Fine-Needle Aspiration Biopsy ofMetastatic Chordoma:Case Report and Review of the LiteratureHala Kfoury, M.D., K.S.U.F., F.R.C.P.A., Abdul Haleem, M.B., M.Phil., F.R.C.Path.,*and Anwaar Burgess, M.S.Sc., C.T. (A.S.C.P.), C.M.I.A.C.

Chordoma is a distinct malignant neoplasm arising from theremnants of the notochord and occurring mostly in the fifth or sixthdecade of life, and occupying most frequently the sacral area(Bibbo, Comprehensive Cytopathology 1997; p 534). Metastases ofthe neoplasm may occur in 10–40% of cases (Jenkins et al., Clin.Radiol. 1995;50:416–417). Because of its rarity, the diagnosis ofchordoma may be diffıcult to render, especially on fine-needleaspiration biopsy (FNAB). However, a clear-cut distinction ofchordoma from other neoplasms is of utmost importance, since theprognosis and treatment of the patient will depend on the finaldiagnosis. This distinction in the case of metastases can be madeeasily, where correlation of previous histology has been doneand/or ancillary studies have been performed.Diagn. Cytopa-thol. 2000;22:104–106. r 2000 Wiley-Liss, Inc.

Key Words:chordoma; soft-tissue mass; FNAB

Fine-needle aspiration biopsy (FNAB) of conventional chor-domas has been described, with special attention given to thedifferential diagnoses.1–4 In this article, we will describe thecytomorphologic features of a case of metastatic chordomato the soft tissue of the shoulder. A search of current theEnglish-language literature to date has not revealed areported case of sacral chordoma metastatic to the soft tissueof the shoulder diagnosed on FNAB. The diagnosis in thecurrent case was made after ruling out the other differentialdiagnoses and after comparison with the previous histologyof the original tumor.

Case ReportHistoryThe patient, a 63-yr-old Saudi male, first presented with apresacral mass in 1993. Initial FNAB on this mass wasreported as a myxoid tumor, which was corrected tochordoma after excision biopsy. The fragmented massmeasured in aggregate 93 5 3 2 cm. On current admission

to the Riyadh Armed Forces Hospital, the patient presentedwith a left shoulder mass, which had increased to the presentsize of 10 cm within a few months. FNAB was performed onthis mass. Four separate passes, without negative pressure,were done by a pathologist using a 25-gauge needle. Air-dried and alcohol-fixed slides were made and stained usingmodified Romanowsky (HemaGurry Rapid Blood SmearStaining Set, Poole, England) and Papanicolaou methods,respectively.

FNAB Cytomorphology of the PresentMetastatic ChordomaThe modified Romanowsky- and Papanicolaou-stainedsmears showed a rich myxoid fibrillary background. Themyxoid fibrillary background stained magenta with theformer and gray to green with the latter (Figs. 1, 2). Round tocuboidal cells with occasional vacuolization of the cyto-plasm were seen in this background. A few cells were larger,with abundant and vacuolated cytoplasm (Fig. 3). Otherclusters showed pleomorphic cells with prominent nucleoli(Fig. 4). At this stage, the differential diagnosis includedmetastatic chordoma, myxoid liposarcoma, myxoid chondro-sarcoma, and metastatic mucinous adenocarcinoma; low onthe list was metastatic myxopapillary ependymoma. How-ever, upon reviewing the initial FNAB and the histologyslides of the previous surgery and based on the cytomorphol-ogy findings of the current FNAB, a diagnosis of metastaticchordoma was made. The patient was started on chemother-apy, which resulted in a good response. The patient remainedstable up to the time of the writing of this article.

Review of Previously Excised Presacral ChordomaHistologic FindingsThe tumor was composed of nests and cords of cuboidalcells lying in a myxoid background. There was only anoccasional large ballooned-up multinucleated cell resem-bling the physalipherous cells of chordoma. The tumor alsoinfiltrated the bone trabeculae.

Riyadh Armed Forces Hospital, Riyadh, Saudi Arabia*Correspondence to: Dr. Abdul Haleem, B101-Military Hospital, P.O.

Box 7897, Riyadh 11159, Saudi Arabia.Received 28 April 1999; Accepted 12 August 1999

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ImmunophenotypingImmunophenotyping was carried out by immunohistochem-istry on the formalin-fixed and paraffin-embedded sections.The use of DAKOy (Dako, Ltd., Buckinghamshire, UK)antibodies showed expression of S-100, cytokeratin MNF116,epithelial membrane antigen (EMA), and vimentin in theneoplastic cells. Carcinoembryonic antigen (CEA) showed anegative reaction.

Electron Microscopy StudyElectron microscopy (EM) study of the tumor showedneoplastic cells with large intracytoplasmic vacuoles anddesmosomes. A number of microvilli were also noted.

DiscussionChordoma is a rare malignant neoplasm deriving fromembryonic remnants of the notochord and arising in the axialskeleton.1,2,5 This neoplasm is common in adults, but well-

known cases have been reported in children.7,8 In this article,we present the case of a 52-yr-old male who presentedrecently with a soft-tissue mass of the left shoulder.

According to the current English-language literature, oneof the cytomorphologic features of chordoma is the presenceof a rich myxofibrillary background within which round orcuboidal cells are seen, either arranged in cohesive clustersor scattered individually. The most important diagnosticfeature is the presence of occasional large, ballooned-upphysalipherous cells.1,4,8–10 These cells characteristicallyshow multiple bubble-like cytoplasmic vacuoles.7,8

The differential diagnosis on FNAB of chordoma includesneoplasms with myxoid background. The most importantentities are myxoid chondrosarcoma, myxoid liposarcoma,metastatic mucinous adenocarcinoma, and myxopapillaryependymoma.5 Myxoid chondrosarcoma and myxoid liposar-coma share the cytomorphology of myxoid background.

Fig. 1. Rich myxoid fibrillary background and clusters of cells with clearcytoplasm (air-dried, Romanowsky stain,3200).

Fig. 2. FNAB smear, showing clusters of chordoma cells (alcohol-fixed,Papanicolaou stain,3100).

Fig. 3. Higher magnification of Figure 2. Multivacuolation of the phy-salipherous cell cytoplasm is highlighted (air-dried, Romanowsky stain,3400).

Fig. 4. In other areas of the FNAB smears are found pleomorphic cells withprominent nucleoli set in the myxoid fibrillary background (air-dried,Romanowsky stain,3400).

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However, the former lacks the presence of physalipherouscells, and the latter shows a prominent plexiform capillarynetwork as well as lipoblasts or spindle or oval nuclei withsyncytial cytoplasm.9,11 Metastatic mucinous adenocarci-noma may have the signet-ring type of cells as a differentiat-ing feature, while myxopapillary ependymoma would havepapillae of ependymal cells, surrounded by myxoid mate-rial.4,9,10,12

Immunohistochemistry and EM examination would alsohelp to differentiate between these neoplasms.3,4,7,9Cytokera-tin would be positive in chordoma and metastatic mucinousadenocarcinoma, while myxopapillary ependymoma andmyxoid chondrosarcoma are negative. On the other hand,S-100 protein is positive in all except metastatic mucinousadenocarcinoma. Ultrastructurally intermediate cytofila-ments, microvilli, and well-formed desmosomes are seen inchordoma.4,5,7 Epithelial differentiation is prominent in mu-cinous adenocarcinoma. Myxoid liposarcoma shows lipidvacuoles in the cytoplasm of tumor cells. Cilia may bedemonstrated in myxopapillary ependymoma.8

Chordoma due to its rarity may be difficult to differentiatefrom other neoplasms with a similar myxoid fibrillarybackground. Ancillary studies such as immunocytochemis-try and electron microscopy, which demonstrate distinctimmunological and electron microscopy profiles, can help inthe diagnosis of chordoma.4,13 The present article illustratesthat FNAB of a patient with a known chordoma should poseno difficulty in diagnosis, provided the previous histology isavailable for comparison and review.

References1. Gupta RK, Arora R, Vashistha R. Chordoma metastatic to the breast

diagnosed by fine needle aspiration. A case report. Acta Cytol1997;41:910–912.

2. Naka T, Fukuda T, Chuman H, Iwamoto Y, Sugioka Y, Fukui M,Tsuneyoshi M. Proliferative activities in conventional chordoma: aclinicopathologic, DNA flow cytometric, and immunohistochemicalanalysis of 17 specimens with special reference to anaplastic chordomashowing a diffuse proliferation and nuclear atypia. Hum Pathol1996;27:381–388.

3. Plate KH, Bittinger A. Value of immunocytochemistry in aspirationcytology of sacrococcygeal chordoma A report of two cases. Acta Cytol1992;36:87–90.

4. Walaas L, Kindblom L. Fine-needle aspiration biopsy in the preopera-tive diagnosis of chordoma: a study of 17 cases with application ofelectron microscopic, histochemical, and immunocytochemical exami-nation. Hum Pathol 1991;22:22–28.

5. Bibbo M. Comprehensive cytopathology. Philadelphia: W.B. SaundersCo.; 1997. p 944–945.

6. Caballero C, Fontaniere B. Sacrococcygeal chordoma: fine needleaspiration cytological findings and differential diagnosis. Cytopathol-ogy 1993;4:311–313.

7. Ali SZ, Semmelmeier SB, Urmacher C. Cytology of cervical chordomain cerebrospinal fluid from a child. A case report. Acta Cytol1995;39:766–769.

8. Nijhawan VS, Rajwanshi A, Das A, Jayaram N, Gupta SK. Fine-needleaspiration cytology of sacrococcygeal chordoma. Diagn Cytopathol1989;5:404–407.

9. Hazarika D, Kumar RV, Muniyappa GD, Mukherjee G, Rao CR,Narasimhamurthy NK, Shenoy AM, Namjundappa. Diagnosis of clivalchordoma by fine needle aspiration of an oropharyneal mass. A casereport. Acta Cytol 1995;39:507–510.

10. Hughes DE, Lamb J, Salter DM, Al Nafussi A. Fine-needle aspirationcytology in a case of chordoma. Cytopathology 1992;3:129–133.

11. Attal H, Jensen J, Reyes CV. Myxoid liposarcoma of the anteriormediastinum diagnosis by fine needle aspiration biopsy. Acta Cytol1995;39:511–513.

12. Kline MJ, Kays DW, Rojiani AM. Extradural myxopapillary ependy-moma: report of two cases and review of the literature. Pediatr PatholLab Med 1996;16:813–822.

13. Gonidi M, Athanassiadou P, Petrakakou E, Zerva CH, Dimou S.Extraskeletal myxoid chondrosarcoma: a case report with fine needleaspiration (FNA) cytology. Cytopathology 1997;8:130–133.

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