Orbital Reconstruction in Neuroectodermal Tumor of the Orbit: Multimodal Treatment Approach.

INTRODUCTION: Primitive neuroectodermal tumors of peripheral origin are very rare, and orbital neuroectodermal tumors are even more uncommon. Only 25 patients with primary orbital involvement in the pediatric age group have been reported. METHODS: In this article, the authors describe their experience in the multimodality treatment approach to treat neuroectodermal tumor of the orbit. The authors also present a male patient 3-year old presenting with a neuroectodermal tumor of the right orbit causing rapidly progressive proptosis. The patient underwent an upper and lateral orbital marginotomy. The entire bone defect was reconstructed with a bone graft, allowing for the reconstruction of the floor and the lateral wall of the middle cranial fossa, the floor of the anterior cranial fossa, the upper and lateral orbital frame, and the right zygomatic bone. Over a period of 16 months, the patient was subjected to chemotherapy. RESULTS: In the postoperative period, a favorable evolution of the disease was observed, with growth in the reconstructed structures, good projection of the orbit and the eyeball, and stable results without tumor recurrence. CONCLUSIONS: The authors present the clinical analysis, surgical management, as well as the chemotherapy treatment established, with follow-ups at 1 and 2 and a half years. This experience shows the effectiveness of multimodality therapy in the treatment of rare tumors of difficult handling.

Open removal of a retained retrohepatic inferior vena cava filter with a residual primary neuroectodermal renal tumoral thrombus.

Primary neuroectodermal renal tumours (PNET) are rare and aggressive neoplasms; thrombosis of the inferior vena cava (IVC) is associated with this entity. We report here the case of a 19-year-old man who experienced a new onset of abdominal pain. A CT scan revealed a large left renal mass, perirenal haematoma and IVC thrombosis. Owing to an acute drop in haemoglobin and subsegmentary pulmonary embolism, he underwent emergency selective renal artery angiography and embolisation of bleeding vessels and IVC filter (IVCF) placement. Once stable, he underwent a left radical nephrectomy and IVC thrombectomy; the pathology report confirmed PNET. 6 months later, imaging revealed a residual tumoral thrombus in the IVCF located in the retrohepatic IVC. The patient underwent removal of this device and the thrombus via a right thoracoabdominal approach. He recovered well and at 4 months, he continues his chemotherapy cycles.

[Primitive neuroectodermal tumor of the kidney. A case report]. / Tumor neuroectodérmico primitivo del riñón. Caso clínico.

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.

[Orbital tumors in children. Transcranial surgical approach]. / Tumores orbitarios en niños. Su abordaje quirúrgico transcraneal.

From March 1980 to December 1995, at the Department of Neurosurgery of the Instituto Nacional de Pediatría (INP), 23 patients were treated for orbital tumors. In this communication, the authors report on the clinical presentation, age, radiological findings, visual-evoked potentials, type of surgery, histological diagnosis, and results. Two kinds of surgical techniques were employed: craniotomy with orbital roof removal (NAFZIGER) and fronto-orbitotomy. Eleven patients had optic nerve gliomas, 3 had fibrous osseous dysplasia, 2 meningiomas, neurofibromas, Langerhans cells histiocytosis, and 1 case of Anaplastic astrocytoma, Teratoma, primitive Neuroectodermal tumor, germinoma with areas of choriocarcinoma. The results were evaluated for the quality of life in regard to the Karnofsky scale, tumor recurrence and postoperative visual-evoked potentials. It is important to note the very variable histological kinds of lesions found in this series that can be approached surgically with cranio orbitotomy with good results.

Tumores orbitarios en niños. Su abordaje quirúrgico transcraneal / Orbitary tumors in children. The transcranial surgical approach

De marzo de 1980 a diciembre de 1995 se trataron 23 pacientes con tumores orbitarios en el Departamento de Neurocirugía del Instituto Nacional de Pediatría (INP). En este trabajo se analiza edad, características clínicas, alteraciones radiológicas y de potenciales evocados; tipo de cirugía, diagnóstico histopatológico y resultados. En todos ellos se efectuó un abordaje craneoorbitario, tipo destechamiento simple, o bién, por fronto-orbitotomía. De estos pacientes, 11 tenían glioma del nervio óptico, tres displasia ósea fibrosa, dos meningioma, neurofibroma, histocitosis de células de Languerhans; uno astrocitoma anaplásico, teratoma, tumor neuroectodérmico primitivo y germinoma con áreas de coriocarcinoma. Se realizó, craneotomía con destechamiento orbitario (Nafziger) en 19 casos y fronto-orbitotomía en 4. Los resultados fueron clasificados de acuerdo a la escala de karnofsky; potenciales evocados postoperatorios, presencia de defecto estético y proptosis pulsátil. Buenos resultados fueron: Karnofsky mayor de 70, no recurrencia tumoral y no emperoramiento de potenciales Visuales; regulares: sin recurrencia tumoral y no empeoramiento de Potenciales Visuales; regulares: sin recurrencia tumoral, cambio en potenciales evocados, escala de Karnofsky entre 50 y 60; malos: recurrencia, lesión visual agregada en potenciales evocados y Karnofsky menor de 50. El reporte muestra la variedad de lesiones intraorbitarias que pueden ser abordadas por vía transcraneal