KEM - DEPARTMENTS
Home College Hospital Alumni Contact Departments Feedback
KEM LOGO



Radiology

Case of the Month


Case No. : 55
Month : July
Year : 2003
Contributor : Dr. Nadeem Ahmed.

Other Cases

Radiological Findings

CLINICAL PROFILE :

A 45-year-old man presented with progressive pain and weakness in the right upper limb over a period of four months. Neck movements were restricted specially on right lateral flexion. There were no sensory or bowel-bladder complaints. On examination, there was atrophy of the hand muscles on the right. Mild hypotonia with grade 3/5 power was present in the right upper limb. Reflexes were normal. The lower limbs were normal.

RADIOLOGICAL FINDINGS:

Plain cervical spine radiograph in frontal (Fig 1) and lateral (Fig 2) projections showed an expansile osteolytic lesion involving the right half of the vertebral body and pedicle of the C4 vertebra with well-defined margins and narrow zone of transition.

Fig.1
Fig.2
Fig.1
Fig.2

Plain and contrast enhanced CT scan of the cervical spine showed a right-sided mildly enhancing soft tissue lesion expanding the C4 neural foramen (Fig 3). There was expansion of the C4 vertebral body, pedicle and lamina (Fig 4).

Fig.3
Fig.4
Fig.3
Fig.4

MRI showed an extradural dumbbell shaped mass involving the right half of C4 vertebra. It was isointense on T1WIand homogeneously hyperintense on T2WI The mass extended from C3 to C5 levels and showed homogenous and brilliant enhancement.

Fig.5
Fig.6
Fig.5
Fig.6
Fig.7
Fig.7

There was mild effacement of anterior subarachnoid space with intrinsic abnormal hyperintense signal seen within the cervical cord suggesting cord edema / ischaemia.

On the basis of the enhancement characteristics and intervertebral foramen widening, the possibility of a schwannoma was entertained. However, with this degree of bone destruction other possibilities such as a giant cell tumourand aneurysmal bone cyst were considered in the differential diagnosis.

At surgery (posterior approach) a large fleshy tumour, separate from the meninges and the nerve roots was removed.

Gross pathological examination revealed grey-white glistening tissue. Histopathology of the resected mass showed areas of nuclear pallisading and dense areas of tumour cells alternating with loosely textured myxoid tissue. These findings were consistent with Antoni type A and Antoni type B tissue respectively. Immunohistochemical testing for S-100 protein was diffusely positive, whereas glial fibrillary acidic protein (GFAP) was negative confirming the diagnosis of schwannoma. There was no evidence of any malignant change within the resected tumour.

DIFFERENTIAL DIAGNOSIS:

A wide variety of benign and malignant neoplasms can involve the spine and cause expansion of the vertebra. These include giant cell tumour, osteoblastoma, aneurysmal bone cyst, solitary plasmacytoma and metastases. Certain spinal tumours involve one or more contiguous vertebral levels or present as an expansile lesions in a specific region of the spine providing an important clue to the diagnosis . Giant cell tumors in spine most commonly occur in sacrum . When GCT occurs in spine above the sacrum it usually affects the vertebral body. On conventional radiograph, spinal GCTs demonstrate osteolytic expansile lesions with no evidence of mineralisation. On CT, these tumors have soft tissue attenuation with well defined margins that may show a thin rim of sclerosis. On MRI, hypointensity with heterogeneous hyperintense foci is seen on T1WI and iso to hyperintense signals on T2WI. They commonly enhance and frequently show presence of blood degradation products. An osteoblastoma is fairly common in the spine and most frequently involves the posterior vertebral elements. Extension into the vertebral body is also common. The most common radiographic pattern is expansile lesion with multiple small calcifications and a peripheral sclerotic rim. On MRI it shows low to intermediate signal intensity on T1WI and intermediate to high signal intensity on T2WI . Aneurysmal bone cysts most commonly affect the thoracic spine .These lesions typically affect young patients with 80% being less than 20 years of age. Radiographs generally show marked expansile remodeling of bone centered in posterior elements, although extension into vertebral body frequently occurs . CT and MR imaging suggest the cystic nature of lesion and often show fluid - fluid levels within ABCs. On T1 and T2 -weighted images they have increased signal intensity. Solitary plasmacytoma occurs most commonly in thoracic spine . They usually predominate in vertebral body. In one third of cases,a multicystic soap-bubble appearance may be seen. At MR imaging, a solitary plasmacytoma of the bone typically exhibits high homogenous signal intensity on T2W images and homogenous marked enhancement post gadolinium images . Uncommonly, metastasis in cervical spine, from kidney and thyroid, can also present in this fashion.

In this case, with neural foramen enlargement with the dumbbell shape of the extradural tumor and uniform contrast enhancement, a schwannoma was thought as a possibility which was confirmed by histopathology of the resected specimen.

DISCUSSION:

Schwannomas and neurofibromas are the most common nerve sheath tumours found in the spine. These are benign tumours arising from the Schwann cell or perineural fibroblasts respectively. These tumours usually present as extramedullary intradural tumours (70-75%), less commonly both intra and extradural (15%), extradural (15%) and intramedullary (< 1%). The distribution of these is fairly uniform throughout the spine, with a slight lumbar predominance. Schwannomas are typically lobulated, encapsulated masses. Nerve fibers do not course through them. They have a variable clinical presentation which includes pain along the distribution of nerve and/ or localized mass. Often they are detected incidentally.


On MR imaging, nerve sheath tumors are isointense to hypointense on T1WI and hyperintense on T2WI. They show uniform and marked enhancement on contrast administration. Spinal schwannomas may undergo cystic degeneration. Cystic, hemorrhagic, or necrotic degeneration is seen as hyperintense and variably inhomogeneous central signal intensity on T2 WI. They rarely ever show calcifications.

Schwannomas can cause pressure erosion of the pedicle and the vertebral body resulting in enlarged foramen and scalloping of the vertebra. However extensive vertebral destruction is unusual for a schwannoma.

Three possible mechanisms have been postulated by which a nerve sheath tumour can involve bone:
1. An extra-osseous tumor causing secondary erosion of bone,
2. Tumor arising centrally within the bone (intra-osseous neurinomas),
3. Tumour arising in the nutrient canal and growing in a dumbbell- shaped configuration, resulting in enlargement of the canal

In conclusion extradural schwannoma should be included in the differential diagnosis of expansile vertebral lesions, especially with characteristic imaging findings like neural foramina enlargement and uniform enhancement. MR imaging with its multiplanar capability and excellent soft tissue differentiation is invaluable in offering the correct diagnosis.



Home | College | Hospital | Alumni | Contact | Departments | Search | Radiology