N.U. Bahri, MD

Case History: A 55-year-old female presents with pain in cervical region radiating to bilateral upper limbs (right > left) for one year; also associated with tingling and numbness in both upper limbs.

Slideshows

Case History: 55-year-old female with pain in cervical region radiating to bilateral upper limbs.

Cervical Pain, Numb Upper Limbs

Case History: 7-year-old female with complaints of convulsions for one year. Last attack was six months prior, after which the patient was put on regular anticonvulsive therapy and has shown improvement.

Case History: 7-year-old female with complaints of convulsions for one year. 

Convulsions

Case History: Two-year-old female born from nonconsanguineous marriage presented with cough with expectoration and high-grade fever for six days.

Case History: Two-year-old female with cough with expectoration and high-grade fever.

Cough, Fever

Case History: A 21-year-old female presented with the complaints of swelling of right shoulder for one year.

Case History: 21-year-old female presented with the complaints of swelling of right shoulder for one year.

Swollen Shoulder

 2-year-old female present with complaints of swelling of vulva, gluteal region, and left thigh since birth which gradually increased in size. No evidence of discoloration of skin or varicosity is seen and non-tender.

 Congenital vascular malformation, lymphangioma of upper thigh and vulva.

External appearance of child showing hypertrophy of left thigh and vulva. No e/o of skin discoloration or varicosity is noted.

X-ray left thigh (AP view) showing increased soft tissue shadow involving left thigh, however bones and joint undervesion appear normal. No e/o lytic and sclerotic lesion is noted.

. Ultrasonography of local part showing fluid filled nonvascular cystic areas within the subcutaneous plane, no evidence of muscular involvement is noted.

Coronal T2WI of hip and both thighs showing increased signal intensity in soft tissue involving subcutaneous tissue of left thigh, gluteal region, both inguinal regions, and vulva. Bones and muscle show normal signal intensity.

Coronal T1FS postcontrast images showing heterogenous enhancement of internal septas, no evidence of pooling of blood in subcutaneous cystic spaces is noted.

: A large soft tissue shadow is noted in the left thigh and perineal region. No evidence of calcification is seen. Bones and joint under vision appears normal.

: Large altered signal intensity (hyperintense on T2WI, STIR, and PD FS images, hypointense on T1WI). Soft tissue lesion in labia majora, labia minora, subcutaneous plane of perineal region, both inguinal regions, lower part of anterolateral abdominal wall, anterolateral part of left gluteal region, and whole circumference of left thigh up to the knee.

The lesion shows heterogenous postcontrast enhancement of internal septas. No e/o pooling of contrast in the above mentioned soft tissue lesions is noted. Underlying muscles and bones show normal signal intensity. S/o congenital vascular malformation possibly lymphangioma.


Soft-tissue lymphangiomas are relatively rare tumors that may involve almost any organ system. Approximately 90% of lymphangiomas are diagnosed in children younger than age 2 and lymphangiomas account for only 6% of benign childhood tumors. Although the specific origin is unclear, most experts consider lymphangiomas to be true congenital neoplasms resulting from erroneous embryogenesis. Soft-tissue lymphangiomas most commonly occur in cervicofacial (up to 75%), axillary (up to 25%), or mediastinal (3%-10%) areas. Patients usually present with a soft, fluctuant mass that has progressively increased in size. Rapid enlargement of the lesion may be seen when there is associated bleeding or infection. Histologically, lymphangiomas consist of ectatic lymphatic channels, occasionally containing a mild lymphocytic infiltrate. Classification by histologic appearance includes three subtypes: capillary, cavernous, and cystic. The capillary form is composed of small thin-walled lymphatic vessels. Cavernous lymphangioma consists of large lymphatic channels with adventitial coats. The third and most common type, cystic lymphangioma or cystic hygroma, is composed of macroscopic lymphatic spaces. These lesions are often multiseptated, which is suggestive of infiltration across tissue planes. The different classifications of this lesion may represent various stages of disease. In clinical practice, vascular malformations are classified as high-flow or low-flow lesions. Those containing arterial components are considered high-flow lesions. Capillary, venous, and lymphatic malformations and their combinations are considered to be low-flow lesions.

Radiography typically shows nonspecific findings. Sonography generally shows excellent through transmission because of the cystic nature of this process. Other sonographic findings may include multiple septations of varying thickness and fluid-fluid levels that represent layering of the hemorrhagic components of the lesion. Sonography may help in differentiating a lymphangioma from a hemangioma, as there generally is no significant Doppler flow in a lymphangioma. CT findings are often nonspecific but can demonstrate the cystic nature of lymphangiomas. MRI is superior to other techniques for defining soft tissues and is useful for further characterization of the composition and extent of soft-tissue lymphangiomas. Lymphangiomas characteristically appear as heterogeneous, fluid-filled masses with low signal intensity (isointense to muscle) on T1-weighted images and show high signal intensity (greater than fat) on T2-weighted images. These fluid-containing spaces correspond to the histologic findings of ectatic lymphatic channels. Additional findings may include fibrous septations seen as linear hypointensities on T2-weighted images, fluid-fluid levels representing hemorrhage, and aneurysmal dilatation of adjacent venous structures. Neither cystic enhancement nor mild septal enhancement is seen in true lymphatic malformations. However, enhancement may be seen in mixed lymphatic-venous lesions.


1. Carpenter CT, Pitcher JD, Davis BJ, Gomez R, Scholfield TD, Youngberg RA. Cystic hygroma of the arm: a case report and review of the literature. 

ol. 1996; 25:201-204.
2. Rautio R, Keski-Nisula L, Laranne J, Laasonen E. Treatment of lymphangiomas with OK-432 (Picibanil). 

l. 2003; 26:31-36.
3. Van Cauwelaert P, Gruwez JA. Experience with lymphangioma. 

 1978;11:43-48.
4. Lev S, Lev MH. Imaging of cystic lesions. Radiol Clin North Am. 2000;38:1013-1027.
5. Siegel MJ. Magnetic resonance imaging of musculoskeletal soft tissue masses. 

. 2001;39:701-720.
6. Brown JV, Stenchever MA.Cavernous lymphangioma of the vulva. 

. 1989;73:877-879.
7. Virgili A, Corazza M. Lymphangioma and lymphangiectasis of the vulva. 

. 1997; 8: 229-232.
8. Peachey RD, Lim CC, Whimster LW. Lymphangioma of the skin: a review of 65 cases. 

 1970;83:519-527.
7. Akimoto K, Nogita T, Kawashima M. A case of acquired lymphangioma of the vulva. 

. 1993; 20:449-451.
8. Handfield-Jones SE, Prendiville WJ, Norman S. Vulval lymphangiectasia. 

. 1989;65:335-337.
9. Sood M, Mandal AK, Ganesh K. Lymphangioma circumscriptum of the vulva. 

 1991; 89:262-263.
10. Fisher I, Orkin M. Acquired lymphangioma (lymphangiectasis). Report of a case. Arch Dermatol. 1970; 101:230-234.
11. Vlastos AT, Malpica A, Follen M. Lymphangioma circumscriptum of the vulva: a review of the literature. 

. 2003;101: 946-954.
12. Whimster IW. The pathology of lymphangioma circumscriptum. 

. 1976;94: 73-486.
13. Mehta V, Nayak S, Balachandran C, Monga P, Rao R. Extensive congenital vulvar lymphangioma mimicking genital warts. 

. 2010;55:121-122.
14. Wong R, Melnyk M, Tang SS, Nguan C. Scrotal lymphangiomatosis: a case report. 

Articles

Case History: 2-year-old female with complaints of swelling of vulva, gluteal region, and left thigh since birth.

Rare Case of Lymphangioma

 A 75-year-old male with complaints of occasional headache for last two years. Laboratory examination, ophthalmic examination and family history were unremarkable. Neurophysician sent patient for routine brain MRI.

 T2WI axial and sagittal images show a small 12x8 mm well-defined, well-circumscribed heterogenous signal intensity lesion to adjacent grey matter with tiny focal area of CSF intensity in left lateral ventricle along inter ventricular septum in the region of foramen of monro (arrow).

 FLAIR axial and coronal images show a small well-defined, well-circumscribed hyperintense signal intensity lesion to adjacent grey matter in left lateral ventricle along inter ventricular septum in the region of foramen of monro (arrow).

 T1W axial image shows isointense signal intensity lesion to adjacent grey matter with focal cystic area in left lateral ventricle along inter ventricular septum in the region of foramen of monro.

 Diffusion weighted axial image shows no restricted diffusion within the lesion (arrow).

 T1 weighted FAT SAT axial imges show minimal to mild enhancement on post contrst study within lesion.

On MRI, a small 12x8 mm well-defined, well-circumscribed lesion was noted in the left lateral ventricle along inter ventricular septum in the region of foramen of monro. It showed heterogenous signal with adjacent grey matter on T2WI (Figure 1) with a tiny focal cystic area, which followed CSF signal, hyperintense signal on FLAIR weighted images (Figure 2) and isointense to white matter on T1WI (Figure 3) and did not show restricted diffusion on diffusion weighted images (Figure 4). No susceptibility artifact was noted. On post contrast study, the lesion showed minimal to mild enhancement (Figure 5). No evidence of obstructive hydrocephalus was seen.

: Subependymoma are uncommon, benign (WHO grade I) tumors, which are slow growing and non-invasive. Subependymoma tend to present in middle-aged to older individuals. There is a slight male predilection (M: F 2.3:1). Typically, patients are asymptomatic and small lesions are discovered incidentally. In some cases, especially when the tumors are larger, presentation is with symptoms of raised intracranial pressure due to obstructive hydrocephalus.


CT shows isodense intraventricular mass compared to adjacent brain, which does not usually enhance. Surrounding vasogenic edema is usually absent. MRI-T1 iso shows hypointense to white matter, T2-heterogenous to adjacent white and grey matter, with susceptibility related signal drop out due to calcifications occasionally seen no adjacent parenchymal edema. On post contrast-T1 fat sat images there is usually no enhancement, although at times may demonstrate mild enhancement


1. Ragel BT, Osborn AG, Whang K, et al. Subependymomas: an analysis of clinical and imaging features. 

. 2006;58: 881–890.
2. Russell JH, Gaillard F, Drummond KJ. A mass in the fourth ventricle. 

. 2009;16: 425,482.
3. Koral K, Kedzierski RM, Gimi B, et al. Subependymoma of the cerebellopontine angle and prepontine cistern in a 15-year-old adolescent boy. 

. 2008;29:190-191.
4. Tonn J, Westphal M, Rutka JT. Oncology of CNS Tumors. Springer Verlag. 2009.
5. Keating RF, Goodrich JT, Packer RJ. Tumors of the pediatric central nervous system. George Thieme Verlag. 2001.
6. Smith A, Smirniotopoulos J, Horkanyne-Szakaly I. From the Radiologic Pathology Archives: Intraventricular Neoplasms: Radiologic-Pathologic Correlation. 

Case History: 75-year-old male with complaint of occasional headaches for last two years.

Subependymoma of Lateral Ventricle

 A 75-year-old male presented with complaint of mucus discharge from an external opening on skin at anterior aspect of mid part of neck on left side. No history of trauma or surgery and unremarkable family history. Upon examination, there was an external opening on skin at anterior aspect of mid part of neck on the left side. Naked eye examination of the oropharynx did not reveal any visible opening at posterior to tonsillar pillar.

The patient was subjected to radiologic investigations. A fistulogram was performed but did not reveal internal opening. Further, on contrast CT, observed an elongated fusiform-shaped approximately 8-10 cm long, well-defined, smoothly marginated thin-walled, peripherally rim enhancing cystic lesion extending from the anterior surface of the mid part of the neck on the left side up to lateral wall of hypopharynx at the hypoid bone level. It had an external opening on the anterior surface of the mid part of the neck on the left side (Figure 1A). It lies anterior to carotid vessels and lateral to thyrohyoid membrane and antero-medial to sternocleidomastoid muscle (Figure 1B). Cranially, it terminated in the lateral wall of left pyriform fossa at hyoid level. It did not open into posterior to tonsillar pillar, so it ruled out second branchial fistula (Figure 2–4). On the basis of these radiological findings, final diagnosis was made of complete fistula of third branchial cleft. The patient was operated on and complete excision of the cord-like fistulous tract was done, which followed up to the lateral wall of left pyriform fossa. The excised tract measured 9 cm in length. It was found to lie over the carotid sheath and was not going up to tonsillar pillar or in between the internal and external carotid arteries, so it ruled out second branchial cleft fistula which is most common in literature (Figure 5).

 Contrast CT axial images at thyroid gland level (A) show external opening of cystic lesion in anterior surface of the mid part of the neck on the left side (small arrow). In B, at thyroid cartilage level, the cystic lesion (large arrow) lies anterior to carotid vessels and lateral to thyrohyoid membrane and antero-medial to sternocleidomastoid muscle.

 Contrast CT axial images of upper neck at hyoid bone level show a well-defined smoothly marginated peripherally rim enhancing cystic lesion (large arrow in A) opens in to lateral wall of left pyriform fossa (B, small arrow). It does not open into posterior to tonsillar pillar, so it ruled out possibility of second branchial cleft fistula.

 Contrast CT sagittal images of the neck show an elongated fusiform-shaped long well-defined smoothly marginated peripherally rim enhancing cystic lesion extending from anterior surface of the middle part of the neck (long arrow in A) and opens in to lateral wall of left pyriform fossa (small arrow in B).

 Contrast CT coronal images of the neck show an elongated fusiform shaped long well-defined smoothly marginated peripherally rim enhancing cystic lesion extending from anterior surface of the middle part of the neck and opens in to lateral wall of left pyriform fossa.

 Postoperative specimen. Complete excision of cord-like tract was performed, which followed up to the lateral wall of left pyriform fossa. The excised tract measured 9 cm in length. It was found to lie over the carotid sheath and was not going up to the tonsillar pillar or in between the internal and external carotid arteries, so it ruled out second branchial cleft fistula which is most common in literature.

: Complete fistula of third branchial cleft

 Third branchial cleft cysts are rare. A third branchial fistula extends from the same skin location as a second branchial fistula; however, a third branchial fistula pierces the thyrohyoid membrane to enter the larynx, terminating on the lateral aspect of the pyriform sinus. Third branchial cleft cysts occur anywhere along that course, but they are characteristically located deep to the sternocleidomastoid muscle. The external opening occurs about two-thirds of the way down the SCM anteriorly, similar to second branchial cleft cysts. The tract ascends along the carotid sheath posteriorly to the internal carotid artery, under the glossopharyngeal nerve, and over the vagus nerve and hypoglossal nerve to open into the pyriform sinus. Second branchial cleft anomalies are most common. The majority of these have an external opening in the neck along the anterior border of sternocleidomastoid at the junction of the upper two-thirds with the lower one-third and the internal opening extends up to the posterior pillar of the tonsil. In our case, the fistulous tract had external opening in the mid part of the neck along the sternocleidomastoid muscle and terminated in the lateral wall of pyriform fossa.

 CT - rounded, sharply circumscribed, fluid density centrally, thin wall. Ultrasound - sharply demarcated, posterior acoustic enhancement, imperceptible walls. MRI-T1 - variable signal dependant on protein content, high protein content; high signal, low protein content: low signal, T2 - usually high signal, T1 C+ (Gd) - no enhancement in uncomplicated lesions.

 Paramedian thyroglossal duct cyst, cystic lymph nodes, necrotic nodal metastases, esp. SCC and papillary thyroid cancer, tuberculous adenitis.


1. Koch BL. Cystic malformations of the neck in children. 

. 2005;35:463-477.
2. Goff CJ, Allred C, Glade RS. Current management of congenital branchial cleft cysts, sinuses, and fistulae. 

. 2012;20:533-539.
3. Zaifullah S, Yunus MR, See GB. Diagnosis and treatment of branchial cleft anomalies in UKMMC: a 10-year retrospective study. 

. 2012.
4. Guldfred LA, Philipsen BB, Siim C. Branchial cleft anomalies: accuracy of pre-operative diagnosis, clinical presentation and management.

. 2012;126:598-604.
5. Lanham PD, Wushensky C. Second brachial cleft cyst mimic: case report. AJNR 

. 2005;26:1862-1864.
6. Harnsberger HR, Mancuso AA, Muraki AS, et al. Branchial cleft anomalies and their mimics: computed tomographic evaluation. 

. 1984;152:739-748.
7. Ahuja AT, King AD, Metreweli C. Second branchial cleft cysts: variability of sonographic appearances in adult cases. 

Case History: 75-year-old male presents with complaint of mucus discharge from external opening on skin at anterior aspect of mid part of neck on the left side.

Complete Fistula of Third Branchial Cleft

A 58-year-old patient presented with complaint of dyspnoea for eight months. No history of fever and laboratory examination, and individual and family history were unremarkable. On chest X-ray (frontal and lateral view), a large well-defined, smooth marginated homogenous opacity was seen in posterior mediastinum with obliteration of right ascending aorta border (Figure 1). Further chest CT was performed, which showed two large saccular aneurysms arising from ascending aorta and arch of aorta with mural thrombus and peripheral calcification (Figure 2). Aneurysmal segment of aorta causes mass effect in form of compression of adjacent vascular structures and compression over trachea and oesophagus and causing posterior displacement of them (Figure 3). There was no evidence of contrast leakage or impending rupture of aorta. Diffuse changes of atherosclerosis were also noted in visible aorta.

: Chest radiograph (A, frontal view and B, lateral view) shows a large, well-defined, smooth marginated homogenous opacity in posterior mediastinum with obliteration of right ascending aorta border. The lesion is forming an obtuse angle with right lung parenchyma.

: Contrast CT angiography shows two large saccular aneurysms (large and small arrows) arising from ascending aorta and arch of aorta with mural thrombus and peripheral calcification in its wall. There is no evidence of contrast leakage or impending rupture of aorta. Diffuse changes of atherosclerosis in visible aorta.

: Contrast CT angiography shows aneurysmal segment of aorta causes mass effect in form of compression of adjacent vascular structures (large arrow) and compression over trachea (small arrow) and oesophagus and causing posterior displacement of them. There is no evidence of contrast leakage or impending rupture of aorta. Diffuse changes of atherosclerosis in visible aorta.

Aneurysm of ascending aorta and arch of aorta

Aneurysms in thoracic aorta are relatively uncommon compared to their counterparts below the diaphragm. Majority of thoracic aortic aneurysms are atherosclerotic. Symptomatic presentation may be due to mass effect on airway of oesophagus. Alternatively, they may present due to a complication, including rupture, aorto-bronchial or aorto-oesophageal fistulae. Thoracic aortic aneurysms can be divided pathologically according to their relationship to the aortic wall: o True aneurysm-atherosclerotic aneurysms (most common), inflammatory/aortitis
• Rheumatoid arthritis (RA), ankylosing spondylitis, takayasu arteritis, giant cell arteritis (GCA), syphiliss
• Structural: cystic medial necrosis, Marfan disease, Ehlers-Danlos syndrome, false aneurysms
• Traumas
• Mycotic aneurysms

Radiographic features: The location and shape of thoracic aortic aneurysms is variable. Aneurysms elsewhere can be described as saccular or fusiform. In the case of fusiform dilatation, the term aneurysm should be applied when the diameter is greater than 4 cm.

Plain film: The thoracic aorta can usually be seen on both frontal and lateral chest radiographs, and aneurysms are often obvious. Additionally, mediastinal masses may mimic aortic aneurysms. Mural calcification is seen both in atherosclerotic disease as well as various causes of aortitis.

CT: Typically aneurysms appear as dilatations of the lumen. The walls may be thin or thickened by presence of mural thrombus (circumferential or more frequently eccentric). Calcified atherosclerotic disease is often identified not only in the wall of the aneurysm but in adjacent arteries. If rupture or leak has occurred haematoma/fluid may be seen adjacent to the aorta. MRI has the advantage of not requiring ionizing radiation or large volumes of iodinated contrast.

 This is particularly advantageous in young patients with connective tissue disorders.

Digital subtraction angiography (DSA): Although angiography has long been considered the gold standard for vascular imaging, it has largely been superseded by CTA and MRA, which are able to obtain 3D volumetric data, and able to assess the extra luminal soft tissues. Angiography is however used during endovascular repair.

Complications

• Rupture
• Distal embolization
• Fistula formation
• Aorto-oesophageal fistula
• Aorto-bronchial fistula Differential diagnoses

• Aortic dissection
• Penetrating atherosclerotic ulcer
• Intramural aortic haematoma
• Kommerell diverticulum

References
1. Collins J, Stern EJ. Chest radiology, the essentials. Lippincott Williams & Wilkins. 2007.
2. Atar E, Belenky A, Hadad M, Ranany E, Baytner S, Bachar GN. MR angiography for abdominal and thoracic aortic aneurysms: assessment before endovascular repair in patients with impaired renal function. 

 2006;186:386-393.
3. Masuda Y, Takanashi K, Takasu J, Morooka N, Inagaki Y. Expansion rate of thoracic aortic aneurysms and influencing factors. Chest 1992;102: 461-466.
4. Kato N, Dake MD, Miller DC, Semba CP, Mitchell RS, Razavi MK, Kee ST. Traumatic thoracic aortic aneurysm: treatment with endovascular stent-grafts. Radiology 1997;205:657-662.
5. Reeder MM, Felson B. Reeder and Felson's gamuts in radiology, comprehensive lists of roentgen differential diagnosis. Springer Verlag. 2003.
6. Agarwal P, Chughtai A, Matzinger F, Kazerooni EA. Multidetector CT of thoracic aortic aneurysms1. 

Case History: 58-year-old patient with dyspnoea for eight months. No history of fever. Lab exam and history were unremarkable.

Aneurysm of Ascending Aorta and Arch of Aorta

 A 45-year-old female patient was referred to our hospital with painless swelling on left side of neck. She had been aware of the swelling for the last eight months which was gradually increasing in size. Initial examination revealed an evident swelling with smooth contour on left side of neck. No sign of inflammation of overlying skin was noted. On palpation, it was non-pulsatile, soft to firm in consistency and was not adherent to overlying skin. The swelling was not tender. Clinically it was suspected to be an enlarged lymph node. Cytological examination was non-conclusive. The patient was then referred to radiology department for CT scan.

Non contrast CT images demonstrated an oblong lesion in left carotid space with well-defined boundaries and a homogenous low density (average 16 HU) (Figure 1).

 Non-contrast axial CT image: fluid ttenuation lesion in left carotid space (blue arrow)

On post contrast CT, the lesion did not show any significant enhancement (Figure 2).

 Contrast axial CT image: Non enhancing lesion in left carotid space (blue arrow).

The lesion appears to displace common carotid and its external and internal branches medially and internal jugular vein laterally (Figure 3).

 Contrast axial CT image: non-enhancing lesion causing separation of common carotid artery and internal jugular vein (red arrows).

Because of the location, characteristic displacement of cervical major vessels, provisional diagnosis of peripheral nerve sheath tumor of cervical vagus nerve was considered. However, because of the non-enhancing nature of the lesion, possibility of 2nd branchial cleft cyst was also considered as a differential diagnosis. So, ultrasound examination with colour doppler study was performed for further evaluation.

On ultrasound, the lesion showed solid appearing peripheral hypoechogenicity and central hyperechogencity (Figure 4).

Solid heterogeneously hypoechoic lesion on left side of neck. Vagus nerve was not clearly delineated.

On color Doppler study, presence of internal vascularity was noted within (Figure 5). The lesion showed displacement of carotid arteries medially and internal jugular vein laterally.

 Color Doppler imaging: Significant internal vascularity was noted.

To further characterise this lesion, MRI was done.

T2WI and STIR images showed homogenous hyperintensity (Figure 6 &7).

 Axial T2WI: Hyperintense lesion in left carotid space (blue arrow).

 Coronal Short Tau Inversion Recovery Image: Homogenonously hyperintense lesion (blue arrow)

T1WI showed homogenous hypointensity (Figure 8).

 Axial T1WI:Homogenously hypointense lesion (blue arrow)

So, further contrast MRI was performed. On contrast studies, the lesion showed heterogenous and progressive delayed enhancement (Figure 9 and 10).

 Contrast axial image: (early phase); Early mild heterogenous enhancement is noted. (blue arrow)

 Contrast axial image: (delayed); Progressive delayed enhancement is noted. (blue arrow)

 Wide local excision was done under general anaesthesia. The lesion was well encapsulated and was easily separated from vagus nerve (nerve of origin). No post-operative Horner’s syndrome was noted. Gross specimen appeared well-circumscribed, firm rubbery mass.

 Intraoperative photograph of the lesion following excision from the parent nerve.

 Haematoxylin and eosin stained section shows neural and fibrous elements intermingled in loose stroma. Findings were suggestive of neurofibroma.

 Peripheral Nerve Sheath Tumor of Cervical Vagus Nerve

In our case, the lesion simulated Type II Branchial cyst on plain and contrast enhanced CT scan and non-contrast MRI study. However, ultrasound played an important role which clearly depicted it as a solid lesion with internal vascularity. Contrast MRI study further confirmed the diagnosis of peripheral nerve sheath tumor of cervical part of vagus nerve.

Neurogenic tumors can be divided into three groups according to their tissue of origin:[3]
• Peripheral nerve: (schwannoma, neurofibroma, malignant schwannoma)
• Sympathetic ganglia: (ganglioneuroma, ganglioneuroblastoma, neuroblastoma)
• Paraganglia: (chemodectoma, pheochromocytoma)

25 – 45% of nerve sheath tumors occur in head and neck region.In the neck region, these tumors may arise from vagus nerve, sympathetic chain or cervical nerve roots including the brachial plexus. [1]

The vagus nerve exits the posterior cranial fossa through the jugular foramen; before entering the thorax, it runs its entire course in the neck within the carotid sheath. Tumors arising from this cervical segment of the vagus nerve are rare and are often accidentally detected at surgery, with most being asymptomatic [5,6]

Neurofibromas arise either from nerves of dermis, subcutaneous tissue or other deeper tissues. They are most commonly seen in patients between the ages of 20 and 40 years. Most neurofibromas are slowly growing painless masses causing no particular symptoms until they reach a considerable size. The nerve of origin is usually incorporated within the neurofibroma and a fusiform configuration is typically seen on gross inspection. Cervical neurofibromas may be solitary lesions (although rare) or they may be seen in patients with Neurofibromatosis1 (Von Recklinghausen’s disease) [4]

Ultrasound features: both schwannomas and neurofibromas show a heterogenous hypoechoic pattern. A pseudocystic appearance with posterior enhancement is commonly seen with both tumors and this may lead to confusion. Neurofibromas however, do not always show posterior enhancement. Neurofibromas may be lobulated and schwannomas may show true well defined cystic component within the tumor. The contours of the tumor are well defined; irregular borders may suggest malignant changes. The nerve immediately adjacent to the tumor may be thickened producing tapering appearance to the tumor. Presence of nerve thickening is the best clue to the nature of these tumors. Histologically, schwannomas are more vascular than neurofibromas. Colour doppler imaging also demonstrates this increased vascularity, which is extremely sensitive to pressure. Even mild transducer pressure obliterates intra-tumoral vascularity. Despite the histological differences between neurofibromas and schwannomas, sonographic features have not allowed reliable distinction between the two.[9]

The typical appearance of peripheral nerve sheath tumor on MRI appears hyperintense on T2WI; the lesion can appear heterogenous due to haemorrhagic or cystic degeneration. After the administration of contrast agent marked contrast enhancement may lead to a misdiagnosis of hypervascular tumor. [2] Actually, peripheral nerve sheath tumor is a relatively hypovascular lesion and the enhancement is due to extravascular leakage through abnormally permeable vessels with poor venous drainage.

Typical signs on MRI:
• The “split-fat” sign - displacement of fat in the neurovascular bundle by the slow-growing tumor, and seen as a thin rim of hyperintense signal on T1 weighted sequences.[10]
• The “fascicular” sign – nerve fascicles can be seen as multiple ring like structures within.[10]
• The “target” sign – low signal intensity with a ring of high signal intensity peripherally on T2WI. This pattern reflects the histologic features of the tumor with peripheral myxomatous tissue with surrounding a fibrocollagenous core.[10]

Imaging findings of schwannomas are similar to those seen with neurofibromas and, in many cases, cannot be distinguished. However, some features can help differentiate these two lesions. When the parent nerve is identified, an eccentrically positioned lesion (in relation to the nerve) suggests a schwannomas, whereas a centrally located mass suggests a neurofibroma. Heterogeneous appearance with degeneration and cystic cavitation are much more common in schwannomas than in neurofibromas. “Ancient” schwannomas refer to long-standing lesions with advanced degeneration exhibiting calcification, hyalinization, and cystic cavitation, findings that can be identified on imaging.[11]

Metastatic lymph nodes, paragangliomas, and schwannoma of the cervical sympathetic chain are the usual differential diagnoses to be considered. Metastatic lymph nodes are often multiple with a known primary facilitating the diagnosis [6] Paragangliomas can be differentiated by dynamic contrast study: a schwannoma will always show slow steady increase enhancement while paragangliomas show rapid enhancement.[2] Another important point of differentiation is displacment of neck vessels: splaying of the carotid bifurcation with hypervascularity suggests a carotid body tumor, whereas in cases without hypervascularity, a cervical sympathetic chain schwannoma is another possibility. Vagus nerve schwannomas can separate the internal jugular vein and internal carotid artery, but seldom widen the carotid bifurcation. [7,8]

The accepted treatment of a cervical nerve sheath tumor is surgical excision, and less often an observational approach is adopted. The benefits of surgical excision must be carefully weighed against the risk of postoperative neurologic deficit especially in view of the indolent course seen with many of these tumors. Hence, an accurate and prompt diagnosis of cervical nerve sheath tumors is crucial. [6]

On gross examination, neurofibromas are white gray and lack degenerative changes. Less frequently, these tumors may arise in and expand a large nerve to form fusiform mass, when if confined by the epineurium, a true cpsule may be present. There is prominent increase in endoneurial myxomatous material that separates myelinated and non-myelinated axons; a disorderly proliferation of fusiform Schwann cells, fibroblasts and axons as well as perineurial fibrous thickening. [12]

In summary, there are certain imaging characteristics that may aid the radiologist in establishing a focused preoperative diagnosis of a peripheral nerve sheath tumor and differentiating from other solid lesions of carotid space.


1) Robert B. Lufkin et al. Teaching Atlas of Head and Neck Imaging. Thieme publications. Page 148.
2) A.LBaert et al. Head and Neck Cancer Imaging. Second edition. Springerlinks publications. Pages 188-192
3) Joseph K.T.Lee et al. Computed body tomography with MRI correlation. Lippincott Williams and Wilkins Publication. Fourth Edition. Volume 1. Page 339
4) Mahmood Mafee, Minerva Becker et al.Valvasorrie’s Imaging of the Head and Neck.Revised 2nd edition. Thieme Publications. Chapter12, page no 112
5) Green JD, Olsen KD, DeSanto LW, Scheithauer BW. Neoplasms of the vagus nerve. Laryngoscope 1988; 98:648–654 [CrossRef] [Medline]
6) Gopinathan Anil and Tiong-Yong Tan. CT and MRI Evaluation of Nerve Sheath Tumors of the Cervical Vagus Nerve. American Journal of Roentology. 2011 July, Volume 197, number 1.
7) Wang CP, Hsiao JK, Ko JY. playing of the carotid bifurcation caused by a cervical sympathetic chain schwannoma. Ann Otol Rhinol Laryngol. 2004. Sep;113(9):696-9.
8) Furukawa M, Furukawa MK, Katoh K, Tsukuda M. Differentiation between schwannoma of the vagus nerve and schwannoma of the cervical sympathetic chain by imaging diagnosis. Laryngoscope. 1996 Dec;106:1548-52
9) Anil T. Ahuja, Rhodri M. Evans. Practical Head and Neck Ultrasound. Masses along carotid artery. Page 99.
10) B.J. Manaster, David A.May et al. Musculoskeletal imaging. Elsevier Publications. Fourth edition. Chapter 36. Page 432.
11) John Lin1 and William Martel. Cross-Sectional Imaging of Peripheral Nerve Sheath Tumors. Characteristic Signs on CT, MR Imaging, and Sonography. American Journal of Roentgenology. 2001;176:75-82
12) Leon Barnes. Surgical Pathology of head and neck. Revised 2nd edition. Volume 2. Tumors of the nervous system. Page 798-801.

Case history: A 45-year-old female patient was referred to our hospital with painless swelling on left side of neck. She had been aware of the swelling for the last eight months which was gradually increasing in size

Peripheral Nerve Sheath Tumor of Cervical Vagus Nerve

 An 8-year-old female presented by his parents in pediatric emergency with seizures, right side hemiplegia and mental retardation. She had hypoxic ischemic insult. On examination she had spasticity in right side of body and mental retardation.

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 MRI T2W axial images show left cerebral hemiatrophy, dilatation of ipsilateral lateral ventricle and ipsilateral mid line shift of 7 mm.

 FLAIR axial and coronal images at the level of lateral ventricle show gliotic area with atrophy of left parietal lobe with relative preservation of deep grey nuclei.

 MRI diffusion weighted and ADC mapping images does not show restricted diffusion in concerned area.

Brain MRI was performed which revealed atrophy of left cerebral hemisphere, cerebral peduncle. There was ipsilateral midline shift and ventricular dilatation along with skull vault thickening and prominent frontal sinus, suggestive of congenital type of cerebral hemiatrophy or Dyke-Davidoff-Masson Syndrome.

MRI findings: There is presence of left cerebral atrophy involving left frontal, parietal and temporal lobe with dilatation of ipsilateral lateral ventricle and ipsilateral mid line shift of 7 mm towards left side with gliotic area in left parietal lobe with ipsilateral calvarial thickening.

 Patient presented with seizures, facial asymmetry, contralateral hemiparesis and mental retardation. The underlying etiology is cerebral insult that may occur in utero or early in life. Prenatal causes include congenital anomalies, cerebral infarction, vascular malformations and infections.

Perinatal causes are birth trauma, hypoxia and intracranial bleed. Postnatal hemiatrophy can develop secondary to cerebral trauma, tumors, infections and febrile seizures. Infantile (congenital) type of DDMS, in contrast to adult (acquired) DDMS, shows enlargement of calvarium, diploic space and paranasal sinuses. These compensatory cranial changes occur to take up the relative vacuum created by the atrophied cerebral hemisphere. Shen et al. depicted three MR imaging patterns of cerebral hemiatrophy: MR imaging pattern I corresponds to diffuse cortical and subcortical atrophy; pattern II corresponds to diffuse cortical atrophy coupled with porencephalic cysts; and pattern III corresponds to previous infarction with gliosis in the middle cerebral artery (MCA) territory. In our case, pattern III was present. The atrophied cerebral hemisphere will have prominent sulcal spaces if the vascular insult occurs after birth or after end of sulcation. However, if ischemia occurs during embryogenesis when the formation of gyri and sulci is deficient, prominent sulcal spaces will be absent.

Children with medically refractive epilepsy and hemiplegia may be candidates for hemispherectomy, which is helpful in eradicating or significantly reducing seizures in 85 percent of patients.

MRI is a valuable method of examination in the analysis of cerebral hemiatrophy as it has the ability to bring to light changes in the cerebral hemispheres as well as highlighting bony structural changes and thus differentiating between congenital and acquired types of DDMS.

Madan Mohan Gupta, Radiology Resident; Harinder Yant, Radiology Resident; N. U. Bahri, MD, S. L. Chudasama, MD; and Reeta Gupta MD
Department of Radiodiagnosis, M.P. Shah Medical College, Jamnagar, Gujarat, India

1. Atalar MH, Icagasioglu D, Tas F. Cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome) in childhood: Clinicoradiological analysis of 19 cases. Pediatr Int 2007;49:70-5.
2. Kochar DK, Jain N, Sharma BV. Dyke-Davidoff-Masson syndrome-Neuroimage. Neurol India 2001;49:417-8. [PUBMED]
3. Shen WC, Chen CC, Lee SK, Ho YJ, Lee KR. Magnetic resonance imaging of cerebral hemiatrophy. J Formos Med Assoc 1993;92:995-1000.
4. Zilkha A. CT of cerebral hemiatrophy. Am J Roentgenol 1980;135:259-62 .
5. Pedley TA, De Vivo DC. Seizure disorders in infants and children. In: Rudolph AM, Hoffman JI, editors. Pediatrics. 17th ed. Norwalk: Appleton-Century-Crofts; 1982. p. 216-39.

N.U. Bahri, MD

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