Ann Thorac Surg 2003;75:566-568
© 2003 The Society of Thoracic Surgeons
Case report
Liposarcoma of the thyroid gland mimicking retrosternal goiter
Wael I. Awad, FRCSa,
Peter H. Rhys Evans, FRCSb,
Andrew G. Nicholson, FRCPathc,
Peter Goldstraw, FRCS*a
a Department of Thoracic Surgery, Royal Brompton Hospital, London, England, United Kingdom
c Department of Histopathology, Royal Brompton Hospital, London, England, United Kingdom
b Department of Head and Neck Surgery, Royal Marsden Hospital, London, England, United Kingdom
Accepted for publication July 31, 2002.
* Address reprint requests to Mr Goldstraw, Department of Thoracic Surgery, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK.
e-mail: pgoldstraw{at}rbh.nthames.nhs.uk
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Abstract
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Primary liposarcoma of the thyroid gland is extremely rare with only two previous reports in the literature. We report two further cases, both patients presenting with rapid airways compression. Patient 1 had clinical, radiographic, and biopsy appearances suggesting benign goiter. Patient 2 had a long-term history of benign goiter, a previous partial thyroidectomy, and more recent biopsies showing liposarcoma. The management of such rare conditions is always challenging.
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Introduction
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Liposarcoma of the thyroid gland is exceedingly rare. It may mimic a retrosternal goiter in its presentation and thus represents an important pitfall for surgeons treating patients who have the clinical features of goiter.
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Case reports
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Patient 1
A 50-year-old woman presented with increasing swelling of the right side of her neck, dyspnea, and dysphagia for more than 3 months. She had a partial thyroidectomy 15 years previously for multinodular goiter. Biopsy of the thyroid mass revealed a myxoid liposarcoma. Within a week she developed stridor and the swelling had become painful. Chest radiograph showed a retrosternal mass and tracheal compression. A subsequent computed tomography (CT) scan of the thorax confirmed a large thyroid mass causing deviation and compression of her trachea and extending down to the aortic arch. Complete surgical excision was thought to offer the best chance of relieving the obstruction.
Rigid bronchoscopy revealed an immobile right vocal cord and external compression of the mid third of the trachea. The mucosa was intact. The previous thyroidectomy scar was then reopened and a vertical extension was carried down over the manubrium. A J-shaped sternotomy was carried out into the right second in-terspace. The tumor was dissected free from the internal jugular vein. The recurrent laryngeal nerve was encased in the tumor and was sacrificed. The tumor was then carefully dissected from the right pyriform fossa musculature, where there appeared to be invasion, and also the upper esophageal muscular wall. The tumor was delivered into the neck from the superior mediastinum and was completely removed with the residium of the thyroid gland. The tumor measured 12 x 7 x 5 cm and had a white lobulated appearance. Microscopically the tumor was a pleomorphic grade II liposarcoma extending to the margins of excision.
The postoperative course was uneventful and the patient had no dysphagia or breathing problems. She underwent radiotherapy to the neck 6 weeks later and remained well for about 8 months and then cutaneous, osseus, pulmonary, and hepatic metastases developed. The patient underwent a course of chemotherapy without response and radiotherapy to the bone lesions. She died 9 months after surgery.
Patient 2
A 71-year-old man presented with a 1-month history of shortness of breath, hoarse voice, and dysphagia to solids associated with neck swelling. His chest radiograph showed tracheal compression by a retrosternal mass (Fig 1A).
A cervical biopsy of this mass before referral had shown a benign goiter. All hematologic and biochemical values were normal. He was referred for urgent thyroidectomy but his symptoms had worsened with stridor and swelling of his left arm. CT scans with contrast showed occlusion of the left internal jugular and left subclavian veins in addition to a large mass extending from the region of the thyroid into the mediastinum, suggestive of a goiter (Fig 1B). The trachea was shifted to the right and externally compressed by this mass. The patient was given anticoagulation therapy with heparin and was assessed for surgery.
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Fig 1. (A) Chest radiograph demonstrating a large retrosternal mass deviating and constricting the trachea in patient 2. (B) Computed tomography chest scan demonstrating the liposarcoma in patient 2 with gross tracheal and esophageal compression.
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Rigid bronchoscopy revealed compression of the upper trachea from the left lateral aspect. The mass, approached through a transverse cervical incision, arose from the inferior aspect of the left lobe of the thyroid gland and was tightly wedged in the superior mediastinum. Posteriorly the mass was invading the wall of the cervical esophagus. The mass was retracted into the neck without the need for a sternotomy and a left hemithyroidectomy was performed. The mass was lobulated and measured 5 x 7 x 5.5 cm. The cut surface was firm and gray-yellow in color. Microscopically the tumor was classified as a liposarcoma of pleomorphic type (Fig 2).
There was minimal invasion into the thyroid parenchyma.
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Fig 2. A pleomorphic liposarcoma containing abundant lipoblasts (long arrows) and atypical mitoses (short arrow), diffusely infiltrates between entrapped thyroid follicles.
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The postoperative course was uncomplicated. The patient received anticoagulation therapy for 8 weeks for the subclavian vein thrombosis. He received radical postoperative radiotherapy to both sides of the neck and the superior mediastinum over a 6-week period commencing 5 weeks after surgery and he remained euthyroid. The patient was alive and well at 6-month follow-up without any sign of disease and with no problems with swallowing or beathing.
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Comment
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Retrosternal goiter may present with compression syndrome when the most common complaints are dyspnea and wheeze due to tracheal compression, hoarseness, and dysphagia. They rarely cause superior vena cava syndrome and when that occurs, it is usually indicative of malignancy.
Liposarcoma is the most common soft tissue sarcoma and accounts for 20% of all mesenchymal malignancies. Liposarcomas can be divided into several subtypes: well differentiated, myxoid, round cell, pleomorphic, and dedifferentiated, with the latter representing a distinct type of liposarcoma in which transition from low-grade to high-grade nonlipogenic morphology within a well-differentiated liposarcoma is observed.
Liposarcoma of the thyroid gland is an extremely rare malignancy. Review of the literature reveals only three previous reports—two of primary liposarcoma [1, 2] and one of a liposarcoma that arose 12 years after high-dose radiotherapy for lymphoepithelioma of the nasopharynx [3]. These neoplasms are often voluminous at the time of diagnosis and thus become symptomatic by compression of nearby structures. In both of our cases the tumors were quite large and the development of stridor was of acute onset. That reflects the rapid growth of these tumors although hemorrhage—both spontaneous and associated with a biopsy—were also considered in our cases.
There are few studies that give the results of therapy for thyroid liposarcoma. Complete surgical excision in addition to relieving compression symptoms and establishing a histologic diagnosis provides the most effective local treatment for liposarcomas of the head and neck with an overall 5-year survival of 67% [4]. Local recurrence rates are as high as 44% at 3 years [5] and adjuvant radiotherapy or chemotherapy would seem reasonable. However in most series the use of adjuvant therapy has been reserved for patients with incomplete excision.
The prognosis of liposarcomas depends on tumor characteristics, especially histologic subtype and tumor grade and location [4–6]. The prognosis for patients with liposarcomas in the head and neck appears to be better than for those with liposarcomas arising elsewhere, particularly in the peritoneum, although that may reflect earlier presentation.
Our cases illustrate the problems associated with the management of liposarcoma of the thyroid gland. This rare condition represents an important pitfall for surgeons treating patients with the radiographic features of a goiter. The development of superior vena cava obstruction, recurrent laryngeal nerve palsy, and rapid growth should alert the clinician to something more sinister. Surgery was of value in both our cases.
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References
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- Nielsen V.T., Knudsen N., Holm I.E. Liposarcoma of the thyroid gland. Tumori 1986;72:499-502.[Medline]
- Andrion A., Gaglio A., Dogliani N., Bosco E., Mazzucco G. Liposarcoma of the thyroid gland. Fine-needle aspiration cytology, immunohistology, and ultrastructure. Am J Clin Pathol 1991;95:675-679.[Medline]
- Griem K.L., Robb P.K., Caldarelli D.D., Templeton A.C. Radiation-induced sarcoma of the thyroid. Arch Otolaryngol Head Neck Surg 1989;115:991-993.[Abstract/Free Full Text]
- Golledge J., Fisher C., Rhys-Evans P.H. Head and neck liposarcoma. Cancer 1995;76:1051-1058.[Medline]
- Dudhat S.B., Mistry R.C., Varughese T., Fakih A.R., Chinoy R.F. Prognostic factors in head and neck soft tissue sarcomas. Cancer 2000;89:868-872.[Medline]
- Nijhuis P.H., Sars P.R., Plaat B.E., Molenaar W.M., Sluiter W.J., Hoekstra H.J. Clinico-pathological data and prognostic factors in completely resected AJCC stage I-III liposarcomas. Ann Surg Oncol 2000;7:535-543.[Abstract]
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Abstract
Introduction
