Ann Thorac Surg 2006;81:2289-2291
© 2006 The Society of Thoracic Surgeons
Case report
Mediastinal and Pulmonary Metastases of Malignant Ossifying Fibromyxoid Tumor
Kotaro Suehiro, MD
a
,
Patrick Pritzwald-Stegmann, MD
a
,
Kenneth M.L. Lee, MBChB
b
,
Hoon Heng Teoh, MBBS, FRCPA
b
,
Peter M. Alison, MD, FRACS
a
,
*
a Cardiothoracic Surgical Unit, Auckland City Hospital, Auckland, New Zealand
b Department of Anatomical Pathology, Auckland City Hospital, Auckland, New Zealand
Accepted for publication July 25, 2005.
* Address correspondence to Dr Alison, Cardiothoracic Surgical Unit, Auckland City Hospital, Main Building Level 4, Room 43, Park Rd, Auckland, 1001 New Zealand (Email: petera{at}adhb.govt.nz).
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Abstract
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Ossifying fibromyxoid tumor is usually a benign tumor. However some of these tumors with histologic and clinical evidence of malignancy have also been reported and little information is available regarding surgery for metastatic ossifying fibromyxoid tumor. We present a case involving extensive excision of a huge metastatic ossifying fibromyxoid tumor occupying the upper mediastinum and upper half of the right hemithorax.
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Introduction
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Ossifying fibromyxoid tumor (OFMT) is usually a painless well-circumscribed, benign tumor in the subcutis or muscle. However some tumors behave in a malignant manner and cause death. Because it is rare, little information is available regarding surgery for metastatic OFMT. We present a patient who underwent excision of mediastinal and pulmonary metastasis of the OFMT.
A 38-year-old woman presented with wheeze, productive cough, and early signs of superior vena cava obstruction that worsened in 1 month. Six years previously she had what was initially believed to be a lipoma, which was excised on three occasions by her family physician, with subsequent wide local excision demonstrating a non-ossifying variant of an OFMT and 3 years of follow-up. Chest roentgenogram and computed tomographic scan showed a large heterogeneous mass that filled the upper two thirds of the right hemithorax, displacing the great vessels to the left and compressing the trachea and superior vena cava (Figs 1A, 1B). The only blood test abnormality was a borderline elevated alkaline phosphatase (122 U/L). The histology results of an ultrasound-guided core biopsy were similar to that of the original scalp lesion, which showed an atypical OFMT of overall low cellularity and 5 to 6 mitotic figures per 10 high power fields.
In light of the overall low cellularity on core biopsy of the tumor, it was believed that there was little role for radiotherapy and very limited information available in the literature to suggest effective chemotherapy. Therefore surgical management was pursued. The tumor was approached through a median sternotomy with a lateral extension of the incision into the right fourth intercostal space and a small extension into the right lateral lower neck. An extensive, soft, encapsulated tumor with a largely necrotic center surrounded both the innominate veins and the superior vena cava with probable invasion into the pericardium. The tumor also extended posteriorly onto the trachea and to its right lateral border invading the right upper lobe of the lung.
The tumor was first grossly cleared from the mediastinum preserving the superior vena cava and innominate vein. Most of the right lateral pericardium involving the right phrenic nerve was removed. Although it had been the initial intention to preserve the right lower and middle lobes, bleeding became very vigorous, and therefore the pulmonary artery and both veins were taken sequentially, which enabled removal of the mass and the whole right lung as a confluent mass. The heavy blood loss seemed to be a combination of the extensive and vascular nature of the tumor upon entering the capsule, and the venous obstruction caused by the tumor itself. In total, 32 units of red blood cells in addition to fresh frozen plasma, platelets, cryoprecipitate, and factor VIIa were administered intraoperatively. The right pericardium was reconstructed using a 2-mm polytetrafluoroethylene sheet to prevent herniation of the heart.
Macroscopically the tumor measured 180 mm in maximum aggregate and was multinodular with a smooth gelatinous cut surface that abutted the pleural surface. Microscopically the tumor had isolated areas of hypercellularity and was arranged in sheets separated by thin fibrovascular septa with occasional hyalinized areas. The tumor displayed moderate to marked nuclear pleomorphism, abundant apoptotic cells, and a mitotic count of 5 mitoses per 10 high power fields (Fig 2). Lymphovascular channel invasion was also noted. There were no osteoid or convincing myxoid formations seen. Vimentin immunohistochemical stain was positive for 50% of the tumor cells, whereas neuron specific enolase and S100 were focally and moderately positive, respectively. Glial fibrillary acidic protein, pancytokeratin and desmin were all negative. The presence of high nuclear grade or high cellularity and mitoses of greater than 2 mitoses per 10 high power fields classified this a malignant ossifying fibromyxoid tumor [1].
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Fig 2. Malignant ossifying fibromyxoid tumor. High power shows the tumor is hypercellular, containing pleomorphic nuclei with abundant apoptotic cells and mitoses.
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Preoperative symptoms related to mechanical compression of the superior vena cava, right lung, and trachea essentially resolved immediately postoperatively. Her hospital stay was prolonged due to wound healing problems, which were managed using vacuum dressings and delayed closure by secondary intention. At 5 months postoperatively, two thirds of the way through her course of radiotherapy for likely residual mediastinal disease, she was readmitted with seizures related to multiple brain metastasis demonstrated on urgent computed tomographic scan. Multiple lung metastases in the opposite lung were also noted at 6 months postoperatively. She died 8 months after surgery, after a further course of radiotherapy to the chest.
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Comment
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Since the first report by Enzinger and colleagues [2], approximately 170 cases of OFMT have been reported. However, only two detailed reports are available regarding surgical excision of metastatic OFMT. Kilpatrick and colleagues [3] reported lung metastasis discovered 25 months after surgery for a primary hip lesion. Five months after excision, this patient was alive without further tumor. The other case reported by Nishio and colleagues [4] presented with multiple bilateral pulmonary and thigh metastases 3 years after right foot OFMT excision. Despite re-excision followed by chemotherapy, this patient had pulmonary recurrence and renal metastasis develop, and then died 3 years after surgery.
Ossifying fibromyxoid tumor is often described as a benign tumor, but atypical and malignant variants have been described [1]. This case clearly represents an atypical OFMT that behaved in an aggressive fashion leading to death from metastasis. Importantly the tumor had areas of hypercelluarity both at first diagnosis and subsequent removal of metastases, increased mitotic figures per high power fields, and some nuclear atypia described by Folpe and Weiss [1] as risks for metastasis. However, when comparing our findings to the histologic features of the cases previously mentioned, there does not seem to be a consistent pattern. For example, Kilpatrick noted hypocellularity with scarce mitotic figures. Therefore this case further indicates that OFMT should not be considered a simple benign lesion, but one that may have serious ramifications for patients apart from local recurrence. This case has demonstrated that a computed tomographic head scan should be included as part of complete preoperative staging. Indications for surgical excision remain controversial, and the roles of radiotherapy or chemotherapy, or both, remain ill defined; however it was believed that in this instance surgery was a reasonable option due to the enormous size of the tumor and its associated symptoms in a young woman.
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References
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- Folpe AL, Weiss SW. Ossifying fibromyxoid tumor of soft partsa clinicopathologic study of 70 cases with emphasis on atypical and malignant variants. Am J Surg Pathol 2003;27:421-431.[Medline]
- Enzinger FM, Weiss SW, Liang CY. Ossifying fibromyxoid tumor of soft parts. A clinicopathological analysis of 59 cases Am J Surg Pathol 1989;13:817-827.[Medline]
- Kilpatrick SE, Ward WG, Mozes M, Miettinen M, Fukunaga M, Fletcher CD. Atypical and malignant variants of ossifying fibromyxoid tumor. Clinicopathologic analysis of six cases Am J Surg Pathol 1995;19:1039-1046.[Medline]
- Nishio J, Iwasaki H, Ohjimi Y, et al. Ossifying fibromyxoid tumor of soft partscytogenetic findings. Cancer Genet Cytogenet 2002;133:124-128.[Medline]
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Abstract
Introduction
