|Year : 2013 | Volume
| Issue : 3 | Page : 497-499
Multifocal thoracic chordoma mimicking a paraganglioma
Giovanni Conzo1, Claudio Gambardella1, Daniela Pasquali2, Giuseppe Ciancia3, Nicola Avenia4, Cristina Della Pietra1, Salvatore Napolitano1, Antonietta Palazzo1, Claudio Mauriello1, Domenico Parmeggiani5, Guido Pettinato3, Vincenzo Napolitano6, Luigi Santini1
1 Department of Anaesthesiologic, Surgical and Emergency Science, VII Division of General and Endocrine Surgery, Second University of Naples, Naples, Italy
2 Division of Endocrinology, Second University of Naples, Naples, Italy
3 Department of Surgical Pathology and Cytopathology, University "Federico II" of Naples, Naples, Italy
4 Endocrine Surgery Unit, University of Perugia, Perugia, Italy
5 Department of Anaesthesiologic, Surgical and Emergency Science, XI Division of General Surgery, Second University of Naples, Naples, Italy
6 Department of Anaesthesiologic, Surgical and Emergency Science, Endoscopic Unit, Second University of Naples, Naples, Italy
|Date of Web Publication||8-Oct-2013|
Department of Anaesthesiologic, Surgical and Emergency Science, VII Division of General and Endocrine Surgery - Second University of Naples - Italy. Via Gen. Giordano Orsini 42 - 80132 Naples
Source of Support: None, Conflict of Interest: None
Chordoma of thoracic vertebras is a very rare locally invasive neoplasm with low grade malignancy arising from embryonic notochordal remnants. Radical surgery remains the cornerstone of the treatment. We describe a case of multifocal T1-T2 chordoma, without bone and disc involvement, incidentally misdiagnosed as a paraganglioma, occurring in a 47-year-old male asymptomatic patient. Neoplasm was radically removed by an endocrine surgeon through a right extended cervicotomy. A preoperative reliable diagnosis of chordoma, as in the reported case, is often difficult. Radical surgery can provide a favorable outcome but, given the high rates of local recurrence of this neoplasm, a strict and careful follow-up is recommended. Although very rare, chordoma should be suggested in the differential diagnosis of the paravertebral cervical masses of unknown origin. Spine surgeon consultation and a FNB should be routinely included in the multidisciplinary preoperative work-up of these neoplasms.
Keywords: Chordoma, paraganglioma, thoracic spine chordoma
|How to cite this article:|
Conzo G, Gambardella C, Pasquali D, Ciancia G, Avenia N, Pietra CD, Napolitano S, Palazzo A, Mauriello C, Parmeggiani D, Pettinato G, Napolitano V, Santini L. Multifocal thoracic chordoma mimicking a paraganglioma. J Can Res Ther 2013;9:497-9
|How to cite this URL:|
Conzo G, Gambardella C, Pasquali D, Ciancia G, Avenia N, Pietra CD, Napolitano S, Palazzo A, Mauriello C, Parmeggiani D, Pettinato G, Napolitano V, Santini L. Multifocal thoracic chordoma mimicking a paraganglioma. J Can Res Ther [serial online] 2013 [cited 2020 Jul 15];9:497-9. Available from: http://www.cancerjournal.net/text.asp?2013/9/3/497/119312
| > Introduction|| |
Chordoma is a slow-growing rare primary bone tumor, arising from embryonic notochordal remnants, highly recurrent, with an incidence assessed as 0.08/100.000 people/year.  Because of the poor response to adjuvant therapies, a wide surgical tumor-free margins resection remains the cornerstone treatment.
We describe a rare case of multifocal T1-T2 chordoma, misdiagnosed as a cervical paraganglioma and surgically treated.
| > Case Report|| |
An asymptomatic 47-year-old male patient underwent a spine computed tomography (CT) for a post-traumatic lumbar (L4) vertebral fracture, showing a solid, hypodense, prevertebral mass between T1-T2, 54 ΄ 30 mm in size, adherent to the posterior wall of the esophagus, with regular margins and a small calcified core inside, without significant enhancement after contrast injection. Bone and disc involvement were not present. An esophageal leyomioma was suspected and an endoscopic ultrasonography (EUS) showed, in the upper part of the posterior mediastinum, a triangular-shaped hypoechoic mass. The lesion was located posteriorly to the esophagus, close to the right side of the vertebral bodies, without relationship with the esophageal wall. It had regular margins with a slight heterogeneous internal texture due to the presence of hyperechoic foci, suspicious for calcifications. A diagnosis of paraganglioma was suggested, and EUS-guided fine needle biopsy (FNB) was not performed. Low-field (O.2T) MRI detected a median and right paramedian prevertebral masses located at level T1-T2, homogeneously hypointense in T1-weighted (w) images and hyperintense in T2-w images, adjacent to the vertebral bodies [Figure 1]. Cervical US examination identified two hypoechoic masses posterior to thyroid, respectively of 38 × 22 mm (right mass) and 39 × 20 mm in size (left mass), consistent with parathyroid neoplasms. The patient refused medical treatments, remained asymptomatic, and 17 months later, was referred to a tertiary endocrine center. Intact parathyroid hormone and calcium levels were normal, and parathyroid 99mTc-sestamibi scintigraphy was negative as well. A multiple endocrine neoplasm (MEN) syndrome was suspected. Blood pressure, chromogranin A, and urinary metanephrines levels were normal, and somatostatin receptor scintigraphy showed no uptake. A further cervico-thoracic CT identified a solid median and paramedian prevertebral masses between T1-T2, 45 × 20 × 35 mm in size, with similar features, already present in the previous CT [Figure 2]. In the differential diagnosis, a cervico-thoracic paraganglioma was considered, and a surgical treatment was indicated with diagnostic purposes. Six months later, through a right extended cervicotomy, along the anterior edge of the sternocleidomastoid muscle, an explorative operation was performed by an experienced endocrine surgeon. Thyroid lobes and parathyroid glands appeared of normal size, volume, and stiffness, without macroscopic nodules. T1 and T2 vertebral bodies were exposed, and we radically dissected out a grayish-white encapsulated huge paramedian lump, 50 mm in size, moderately vascularized, of hard consistence and firm on the right anterior lateral vertebral wall. No hemorrhagic features were present. Subsequently, we totally excised another lump, showing similar features, growing on the left paramedian side, 4 cm in size. Post-operative course was uneventful. A spinal MRI, performed 18 months after surgery, did not identify residual or relapsing disease [Figure 3]. Adjuvant chemo-radio therapy was not administered.
|Figure 1: Sagittal T1 (a), and T2 (b), - weighted MR images showing the right paramedian prevertebral mass at T1-T2 level (arrow)|
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|Figure 2: Contrast-enhanced neck and chest CT showing a solid mass between T1 and T2 (arrow), with regular margins and a small calcified core inside, without significant enhancement after contrast injection|
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|Figure 3: Sagittal T2 – weighted 18-months post-operative MR image, showing complete removal of the prevertebral masses|
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Macroscopically, a right nodule measuring 50 × 30 mm and a left one of 40 × 20 mm, both enveloped, were observed. On cross-section, the nodules appeared grayish-white, translucent, and lobulated. Their texture was soft. The histomorphological appearance [Figure 4] of both lesions showed encapsulated, lobulated architecture for the presence of fibrous septa. The lobules appeared to consist of cells showing an eosinophilic cytoplasm, often vacuolated with a central round nucleus. A low index of proliferation (<1/Hpf) was observed. The immunohistochemical study of the lesions showed positivity for pan-cytokeratin, vimentin, and S-100 protein. These aspects were consistent with a diagnosis of multifocal spine chordoma.
|Figure 4: Lobulated architecture with fibrous septa and (insert) physaliphorous cells - Staining: hematoxylin/eosin; magnification 100x (insert: 630X)|
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| > Discussion|| |
Chordoma is considered locally invasive and, multifocality, as in our case, is reported as well. Cases of metastases are reported,  and survival rate is related to tumor size and grading. The overall median survival rate, for all forms, is of 6.3 years. 
It commonly involves vertebral column: 50% sacral, 35% spheno-occipital, and 15% spinal, arranged such as 61% in lumbar, 11% in thoracic, and 28% in cervical vertebras. Only 30 cases of thoracic chordoma have been identified since 1902.  Symptoms are related to the site of growth and to the size. On computed tomography, chordoma appears heterogeneous, and CT-guided biopsy by trocar allows a correct diagnosis. MRI more accurately identifies tumor margins, and is particularly useful postoperatively and in the long-term follow-up.  In our case, EUS was very suggestive of a paraganglioma, mainly for its location, although EUS features were not specific, and an EUS-guided FNB, probably very useful in obtaining a correct pre-operative diagnosis, was not performed. In this kind of patients, a multiple endocrine neoplasia syndrome must be excluded. , Also, T2w MR images did not allow a certain diagnosis, and the patient was referred to an explorative cervical operation. In the differential diagnosis, a spine surgeon consultation and a guided FNB would have been very useful.
According to the principles outlined in Enneking's classification,  the best results are obtained by wide tumor-free resection margins, often by an en bloc resection, and the extension of margins remains a very important prognostic factor. In any case, radical surgery is still the treatment of choice. Although chordoma is relatively radio-resistant, not operable masses, as well as lesions only partially removed, can be treated by adjuvant radiotherapy. Advances in radiation technology allowed better outcomes with charged particles, protons, helium, carbon, and neon ions.  In cases not suitable for surgery, RT is performed for pain relieving.
It is a common knowledge that no chemotherapy is proved to be effective; however, Neo et al., in 2007, proved anti-cancer activity of Imatinib -Tyrosine Kinase inhibitor.  Although very rare, chordoma should be suggested in the differential diagnosis of paravertebral cervical and thoracic masses of unknown origin.
| > Acknowledgments|| |
Special thanks to Prof Luigi Santini department chair of Department of Anaesthesiologic, Surgical and Emergency Science.
| > References|| |
|1.||Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: Current concepts, management, and future directions. Lancet Oncol 2012;13:e69-76. |
|2.||McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM. Chordoma: Incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control 2001;12:1-11. |
|3.||Fontes R, O'Toole JE. Chordoma of the thoracic spine in an 89-year-old. Eur Spine J 2012;21 Suppl 4:S428-32. |
|4.||Coffin CM, Swanson PE, Wick MR, Dehner LP. An immunohistochemical comparison of chordoma with renal cell carcinoma, colorectal adenocarcinoma, and myxopapillary ependymoma: A potential diagnostic dilemma in the diminutive biopsy. Mod Pathol 1993;6:531-8. |
|5.||Pasquali D, Circelli L, Faggiano A, Pancione M, Renzullo A, Elisei R, et al. CDKN1B V109G polymorphism a new prognostic factor in sporadic medullary thyroid carcinoma. Eur J Endocrinol 2011;164:397-404. |
|6.||Conzo G, Circelli L, Pasquali D, Sinisi A, Sabatino L, Accardo G, et al. Lessons to be learned from the clinical management of a MEN 2A patient bearing a novel 634/640/700 triple mutation of the RET proto-oncogene. Clin Endocrinol (Oxf) 2012.;77:934-6 |
|7.||Chan P, Boriani S, Fourney DR, Biagini R, Dekutoski MB, Fehlings MG, et al. An assessment of the reliability of the Enneking and Weinstein-Boriani-Biagini classifications for staging of primary spinal tumors by the Spine Oncology Study Group. Spine (Phila Pa 1976) 2009;34:384-91. |
|8.||Neo M, Asato R, Honda K, Kataoka K, Fujibayashi S, Nakamura T. Transmaxillary and transmandibular approach to a C1 chordoma. Spine (Phila Pa 1976) 2007;32:E236-9. |
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