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. 2024 Oct 1;76(6):6080–6089. doi: 10.1007/s12070-024-05105-9

Extraosseous Ewing’s Sarcoma Masquerading as Adolescent Neck Mass–a Case Report and Review of Literature

Raja Ram Khenhrani 1,✉, Sapna Devi 2, Maha Veer 2, Sehrish Gaho 1, Fnu Sonia 2
PMCID: PMC11569049  PMID: 39559010

Abstract

Ewing sarcoma (ES) is a rare type of malignant bone tumor that arises from mesenchymal stem cells. It commonly occurs in the diaphysis and diaphyseal-metaphyseal portions of long bones, pelvis, and ribs, although any bone can be affected. A minority of the Ewing sarcoma also arise in soft tissues, and a primary tumor in the neck with metastasis is extremely uncommon. We are reporting the case of a 12-year-old female with a history of fever for 4 months and a neck mass for 3 months. Given the broad range of inflammatory and neoplastic etiologies, an extensive laboratory and imaging workup was conducted. Cervical lymph node biopsy for histopathology showed sheets of round blue cells consistent with the diagnosis of Ewing sarcoma in the neck, while a positive immunohistochemical assays for NKX2.2, and negative assay for PAX-5, TdT markers neurofilament, neuron-specific enolase, and S100 protein ruled out the alternative etiologies such as rhabdomyosarcoma and lymphoma. After surgical excision of tumor, chemoradiotherapy was initiated. Despite the typically benign nature of extraosseous Ewing tumors, they can rarely metastasize in less than 20% of cases, as exemplified by this rare case. Accurate diagnosis requires a combined clinic-radio-immunohistochemical approach, and general practitioners should be aware of this clinical entity in neck masses considering its variable clinical presentation and poor prognosis in certain patient’s population.

Keywords: Adolescent, Neck mass, Extraosseous ewing sarcoma, Metastasis, Lungs, Vertebrae

Background

ES is a poorly differentiated and a highly malignant tumor that primarily presents as tumor of bone. Less commonly, it also arises in the soft tissues and is termed Extraosseous Ewing Sarcoma (EES) [1]. The annual incidence of ES is approximately one case per million in the United States, with higher rates in patients aged between 10 and 19 years. Caucasians and whites are affected more frequently than Asians and Africans. The median age of diagnosis is 15 years, with males being the most commonly affected gender [2, 3]. In comparison, patients with EES typically presents at higher mean age of 19.5 years and less likely to be a male or of the white race [4]. The bones of the lower extremity and pelvis are the most common primary sites of ES. Conversely, the trunk is the prime site of involvement in EES [5]. The majority of ES cases are sporadic in nature; however, the translocation of Ewing sarcoma breakpoint region 1 (EWSR1) gene on short arm of chromosome 22 has also been detected in suspected cases [6].

The clinical features of EES are highly variable and non-specific, thus posing a diagnostic challenge to the clinicians with a broad range of possible diagnosis. The most frequently reported presenting symptom is a growing mass with local pain [7]. Similarly, the imaging findings of EES are non-specific, and may range from a low echogenicity mass with intratumor flow signals on Doppler study [4], contrast- enhancement of mass and hypodense foci (intratumor necrosis) on computed tomography (CT) [8], and signal abnormalities on magnetic resonance imaging (MRI) [9]. Therefore, there is a compelling need for tissue biopsy and histopathological confirmation of the diagnosis.

We are hereby reporting a rare case of primary EES in the neck with metastatic spread to the lumbar vertebrae and lungs in a 12-year-old female who presented to us in an outpatient setting with a history of fever and swelling in the neck. Based on history and non-specific finding of unilateral swelling in neck, a broad range of pathological conditions were entertained until the cervical lymph node biopsy turned out to contain sheets of round blue cells and immunohistochemical NK2 homeobox 2 (NKX2.2) marker was found to be consistent with the diagnosis of malignant EES. Additionally, a contrast CT-scan of the neck and chest revealed a primary tumor in the neck with metastatic nodules in both lungs, and an increased uptake of radiotracer on a bone scan, which is consistent with the metastasis of the tumor in vertebral column. To the best of our knowledge, only a few cases have been described on the rare existence of primary EES in the neck and this is the eighth case reported with distant metastasis. In this article, we have also reviewed reported cases of metastasis found in the literature.

Case Presentation

A 12-year-old female Pakistani patient with no past medical history attended outpatient department on February 26, 2023, with complaints of fever for 04 months and swelling in the neck for 03 months. During the early stages of her illness, she experienced low-grade intermittent fever, for which her parents self-medicated with over-the-counter paracetamol. A month later, she noticed a small painless swelling on the right side of her neck, which progressively increased to the size of a tennis ball, prompting her to seek immediate medical attention. There was no significant family history of malignancy, and she had no history of smoking or alcohol addiction. The review of systems was unremarkable.

On physical examination, significant findings included enlarged anterior cervical lymph nodes that were firm in consistency and non-tender to touch. A localized swelling with visible veins below the mandible on the right side of neck was noted. The swelling was single, oval-shaped, non-mobile, non-tender, measuring 8 × 7.5 cm in size, and hard in consistency without overlying bruit, discharge or skin discoloration [Fig. 1A]. The rest of systemic examinations were unremarkable. Vitals were significant for blood pressure of 105/60 mm Hg (reference: 50th – 90th percentile for her age), heart rate of 96 beats per minute, temperature of 100 °F, respiratory rate of 21 breaths per minute, oxygen saturation of 98% on room air, and body mass index of 11.1 kg/m2 (healthy BMI range is 14.8–21.7 kg/m2). Additionally, she had pallor conjunctiva without icterus, cyanosis, or any signs of distress.

Fig. 1.

Fig. 1

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A Lump on right side of neck on admission, B Reduced lump size after surgery and chemotherapy

Based on history and examination, this patient was admitted to the hospital with presumed neoplastic, congenital, or inflammatory etiologies as a possible cause for origin of her neck swelling. Upon admission, she underwent an extensive blood workup and imaging, and was evaluated by the otolaryngologist and surgery teams. Her blood investigations revealed low haemoglobin (Hb) of 8 g/dL with normocytic and normochromic cells, high erythrocyte sedimentation rate (ESR) of 110 mm/hr (reference range: <20 mm/hr), and elevated serum lactate dehydrogenase (LDH) levels of 1143 (reference range: 110–265). Comprehensive metabolic panel, uric acid levels, and serum calcium levels were found to be normal.

An ultrasound of her neck showed an 8.9 × 7.9 cm mass with irregular margins and increased vascularity – suggestive of possibly a nodal mass or matted (conglomerated) cervical lymph nodes. Her CXR showed multiple nodular opacities [Fig. 2]. The induced sputum samples for nucleic acid amplification test (Gene-Xpert) were negative for Tuberculosis. Thyroid levels were within the normal limits. Electrocardiography and echocardiography were normal. To determine the cause of the patient’s symptoms, an excisional biopsy of her cervical lymph node and a bone marrow aspirate with biopsy were performed. Given the unknown cause of her fever, she was commenced on IV ceftriaxone and paracetamol as pro re nata.

Fig. 2.

Fig. 2

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X-ray Chest showing diffuse nodular opacities in both lung parenchyma

On March 21, 2023, the results of cervical lymph node biopsy showed multiple sheets of mitotic round blue cells positive for NKX2.2 on immunostaining, unveiling the diagnosis of EES [Fig. 3]. The Computed tomography (CT) scan showed a well-defined, lobulated, and heterogeneously-enhancing lesion in the right neck, measuring 8.0 × 8.6 × 8.4 cm in size. This lesion extended from the base of skull up to 4th cervical (C) vertebrae, encasing the styloid process superiorly, abutting the right pterygoid muscle medially, and displacing the parapharyngeal space anteriorly. The lesion obliterated fossa of Rosenmüller and torus tubarius, causing deformed changes in pharynx and larynx. Anteriorly, the lesion also abutted the masseter, buccinator muscle, ramus of mandible, and laterally displaced the deep lobes of parotid gland. Posteriorly, it abutted the paraspinal muscles and encroached the neural foramen from C1 to C4. Adjacent internal jugular vein and common carotid artery were compressed along with the formation of multiple collaterals on lateral and inferior aspect of lesion. Multiple cervical lymph nodes were enlarged on right side of neck. Diffuse lytic lesions were found in the C-vertebrae, accompanied by destructive changes in the occipital condyle of the C1 and C2 vertebrae [Fig. 4]. The plain CT chest showed well-circumscribed metastatic nodules of varying sizes on both lung fields [Fig. 5]. Bone scintigraphy was ordered in an attempt to determine if another focus of disease could be detected, and it revealed increased uptake of Tc-99 m labelled (MDP) over L1 and L5 vertebrae [Fig. 6], suggesting metastatic spread. There was no evidence of malignant cells infiltration on the bone marrow aspirate and trephine biopsy, and cerebrospinal fluid analysis did not show any malignant cells.

Fig. 3.

Fig. 3

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A Histomorphology of Ewing sarcoma with classic composed of small round blue cells in cervical lymph node tissue, B showing diffuse nuclear immunoreactivity for NKX2-2

Fig. 4.

Fig. 4

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A CT-scan neck showing well-defined, lobulated, heterogeneously enhancing lesion on right side of neck (red arrow), B Sagittal view of lesion encasing and compressing nearby structures (red arrow)

Fig. 5.

Fig. 5

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Non-contrast-enhancing CT-scan showing cannon-ball appearing metastatic nodules

Fig. 6.

Fig. 6

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Bone scintigraphy showing variably increase uptake in lumbar region (L1-L5)

During hospitalization, a multimodal approach was taken to manage her metastatic tumor. On April 1, 2023, the neck mass was biopsied, which revealed a translocation of 22q12 on fluorescence in situ hybridization (FISH) assay for ESWR1 gene rearrangement as shown in Fig. 7, further confirming our final diagnosis of primary ESS in the neck. Given her metastatic disease, the oncology team was taken on board after the surgical excision of the lesion. The patient was started on VDC-based intravenous (IV) chemotherapeutic regimen with Vincristine 2 mg/m2, Doxorubicin 75 mg/m2, and Cyclophosphamide 1200 mg/m2. Mesna cycles at 2.1 g/m2/day were also added to reduce cyclophosphamide-induced side-effects. While on this regimen, the patient developed nausea and vomiting, which were managed with IV ondansetron. Upon follow-up, her fever had subsided, and the lesion became softer and regressed in size, as shown in Fig. 1B. However, she developed worsening respiratory distress, severe bone pain, and ultimately, succumbed to metastasis after six months.

Fig. 7.

Fig. 7

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FISH showing translocation of 22q12 on EWSR1 gene rearrangement

Discussion

The EFTs: Ewing Sarcoma family of tumors (EFTs) comprising ES, primitive neuroectodermal tumor, and Askin’s tumor, are a group of highly malignant small round cell tumors that typically affects bone, soft tissues, and chest wall, respectively [1, 10]. After the discovery of undifferentiated Ewing’s tumors of bone by James Ewing in 1921, the term EES was then coined in literature by Tefft in 1969 [11]. It is estimated that nearly 20–30% of EES constitutes about 400 annually diagnosed cases of the Ewing sarcoma family of tumors in the United States [12].

In 2011, the Surveillance, Epidemiology, and End Results Program database conducted compared the characteristics of EES patients with skeletal ES, and found that ESS is usually diagnosed in late adolescent at the median age of 20 (range 0–39) years, with males being the less commonly affected gender, and axial skeleton being the primary site of involvement. The rare association with head and neck is seen in 18% of cases [4].

For the literature review, we used PubMed, Google Scholar, Web of Sciences, Scopus, Cochrane library, and MEDLINE as our databases to search the terms Ewing sarcoma, Extra-osseous Ewing sarcoma, Extra-skeletal Ewing sarcoma, Cervical Ewing sarcoma, Thyroid sarcoma, Submandibular sarcoma, Neck Sarcoma from inception to March, 27, 2024. EES of the neck is exceedingly rare, with only forty cases identified in the literature as shown in Table 1. Of these cases, 22 (61%) were males and 14 (39%) were females, with a M: F ratio of 1.6:1. In four cases, gender was not reported. Metastasis was reported in 7 (17.5%) cases, and lungs being the most common sites of involvement observed in four cases (57%). Out of the cases reviewed, 29 patients showed no evidence of disease after treatment, four died of metastatic disease, and information regarding the remaining eight patients was unavailable. Our patient was also found to have metastatic disease in lungs and vertebral column.

Table 1.

Literature review of previously published cases of EES cases in the neck

References Age (years) Gender Primary site (± size) Metastasis Treatment Status
McCreary DJ et al., 2022 [15] 5-month-old F Neck mass (5 × 4.1 × 3.6 cm) Unknown CT+PBT Unknown
De Sa, Hong et al., 2022 [16] Newborn F Neck mass - CT NED
AlAtwan, Abrar A et al., 2022 [12] 42 M Posterior aspect of neck (13.8 × 12.5 × 7 mm) - NAC NED
Wang, Hanrui et al., 2022 [17] 29 F Larynx (3.8 × 2 cm) - S+CT+RT+DC-CTL NED
Alhomsi, Dima et al., 2022 [18] 41 M Larynx (3.5 × 2 × 3 cm) - S Unknown, referred to oncology department
Malvika Shastri et al., 2022 [19] 18 F Thyroid (10 × 10 cm) - None DOD (within a month after diagnosis)
Seipel, Amanda H et al., 2022 [20] 54 F Thyroid (3.7 × 3.1 × 2.1 cm) - S+CT+RT NED
Wang, Shuang et al., 2022 [21] 36 F Left neck mass (9 × 8 × 6 cm) - S+CT+RT NED
Zhu, Weiyu et al., 2021 [22] 30 F Thyroid - S+CT+RT NED
Pasha, Hamdan Ahmed et al., 2020 [23] 5 M Larynx (18 × 16 mm) - S NED
Ansari, Mohammed- Humaam et al., 2019 [24] 3 F Right side of neck (46 × 40 mm) - S+RT NED
Maroun, C A et al., 2019 [25] 53 M Larynx (5.0 × 3.8 × 3.8 cm) Lungs CT+RT NED
Khosla, Divya et al., 2019 [26] 8 F Left parapharyngeal space Lungs, vertebrae CT + RT DOD
Gazula, S et al., 2019 [27] 4-month-old F Left submandibular (2.9 cm × 2.8 cm × 2.2 cm) - S+CT NED
Yildiz, Gulsah Aynaoglu et al., 2018 [28] Intrauterine fetus M Right neck mass (15 × 10 × 9 cm) Liver S+CT Unknown
Rama-López, Julio et al., 2017 [29] 70 M Left supraclavicular fossa (6.1 × 6.7 × 7.1 cm) - S+CT+NAC+RT NED
Van Der Meer, Graeme et al., 2017 [30] 12 M Mass posterior to trachea and anterior to vertebrae (6.6 × 7.5 × 3.7 cm) - CT + RT NED
Kabata, Pawel et al., 2017 [31] 34 M Lower left neck (5.8 × 5.8 × 6.0 cm) - S+NAC NED
Ijichi, Kei et al., 2016 [32] 33 F Larynx - S+CT+RT NED
Lynch, Michael C et al., 2014 [33] 45 F Larynx (2.9 cm) - CT + RT NED
Chirila, Magdalena et al., 2013 [34] 48 M Thyroid (10 × 10 cm) Cerebral S+CT DOD
Wygoda, A et al., 2013 [35] 68 M Larynx (20 × 19 × 17 mm) - CT+RT NED
Maldi, Elena et al., 2012 [36] 66 M Thyroid (4 × 5 cm) Discovered S Unknown
Whaley, J Taylor et al., 2010 [37] 19 M Posterior neck muscle mass (4 cm) - CT+RT NED
Abdel Rahman, Hany et al., 2010 [38] Unknown (4 Patients) Unknown Neck Unknown Unknown Unknown
Schmidt, Sandrin et al., 2010 [39] 16 M Neck - S+CT+RT NED
Adapa, Pavani et al., 2009 [40] 9 F Thyroid (4 × 4.5 × 6 cm) - S+CT+RT NED
Weinreb, Ilan et al., 2008 [41] 29 M Right neck mass (2.5 cm) - S+CT NED
Ali S et al., 2008 [42] 14 M Right neck mass (12 × 5 cm) Unknown CT+RT Unknown
Cho, Sung In et al., 2007 [43] 49 M Parapharynx Lungs, dural venous sinuses CT+RT NED, however, visual loss didn’t improve
Agir, Hakan et al., 2006 [44] 22 M Submandibular gland (4 × 5 cm) Pelvis, skull, clavicle, sternum, meninges, brain, lungs S+CT+RT DOD
Chung, Cheng- Ho et al., 2006 [45] 30 F Right side of neck anteriorly (2 × 2 × 2.8 cm) - S Unknown
Yang YS et al., 2004 [46] 74 M Larynx (3.53 × 2.0 cm) - S+CT+RT NED
Ng, S-H et al., 2004 [47] 53 M Right Parapharyngeal space (5 × 4 cm) Intracranial extension (Cranial nerve V, IX to XII) CT+RT DOD
Yi-liang HOU et al., 2002 [48] 18 M Root of right neck (1.4 × 1.0 × 0.8 cm) - S+CT+RT NED
Kennedy, J G et al., 2000 [49] 24 M Left side of neck (12 × 8 cm) - S+RT NED
Gustafson, R O et al., 1982 [50] 18 M Ride side of neck (14 × 10 × 7 cm) - S+CT+RT NED
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F: Female; M: Male; S: Surgery; CT: Chemotherapy; NAC: Neoadjuvant chemotherapy; PBT: proton beam therapy; RT: radiotherapy; DC-CTL: dendritic cells-cytotoxic T-lymphocytes; NED: No evidence of disease at follow-up; DOD: Died of disease; FU: Follow-up

The clinical features of EES, including painless lump and B symptoms, closely resemble those of lymphoma, embryonal rhabdomyosarcoma, and neuroblastoma, among various neoplastic etiologies. Similarly, non-specific imaging findings and the microscopic presence of small, round, blue cells with a sheet-like arrangement of mitotic cells and scant clear cytoplasm make their differentiation challenging for clinicians [2].

Unlike lymphoma and rhabdomyosarcoma, the presence of a cluster of differentiation (CD)-99 (MIC2) cell surface glycoprotein and FLI-1 are typically seen in ES and ESS. It is noteworthy here that CD99 lacks specificity and also seen in synovial sarcoma, non-Hodgkin lymphoma, and gastrointestinal stromal tumors, thus making its accuracy controversial for the definitive diagnosis of EES on immunohistochemistry [4]. Additionally, negative staining for leukocyte common antigen (LCA), myoglobin, actin, CD 30, and myosin helps rule out rhabdomyosarcoma and lymphoma, as seen in our case. Furthermore, the neuroblastoma is characterized by negative staining for neurofilament, neuron-specific enolase, and S100 protein [24], and were also negative in our case. The highly sensitive and specific NK2 homeobox 2 (NKX2.2) acts as a target gene for EWS-FLI-1 and upregulate in ESS, and helps in ruling out other tumors by its variable sensitivity in them [13]. Our patient had positive NKX2.2, whereas immunonegative PAX-5, terminal deoxynucleotidyl transferase (TdT) markers without involvement of bone marrow (i.e., negative CD-3). The presence of a common chromosomal translocation t(11;22)(q24;q12) forms an anti-apoptotic transcription factor EWSR1- FLI1 has been detected as 95% sensitive and 90% specific on molecular genetics in nearly 85% of EFTs [14]. With regards to our case, FISH detection of t(22q12) in our patient added additional value to the confirmation of diagnosis.

Imaging options such as Doppler, CT, MRI, and bone scans (also known as positron emission tomography) are used in the diagnosis of tumor status, local resectability, and distant metastasis. Regarding radiological findings, there are no specific features. They usually appear as well circumscribed, non-calcific soft tissue tumors and demonstrate either internal heterogeneity with areas of low attenuation secondary to necrosis or hyper-attenuation secondary to hemorrhage on CT scan [12]. Sonographic findings may show hypoechoic mass with internal vascularity [4], as seen in our patient. Additional findings of lung metastatic nodules missed on CXR can be detected by CT scan, as seen in our patient. Moreover, the bony metastasis can be detected best by the increased uptake of radiotracer technetium (Tc)-99 m labelled methylene diphosphonate (MDP) [12]. Our patient was found to have increase tracer uptake over L1 and L5 vertebrae.

Upon diagnosis of EES, the initial treatment of tumor in neck can be particularly challenging due to limited number of cases. Though treatment strategies are being devised, the complex anatomy of the region, size and location of tumor, and stage of metastasis helps determine tumor therapy. The multimodal approach including chemotherapy, surgery, and/or radiation therapy are standard strategies while treating metastasized ESS sarcoma. The VIDE-based chemotherapy with Vincristine, Ifosfamide, Doxoroubicin, and Etoposide has been the most renowned initial regimen and consists of 6 cycles at 2–4 weeks intervals. After induction chemotherapy, surgery and radiotherapy are the recommended treatments depending upon the severity of disease [12]. With recent advancement in treatment modalities, new therapies such as DC-CTL has also proven to be beneficial [17]. Our patient underwent excision of tumor. Subsequently, due to limited resources, she was started on VDC-based chemotherapy with vincristine, doxorubicin, and cyclophosphamide. Six months later, she succumbed to metastatic disease.

Conclusions

In conclusions, EES of neck is a rare entity that can imitate a broad range of inflammatory, neoplastic, vascular, and congenital pathologies. Given the scarcity of literature on this condition, a high index of clinical suspicion is necessary for its early detection. The tissue biopsy for histopathology is crucial in the diagnosis of this disease, whereas molecular markers can aid in differentiating it from similar pathologies. Additionally, imaging techniques like MRI and CT scans, play a vital role in staging the disease and directing surgical excision of the lesion. A multifaceted approach to the treatment, including administration of new immunomodulatory therapies, is essential in cases with metastasis. Moreover, extensive research on the biological nature of this tumor would help us discover new therapeutic regimens and improve patient outcomes.

Acknowledgements

None.

Author Contributions

Conception, RRK, SD, and SG. Design of the work, RRK, SG, and FS. Investigation, RRK, SD. Formal analysis, RRK. Supervision and visualization, RRK and SG. Drafted the manuscript and data curation, RRK, SG, MV, FS. Critical revision of the manuscript, RRK, SG, and MV. Final approval, all authors.

Funding

The authors received no financial support for the research, authorship, and/or publication of this report.

Declarations

Ethics Approval and Consent to Participate

The work was approved by the Research Ethical Committee at the Sindh Government Lyari General Hospital.

Consent for Publication

Written informed consent was taken from the patient for publication of this case report and any accompanying images.

Competing Interests

None.

Footnotes

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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