Extremities Soft Tissue Sarcomas, more Common and as Dangerous as Bone Sarcomas / Sarcomas de partes moles nos membros, mais comuns e tão graves quanto os sarcomas ósseos

Abstract Musculoskeletal sarcomas are rare diseases that require attention. They often present high degree of malignancy at diagnosis and, if underestimated, they can evolve aggressively locally and systemically. They present as soft tissues arcoma and bone sarcomas, with soft tissue being four to five times more common. Most soft tissue sarcomas occur in the extremities. The most common subtypes in children and adolescents are rhabdomyosarcoma and synovial sarcoma; in adults, undifferentiated pleomorphic sarcoma, liposarcoma, leiomyosarcoma, myxofibrosarcoma and synovial sarcoma; all with a high degree of histological malignancy. Many soft tissue sarcomas are confused with benign soft tissue tumors, 100 times more common, so they are resected without the necessary planning, resulting in amputation of a limb that could have been preserved. As in all cancers, the most important prognostic factor is metastatic disease. When it is present, the overall survival rate falls around 20 to 30%. Survival rates are generally similar between bone and soft tissue sarcomas. So soft tissue sarcomas, in addition to being more prevalent, are as aggressive as bones arcomas, deserving a lot of attention from orthopedic surgeons, who are often the first line of care of carriers of these tumors.

Resumo Os sarcomas musculoesqueléticos são doenças raras que exigem atenção. Frequentemente, apresentam alto grau de malignidade ao diagnóstico e se subestimados podem evoluir de forma agressiva local e sistemicamente. Apresentam-se como sarcoma de partes moles e sarcomas ósseos, sendo os de partes moles quatro a cinco vezes mais comuns. A maioria dos sarcomas de partes moles ocorre nos membros. Os subtipos mais comuns nas crianças e adolescentes são o rabdomiossarcoma e o sarcoma sinovial, nos adultos o sarcoma pleomórfico indiferenciado, lipossarcoma, leiomiossarcoma, mixofibrossarcoma e sarcoma sinovial; todos de alto grau de malignidade histológica. Muitos sarcomas de partes moles são confundidos com tumores benignos de partes moles, 100 vezes mais comuns, por isso são ressecados sem o planejamento necessário, acarretando em amputação de um membro que poderia ter sido preservado. Como em todos os cânceres, o fator prognóstico mais importante é a doença metastática. Na sua vigência, a taxa de sobrevida global cai em torno de 20 a 30%. As taxas de sobrevida no geral são parecidas entre os sarcomas ósseos e de partes moles, portanto o sarcoma de partes moles, além de mais prevalente, mostra-se tão agressivo quanto os sarcomas ósseos, por isso merece muita atenção dos ortopedistas que frequentemente são a primeira linha de atendimento dos portadores destes tumores.

Tumores hepáticos primarios malignos no hepatocarcinoma ni colangiocarcinoma / Primary malignant hepatic tumors other than hepatocarcinoma or cholangiocarcinoma: a series of cases

Background: There are several types of primary malignant hepatic tumors (PMHT) other than hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC): they are infrequent and poorly known. Imaging studies could help characterize the lesions and may guide the diagnosis. However, the definitive diagnosis of PMHT is made by pathology. Aim: To review a registry of liver biopsies performed to diagnose hepatic tumors. Patients and Methods: Review of a pathology registry of liver biopsies performed for the diagnosis of liver tumors. Among these, 25 patients aged 57 ± 17 years, 60% males, in whom a liver tumor other than a HCC or CC was diagnosed, were selected for review. The medical records of these patients were reviewed to register their clinical characteristics, imaging and the pathological diagnosis performed during surgery and/ or with the percutaneous liver biopsy. Results: Ten patients (40%) had neuroendocrine tumors, six (24%) had a lymphoma and four (16%) had hepatic hemangioendothelioma. Angiosarcoma and sarcomatoid carcinoma were diagnosed in one patient each. In 22 patients (88%), neither clinical features nor imaging studies gave the correct diagnosis. Four patients (16%) had chronic liver disease. The most frequent symptoms were weight loss in 28% and abdominal pain in 24%. Conclusions: The most common PMHT other than HCC and CC were neuroendocrine tumors and lymphomas. Imaging or clinical features were not helpful to reach the correct diagnosis.

Cáncer Bucomaxilofacial en Niños III: Tumores Malignos del Tejido Blando / Oral and Maxillofacial Cancer in Children III: Malignant Soft Tissue Tumors

RESUMEN: El grupo de neoplasias malignas de tejido blando de la región de cabeza y cuello en pacientes pediátricos está representado por carcinomas, sarcomas, melanomas y tumores de diferenciación incierta. La neoplasia más prevalente en la población pediátrica es el Rabdomiosarcoma, seguido por el carcinoma de células escamosas. Los rangos de presentación son muy amplios, siendo los grupos entre 2-6 años y 15-19 años los que presentan mayor incidencia. Se ha planteado que la etiología de estas neoplasias es incierta. El tratamiento de estas neoplasias es comúnmente de enfoque multimodal, combinando un procedimiento quirúrgico con quimioterapia y radioterapia. El pronóstico y sobrevida del paciente dependerán principalmente del momento en que se realice el diagnóstico de la lesión. Un diagnóstico y tratamiento temprano favorecen las posibilidades de sobrevida y el pronóstico del paciente. Este estudio corresponde a la 3ra parte de "Cáncer bucomaxilofacial en niños". Se hará referencia a los distintos tumores malignos del tejido blando en la población pediátrica en el territorio de cabeza y cuello, abarcando sus generalidades, etiología, epidemiología, tratamiento y pronóstico.

ABSTRACT: Head and neck malignant tumors in pediatric patients comprise carcinoma, sarcoma, melanoma and tumours of uncertain differentiation. Within the pediatric population, the most prevalent neoplasm is rhabdomyosarcoma, followed by squamous cell carcinoma. There is a wide range in the presentation, and it varies significantly with age groups of 2-6 and 15-19 year-olds who present the higher incidence rates. For this reason, it has been suggested that the etiology of head and neck neoplasms remains unclear. Treating these pathologies usually involves a multimodal approach that combines surgery, radiation and chemotherapy. Prognosis and survival rates depend mainly of the stage at the time of diagnosis. Early diagnosis and treatment can improve prognosis and survival rates. In this 3rd part of "Maxillofacial Cancer in Pediatric Patients", we studied a variety of malignant tumors in head and neck soft tissue from a paediatric sample. Specifically, we aim to analyze their etiology, epidemiology, treatment and prognosis.

Liposarcoma: A Rare Case Report with Review of Literature.

Liposarcoma is one of the most common malignant mesenchymal neoplasms, comprising approximately 15% of all soft-tissue sarcomas. First described by “Virchow” in 1857, it has been extensively reported in the literature, although its incidence remains exceedingly rare in the head and neck region with an annual incidence estimated to be 2.5/1 million inhabitants in population-based studies. It is one of the most common malignant mesenchymal neoplasms, comprising approximately 15% of all soft-tissue sarcomas. It is a heterogeneous disease with distinct sub-entities presenting with differential clinical behavior. The purpose of this article is to report an additional case of liposarcoma of the buccal vestibule and to review the literature.

Comorbidity is Independently Associated with Poor Outcome in Extremity Soft Tissue Sarcoma

BACKGROUND: Comorbidity has not been examined as an independent prognostic factor in soft tissue sarcoma (STS). We examined the prognostic impact of comorbidity on oncologic outcome in STS with an adjustment for possible confounding factors. METHODS: A retrospective review was performed on 349 patients who had undergone surgery for high-grade localized STS of extremity at our institute. Conditions known to alter the risk of mortality, as defined in the Charlson comorbidity index, were classified as comorbidities and 43 patients (12%) had at least one comorbidity at the time of surgery. The association of comorbidity and oncologic outcomes of local recurrence-free survival (LRFS) and disease-specific survival (DSS) were tested with adjustment for confounding factors. RESULTS: Comorbidity was associated with old age, high tumor grade, and large tumor size. The presence of comorbidity was independently associated with poor LRFS and DSS, even after adjusting for confounding factors including age and treatment variables. CONCLUSIONS: Our data suggest that the presence of comorbidity is an independent prognostic factor for extremity STS.

A fifty-year review of soft tissue sarcomas in Jamaica: 1958-2007 / Cincuenta años de revisión de los sarcomas de tejidos blandos en Jamaica: 1958-2007

OBJECTIVE: To determine the distribution of histologic subtypes of soft tissue sarcomas (STS) in Kingston and St Andrew, Jamaica, according to age and topography. METHODS: From the Jamaica Cancer Registry (JCR) archives, all cases of STS diagnosed between 1958 and 2007 were extracted. For each case, age, gender, histological diagnosis and anatomical site of tumour were recorded. Patients were categorized according to age at diagnosis as: children (0-14 years) and adults (> 14 years), and the distribution of histologic diagnoses with respect to age and anatomical site were analysed. RESULTS: There were 432 cases (67 children, 364 adults, one person of unknown age) of STS recorded in the JCR over the 50-year period (218 males, 214 females). The commonest STS in adults were "sarcoma, not otherwise specified [NOS]" (20.1%), malignant fibrous histiocytoma [MFH] (17.9%), fibrosarcoma (12.4%), liposarcoma (10.7%) and malignant peripheral nerve sheath tumour [MPNST] (10.2%). In children, they were neuroblastoma (38.8%), rhabdomyosarcoma (23.9%), "sarcoma, NOS" (9%), fibrosarcoma (6%) and MFH (6%). In adults, the lower limb was the commonest location, followed by trunk and/or upper limb for MFH, fibrosarcoma and liposarcoma, and head and neck for MPNST. In children, head and neck was the commonest site for rhabdomyosarcoma, head and neck and upper limb for MFH, retroperitoneum for neuroblastoma and trunk for fibrosarcoma. CONCLUSION: A high proportion of soft tissue sarcomas in Jamaica are unclassified and the anatomical distribution of common classified sarcomas shows some differences with the literature. Limited access to immunohistochemistry/molecular diagnostics and increasing core biopsy diagnosis may contribute to these phenomena.

OBJETIVO: Determinar la distribución de subtipos histológicos de sarcomas de tejido blando (STB) en Kingston y Saint Andrew, Jamaica, según la edad y la topografía. MÉTODOS: De los archivos del Registro de Cáncer de Jamaica, se extrajeron todos los casos de STB diagnosticados entre 1958 y 2007. Para cada uno de los casos, se registró la edad, el género, el diagnóstico histológico, y el sitio anatómico del tumor. Los pacientes fueron clasificados de acuerdo con la edad en el momento del diagnóstico, bajo las categorías de niños (0-14 años) y adultos (> 14 años), y se analizó la distribución de diagnósticos histológicos con respecto a la edad y el sitio anatómico. RESULTADOS: Se registraron 432 casos de STB (67 niños, 364 adultos, una persona de edad desconocida) en el JCR en un período de 50 años (218 varones, 214 hembras). Los STB más comunes en los adultos fueron "el sarcoma no especificado [NE]" (20.1%), el histiocitoma fibroso maligno [HFM] (17.9%), el fibrosarcoma (12.4%), el liposarcoma (10.7%), y el tumor maligno de la vaina del nervio periférico [TMVNP] (10.2%). En los niños, se trató de los neuroblastomas (38.8%), los rabdomiosarcomas (23.9%), "los sarcomas NE" (9%), los fibrosarcomas (6%), y los HFM (6%). En los adultos, los miembros inferiores fueron el lugar más común, seguido del tronco y/o los miembros superiores para el HFM, el fibrosarcoma y el liposarcoma; y la cabeza y el cuello para el TMVNP. En los niños, la cabeza y el cuello fueron el sitio más común para el rabdomiosarcoma; la cabeza, el cuello y los miembros superiores para el HFM; el retroperitoneo para el neuroblastoma; y el tronco para el fibrosarcoma. CONCLUSIÓN: Una proporción alta de sarcomas de tejidos blandos en Jamaica no están clasificados, y la distribución anatómica de sarcomas clasificados comunes muestran algunas diferencias con la literatura. El acceso limitado a los diagnósticos moleculares/inmunohistoquímicos, y el aumento de los diagnósticos centrales, pueden contribuir a estos fenómenos.

Whole saliva physico-biochemical changes and quality of life in head and neck cancer patients following conventional radiation therapy: A prospective longitudinal study.

Background: We investigated the physico-biochemical changes in saliva and its relation to quality of life (QOL) in head and neck cancer patients following conventional radiation therapy (RT). Materials and Methods: 53 consecutive head and neck cancer patients underwent conventional RT using telecobalt photons. We analyzed objective sialometry and sialochemical parameters of salivary gland function and a physician reported Oral Assessment Protocol to assess the patients' QOL during (baseline, 3 and 6 weeks) and post RT (3 and 6 months). Statistical analysis was done using SPSS software (version 15.0; SPSS, Inc., Chicago, IL, USA). Results: Stimulated salivary flow rates had shown a consistent decline during and in post-RT analysis (P < 0.001). A significant correlation was seen between mean salivary flow rates at 6 months post-RT and mean salivary electrolytes and amylase levels during the same period (P < 0.001). Mean global QOL scores had significantly worsened during RT and were still significantly poorer at 6 months than initial pre-RT levels (P < 0.001). Further, significant correlation was established between salivary pH values with global QOL scores at 6 months (P = 0.05). Conclusions: Radiation-induced hyposalivation invariably persists and correlates with poor global QOL scores seen during and following conventional RT. Post RT, there is a trend for biochemical reversal toward pre-irradiation levels suggesting a subsiding inflammation or a probable functional recovery.

Cáncer laríngeo de histología inhabitual / Unusual histology laryngeal cancer

Introducción: El cáncer laríngeo en 95 por ciento de los casos corresponde a carcinomas escamosos, siendo el porcentaje restante de diversos tipos histológicos. Objetivo: Determinar la frecuencia de cáncer laríngeo de histología inhabitual en nuestro centro. Material y método: Estudio retrospectivo, revisión de fichas clínicas de pacientes con diagnóstico histopatológico de cáncer laríngeo evaluados en el Servicio Otorrinolaringología Hospital San Juan de Dios, entre años 2000-2008. Se describen sexo, edad, tipo histológico, hábito tabáquico y localización tumoral, realizándose un análisis descriptivo de los datos. Resultados: Se incluyeron 85 pacientes; 74 casos (87,1 por ciento) de sexo masculino; promedio edad 64 años; se encontraron 5 casos de tumores de histología inhabitual (5,9 por ciento); un caso respectivamente de: condrosarcoma, tumor carcinoide, carcinoma adenoideo-quístico, carcinoma sarcomatoide y carcinoma verrucoso. Discusión: Se podría sospechar histología inhabitual con base estadística ante un paciente menor de 60 años y con un tumor de localización subglótica. Además, sexo femenino, no fumador y más de una toma de muestra para biopsia. Conclusión: Los tumores de histología inhabitual se encontraron en 5,9 por ciento de los casos, similar a lo reportado en la literatura.

Introduction: The laryngeal cancer in 95 percent of the cases corresponds to squamous cell carcinomas, being the remaining percentage several histological different types. Aim: To determine the frequency of laryngeal cancer of unusual histology in our center. Material and method: Retrospective study review of clinical data of patients with histophatological diagnosis of laryngeal cancer evaluated in Otolaryngology Department ofSan Juan de Dios's Hospital between years 2000-2008. Sex, age, histological type, tobacco smoking and tumor location are described, being carried outa descriptive analysis ofthe data. Results: 85 patients were included; 74 cases (87,1 percent) ofmasculine sex; mean age 64 years; there were 5 cases of unusual histology tumors (5,9 percent); a case respectively of: chondrosarcoma, carcinoid tumor, adenoid-cystic carcinoma, sarcomatoid carcinoma and verrucous carcinoma. Discussion: It might suspect unusual histology with statistical base in patient younger than 60 years and tumor of subglotic location. Also, in feminine patient, not smoking and more than one capture ofsample forbiopsy Conclusion: The tumors of unusual histology viere found in a 5.9 percent of the cases, similar to the reponed in the literature.

P53 labeling index as a prognostic factor in adult soft tissue sarcomas an analysis of 97 patients

Soft tissue sarcomas are rare neoplasms with a dismal prognosis, accounting for 1 percent of all malignancies. Their management presents several challenges, particularly when the tumor is over 5 cm in diameter, when histological grade is high, when the tumor is localized in the trunk and when it has metastasized. Immunohistochemical findings have suggested a possible prognostic role for the immunodetection of p53 protein in soft tissue sarcomas. The present study investigated the possible association between p53 protein immunodetection and prognosis in a group of patients with soft tissue sarcomas receiving care at Hospital Araújo Jorge in Brazil. Ninety-seven adult patients were included in the study. Formaldehyde-fixed and paraffin-embedded tissue sections were used in the immunohistochemical analysis, which was carried out using DO-7 monoclonal antibody and the avidin-biotin-peroxidase complex (ABC) technique. Forty-one cases (42.2 percent) were positive for p53 protein immunodetection. No statistically significant association was found between p53 immunodetection and clinical or pathological features. The p53 protein immunodetection in tumor cells revealed a labeling index > 50 percent in 32 cases (78 percent) and < 50 percent in 9 cases (32 percent). The 5-year survival rate was higher for patients in whom p53 immunodetection was negative (52.8 percent) compared to those with positive immunodetection (38.9 percent); however, the difference between the two groups was not statistically significant (p=0.159). A p53 labeling index > 50 percent was significantly associated with a lower 5-year survival rate (85.7 percent versus 27.6 percent) (p= 0.017). These results suggest that the p53 labeling index is a negative prognostic factor for soft tissue sarcoma.

Recidiva local nos sarcomas de tecidos moles: fatores prognósticos / Local recurrence in soft tissue sarcoma: prognostic factors

OBJETIVO: Determinar os fatores prognósticos associados com a recorrência local nos pacientes submetidos a tratamento de sarcoma de tecidos moles em extremidades. MÉTODOS: Foram avaliados retrospectivamente 30 pacientes tratados por ressecção cirúrgica de sarcoma de tecidos moles localizados em extremidades, com seguimento 36,5 ± 12,2 meses. Foram avaliados como fatores prognósticos: idade, sexo, localização, profundidade, localização em compartimentos anatômicos, tamanho, manipulação prévia ao tratamento definitivo, margens cirúrgicas, grau de malignidade histológica, presença de necrose e invasão vascular à histologia. Estes fatores foram correlacionados com a ocorrência de recidiva local do tumor por uma análise univariada e multivariada, sendo considerados significativos valores de p = 0,05. RESULTADOS: A recorrência local apresentou correlação significativa, tanto na análise univariada como multivariada, com a localização extra-compartimental (p = 0,001) e com o alto grau de malignidade (p = 0,024). Não houve associação com a idade (p = 1,000), com o sexo (p = 1,000), com a localização em segmentos corporais (p = 0,544), com a profundidade (p = 0,288), com o tamanho (p = 0,694), com as margens cirúrgicas (p = 0,384), com a manipulação prévia (p = 0,461), com a presença de necrose (p = 0,461) ou invasão vascular (p = 1,000). CONCLUSÃO: A recorrência local apresentou correlação com a localização extra-compartimental e com o alto grau de malignidade dos tumores.

OBJECTIVE: To determinate the prognostic factors associated with the local relapse in patients treated for soft tissue sarcoma in extremities. METHODS: Retrospectively, 30 patients were evaluated after being treated with surgical resection of soft tissue sarcoma located in extremities, with a outcome of 36,5 ± 12,2 months. The prognostic factors evaluated were: age, sex, location, depth, location in the anatomic compartments, size, tumor previous manipulation, surgical margins, grade of histological malignance, presence of histological necrosis or histological vascular invasion. These factors were correlated with the local relapse of the tumor with a univariated and multivariated analysis with significance when p = 0,05. RESULTS: Local relapse presented significant correlaction in univariated and multivariated analysis with extra-compartimental location (p = 0,001) and with high grade histological malignance (p = 0,001). There was no significant association with age (p = 1,000), sex (p = 1,000), body location (p = 0,544), depth (p = 0,288), size (p = 0,694), surgical margins (p = 0,384), previous manipulation (p = 0,461), necrosis (p = 0,461) or with the presence of vascular invasion (p = 1,000). CONCLUSION: Local relapse presents correlaction with extra-compartimental location and with high grade malignancy tumors.

Patho-epidemiology of cancer corpus uteri in Karachi South '1995-1997'.

AIM: To provide demographics and pathology of cancer of the uterine corpus in Karachi. METHODOLOGY: Data for 66 incident cases of cancer corpus uteri, ICD-10 category C54-5 registered at the Karachi Cancer Registry, for Karachi South, during a 3 year period, 1st January, 1995 to 31st December 1997 were reviewed. RESULTS: Cancer uterine corpus (1995-97) was the sixth most common malignancy, following breast, oral cavity, ovary, esophagus and cervix. The age standardized incidence rate (ASR) world and crude incidence rate (CIR) per 100,000 were 6.4 (4.73 to 8.01) and 2.9 (2.18 to 3.57). The mean age was 53.7 years (SD 15.6; range 6-90 years). Fifty eight cases were endometrial carcinoma with ASR world and CIR per 100,000 of 5.77 (4.20 to 7.33) and 2.53 (1.88 to 3.18) respectively. Sarcomas comprised 6% of the cases. Approximately a third of the females (28.8%) were below 50 years of age. The age-specific curves showed a gradual increase from the fourth till the seventh decade, followed by an actual apparent decrease in risk after 70 years. Peak incidence was observed in the 65-69 year age group. Presenting symptoms were post-menopausal bleeding (86.4%) and purulent discharge (4%). Associated pathologies included adenomyosis, adenomatous hyperplasia (12% each) or leiomyoma (8%). Associated clinical conditions were diabetes mellitus and hypertension (4% each). The majority of the cases presented as well differentiated (39.4%), localized (59.1%) lesions. CONCLUSION: The incidence of cancer corpus uteri in Karachi South reflects a moderate risk population, predominantly middle aged with a higher socio-economic status. On the average the malignancy is observed a decade earlier then reported elsewhere. This calls for in-depth investigation of risk factors and identification of underlying etiology.

A review of soft tissue sarcoma

Background : Soft tissue sarcomas (STS) are a heterogeneous group of rare tumours that arise predominantly from the embryonic mesoderm. They present most commonly as an asymptomatic mass originating in an extremity but can occur anywhere in the body; particularly the trunk; retroperitoneum; or the head and neck. They account for about 0.7of all adult malignancies. Method : A review of the literature of STS was undertaken with emphasis on current approach in management. Result : Despite recent advances in the knowledge of the molecular biology of STS; there is yet no identifiable aetiology in most cases. Tru-cut biopsy is a safe; accurate and economical procedure for diagnosing STS. Enough tissue is usually obtained for use in several diagnostic tests such as electron microscopy and cytogenetic analysis. With the advent of Computed Tomography (CT); Resonance Imaging (MRI) and Magnetic Resonance Angiography (MRA); tumours can easily be delineated from muscle groups; bone and neurovascular structures. Surgery remains the main potentially curative therapy for STS. In the last two decades; the role of adjuvant radiotherapy has revolutionized the treatment from a situation where amputation was the standard treatment for extremity STS to the present time where limb sparing surgery is appropriate in more than 90of patients. Postoperative adjuvant chemotherapy significantly improves the overall and disease free survival for patients with large size and high grade sarcomas. Conclusion : optimal results of treatment require multidisciplinary interaction between the referring practitioner; the treating surgeon; the pathologist; the radiotherapist and the chemotherapist.1

Sarcomas primarios de mama / Primary sarcomas of the breast

Los sarcomas primarios de mama son extremadamente raros y representan menos del 1% de lostumores mamarios comunicados en la literatura. Entre los años 1999 y 2004 se diagnosticaron ennuestra institución 1315 tumores malignos de mama, entre ellos nueve correspondieron a sarcomas primarios:tres angiosarcomas, un leiomiosarcoma, un sarcoma fibromixoide de bajo grado, un dermatofibrosarcoma, unliposarcoma, un osteosarcoma y un tumor maligno de la vaina de los nervios periféricos. Se revisaron los preparadoshistológicos, teñidos con técnicas de rutina y de inmunoperoxidasa, estableciéndose la fracción de crecimiento(FC) y sobre-expresión de proteína p53. Se estudiaron también las historias clínicas de las pacientespara determinar tipos de evolución (favorable y desfavorable). La incidencia observada (0.7%) es similar a lasya publicadas por otros autores. La FC se correlacionó con la evolución, siendo un factor pronóstico desfavorablecuando fue mayor o igual al 30%. La mayoría de los tumores (67%) mostró sobre-expresión de proteína p53(mayor o igual al 20% de tinción nuclear) pero esto no demostró tener una relación directa con la evolución decada neoplasia.

Primary sarcomas of the breast are extremely rare with less than1% of all malignant tumours of the breast reported in literature. At our Institution 1315 malignanttumours of the breast were diagnosed between 1999-2004; nine of them corresponded to primary sarcomas:angiosarcoma (3), leiomyosarcoma (1), low-grade fibromyxoid sarcoma (1), dematofibrosarcoma protuberans (1),liposarcoma (1), osteosarcoma (1), malignant peripheral nerve sheath tumour (1). Histopathological specimensstained with routine techniques and immunoperoxidase were reviewed; proliferation index and p53 over-expressionwere also determined. Patients´ clinical reports were also reviewed to determine prognosis (favorable andunfavorable). The incidence observed (0.7%) is similar to those already published by others authors. Proliferationindex was correlated with type of evolution, being an unfavourable prognosis factor when it was equal ormajor to 30%. Most of the tumours (67%) showed p53 (mayor or equal to 20% of nuclear staining) over-expressionbut this did not show a direct relationship with the evolution of each neoplasm.

Tasas de incidencia y caracterización de sarcomas en la provincia de Valdivia / Incidence rate and characterization of sarcomas in the province of Valdivia

Antecedentes: Los sarcomas primarios de hueso y de tejidos blandos son neoplasias infrecuentes. Los sarcomas de tejidos blandos más comunes son fibrohistiocitoma maligno, liposarcoma y leiomiosarcoma, los sarcomas de hueso más comunes son osteosarcoma, condrosarcoma y sarcoma de Ewing. La frecuencia relativa de cada tipo histológicos presenta variaciones etáreas. Método: Identificamos los casos de sarcomas diagnosticados en la provincia de Valdivia en el período 1999-2003, analizando incidencia y características generales. Resultados: Encontramos una incidencia de entre 2,8-6,2/100.000 hab. Hubo predominio de la población masculina (55 por ciento). La edad de presentación promedio fue 50,4 años. El tipo histológico mas frecuente fue el fibrohistiocitoma maligno y la localización más frecuente fue la visceral. Discusión: Encontramos similitudes con la literatura internacional en cuanto a incidencia, edad de presentación, tipo histológico más frecuente y tipo de tejido de origen. Se observó diferencias en cuanto a la localización anatómica y distribución por sexo.

Sarcoma in Iran.

PURPOSE: To review the clinical characteristics of 1470 sarcoma cases and to define the factors in patients that predict out come, relapse and survival. METHODS: Retrospective analysis of the database for the period 1991-2002, focusing on demographic, tumor related and treatment related variables, relapse free survival (RFS) and overall survival (OS) using the Kaplan- Meier method. Statistical significance was evaluated using the chi square and t- tests for univariate influence and a Cox regression model for multivariate influence. RESULTS: Mean age was 30 years. The male to female ratio was 3/2 and 23% of the cases were under 16 years of age. Median tumor diameter was 10.5 centimeters. The bone to soft tissue sarcoma ratio was 3/1 in children and 1/3 in adults. Osteosarcoma, Ewing's tumours and rhabdomyosarcomas accounted for 83% of childhood tumors. In adults osteosarcomas, synovial sarcomas and malignant fibrous histocytomas (MFHs) were the most common subtypes. Mean follow up time was 56 months. Of the total, 25% had initial metastasis, 86% received chemotherapy and 41 % underwent radiotherapy. The main prognostic factors for survival were tumor size, margin of surgery, neurovascular involvement in the pathological report, initial metastasis and no complete response to first therapy. Adjuvant radiotherapy, small tumor size, curative surgery with chemotherapy and free surgical margins were significantly associated with reduced recurrence. CONCLUSION: Our patients are characterised by diagnosis with a large tumor size, advanced stage of disease and short survival. A complete response to primary therapy is the main independent variable for overall survival. Earlier diagnosis and an experienced team including surgical, medical and radiotherapy oncologists are needed for a better response and longer survival of patients.