Sacrococcygeal chordoma

Case contributed by Mostafa Elfeky
Diagnosis certain

Presentation

Constipation and painful sacral mass.

Patient Data

Age: 25 years
Gender: Female

Lumbosacral spine and pelvis

x-ray

Mid-level sacral osteolytic lesion with smooth bone scalloping of the sacrum, more extending on the right side. Preserved coccyx.

Pelvis

mri

Evidence of soft tissue space occupying lesion epicentered upon the right side of the sacrum with multispatial extensions showing the following criteria:

  • size: it measures roughly 9.3 x 11.3 x 8.1 cm regarding its maximum dimensions 
  • signal pattern: heterogeneous signal intensities are noted, mainly T1 hypo, T2 hyperintense signal intermingled with T1 hyper and T2 hypointense foci ( suspected calcific rather than hemorrhagic)
  • extensions: the lesion is expanding the right sacral foramina with anterior extension into the pelvic cavity displacing the uterus and the rectum, superiorly, extending upward within the intraspinal canal to the level of SV2, inferiorly to the right side of the perineum, laterally to the left sacral neural foramina and bounded by both iliac muscles and iliac bones
  • enhancement pattern: postcontrast heterogeneous enhancement 
  • DWI: foci of restriction are noted

The right ovarian hemorrhagic cyst is noted measuring 3 x 4 cm.

Case Discussion

The patient was submitted to debulking surgery. Pathology revealed Chordoma.

Microscopic picture

Sections through an angioinvasive tumor tissue, formed of lobules showing cuboid cells strung together in hepatocyte-like cords. Numerous physaliphorous cells are seen. A predominately myxoid matrix is visualized. Mitotic activity is indiscernible. No undifferentiated component is detected in the material submitted. The tumor invades bone and infiltrates surrounding skeletal muscle fibers.

Differential diagnosis includes sacrococcygeal teratoma and neurogenic tumors.

 

Additional contributor Dr. Mohamed Kaed, Radiology consultant, Alexandria, Egypt.

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