Primary Peripheral Primitive Neuroectodermal Tumor of the Prostate on 18F-DCFPyL PET/CT.

Peripheral primitive neuroectodermal tumor (PNET) is a group of malignant tumors composed of small round cells. Peripheral PNET usually originates in the skeletal system. However, the presence of PNET lesion in prostate is extremely rare. We report a case of a 40-year-old man who presented with dysuria for more than 2 months. Pelvic MRI indicated prostatic malignant tumor, and F-DCFPyL PET/CT showed an isolated prostatic mass with high uptake of F-DCFPyL. Although F-DCFPyL is very specific for prostatic adenocarcinoma, a final diagnosis of peripheral PNET was made by pathology examination.

Primitive neuroectodermal tumor arising from an untreated congenital undescended testicle.

A 26-year-old male with a personal history of schizophrenia initially presented with a 13 cm pelvic mass corresponding to a cryptorchidic testis. The patient was treated with primary and second-line chemotherapy for metastatic germ-cell tumor followed by surgical consolidation. Final pathology revealed a primitive neuroectodermal tumor (PNET) mixed with mature teratoma. Despite multidisciplinary management, significant patient non-compliance led to inadequate follow up and treatment delays ultimately resulting in death. To our knowledge, this is the only reported case of teratoma with malignant transformation arising from an untreated congenital undescended testicle.

Ewing's sarcoma/primitive neuroectodermal tumor arising from the adrenal gland: a case report and literature review.

We report a rare case of Ewing's sarcoma (ES)/primitive neuroectodermal tumor (PNET) arising from the adrenal gland. A 17-year-old Japanese woman presented with left upper abdominal pain and high fever. Computed tomography and magnetic resonance imaging revealed a 15 × 10 cm tumor replacing the adrenal gland. Preoperative diagnosis was an adrenocortical carcinoma. Resection of the tumor was performed. We obtained the final diagnosis of ES/PNET by immunohistochemical molecular study with positive staining for the MIC2 gene product (CD99) and a Ewing sarcoma breakpoint region 1 (EWSR1) gene rearrangement. Local recurrence was observed one month after the surgery. The patient was then treated with systemic chemotherapy and localized radiotherapy.

Presentación de un sarcoma extraóseo de Ewing como una masa mediastínica posterior / Extraskeletal Ewing's Sarcoma Presenting as a Posterior Mediastinal Mass

La familia de tumores del sarcoma de Ewing es un grupo poco habitual de neoplasias malignas que pueden localizarse en regiones tanto óseas como extraóseas. El sarcoma de Ewing extraóseo (SEE) es poco frecuente y afecta de modo predominante a los tejidos blandos del tronco o de las extremidades. Describimos a una paciente de 19 años de edad que refirió dolor en el brazo izquierdo. La radiografía de tórax simple reveló una opacidad que ocupaba casi todo el hemitórax izquierdo y, tras realizar modalidades de diagnóstico por imagen, se demostró una lesión de masa realzada para el contraste, sólida, en el mediastino posterior. Era evidente una desviación mediastínica y el pulmón izquierdo estaba colapsado. Aunque, como diagnóstico inicial, se consideró un linfoma, la paciente se sometió a una biopsia y el análisis histopatológico reveló un SEE. Entre los estudios publicados, solo se han descrito unos pocos casos de SEE localizados en el mediastino. Concluimos que, aunque es una localización insólita del SEE, debe tenerse en cuenta en el diagnóstico diferencial de las masas mediastínicas(AU)

The Ewing's sarcoma family of tumors is an uncommon group of malignant neoplasms that may be located in both skeletal and extraskeletal regions. Extraskeletal Ewing's sarcoma (EES) is quite rare and predominantly involves the soft tissues of the trunk or the extremities. Herein, we report the case of a 19-year-old female patient who complained of left arm pain. Simple chest radiography revealed an opacity occupying almost the entire left hemithorax. Diagnostic imaging techniques demonstrated a solid contrast-enhanced mass in the posterior mediastinum. There was an evident mediastinal shift, and the left lung was collapsed. Even though lymphoma was considered as an initial diagnosis, a biopsy was taken and its histopathological analysis revealed EES. In the literature, there have been only a few case reports of ESS located in the mediastinum. We conclude that, although this is an unusual location, EES should be contemplated in the differential diagnosis of mediastinal masses(AU)

Varón de 43 años con masa pélvica derecha / No disponible

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Tumor neuroectodérmico primitivo (tumor de Askin) en la pared torácica / Primitive neuroectodermal tumour (Askin tumour) in the chest wall

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Sarcoma de Ewing renal primario / Primary renal Ewing`s sarcoma

OBJETIVO: Presentación de dos nuevos casos de sarcoma de Ewing / tumor neuroectodérmico primitivo renal primario, uno de ellos con trombo en cava.MÉTODO: Caracterización de los dos casos clínicos y revisión bibliográfica mediante búsqueda en pubmed.RESULTADO: Presentamos los casos de dos varones diagnosticados de sarcoma de Ewing renal primario, que han sido tratados con nefrectomía y quimioterapia adyuvante; encontrándose en remisión completa hasta la fecha.CONCLUSIÓN: El sarcoma de Ewing / tumor neuroectodérmico primitivo renal primario es una entidad rara que afecta mayoritariamente a adultos jóvenes. La historia natural de estos tumores es la evolución hacia una enfermedad metastásica y la muerte. El tratamiento es multimodal, y combina cirugía y quimioterapia. El papel de la radioterapia no está bien establecido(AU)

OBJECTIVE: To report two new cases of Ewing`s sarcoma/ primitive neuroectodermal tumor of the kidney, one of them with tumor thrombus in cava.METHOD: Characterization of two new cases and literature review by PubMed search.RESULTS: We report the cases of two men diagnosed with primary renal Ewing`s sarcoma, who have been treated with nephrectomy and adjuvant chemotherapy, being in complete remission to date.CONCLUSION: Ewing`s sarcoma / primitive neuroectodermal tumor of the kidney is a rare condition that mainly affects young adults. The natural history of these tumors is the evolution towards metastatic disease and death. Treatment is multimodal, combining surgery and chemotherapy. The role of radiotherapy is not well established

Intramedullary primitive neuroectodermal tumor presenting with rapidly-progressive cauda equina syndrome.

A 39-year-old male presented with gait disturbances with rapid deterioration for 2 weeks. Neurological examination found paraparesis, sensory loss in the L1-S5 dermatomes, and vesicorectal dysfunction. Magnetic resonance (MR) imaging revealed a fusiform intramedullary tumor at T12-L1 levels with heterogeneous enhancement. The patient underwent microsurgical tumor resection. A myelotomy exposed a highly vascular tumor that was subtotally resected. Histological examination demonstrated hypercellular tumor accompanied by significant cell atypism and mitotic figures. Immunohistochemical staining was positive for glial fibrillary acidic protein, S-100 protein, synaptophysin, and INI-1, consistent with primitive neuroectodermal tumor (PNET). Postoperatively, the patient underwent irradiation to the whole craniospinal axis. He experienced local recurrence 7 months after surgery. MR imaging performed at 10 months revealed holocord progression and intracranial dissemination. The patient died 13 months after the onset of the disease. PNET should be considered in the differential diagnosis of an intramedullary spinal cord tumor.

Sarcoma de Ewing pulmonar/tumor neuroectodérmico primitivo (PNET): aportación de un caso y revisión de la bibliografía / Pulmonary Ewing sarcoma/primitive neuroectodermal tumor: a case report and a review of the literature

Los sarcomas primarios de tórax son muy poco frecuentes. Sarcoma sinovial, angiosarcomas, leiomiosarcomas, rabdomiosarcomas y mesoteliomas sarcomatoides son las variantes intratorácicas más comunes. Aunque el sarcoma de Ewing/tumor neuroectodérmico primitivo (PNET) torácico se desarrolla habitualmente en la pared torácica, se ha descrito en la literatura médica algún caso de localización pulmonar primaria. Presentamos el caso de una mujer de 22 años diagnosticada de sarcoma de Ewing/PNET pulmonar mediante muestra broncoscópica por sus características histológicas, inmunohistoquímicas y técnicas de hibridación in situ. Se excluyó el origen metastásico mediante radiografía, gammagrafía y biopsia de médula ósea. Se inició quimioterapia según el esquema VACD-IE (vincristina, actinomicina D, ciclofosfamida, doxorrubicina, ifosfamida y etopósido), con buena respuesta. En la actualidad acude de forma regular a consultas ambulatorias(AU)

Primary thoracic sarcomas are very rare. The most common intrathoracic variants are synovial sarcoma, angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid mesothelioma. Although thoracic Ewing sarcoma/primitive neuroectodermal tumor (PNET) usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung. We present the case of a 22-year-old woman with Ewing sarcoma/PNET diagnosed following histologic, immunohistochemical, and in situ hybridization studies of a bronchial biopsy specimen. Radiography, ventilation-perfusion scintigraphy, and a bone marrow biopsy confirmed that the tumor was not metastatic. The patient was started on a chemotherapy regimen of vincristine, actinomycin, cyclophosphamide, doxorubicin, ifosfamide, and etoposide and responded well. She is now being seen regularly at our outpatient clinic(AU)

Peripheral Primitive Neuroectodermal Tumour (pPNET) in the cervical spine.

Primary spinal primitive neuroectodermal tumours are rare. We present a 45-year-old man with a peripheral primitive neuroectodermal tumour arising in the cervical spine. We believe this to be the first report of this type of tumour in the cervical spine.

Solución del caso 9. Tumor de Askin / Solution to case 9. Askin tumor

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Familial Currarino syndrome presenting with peripheral primitive neuroectodermal tumour arising with a sacral teratoma.

This report illustrates a rare genetic disorder, Currarino syndrome, in association with an unusual malignant transformation to a peripheral primitive neuroectodermal tumour within a sacral teratoma. The triad of features consists of a presacral mass, partial sacral agenesis and anorectal anomalies. The most common presentation is constipation. In this case there was a history of constipation, teratomas and spinal abnormalities in many of the family members over three generations. Detailed family history taken at time of initial presentation may have prevented delay in diagnosis and averted the need for intensive treatment, which may well cause late sequelae.