Tumor neuroectodérmico maligno del tracto gastrointestinal: a propósito de un caso / Malignant neuroectodermal tumor of the gastrointestinal tract: case report

El tumor neuroectodérmico maligno del tracto gastrointestinal es una neoplasia rara con pocos casos reportados en la literatura, especialmente en América Latina. Descrito por primera vez en 2003, se trata de una entidad sin tratamiento estandarizado y de pobre pronóstico. Se presenta el caso de una paciente de 22 años de edad que acude a la consulta por dolor abdominal, anemia y masa abdominal palpable. Luego de estudios pertinentes se decide la conducta resectiva y el posterior tratamiento oncológico. (AU)

Malignant gastrointestinal neuroectodermal tumor (GNET), formerly known as clear cell sarcoma of the gastrointestinal tract, is an extremely rare tumor of mesenchymal origin, which presents great microscopic and molecular similarity to clear cell sarcoma found in other parts of the body, such as tendons and aponeurosis. It is characterized by its rapid evolution, high recurrence rate and frequent diagnosis as metastatic disease.1,2 (AU)

Papel da Ressonância Nuclear Magnética na Predição de Grau Histológico em Sarcomas de Partes Moles / Role of magnetic resonance imaging in the prediction of histological grade in soft tissue sarcomas

INTRODUÇÃO: Os sarcomas são tumores malignos raros e compreendem um grupo heterogêneo de tumores, com mais de 60 subtipos histológicos. A importância da classificação do grau histológico é fundamental para a definição da linha de tratamento a ser seguida. A busca por métodos que aumentem a acurácia na definição de alto grau deve ser constante. OBJETIVO: Avaliar o papel da Ressonância Nuclear Magnética na predição do grau histológico nos sarcomas de partes moles. MATERIAIS E MÉTODOS: Estudo observacional, transversal e retrospectivo. Critérios de inclusão foram pacientes com mais de 18 anos, submetidos a biópsia por agulha grossa guiada ou revisão de biópsia externa, exame de RNM e a ressecção do tumor primário no ACCCC. Exclusão de pacientes submetidos a biópsia cirúrgica. Principais variáveis foram: tamanho do tumor, idade, localização, subtipo histológico, grau histológico da biópsia e da peça cirúrgica ­ sendo utilizado classificação em alto grau (G2 e G3 da FNCLCC) e baixo grau (G1). Os critérios radiológicos da RNM (tamanho, definição das margens (regular ou irregular), heterogeneidade de sinal em T2 (maior 50% ou menor 50%), sinal de alta intensidade peritumoral em T2 (edema peritumoral presente ou ausente), realce pós-contraste peritumoral (presente ou ausente) foram avaliados por dois radiologistas da nossa instituição. Além disso, foi realizado o cálculo de um score para avaliação de predição do alto grau histológico, por meio da somatória dos critérios positivos analisados (considerando ser de 0 (zero), quando nenhum critério foi identificado a 5 (cinco) quando todos os critérios forem positivos). Utilizamos teste de associação Qui-quadrado entre os itens da RNM e o grau histológico da peça cirúrgica e, em seguida, utilizamos teste de regressão logística a fim de identificar a associação independente, de modo a avaliar o valor da RNM na predição do grau histológico. O nível de significância adotado foi de 5%. RESULTADOS: Sessenta e oito pacientes foram incluídos no estudo. Trinta mulheres e 38 homens. A biópsia identificou 54 tumores de alto grau e 14 tumores de baixo grau. Já na peça cirúrgica tivemos 52 tumores de alto grau e 16 de baixo grau. A via mais comum de biópsia foi biópsia por agulha grossa guiada por ultrassonografia com 54,4% dos casos. O subtipo mais comum foi o lipossarcoma com 17(25%) casos, seguido pelo sarcoma sinovial 17,6% e leiomiossarcoma 10,3%, assim como localização em extremidade com 64,7%. Identificamos uma maioria de tumores maiores que 5cm, correspondendo à 89,7%. A regularidade das margens 43 (63,2%) foram regular, e apenas 25 (36,8%) consideradas irregular. Já na avaliação da heterogeneidade tumoral em T2 foram 36 (52,9%) heterogêneos contra 32 (47,1%) com menos 50% de heterogeneidade. O sinal de alta intensidade peritumoral em T2 estava presente em 45 (66,2%) das vezes, ao passo que, o realce pós-contraste peritumoral estava presente em 31 (45,6%). O score ficou: dois com score 0 (zero), 10 com score 1 (um), 16 com score 2 (dois), 13 com score 3 (três), 18 com score 4 (quatro) e nove com score 5 (cinco). Os critérios da RNM que demonstraram associação com grau histológico foram sinal de alta intensidade peritumoral em T2 (p <0,001) e realce peritumoral pós-contraste (p=0,006). Na regressão logística a presença de sinal de alta intensidade peritumoral em T2 (OR 11,8) e o realce pós-contraste peritumoral (OR 8,8) também foram preditivos de tumor de alto grau. Apesar de os demais critérios da RNM não apresentarem associação com o grau histológico, ao se aplicar o score (somatório dos achados dos critérios na RNM) encontramos, que a cada ponto a mais aumentase a chance de tumor de alto grau em 2 vezes (OR 2,0; p 0,014). CONCLUSÃO: A utilização da RNM como ferramenta para predizer o grau histológico dos sarcomas de partes moles se demonstrou eficiente. Os critérios relacionados ao padrão de crescimento infiltrativo tumoral identificados na RNM como sinal de alta intensidade peritumoral em T2 e realce pós-contraste peritumoral foram positivos para predizer alto grau histológico.

INTRODUCTION: Sarcomas are rare malignant tumors and comprise a heterogeneous group of tumors, with more than 60 histological subtypes. The importance of classifying the histological grade is fundamental for defining the line of treatment to be followed. The search for methods that increase the accuracy in the definition of high degree must be constant. OBJECTIVE: To evaluate the role of Magnetic Resonance Imaging in predicting the histological grade in soft tissue sarcoma. MATERIALS AND METHODS: This is an observational, cross-sectional retrospective study. Inclusion criteria were patients over 18 years old who underwent guided core needle biopsy or external pathology review, MRI exam, and primary tumor surgery at the ACCCC from January 2015 to June 2022. Patients who underwent surgical biopsy were excluded. The main variables were tumor size, age, location, histological subtype, histological grade of the biopsy and of the surgical specimen ­ using a classification of high grade as G2 and G3 of the FNCLCC and low grade as G1. MRI radiological criteria (size, margin definition (regular or irregular), heterogeneity of the T2 signal (greater than 50% or less than 50%), high intensity peritumoral signal on T2 (peritumoral edema present or absent), post- peritumoral contrast (present or absent) were evaluated by two radiologists from our institution. In addition, a score was calculated to evaluate the prediction of histological grade, through the sum of the positive criteria analyzed (considering it to be 0 (zero), when no criterion was identified to 5 (five) when all criteria are positive). We used the Chi-square association test between the MRI items and the histological grade of the surgical specimen, and then we used the logistic regression test to identify the independent association. P values < 0.05 were considered significant. RESULTS: After applying the inclusion and exclusion criteria, sixty-eight patients were included. Thirty women and 38 men. The biopsy identified 54 highgrade tumors and 14 low-grade tumors. In the surgical specimen, we had 52 high-grade and 16 low-grade tumors. The most common type of biopsy was ultrasound-guided core needle biopsy with 54.4% of cases. Liposarcoma with 17 (25%) cases were most frequently, followed by synovial sarcoma 17.6% and leiomyosarcoma 10.3%, as well as location in the extremity with 64.7%. We identified most tumors larger than 5cm, corresponding to 89.7%. The regularity of the margins 43 (63.2%) were regular, and only 25 (36.8%) considered irregular. In the evaluation of tumor heterogeneity in T2, there were 36 (52.9%) against 32 (47.1%). Peritumoral high-intensity T2-weighted signal was present in 45 (66.2%), whereas peritumoral post-contrast enhancement was present in 31 (45.6%). The score was: two with score 0 (zero), 10 with score 1 (one), 16 with score 2 (two), 13 with score 3 (three), 18 with score 4 (four) and nine with score 5 (five). The MRI criteria that demonstrated an association with histological grade were peritumoral high-intensity T2-weighted signal (p <0.001) and peritumoral post-contrast enhancement (p=0.006). In logistic regression, the presence of peritumoral high-intensity T2weighted signal (OR 11.8) and peritumoral post-contrast enhancement (OR 8.8) were also predictive of high-risk tumor. Although the other MRI criteria are not associated with the histological grade, when applying the score (sum of the MRI findings of the criteria), we found that each additional point increases the chance of a high-grade tumor by 2 times (OR 2.0; p 0.014). CONCLUSION: The use of MRI as a tool to predict the histological grade of soft tissue sarcomas proved to be efficient. The criteria related to the infiltrative tumor growth pattern identified on MRI as peritumoral high-intensity T2-weighted signal and peritumoral post-contrast enhancement were positive for predicting histological high grade.

Factores hereditarios y síndromes de predisposición genética relacionados con sarcomas de partes blandas / Hereditary factors and syndromes of genetic predisposition related to sarcomas of soft parts

Los sarcomas de partes blandas son tumores malignos que se originan en el tejido conectivo, a partir del mesénquima embrionario. Teniendo en cuenta la existencia de nuevos y constantes cambios en la naturaleza de estos tumores, se realizó una revisión de las publicaciones más recientes para profundizar en las alteraciones genéticas, los síndromes de predisposición y su relación con los sarcomas. Se pudo concluir que aún siguen siendo muy pocos los registros que describen la identificación de dichos síndromes como principal eslabón en el desarrollo de los sarcomas.

The sarcomas of soft parts are malignancies that originate in the connective tissue, starting from the embryonic mesenchyme. Taking into account the existence of new and constant changes in the nature of these tumors, a review of the most recent publications was carried out to deepen in the genetic disorders, the predisposing syndromes and its relationship with sarcomas. It was concluded that the records that describe the identification of these syndromes are still very few as main link in the development of sarcomas.

Sarcoma de Células Claras en pierna de mujer de 28 años / Clear cell sarcoma on 28 years old woman leg

Los tumores de partes blandas son tumores que se presentan en adultos, aproximadamente a los 60 años. De estos tumores los sarcomas son poco frecuentes y tienen preferencia en las extremidades y muchas veces invaden estructuras músculo aponeuróticas cercanas, y poca invasión vascular o nerviosa lo que las hace de fácil acceso con poca morbilidad para su biopsia. El diagnóstico de los tumores de partes blandas se basa en el estudio histológico de anatomía patológica y, a veces se debe asociar a estudios de citogenética para poder tener un diagnóstico específico. El tratamiento principal es quirúrgico y puede llegar estar asociado a terapia adyuvante.

Soft tissue tumors are tumors that occur in adult's age, at about 60 age. Of these tumors, sarcomas are rare and have preference in the extremities, and often invade nearby muscle-fascia structures, and little vascular or nerve invasion, making them easily accessible with little morbidity for biopsy. The diagnosis of soft tissue tumors is based on the histological study of pathological anatomy and, sometimes, it must be associated with cytogenetic studies in order to have specific diagnosis. The main treatment is surgical and may end up being associated with adjuvant therapy.

Sarcoma neurogénico de la mama masculina. Presentación de un caso / Male breast neurogenic sarcoma. A case

RESUMEN El cáncer de mama en el sexo masculino es una entidad clínica poco frecuente, tiene una presentación unimodal a los 71 años de edad, generalmente se presenta de manera similar a la forma en que se presenta en el sexo femenino. Su causa es poco conocida. Los sarcomas son tumores de componentes mesenquimatoso que constituyen del 0,2-1 % de todos los tumores de mama, y menos del 5 % del total. El sarcoma neurogénico, a su vez, es un tumor extremadamente raro. Representa del 1-2 % aproximadamente, de los tumores de los nervios periféricos con transformación maligna. Debido a la rareza geográfica e histopatológica de este tipo y mucho más en pacientes masculinos se presentó este caso. Paciente masculino de 57 años de edad, con el diagnóstico de un sarcoma de la mama derecha. Se le realizó una mastectomía radical más quimioterapia y radioterapia adyuvante. Los estudios de inmunohistoquímicos permitieron llegar al diagnóstico de sarcoma neurogénico.

ABSTRACT Breast cancer in men (BCM) is a rare clinical entity that has a unimodal presentation at the age of 71 years, and generally presents in a similar way it presents in the female sex. Its etiology remains almost unknown. Sarcomas are tumors of mesenchymal components representing from 0.2 to 1 % of all the breast tumors and less than 5 % of the total. The neurogenic sarcoma is also an extremely rare tumor. It represents around 1-2 % of the peripheral nerves tumors with malignant transformations. Due to location and histopathological rarity of this kind of tumors, much more in male patients, the authors presented the case of a male patient, aged 57 years, with the diagnosis of a left breast sarcoma. He undergone a radical mastectomy plus adjuvant chemotherapy and radiotherapy. The immunohystochemical studies allowed arriving to the diagnosis of neurogenic sarcoma.

Melanoma del canal anal: a propósito de un caso / Melanoma of the anal canal: a propos of a case

RESUMEN Los melanomas mucosos son tumores poco frecuentes y de mal pronóstico. Presentan un comportamiento agresivo, y pueden tener varias localizaciones en el aparato digestivo. Este tipo de tumores es más frecuente en la región anorectal. Se presentó un caso de una paciente femenina de 59 años, con un cuadro de varios meses de evolución; caracterizado por ardor, prurito anal y sensación de masa que prolapsaba a través del ano. Se constató al examen físico masa tumoral aspecto polipoideo. Se realizó polipectomía donde la biopsia arrojó como resultados el aspecto histológico de un melanoma mucoso de canal anal.

ABSTRACT Mucous melanomas are few frequent and have a bad prognosis. They present an aggressive behavior and might have several locations in the digestive system. This kind of tumors is more frequent at the anus-rectal region. The authors presented the case of a female patient, aged 59 years, with clinical conditions of several months of evolution, characterized by burning, anal pruritus and the sensation of a mass prolapsing through the anus. A tumor mass of polypoid aspect was found at physical examination. Polypectomy was carried out and the biopsy showed the histologic aspect of a mucous melanoma of the anal canal.

Sarcoma de células claras del pie. Presentación de un caso / Clear cell sarcoma of the foot. Case presentation

RESUMEN El sarcoma de células claras fue descrito por primera vez por Franz M. Enzinger en 1965. Está íntimamente asociado a tendones y aponeurosis, excepcionalmente compromete la epidermis. Afecta fundamentalmente a pacientes jóvenes y se caracteriza por múltiples recurrencias locales y metástasis tardías. Se presenta un paciente de 22 años de edad, masculino que fue sometido a tratamiento quirúrgico radical (amputación transmetatarseana del 1er y 2do rayo). Los estudios anatomopatológicos confirmaron el diagnóstico de un sarcoma de células claras. El paciente se encuentra libre de la enfermedad después de 6 años de operado e incorporado a su vida social (AU).

ABSTRACT The clear cell sarcoma was firstly described by Franz M. Enzinger in 1965. It is intimately associated to tendons and aponeurosis, exceptionally compromising the epidermis. It mainly affects young patients and is characterized by multiple local recurrences and late metastases. We present a male patient, aged 22 years, who underwent a radical surgical treatment (transmetatarsal amputation of the 1st and 2nd rays). The anatomic-pathological studies confirmed the diagnosis of clear cell sarcoma. 6 years after surgery, the patients is free of the disease and reincorporated to his social life (AU).

Renal Tumors in Children / 임상소아혈액종양

Pediatric renal tumors represent a diverse group, which include Wilms' tumor (WT), renal cell carcinoma (RCC), clear cell sarcoma of the kidney (CCSK), congenital mesoblastic nephroma, malignant rhabdoid tumor of the kidney (MRTK) and primitive neuroectodermal tumor. WT (85%) and RCC (8%) are the most prevalent types. WT predominates among the 1- to 10-year age group, but RCC exceeds WT in children over age 10 years. Pediatric renal tumors are genetically, histologically and clinically heterogeneous. The overall survival for children with localized WT is currently more than 90%, whereas poorer survival rates are observed for anaplastic WT, metastatic WT, metastatic CCSK, MRTK, metastatic RCC and relapsed WT. Therefore risk-stratified treatment is important to minimize treatment morbidity while preserving survival. This review focuses on distinct characteristics of each tumor type and optimal stratified treatment.

Clinical Features and Treatment Outcome of Soft Tissue Clear Cell Sarcoma / 임상소아혈액종양

BACKGROUND: Soft tissue clear cell sarcoma is a rare tumor which originates from neural crest cells. Due to its rarity and lack of established treatment, the prognosis of clear cell sarcoma is poor. Here, we reviewed the clinical data and outcome of patients diagnosed with soft tissue clear cell sarcoma in our institution. METHODS: A retrospective study was conducted on pediatric patients who were treated for pathologically confirmed soft tissue clear cell sarcoma at the Seoul National University Hospital, between January 2000 and July 2017. RESULTS: Six patients (3 boys and 3 girls) were diagnosed with soft tissue clear cell sarcoma at a median age of 14 years 4 months (range 11 years 7 months - 19 years 3 months). The median size of the tumor was 5.6 cm (range, 0.6 cm to 7.9 cm). The most frequent symptom was pain (67%), and the most common primary site was the lower limb (67%). Three patients (50%) presented with metastases at diagnosis. Four patients underwent chemotherapy with various therapeutic combinations. Four patients received surgical resection. Only one patient received local radiotherapy. One patient died of primary refractory disease, three patients relapsed, while the remaining two survive event-free. CONCLUSION: Soft tissue clear cell sarcoma is a rare and highly aggressive tumor, for which there is no established treatment. All surviving patients received surgery, indicating that surgery is a key treatment modality. Further genetic studies of soft tissue clear cell sarcoma are needed to find a better treatment strategy.

Clinical Features and Treatment Outcome of Soft Tissue Clear Cell Sarcoma / 임상소아혈액종양

BACKGROUND: Soft tissue clear cell sarcoma is a rare tumor which originates from neural crest cells. Due to its rarity and lack of established treatment, the prognosis of clear cell sarcoma is poor. Here, we reviewed the clinical data and outcome of patients diagnosed with soft tissue clear cell sarcoma in our institution.METHODS: A retrospective study was conducted on pediatric patients who were treated for pathologically confirmed soft tissue clear cell sarcoma at the Seoul National University Hospital, between January 2000 and July 2017.RESULTS: Six patients (3 boys and 3 girls) were diagnosed with soft tissue clear cell sarcoma at a median age of 14 years 4 months (range 11 years 7 months - 19 years 3 months). The median size of the tumor was 5.6 cm (range, 0.6 cm to 7.9 cm). The most frequent symptom was pain (67%), and the most common primary site was the lower limb (67%). Three patients (50%) presented with metastases at diagnosis. Four patients underwent chemotherapy with various therapeutic combinations. Four patients received surgical resection. Only one patient received local radiotherapy. One patient died of primary refractory disease, three patients relapsed, while the remaining two survive event-free.CONCLUSION: Soft tissue clear cell sarcoma is a rare and highly aggressive tumor, for which there is no established treatment. All surviving patients received surgery, indicating that surgery is a key treatment modality. Further genetic studies of soft tissue clear cell sarcoma are needed to find a better treatment strategy.

Renal Tumors in Children / 임상소아혈액종양

Pediatric renal tumors represent a diverse group, which include Wilms' tumor (WT), renal cell carcinoma (RCC), clear cell sarcoma of the kidney (CCSK), congenital mesoblastic nephroma, malignant rhabdoid tumor of the kidney (MRTK) and primitive neuroectodermal tumor. WT (85%) and RCC (8%) are the most prevalent types. WT predominates among the 1- to 10-year age group, but RCC exceeds WT in children over age 10 years. Pediatric renal tumors are genetically, histologically and clinically heterogeneous. The overall survival for children with localized WT is currently more than 90%, whereas poorer survival rates are observed for anaplastic WT, metastatic WT, metastatic CCSK, MRTK, metastatic RCC and relapsed WT. Therefore risk-stratified treatment is important to minimize treatment morbidity while preserving survival. This review focuses on distinct characteristics of each tumor type and optimal stratified treatment.

Frecuencia de staphylococcus aureus meticilino resistente y del factor de virulencia PVL en pacientes ambulatorios con infección de piel y partes blandas de Asunción, Paraguay / Frequency of methicillin-resistant staphylococcus aureusand PVL virulence factor in ambulatory patients with skinand soft tissue infections of Asuncion, Paraguay

Staphylococcus aureus (S. aureus) es actualmente el agente etiológico más frecuente en infecciones de piel y tejidos blandos. El S. aureus meticilino resistente (SAMR) aislado en infecciones de pacientes de la comunidad ha ido aumentando, constituyéndose en un problema de salud pública a nivel mundial. En Paraguay existen pocos registros sobre la meticilino resistencia y factores de virulencia a nivel comunitario, por lo que se realizó este estudio observacional descriptivo para determinar la frecuencia de SAMR y del factor de virulencia leucocidina de Panton Valentine (PVL-Panton Valentine leukocidin), así como el perfil de resistencia antimicrobiana acompañante a la meticilino resistencia en S. aureus aisladosde infecciones de piel y partes blandas de pacientes ambulatorios de dos laboratorios de Asunción, Paraguay, entre octubre de 2012 a febrero de 2014. La identificación bacteriana se realizó mediante técnicas microbiológicas convencionales y la susceptibilidad antimicrobiana por la prueba de difusión en disco. Elgen mecA y luk-PV fueron detectados por la técnica de PCR. De los 70 aislados de S. aureus estudiados, el 54,3% (38/70) fue SAMR tanto por método fenotípico como molecular. La frecuenciade PVL fue de 15,7% (11/70), siendo mayor en los SAMR (21%; 8/38) que en los SAMS (9,4%; 3/32). El 2,6% de los SAMR presentó resistencia a ciprofloxacina, no se observó multiresistencia en ningún aislado. Se encontró alta frecuencia de SAMR comparado con reportes previos en Paraguay. Se requiere fortalecer estrategias de vigilancia, prevención y control de la resistencia bacteriana en ambientes hospitalarios y de la comunidad.

Staphylococcus aureus (S. aureus) is currently the most common etiologic agentof skin and soft tissue infections. The isolation of methicillin-resistant S. aureus(MRSA) from infections of patients in the community has increased, becoming a public health problem worldwide. In Paraguay, there are few records aboutmethicillin resistance and virulence factors at community level. Therefore, this descriptive observational study was performed to determine the frequency of MRSA and factor virulence of Panton-Valentine leukocidin (PVL) as well as the antimicrobial resistance profile accompanying methicillin resistance in S. aureusisolated from skin and soft tissue infections in ambulatory patients from two laboratories of Asuncion, Paraguay from October, 2012 to February, 2014. The bacterial identification was performed using conventional microbiological techniques and the antimicrobial susceptibility was determined by disk diffusion. The mecA andluk-PV genes were detected by PCR technique. Out of the 70 S. aureus isolates studied, 54.3% (38/70) was SAMR by phenotypic and molecular methods. PVL frequency was 15.7% (11/70) being higher in MRSA (21%; 8/38) than in the SAMS (9.4%; 3/32), 2.6% of the MRSA was resistant to ciprofloxacin and multidrug resistance was not observed in any isolates. A high frequency of MRSA was found compared with previous reports in Paraguay. It is required to strengthen surveillance, prevention and control of bacterial resistance strategies in hospital and community environments.

Clear Cell Sarcoma of the Wrist: MRI Findings with Diffusion-Weighted Image and Histopathologic Correlation

Clear cell sarcoma is rare and difficult to diagnose. Herein, we present a case of clear cell sarcoma in the dorsum of the wrist with MRI findings, including diffusion-weighted imaging, and histopathologic correlation, which was initially diagnosed as giant cell tumor of tendon sheath.

A Case of Clear-Cell Sarcoma in the Stomach / 대한내과학회지

Clear-cell sarcoma (CCS) is a rare soft tissue sarcoma that usually develops in the lower extremities of young adults. CCS of the gastrointestinal tract is extremely rare. We report here the first case of CCS of the stomach in Korea. A 28-year-old female developed a large tumor of the stomach that was initially considered Ewing sarcoma, based on the results of a needle biopsy. She had paraneoplastic syndrome; fever, anemia, and hyperglycemia. Follow-up abdominal computed tomography after four-cycles of chemotherapy with vincristine-doxorubicin-cyclophosphamide or ifosfamide-etoposide showed no tumor shrinkage and revealed a fistula between the tumor and the gastric lumen. The infected tumor prompted debulking surgery, which resulted in improvement of her symptoms. The pathologic findings were consistent with CCS, and fluorescence in situ hybridization for EWS gene rearrangement was positive, confirming the diagnosis. This case highlights the importance of clinical suspicion for CCS in chemotherapy-refractory Ewing sarcoma.

Tumor de partes blandas de bajo potencial maligno: caso clínico y revisión de la literatura / Soft tissue tumor of low potential malignancy: clinic case and review of literature

El tumor de células gigantes de partes blandas de bajo potencial de maligno, es una neoplasia poco frecuente, clasifica dentro de las lesiones fibrohistiocíticas. Histológicamente es un tumor con hallazgos idénticos al tumor de células gigantes del hueso. Presentamos un caso correspondiente a esta neoplasia. Se trató de paciente de 26 años con una lesión tumoral 20 cm x15 cm x15 cm, en ambas regiones lumbares y región sacra. El estudio histológico reveló una neoplasia con abundantes células gigantes, células fusiformes, hemosiderina y hueso metaplásico. Los estudios inmunohistoquímicos demostraron fuerte positividad de CD68 para las células osteoclásticas. La evolución del paciente fue favorable, sin evidencia de recidivas. Es imprescindible realizar el diagnóstico diferencial de este tumor con otras neoplasias con abundantes células gigantes, como el tumor de células gigantes de la vaina tendinosa y el fibrohistiocitoma maligno rico en células gigantes, el cual es un sarcoma de alto grado

The giant cell tumor of soft tissues of low potential malignancy is a very rare tumor. It’s classified in fibrohistiocytic neoplasm and has features identical to giant cell tumor of bone. We present a clinical case for this less frequent malignancy. Patient 26 years old man with a tumor of 20 cm x 15 cm x 15 cm, in both lumbar and the sacral regions. Histological examination revealed a neoplasm with abundant giant cells, spindle cells, hemosiderin and metaplastic bone. The immunohistochemistry studies practice showed strong positivity of CD68 for the osteoclastic cells. The patient outcome was favorable, without evidence of recurrence. It is essential to make the differential diagnosis of this kind of tumor with other neoplasm with abundant giant cells, such as the giant cell tumor of the tendon sheath and malignant giant cell, also malignant fibrous hystiocitoma, which is rich in giant cells and high grade sarcoma

Fibrohistiocitoma maligno en pared abdominal: reporte de un caso / Malignant fibrohystiocytoma of the abdominal wall: a case report

El fibrohistiocitoma maligno es el sarcoma de partes blandas más común en la edad adulta, en un grupo etario mayor de 50 años y afecta principalmente a hombres. Estos tumores mesenquimales, con sus distintas variedades morfológicas, precisan de un análisis inmunohistoquímico para su diagnóstico diferencial, fundamentalmente con el carcinoma sarcomatoide, el tumor miofibroblástico inflamatorio y el leiomiosarcoma. Macroscópicamente son neoplasias voluminosas, con múltiples áreas de necrosis en la superficie de corte; desde el punto de vista microscópico el tumor muestra una proliferación desordenada de células fusocelulares, con un patrón esteliforme y/o presencia de células multinucleadas con atipia, mitosis atípicas y un estroma que muestra gran cantidad de colágeno así como un número variable de células inflamatorias mononucleadas e histiocitos espumosos. Su diagnóstico es realizado en base a su morfología e inmunohistoquímica. Presentamos el caso de un paciente masculino de 54 años y la forma como se manejó en nuestro departamento.

The malignant fibrohystiocytoma is the most frequent soft tissue sarcoma in the adult age, in an age group mayor of 50 years old, and affect principal to the man. These mesenquimal tumors with distinct morphological varieties need an immunohistochemistry analysis for his differential diagnostic, principal with the sarcomatoide carcinoma, the miofibroblastic inflammatory tumor and the leiomiosarcomas. Macroscopically there are bulky neoplasm with multiples necrosis areas in the superficial incision, of the microscopically point of view, the tumor show a no order proliferation of the fusocellular cells, with a esteliform patron and or the presence of multinucleate cells with atypical mitosis and a stroma, with show a big quantities of collagen and a variety numbers of inflammatory cells mononucleotic and spumoni hystiocyts. The diagnostic is realized in base to his morphologic and the immunohistochemistry. We present the case of a male patient 54 years old and the form of his management.

Renal tumors presentation: Changing trends over two decades.

Purpose: We have analyzed the changing trends in surgical treatment of renal tumors over the last 2 decades with regard to age incidence, presentation, incidental detection, and histopathology. Materials and Methods: Records of renal tumors were analyzed from January 1, 1988 to December 31, 2007. Data were split into 4 parts based on a 5-year time period, 1 for each cohort of patients: cohort 1 (1988-1992)-103 patients, cohort 2 (1993-1997)-161 patients, cohort 3 (1998-2002)-243 patients, and cohort 4 (2003-2007)-304 patients. A comparative study was performed with regard to age incidence, presentation, incidentallomas, histopathology, and management with statistical analysis. Results: Out of 811 renal tumors, 17.63% cases were benign and 82.37% were malignant. In the first cohort, 34.95% cases were detected in the seventh decade as compared with cohort 4 in which these were detected in the sixth (34.86%) and fifth decades (21.38%). Incidentallomas increased from 11 (10.67%) in cohort 1 to 84 (27.63%) in cohort 4 (P = 0.001). The cases of surgically treated tumors increased in number from 103 to 304 in cohort 4. Among the presenting features, incidence of weight loss, flank pain, and lump decreased while other clinical syndromes were constant. Only open radical nephrectomy was performed in the first 2 consecutive timeperiods. Laparoscopic radical nephrectomy was increasingly used in cohort 4 as compared with cohort 3 (121 vs 32, respectively). Similarly, open nephron sparing surgery (NSS) was increasingly used in cohort 4 as compared with cohort 3. Among the histopathologies, clear cell carcinoma was most common (73.35 %), but Fuhrman grading showed a trend toward more cases detected with grade 1 and 2 in cohort 4; 23.73% and 61.86%, respectively, as compared with 15.85% and 45.12% in cohort 1 (P = 0.001); more T1 tumors were detected (63.42% in cohort 4 as compared with 41.46% in cohort 1). Conclusions: A majority of renal tumors presented as symptomatic tumors. Recently, tumors are being detected at an early stage and grade; in the younger patients, with an increasing trend of laparoscopic and open NSS.