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Year : 2010  |  Volume : 6  |  Issue : 3  |  Page : 327-329

Imaging of parotid gland primitive neuroectodermal tumor

Department of Diagnostic Imaging, KK Women's and Children's Hospital, Singapore

Date of Web Publication29-Nov-2010

Correspondence Address:
Niketa C Chotai
Department of Diagnostic Radiology, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng, Singapore - 308 433
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.73345

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 > Abstract 

A seven-years-old boy presented with recurrent episodes of right parotid gland swelling that was presumptively being treated as sialoadenitis. Interrogation with ultrasonography, computerized tomography and magnetic resonance imaging revealed a heterogeneous mass occupying the right parapharyngeal space, imperceptibly merging with adjoining parotid gland, scalloping the vertical ramus of the mandible and involving the base skull with widening of the foramen ovale. The findings at surgery and histopathology provided a final diagnosis of parotid gland primitive neuroectodermal tumor. This report emphasizes on the imaging findings of this rare tumor occurring in such an unusual location.

Keywords: Imaging of primitive neuroectodermal tumor, Parotid space, parapharyngeal space

How to cite this article:
Chotai NC, Tang P, Gopinathan AN. Imaging of parotid gland primitive neuroectodermal tumor. J Can Res Ther 2010;6:327-9

How to cite this URL:
Chotai NC, Tang P, Gopinathan AN. Imaging of parotid gland primitive neuroectodermal tumor. J Can Res Ther [serial online] 2010 [cited 2021 Jan 21];6:327-9. Available from: https://www.cancerjournal.net/text.asp?2010/6/3/327/73345

 > Introduction Top

Primitive neuroectodermal tumor (PNET) is a malignant small round cell tumor, characterized by unique chromosomal translocations and MIC-2 antigen expression. [1] When these tumors arise outside the central and sympathetic nervous system, they are termed as peripheral PNET. [2] Common locations of peripheral PNET (pPNET) include thoracopulmonary region, the retroperitoneum and pelvis. We report a case of parotid gland PNET that is extremely unusual for its location, with emphasis on the role of pre-operative radiological imaging.

 > Case Report Top

A seven-year-old boy presented with history of recurrent right parotid gland swelling, of two years duration, presumptively being treated as sialoadenitis. An ultrasound was performed to exclude sialolithiasis.

Ultrasound (US) showed a 3 cm, heterogeneous mass in the right side of the neck, inseparable from the deep surface of the right parotid gland. The complete extent of the mass was not sufficiently evaluated on US. Subsequently a contrast enhanced CT scan was done with z-axis coverage from the base skull to arch of aorta. It demonstrated about 4 cm relatively well-defined heterogeneous, moderately enhancing soft tissue mass, isodense to the muscle involving the deep lobe of the parotid gland and completely filling the parapharyngeal space (PPS) [Figure 1]a. There was widening of the stylomandibular tunnel with involvement of the deep aspect of the superficial lobe of parotid gland. There was extension into the masticator space along with smooth scalloping of the ramus of mandible. Widening of foramen ovale [Figure 1]b and equivocal intracranial extension was noted. The carotid space was displaced posteriorly without encasement or infiltration of the great vessels within.
Figure 1: Axial (a) soft tissue window and coronal (b) bone window CT showing the mass filling up the right parapharyngeal space with widening of stylomandibular tunnel (thin arrows). Note the scalloping of the ramus of the mandible (thick arrows in a) and widened foramen ovale (arrow heads in b)

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Multiplanar contrast enhanced MRI was done to ascertain the anatomical extent of the tumor and exclude intracranial involvement. MRI confirmed the soft tissue extent of the tumor as seen at CT. There were several T2 bright, rim-enhancing areas suggesting necrosis, in otherwise moderately enhancing lesion [Figure 2]a. Besides, it also demonstrated medial displacement of the PPS. Smooth, linear, dural enhancement was seen in the floor of the middle cranial fossa as the tumor widened the foramen ovale and abutted the dura [Figure 2]b. An intervening sliver of fluid was still present between brain and dura.

After excluding distant metastasis by bone scan, interval excision of the mass was done. Complete surgical clearance was achieved. Findings at surgery and histology with immuno-histochemistry (positive for CD 99, CD56, synaptophysin) were reminiscent of parotid gland PNET. Twelve cycles of chemotherapy were administered. The patient is on biannual follow- up since last two years and is disease free till date.
Figure 2: Post contrast T1-weighted axial and coronal MRI showing large heterogeneously enhancing mass with necrotic areas (arrow in a). Note the smooth, linear dural thickening in foramen ovale region (arrow heads in b) without overt intracranial extension

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 > Discussion Top

Current evidence indicates that both PNET and Ewing's sarcoma represent two ends of the spectrum of the same entity. [3] pPNET is rare and comprise only around 1% of all the sarcomas. [2] Rare occurrences of this tumor in the head and neck region include those arising from the maxilla, mandible, orbits, nasal cavity, paranasal sinuses, temporal region and rarely parotid gland. To the best of our knowledge, there are only four prior reports on parotid PNET in medical literature in English language. [4],[5],[6],[7] All four of them dealt with pathological findings with limited literature on the scope of diagnostic imaging. The previously reported cases of parotid PNET were seen in adults in the fourth to sixth decade of life. In comparison, this case is unique as our patient presented in the first decade of life.

Suprahyoid deep neck spaces are not easily accessible to clinical examination and hence imaging plays crucial role in this region. The role of ultrasound is limited as the complex regional anatomy restricts the acoustic window for adequate evaluation and CT is usually the first choice of investigation. However, the young age of this patient and clinical suspicion of sialoadenitis had prompted US over CT. The heterogeneously hypoechoic appearance on US with cystic areas, seen here, has been described earlier in PNET. [2] The primary role of USG was of excluding more common pathologies and indicating the presence of neoplastic lesion rather than infective/inflammatory pathology.

CT and MRI is the mainstay when investigating head and neck masses. The rim enhancing, T2 bright foci seen at MRI, in this patient were corresponding to the several necrotic areas found at pathological examination. PNETs are known to have internal hemorrhage and necrosis. [8] They are usually rapidly growing, locally aggressive tumors with invasion of adjacent bone/ soft tissues and neovascularization. Probably this tumor was slow- growing and less aggressive than a usual PNET, as was suggested by the scalloping of the mandible and smooth widening of the foramen ovale as against permeative bone destruction in a rapidly growing, aggressive lesion. In malignant head and neck tumors, the primary role of CT and MRI is in providing an accurate estimate of the extent of the disease and presence of adequate margins for resection. CT may show cortical bone involvement better, but MRI offers better soft tissue resolution and may be mandatory for intracranial extension and marrow infiltration.

The differential diagnoses in this patient would include embryonal rhabdomyosarcoma (ERMS), salivary neoplasms, lymphoma, malignant peripheral nerve sheath tumors and hemangioma. Salivary gland tumors are very uncommon at young age. Though ERMS is the most common soft tissue sarcoma seen in the head and neck region in pediatric population, they are more often found in orbits, nasopharynx, paranasal sinuses and infratemporal fossa with primary parotid rhabdomyosarcoma extremely rare. However in this case, excluding an ERMS at imaging was not possible. Similarly lymphomas and malignant peripheral nerve sheath tumours are other rare conditions that can occur in this region with no pathognomonic imaging findings. Hemangiomas are generally brighter on T2WI and enhancement may vary as per their flow characteristics.

 > Conclusion Top

Our experience with USG, CT and MRI in this case of pPNET of the parotid gland, defined the tumor as a heterogeneous mass with necrotic areas within, moderate contrast enhancement and scalloping of the adjoining bones. With such findings on cross-sectional imaging, PNET should be considered in the differential diagnosis of moderately aggressive soft tissue tumors in pediatric age group, even when present in unusual locations.

 > References Top

1.Ambros IM, Ambros PF, Strehl S, Kovar H, Gadner H, Salzer-Kuntschik M. MIC-2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Cancer 1991;67:1886-93.  Back to cited text no. 1
2.Kim MS, Kim B, Park CS, Song SY, Lee EJ, Park NH, et al. Radiological findings of peripheral primitive neuroectodermal tumor arising in the retroperitoneum. AJR 2006;186:1125-32.  Back to cited text no. 2
3.Dehner LP. Peripheral and Central primitive neuroectodermal tumors: A nosologic concept seeking a consensus. Arch Pathol Lab Med 1986;110:997-1005.   Back to cited text no. 3
4.Mackay B, Luna MA, Butler JJ. Adult neuroblastoma: electron microscopic observations in nine cases. Cancer 1976;37:1334-51.  Back to cited text no. 4
5.Jones JE, McGill T. Peripheral primitive neuroectodermal tumors of head and neck. Arch Otolaryngol Head Neck Surg 1995;121:1392-5.  Back to cited text no. 5
6.Deb RA, Desai SB, Amonkar PP, Aiyer PM, Borges AM. Primary PNET of the parotid gland. Histopathology 1998;33:375-8.  Back to cited text no. 6
7.Helsel JC, Mrak RE, Hanna E, Parham DH, Bardales RH. Peripheral primitive neuroectodermal tumor of the parotid gland region: Report of a case with fine-needle aspiration findings. Diagn Cytopathol 2000;22:161-6.   Back to cited text no. 7
8.Ibarburen C, Haberman JJ, Zerhouni EA. Peripheral primitive neuroectodermal tumors: CT and MRI evaluation. Eur J Radiol 1996;21:225-32.  Back to cited text no. 8


  [Figure 1], [Figure 2]

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