Tumor neuroectodérmico de seios paranasais: diagnóstico e tratamento / Primitive neuroectodermal tumor of paranasal sinuses: diagnosis and treatment

Tumores neuroectodérmicos primitivos (PNET) são neoplasias raras e extremamente agressivas encontradasprincipalmente em crianças e adultos jovens. São classificados em periféricos ou centrais, de acordo com sua origem. O diagnóstico baseia-se na história clínica, sendo essenciais exames de imagem, como tomografia computadorizada e ressonância magnética, e, para a confirmação, estudo anatomopatológico. É importante distingui-los de outros tumores de células redondas pequenas,como linfoma, sarcoma de Ewing extraósseo e rabdomiossarcoma,exigindo diferenciação imunoistoquímica através de marcadores específicos. O tratamento envolve cirurgia, quimioterapia e radioterapia, sendo o prognóstico pobre e a sobrevida bastante reservada. O objetivo deste artigo é discutir as características clínicas, radiográficas ehistológicas dos tumores neuroectodérmicos primitivos e seu tratamento...

Primitive neuroectodermal tumours (PNET) are rare and highly aggressive neoplasms found mainly in children and young adults. They are classified in peripheral or central according to their origin. The diagnosis is based on clinical history, computed tomography and magnetic resonance imaging, but the pathological study is the only way to confirm it. It´s very important to distinguish the PNET from other small cell round cell tumors - such as lymphoma, Ewing´s sarcoma and rhabdomyosarcoma – through immunohistochemical specific markers. The treatment includes surgical resection, chemotherapy and irradiation. The prognosis is poor and the survival rate is variable. This article aims to discuss the clinical, imagingand histological features of the primitive neuroectodermal tumors and their treatment...

Tumor neuroectodérmico primitivo: relato de caso / Primitive neuroectodermal tumor: case report

JUSTIFICATIVA E OBJETIVOS: O tumor neuroectodérmico primitivo (PNET) pertence a um grupo de tumores de pequenas células redondas e, devido a sua semelhança biológica com os sarcomas de Ewing, tem sido incluído nesta família. O objetivo deste estudo foi descrever o quadro clínico, características de imagem e evolução de um caso de processo expansivo em coxa direita, com histologia consistente com sarcoma de Ewing extraesquelético. RELATO DO CASO: Paciente do sexo feminino, 21 anos, branca, apresentava queixa de dificuldade para deambular e dor na perna direita há aproximadamente seis meses. Ao exame físico foi detectada massa sólida na raiz da coxa direita. Exames de imagens identificaram formação expansiva sólida, sendo o estudo histopatológico/imuno-histoquímico compatível com PNET/sarcoma de Ewing extraesquelético. Foi instituído tratamento quimioterápico, que nãofoi suspenso mesmo após diagnóstico de gestação. A gravidez evoluiu com oligodramnia e óbito fetal. Dois meses após o diagnóstico, a paciente apresentou depressão, piora do estado geral e óbito. CONCLUSÃO: O estudo relatado reflete a agressividade desta doença apesar da possibilidade de multimodalidade terapêutica. A aplicação da genética molecular em estudos recentes correlaciona o rearranjo genético da translocação entre os cromossomas 11e 22 com o prognóstico.

BACKGROUND AND OBJECTIVES: Primitive neuroectodermal tumor (PNET) belongs to a family of small round cell tumors, and because of their biological similarity has been includedin the family of Ewing's sarcomas. The objective of this case was to describe the clinical, imaging characteristics and outcome of a case of an expansive process in the right thigh, with histology consistent with extraskeletal Ewing's sarcoma. CASE REPORT: Female patient, 21 years, white, featured a complaint of difficulty in walking and pain in his right leg for about six months. On examination a solid mass was detected at the root of the right thigh. Imaging techniques identified an expansive solid mass and histopathology was compatible with PNET/extra-skeletal Ewing's sarcoma. Chemotherapy was instituted, which was not suspended even after pregnancy diagnosis. It progressed with oligohydramnios and fetal death. In two months, the patient had depression, poor general condition and death. CONCLUSION: The study reported reflects the aggressive nessof this disease despite multimodality therapy. The application of molecular genetics in recent studies correlating the genetic rearrangement of the translocation between chromosomes 11:22 prognosis.

Metástasis de calota / Calvarial metastasis

Objective. To describe five cases of cranial vault metastasis. Materials and methods. Clinical records and images of five patients with cranial vault metastasis were reviewed at the “Hospital de Clinicas” and “Hospital Español” from Buenos Aires, between January 2009 to June 2010. Results. Case 1, 63 years old female, complains for headache with aleatory response to medical therapy; MRI: right occipital bone lession; Pathological Finding: breast carcinoma metastasis. Case 2, 70 years old female, complains for palpable lession of the scalp; MRI: right fronto-parietal intraxial lession and right parietal cranial vault lession; Pathological Finding: endometrial carcinoma metastasis (primary, diagnosed 4 months before). Case 3, 76 years old female, with palpable lession in the scalp; MRI: left parietal cranial vault lesion. Pathological Finding: kidney carcinoma metastasis (primary, diagnosed in 2008). Case 4, 50 years old female and Case 5, 78 years old male, both complains for bone pain; serological findings and marrow bone biopsy compatible with multiplemyeloma; screening of the long bones and cranial vault demonstrate evidence of bone infiltration. Conclusion. Metastasis at the cranial vault often presents with headache or pain upon inspection of a palpable scalp lession. Images of the CNS involves CT scan (delimitate lession and differentiates litic from blastic metastasis) and MRI (shows the degree of dural and brain infiltration). Prevalence of cranial vault metastasis is greater than brain metastasis, but this underdiagnosis is due that the first are frequently course without symptoms.

Sarcoma de Ewing extraesquelético que semeja abdomen agudo / Extra-skeletal Ewing's sarcoma resembling acute abdomen: case report

Introducción: El sarcoma de Ewing extraóseo es un tumor raro de origen neuroectodérmico que se ha presentado principalmente en las partes blandas de las extremidades y del tórax; histológicamente es similar al sarcoma de Ewing en el tejido óseo. Caso clínico: Paciente con abdomen agudo y leucocitosis, en quien por imagen (ultrasonido y tomografía axial computarizada) se diagnosticó enfermedad diverticular complicada del colon, por lo que fue intervenido quirúrgicamente, encontrando lesión localizada en el espacio de Retzius con extensión a la cavidad abdominal de 20 x 15 x 15 cm. El tratamiento consistió en extirpación del tumor, separándolo de las vísceras adyacentes y el paciente fue tratado con quimioterapia: epirrubicina, ciclofosfamida y vincristina por seis ciclos; la tomografía abdominal de control mostró actividad tumoral en el retroperitoneo, adyacente al colon ascendente y ciego, por lo que se requirió nuevo tratamiento resectivo. Conclusiones: En la revisión de la literatura no hay informes previos de sarcoma de Ewing extraóseo cuya presentación sea abdomen agudo. Por la rareza de esta lesión, en la literatura solo hay registro de casos o algunas series de casos, por lo que no fue posible llevar a cabo comparaciones; la extirpación de la lesión es el pilar del tratamiento. No se define el papel de la quimioterapia preoperatoria. La biopsia percutánea guiada por algún método de imagen es de utilidad para otorgar al paciente un tratamiento óptimo que le permita un mejor pronóstico.

BACKGROUND: Extraosseous Ewing's sarcoma is a rare tumor of neuroectodermal origin. It presents mainly in the soft tissue of the extremities and thorax. Histologically, it is similar to Ewing's sarcoma of the bone. CLINICAL CASE: We present the case of a male who arrived at the emergency room with acute abdomen, leucocytosis and imaging techniques (abdominal ultrasound and computed tomography) suggestive of complicated diverticular disease. He was treated with emergency surgery. Intraoperative findings were an unsuspected tumor (20 x 15 x 15 cm). Treatment consisted of extirpation of the tumor, separating it from the adjacent viscera and followed by chemotherapy based on epirubicin, cyclophosphamide and vincristine for six cycles. Because the control abdominal CT demonstrated tumor activity in the retroperitoneum adjacent to the ascending colon and cecum, further resection was decided upon. CONCLUSIONS: In a review of the literature, no previous reports of extraosseous Ewing's sarcoma were found presenting as acute abdomen. Due to the rarity of this tumor, only case reports or series have been found in the literature without randomized or comparative studies. Surgery was the cornerstone of treatment, without reports of preoperative chemotherapy. If the patient's condition permits, percutaneous needle biopsy is mandatory to obtain optimum treatment as well as to improve prognosis.

Radioterapia como tratamiento de las metástasis tumorales: reporte de dos casos / Radiotherapy as treatment of tumor metastasis: report of two cases

Se presentan dos casos de pacientes con neuroblastoma y sarcoma de Ewing respectivamente, los cuales presentan lesiones metastásicas. El primer caso presenta una lesión a nivel de cuerpo vertebral, el cual a su vez le produjo un síndrome de compresión medular. El paciente presenta alteración en la movilidad y sensibilidad de miembros inferiores, y alteración en el control de sus esfínteres. En el segundo caso se encontró una lesión metastásica a nivel de tejidos blandos en la zona parietal derecha. En ambos cuadros se valora el tratamiento por medio de la radioterapia para el control y reversión de los síntomas

Extraskeletal Ewing`s sarcoma Extraskeletal Ewings Sarcoma Arising in the Chest Wall / 결핵

Extraskeletal Ewing's sarcoma is a very rare tumor which was first reported by Angervall and Enzinger in 1975. The common sites of extraskeletal Ewing s sarcoma are bony structures of lower extremities, paravertebral region, and pelvis, but rarely chest walL Microscopically, extraskeletal Ewing's sarcoma is indistinguishable from the Ewing's sarcoma of bone. We present here a case of extraskeletal Ewing's sarcoma of the left lateral chest wall in a 19-year-old male. Wide extirpation and postoperative combined chemotherapy were done, and we discuss the clinical picture, histopathology, therapeutic management, and prognosis with review of the literature.