Eur J Cardiothorac Surg 1999;16:573-575
© 1999 Elsevier Science NL
Chest wall parachordoma
Josep M. Gimferrera,
Xavier Baldob,
Carlos A. Monteroa,
Jose Ramirezc
a Department of Thoracic Surgery, Hospital Clínic, University of Barcelona, C/Villarroel 170, 08036 Barcelona, Spain
b Department of Thoracic Surgery, Hospital de Girona ‘Dr. Josep Trueta’, Girona, Spain
c Department of Pathology, Hospital Clínic, University of Barcelona, Barcelona, Spain
Corresponding author. Tel.: +34-93-227-5400; fax: +34-93-227-5431
e-mail: jmgimferr{at}medicina.ub.es
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Abstract
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A 21-year-old woman with a painful chest wall mass was found to have a parachordoma (PC). The tumor arose from the fifth intercostal space. A wide chest wall resection including the tumor and a 2.5 cm free margin and the subsequent reconstruction with a Gore-Tex® soft tissue patch covered with a latissimus dorsi rotational flap was performed. To our knowledge, chest wall parachordoma has not been previously reported in the medical literature.
Key Words: Parachordoma • Chest wall tumor • Chest wall resection
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1. Introduction
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Parachordoma (PC) was first reported by Dabska in 1977 [1,2], and represents an extremely uncommon soft tissue tumor consisting of cells with histology and ultrastructure similar to those of chordoma cells but with immunohistochemistry similar to that of chondroid tumor cells [3,4]. It is most frequently located in the upper or lower extremities [4,5]. Here we describe a recurrent PC originating in the anterior chest wall.
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2. Case report
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A 21-year-old woman, noted a painful anterior chest wall mass arising over the scar developed after the surgical excision of a 1-cm chest wall tumor 10 years earlier. No information with regard to the pathological diagnosis was available. Physical examination showed a firm 3-cm tumor mass extending over the fifth anterior left intercostal space, firmly adhered to the rib on palpation without adherence to the skin.
Computed tomography of the chest confirmed a well defined homogeneous soft tissue density tumor without signs of rib erosion or pulmonary connection. Percutaneous needle biopsy yielded the possible diagnosis of local parachordoma recurrence. At surgery, a well circumscribed mass attached to the 5th and 6th ribs in the middle axillary line was found. The skin was not involved. After opening of the pleura, the tumor was found to be protruding over the pleura but there were no signs of local infiltration. The lung parenchyma was also free. The tumour and a 2.5 cm free margin was resected. The whole resected sample included 10 cm of the 5th and 6th ribs, and 4th and 6th intercostal muscles. The 10x9 cm anterior chest wall defect was reconstructed with a 18x12 cm Gore-Tex® soft-tissue patch, sutured over the costal plane, and covered with a latissimus dorsi rotational flap. The postoperative course was uneventful and the patient was discharged on the seventh postoperative day. Four years after the operation the patient is leading a normal active life with no signs of tumour recurrence.
2.1. Pathological findings
The tumour size was 5x4x3 cm with signs of rib invasion. The lesion consisted in a polilobulated infiltrative tumour with mixoid and chondroid areas (Figs. 1 and 2). Immunohistochemical studies were positive for S-100 protein, vimentin and low molecular weight keratins (cam 5.2).
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Fig. 2. High magnification of nests of irregular cells, without atypia growing in a myxoid matrix. H&E 250x.
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3. Discussion
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Parachordoma is a very rare soft tissue neoplasm most often arising from the upper or lower extremities [4,5] Although it is considered to be a benign lesion, PC may be locally aggressive and can infiltrate the surrounding tissues [1,4,5]. To our knowledge no previous cases of chest wall PC have been reported in the English literature. Symptoms are rare and they result mainly from the local mass effect of tumour growth on the surrounding structures or erosion of bony tissues [1,4,5].
Complete resection of the tumour with a clear free surgical margin can be considered as a curative treatment [6–10]. We performed a wide chest wall resection with a 2.5-cm free margin because it was a recurrent tumoral lesion and intraoperative pathological examination could not identify the exact nature of the lesion. In our patient the first operation was performed under local anesthesia. To our belief that could represent an incomplete excision and this was the reason of the late local recurrence.
Clinical differential diagnosis has to be made with chondrosarcoma. Fibrous dysplasia is usually not painful but it may grow larger leading to compression of the surrounding structures. Pathological differential diagnosis has to be made with chordoma, extraskeletal myxoid chondrosarcoma and subcutaneous sacrocoxccygeal myxopapillary ependymoma [1,3–5]. Both morphological criteria and inmunohistochemistry are essential for differential diagnosis. Positive results for keratins, as in our case, has also been discussed in the literature [11].
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References
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Received May 3, 1999;
received in revised form July 16, 1999;
accepted August 24, 1999.
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Abstract
1. Introduction
