KING EDWARD MEMORIAL HOSPITAL

Seth Gordhandas Sunderdas Medical College

Acharya Donde Marg, Parel,
Mumbai 400 012. India.
Tel.: 91-22-2410 7000 Fax: 91-22-2414 3435

Case of the Month

 
Case of the Month
Case No. : 142
Month : December
Year : 2010
Contributor :
Dr. Kiran Sahane

Other Cases

CLINICAL PROFILE:

A three-year-old girl presented with dry cough since two months. she had been treated elsewhere for this with a diagnosis of a ‘pneumonia’. There were no other complaints. A chest radiograph was taken as initial step of evaluation.

RADIOLOGICAL FINDINGS:

Chest Radiograph:

Fig. 1

Chest radiograph revealed an approximately 8 x 5 cm size well-defined soft tissue opacity in right paraspinal region with a broad base towards the mediastinum. The right heart border was seen separately – suggesting a posterior mediastinum mass. There was widening of intercostal spaces in the region of opacity, suggestive of benign nature of posterior mediastinal mass. However there was no evidence of rib or vertebral body erosion.

Plain and contrast enhanced CT examination was performed to characterize the mass.

Computer Tomography Images:

Fig. 2.

Fig. 3.

Fig.4. (Postcontrast)

Fig. 5. (Postcontrast)

This showed a right sided posterior mediastinal mass that measured approximately 8 x 5 x 4 cm. It was well circumscribed. The mass arose in the paraspinal space. The surrounding structures appeared unaffected by the mass. The mass demonstrated mild heterogeneous contrast enhancement. There was no mediastinal lymphadenopathy. The differential diagnosis for these CT findings included a benign or malignant tumor of neural origin, sarcoma and lymphoma.

MR study was performed for further characterization and to assess intraspinal extension.

Magnetic Resonance Imaging:

Fig. 6 (T1WI)

Fig. 7 (T2WI)

Fig. 8 (sag)

Fig. 9 (cor)

T1WI reveals a hypointense mass in the right paraspinal region measuring approximately 8 x 5 x 4 cm. It is hyperintense on T2WI. There was no intraspinal extension. There was no invasion of surrounding structures. Differentials remain the same on MR imaging with most likely diagnosis being benign or malignant tumor of neurogenic origin.

POST RADIOLOGIC WORKUP:-

CT guided biopsy was performed to know the nature of tumour.

Fig. 10.

It reveals mature ganglion cells along with Schwann cells (mature stroma). There is no evidence of neuroblastic cells. There is no evidence of nuclear atypia or polymorphism. This is suggestive of ganglioneuroma.

MANAGEMENT:-

A right thoracotomy with complete resection of tumour was performed. The tumour was abutting the spine. However there was no invasion of surrounding structures. A well circumscribed lobulated mass was excised.

Fig 11

Fig 12

DISCUSSION:

Introduction:

Ganglioneuroma are benign tumours that originate from neural crest cell. They share common histogenic linage with neuroblastoma and ganglioneuroblastoma and represent the most benign and differentiated form of this group. A tumor composed primarily of neuroblasts is referred to as neuroblastoma, a tumor composed entirely of mature ganglion cells and other mature tissue is a ganglioneuroma, and a tumor with both immature and mature cell types is a ganglioneuroblastoma. Neuroblastoma and ganglioneuroblastoma may mature to Ganglioneuroma. Ganglioneuroma are also documented to have arisen in neuroblastomas that were treated with chemotherapy, and they may arise de novo.

Clinical Presentation:

Ganglioneuromas occurs most commonly in young patients and represents the most common tumour arising in posterior mediastinum in this group. They most often manifests as an asymptomatic mass discovered incidentally. Sometimes ganglioneuroma causes local mass effect and patients may present with cough, abdominal pain or dyspnea. In rare cases, Ganglioneuroma secretes sufficient quantities of VMA or HVA to manifest with flushing and other symptoms of catecholamine excess.

Radiological Features:

Although Ganglioneuroma tends to be relatively homogeneous, the imaging characteristics of Ganglioneuroma are similar to those of ganglioneuroblastoma and neuroblastoma; hence, they cannot be discriminated at imaging evaluation. However presence of metastasis excludes ganglioneuroma.

Plain radiographs typically show a posterior mediastinal mass, which may cause rib spreading and foraminal erosion. A mass may also be noted in the retroperitoneum, pelvis, or neck.

Ultrasonogrphy reveals a homogeneous, hypoechoic mass with well-defined borders.

Computer Tomography shows calcifications in approximately half of Ganglioneuromas. Calcification is typically fine and speckled but may be coarse. Ganglioneuromas are low attenuation and homogeneous on unenhanced CT scans and demonstrate slight to moderate enhancement, which may be heterogeneous or homogeneous.

At Magnetic Resonance Imaging, Ganglioneuromas have low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images, the appearance is presumed to be caused by a combination of myxoid material and relatively low amounts of ganglion cells. MR imaging enhancement varies from mild to marked. Delayed images may show increasing enhancement. MR imaging is the preferred modality for detection of intraspinal extension.

Histopathologic Features:

Ganglioneuromas may appear encapsulated, although a true capsule is infrequent. The tumors are firm, white to yellow in color, and may appear trabeculated or whorled.

Ganglioneuroma is composed of ganglion cells and mature schwann cells (mature stroma). Cellular atypia, mitotic activity, and necrosis are not features of ganglioneuroma. The presence of neuroblasts indicates that the tumor is a ganglioneuroblastoma or neuroblastoma and excludes Ganglioneuroma.

Treatment & Prognosis:

Treatment consists of complete surgical resection when possible. Complete resection ensures thorough sampling of the tumor, such that a confident diagnosis of GN can be made. Local recurrence has been reported, so periodic radiologic surveillance is performed after resection.

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