Recurrent sacrococcygeal chordoma

Case contributed by RMH Neuropathology

Presentation

Recurrent sacral chordoma.

Patient Data

Age: 69
Gender: Male
CT

CT Pelvis (2 years ago)

The known, lobulated, moderately well-defined, infiltrating soft tissue mass is again demonstrated involving the tip of the coccyx and extending inferiorly and anteriorly into both ischiorectal fossae as well as posteriorly into the surrounding fat, deep to the superior aspect of the natal cleft. The perirectal fat, although narrowed in places, is preserved with no definite breach.

The mass measures up to 9.8cm in maximum craniocaudal dimension and inferiorly on the right side measures up to 7.7 x 4.2cm.

Superiorly, there is evidence of invasion of the coccyx. Ill-defined soft tissue infiltrate continues superiorly into the right side and midline of the coccyx over a distance of approximately 5.2cm with associated erosion.

No free intraperitoneal fluid or focal collection. No pelvic or inguinal lymphadenopathy.

Features suggest a previous TURP.

Mild degenerative change in the lower lumbar spine and sacroiliac joints bilaterally.

Conclusion: The features would be in keeping with the clinical history of recurrent chordoma and are unaltered from the recent MRI.​

MRI

MRI pelvis (2 years ago)

Comparison is made to an outside MRI and the previous CT from this hospital.

Recurrent chordoma is documented-with markedly hyperintense signal on T2 weighted scans, best demonstrated on the fat saturated axial and coronal T2 images, with tumour in the midline and on the right 6.7 x 3.2 cm, in continuity with multiple contiguous nodules in both ischio rectal fossae, on the left with displacement of the levator ani complex superiorly, and with a high suspicion for tumour invasion across this fascial plane. More inferiorly from the ischio rectal fossa, tracking between the gluteus maximus and medius muscles, is a tongue of tumour that reaches to the subcutaneous tissues laterally on the buttock, 1.3 cm wide, and 12 cm long (extending along a surgical plane? ). Tumour extends into the central and right side of the sacrum - both bone and canal.

Enhancement is moderate. Tumour does not reach to the lumbar spine, and there is no involvlement of the bony pelvis outside the sacrum.

Conclusion: Local tumour spread demonstrated, in gluteal region, perineum, sacrum, ischiorectal fossa, and likely spread through the left levator ani abutting the rectum.​

MRI

MRI pelvis (10 months ago)

Reference is made to the prior scan.

As previously demonstrated, there is a large chordoma arising from the inferior aspect of the right sacral ala with inferolateral extension into the mesorectal fat, the ischiorectal fossa and the gluteus maximus muscle. There has been increase in extent and dimensions of the mass. Superiorly, there is increase in the component within the right posterolateral aspect of the mesorectal fat with obliteration of the fat plane between the mass and the rectum. There is no extension within the lumen of the rectum. There is increase in the component within piriformis muscle and also increase in the AP diameter of the gluteus maximus muscle invasive component.

There is interval small exophytic nodule extending from the mass in the right ischiorectal fat.

Prior sacral surgery and fat grafting in the sacral spinal canal are unchanged.

Conclusion: There is further increase in the size of the right sacral chordoma as detailed.

 

MRI

MRI lumbar spine and pelvis (6 months ago)

MRI lumbar spine, sacrum and pelvis

Pre-and post contrast sequences have been obtained, with comparison to the previous MRI.

The sacrum is again truncated at the S3 level, consistent with previous tumour debulking. The lobulated T2 hyperintense tumour in the ischiorectal fossae, right piriformis and abutting partially resected sacrum remains in situ, but the large component which had previously extended inferolaterally posterior to the right ischial tuberosity and right hip joint has been debulked. Mild oedema in the operated region is expected postsurgical finding.

There is stable indentation of the mesorectal fascia by the right ischiorectal fossa component of tumour. No proximal extension into the remaining portion of the sacral canal.

Conclusion: Debulking of the right gluteal portion of the large sacral chordoma.​

MRI

MRI pelvis (current)

No evidence of distal sacrectomy at the level of S3. The lobulated T2 hyperintense and enhancing tumour in the right gluteal region involving the ischiorectal fossae, right piriformis and abutting partially resected sacrum is larger. Today the largest more central component measures 11 x 11.7 x 10.7 cm (AP x LR x CC) and there 2 large tongues of tumour extending inferiorly and laterally into the gluteus musculature on the right which have markedly increased in size.

The tumour no above the right lateral wall of the rectum however there is preservation of the mesorectal fat plane. This

Enlarging enhancing bony lesions in the posterior right ilium in keeping with progressive metastatic disease.

Conclusion:  Progressive local and metastatic disease.​

Pathology

MICROSCOPIC DESCRIPTION: Sections show a multi-lobulated, circumscribed tumour with a fibrous pseudocapsule in areas. The tumour is moderately cellular composed of sheets of eosinophilic tumour cells set within extracellular matrix material. There are focal areas of haemorrhage and necrosis present. Tumour cells contain round-to-oval nuclei with finely granular chromatin and prominent nucleoli. Physalliferous cells containing vacuolated cytoplasm are present. No atypia or mitoses are seen. No skeletal muscle, lymphovascular or perineural invasion is seen. Margins are unable to be accurately assessed due to fragmentation.

DIAGNOSIS: Sacrum: Recurrent chordoma.

Case Discussion

Chordomas correspond to ~4% of all primary bone tumours. 

Traditionally surgical resection has been the first line of treatment in feasible scenarios with radiotherapy offered for recurrent cases. Recurrence, such happened in this case, including seeding along the operative tract, is common.

Prognosis is typically poor, due to the locally aggressive nature of these tumours, with the 10 year survival approximately 40%.

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Case information

rID: 40476
Case created: 22nd Oct 2015
Last edited: 24th Oct 2015
System: Spine
Inclusion in quiz mode: Excluded

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