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Hurthle Cell Carcinoma
Thyroid Cancer Type Hurthle Cell Carcinoma

Hurthle cell carcinoma is rare, but usually, an aggressive tumor of the thyroid gland, as it possesses a rather high rate of metastatic spread compared to other thyroid malignancies. Dysphagia, dyspnea, voice changes and an apparent mass in the neck are main findings. The initial diagnosis can be made through a thorough clinical exam, ultrasonography, and a fine-needle aspiration biopsy, but a histological exam showing > 75% of hurthle cells is necessary in order to confirm this malignancy.

Presentation

Hurthle cells (large, polygonal and well-demarcated cells containing a granular cytoplasm, a large hyperchromatic nucleus, and a prominent nucleolus) are considered to be a rather common finding on histological samples of thyroid nodules, which are present in up to 8% of the population [1]. Many different disorders of the thyroid gland have been associated with the appearance of hurthle cells, including chronic lymphocytic thyroiditis (CLT), Graves' disease, and multinodular goiter, but also a range of benign and malignant neoplasms [1] [2] [3]. Hurthle cell carcinoma accounts for < 5% of all differentiated thyroid tumors and is suspected when the biopsy specimen contains > 75% of hurthle cells, with the absence of a complete lesional capsule [1] [2] [3] [4] [5]. Hurthle cell carcinoma must be included in the differential diagnosis when patients present with a hard and immobile mass that may be accompanied by dysphagia, voice changes, or even dyspnea [2], the reason being mechanical compression of the esophagus and/or the trachea. Unfortunately, hurthle cell carcinoma is known for its potentially aggressive clinical course with a higher rate of metastatic spread when compared with other malignancies of the thyroid gland, and in addition to male gender and increased age, both being established as poor prognostic factors, the size of the tumor is important in determining the survival rates [6].

Workup

An early diagnosis of hurthle cell carcinoma may significantly improve patient outcomes, which is why physicians must obtain a detailed patient history and conduct a thorough physical examination [2]. The presence of previously mentioned symptoms of dysphagia/dyspnea and detection of a palpable mass in the neck mandates a more advanced workup. Ultrasonography of the neck is a valuable initial tool in the assessment of thyroid masses (and examination of proximal lymph node spread), but because both hypoechoic and hyperechoic (and sometimes isoechoic) appearance of hurthle cell carcinoma has been observed (as well as marked variations in vascularity of the tumor) [7], the use of fine-needle aspiration biopsy (FNAB) is imperative [1] [2] [3] [4]. When an abundance of hurthle cells is observed in biopsy samples (>75%), a lesion of > 4 cm is highly suggestive of a hurthle cell carcinoma [2], in which case additional imaging studies, such as computed tomography (CT) and magnetic resonance imaging (MRI) are recommended for assessing potential spread to the adjacent structures in the neck and distal organs [2]. To solidify the diagnosis, a histological examination must show capsular and/or vascular invasion of the tumor [1] [2].

Treatment

The primary treatment for Hurthle Cell Carcinoma is surgical removal of the tumor. This often involves a total thyroidectomy, which is the removal of the entire thyroid gland. Depending on the extent of the disease, lymph node dissection may also be performed. Radioactive iodine therapy is less effective for HCC compared to other thyroid cancers, but it may be considered in certain cases. Post-surgical treatment may include thyroid hormone replacement therapy to maintain normal metabolism and suppress any remaining cancer cells. Regular follow-up and monitoring are essential to detect any recurrence.

Prognosis

The prognosis for patients with Hurthle Cell Carcinoma varies based on several factors, including the size of the tumor, the presence of metastasis, and the patient's overall health. Generally, the prognosis is favorable if the cancer is detected early and treated promptly. However, HCC can be more aggressive and has a higher risk of recurrence compared to other types of thyroid cancer. Long-term follow-up is crucial to monitor for any signs of recurrence or metastasis.

Etiology

The exact cause of Hurthle Cell Carcinoma is not well understood. Like other thyroid cancers, it may be associated with genetic mutations and environmental factors. Exposure to radiation, particularly during childhood, is a known risk factor for thyroid cancer. There may also be a genetic predisposition, as some cases of HCC occur in families with a history of thyroid cancer or other endocrine disorders.

Epidemiology

Hurthle Cell Carcinoma is a rare form of thyroid cancer, accounting for approximately 3-5% of all thyroid malignancies. It is more commonly diagnosed in women than men and typically occurs in individuals over the age of 50. The incidence of HCC varies geographically, with higher rates reported in areas with iodine deficiency.

Pathophysiology

Hurthle Cell Carcinoma arises from Hurthle cells, which are altered follicular cells in the thyroid gland. These cells are characterized by their large size and abundant mitochondria, giving them a granular appearance under the microscope. The pathophysiology of HCC involves genetic mutations that lead to uncontrolled cell growth and tumor formation. Unlike other thyroid cancers, HCC is less responsive to radioactive iodine due to the reduced ability of Hurthle cells to uptake iodine.

Prevention

There are no specific measures to prevent Hurthle Cell Carcinoma, but general strategies to reduce the risk of thyroid cancer include avoiding unnecessary radiation exposure, particularly in children, and maintaining a diet sufficient in iodine. Regular medical check-ups and monitoring for individuals with a family history of thyroid cancer may aid in early detection and treatment.

Summary

Hurthle Cell Carcinoma is a rare and potentially aggressive form of thyroid cancer that arises from Hurthle cells. It presents with symptoms related to a neck mass and may require a comprehensive diagnostic workup for accurate diagnosis. Treatment primarily involves surgical removal of the thyroid gland, with careful follow-up to monitor for recurrence. While the prognosis can be favorable with early detection, the risk of recurrence necessitates ongoing vigilance.

Patient Information

If you have been diagnosed with Hurthle Cell Carcinoma, it is important to understand that this is a rare type of thyroid cancer that requires specialized care. Treatment usually involves surgery to remove the thyroid gland, and you may need to take thyroid hormone replacement medication afterward. Regular follow-up appointments with your healthcare provider are essential to monitor your health and ensure any recurrence is detected early. If you notice any new symptoms or changes, be sure to report them to your doctor promptly.

References

  1. Cannon J. The significance of hurthle cells in thyroid disease. Oncologist. 2011;16(10):1380-1387.
  2. Ahmadi S, Stang M, Jiang X “Sara”, Sosa JA. Hürthle cell carcinoma: current perspectives. Onco Targets Ther. 2016;9:6873-6884.
  3. Gross M, Eliashar R, Ben-Yaakov A, Weinberger JM, Maly B. Clinicopathologic features and outcome of the oncocytic variant of papillary thyroid carcinoma. Ann Otol Rhinol Laryngol. 2009;118(5):374–381.
  4. Hanief MR, Igali L, Grama D. Hürthle cell carcinoma: diagnostic and therapeutic implications. World J Surg Oncol. 2004;2:27.
  5. Barnabei A, Ferretti E, Baldelli R, Procaccini A, Spriano G, Appetecchia M. Hurthle cell tumours of the thyroid. Personal experience and review of the literature. Acta Otorhinolaryngol Ital. 2009;29(6):305-311.
  6. Bhattacharyya N. Survival and prognosis in Hürthle cell carcinoma of the thyroid gland. Arch Otolaryngol Head Neck Surg. 2003;129(2):207-210.
  7. Maizlin ZV, Wiseman SM, Vora P, et al. Hurthle cell neoplasms of the thyroid: sonographic appearance and histologic characteristics. J Ultrasound Med. 2008;27(5):751–757. quiz 759
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