1. Abstract Ewing’s Sarcoma (ES) is a solid tumor of bone and soft tissue, affecting mainly adolescent and young adults. It is the second most common primary bone tumor in childhood and the most lethal bone tumor. ES is more common in the axial skeleton, head and neck localization is extremely rare (2 to 4%). The purpose of this article is to discuss the case of a young patient diagnosed with mandibular Ewing’s sarcoma and highlight the importance of early diagnosis.
2. Introduction ES a rare malignant neuro-ecto-dermal tumor. It accounts for 4 to 10% of all types of bone cancer, with a predilection for long bones and pelvis [1, 2]. Facial bones are affected in 2 to 4% of cases with nonspecific symptoms. SE affects adolescents and young adults and is uncommon before 8 years and after 25 years [3]. The reported case is that of a young patient with mandibular Ewing sarcoma having benefited from multidisciplinary treatment, with an overall survival of 4 years without local or distant recurrence.
3. Case Presentation This is a young 14-year-old patient, with no notable history, referred by her dental surgeon for a left mandibular swelling evolving for 2 months. The clinical examination revealing an expansive swelling of the left mandibular region, associated with left angulo-mandibular lymphadenopathy of about 3 cm. The intraoral examination shows a mass not invading the dental structures, the overlying mucosa was slightly erythematous without ulceration. On palpation, the tumoral masse was hard, firm, painless and with out any sign of inflammation. The mouth opening was normal. A computed tomography (CT) scan with three-dimensional CT of the facial massif revealed the presence of a manifestly malignant lesion process of the left horizontal branch of the mandible (Figure 1) associated with a left Angulo-mandibular lymphadenopathy of about 4 cm (Figure 2), may be compatible with Ewing’s sarcoma or osteosarcoma.
For diagnostic confirmation, the patient beneficed from a surgical biopsy of the lesion. Microscopic analysis of the specimen showed solid sheet of small-round cell population scattered in a fibrovasculat stroma. Nuclei are rounded, hyperchromatic, nucleolate and richly mitotic. The immunohistochemical complement was performed and revealed positive for CD99, vimentin and pancytokeratin, confirming the diagnosis of Ewing’s sarcoma. A whole body bone scan and a bone marrow aspiration biopsy was made and no abnormalities or distant metastases were revealed. The patient received induction chemotherapy combining Vincristine (V), Ifosfamide (I), Adriamycin (A), Cyclophosphamide (C) and Etoposide (E) (VAC-IE) without significant adverse effects. The evaluation scan showed a tumor regression of 40%. The excisional surgery consisted of a left hemi-mandibulectomy without reconstruction. The anatomo-pathological analysis of the excisional piece showed the presence of a tumor residue estimated at 5% with a tumor jugal limit. Consolidation chemotherapy was started postoperatively. The use of radiotherapy was necessary because of the tumor margins. Radiotherapy was administered concurrently with the start of consolidation chemotherapy. The patient received a dose of 45 Gy in 1.8Gy per fraction, spread over 35 days, in technique volumetric modulated arc therapy (VMAT) (Figure 3).
4. Discussion First described by James Ewing in 1921, ES is an aggressive primitive malignant bone tumor that is radiation sensitive in contrast to osteosarcoma [4]. It affects children and young adults preferentially between 5 and 20 years, peak age is approximately 15 years [5], with a male predominance of (2:1) [6] and preferably persons of Caucasian origin [7]. ES most often develops in the lower extremity (femur 15–20%, tibia or fibula 5–10%), pelvis (20–30%), upper extremity (humerus 5–10%), ribs (9–13%), and spine (6–8%). Long bone tumors usually present in diaphysis. Cervical and facial locations are very rare, mandibular involvement represents only 0.7% of all locations observed [8]. At the time of diagnosis, 20-25% of patients have gross metastases, and nearly all patients have micrometastases. Metastasis is more in pelvic primary tumors as compared with tumors of the extremities or ribs [9, 10]. Common sites of metastases are the lungs (40%) and bones (40%), and infrequently to other sites [11]. Locoregional pain is the most common symptom presented in patients with ES, associated with mass syndrome and pathological fractures. At the mandibular level, the symptoms are misleading and non-specific causing delayed diagnosis. It is most often a mass rapidly progressive more or less painful mimicking a dental infection [12, 13]. Other symptoms may be observed such loosing teeth, otitis media, trismus, and paresthesia if the tumor is near to the inferior alveolar nerve [14]. As others bone tumors, systemic symptoms such fever, anemia, and weight loss are found in < 10% of cases [15]. The mucous plane is usually healthy or erythematous and rarely ulcerated. On a plain X-ray, the ES is defined as a destructive lesion of the bone, and takes the appearance of a lytic or sclerotic lesion. Other forms can be observed such “moth-eaten,” “onion skinning”, or “Codman’s triangle” reaction. The presence of a periosteal bone reaction and displacement or destruction of unerupted tooth follicles have been described as the commonest radiological features for ES affecting jaw bones [16, 17] “Onion skinning” reaction which suggested as a common radiological feature for lots of ES of the long bones, is rarely seen in jaws lesions [18, 19]. Local computed tomography (CT) and magnetic resonance imaging (MRI) are complementary needed to make the diagnosis and assess the resectability of the tumor. The CT can be used to identify bone destruction and soft tissue extent associated with Ewing’s sarcoma. Tumor size is evaluated after contrast injection, which may be a means of assessing tumor response after chemotherapy. The other advantage of the scanner is to make the differential diagnosis with osteomyelitis, fibrosarcoma or osteosarcoma. MRI allows a precise definition of the local extension of the tumor, especially the intramedullary portion, and the relation of the tumor with the neighboring structures namely the vessels and the nerves [20]. Based on the use of FDG as a marker of tumor viability, FDGPET-CT help to detect bone metastases no depicted on traditional bone scans, both at diagnosis and at recurrence [21]. In addition, PET-CT is the most sensitive modality for therapeutic follow-up, and detection of early changes in tumor metabolism, which is the consequence of a therapeutic response. Because few lesions < 8 mm are not detectable using FDG-PET-CT imaging, CT scans are still more accurate for the screening of lung metastases [21].
5. Conclusion ES is a highly aggressive tumor that very rarely affects the mandible. Because of its important metastatic power that conditions the prognosis, the diagnosis of ES must be accurate and early. Modern multimodality treatment strategy based on chemotherapy regimens neoadjuvantly and adjuvantly, in combination with surgery and/or radiotherapy for local control will aim to achieve maximum possible outcome for these patients.
6. Acknowledgment We thank the Radiotherapy department and our radiotherapist‘s colleagues at the National Institute of Oncology of Rabat who provided care and support for the patient.
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Imane Mbarki. Mandibular Localizations of Ewing Sarcomas in Children: A Rare Presentation. Annals of Clinical and Medical Case Reports 2021