Chordoma News Headlines

Sacral Chordoma: Can Local Recurrence After Sacrectomy Be Predicted?

Clin Orthop Relat Res. 2008 Jun 27;

Authors: Hanna SA, Aston WJ, Briggs TW, Cannon SR, Saifuddin A

Surgical resection margins are reportedly the most important predictor of survival and local recurrence with sacral chordomas. We examined the relevance of invasion of the surrounding posterior pelvic musculature (piriformis and gluteus maximus) at initial diagnosis to local recurrence after sacrectomy. We retrospectively reviewed 18 patients with histologically verified sacral chordoma seen at our institution between 1998 and 2005. There were 14 men and four women with a mean age of 65.1 years (range, 31-78 years). The average overall followup was 4.4 years (range, 0.5-10 years), 5.4 years for the living patients (range, 3-10 years), and 2.8 years for the deceased (range, 0.5-5.4 years). Local recurrence occurred in 12 patients (66%) 29 months postoperatively (range, 2-84 months). Six of these patients had wide excisions at initial surgery, five had marginal excisions, and one had an intralesional excision. Ten patients had wide surgical margins, six of whom (60%) had local recurrences. Tumor invasion of adjacent muscles at presentation was present in 14 patients, 12 of whom (85%) had local recurrences. Sacroiliac joint involvement was seen in 10 patients, nine of whom (90%) had local recurrences. The findings suggest obtaining wide surgical margins posteriorly, by excising parts of the piriformis, gluteus maximus, and sacroiliac joints, may result in better local disease control in patients with sacral chordoma. Level of Evidence: Level IV, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.

PMID: 18584264 [PubMed - as supplied by publisher]

Malignant myoepithelioma of soft tissue: a case report with cytogenetic findings.

Cancer Genet Cytogenet. 2008 Jun;183(2):121-4

Authors: Balogh Z, Deák L, Sápi Z

Soft tissue malignant myoepithelioma (STMM) is a particularly rare tumor displaying myoepithelial elements and lacking obvious ductal differentiation. From the one case report with cytogenetic data available in the literature, STMM seems to be a distinct entity with some resemblance to chordoma on the one hand and myoepithelioma on the other. The present case of STMM yielded novel data from high-resolution comparative genomic hybridization (HR-CGH) analysis. An 82-year-old female patient presented with a soft tissue tumor within the deep soft tissues in the right gluteal muscle measuring 16 x 13 x 11 cm. Histologically, the lesion was diagnosed as a myoepithelial carcinoma. Immunohistochemistry was focally positive for pancytokeratin, EMA, S-100 protein, and alpha smooth muscle actin. HR-CGH analysis revealed gains of 1p31 approximately p34, 1q21 approximately q23, 9q12 approximately q33, and 16q22 and losses of 1p11 approximately p22, 1q24 approximately q44, 3p, 10q11.1 approximately q22, 13q, 14q13 approximately q24, and 15q. Subsequent fluorescence in situ hybridization analysis confirmed deletion of 3p, gain of 16q, and monosomy of chromosomes 13 and 15. These results support the hypothesis that STMM is a distinct entity, not sharing the cytogenetic alterations of salivary gland myoepithelial carcinomas and ductal carcinomas of breast with myoepithelial differentiation.

PMID: 18503832 [PubMed - in process]

Differential diagnosis of dumbbell lesions associated with spinal neural foraminal widening: Imaging features.

Eur J Radiol. 2008 May 14;

Authors: Kivrak AS, Koc O, Emlik D, Kiresi D, Odev K, Kalkan E

Computed tomography (CT) and magnetic resonance imaging (MRI) reliably demonstrate typical features of schwannomas or neurofibromas in the vast majority of dumbbell lesions responsible for neural foraminal widening. However, a large variety of unusual lesions which are causes of neural foraminal widening can also be encountered in the spinal neural foramen. Radiologic findings can be helpful in differential diagnosis of lesions of spinal neural foramen including neoplastic lesions such as benign/malign peripheral nerve sheath tumors (PNSTs), solitary bone plasmacytoma (SBP), chondroid chordoma, superior sulcus tumor, metastasis and non-neoplastic lesions such as infectious process (tuberculosis, hydatid cyst), aneurysmal bone cyst (ABC), synovial cyst, traumatic pseudomeningocele, arachnoid cyst, vertebral artery tortuosity. In this article, we discuss CT and MRI findings of dumbbell lesions which are causes of neural foraminal widening.

PMID: 18485652 [PubMed - as supplied by publisher]

Related Articles

Dedifferentiated chordoma: A REPORT OF FOUR CASES ARISING 'DE NOVO'.

J Bone Joint Surg Br. 2008 May;90(5):652-6

Authors: Hanna SA, Tirabosco R, Amin A, Pollock RC, Skinner JA, Cannon SR, Saifuddin A, Briggs TW

Dedifferentiated chordoma is a rare and aggressive variant of the conventional tumour in which an area undergoes transformation to a high-grade lesion, typically fibrous histiocytoma, fibrosarcoma, and rarely, osteosarcoma or rhabdomyosarcoma. The dedifferentiated component dictates overall survival, with smaller areas of dedifferentiation carrying a more favourable prognosis. Although it is more commonly diagnosed in recurrences and following radiotherapy, there have been a few reports of spontaneous development. We describe four such cases, which were diagnosed de novo following primary excision, and discuss the associated clinical and radiological features.

PMID: 18450635 [PubMed - in process]

Primary reconstruction of pelvic floor defects following sacrectomy using Permacoltrade mark graft.

Eur J Surg Oncol. 2008 Apr 23;

Authors: Abhinav K, Shaaban M, Raymond T, Oke T, Gullan R, Montgomery AC

AIMS: The large pelvic floor defect following sacrectomy for sacral masses leaves the challenging problem of primary closure and herniation. We present the outcome of primary repair using Permacoltrade mark, a biomaterial made of acellular porcine cross-linked dermal collagen and with similar tensile strength to polypropylene mesh. It is non-allergenic and possibly less likely than synthetic mesh to cause inflammation leading to small bowel adherence; fistula formation and graft extrusion. Following implantation, Permacol is colonized by host cells and resists degradation by host enzymes. METHODS: Three patients had sacrectomy with primary repair of pelvic floor defects between March 2004 and August 2005. Two had excision of sacral chordomas and one excision of a sacrococcygeal teratoma. Repair of the defect was carried out using the Permacol graft, suturing to the sacrum, anococcygeal raphe and ischial spines. Two suction drains were placed superficial to the mesh. RESULTS: All patients had gross en-bloc tumour resections and over a median follow-up period of 1year (range 8-25months), there were no complications related to primary repair. CONCLUSION: Primary closure of a large defect following sacrectomy using a Permacol graft, in our early experience seems to be convenient and safe without the development of herniation.

PMID: 18439796 [PubMed - as supplied by publisher]

Extranotochordal extralaryngeal chordoma: A case report.

Auris Nasus Larynx. 2008 Mar 20;

Authors: Bumber Z, Krizanac S, Janjanin S, Bilic M, Bumber B

Chordomas are rare, malignant, slowly growing neoplasms which develop from vestigial remnants of the fetal notochord. Most chordomas arise in the sacrococcygeal and spheno-occipital region. Extranotochordal chordomas are extremely unusual. A case of extranotochordal chordoma with extralaryngeal localization is described. A 73-year-old male presented with swallowing difficulties and hoarseness. Contrast-enhanced magnetic resonance imaging of the neck revealed a well-encapsulated tumor mass that was well enhanced and located in the left retrolaryngeal space at the level of C4, dislocating the larynx to the right. Left radical neck dissection and tumor extirpation were performed. The tumor had not invaded cervical vertebra and the surrounding soft tissue but superficial erosions of the ossificated thyroid and cricoid cartilage were found. High-power pathologic examination and immunohistochemistry defined the lesion as a dedifferentiated type of chordoma. The patient received adjuvant radiotherapy. Four years after the surgery, the patient has been free from tumor recurrence.

PMID: 18358656 [PubMed - as supplied by publisher]

[Extensive lower clivus chordomas.]

Neurochirurgie. 2008 Mar 20;

Authors: Fournier HD, Hue AS, Laccourreye L

BACKGROUND AND PURPOSE: Clival chordomas are rare skull-base tumors with local malignant behavior. Their control and removal remain difficult because of their anatomical location and because of their extensions. The goal of the treatment is complete surgical removal in a single stage if possible, with minimal deficits, followed by proton therapy. If the tumor remains extradural for a while, it finally progresses through the dura backwards to reach and displace the brain stem and upper cervical cord. Its anterior extension in the retropharyngeal space offers a logical opportunity and many advantages to use an anterior approach. METHODS: With three consecutive cases, we try to demonstrate that the unilateral transmandibular approach offers a large exposure of the lower clivus, the foramen magnum in its ventral part, the ipsilateral infratemporal fossa and C1 to C3. Surgical complications concern the lower cranial nerves, including the hypoglossal. Serous otitis media is possible in case of opened Eustachian tube. Tracheostomy is needed because of a transient tongue oedema. RESULTS: The unilateral transmandibular approach enabled to anatomical and physiological nasal preservation, large operative field facilitating dural closure and tumor removal, with acceptable cosmetic results and sequellae considering the natural course and prognosis of the tumor. CONCLUSIONS: This approach seems to be very useful to reach and removed extensive lower chordomas.

PMID: 18359050 [PubMed - as supplied by publisher]