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.

Diffuse Ganglioneuromatosis of the Colon Presenting as a Large Subepithelial Tumor in Adults: Report of Two Cases / 대한소화기학회지

Colonic diffuse ganglioneuromatosis is a benign neoplastic condition characterized by disseminated, intramural, or transmural proliferation of neural elements involving the enteric plexuses, sometimes associated with von Recklinghausen's disease and other multiple tumor syndromes. Colonic diffuse ganglioneuromatosis is usually large, ranging from 1 to 17 cm, and thus can distort the surrounding tissue architecture as well as infiltrate the adjacent bowel wall. However, colonic diffuse ganglioneuromatosis is an exceptional finding in adults and only individual cases are reported in the literature. Herein, we report two unusual cases of adult patients with colonic diffuse transmural ganglioneuromatosis presenting as a large subepithelial tumor.

Composite pheochromocytoma-ganglioneuroma of the adrenal gland: a case report with immunohistochemical study

Composite tumors of the adrenal medulla consisting of pheochromocytoma and ganglioneuroma are rare tumors accounting for less than 3% of all sympathoadrenal tumors. These tumors display more than one line of differentiation in which normal and neoplastic chromaffin cells are capable of differentiating into ganglion cells under the influence of nerve growth factors. To the best of our knowledge, we report the second case with a composite tumor of the adrenal medulla in a normotensive patient from India

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.

Ganglioneuroma of the adrenal gland: a rare case.

Ganglioneuromas, which are benign tumors of sympathetic nervous system, are rare to arise in adrenal gland. They are usually clinically silent and detected during work-up for other unrelated conditions. Currently, histopathology is the only tool to diagnose ganglioneuroma and to differentiate it from ganglioneuroblastoma and neuroblastoma. We discuss the case of a 40-year-old female found to have a right adrenal mass during investigation of dull ache in right upper abdomen. Clinico-radiological assessment was non-confirmatory on the nature of the mass, and excision was done. Histological examination showed the mass to be a ganglioneuroma arising in the adrenal gland. In conclusion, ganglioneuroma occurs rarely in adrenal gland and pre-operative diagnosis is difficult since the symptoms are usually non-specific. Histological examination is the mainstay of diagnosis and should be thorough to exclude neuroblastomatous foci, portending a worse outcome.

Retroperitoneal ganglioneuroma with lymphnode metastasis: a case report.

Ganglioneuroma is a benign, most differentiated tumor of neural crest origin. These tumors though benign, can very rarely metastasize to regional lymphnodes or to distant sites. We report a case of a 52 year old lady who presented to the surgical OPD with dyspeptic symptoms of 2 months duration. Ultrasonography and CT scan of the abdomen showed a homogenous mass in the region of the right adrenal gland, extending between the inferior vena cava and the aorta. A clinical and radiological diagnosis of an adrenal tumor was made. Microscopically, the tumor histology was that of a ganglioneuroma (schwannian stroma-dominant) mature type. The lymph node attached to the tumor showed metastasis, the metastatic foci having the same morphology as the primary tumor. The right adrenal gland was free of tumor. The case is illustrative due to its clinical presentation mimicking an adrenal incidentaloma, and a rare phenomenon of a benign tumor exhibiting completely differentiated lymph node metastasis.

Ganglioneuroma of pelvis--an unique presentation in a young man.

Ganglioneuromas presented as a pelvic tumor around the pelvic organs is a rare entity. A case with unusual presentation is reported. Young man of 18 years old presented with a complaint of lower abdominal mass increasing in size for last 3 years. It was treated with partial resection for debulking purpose after the conformation during surgery with frozen section. Debulking surgery with preservation of organ functions is feasible in these slow growing tumors for better quality of life.

A Case of a Composite Adrenal Medullary Tumor of Pheochromocytoma and Ganglioneuroma Masquerading as Acute Pancreatitis

Composite adrenal medullary tumors, composed of both pheochromocytoma and ganglioneuroma, are extremely rare, as are pheochromocytomas masquerading as acute relapsing pancreatitis. We recently experienced a case of a 48-year-old male with both these phenomena. The patient complained of an acute onset of intense abdominal discomfort. At the same time, pancreatic enzymes were increased in concentration. An abdominal computed tomographic scan revealed an enlarged pancreas and a 3-cm left adrenal incidentaloma. Biochemical and 131I-MIBG scintigraphic findings were compatible with a pheochromocytoma. Yet, he had no clinical manifestations suggesting pheochromocytoma. An adrenalectomy was performed and a composite adrenal medullary tumor of pheochromocytoma and ganglioneuroma was confirmed during a pathologic examination. This case illustrates two points: 1) acute abdominal intense discomfort and hyperamylasemia may be unusual presentations of pheochromocytomas; and 2) the possibility of the pheochromocytoma, albeit rare, should be considered when a relapsing pancreatitis of uncertain etiology develops.

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

Ampullary ganglioneuroma: an unusual feature of neurofibromatosis type 1 - A case report

The case of a 65 year old man is presented. He had a history of dyspepsia and was diagnosed as neurofibromatosis clinically. Upper G.I. endoscopy showed thickened folds in duodenum. A polyp was seen at the junction of D1 and D2 adjacent to the ampulla. Duodenal biopsy results were suggestive of ganglioneuroma

Dysplastic gangliocytoma of the cerebellum

The authors report a case of Lhermitte-Duclos disease [Dysplastic gangliocytoma of the cererbellum] in a child, revealed by symptoms of raised intracranial pressure and diagnosed preoperatively by magnetic resonance imaging. The diagnosis of Lhermitte-Duclos disease was confirmed by histological study of the surgical specimen. Clinical imaging and pathological features of this rare entity are discussed, the literature regarding this disease is also reviewed

Ganglioneuroma of small intestine presenting with perforation peritonitis.

We report a 42-year-old man with benign solitary small intestinal ganglioneuroma presenting with perforation peritonitis. The patient had no evidence of MEN IIB syndrome. Simple segmental resection was done; the patient is well on follow up one year later.

Gangliocytoma Mimicking Extra-axial Tumor: A Report of Two Cases

We report two cases of supratentorial gangliocytomas mimicking an extra-axial tumor. MR imaging indicated that the tumors were extra-axial, and meningiomas were thus initially diagnosed. Relative to gray matter, the tumors were hypointense on T1-weighted images and hyperintense on T2-weighted images. On contrast-enhanced T1-weighted images, homogeneous enhancement was observed, while CT scanning revealed calcification in one of the two cases.

Enfermedad de Lhermitte Duclos: reporte de un caso y revisión de la literatura / Duclos Lhermite disease: report of one case and literature review

La enfermedad de Lhermitte Duclos o gangliocitoma displásico del cerebelo es una neoplasia infrecuente, asociada a Síndrome de Cowden, que ocurre principalmente en pacientes jovenes. Reportamos el caso de un varón de 31 años que presenta esta rara enfermedad, asociado a hamartomas de colon, que fue operado y controlado hasta la actualidad sin que presente evidencia de recidiva. Siendo una patología poco común, se hace una revisión de la literatura sobre esta enfermedad.