AI Article Synopsis

  • * Two pediatric cases presented with invasive tumors affecting the skull base and upper spine, showing unique cell morphology and specific immunohistochemical markers, leading to a diagnosis of PDC.
  • * The study emphasizes the importance of understanding PDC's clinical and histological characteristics to improve diagnostic accuracy and awareness.

Article Abstract

Introduction: Poorly differentiated chordoma (PDC) is an uncommon subtype of chordoma, distinct in its occurrence in paediatric age group, location, variable epithelioid/rhabdoid/spindled histomorphology and the lack of physaliphorous cells (classical of chordoma) and immunohistochemistry (INI-1 loss, brachyury positive). We describe two cases of PDC.

Case Reports: A 3-year-old male and 4-year-old female child presented with neck stiffness and infiltrating tumour involving the skull base and upper cervical vertebral segments. Histopathology showed a tumour with sheets of cells having epithelioid to rhabdoid morphology and absence of physaliphorous cells. The tumour cells were positive for pan-cytokeratin, EMA, CD99 and vimentin and showed loss of INI-1 suggesting differentials of epithelioid sarcoma and atypical teratoid/rhabdoid tumour. On careful review of the clinical, radiological and pathological features, the additional immunohistochemistry for brachyury was performed, and its positivity clinched the diagnosis of PDC. Both the patients succumbed within a short span post-surgery.

Conclusion: The present case study helps in creating an awareness and attempts to expand our knowledge in relation to the spectrum of chordoma (clinico-histological) and its immunohistochemical profile.

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00381-024-06641-zDOI Listing

Publication Analysis

Top Keywords

differentiated chordoma
8
immunohistochemical profile
8
physaliphorous cells
8
tumour
5
chordoma recognising
4
recognising complex
4
complex rare
4
rare aggressive
4
aggressive tumour
4
tumour characteristic
4

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!