Fine-needle aspiration of a chordoma and its recurrence 19 years after

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Fine-Needle Aspirationof a Chordoma and itsRecurrence 19 Years AfterDear Dr Bedrossian:Chordoma is an uncommon malignant mesenchymal tu-mor of fetal notochord origin that occurs exclusivelyalong the spinal axis, with a predilection for the sacrum,base of the skull, and, occasionally, the mobile spine.1 Itusually occurs in the fth to seventh decades and shows amale predominance. Although it is a slow-growing neo-plasm that recurs locally if incompletely excised, distantmetastasis may occur in late stages of the disease. Betterpatient survival is achieved with complete surgical exci-sion of the primary tumor, currently the only curativeform of treatment.2 The clinical features are related to thelocation and spread of the neoplasm; usually, as a slowgrowing-tumor, chordoma produces nonspecic symptomsfor months to years before the diagnosis is made. Charac-teristically, the most frequent symptoms are pain referredto the lower back and nerve dysfunctions such as anesthe-sia and paresthesia as late manifestations.3 Patients tendto live for a prolonged time but have signicant morbiditybecause of neurologic problems. The most common radio-graphic appearance was a solitary, lytic destructive mid-line mass, often with lobulations.4 Chordomas are dividedinto three subtypes, based on microscopic morphology:(1) conventional, (2) chondroid, and (3) dedifferentiated.Chondroid and dedifferentiated chordomas account forless than 5% of all chordomas.5 Macroscopically, they aresoft, tan, myxoid masses that frequently show areas ofhemorrhage. Patients with chondroid chordomas have alonger survival than those with conventional chordoma,whereas patients with dedifferentiated chordoma have avery poor prognosis.5 The natural history of the dediffer-entiated chordoma is unknown. Some researchers6 sug-gested that the dedifferentiated component arose denovo in conjunction with conventional chordoma and usu-ally portends an accelerated clinical course, but otherresearchers7 reported a dedifferentiated chordoma arisingin an irradiated sacral conventional chordoma, suggestingthe possible malignant transformation of chordoma.In this article we describe the cytomorphological fea-tures of a conventional chordoma and its local recurrenceas a dedifferentiated chordoma 19 yr after the diagnosis.In both cases the diagnosis was made using ne-needleaspiration biopsy, which was conrmed after excisionalbiopsy.At the rst diagnosis in 1986, the patient (a 53-year-oldman) presented a sacrococcygeal mass with extension intothe adjacent soft tissue. X-ray studies showed an aggres-sive mass with osteolytic areas. Initial ne-needle aspira-tion (FNA) on this mass was reported as a conventionalchondroma, which was conrmed after excision biopsy.Multiple Diff-Quik-stained smears showed high cellular-ity, with classic physaliphorous cells arranged in clustersand single cells in a brillary, myxoid background. This*Correspondence to: Ana Saiz, M.D., Departamento de AnatomaPatologica, Hospital Doce de Octubre, Avenida de Codoba s/n, 28041Madrid, Spain. E-mail: vmpozuelo@hotmail.comDOI 10.1002/dc.20472Published online in Wiley InterScience ( 1. Cytomorphology of a conventional chordoma: classic physalipho-rous cells arranged in clusters and single cells in a brillary, myxoid,metachromatic background (MGG, 340). [Color gure can be viewed inthe online issue, which is available at]' 2006 WILEY-LISS, INC. Diagnostic Cytopathology, Vol 34, No 9 663myxoid stroma showed the distinctive, but nonspecic,metachromatic staining (Fig. 1). At the time of the origi-nal histopathological diagnosis, there were no cellularpleomorphisms, multinucleation, nuclear holes, or mito-ses. These features have been described as markers ofatypical and aggressive tumors.8,9 Papanicolaou (Pap)-stained smears showed the same features. Immunocyto-chemical studies were performed on one Pap smear. Thetumor cells were positive for cytokeratin CAM 5.2 imu-nostaining. The patient was free of disease for 19 yr,when it recurred in the same location. New FNA wasmade, with the diagnosis of dedifferentiated chordoma. Inthis case the Diff-Quik-stained smears showed high cellu-larity with intense pleomorphism, cells with nucleoli andintranuclear cytoplasmic inclusions resulting in nuclearholes. Typical phisaliphorous cells were uncommon, andwhen present, usually appeared as single cells. Abundantbrillary, mixoid, metachromatic background was presentin all smears. Pap-stained smears showed the same fea-tures (Fig. 2).According to the current English literature, the mostimportant cytomorphologic diagnostic feature of chor-doma is the presence of occasional large physalipherouscells.4,59 Other cytomorphological features are the pres-ence of a rich myxobrillary background within cuboidalcells arranged in cohesive clusters or scattered individu-ally.4,59 The common cytological differential diagnosesof chordoma include myxochondrosarcoma, myxoliposar-coma, myxopapillary ependimoma, alveolar soft-tissuesarcoma, and metastatic clear-cell malignancies fromother primary sites.5,9 At this point immunocytochemistrywould help us: cytokeratin (AE-1/AE-4; CAM 5.2) wouldbe positive in chordoma and metastatic mucinous adeno-carcinoma and negative in the other differential diagnoses,and on the other hand S-100 would be positive in allexcept metastatic mucinous adenocarcinoma.5To the best of our knowledge, this is the rst case inthe literature of a conventional chordoma with a recur-rence 19 years after with pleomorphic cytology, resultingin the diagnosis of a dedifferentiated chordoma.Ana Saiz, M.D.*Department of PathologyHospital Doce de OctubreMadrid, SpainPedro de Agustn, Ph.D., F.I.A.C.Andres Perez Barrios, M.D.Nuria Alberti, M.D.Division of CytopathologyDepartment of PathologyHospital Doce de OctubreMadrid, SpainReferences1. Dorfman HD, Czerniak B. Chordoma and related lesions. In: Bonetumors. St. Louis, MO: Mosby; 1998. P 9741007.2. Mirra JM, Nelson SD, Della Rocca C, Mertens F. Chordoma. In:Fletcher C, Unni K, Mertens F, editors. World Health Organizationclassication of tumours: Tumours of soft tissue and bone. Lyon,Paris: IARC Press; 2002. p 316317.3. Kay PA, Nascimento AG, Krishnan Unni K, Salomao DR. Chor-doma. Cytomorphologic ndings in 14 cases diagnosed by ne nee-dle aspiration. Acta Cytol 2003;47:202208.4. Crapanzano JP, Ali SZ, Ginsberg MS, Zakowski MF. Chordoma. Acytologic study with histologic and radiologic correlation. Cancer(cancer cytopathol) 2001;93:4051.5. Kfoury H, Haleem A, Burgess A. Fine-needle aspiration biopsy ofmetastatic chordoma: Case report and review of the literature. DiagnCytopathol 2000;22:104106.6. Meiss JM, Raymond AK, Evans HL, Charles RE, Giraldo AA.Dedifferentiated chordoma. A clinicopathologic and immunohisto-chemical study of three cases. Am J Surg Pathol 1987;11(7):516525.7. Ikeda H, Honjo J, Sakurai H, Mitsuhashi N, Fukuda T, Niibe H.Dedifferentiated chordoma arising in irradiated sacral chordoma.Radiat Med 1997;15(2):109111.8. Waalas L, Kindblom LG. Fine-needle aspiration biopsy in the preop-erative diagnosis of chordoma: A study of 17 cases with applicationof electron microscopic, histochemical, and immunocytochemical ex-amination. Human Pathol 1991;22:2228.9. Plaza JA, Ballestin C, Perez-Barrios A, Martinez MA, de Agustin P.Cytologic, cytochemical, immunocytochemical and ultrastructuraldiagnosis of a sacrococcygeal chordoma in a ne-needle aspirationbiopsy specimen. Acta Cytol 1998;33(1):8992.Fig. 2. Cytomorphology of a dedifferentiated chordoma: high cellularitywith intense pleomorphism, cells with nucleoli and intranuclear cytoplas-mic inclusions resulting in nuclear holes. Scattered phisaliphorous cells(Papanicolaou, 340). [Color gure can be viewed in the online issue,which is available at]SAIZ ET AL.664 Diagnostic Cytopathology, Vol 34, No 9Diagnostic Cytopathology DOI 10.1002/dc


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