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Aggressive metastatic chordoma in the wrist.

Abraham J, Clemens MW, Oates S - Plast Reconstr Surg Glob Open (2014)

View Article: PubMed Central - PubMed

Affiliation: The University of Texas Medical School at Houston Houston, Tex.

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Chordoma is a rare, malignant, primary bone tumor (1–4), which arises from remnant cells of the developmental notochord... We report an extremely rare case of a metastatic sacral chordoma in the wrist... The patient had a history significant for a 14 × 12 cm sacral chordoma, which was surgically excised in 2011, with a total sacrectomy and pelvic reconstruction and had been disease free for 1 year... The wrist is now 15 months post-op and is without signs of local recurrence, but patient continues to demonstrate vertebral metastases... There are no further surgical interventions planned for the patient... Chordomas are extremely rare with an annual incidence of 0.0001% in the general population, with approximately 300 new patients diagnosed each year in the United States... Chordomas are slow growing, locally aggressive, and typically found at the skull base and sacrococcygeal region... Local recurrence rates greater than 70% have been reported in surgically treated chordomas with nonnegative margins, and although chordoma metastasis is rarely evident during initial diagnosis, it presents later in the disease process in 40–60% of patients... Common sites for metastasis include the lungs, liver, heart, lymph nodes, and vertebrae... This case illustrates the need for increased physician vigilance in monitoring newly identified masses in patients with a history of chordoma, by obtaining a biopsy and subsequent surgical excision of the metastatic mass... The authors have no financial interest to declare in relation to the content of this article... The Article Processing Charge was paid for by the authors.

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Intraoperative view of excisional biopsy. Note the clean separation of the vascularized myxoid tumor from the underlying soft tissue without evidence of invasion of the deep fascia.
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Figure 2: Intraoperative view of excisional biopsy. Note the clean separation of the vascularized myxoid tumor from the underlying soft tissue without evidence of invasion of the deep fascia.

Mentions: A 59-year-old man presented in 2012 with a gradually enlarging 3 cm bulbous, transilluminating, firm mass over the left volar radial wrist. The mass was suspicious for a ganglion cyst and was aspirated at an outside institution with indeterminate pathology (Fig. 1). The patient had a history significant for a 14 × 12 cm sacral chordoma, which was surgically excised in 2011, with a total sacrectomy and pelvic reconstruction and had been disease free for 1 year. An excisional biopsy was performed (Fig. 2), and pathologic analysis revealed a 3 × 3 cm metastatic myxoid chordoma positive for brachyury. Magnetic resonance imaging of the left wrist for staging, 2 months after initial diagnostic excision, demonstrated a recurrent 1.3 × 0.7 cm lobulated mass. A second resection was performed with a 1-cm wide margin. In 2013, patient developed recurrence iliac and lumbar vertebrae disease treated with Gleevec. The wrist is now 15 months post-op and is without signs of local recurrence, but patient continues to demonstrate vertebral metastases. There are no further surgical interventions planned for the patient.


Aggressive metastatic chordoma in the wrist.

Abraham J, Clemens MW, Oates S - Plast Reconstr Surg Glob Open (2014)

Intraoperative view of excisional biopsy. Note the clean separation of the vascularized myxoid tumor from the underlying soft tissue without evidence of invasion of the deep fascia.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4255888&req=5

Figure 2: Intraoperative view of excisional biopsy. Note the clean separation of the vascularized myxoid tumor from the underlying soft tissue without evidence of invasion of the deep fascia.
Mentions: A 59-year-old man presented in 2012 with a gradually enlarging 3 cm bulbous, transilluminating, firm mass over the left volar radial wrist. The mass was suspicious for a ganglion cyst and was aspirated at an outside institution with indeterminate pathology (Fig. 1). The patient had a history significant for a 14 × 12 cm sacral chordoma, which was surgically excised in 2011, with a total sacrectomy and pelvic reconstruction and had been disease free for 1 year. An excisional biopsy was performed (Fig. 2), and pathologic analysis revealed a 3 × 3 cm metastatic myxoid chordoma positive for brachyury. Magnetic resonance imaging of the left wrist for staging, 2 months after initial diagnostic excision, demonstrated a recurrent 1.3 × 0.7 cm lobulated mass. A second resection was performed with a 1-cm wide margin. In 2013, patient developed recurrence iliac and lumbar vertebrae disease treated with Gleevec. The wrist is now 15 months post-op and is without signs of local recurrence, but patient continues to demonstrate vertebral metastases. There are no further surgical interventions planned for the patient.

View Article: PubMed Central - PubMed

Affiliation: The University of Texas Medical School at Houston Houston, Tex.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Chordoma is a rare, malignant, primary bone tumor (1–4), which arises from remnant cells of the developmental notochord... We report an extremely rare case of a metastatic sacral chordoma in the wrist... The patient had a history significant for a 14 × 12 cm sacral chordoma, which was surgically excised in 2011, with a total sacrectomy and pelvic reconstruction and had been disease free for 1 year... The wrist is now 15 months post-op and is without signs of local recurrence, but patient continues to demonstrate vertebral metastases... There are no further surgical interventions planned for the patient... Chordomas are extremely rare with an annual incidence of 0.0001% in the general population, with approximately 300 new patients diagnosed each year in the United States... Chordomas are slow growing, locally aggressive, and typically found at the skull base and sacrococcygeal region... Local recurrence rates greater than 70% have been reported in surgically treated chordomas with nonnegative margins, and although chordoma metastasis is rarely evident during initial diagnosis, it presents later in the disease process in 40–60% of patients... Common sites for metastasis include the lungs, liver, heart, lymph nodes, and vertebrae... This case illustrates the need for increased physician vigilance in monitoring newly identified masses in patients with a history of chordoma, by obtaining a biopsy and subsequent surgical excision of the metastatic mass... The authors have no financial interest to declare in relation to the content of this article... The Article Processing Charge was paid for by the authors.

No MeSH data available.


Related in: MedlinePlus