported in the literature. This is the first case of JFS of the eyelid in Tribhuvan University Teaching Hospital and the first diagnosed case in Nepal. Histologically, it is similar to adult fibrosarcoma but it has an excellent prognosis. Surgical excision is the mainstay of treatment; however chemotherapy has been proved effective. Here we present a case of juvenile fibrosarcoma of the eyelid in a four year female child presenting with a rapidly enlarging, painless, left upper eyelid mass. An incisional biopsy, from the eyelid mass, showed typical histopathological features of juvenile fibrosarcoma.

Cáncer experimental e inflamación sistémica en un modelo murino / Systemic inflammation and experimental cancer in a murine model

La asociación entre cáncer e inflamación en un órgano o tejido se encuentra sólidamente establecida. En efecto, se sabe que en sitios de inflamación crónica, existe una mayor probabilidad de que se origine un tumor y que procesos inflamatorios locales pueden acelerar el crecimiento de tumores preexistentes en animales y seres humanos. Por otro lado, la relación entre cáncer e inflamación sistémica ha sido menos estudiada. En este trabajo, demostramos que el crecimiento de un fibrosarcoma de ratón (MC-C) fue acompañado por inflamación sistémica, evidenciada por neutrofilia y por un aumento de la concentración sérica de las citoquinas pro-inflamatorias interleuquina-1 beta (IL-1 beta), interleuquina-6 (IL-6) y factor de necrosis tumoral-alfa (TNF-alfa) y de las proteínas de fase aguda C reactiva (CRP) y A amieloide (SAA). Hubo un pico de estas moléculas poco después de la inoculación del tumor, que cayó a valores normales después de la primera semana, para luego comenzar a incrementarse progresivamente en función del tamaño tumoral. Una variación similar fue vista en el porcentaje de neutrófilos polimorfonucleares (PMN) circulantes. En ratones portadores de tumores grandes la mayoría de los PMN exhibían activación evidenciada por aumento en la generación de especies reactivas del oxígeno y alta expresión de los marcadores Gr1+/Mac1+. La inoculación de tioglicolato, que produce una inflamación sistémica transitoria, aceleró el crecimiento de MC-C, mientras que el tratamiento anti-inflamatorio con indometacina revirtió ese efecto. Esto sugiere que MC-C podría utilizar el fenómeno de inflamación sistémica que genera por sí mismo, como parte de su estrategia de crecimiento.

The link between cancer and inflammation in an organ or tissue has firmly been established on the basis that cancer tends to occur at sites of chronic inflammation and that local inflammatory processes can accelerate the growth of preexisting tumors in both animals and human beings. In contrast, the relationship between cancer and systemic inflammation has been less studied. In this work, we demonstrated that the growth of the murine fibrosarcoma MC-C, was accompanied by manifestations of systemic inflammation, as demonstrated by an increase in both the number of circulating polymorphonuclear neutrophils (PMN) and the serum concentration of the proinflammatory cytokines interleukin-1 beta (IL-1 beta), interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-alpha) and the acute phase proteins C reactive (CRP) and serum A amyloid (SAA). Two temporally separate peaks of systemic inflammation were detected during tumor development. The first was displayed during the first week after tumor inoculation. The second peak began around day 14 and its intensity was proportional to tumor size. In mice bearing a large MC-C tumor, a high number of circulating PMN and myeloid precursors were evident. Most of these cells exhibited activation evidenced by an increased reactive oxygen species generation and high expression of the Gr1+/Mac1+ markers. Inoculation of thioglycolate -which generates a transient systemic inflammation- accelerated the growth of MC-C tumor and reciprocally, inhibition of such systemic inflammation by using indomethacin, prevented that enhancing effect. This suggests that the systemic inflammation that the tumor generates on its own, could be part of its growth strategy.

Fibrosarcoma / Fibrosarcoma

Este caso clínico hace referencia a un tumor del tejido blando, el fibrosarcoma. Si bien este tipo de tumor no es muy frecuente en la población, el fibrosarcoma es uno de los más comunes dentro de este grupo. Debido a esto hemos querido complementarlo con una revisión bibliográfica que incluye sus características generales, los hallazgos histopatológicos, el diagnóstico y tratamiento propuesto en la literatura.

Lesión tumoral músculo-esquelética de evolución rápida: a propósito de un caso / Lesion skeletal tumoral muscle of quick evolution: a purpose of a case

El Fibrosarcoma es un tumor maligno de partes blandas primario o secundario a lesiones benignas pre-existentes, pueden ser endostal o parostal, comprende aproximadamente el 6.2 por ciento de los tumores primarios del hueso, predomimante en hombres, entre la 3era y 6ta década de vida, tiene predilección por el fémur, húmero, tibia y pelvis. Se describe paciente masculino de 25 años de edad quien inicia enfermedad en noviembre 2002 con aumento de volumen progresivo y dolor en rodilla derecha, acompañandose de limitación funcional; consulta en mayo 2003 con clínica exacerbada iniciándose su estudio.

Fibrosarcoma of penis--a case report.

Malignant mesenchymal tumours of penis are rare. A rare case of fibrosarcoma of penis in 60 year male is presented herewith.

Combined primary fibrosarcoma--malignant fibrous histiocytoma of the breast: a case report.

Malignant mesenchymal tumours of the breast are relatively rare tumours that have yet to receive universal approval in terms of terminology. With classification based on histogenesis rapidly gaining acceptance, we present a case with histological features of both fibrosarcoma and malignant fibrous histiocytoma, with the former predominating. An unusual finding was the presence of large areas with haemangiopericytomatous appearance. The literature on changing concepts regarding classification of malignant mesenchymal tumours of the breast is reviewed.