Imaging Sci Dent.  2014 Dec;44(4):307-314. 10.5624/isd.2014.44.4.307.

Primitive neuroectodermal tumor of the maxillary sinus in an elderly male: A case report and literature review

Affiliations
  • 1Department of Dental Public Health, Bangladesh Dental College, Dhaka, Bangladesh.
  • 2Department of Oral and Maxillofacial Radiology and Dental Research Institute, School of Dentistry, Seoul National University, Seoul, Korea. future3@snu.ac.kr

Abstract

Primitive neuroectodermal tumor (PNET), which belongs to the Ewing's sarcoma (ES) family of tumors, is mainly seen in children and young adults. PNETs are extremely rare in the maxilla. Here, we report a case of PNET of the left maxillary sinus in an elderly male. Magnetic resonance imaging (MRI) revealed a slightly enhanced solid mass occupying the left maxillary sinus and infiltrating into the retroantral space. A partial maxillectomy was performed. Despite postoperative chemotherapy, follow-up computed tomography (CT) and MRI revealed a nodal metastasis in the submandibular space. Neck dissection was performed. However, the patient died 10 months after the second surgery because of distant metastasis to the liver. MRI and CT were particularly useful in detecting the extent of the tumor, recurrence, and metastasis. Further, a literature review of the previously reported PNET cases of the maxilla was carried out. In this paper, we also discuss the current approach for the diagnosis and management of these tumors.

Keyword

Neuroectodermal Tumors, Primitive; Maxillary Sinus; Magnetic Resonance Imaging

MeSH Terms

Aged*
Child
Diagnosis
Drug Therapy
Follow-Up Studies
Humans
Liver
Magnetic Resonance Imaging
Male
Maxilla
Maxillary Sinus*
Neck Dissection
Neoplasm Metastasis
Neuroectodermal Tumors, Primitive*
Recurrence
Sarcoma, Ewing
Young Adult

Figure

  • Fig. 1 Panoramic radiograph shows increased radiopacity in the left maxillary sinus with thinning of the innominate line of the zygomatic process of the left maxilla.

  • Fig. 2 Posteroanterior skull radiograph demonstrates erosion of the lateral wall of the left maxillary sinus.

  • Fig. 3 Magnetic resonance images reveal hyperintense T2 (A) and hypointense T1 signal lesion (B) with minimal enhancement occupying left maxillary sinus (C, D). Note the infiltration into the retroantral space posteriorly, and gingivobuccal sulcus inferiorly. The central low signal intensity with a dark rim is possibly due to a hematoma from the initial curettage/surgical procedure.

  • Fig. 4 Bone scans show hot spots in the left maxilla.

  • Fig. 5 Photomicrograph of a histopathological slide of primary tumor shows tumor cells with round to oval outlines, arranged in a lobular pattern. The nuclei are well-defined and hyperchromatic, while the cytoplasm is very scanty (H&E stain, 200×).

  • Fig. 6 Photomicrographs A. Tumor cells are stained positively for CD99, (CD99 stain, 200×). B. The majority of tumor cells are positive for neuron-specific enolase (NSE stain, 200×). C. A few tumor cells are positive for S-100 protein (S-100 stain, 200×). D. Tumor cells show a focal positive reaction for cytokeratin (CK stain, 200×).

  • Fig. 7 Panoramic radiograph reveals partial maxillectomy.

  • Fig. 8 Follow-up enhanced computed tomography (CT) image, taken 3 months after the operation, reveals a minimally enhancing 2-cm mass in left submandibular space compressing the submandibular gland.

  • Fig. 9 Follow-up fat-suppressed T2-weighted MR image, taken after 4 months after the follow-up CT scan, shows an increase in size of the metastatic lymph node in the left submandibular space.

  • Fig. 10 Photomicrograph of histopathological slide of the metastatic tumor, reveals tumor cells with round nuclei and ill-defined cytoplasm. Numerous mitoses are observed (H&E stain, 200×).


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