Cerebellar Dysplastic Gangliocytoma: Case Report

Lhermitte-Duclos disease (LDD), or cerebellar dysplastic gangliocytoma, is a rare type of cerebellar tumor, from unknown origin. Patients can be asymptomatic for several years, but there are usually imprecise neurological signs for long periods.

Robotic excision of complex adrenal mass with retrocaval extension and encasement of renal hilum with renal preservation

ABSTRACT Objective: The purpose of this video is to present robotic excision of a complex adrenal mass with retrocaval extension and encasement of renal hilum in a 16 year old boy. Biochemical screening was negative for metabolically active component. Computerized tomographic scan with contrast revealed a homogenous mass of approximately 10.8 cm x 6.2 cm x 4.2 cm in the suprarenal area on right side that was extend-ing behind inferior vena cava and encasing renal hilar vessels. Imaging findings were that of a classical ganglioneuroma. Material and methods: Robot assisted laparoscopic adrenalectomy with sparing of renal hilar vasculature was performed. With patient in lateral position, five ports were used, including one for liver retraction. Da Vinci® system with four arms was docked from over the right shoulder. The displaced renal hilar structures were identified by opening Gerota's fascia. Mass was dissected completely and removed through Pfan-nensteil incision. Results: Duration of procedure was 345 minutes and console time was 290 minutes. Blood loss was 250 mL. Post-operative renal doppler showed normal blood flow. He was discharged on post-operative day three. Histopathologic examination of specimen revealed ganglioneuroma arising from adrenal gland. Conclusion: Ganglioneuroma is a rare adrenal tumor with good prognosis on surgical removal. The advent of robotic surgery has made complex surgical procedures involving vital structures like inferior vena cava be performed using minimally invasive techniques without compromising oncologic principles.

Diffusion-Weighted Magnetic Resonance Imaging Findings in a Patient with Trigeminal Ganglioneuroma

A case of intracranial ganglioneuroma arising from the trigeminal nerve in the pontine and cerebellopontine angle cistern, in a 44-year-old female, is presented with an emphasis on diffusion-weighted imaging findings. We will discuss on how the tumor in the very unusual location should be differentiated particularly focused on diffusion-weighted imaging findings.

Adrenal ganglioneuroma / Ganglioneuroma de adrenal

Adrenal incidentalomas (AI) are unsuspected adrenal masses discovered during investigation of unrelated diseases, and are increasing in frequency. The majority of the AI is non-secretory adenomas, although it can also represent primary or metastatic malignant neoplasia. However, less frequent diseases should not be forgotten in the differential diagnosis. We describe a case of a young woman with an adrenal mass without clinical or laboratorial signs of hormonal hyperfunction. Diagnosis was performed after an episode of acute pyelonephritis in which the imaging study was carried out in order to exclude local complications. During the surgical procedure, the fish flesh aspect of the mass raised the suspicion of a neurogenic tumor, which was diagnosed in the histophatological analysis. The patient presented the most common manifestation of adrenal ganglioneuroma, an incidentaloma.

Incidentalomas adrenais (IA) são massas descobertas ao acaso na investigação de distúrbios não relacionados a patologias das glândulas adrenais e tem se tornado cada vez mais frequentes. A maioria dos casos é constituída por adenomas não secretores, embora possa também representar uma neoplasia maligna primária ou metastática. Porém, no diagnóstico diferencial, doenças menos prevalentes não podem ser esquecidas. Relatamos um caso de massa adrenal em mulher jovem, sem sinais de hiperfunção clínica ou laboratorial. O diagnóstico foi feito após episódio de pielonefrite aguda, em que o exame de imagem foi realizado para excluir complicações locais. Durante a abordagem cirúrgica, o aspecto em carne de peixe da lesão fez suscitar a hipótese de uma lesão de origem neural, a qual foi prontamente definida pelo exame histopatológico. A paciente se apresentava com a manifestação mais comum do ganglioneuroma de adrenal, um incidentaloma.

Extra-adrenal retroperitoneal ganglioneuroma

Ganglioneuromas are benign tumors that originate from primordial neural crest cells. We report a retroperitoneal ganglioneuroma arising from the left paraspinal region. A twenty-year old woman presented with left lumbar pain. Neurological workup was normal. Imaging revealed a left paraspinal retroperitoneal mass measuring 9.74 x 6.19 x 4.30 centimeters. Complete surgical removal was uneventful. During follow-up, left lumbar pain improved and imaging showed no evidence of the disease

Primary orbital ganglioneuroma in a 2-year-old healthy boy

A 2-year-old healthy child presented with progressive unilateral proptosis. Complete work up including: general examination, detailed ophthalmic evaluation and radiological imaging were done. He underwent orbital exploration via anterior orbitotomy incision and debulking of the tumor was done. The histopathological examination confirmed the diagnosis of orbital ganglioneuroma. Ganglioneuroma is an unusual benign tumor of neuroplastic origin with extremely rare orbital involvement with only one prior reported case in a youth. The tumor is slow growing and non-metastasizing. Biopsy is necessary to differentiate it from the malignant neuroblastoma and excision is usually curative

Extra-adrenal composite pheochromocytoma-ganglioneuroma

Composite tumors containing pheochromocytoma and ganglioneuroma are rare tumors typically arising in the adrenal glands. Very few were reported outside of the adrenals. We present the case of a middle-aged woman with a retroperitoneal mass that was discovered when she complained of local signs of compression and systemic signs of catecholamine hypersecretion. On pathology the mass was composed of large polygonal and pleomorphic cells arranged in nests characteristic of pheochromocytoma that were mixed with clusters of mature ganglion cells and bundles of spindle cells characteristic of ganglioneuroma. The histological features were benign, but due to its invasion of the inferior vena cava and of the right renal artery, we had concerns about the malignant potential of the pheochromocytoma component, which is extremely rare in these composite tumors

MR Imaging and Histopathologic Findings of A Case of Cerebral Ganglioneurocytoma

We report a case of ganglioneurocytoma manifesting as a complex partial seizure in a young adult male. MR images depicted a well-marginated cystic mass with a heterogeneous solid portion abutting the dura in the parietal lobe. The solid portion showed minimal heterogeneous enhancement, and pressure erosion of the overlying calvarium had occurred. Following gross total resection, the clinical outcome was satisfactory, with no further seizures, and during the five-year follow-up period, the tumor did not recur.

Cytopathology of neuroblastoma, ganglioneuroblastoma and ganglioneuroma.

Four cases of neuroblastomas, two cases of ganglioneuroblastomas and one case of ganglioneuroma diagnosed by fine needle aspiration cytology (FNAC) are presented. Five tumors were in the retroperitoneum and two tumors occurred in the posterior mediastinum. Complete clinical history, physical examination, radiological investigation, laboratory parameters, cytological study of multiple slides aided with special stains and cell block preparation of aspirated material helped in diagnosis of these tumours. Cytohistopathological correlation was carried out from surgically resected specimens and from autopsy studies.

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease)--a case report with review of the literature.

Dysplastic gangliocytoma of the cerebellum is an uncommon malformative lesion and clinically manifest as a slow growing space occupying lesion. In this report, the characteristic clinical and histopathological features of a case of dysplastic gangliocytoma of the cerebellum are described.

Regresión espontánea de neuroblastoma / Spontaneus regression of neuroblastoma

Se presenta un caso de regresión espontánea de ganglioneuroblastoma a la forma benigna de ganglio neuroma. Es el primero que se reporta en Panamá. Las formas tempranas de neuroblastoma tienen el mejor pronóstico

Glanglioneuroma de mediastino: presentación de un caso y revisión de la literatura / Mediastium ganglio neuroma: report of the case and review of the literature

El mediastino puede ser asiento de una gran variedad de patología tumoral. Las masas mediastínicas son asintomáticas en un 50% de los casos, pero debido a la alta incidencia de tumores malignos y al riesgo potencial de albergar un tumor maligno en el mediastino, estas lesiones deben ser diagnosticadas con presión y tratadas adecuadamente. Existen clasificaciones de las formaciones que ocupan espacio en el mediastino de acuerdo a su localización preferentes en los comportamientos del mismo. Estas tienen gran interés prácticos ya que los distintos tumores muestran una extraordinaria predilección por determinadas localizaciones; así este simple dato, permite sospechar la naturaleza del tumor. Presentamos un caso de lesión ocupante de mediastino posterior, que correspondió a un GANGLIONEUROMA, tumor derivado del sistema nervioso autónomo, benigno, asintomático, descubierto en una radiografía de tórax preoperatoria para una colecistectomía electiva. Mostramos la metodología, técnica quirúrgica y se comenta una complicación postoperatoria: síndrome de Bernard-Horner. Se revisó la literatura acerca de esta infrecuente patología

Ganglioneuroma duodenal associado à pâncreas ectópico: apresentaçäo de caso de revisäo de literatura, diagnostico diferencial das ganglioneuromatosis e dos ganglioneuromas / Duodenal ganglioneuroma associated with ectopic pancreas: report of a case of review of literature; differential diagnosis of ganglioneuromatoses and ganglioneuromas

De acordo com a histogênese do tecido nervoso é possível compreender a íntima relaçäo entre os vários tumores da linha neurogênica. A literatura nos relata que os tumores aqui discutidos podem evoluir para uma linha maligna ou ao contrário podem involuir para a benignidade por fatores desconhecidos. Ao mesmo tempo em que revemos as generalidades dos ganglioneuromas, apresentamos um caso raro de ganglioneuroma duodenal associado à pâncreas ectópico. Procuramos estabelecer parâmetros para o diagnóstico diferencial entre duas entidades até aqui relatadas associadamente, os ganglioneuromas e as ganglioneuromatoses gastro-intestinais. Em última análise estabelecemos o diagnóstico diferencial entre enfermidade potencialmente benigna e uma enfermidade maligna e consequentemente uma atitude terapêutica definida