Joint Program in Nuclear Medicine

Hurthle Cell Tumor

Kavitha Vadde, MD
J. Stevan Nagel, MD

November 23, 1999

Presentation

A 57 year old white female presented with a 5 cm right thyroid mass. Fine needle aspiration cytology was consistent with Hurthle cell tumor.

Imaging Technique

Imaging Findings

Octreotide scintigraphy shows uptake in the right anterior neck in the region of the right lobe of the thyroid (shown by arrow).

Follow up

Total thyroidectomy was performed. Pathology showed a 3.5 cm colloid nodule with Hurthle cell change and nodular hyperplasia (goiter).

Discussion

Hurthle cell tumor is an uncommon and controversial thyroid neoplasm. Hurthle cells, also known as oxyphil cells, are large polygonal cells with pleomorphic hyperchromatic nuclei and eosinophilic granular cytoplasm. They are thought to arise form follicular thyroid epithelium. The World Health Organization (WHO) classifies malignant Hurthle cell tumors as follicular carcinoma, oxyphilic cell type (1). Hurthle cells are seen in association with a number of benign thyroid conditions including nodular hyperplasia, Gravesí disease, and thyroiditis. Hurthle cell tumors are nodular aggregates of Hurthle cells that may or may not be encapsulated. Hurthle cell carcinoma occurs with an incidence ranging form 13 - 35% in various series of these tumors. The pathologic determination of malignancy is difficult as the architectural and cytological features of the benign and malignant tumors overlap. Fine needle aspiration is useful only to establish the presence of a Hurthle cell neoplasm. The final diagnosis is dependent on histological analysis of the surgical specimen. If vascular invasion, invasion of adjacent tissues or complete capsular penetration (if the tumor is encapsulated) is demonstrated the tumor is considered malignant. The size of the tumor is not an independent predictor of malignancy but large (> 4 cm) tumors are more likely to be malignant.

Hurthle cell carcinoma accounts for approximately 6% of all differentiated thyroid cancers. It is primarily a disease of adults with a mean age at diagnosis of 50 years. Hurthle cell tumors occur more commonly in women than men (>2:1), but the relative incidence of carcinoma is greater in men. The most common clinical presentation is a single palpable, cold nodule. It can occur concurrently with benign thyroid disease, e.g. multinodular goiter, Gravesí disease. Some case series have reported an association with prior head and neck irradiation.

Hurthle cell carcinoma is a more aggressive neoplasm than papillary or other follicular cancers. Distant metastatic disease is seen in 10-15% of patients at initial diagnosis of the tumor in the neck. The lungs and bone are the most frequent sites of distant metastases. Distant metastatic disease is seen in approximately one third of patients and has a five year mortality rate of 80%.

Therapy

The only effective treatment for Hurthle cell neoplasm is surgery, due to the difficulty in making the pathologic diagnosis of malignancy from fine needle aspiration. Total thyroidectomy is advised for all malignant tumors possibly with internal jugular node sampling. Hurthle cell adenomas are treated by some surgeons with lobectomy and close follow up. Chemotherapy and external radiation treatment have not proven effective. The overwhelming majority of Hurthle cell carcinoma is not iodine avid, so I-131 is ineffective in treating or diagnosing metastatic disease. Prognosis depends mainly on extent of disease at initial diagnosis and the adequacy of surgical intervention. There is no effective treatment for metastatic disease.

In-111 Octreotide Scintigraphy

Indium-111 labeled octreotide has proven useful in identifying the extent of tumor burden. Hurthle cell tumors (adenomas and carcinomas) express somatostatin receptors (2, 3). Normal thyroid tissue also expresses somatostatin receptors. However, the concentration of somatostatin receptors is higher in Hurthle cell tumors. False positives can result with excessive uptake of tracer in benign thyroid disease. In one series of 10 patients there is no difference in octreotide uptake between Hurthle cell adenomas and carcinomas (2).

The main role of octreotide scintigraphy in the management of Hurthle cell carcinoma is to assess for metastatic disease. Possibly in the future somatostatin receptor analogs can be used to direct radiotherapy to treat metastatic disease, similar to I-131 treatment of metastatic thyroid follicular and papillary cancer.

References

1. Cooper DS and Schneyer, CR. Follicular and Hurthle cell carcinoma of the thyroid. Endocrinol Metab Clin North Am. 1990: 577-91.

2. Carcangiu ML, Bianchi S, Savino D, Voynick IM, Rosai J. Follicular Hurthle cell tumors of the thyroid gland. Cancer 1991;68:1944-1953.

3. Chen H, Nicol TL, Zeiger MA, Dooley WC, Ladenson PW, Cooper DS, Ringel M , Parkerson S, Allo M, Udelsman R. Hurthle cell neoplasms of the thyroid. Are there factors predictive of malignancy? Ann Surg, 1998;227:542-546.

4. Gulec SA, Serafini AN, Kasi SS, Peker KR, Gupta A, Goodwin WJ, Sfakianakis GN, Moffat FL. Somatostatin receptor expression in Hurthle cell cancer of the thyroid. J Nucl Med 1998;39:243-245.

5. Wilson CJ, Woodroof, JM, Girod DA. First report of Hurthle cell carcinoma revealed by octreotide scanning. Ann Otol Rhinol Laryngol, 1998; 107:847-849.

6. Tisell LE, Ahlman H, Wangberg B, Kolby L, Fjalling M, Forssell-Aronsson E, Molne J, Nilsson O. Expression of somatostatin receptors in oncocytic (Hurthle cell) neoplasia of the thyroid. British Journal of Cancer, 1999; 79:1579-1582.

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J. Anthony Parker, MD PhD, Tony_Parker@CareGroup.Harvard.edu