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.

A Rare WHO Grade I Lesion of the Posterior Fossa with Recurrence Biological Behavior ­ Dysplastic Gangliocytoma of the Cerebellum: Case Report

Dysplastic gangliocytoma of the cerebellum (DGC) or Lhermitte-Duclos Disease is a rare lesion (World Health Organization [WHO] grade I) characterized by thickened folia and replacement of the internal granular layer by abnormal ganglion cells. More commonly, the compromised patients are young adults presenting ataxia, seizures, obstructive hydrocephalus, and increased intracranial pressure. Dysplastic gangliocytoma of the cerebellum is intimately associated with Cowden syndrome, a hereditary disorder caused by a germline mutation in the PTEN tumor suppressor gene on chromosome 10q23. Large neurons of DCG show vesicular nuclei with prominent nucleoli. Expansion of the internal granular layer determines vacuolization of the molecular layer and white matter, which can be related to the bright stripes identified on T2-weighted magnetic resonance imaging. Herein, the authors report a female patient who developed long- time recurrence of DGC and discuss pathological findings and differential diagnosis of this rare cerebellar lesion.

Ganglioneuroma suprarrenal. Reporte de un caso / Adrenal ganglioneuroma. A case report / Ganglioneuroma suprarrenal. Relato de caso

Resumen: Introducción: el ganglioneuroma suprarrenal es un tumor benigno excepcional originado en las células nerviosas derivadas de la cresta neural. El objetivo de este trabajo es comunicar un caso clínico de un ganglioneuroma suprarrenal derecho sintomático. Caso clínico: paciente de 37 años, sexo femenino, que consultó por dolor abdominal inespecífico que luego de la valoración imagenológica y estudio funcional hormonal, se diagnosticó una tumoración suprarrenal derecha no funcionante. Con dicho diagnóstico se realizó la adrenalectomía por abordaje convencional retroperitoneal y el estudio anatomopatológico de la pieza diagnosticó un ganglioneuroma. Discusión: los ganglioneuromas habitualmente son asintomáticos y no funcionantes, por lo que su diagnóstico es incidental en un alto porcentaje de casos. Otros se presentan con una sintomatología inespecífica. Su diagnóstico etiológico preoperatorio raramente es realizado y su confirmación es anatomopatológica. Tienen indicación quirúrgica los sintomáticos, los que superan los 6 cm, o persisten dudas diagnósticas de malignidad. El pronóstico es bueno, siendo excepcional la recidiva.

Summary: Introduction: adrenal ganglioneuroma is an exceptional benign tumour which originates from neural crest cells. The study aims to inform about a clinical case of asymptomatic right adrenal ganglioneuroma. Clinical case: 37-year old patient, female, who consulted for non-specific abdominal pain which, after imaging assessment and functional and hormone test was diagnosed with non-functioning tumour of the right adrenal gland. Upon this diagnosis, adrenalectomy using the conventional retroperitoneal approach was performed, and the pathology study of the piece confirmed the diagnosis of ganglioneuroma. Discussion: ganglioneuromas are usually asymptomatic and non-functioning, so diagnosis is incidental in a large percentage of cases. Others present non-specific symptoms. Preoperative etiological diagnosis is rare, and confirmation is usually after pathology study. Symptomatic tumours over 6cm long have an indication of surgery, as well as those suspicious of malignancy. Prognosis is good, relapses being exceptional.

Resumo: Introdução: o ganglioneuroma suprarrenal é um tumor benigno excepcional originado nas células nervosas derivadas da crista neural. O objetivo deste trabalho é descrever o caso clínico de um ganglioneuroma suprarrenal direito sintomático. Caso clínico: paciente de 37 anos, sexo feminino que consultou por dor abdominal inespecífica que foi diagnosticado como uma tumoração suprarrenal direita não funcionante depois da avaliação de estudos de imagem e funcional hormonal. Com este diagnóstico realizou-se adrenalectomia por abordagem convencional retroperitoneal e o laudo anatomopatológico diagnosticou um ganglioneuroma. Discussão: os ganglioneuromas geralmente são assintomáticos e não funcionantes por isso seu diagnóstico é acidental em uma alta proporção dos casos. Outros se apresentam com sintomatologia inespecífica. O diagnóstico etiológico pré-operatório raramente é realizado e sua confirmação é feita pela anatomia patológica. Quando são sintomáticos, têm mais de 6 cm ou se persistem dúvidas sobre sua malignidade têm indicação cirúrgica. O prognóstico é bom, e a recidiva é excepcional.

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.

Retroperitoneoscopic resection of retroperitoneal nonadrenal ganglioneuromas: our technique and clinical outcomes

ABSTRACT Objective: To report our experience of retroperitoneoscopic technique in semi-lateral decubitus position for the retroperitoneal nonadrenal ganglioneuromas in 18 patients, and to evaluate its clinical outcomes. Materials and Methods: From January 2012 to May 2016, 18 patients with retroperitoneal nonadrenal ganglioneuromas underwent retroperitoneoscopic resection. With the patients in semi-lateral decubitus position, a 4-port retroperitoneal approach was used. Data were collected on the tumor size, tumor location, perioperative outcomes, pathology, and last-known disease status. We reviewed the operative videos to identify surgical tips and tricks. Results: All procedures were carried out successfully without converting to open surgery. The tumors had an average size of 5.2cm. The mean operative time was 86.5 min, with a mean estimated blood loss of 85.4mL. There were three patients suffering from intraoperative complications. Postoperatively, all patients achieved an uneventful recovery; the mean postoperative hospital stay was 5.5 days. The postoperative pathology revealed to be retroperitoneal ganglioneuromas. With a mean follow-up of 39.5 months, all patients were recurrence free. The review of the operative videos revealed several tips and tricks, including keeping peritoneum and posterior Gerota fascia intact to provide a favorable operative exposure of tumors, and placing the harmonic scalpel through different ports during tumor dissection. Conclusions: With the patient in semi-lateral decubitus position and a 4-port retroperitoneal approach, retroperitoneoscopic resection of retroperitoneal nonadrenal ganglioneuroma is a feasible, effective, and safe procedure. This approach has distinct advantages including direct access to the tumor, optimal exposure of tumor and less intraperitoneal interference.

Ganglioneuroma retroperitoneal abdomino-pélvico / Abdomino-pelvicretroperitoneal ganglioneuroma

Los ganglioneuromas son los tumores benignos, formados por células maduras de la cresta neural, más frecuentes del sistema nervioso simpático en adultos jóvenes. Se presenta el caso de un varón de 25 años con una lesión retroperitoneal extensa y exéresis parcial, con buena evolución durante 6 meses(AU)

Ganglioneuromas are the benign tumors from mature cells of the neural crest more often seen from the sympathetic system on young adults. We present a young male with and extent retroperitoneal tumor with partial removal with a good evolution for 6 month(AU)

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

Ganglioneuroma cervical: reporte de un caso / Cervical ganglioneuroma: a case report

Se presenta el caso clínico de un paciente, sexo femenino, 52 años de edad, que al examen clínico presentaba un aumento de volumen a nivel del ángulo mandibular derecho. Los estudios imagenológicos mostraron una extensa masa cervical paravertebral, encapsulada, de límites netos, sin evidencias de calcificaciones en el cuerpo de la lesión como tampoco compromiso del tejido óseo vecino, que se extiende desde la base de cráneo hasta la región de la cara interna del ángulo mandibular derecho. Se determinó la cirugía, como tratamiento, la cual presentó como complicaciones post operatorias, trastornos en la deglución y síndrome de Horner. Por último la biopsia post operatoria arrojó ganglioneuroma y linfonodos sin lesiones histológicas.

A case report of a patient, female, 52 years old, clinical examination showed a volume increase at the right mandibular angle. Imaging studies showed a large cervical paravertebral mass, encapsulated, net limits without evidence of calcification in the body of the lesion nor involvement of neighboring bone tissue, extending from the base of the skull to the region of the inner right mandibular angle. Surgery was determined as treatment, presenting, impaired swallowing and Horner syndrome as post-operative complications. Finally, the postoperative biopsy showed, ganglioneuroma and lymph nodes without histological lesions.

Retroperitoneal ganglioneuroma: a case report

Ganglioneuroma is a rare, benign, neurogenic tumor originating from the neural sheath and frequently localized in the retroperitoneum. Report of a new case. We report a case of a 36-year-old woman presenting isolated right lumbar pain. Computed tomography revealed a heterogeneous, right, retroperitoneal mass, 13 cm in diameter. Complete resection of the tumor and right nephrectomy were performed. Histology confirmed the diagnosis of ganglioneuroma. Follow-up was 10 months with no recurrence. Through this case, we discuss diagnostic, therapeutic and prognostic aspects of this disease

[Pelvic ganglioneuroma. Report of a case]

Ganglioneuroma is a rare benign tumor. It is the most mature of neurogenic tumors. We report a case of a pelvic ganglioneuroma diagnosed in 24-year-old pregnant women who presented with an urinary infection. Echographic examination suggested an ovarian mass. At surgical operation, the tumor was close to the sacrum. A total resection of the tumor was performed. Pathological examination proved it as a ganglioneuroma. Sixteen months later, the patient is free from disease

Lhermitte Duclos disease [dysplastic cerebellar gangliocytoma]

This case report describes an adult male presenting with ataxia. Dysplastic cerebellar gangliocytoma, the Lhermitte- Duclos disease, was diagnosed on neuroimaging. Diagnosis was confirmed on histopathology of surgically removed lesion. Patient underwent an uneventful recovery following operative treatment

Ganglioneuroma of the cervical spine

This case report describes a young lady who presented with complaints of neck pain and weakness of all four limbs for the last eight months. Magnetic Resonance Imaging [MRI] scan of cervical spine revealed dumbbell shaped extramedullary lesion at C-3 and C-4 level extending outside through neural foramen of C-3. She underwent extended cervical laminectomy from C-3 to C-7 level. Postoperatively power was gradually regained in all four limbs. Histopathology confirmed a case of ganglioneuroma

Ganglioneuroma del torax / Thorax ganglioneuroma

Presentamos el caso clínico de un ganglioneuroma mediastínico en un menor de 12 años de edad, estudiado en cardiología infantil y hematología infantil del Hospital Gustavo Fricke de Viña del Mar. La revisión bibliográfica permite corroborar la rareza de la lesión, la importancia de una buena imagenología para una aproximación diagnóstica y su buen pronóstico al ser extirpados en su totalidad

Ganglioneuroma de la región cervical C1-C3: informe de un caso / Ganglioneuroma of cervical C1-C3 cervical region: one case report

Se obtuvo la completa recuperación neurológica de un paciente cuadriparético, con la exéresis radical de un ganglioneuroma intrarraquideo de C1 a C3 mediante una laminectomía estándar. El ganglioneuroma es un tumor poco frecuente y de localización generalmente en la región torácica. La asociación de ganglioneuroma y neurofibroma puede encontrarse en la enfermedad de Von Recklinhausen. El pronóstico es favorable cuando se logra la completa resección del tumor, particularmente en lesiones bien encapsuladas.

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