Neurogenic tumors (sympathetic ganglion-deriving), radiographic manifestations of neurofibromatosis

Neurogenic tumors (from nerve sheath origin)Imaging findings

Bilingual caseParavertebral neurofibroma (NF-1)

【Bilingual case】Abdominal tumor complicated by NF-1

Neurogenic tumors (sympathetic ganglionic origin)

Neurogenic Neoplasms of the Sympathetic Ganglia

overview

Image representation:

X-rays:

CT：

differential diagnosis

Clinical manifestations

(left) 30-year-old female, right-sided gangliocytoma; PA chest radiograph shows a right paravertebral mass, causing enlargement of the 5th and 6th posterior costal spaces on the right posterior, and hilar overlapping signs
can be seen.

(Right) Lateral chest x-ray of the same patient shows a mass in the paravertebral
region.
Gangliocytomas are benign tumors, usually asymptomatic, more common in adolescents and young adults, and most are found
incidentally on imaging.

(left) 27-year-old man, paraganglioma
.
CECT shows a left paravertebral mass with a markedly strengthened longitudinal axis and at least 3 vertebral bodies
in the upper and lower ranges.

(Right) In the same patient, axial T2WI, T1WI+C and nuclide imaging showed T2 high-intensity soft tissue mass with empty blood vessels at the edges, which was significantly enhanced and MIBG was taken up.

Neurofibromatosis

Neurofibromatosis

Overview:

Best clues: multiple subcutaneous and intrathoracic neurofibromas

Differential diagnosis:

(Left) PA chest x-ray from neurofibromatosis type 1 (NF1) shows typical skeletal findings, with dysplasia and deformity
of the ribs.
Upper thoracic vertebra right kyphoscoliosis
.

(Right) In the same patient, there are extensive bullae in the upper lobes of both lungs, which are more severe
on the right side than on the left.
Approximately 20% of adults with NF1 have pulmonary manifestations
.

(Left) NF1 patients, a large number of neurofibromas (the largest paravertebral area) and multiple subcutaneous neurofibromas (curved arrows).

(Right) In the same patient, coronary T2WI shows a large number of neurofibromas
in the subcutaneous and deep soft tissues of the head and neck, upper extremities, and chest.
Most neurofibromas present as T2 "targets" with low central and peripheral high intensity
.

(Left) In patients with NF1, AP chest x-ray shows a right upper mediastinal soft-tissue mass
.

(Right) In the same patient, CECT shows a large, unreinforced watery density mass with an adjacent right foramina and spinal canal enlargement, suggesting thoracic paravertebral meningocele
.

(left) In patients with NF1, axial HRCT shows subpleural reticular opacities, bronchiectasis, and cellular lung
.

(Right) In NF1 patients, chest x-rays show a large mass (neurofibromas) on the outside of both lungs and a widened
mediastinum.
These findings may mimic
lymphadenopathy and other mediastinal masses on chest radiograph.

(Left) NF1 patient, a slightly lower-density mass in the left armpit
.

(Right) PET/CT shows massive uptake of FDG by mass, suggesting neurogenic tumors or malignant transformation of peripheral nerve sheath tumors (MPNST).

PET/CT is valuable
in detecting recurrent or metastatic disease in patients with known MPNST.