Extraskeletal Ewing's sarcoma masquerading as infantile benign neck mass

Gazula, Suhasini; Rani, Vidya; Jonathan, G. T.; Kumar, Navin

doi:10.4103/jiaps.jiaps_98_18

Cited by 6 publications

(3 citation statements)

References 5 publications

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“…The best indicator, a direct invasion of bone usually happens in the terminal stage, is that MRI can help in clinical staging and follow-up for EES recurrence[ 12 ]. Despite the benign-like appearance, sometimes nonspecific imaging features of large, deep in soft-tissue and well-defined may aid the EES diagnosis[ 6 , 8 , 13 , 14 ].…”

Section: Discussionmentioning

confidence: 99%

Imaging-based diagnosis for extraskeletal Ewing sarcoma in pediatrics: A case report

Chen¹,

Guo²,

Chen³

et al. 2022

WJCC

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BACKGROUND Extraskeletal Ewing sarcoma (EES) is a member of the Ewing sarcoma family of tumors which is pathologically known as a small, round, blue cell tumor involving bone and soft tissue. The prevalence of EES is only 15%-25% of all Ewing sarcoma and EES often occurs in patients aged from 20-mo-old to 30-years-old resulting in an unfavorable prognosis. CASE SUMMARY The present case report described a 7-year-old patient with a palpable EES mass of 33 mm × 27 mm × 28 mm in the deep neck with symptoms of persistent dyspnea over the past 5 mo. After laboratory examinations, abnormal physiological and biochemical indicators were not found. Ultrasound images presented the mass to be complex, solid and fluid-filled with circumscribed margins and posterior acoustic enhancement. The mass also presented with partial internal vascularity. The contrast-enhanced magnetic resonance imaging scan illustrated the outstanding enhancement with fast perfusion mode in the early arterial phase. CONCLUSION Our study suggested that a quick-growing mass in the pediatric patient is possibly a malignant tumor whether the mass has well-defined margins or not.

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Section: Discussionmentioning

confidence: 99%

Imaging-based diagnosis for extraskeletal Ewing sarcoma in pediatrics: A case report

Chen¹,

Guo²,

Chen³

et al. 2022

WJCC

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“…Infant diagnosis is rare, with one study showing only 2.6% of all EWS cases presenting before 3 years of age11; soft-tissue or extraskeletal EWS is also relatively rare, only representing 4% of all ESFTs 12. Thus, congenital soft-tissue EWS is exceedingly rare and has been described exclusively in case reports—as few as 19 times in the English-language literature 3,12–16. Congenital soft-tissue EWS is rare; however, because of the aggressive nature and subsequent risk for airway compromise, neonatal nurses and physicians must consider malignancy on their differential when evaluating neonates with neck masses and involve appropriate levels of subspecialty care.…”

Section: Discussionmentioning

confidence: 99%

“…12 Thus, congenital softtissue EWS is exceedingly rare and has been described exclusively in case reports-as few as 19 times in the English-language literature. 3,[12][13][14][15][16] Congenital softtissue EWS is rare; however, because of the aggressive nature and subsequent risk for airway compromise, neonatal nurses and physicians must consider malignancy on their differential when evaluating neonates with neck masses and involve appropriate levels of subspecialty care.…”

Section: Casementioning

confidence: 99%

Congenital Ewing Sarcoma Presenting as a Rapidly Growing Neck Mass in a Newborn

Pearl

Hibbs

et al. 2022

Advances in Neonatal Care

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Background: Ewing sarcoma (EWS) is an aggressive soft-tissue and bone malignancy. Congenital EWS is extremely rare, and its presenting features can be unique from that of EWS occurring in older children. Clinical Findings: A full-term female infant with a neck mass present at birth was admitted to a level I nursery with an otherwise well appearance and normal vital signs. After consultation with a neonatologist, she was transferred to a neonatal intensive care unit where she developed sudden respiratory collapse from rapid growth of the mass causing airway obstruction, leading to emergent intubation. Ultrasound and MRI scans of the neck mass demonstrated cystic and vascular components, and a timely biopsy revealed small round blue cells with diffuse CD99 expression and chromosomal translocation 11;22. Primary Diagnosis: Ewing sarcoma. Interventions: An accelerated workup for EWS was done due to the patient's critical status. On day of life (DOL) 8, she was started on treatment of EWS as per the current standard-of-care AEWS0031. On DOL 24, she underwent tracheostomy placement. Outcomes: The patient completed 14 total cycles of chemotherapy and is more than 12 months old. Her tracheostomy was decannulated at 6 months of age. Practice Recommendations: The rarity of EWS in neonates and its presentation as a neck mass make this disease difficult to recognize unless clinicians have a high index of suspicion. The aims of this case report are to increase awareness of malignancy as a potential cause of neck masses in neonates and to prompt nurses and physicians to prepare for airway stabilization at appropriate levels of care if a neck mass is present at birth.

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