Sarcoma de Ewing multifocal: presentación de un caso pediátrico / Multifocal Ewing sarcoma: presentation of a pediatric case / Sarcoma de Ewing Multifocal: apresentação de um caso pediátrico

Introducción: el Sarcoma de Ewing es una neoplasia maligna de origen mesenquimático. Al momento del diagnóstico el 75% se presentan en forma localizada. Objetivo: comunicar un caso que por su presentación multifocal, generó dificultades diagnósticas. Caso clínico: niña de 6 años. Consulta por traumatismo de mano derecha tras caída de su altura 24 horas previas, constatándose en mano y puño derecho edema, calor y eritema, movilidad conservada. No fiebre. Radiografía: aumento del diámetro del tercer metacarpiano, imagen esmerilada, no trazos de fracturas. Ingresa con planteo de celulitis. Anemia leve microcítica, hipocrómica. Proteína C reactiva 82 mg/l. Recibe clindamicina intravenosa 72 horas, completa 14 días vía oral. Persistencia de alteraciones en puño y mano derecha, agrega tumoración de raíz nasal con desviación del eje, indolora. Fosfatasa alcalina, lactato deshidrogenasa, fosfatemia, calcemia normales. Resonancia magnética: alteración morfoestructural de radio, olecranon y tercer metacarpiano, fractura de olecranon y radio, reacción perióstica. Pet-Scan: lesión extensa ósea en macizo facial, tibias, cúbitos, humero derecho y clavícula. Biopsia 3er metacarpiano: tumor de células pequeñas, redondas azules, CD99 y vimentina positivo. Comienza poliquimioterapia y radioterapia sin complicaciones. Conclusiones: es frecuente que las manifestaciones clínicas iniciales sean confundidas con entidades más frecuentes, como post-traumáticas y/o inflamatorias, tal como ocurrió en este caso. Posteriormente, la aparición de nuevas lesiones y compromiso del estado general orientó el abordaje diagnóstico de la patología tumoral. La confirmación exige el estudio anatomopatológico con estudio inmunohistoquímico. La presencia de metástasis óseas constituye un factor de mal pronóstico y dificulta el abordaje terapéutico.

Introduction: Ewing's sarcoma is a malignant neoplasm of mesenchymal origin. At the time of diagnosis 75% of the cases are localized. Objective: to report a case that, due to its multifocal presentation, generated diagnostic difficulties. Clinical case: 6-year-old girl. She consulted for right hand trauma after a fall from her height 24 hours earlier, with edema, warmth and erythema in the right hand and fist, with preserved mobility. No fever. X-ray: increase in the diameter of the 3rd metacarpal, frosted image, no traces of fractures. Admitted with cellulitis. Mild microcytic anemia, hypochromic. C-reactive protein 82mg/l. Receives intravenous clindamycin 72 hours, completes 14 days orally. Persistence of alterations in fist and right hand, adds tumor of nasal root with deviation of the axis, painless. Alkaline phosphatase, lactate dehydrogenase, phosphatemia, normal calcemia. MRI: morphostructural alteration of radius, olecranon and 3rd metacarpal, fracture of olecranon and radius, periosteal reaction. Pet-Scan: extensive bone lesion in facial mass, tibiae, ulnae, right humerus and clavicle. Biopsy 3rd metacarpal: small cell tumor, blue round, CD 99 and vimentin positive. Polychemotherapy and radiotherapy were started without complications. Conclusions: it is frequent that the initial clinical manifestations are confused with more frequent entities, such as post-traumatic and/or inflammatory, as occurred in this case. Subsequently, the appearance of new lesions and compromise of the general condition guided the diagnostic approach of the tumor pathology. Confirmation requires anatomopathological study with immunohistochemical study. The presence of bone metastases constitutes a poor prognostic factor and hinders the therapeutic approach.

Introdução: O sarcoma de Ewing é um neoplasma maligno de origem mesenquimatosa. No momento do diagnóstico, 75% dos casos são localizados. Objetivo: Relatar um caso que, devido a sua apresentação multifocal, causou dificuldades diagnósticas. Caso clínico: Menina de 6 anos. Ela consultou por traumatismo à mão direita após cair de sua altura 24 horas antes, com edema, calor e eritema na mão direita e punho, com mobilidade preservada. Sem febre. Raio-X: aumento do diâmetro do 3º metacarpo, imagem fosca, sem vestígios de fraturas. Admitido com a sugestão de celulite. Anemia microcítica leve, hipocrómica. Proteína C reativa 82mg/l. Recebe clindamicina intravenosa por 72 horas, completa 14 dias por via oral. Persistência de alterações no punho e mão direita, tumor indolor da raiz nasal com desvio do eixo. Fosfatase alcalina, desidrogenase láctica, fosfataemia, calcemia normal. IRM: alteração morfo-estrutural do rádio, olecrânio e 3º metacarpo, fratura do olecrânio e do rádio, reação periosteal. Pet-Scan: extensa lesão óssea na massa facial, tíbia, ulnae, úmero direito e clavícula. Biópsia do 3º metacarpo: tumor de pequenas células, redondo azul, CD 99 e vimentina positiva. Ela iniciou a poli-quimioterapia e radioterapia sem complicações. Conclusões: É comum que as manifestações clínicas iniciais sejam confundidas com entidades mais freqüentes, tais como pós-traumáticas e/ou inflamatórias, como ocorreu neste caso. Posteriormente, o aparecimento de novas lesões e o envolvimento do quadro geral levaram a uma abordagem diagnóstica da patologia tumoral. A confirmação requer um estudo anatomopatológico com estudo imuno-histoquímico. A presença de metástases ósseas é um fator de mau prognóstico e dificulta a abordagem terapêutica.

Respuesta patológica completa al tratamiento multimodal en sarcoma de Ewing del adulto / Pathological complete response after multimodal treatment of adult Ewing sarcoma

Resumen El sarcoma de Ewing es una neoplasia rara y altamente agresiva que afecta con cierta predilección adolescentes varones. La incorporación de terapia neoadyuvante y nuevas técnicas quirúrgicas ha mejorado la supervivencia. Presentamos el caso de un varón de 41 años con sarcoma de Ewing de pared torácica, quien recibió tratamiento multimodal consistente en quimio-radioterapia concurrente y tratamiento qui rúrgico, y alcanzó respuesta patológica completa. El sarcoma de Ewing rara vez se presenta en la edad adulta y, cuando lo hace, suele tener mal pronóstico. El tratamiento multimodal de pacientes mayores de 40 años ha probado mejorar los resultados oncológicos.

Abstract Ewing sarcoma is a rare and highly aggressive neoplasm that occurs most frequently in male adolescents. The incorporation of neoadjuvant therapy and new surgical techniques has improved survival. We present the case of a 41-year-old man diagnosed with Ewing sarcoma of the chest wall, whose tumor showed a pathological complete response to a multimodal treatment consisting of concurrent chemotherapy, radiotherapy, and surgical resection. Ewing sarcoma rarely occurs in adults, who generally have a worse prognosis. A multimodal approach for the treatment of patients older than 40 years has proven to improve oncological results.

Actualización en el uso del PET/CT en sarcoma de Ewing, a propósito de un caso

The use of positron emission tomography with computed tomography (PET/CT) for adult cancer patients is widespread, however, its use in pediatric patients is limited by fear of radiation, monetary cost and lack of awareness of its greater sensitivity in the evaluation of some types of tumors. Ewing's sarcoma is one of the primary pediatric malignancies in which PET/CT with 18F-labeled fluorodeoxyglucose (18F-FDG) has demonstrated greater sensitivity in the evaluation of bone metastases compared to scintigraphy, as well as in the evaluation of treatment response. We report a 13 years old female consulting for retrosternal pain. A chest CT scan showed an infiltrating mass originating in the sternum. A biopsy confirmed the presence of an Ewing sarcoma. The tumor was staged with PET/CT which showed multiple bone lesions not visible in previous studies.

Manual and semiautomatic segmentation of bone sarcomas on MRI have high similarity

The aims of this study were to evaluate the intra- and interobserver reproducibility of manual segmentation of bone sarcomas in magnetic resonance imaging (MRI) studies and to compare manual and semiautomatic segmentation methods. This retrospective study included twelve osteosarcoma and eight Ewing sarcoma MRI studies performed prior to any therapeutic intervention. All cases were histopathologically confirmed. Three radiologists used 3D-Slicer software to perform manual segmentation of bone sarcomas in a blinded and independent manner. One radiologist segmented manually and also performed semiautomatic segmentation with the GrowCut tool. Segmentation exercises were timed for comparison. The dice similarity coefficient (DSC) and Hausdorff distance (HD) were used to evaluate similarity between the segmentation results and further statistical analyses were performed to compare DSC, HD, and volumetric results. Manual segmentation was reproducible with intraobserver DSC varying from 0.83 to 0.97 and HD from 3.37 to 28.73 mm. Interobserver DSC of manual segmentation showed variation from 0.73 to 0.97 and HD from 3.93 to 33.40 mm. Semiautomatic segmentation compared to manual segmentation resulted in DSCs of 0.71−0.96 and HDs of 5.38−31.54 mm. Semiautomatic segmentation required significantly less time compared to manual segmentation (P value ≤0.05). Among all situations compared, tumor volumetry did not show significant statistical differences (P value >0.05). We found excellent intra- and interobserver agreement for manual segmentation of osteosarcoma and Ewing sarcoma. There was high similarity between manual and semiautomatic segmentation, with a significant reduction of segmentation time using the semiautomatic method.

Sarcoma de Ewing congênito: relato de um caso / Congenital Ewing's sarcoma: a case report

O sarcoma congênito da família Ewing é uma doença extremamente rara, que se manifesta através do surgimento de uma massa e sinais sistêmicos como dor e febre. Cerca de 70 a 80% dos casos ocorrem antes dos 20 anos de idade. Histologicamente se caracteriza pela presença de pequenas células arredondadas e azuis. Além disso, pode se manifestar em tecidos moles ou osso e em qualquer parte do corpo, sendo a forma congênita mais comumente encontrada nas extremidades. O diagnóstico é estabelecido através de exames de imagem, histopatologia e imuno-histoquímica. Esses tumores costumam ser agressivos e apresentar elevada mortalidade. Aqui, relatamos o caso de um recém-nascido que apresentou uma massa no membro superior esquerdo diagnosticada como sarcoma congênito da família Ewing por biópsia cutânea por punch e pela presença do marcador CD99 (AU)

Congenital Ewing's sarcoma is an extremely rare disease, manifested by the appearance of a mass and systemic signs such as pain and fever. About 70-80% of cases occur before 20 years of age. This condition is histologically characterized by the presence of small round blue cells and can manifest in bone or soft tissue, anywhere in the body, with the congenital form being the most commonly found in the extremities. The diagnosis is established by imaging, histopathology, and immunohistochemistry. These tumors tend to be aggressive and have high mortality rates. Here, we report the case of a newborn who presented a mass in the left upper limb diagnosed as congenital Ewing's sarcoma by punch skin biopsy and the presence of the CD99 marker (AU)

Ewing’s Sarcoma of Zygomatic Bone.

Ewing’s sarcoma is an uncommon malignancy usually arising from the skeletal system particularly pelvic bones and long bones of lower extremities and mostly affecting children and young adults. Its occurrence in the maxillofacial region with soft tissue involvement is struck with rarity. It carries a poor prognosis due to its uncontrolled potential for metastatic spread; therefore an early and integrated treatment approach must be adopted to improve patient’s long term survival.

Cytological Diagnosis of Ewing Sarcoma – Clavicle.

Ewing sarcoma is a rare malignant round cell tumor of the bone. It is the second most common primary malignant tumor of bone found in children. It commonly affects long bones, pelvis and ribs. An 11 year old boy presented with painful swelling at the medial end of left clavicle. X-ray revealed a diaphyseal lesion of the medial end of the left clavicle with destruction of bone and mottled appearance. FNAC was done and reported as Malignant round cell tumor suggestive of Ewing sarcoma. Histopathologically the diagnosis of Ewing sarcoma was confirmed with special stains. Ewing sarcoma affecting clavicle is uncommon. Clavicle can be often resected with no need of reconstruction in young children.

many faces of Ewing sarcoma: difficult to diagnose pediatric cases

Ewing sarcoma [ES] is the second most frequent primary malignant bone cancer, following osteosarcoma. ES is a small round-cell tumor typically arising in the bones, rarely in soft tissues, of children and adolescents. We describe four children aged 3, 3.5, 9, and 9.5 years, who presented with two femur masses simultaneously [patient 1], a huge mediastinal mass [patient 2], an abdomino-mediastinal mass with dysphagia [patient 3], and a huge abdomino-pelvic mass [patient 4]. Our patients were of younger age and had abnormal presentations that made initial diagnosis difficult, but also are representative of the different problems encountered in pediatric practice. Biopsy initially revealed round cell tumor and by immunohistochemistry, CD99 was positive, which confirmed the diagnosis of ES. Our patients were difficult to diagnosis. The patients were misdiagnosed initially, so there was a delay in diagnosis. Definitive diagnosis required use of various radiological imaging methods and immunohistochemistry

Endoprosthetic reconstruction for malignant bone and soft-tissue tumors.

BACKGROUND: Nowadays, the results of the management of malignant bone and soft-tissue tumors have been dramatically improved because of the advance in imaging, chemotherapy, radiation therapy, and surgical techniques. Patients can have longer survival times with limb-salvage surgery. Several techniques of reconstruction have been advocated and gained more popularity following malignant tumor resection by using allograft, tumor prostheses, composite allograft prosthesis, or arthrodesis. OBJECTIVE: To report the preliminary results of 32 endoprosthetic reconstructions following malignant bone and soft-tissue tumor resection. The oncologic results, functional outcomes, and complications from the surgery were assessed in the present study. MATERIAL AND METHOD: Since September 1988, the authors have performed 188 limb-salvage surgical operations for the treatment of musculoskeletal tumors at Siriraj Hospital. From March 1994 to July 2006, 32 endoprosthetic reconstructions were performed on 30 patients following malignant bone or soft-tissue tumor removal. There were 16 males and 14 females with a mean age of 28 years (range 10-73). The diagnosis was conventional osteosarcoma in 16 patients, parosteal osteosarcoma in two patients, chondrosarcoma in two patients, leiomyosarcoma in two patients, failed allograft in two patients and one patient each of periosteal osteosarcoma, Ewing's sarcoma, Gorham's disease, synovial sarcoma, malignant fibrous histiocytoma, metastatic renal cell carcinoma, and prosthetic loosening. Wide excision was performed with a mean length of 18.5 cm (range 10-41). Five proximal femurs, 17 distal femurs, 1 total femur 3 proximal tibias, 1 intercalary tibia, 4 proximal humerus and 1 distal humerus were used for reconstruction. Modular replacement systems (MRS, Stryker/Howmedica/Osteonics) were the most common prostheses used in the present series. RESULTS: The mean follow-up time was 26 months (range 6-128.7). Sixteen patients are continuously free of the disease, two are alive with the disease, two had no evidence of the disease, nine died of the disease, and one patient died from complication of hypertension. The mean Musculoskeletal Tumor Society functional analysis for upper extremity reconstruction was 93% (range 86.7-100) and for lower extremity was 89% (range 63.3-100). Two patients (6.7%) were determined to be a failure. Revision due to aseptic loosening was performed in one patient (3.3%) and one hip disarticulation was done related to local recurrence (3.3%). One patient with sciatic nerve palsy and two seromas was found and successfully treated in the present study. CONCLUSION: Endoprosthetic reconstruction could yield satisfactory results as a wide excision and limb-salvage for patients with malignant bone and soft-tissue tumors. Most patients in the present report had good to excellent functions following surgery and few complications occurred in the present report.

Digital soft tissue Ewing's sarcoma of foot--a case report.

A 22-year-old female presented with rapidly increasing, fungating and painful growth of right foot of 4 months duration. The growth had superadded infection. Radiography revealed soft issue mass between 2nd and 3rd toes without any bony involvement. Histopathologically it was confirmed to be a case of extraskeletal Ewing's sarcoma. Amputation above the ankle was done. She was advised for chemotherapy. She is still alive after 2 years. Here in this case the tumour arose in the subcutaneous tissue between 2nd and 3rd toes and diagnosed as a case of Ewing's sarcoma of the foot.

Ewing's sarcoma of metacarpal. A case report and review of literature.

The radiological features of a rare case of Ewing's sarcoma of right fourth metacarpal in a twenty-five years young male are described. An up date review of literature is also presented.