Infantile undifferentiated sarcomas: a diagnostic and therapeutic challenge - two case reports and literature review

Abstract

Background: Soft tissue undifferentiated sarcomas (STUS) are an ultra-rare and heterogenous group of mesenchymal neoplasms often lacking known genetic abnormalities with a marked vulnerability towards intensive therapy such as invasive surgery and high dose chemotherapy. Despite aggressive treatment, they are usually associated with dismal outcomes.

Case presentation: Here we describe two cases of STUS in 3-week-old and 3-month-old infants localized on the neck and the trunk area.

Discussion: In both cases, the malignancy had a fatal outcome due to the toxicity of intensive therapy in one case and the progression of the disease in the other. The purpose of this report is to discuss the clinical challenges of managing infancy-related STUS such as limited treatment options and poor prognosis.

Figure 1

Lumbosacral MRI of case #1: (A) T1 sequence (coronal), (B) T1 contrast enhanced (coronal), (C) T2 sequence (axial) – at the moment of diagnosis; (D) T2 sequence (axial) at disease progression. MRI: Magnetic Resonance Imaging.

Figure 2

Craniofacial MRI of patient #2 at the time of diagnosis showing the tumor in the left submandibular region, modeling and displacing the left salivary gland, the styloglossus muscle, the posterior belly of the digastric muscle, the root of the tongue, the muscles of the mouth floor, the oral and laryngeal parts of the pharynx, and downwardly reaching the level of the middle part of the larynx: (A) T2 SPACE sequence (sagittal), (B) T2 SPACE sequence (coronal), (C) T1 VIBE sequence (axial), (D) T1 VIBE contrast enhanced sequence (axial). MRI: Magnetic Resonance Imaging.