Definition/General

Introduction:
-Ganglioneuroma is a benign tumor of the sympathetic nervous system
-It represents the mature end of the neuroblastoma-ganglioneuroblastoma-ganglioneuroma spectrum
-It arises from neural crest cells
-FNAC shows mature ganglion cells and Schwannian stroma.
Origin:
-Arises from mature neural crest cells
-Composed of mature ganglion cells and Schwannian stroma
-Represents complete differentiation from primitive neuroblasts
-May arise de novo or from maturation of neuroblastoma.
Classification:
-Classified as mature neuroblastic tumor (WHO classification)
-100% mature ganglion cells and Schwannian stroma
-No primitive neuroblasts
-Benign behavior regardless of age
-Part of neuroblastoma tumor family.
Epidemiology:
-Peak incidence in older children and young adults
-Later age than neuroblastoma (median 7 years)
-No sex predilection
-Can occur at any age
-Excellent prognosis with complete excision.

Clinical Features

Presentation:
-Asymptomatic mass (most common)
-Slowly growing mass
-Posterior mediastinal mass (thoracic location)
-Abdominal mass (adrenal/retroperitoneal)
-Incidental finding on imaging
-Rarely functional.
Symptoms:
-Mass effect symptoms if large
-Respiratory symptoms (thoracic tumors)
-Abdominal discomfort (abdominal tumors)
-Neurological symptoms (spinal extension)
-No constitutional symptoms typically.

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Gross Description

Appearance:
-Well-encapsulated, firm mass
-Gray-white to tan cut surface
-Whorled pattern may be visible
-No hemorrhage or necrosis
-Size variable (few cm to >10 cm)
-Homogeneous appearance.
Characteristics:
-Firm, rubbery consistency
-Gray-white color predominant
-Fascicular pattern on cut surface
-No cystic change
-No calcification grossly
-Well-demarcated from adjacent tissue.
Size Location:
-Variable size (2-15 cm)
-Adrenal medulla (when adrenal primary)
-Posterior mediastinum (common)
-Retroperitoneum along sympathetic chain
-Neck (cervical sympathetic chain)
-Pelvis (presacral location).

Microscopic Description

Immunohistochemistry

Molecular/Genetic

Differential Diagnosis

Sample Pathology Report

Template Format

Sample Pathology Report

Complete Report: This is an example of how the final pathology report should be structured for this condition.

Clinical Information

[Age] year old [male/female] with [slowly growing mass/incidental finding] at [location]

Specimen Information

FNAC from [mass location], performed under [guidance method]

Specimen Adequacy

[Adequate/Inadequate] for cytological interpretation

Cytological Findings

Smears showing [mature ganglion cells] with [abundant cytoplasm] and [large vesicular nuclei]. [Schwannian stroma] elements present

Ganglion Cell Features

[Large polygonal cells/Abundant eosinophilic cytoplasm/Vesicular nuclei/Prominent nucleoli/Nissl substance] observed

Stromal Elements

[Schwannian stroma/Spindle cells/Fascicular pattern] noted in background

Background

Background shows [mature neural elements/no primitive cells/no necrosis]

Cytological Diagnosis

[Benign neural tumor] - Features consistent with ganglioneuroma

Differential Diagnosis

Differential includes [other mature neural tumors/neuroblastoma/paraganglioma]

Recommendations

[Histopathological correlation/Immunohistochemistry/Surgical consultation] as indicated

Note

Ganglioneuroma is benign tumor with excellent prognosis after complete excision