Management

Patients presenting with Hiirthle cell neoplasms usually have had a solitary thyroid nodule evaluated by FNA. In our experience, 35% of these lesion ultimately prove to be malignant, although in some series, up to 60% have been reported to be cancers.8 25 Accordingly, we recommend surgical exploration for patients in whom the FNA demonstrates a Hiirthle cell neoplasm.

Surgical Procedure

A careful exploration is always undertaken to detect the presence of obvious malignant disease—i.e., tumor invasion into adjacent structures, metastatic nodal disease—as well as contralateral nodular thyroid disease. We advocate one-stage total or near-total thyroidectomy if obvious malignant disease or contralateral nodular disease is present or if the patient has a history of childhood head and neck irradiation. This is based on the fact that there is an increased incidence of multifocal disease as well a 50% chance of a concomitant papillary cancer associated with previous head and neck irradiation." Furthermore, Carcangiu and associates have shown that local recurrence of Hiirthle cell carcinoma is correlated with the extent of surgery, with recurrence rates for nodulectomy, thyroid lobectomy, and total thyroidectomy of 75%, 40%, and 15%, respectively.10 In addition, some patients, understanding the 35% risk of carcinoma, may elect to have an initial total thyroidectomy rather than having a completion thyroidectomy should cancer be found.

For the routine patient with a single dominant nodule, surgical management should consist of an ipsilateral lobectomy and isthmusectomy. Surgical procedures involving less than a lobectomy, in our opinion, have no role in the management of neoplastic lesions. Some surgeons advocate intraoperative frozen section evaluation to assess capsular and/or vascular invasion.26 If frozen section reveals a benign process such as thyroiditis or goiter, or a Hiirthle cell neoplasm lacking capsular or vascular invasion, the surgery is terminated. If capsular or vascular invasion is present, then total or near-total thyroidectomy is performed. Although this is theoretically an acceptable approach, because frozen section is inherently unreliable in detecting capsular/vascular invasion,24 one can argue for omitting frozen section evaluation.

Although some recommend random lymph node sampling in all cases,27 we typically do not excise lymph nodes unless they are enlarged. If disease is found outside the thyroid—i.e. soft tissue invasion or lymph node metastasis—resection of all gross/microscopic disease is performed. Because surgical resection is usually the only curative treatment option for Hiirthle cell carcinoma, every effort should be made to resect all disease. This includes a modified radical neck dissection for positive cervical lymph node metastasis. We occasionally advocate more radical surgery for advanced Hiirthle cell carcinoma, including resection of the larynx, trachea, skin, soft tissue, cervical lymph nodes, and esophagus (Fig. 14-6).

In patients who underwent an initial lobectomy/isthmus-ectomy whose final pathology was diagnostic of carcinoma, we generally perform a completion thyroidectomy as soon as possible after the initial surgery. In patients with only partial capsular invasion, the decision is not as clear. Several studies, however, have illustrated that these lesions do not behave in a malignant manner.81115 This has also been our experience. Although some advocate completion

FIGURE 14-6. A patient with very aggressive Hiirthle cell carcinoma. A, MRI demonstrates a large thyroid mass, fi, CT scan of the neck depicts almost complete airway obstruction by tumor. C, A barium swallow study illustrates esophageal deviation and obstruction secondary to the mass. This patient's operative management included total thyroidectomy, total laryngectomy, bilateral modified radical lymph node dissections, esophagectomy, and gastric pull-up reconstruction.

FIGURE 14-6. A patient with very aggressive Hiirthle cell carcinoma. A, MRI demonstrates a large thyroid mass, fi, CT scan of the neck depicts almost complete airway obstruction by tumor. C, A barium swallow study illustrates esophageal deviation and obstruction secondary to the mass. This patient's operative management included total thyroidectomy, total laryngectomy, bilateral modified radical lymph node dissections, esophagectomy, and gastric pull-up reconstruction.

c thyroidectomy in this case, we would favor close clinical follow-up and life-long thyroid suppression. In patients with the diagnosis of Hurthle cell adenoma, no further operative intervention is warranted. However, close clinical follow-up is recommended. There have been reports of recurrence with Hurthle cell adenomas,28 but this is a rare occurrence.

Radioactive Iodine and Other Modalities

Hurthle cell carcinomas generally fail to concentrate 131I and, therefore, in most patients, radioablation does not offer a therapeutic benefit. As a result, the only curative treatment for Hurthle cell carcinoma is surgical resection. However, there have been case reports of metastatic tumors that have 131I uptake. In 4 of 7 patients with pulmonary metastases from Hurthle cell carcinoma, 131I uptake by the metastatic tumors was present.29 Although there have also been reports of resolution of pulmonary metastases with 13II treatment, it is important to note that uptake does not always correlate with tumor responsiveness. However, because results from other modalities of treatment for metastatic disease have been dismal, each patient's case must be individually considered in evaluating the role for 131I. In most patients, it is unlikely that 13II has a therapeutic benefit. In another series, two patients, who had pulmonary metastases responsive to 13'I treatment, also had extraordinarily elevated serum thyroglobulin levels.30 Others have also reported regression of Hurthle cell carcinoma metastases with 13'I in tumors that apparently secreted thyroglobulin.31 Hurthle cell adenomas and carcinomas are known to be able to secrete thyroglobulin. We have also had two patients with pulmonary and hilar lymph node metastases, respectively, who responded to 131I therapy.8 Based on these reports, some advocate 131I scans in patients with elevated thyroglobulin levels 4 to 6 weeks after total thyroidectomy, and treatment if positive. Other modalities of treatment have no efficacy in the primary treatment of Hurthle cell carcinoma. These tumors are not responsive to various regimens of chemotherapy. External-beam radiation to the soft tissue in the neck has shown no effect of survival.10 However, it does have a role for palliation of bony metastasis.

Postoperative Follow-Up

Our philosophy is based on the belief that total or near-total thyroidectomy is the treatment of choice for well-differentiated thyroid cancers including Hurthle cell carcinomas. After thyroidectomy, we advocate 131I ablation if any residual uptake is present. This offers the advantage of screening for recurrence by thyroglobulin levels. After ablation, thyroglobulin levels should be checked at 6-month intervals initially. If thyroglobulin levels are elevated, the patients should be withdrawn from thyroid hormone and a scan performed. If positive, the patient should be treated with therapeutic doses of 131I. If the 131I scan is negative, then we use other modes of imaging, including neck ultrasound, computed tomography scan or magnetic resonance imaging of the neck and chest, and positron-emission tomography (PET) scans. In a recent meta-analysis for the detection of Hurthle cell carcinoma by PET, the reported sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 92%, 80%, 92%, 80%, and 89%, respectively.32 Another emerging method to detect Hurthle cell carcinoma recurrence is octreotide scintiscanning. Gorges and associates recently reported their experience with 29 patients with recurrent Hurthle cell cancers.33 They found that in patients with thyroglobulin greater than 10 ng/mL, 95% had positive "'In-octreotide scans. If any of these studies localize recurrent disease, surgical resection is considered.

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