Presentación atípica de cáncer de tiroides en Hospital Escuela Universitario, Honduras / Atypical Thyroide Cancer, University School Hospital, Honduras

A las masas o protuberancias en la glándula tiroides se les llama nódulos tiroideos, la mayoría son benignos, pero alrededor de 2-3 de 20 son cancerosos. El carcinoma anaplásico, representa alrededor del 2%, es más frecuente en el sexo femenino (proporción 3:1). Objetivo: presentar un caso atípico de tumor canceroso en tiroides, técnicas diagnósticas y tratamiento aplicado. Presentación decaso clínico: paciente femenina de 51 años, procedente de Tegucigalpa, evangélica, ama de casa, con historia de masa en región anterior del cuello de un año de evolución, crecimiento progresivo, dolorosa a la palpación, que presentaba úlcera sangrante de un mes de evolución y 8 cm de diámetro. Examen físico: masa de 15 x 15 cm, móvil, asimétrica, bordes irregulares, consistencia dura, adherida a planos profundos. Exámenes complementarios: T4 libre 1.14 ng/dL, hormona estimulante tiroidea 2.10 mU/mL. Ultrasonido de cuello + Doppler:masa sólida, lóbulos aumentados de tamaño y con microvascularidad. Tomografía contrastada: masa en cartílago tiroides de 7.6 x 6.9 cm, bordes definidos, múltiples calcificaciones, sin áreas de necrosis, sin compresión de estructuras adyacentes; ganglios bilaterales aumentados de tamaño. Biopsia por Aspiración con Aguja Fina:carcinoma anaplásico de tiroides con focos de neoplasia papilar y variante de células altas. Tratamiento: tiroidectomía total, mostrando glándula tiroides aumentada de tamaño, peso aproximado de 200 g; levotiroxina 100 µg vía oral c/día de por vida, calcio 2 tabletas vía oral c/día por un mes. Conclusión: El estudio confirmatorio de cáncer anaplásico de tiroides es la biopsia por aspirado con aguja fina; sin embargo, la biopsia por disección, confiere un diagnóstico definitivo cuando existen dudas sobre la toma de muestra o cuando la clínica del paciente no corresponda con el diagnóstico de la biopsia por aspiración con aguja fina...(AU)

What is the Meaning of Anaphase-Promoting Complex 7 in Malignant Neoplasms?

No abstract available.

Supratentorial Pilocytic Astrocytoma Mimicking Convexity Meningioma with Early Anaplastic Transformation: A Case Report

Meningiomas and pilocytic astrocytomas are benign intracranial tumors. Pilocytic astrocytomas arises frequently at the posterior fossa in childhood. Meningiomas have several image findings, such as a dural tail sign, bony erosion, and sunburst appearance on angiography. However, pilocytic astrocytomas with these findings have been rarely reported. In this report, we describe a mass with typical image findings of a meningioma, but diagnosed as a supratentorial pilocytic astrocytoma with early anaplastic transformation.

Anaplastic Ganglioglioma of the Pineal Region ­ A Case Report

Introduction Gangliogliomas are tumors commonly found in the temporal lobe and related to seizures; their appearance in the pineal region is rarely described. This report characterizes the first case of anaplastic ganglioglioma of the pineal region. Case Report The authors describe the case of a 32-year-old woman that developed progressive headache. An MRI investigation revealed a pineal tumor. The patient tested negative for biomarkers and underwent surgery through supracerebellar infratentorial approach and achieved gross total resection of the tumor in a challenging location. Pathological analysis revealed a biphasic neoplasm with the following two distinct phenotypes in separate fields: an immature neuronal component with several atypical mitoses and a mature astrocytic component with bipolar cells, microcysts, and eosinophilic bodies. The Ki67/MIB1 proliferation index was 20­30% in localized hotspots. Based on the pathological findings, the tumor was defined as an anaplastic ganglioglioma World Health Organization (WHO) grade III. Discussion/Conclusion Gangliogliomas are classified as glioneural neoplasms based on the histologic findings described as a mixture of neoplastic astrocytes and neurons. Moreover, these tumors represent 0.4­1.3% of tumors of the central nervous system. Authors describe de novo anaplastic ganglioglioma as 1% of the largest series. Gross total resection and adjuvant treatment may play important role in patients' prognostic. In this case, due to the malignant anaplastic component of her tumor, the patient received treatment with temozolamide and radiotherapy after gross total resection of the lesion.

Introdução Gangliogliomas são tumores comumente encontrados no lobo temporal e se relacionam com crises epilépticas; o aparecimento desses tumores na região da pineal é raramente descrito. Este relato caracteriza o primeiro caso de ganglioglioma anaplásico da região da pineal. Relato de Caso Paciente do sexo feminino, 32 anos, apresentou-se com cefaleia de piora progressiva. Investigação com ressonância magnética revelou tumor na região da pineal. Os biomarcadores para tumores da pineal foram negativos e a paciente foi submetida a microcirurgia com o acesso supracerebelar e infratentorial atingindo ressecção total da lesão. A análise patológica revelou neoplasia bifásica com dois fenótipos distintos em campos separados: um componente era composto por células neuronais imaturas com inúmeras mitoses atípicas e o segundo componente era composto por astrócitos maduros, microcistos e corpos eosinofílicos. Foi encontrado um índice proliferativo Ki67/M1B1 de 20­30%. Baseado nos achados anatomopatológicos, o tumor foi definido como ganglioglioma anaplásico grau III da OMS. Discussão/Conclusão Gangliogliomas são classificados como neoplasias glioneurais baseado nos achados histológicos descritos como misto de neoplasia neuronal e glial; esses tumores representam 0,4­1,3% de todos os tumores do sistema nervoso central. Ganglioglioma anaplásico de novo tem sido descrido em 1% nas maiores series de gangliogliomas. Ressecção total da lesão e tratamento adjuvante desempenham um papel importante no prognóstico dos pacientes. Devido ao componente anaplásico do tumor em questão, a paciente foi tratada com temozolamida e radioterapia após ressecção total da lesão.

Epidemiology of Oral Lichen Planus in a Cohort of South Indian Population: A Retrospective Study

BACKGROUND: Oral lichen planus (OLP) is an immune-mediated potentially malignant disorder of the oral cavity. Dysplastic OLP has an altered cytogenic profile and can progress into oral squamous cell carcinoma. The epidemiology of OLP is well-described in several relatively large series from various geographic locations, whereas such series from southern India is rare. The aim of the present study was to determine the epidemiology of OLP in a cohort of South Indian population. METHODS: All the case data records of 29,606 patients who visited Mar Baselios Dental College and Hospital, Kerala, India from 2014 to 2015 were retrospectively reviewed. For data review, 122 patients of OLP were selected Estimated were type, number, and location of lesions, clinical manifestation, age of the patient, gender, onset and duration of lesion, stressful life style, habits, skin involvement and associated systemic illness, and presence/absence of dysplasia. RESULTS: When the distribution of OLP among the gender was considered, we found more prevalence in females than males. Fifty-seven percent of patients were associated with stressful lifestyle. Reticular lichen planus was the most common clinical subtype found. Bilateral buccal mucosal was the common site, when the distribution of sites of OLP were compared (P < 0.05). Hypersensitivity reaction was frequently associated with systemic illness with OLP (P < 0.05). Anaplasia was found among 5% of lichen planus lesions. CONCLUSIONS: OLP patients had high incidence of hypersensitivity reactions and 5% of OLP lesions showed anaplasia. Long term follow-up is necessary to monitor the recurrence, prognosis, and malignant transformation of OLP.

Gliosarcoma cerebeloso asociado aneurofibromatosis tipo I: presentación de caso / Cerebellar gliosarcoma associated with neurofibromatosis type I: case presentation

El Gliosarcoma es un raro Glioblastoma que contiene tantos elementos gliales comparable con un Glioblastoma como componentes mesenquimal. Aproximadamente entre 2-8% de todos los Glioblastomas están asociados con elementos sarcomatosos. Clínica y genéticamente muy parecido a los Glioblastomas, excepto por la ausencia de amplificación del EGFR. El gliosarcoma es un tumor de alto grado de malignidad y pobre pronóstico, con alta tasa de recurrencia. Presentamos el caso de un paciente masculino de 54 años de edad con diagnóstico de gliosarcoma cerebeloso, asociado a Neurofibromatosis tipo I. La NF I es el síndrome hereditario más común que predispone a la neoplasia, es una enfermedad polifacética asociado no sólo a tumores benignos.

Gliosarcoma are rare glioblastomas that contain an anaplastic glial component comparable to a glioblastoma, as well as,a mesenchymal component, that have a biphasic pattern. Approximately 2-8% of all glioblastomas are associated with a sarcomatous element. Clinically and genetically close to glioblastomas, except for the absence of EGFR amplification. The Gliosarcoma is a high-grade tumor of malignity and poor prognosis, with high rate of recurrence. We present the case of a masculine patient of 54 elderly years, with diagnosis of Gliosarcoma Cerebellar associate to Neurofibromatosis type I. The NF I is the hereditary syndrome more common that predisposes to the tumor, it is a versatile disease that not only becomes a partner of benign tumors.

WHO Grade IV Gliofibroma: A Grading Label Denoting Malignancy for an Otherwise Commonly Misinterpreted Neoplasm

We report a 50-year-old woman with no relevant clinical history who presented with headache and loss of memory. Magnetic resonance imaging showed a left parieto-temporal mass with annular enhancement after contrast media administration, rendering a radiological diagnosis of high-grade astrocytic neoplasm. Tumour sampling was performed but the patient ultimately died as a result of disease. Microscopically, the lesion had areas of glioblastoma mixed with a benign mesenchymal constituent; the former showed hypercellularity, endothelial proliferation, high mitotic activity and necrosis, while the latter showed fascicles of long spindle cells surrounded by collagen and reticulin fibers. With approximately 40 previously reported cases, gliofibroma is a rare neoplasm defined as either glio-desmoplastic or glial/benign mesenchymal. As shown in our case, its prognosis is apparently determined by the degree of anaplasia of the glial component.

Evaluation and Comparison of the Biopathology of Collagen and Inflammation in the Extracellular Matrix of Oral Epithelial Dysplasias and Inflammatory Fibrous Hyperplasia Using Picrosirius Red Stain and Polarising Microscopy: A Preliminary Study

BACKGROUND: The role of tumour inflammation and the dysplastic epithelial-stromal interactions on the nature of collagen fibres in the extracellular matrix of dysplastic epithelium is not fully understood. The present study was aimed to evaluate and compare the inflammation and pathological stromal collagen (loosely packed thin disorganized collagen) present in mild, moderate and severe epithelial dysplasias with that of inflammatory fibrous hyperplasias. The basement membrane intactness of epithelial dysplasias was also evaluated to determine if dysplastic epithelial mesenchymal interaction has any role in the integrity of stromal collagen in epithelial dysplasia. METHODS: Oral epithelial dysplasias, inflammatory fibrous hyperplasia and normal oral mucosal samples were used for the study. Packing, thickness and orientation of collagen fibres in mild, moderate and severe grades of oral epithelial dysplasias (n = 24), inflammatory fibrous hyperplasia (n = 8) and normal oral mucosal samples (n = 8) were analysed based on the polarisation of collagen fibres in picrosirius red polarising stain under polarising microscope. RESULTS: All the grades of epithelial dysplasias showed greenish yellow birefringence confirming the presence of loosely arranged pathological collagen in the presence of moderate inflammation. All the cases of inflammatory fibrous hyperplasia showed red polarisation hue and moderate inflammation. A statistically significant difference was found in the packing and orientation of collagen when epithelial dysplasias and inflammatory fibrous hyperplasia were compared (P < 0.01). When the intactness of basement membrane integrity was compared in all the groups of epithelial dysplasia, a statistically significant result was obtained (P < 0.05). CONCLUSIONS: Presence of significant amount of loosely packed thin disoriented collagen even in mild epithelial dysplasia suggests that tumourigenic factors are released to connective tissue stroma much earlier than expected. Hence we suggest considering the integrity of extracellular matrix collagen, intactness of basement membrane and inflammation associated with dysplasia along with the anaplasia of epithelial cells in the microscopic assessment of dysplastic epithelium.

A Case Report of Botryoid Wilms Tumor: Has Different Clinical Manifestations / 임상소아혈액종양

A previously healthy, 16-month-old boy was admitted to hospital due to fever, vomiting, and persistent gross hematuria for 2 weeks. His imaging studies showed an inhomogeneous mass, in a left dilated renal pelvis and proximal ureter, which was suggestive of a kidney neoplasm. A stage I Wilms tumor limited to the nephrectomised kidney was diagnosed by histology. The margin of ureter and renal vessels were clear. There was no evidence of anaplasia, angiolymphatic invasion, or skeletal muscle differentiation. In addition, there were polypoid protrusions of tumor into the pelvicaliceal system, resulting in such a 'botryoid' appearance. The patient had been treated with adjuvant chemotherapy using 7 cycles of intravenous actinomycin-D and vincristine (NWTS regimen EE4A) for 5 months. During a 22-month follow-up period, there has been no evidence of tumor recurrence.

A Case Report of Botryoid Wilms Tumor: Has Different Clinical Manifestations / 임상소아혈액종양

A previously healthy, 16-month-old boy was admitted to hospital due to fever, vomiting, and persistent gross hematuria for 2 weeks. His imaging studies showed an inhomogeneous mass, in a left dilated renal pelvis and proximal ureter, which was suggestive of a kidney neoplasm. A stage I Wilms tumor limited to the nephrectomised kidney was diagnosed by histology. The margin of ureter and renal vessels were clear. There was no evidence of anaplasia, angiolymphatic invasion, or skeletal muscle differentiation. In addition, there were polypoid protrusions of tumor into the pelvicaliceal system, resulting in such a 'botryoid' appearance. The patient had been treated with adjuvant chemotherapy using 7 cycles of intravenous actinomycin-D and vincristine (NWTS regimen EE4A) for 5 months. During a 22-month follow-up period, there has been no evidence of tumor recurrence.

Anaplastic transformation of metastatic papillary thyroid carcinoma at shoulder mimicking soft tissue sarcoma.

A 52-year-old woman presented with fracture upper end of the left humerus after trivial trauma and aspiration cytology from the lytic lesion in the upper humerus seen on X-ray revealed a metastatic papillary carcinoma from the thyroid. Total thyroidectomy confirmed the papillary carcinoma thyroid. Post-operatively, she was given radioactive iodine (I-131) ablation therapy for 8 years and was asymptomatic during this period; however, for the last 1 year, she has been complaining of swelling in the shoulder, which did not respond to palliative radiotherapy and rapidly increased in size. Disarticulation of the shoulder joint was performed, which showed anaplastic carcinoma on histopathological examination. Anaplastic transformation of papillary carcinoma at the metastatic sites is well documented in the literature and is rare. However, the same has not been reported at the shoulder and from India before. Although soft tissue sarcomas are most common at this site, however, the possibility of anaplastic transformation should be kept in the differential diagnosis of rapidly enlarging painful mass in a known case of metastatic thyroid carcinoma to prevent misdiagnosis.

Aspiration Cytology of the Osteoclastic Variant of Anaplastic Thyroid Carcinoma: with Special Emphasis on the Undifferentiated Mononuclear Cells

Anaplastic thyroid carcinoma (ATC) is an uncommon aggressive malignant tumor, and the osteoclastic variant of ATC is extremely rare. We report here on the fine needle aspiration cytology of the osteoclastic variant of ATC in an 83-year-old woman. The smear was composed of many oval to slightly elongated undifferentiated mononuclear cells admixed with multinucleated osteoclast-like giant cells. The mononuclear tumor cells revealed inconspicuous nuclear pleomorphism and the nuclei were characterized by vesicular chromatin and an indented or lobulated nuclear membrane with conspicuous nuclear grooves. A few epithelial clusters suggestive of a papillary carcinoma component were also observed. Making the proper cytological diagnosis of the osteoclastic variant of ATC is helpful to determine the proper treatment modality for these patients.

Clinical Characteristics of Wilms' Tumor according to Age

PURPOSE: Wilms' tumor typically affects young children, with more than 80% of patients identified before 5 years of age. We evaluated the clinical features of Wilms' tumor according to age. MATERIALS AND METHODS: According to their age, 27 patients with Wilms' tumor who underwent radical nephrectomy were classified into group I (under 24 months: n=15), group II (24-48 months: n=4), and group III (over 48 months: n=8), respectively. We retrospectively reviewed tumor size, pathologic features, renal vein invasion, renal capsule invasion, bilaterality, lymphovascular invasion, stage, complete resection, and survival rate. RESULTS: The mean age of groups I, II, and III was 12.5+/-7.0 (range, 1-20), 33.3+/-5.7 (range, 24-39) and 126.9+/-89.1 (range, 54-300) months, respectively. Bilaterality (p<0.001) and male sexuality (p=0.039) were significantly related to early diagnostic age. Tumor stage (p=0.036) and blastema component (p=0.027) increased with age. Bilaterality and incomplete resection of Wilms' tumor were related to larger size (p<0.001) and vessel/lymph node invasion (p=0.02), respectively. There were no significant differences in tumor volume, anaplasia, capsule invasion, lymph node invasion, vessel invasion, complete resection, laterality, or recurrence according to age. There was also no significant difference in the survival rate according to age. CONCLUSIONS: Tumor stage and blastema component of Wilm's tumor increased with age. Bilaterality and male sexuality were related to an early diagnostic age. There was no significant difference in the survival rate according to age.

Anaplastic transformation of differentiated thyroid carcinoma

Anaplastic thyroid cancer [ATC] is one of the most aggressive malignancies that arise from transformation of preexisting differentiated thyroid cancer [DTC]. However, the carcinogenic mechanism of anaplastic transformation remains unclear. We describe a case for huge goiter diagnosed as papillary thyroid carcinoma, which underwent thyroidectomy. The final histology showed anaplastic transformation. The clinical and possible etiological aspects are discussed

Osteossarcomas humanos de alto grau: imunoexpressão de p53, erb-2 e P-glicoproteína, e correlação com o parâmetro anaplasia / P-glycoprotein, erb2 and p53 expression in high-grade human osteosarcomas and their correlation with anaplasia

INTRODUÇÃO: Osteossarcoma (OS), o mais freqüente tumor primário maligno do osso, tem comportamento local agressivo e alto índice de disseminação sistêmica. Os eventos que permitem o crescimento e a disseminação tumoral ainda permanecem controversos. Os estudos sobre a carcinogênese e a progressão dessa neoplasia, com base na imunoexpressão de c-erb-B2, P-glicoproteína (P-gp) e p53, apresentam resultados conflitantes acerca do real valor prognóstico e suas correlações com parâmetros histológicos. A anaplasia, em neoplasias na infância, constitui parâmetro histológico de agressividade tumoral e quimiorresistência. Nos OS primários ou metastáticos, seu significado permanece controverso. Por outro lado, em outras neoplasias humanas, a expressão do c-erb-B2 relaciona-se com p53, grau nuclear e outros parâmetros de agressividade. OBJETIVO: Avaliar a imunoexpressão de p53, c-erb-B2 e P-gp em OS, correlacionando os parâmetros entre si e com a presença de anaplasia. MÉTODO: O estudo incluiu 96 biópsias pré-quimioterapia de pacientes com OS de alto grau, diagnosticados entre 1991 e 2000. A pesquisa imuno-histoquímica de p53, P-gp e c-erb-B2 foi feita pela técnica da estreptoavidina-biotina-peroxidase. Foram considerados positivos os casos onde havia imunoexpressão em 10 por cento ou mais das células neoplásicas. Somente colorações membranosa (para cerb-B2 e P-gp) e nuclear (para p53) foram consideradas positivas. Anaplasia foi definida como no tumor de Wilms, sendo considerada presente ou ausente. RESULTADOS: Anaplasia pôde ser avaliada em 82/96 casos, estando presente em 29 (35,36 por cento). Imunoexpressão de p53 foi detectada em 25 dos 60 casos (36,23 por cento); de P-gp, em 30 dos 73 casos (41,1 por cento); e de c-erb-B2, em 22 dos 55 casos (40 por cento). Os resultados demonstraram associação entre as imunoexpressões de c-erb-B2 e p53 (p = 0,042), p53 e o parâmetro anaplasia (p = 0,015), anaplasia e Pg (p = 0.034) CONCLUSÕES: A imunoexpressão...

BACKGROUND: Osteosarcomas (OS), the most frequent primary malignant bone tumors, have aggressive local behavior and high rate of metastatization. The events that allow tumor growth and dissemination are still controversial. The studies about carcinogenesis and tumor progression in this neoplasia, which are based on c-erb-B2, P-glycoprotein (P-gp) and p53 immunoexpression, show conflicting results as to the real prognostic value and its correlations with histological parameters. Anaplasia in childhood neoplasias is a histological parameter of tumor aggressiveness and chemoresistance. In primary or metastatic OS, its meaning remains controversial. On the other hand, in other human neoplasias, c-erb-B2 expression is associated with p53, nuclear grade and other aggressiveness parameters. OBJECTIVE: The aim of the present study was to evaluate p53, c-erb-B2 and P-gp immunoexpression in OS, correlating the parameters with the presence of anaplasia. METHODS: This study included 96 pre-chemotherapy biopsies in patients with high-grade OS diagnosed between 1991 and 2000. The immunohistochemical evaluation of p53 and c-erb-B2 was carried out with the streptavidin-biotin-peroxidase technique. Cases were considered positive when there was immunoexpression in 10 percent or more neoplastic cells. Only membrane staining (for c-erb-B2 and P-gp), and nuclear staining (for p53) were considered positive. Anaplasia was defined as Wilms' tumor, and considered present or absent. RESULTS: Anaplasia was present in 29 out of 82 cases (35.36 percent); p53 immunoexpression was detected in 25 out of 60 cases (36.23 percent); P-gp, in 30 out of 73 cases (41.1 percent); and c-erb-B2, in 22 out of 55 cases (40 percent). The results demonstrated an association between c-erb-B2 and p53 immunoexpression (p = 0.042), p53 and parameter of anaplasia (p = 0.015), anaplasia and P-gp (p = 0.034). CONCLUSIONS: The p53, c-erb-B2 and P-gp immunoexpression is relatively frequent in high-grade, metastic...

Anaplastic Ganglioglioma in a Middle-aged Woman: A Case Report with a Review of the Literature

We report a case of anaplastic ganglioglioma. A 45-yr-old woman was admitted with a 5-month history of headache and dizziness, both of which progressed slowly. Preoperative magnetic resonance imaging revealed a strong enhancing mass in the left frontal lobe extending to the cingulate gyrus. Adjuvant radiation therapy and chemotherapy were given after gross total resection of the tumor. Histological and immunohistochemical studies showed an anaplastic ganglioglioma. Gangliogliomas of the central nervous system are rather uncommon tumors, and anaplastic ones are extremely rare. The pertinent literature regarding gangliogliomas is reviewed.

Sclerosing Mucoepidermoid Carcinoma of the Parotid Gland: A Case Report

Sclerosing mucoepidermoid carcinoma (SMEC) is a very rare tumor of the salivary gland. There have been eight cases of SMEC reported in the medical literature; this is the first reported case in Korea. A 51-year-old woman presented with a right infra-auricular mass that she had for 3 years. We performed superficial parotidectomy. Grossly, the resected parotid gland showed a well-circumscribed firm mass. Histologically, the tumor consisted of central solid or ductal tumor nests within a dense fibrous stroma surrounded by peripheral lymphoid infiltration. The tumor nests were composed of squamous, intermediate and mucin-secreting cells. However, the tumor cells lacked mitosis and nuclear anaplasia. The lymphoid cells were mostly composed of lymphocytes and plasma cells with occasional eosinophils and neutrophils. Immunohistochemically, the tumor cells were positive for high- and low- molecular weight cytokeratins, cytokeratin 7, p16INK4A, Bcl-2 and cyclin D1. The patient also underwent radiation therapy. We report here on a case of SMEC of the parotid gland along with the immunohistochemical characteristics, and we review the relevant literature.

Long Term Follow-up of Wilms' Tumor: 20 Year Experience in a Single Pediatric Center

PURPOSE: Wilms' tumor is the most common malignant tumor of the kidney during the childhood period. This is a potentially curable disease with an excellent prognosis due to the development of the multimodal treatments, including chemotherapy, radiotherapy and nephrectomy. The aim of this study was 1) to analyze our clinical experiences with of Wilms' tumor at a single tertiary medical center, including the survival rate and recurrence of disease after treatment and 2) to investigate the prognostic factors affecting the survival of patients. METHODS: We performed retrospective analysis of the clinicopathologic data of 68 patients who underwent operation for Wilms' tumor during 20 years from March 1986 to March 2005 at Yonsei Medical Center. RESULTS: 40 (58.8%) patients were male and 28 patients (41.2%) were female. The median age at diagnosis was 25 month (range: 2 month to~10 year 10 month). Preoperative neoadjuvant chemotherapy was given in 27 patients (39.7%), and initial nephrectomy followed by chemotherapy was performed in 41 patients (60.3%). Postoperative adjuvant radiotherapy was performed in 27 patients (39.7%). The median duration of follow-up period was 8 year 2 month (range: 24 day to~18 year 6 month). The overall 5-year survival rate and 5-year disease free survival rate were 87.0% and 76.9%, respectively. Univariate analysis showed that the significant prognostic factors affecting survival were age at diagnosis (P=0.016), pathologic stage (P=0.032) and, Tumor spillage or rupture during operation (P=0.005) and tumor cell anaplasia (P=0.031). Multivariate analysis showed that tumor spillage or rupture during operation was the most significant prognostic factor (Relative Risk=4.5). CONCLUSION: Early diagnosis, meticulous surgical technique and proper pre-or postoperative adjuvant chemotherapy and radiotherapy according to the tumor histology significantly increase the patient's survival rates.

Prognóstico de pacientes com tumor de Wilms unilateral no Rio de Janeiro, 1990-2000 / Prognosis for patients with unilateral Wilms' tumor in Rio de Janeiro, Brazil, 1990-2000

OBJETIVO: Analisar a sobrevida e os principais fatores prognósticos entre os pacientes com tumor de Wilms unilateral. MÉTODOS: A coorte de estudo incluiu 132 casos de tumor de Wilms unilateral em menores de 15 anos de idade matriculados em serviço de oncologia pediátrica, de janeiro de 1990 a dezembro de 2000. Curvas de sobrevida foram confeccionadas utilizando-se o método de Kaplan-Meier e fatores prognósticos foram analisados pelo modelo de riscos proporcionais de Cox. RESULTADOS: A estimativa de sobrevida global em cinco anos foi 84,6 por cento. As probabilidades de sobrevida para os estádios I, II, III e IV foram de 100 por cento; 94,2 por cento; 83,2 por cento e 31,3 por cento, respectivamente. A taxa de sobrevida para os pacientes com: histologia favorável foi de 89,4 por cento, para aqueles com anaplasia focal 66,7 por cento e com anaplasia difusa 40 por cento. Todos os pacientes com doença em estádio IV e anaplasia difusa foram a óbito (n=4). Todos os pacientes com doença em estádio I, independente da histologia, permaneceram vivos até o final do período de seguimento. CONCLUSÕES: Entre as variáveis escolhidas para o modelo final apenas o estadiamento e a histologia permaneceram associados ao elevado risco de óbito enquanto que os casos na faixa etária entre 24 e 47 meses apresentaram melhor prognóstico que os demais. Esses resultados mostram a importância do diagnóstico em fases iniciais da doença e que a histologia é fundamental para orientar a terapia adequada.