Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution

OBJECTIVE: To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. MATERIALS AND METHODS: We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for > or = 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. RESULTS: Among the 70 patients (mean age, 35.8 +/- 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). CONCLUSION: Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites.

Unusual Malignant Solid Neoplasms of the Kidney: Cross-Sectional Imaging Findings

Malignant kidney neoplasms are the most frequently encountered solid kidney masses. Although renal cell carcinoma is the major renal malignancy, other solid malignant renal masses should be considered in the differential diagnosis of solid renal masses that do not contain a macroscopic fatty component. In this pictorial essay, we present the imaging findings of a primitive neuroectodermal tumor, primary liposarcoma of the kidney, primary neuroendocrine tumor, leiomyosarcoma, synovial sarcoma, malignant fibrous histiocytoma, sclerosing fibrosarcoma and renal metastasis of osteosarcoma.

Tumor neuroectodérmico primitivo renal: sarcoma de Ewing extraóseo: presentación de un caso / Primitive neuroectodermal renal tumor: Ewing sarcoma extra osseous: presentation of a case

El sarcoma de Ewing o también llamado tumor neuroectodérmico primitivo es una entidad muy rara, cuyo diagnóstico se realiza con la utilización de estudios de inmunohistoquímica, su comportamiento es muy agresivo con una supervivencia limitada. Se presenta el caso de un paciente masculino de 18 años de edad que consultó por presentar un tumor renal, se le realizó nefrectomía radical con diagnóstico definitivo de sarcoma de Ewing extra esquelético. Esta es una patología quirúrgica poco frecuente, lo que nos motivó a su presentación y a la revisión de la literatura.

The Ewing sarcoma is also denominated primitive neuroectodermic tumor and is considered how a rare entity, the diagnostic is realize with the utilization of immunohistochemestry study’s, his clinical curse is very aggressive with a limite superlife. We presented and study a clinical case of a sex male patient of 18 years old which consult us for present a renal tumor. He underwent a radical nefrectomy with a definitive diagnostic of extra osseous Ewing sarcoma. This is a less frequent surgical pathology, we motive us to present and review the literature.

Derrame pleural incomum: metástase pleuropulmonar de tumor neuroectodérmico primitivo / Uncommon pleural effusion: pleuropulmonary metastasis from primitive neuroectodermal tumor

O tumor neuroectodérmico primitivo é uma neoplasia com diferenciação neural de comportamento invasivo que origina metástases para diversos órgãos. Relatamos um caso de tumor neuroectodérmico primitivo primário em axila com metástases para pulmão, pleura, osso, músculo ilíaco e medula óssea. Enfatizamos o achado incomum da análise citológica do líquido pleural.

Primitive neuroectodermal tumor is an invasive neoplasm with neuronal differentiation, which frequently results in metastasis in various organs. We report the case of a patient with primitive neuroectodermal tumor whose primary site was the axilla. The patient presented with metastases in the lung, pleura, bone, iliac muscle and bone marrow. We highlight the uncommon finding in the pleural fluid cytology.

Tumor neuroectodérmico primitivo de maxilar: presentación de un caso / Primitive neuroectodermal tumor of maxilla: presentation of a case

El tumor neuroectodérmico primitivo periférico forma parte de la familia de tumores sarcoma de Ewing representando el 5 % de los mismos, en maxilar superior es infrecuente. Se reporta este caso clínico de un adolescente masculino, de 15 años referido a nuestro centro con pérdida de peso, tumoración en cuadrante superior izquierdo de cavidad bucal, de 6 cm de diámetro, que ocupa el 50 % de la misma, sobre infectada, con áreas de necrosis. La biopsia reportó: tumor de células redondas indiferenciadas. Se observó rápido crecimiento tumoral que triplica su tamaño en 8 días, protruyendo por cavidad oral, la ocupa en totalidad, tendiente a obstrucción de la vía aérea; ameritó traqueotomía y gastrostomía de emergencia. Los estudios de inmunohistoquímica concluyen: tumor neuroectodérmico primitivo (inmunorreactivo a CD99, enolasa neuroespecífica y cromogranina), recibe dos ciclos de ciclofosfamida, vincristina, actinomicina D, ifosfamida y etoposido, con progresión de la enfermedad y deterioro del estado físico, por lo que recibe radioterapia 6 000 cGy con cisplatino, lográndose condiciones favorables a la cirugía: Maxilectomía bilateral con preservación del piso de la órbita. El estudio anatomopatológico reportó tejido fibromixoide con abundante vascularización sin evidencia de malignidad, márgenes libres. Actualmente, 7 meses sin evidencia de enfermedad en espera de prótesis palatina para la restitución de deglución funcional. En localizaciones infrecuentes debe el tratamiento ser multidisciplinario e individualizado, incluyendo las tres modalidades terapéuticas (quimioterapia, radioterapia y cirugía).

Periferical neroectodermic primary tumor is a part of the sarcoma Ewing family and represents 5 % of them. The superior maxillary location is not frequent, we report these clinical case of a male teenage of 15 years old sent to our hospital with loss weigh, tumor in the oral cavity in the left superior quadrant of the oral cavity, with a diameter of 6 cm, that it occupies 50 % of the same one with sobreinfection, with necrosis. The biopsy reported: tumor of undifferentiated round cells. We observed a growth of the tumor that triples its size in 8 days, it occupies it in totality, with tendency to obstruction of the air way, needed emergency traqueostomy and gastrostomy. The inmunohistoquimical studies reveal primary neuroectodermic tumor (inmunoreactive to CD99 enolasa neurospecific and cromagine), received two cycles of ciclofosfamide, vincristine, actinomicyn D, ifosfamida and etoposid, with disease progression and depletory of his physical status; receive radiation therapy 6 000 cGy with platinum, obtaining favorable condition to surgery: A bilateral maxilectomy with preservation of the floor of the orbit. The anatomopathological study report fibromixoide weave a lot of vascularization without evidence of malignity, free margins. Actually, 7 months without evidence of disease waiting for platinum prosthesis to restituted the functional deglution. In the inusual localizations, the treatment has to be multidisciplinary, individualized, and include the three treatment modalities (Quemotherapy, radiation therapy and surgery).

Primary spinal intramedullary primitive neuroectodermal tumour in an adult

Primary spinal primitive neuroectodermal tumour [PNET] is believed to be very rare. A review of the available literature revealed 14 cases of spinal PNET, which affected the paediatric age group but no previously reported cases involved an adult. A 42-year-old patient presented with weakness of both lower limbs and signs of upper motor neuron lesion. Intramedullary PNET was discovered after neurological deterioration following C3-4, C5-6 and C6-7 discectomy. Patient received postoperative radiotherapy, however, he died 8 months later from brain metastasis. As this is the first case of its kind in the literature, we believe it is worth reporting to point out the possibility of the existence of such a lesion in the spinal cord

Primary Intraspinal Primitive Neuroectodermal Tumor at Conus Medullaris

A primary intraspinal primitive neuroectodermal tumor is very rare, with only 24 cases having been reported in the literature. In general this type of tumor is treated with surgery followed by radiotherapy and chemotherapy; however, the prognosis still remains poor. The case of a primary intraspinal primitive neuroectodermal tumor, at the conus medullaris in a 17 year old male patient is presented. He had sufferred from paraparesis, urinary difficulty and lower back pain of 1 month duration. A thoracolumbar MRI demonstrated a 2x2x8cm isointense intraspinal mass, on T1-weighted images, with strong contrast enhancement from the T11 to L2 level. There was no clinical or radiological evidence for the existence of an intracranial tumor. A histological examination revealed a small round cell tumor and immunohistochemical characteristics of PNET. The clinical, radiological and pathological features are discussed with a review of the literatures.

MRI features of intracranial primitive neuroectodermal tumors in adults: comparing with histopathological findings / 华中科技大学学报（医学）（英德文版）

The MRI appearances of 7 adult patients with pathologically proven intracranial primitive neuroectodermal tumors (PNET) were retrospectively analyzed. The MRI features were compared with findings in pathology and surgery. In this group, the tumor masses were most commonly found in the semisphere of cerebrum and in the vermis of cerebellum. They were relatively large and 4 were in lobulated shape. All of them had well-defined margins. MR images showed the tumors to be mildly or obviously hypointense on T1-weighted images and hyperintense on T2-weighted images. Most masses had heterogeneous appearances with some cystic and necrotic areas. Intratumoral haemorrhage and focal calcification were occasionally seen. Mostly, there was no or only mild surrounding edema. Marked inhomogeneous contrast enhancement on MRI was seen in 6 cases except one. Two patients with multiple intracranial metastases were revealed on MR images. In this series, there was good correlation between MRI features and findings in pathology and surgery. These results showed that certain MRI features might suggest the diagnosis of intracranial PNET in adults. MRI is an effective technique to detect these tumors and is helpful to treatment planning and follow-up.

Medulloepithelioma: a case report.

Histological, immunohistochemical, and CT morphological features of medulloepithelioma, a rare embryonal tumor of primitive neuroepithelium, are described.

Malignant teratoid medulloepithelioma in eye.

Malignant teratoid medulloepithelioma is an extremely rare tumor occurring in children younger than 5 years of age, arising from ciliary body epithelium or iris but few arise from optic nerve and retina. This report concerns a 5 years old boy who presented with pain, redness and protrusion of right eye. Histopathologically, the tumor was composed of epithelial and sarcomatoid component. The pseudostratified primitive appearing epithelial cells were arranged mainly in diffuse pattern, nests cords and tubules. At places, pseudo rosette and true rosette were seen. Mitoses were frequent consisting of 7-10/ HPF. The sarcomatoid component consisting of spindle shaped cells arranged in interlacing bundle were also seen. Mitoses counted 5-7/HPF. Massive areas of necrosis and hemorrhage along with calcification, focal area of mature cartilage were present. Vascular and optic nerve invasions were seen. This case of malignant teratoid medulloepithelioma is the second case diagnosed in TU Teaching Hospital within the period of 10 years and reported because of its rarity. The differentiations from other tumors of the orbit such as small cell tumor were discussed.

Primary primitive neuroectodermal tumor of the ovary: a case report.

Ovarian germ cell tumours having neuroectodermal cells, growing in patterns reminiscent of tumours of the central nervous system are rare. We report a case of primary primitive neuroectodermal tumor(PNET) of the ovary detected soon after pregnancy.

Medulloepithelioma of the ciliary body.

A rare case of medulloepithelioma of the ciliary body is described. The tumour necessitated enucleation of the eye; histopathological diagnosis was benign nonteratoid medulloepithelioma of the ciliary body.

Tumor neuroectodérmico primitivo del riñón: caso clínico / Primitive neuroectodermal tumor of the kidney: case report

A 27 years old female presented with diffuse abdominal pain, malaise, weight loss and a palpable mass in the left abdomen. Abdominal CAT scan showed a 18 cm heterogeneous tumoral mass that originated in the kidney. The patient was operated on and it was not possible to completely remove the mass. In the postoperative period the patient received chemotherapy with vincristine, cyclophosphamide and adryamicin but the disease progressed and the patient died 16 months later. The pathological study of the surgical specimen disclosed a tumor with monotonous proliferation of small round cells with a basophilic nucleus and scanty cytoplasm with PAS positive granules. Immunohistochemistry was positive for specific neuronal enolase and CD99. Involvement of the urinary system with primary neuroectodermal tumors is very infrequent

Tumor neuroectodérmico primitivo na infância: relato de 13 casos e revisão da literatura / Primitive neuroectodermic tumor in childwood: a report of 13 cases and literature review

Proposta: a proposta desse estudo é a análise retrospectiva dos pacientes com diagnóstico de tumor neuroectodérmico primitivo (PNET), admitidos no Departamento de Pediatria do Hospital do Câncer no periodo de 1989 a 1996. Pacientes e métodos: Ttdos os 13 pacientes portadores de PNET foram analisados retrospectivamente utilizando uma ficha para obtenção dos dados epidemiológicos clínicos, terapêuticos e seguimento de cada paciente. Resultados: a cirurgia foi a primeira abordagem terapêutica em 2 pacientes. Quimioterapia foi administrada em 12 pacientes e radioterapia em 2. Dos 13 pacientes 5 estão vivos em seguimento médio de 48 meses após o término do tratamento. Conclusão: a análise dos 13 pacientes com PNET e revisão da literatura demonstram a agressividade desse tumor. Devido a raridade dessa patologia, estudos multiinstitucionais, podem ter papel importante para análise de fatores prognósticos e consequente abordagem terapêutica.

Primitive peripheral neuroectodermal tumors Mayagüez Medical Center experience

Primitive neuroectodermal or neuroepithelial tumors are names used to describe neoplasias composed of undifferentiated cells resembling germinal cells of the embryonic neural tube. These tumors are small round cell malignancies of the neural crest origin arising outside the central and sympathetic nervous system. They are described as peripheral and central neuroectodermal tumors related to the original malignant cell. A great number of tumors are described under this classification in spite of the fact that there is no an universal acceptance that these small-cell neoplasms, regardless of their primary site, are derived from immature neuroectoderm tissue. Because one tumor resembles others in terms of its phenotypic expression, multiple specific studies such as clinical profile, ultrastructural, immunocytochemical, and cytogenetic features should be studied, since no single clinical or laboratory marker is by itself diagnostic. However, there is a chromosomal reciprocal translocation, t(11;22)(q24;q12), which is unique to Primitive Neuroectodermal Tumor (PNET)

Tumor neuroectodérmico primitivo supratentorial: estudo de quatro casos / Supratentorial primitive neuroectodermal tumor: study of 4 cases

As alteraçöes clinicopatológicas e imuno-histoquímicas de quatro casos de tumores neuroectodérmicos primitivos do sistema nervoso central foram investigadas. Três pacientes morreram. Todos os casos mostraram células vimentina positivas com morfologia de células neoplásicas e um caso mostrou células neoplásicas com imunoexpressäo para proteína glial fibrilar acídica, enolase neuro-específica e neurofilamento. O presente estudo indica que este grupo de tumores tem mau prognóstico e pode mostrar alteraçöes imuno-histoquímicas que indicam diferenciaçäo glial e/ou neruonal