Ann Thorac Surg 2009;88:e9-e10. doi:10.1016/j.athoracsur.2009.05.018
© 2009 The Society of Thoracic Surgeons
Case Reports
Pulmonary Metastases From Parachordoma
Filippo Lococo, MDa,*,
Alfredo Cesario, MDa,b,
Elisa Meacci, MDa,
Giacomo Cusumano, MDa,
Stefano Margaritora, MDa
a Department of Thoracic Surgery, Catholic University and Scientific Direction, Rome, Italy
b CdC San Raffaele Velletri and IRCCS San Raffaele Pisana, Rome, Italy
Accepted for publication May 4, 2009.
* Address correspondence to Dr Lococo, Department of Thoracic Surgery, Catholic University, Largo F. Vito n 1, Rome, 00168, Italy (Email: filippo_lococo{at}yahoo.it).
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Abstract
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A young woman was referred to our institution for the appearance of a single pulmonary nodule at a routine follow-up computed tomographic scan of the thorax. She had been operated on 4 years earlier for a parachordoma of the iliopsoas muscle. Wedge resections were accessed through a mini-thoracotomy, and the pathologic examination confirmed the diagnosis of parachordoma lung metastases. Adjuvant chemotherapy (Glivec, 400 mg/daily [imatinib; Novartis, Basel, Switzerland]) was indicated and was administered for 10 months. At the time this case report was written, the patient was alive with no sign of tumor recurrence. We believe that metastatic parachordoma to the lung has been previously reported only once in the English literature. Our case adds evidence that parachordoma, despite its generally indolent behavior, can metastasize to the lung.
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Introduction
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The parachordoma is a rare tumor that usually occurs in the deep-seated soft tissue of the extremities or in the skin. The entity was first described by Laskowski [1] in 1955 with less than 60 overall reported cases until now. The histogenesis of a parachordoma is still uncertain; it is considered an indolent neoplasm with some potential for local recurrence (normally in a range from 3 months to 12 years after surgery, as described in the literature [2]).The metastatic potential of this tumor remains poorly defined, and overall there has been very few cases that have been reported (of these, we could find only one within the lung [3]). We report an additional case of locally aggressive recurrent parachordoma metastatic to the lung.
A 39-year-old woman with long lasting (more than 7 months), gradually increasing, drug-resistant localized pain, presented with a mass in her left hip. Medical, personal, and familiar history were uneventful. The physical examination revealed a large mass accompanied by moderate pain that radiated outward to the left leg. A computed tomographic scan showed the presence of a poor-marginated mass with dishomogeneous and partially calcified areas (6 cm x 5 cm of diameter) within the tissue of the left iliopsoas muscle. In the context of this muscle, the mass showed an infiltrating attitude and was tightly adjacent to the fourth lumbar vertebra without any clear intraforaminal extension. No evidence of abdominal or thoracic metastases was present. A magnetic resonance imaging demonstrated an ill-defined mass with T1 intermediate signal intensity and T2 high-signal intensity. The mass involved the muscular tissue, the tendons, and the neurovascular structures. A whole body radionuclide scan proved negative for secondary bone localizations. Histology was bioptically confirmed as consistent with parachordoma tumor. Common cancer markers (CA125, CA15-3, a-FP e-TPA) were within the normal range. The lesion was surgically removed through a lumbotomic access. At the intraoperative assessment, the tumor was clearly invading the iliopsoas muscle, the genito-femoral nerve, and a collateral branch of the femoral nerve. These structures were dissected "en bloc" with the mass. The definitive pathology confirmed the diagnosis of parachordoma: the tumor was composed of vague nodules of large, rounded eosinophilic cells embedded in a matrix that varied from myxoid to densely hyaline, and the latter areas occasionally resembled primitive cartilage; S-100, vimentin, and CAM 5.2 were strongly positive to immunohistochemical staining and the epithelial membrane antigen (EMA) was slightly positive. A strong expression of CK 8/18 was observed, but not for other cytokeratins (unlike the usual immunohistochemical chordoma pattern). The HHF35 and glial fibrillary acidic protein (GFAP3) were negative too.
A 6-month paced follow-up was planned with 18-fluorodeoxyglucose-positron emission tomographic and computed tomographic scan); the patient did not show any pathologic feature for 3 years. Then an examination with a computed tomographic scan showed the presence of solid tissue (round shape, 1.3 cm maximum diameter) in the right paravertebral region (within the previous surgical field), and a centimetric pulmonary lesion (Fig 1). With the double aim of confirming the clinically suspected diagnosis of metastatic parachordoma, and to eventually assess the real extent of the dissemination of the disease while providing a valuable therapeutic action, the patient underwent a vertical axillary right thoracotomy. The manual palpation of the entire lung led to the discovery of four additional lesions and one enlarged lymph node (intra-scissural). All the lesions were removed by stapled wedge resections. These were localized as following: one in the apical segment of the lower lobe (1 cm x 1 cm), two in the anterior segment of the lower lobe (0.6 cm x 0.5 cm and 0.4 cm x 0.5 cm), and two in the upper lobe (0.5 cm x 0.6 cm and 0.2 cm x 0.3 cm) (Fig 2). The pathologic examination and the immunohistochemical features confirmed the diagnosis of metastatic parachordoma in four of five pulmonary nodules, whereas one nodule of the upper lobe and the intrascissural lymph node appeared nonpathologic. Adjuvant chemotherapy (Glivec, 400 mg/daily [imatinib; Novartis, Basel, Switzerland]) was indicated and administered for 10 months. At the time that this report was written, the patient was alive and well with no sign of tumor recurrence or additional metastatic evidence.
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Fig 2. Intraoperative finding of a pulmonary lesion in the apical segment of the lower lobe (1 cm x 1 cm).
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Comment
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Parachordoma is a rare soft tissue tumor with less than 60 overall reported cases. The entity was first described by Laskowski in 1955 [1] and the name "parachordoma" was proposed by Dabska [4] a few decades later.
This initial period was characterised by a certain degree of ambiguity as to whether parachordoma was a discrete entity, rather than an extraskeletal myxoid chondrosarcoma, an ectopic chordoma, or a variant of some other neoplasm. In truth, the actual number of cases could be less, because the accuracy of some of the diagnoses is suboptimal according to the pattern assessed and reported by some of the authors [5]. Previous articles on this subject, predominantly in the form of case reports, have suggested its origin from ectopic (abaxial) rests of notochord or kinship to Schwann cell tumors and synovial sarcoma. Parachordoma histological, immunohistochemical, and cytogenetical profiles are well established and sufficiently characteristic to clearly differentiate it from chordoma and extraskeletal myxoid chondrosarcoma. Although all three tumors are composed of nodules containing cords of rounded eosinophilic cells, the parachordoma has more architectural and cellular heterogeneity. The parachordoma shows a curious blending of the predominant epithelioid cells with immature spindle cells, and small, glomoid rounded cells. Neither chordoma nor extraskeletal myxoid chondrosarcoma possess the small cell or glomoid component, as is seen in parachordoma instead. A definitive differentiation is thus achieved through an immunohistochemistry approach [5, 6]. The pattern (morphological, immunohistochemical) of the tumor case we have reported herein is entirely consistent with the diagnosis of parachordoma, as is currently prevalently accepted (Fig 3). From an oncological point of view, parachordoma is an indolent neoplasm, but one with a known potential for local recurrence. This is probably due to the fact that small trails of the tumor may be present well outside the main mass. Local recurrences have been reported in the literature, but some reports lack any comment regarding the status of the surgical margins [3, 4, 7, 8] in a range from 3 months to 12 years after surgery [3]. The issue of the metastatic potential in this tumor is still poorly defined. A case of metastatic parachordoma to a subcutaneous lymph node has been reported by Limon and colleagues [8]. In addition, a cutaneous, bony, and pulmonary widespread metastatic case has been reported by Abe and colleagues [3].
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Fig 3. The tumor was histologically characterized mainly by epithelioid cells with immature spindle cells, and small, glomoid rounded cells (hematoxylin & eosin stain, x100).
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Our case, as believed to be the second reported of its kind, adds evidence that parachordoma, despite its generally indolent behavior, can metastasize to the lung.
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References
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- Laskowski J. Zarys onkologiiIn: Kolodziejska H, editor. Pathology of tumors. PZWL; 1955. pp. 91-99.
- Imlay SP, Argnyi ZB, Stone MS, et al. Cutaneous parachordoma. A light microscopic and immunohistochemical report of two cases and review of the literature. J Cutan Pathol 1998;25:279-284.[Medline]
- Abe S, Imamura T, Harasawa A, et al. Parachordoma with multiple metastases J Comput Assist Tomogr 2003;27:634-638.[Medline]
- Dabska M. Parachordoma. A new clinical entity. Cancer 1977;40:1586-1592.[Medline]
- Hirowaka M, Manabe T, Sugihara K. Parachordoma of the buttock: an immunohistochemical case study and review Jpn J Clin Oncol 1994;24:336-339.[Abstract/Free Full Text]
- Fisher C, Miettinen M. Parachordoma: a clinicopathologic and immunohistochemical study of four cases of an unusual soft tissue neoplasm Ann Diagn Pathol 1997;1:3-10.[Medline]
- Ishida T, Oda H, Oka T, et al. Parachordoma: an ultrastructural and immunohistochemical study Virchows Archiv A Pathol Anat 1993;422:239-245.
- Limon J, Babinska M, Denis A, et al. Parachordoma: a rare sarcoma with clonal chromosomal changes Cancer Genet Cytogenet 1998;102:78-80.[Medline]
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Abstract
Introduction
