Hugely Recurrent Papillary Thyroid Carcinoma
P Floros, R Grigg
Keywords
hugely, papillary carcinoma, recurrent, thyroid disease
Citation
P Floros, R Grigg. Hugely Recurrent Papillary Thyroid Carcinoma. The Internet Journal of Otorhinolaryngology. 2010 Volume 13 Number 1.
Abstract
Case Report
A 55 year old Fijian lady was referred to an ENT specialist in Toowoomba, Australia for advice and management of locally advanced recurrent thyroid papillary carcinoma. She was first seen in a small Fijian hospital in 1995 for some neck swelling and subsequently underwent a subtotal thyroidectomy. Histology at that time is unavailable. In 2005 she represented to Lautoka hospital, Fiji with recurrent neck swelling which had been present for 2 years. FNA of the lesion showed pathology consistent with a papillary lesion. She was subsequently admitted to Lautoka hospital (Lautoka, Fiji) from 31 st January 2005 to the 14 th of February 2005 and underwent a near total thyroidectomy.
Intraoperative findings indicated tumour invasion of the trachea and outer layer of oesophagus, enlarged bilateral cervical lymph nodes, invasion of tumour involving the carotid sheath and internal jugular vein near the sternal notch and a small tumour emboli in the internal jugular notch. Histopathology confirmed papillary carcinoma with lymphovascular invasion. She was reported to have hypertension during this admission and postoperatively commenced on thyroxine. She received follow up in the surgical clinic.
In 2009, the patient represented to the surgical clinic at the Colonial War Memorial hospital (Suva, Fiji) for recurrent local disease. She failed to return for prompt review when recurrent neck swellings were first noticed in 2007, two years following her second operation. Clinically in addition to a change in the quality of her voice from prior surgery, she reported regular forceful clearing of her larynx. She denied any shortness of breath and Pemberton’s sign was negative. She reported no difficulty in swallowing. The neck masses at this time measured 19 X 12cm on the right and 17 X 9cm on the left (Figure 1). CT with contrast reported multiple nodular masses of soft tissue density and septated in nature along with encapsulated cystic masses bilaterally in the neck region. The superior extent of these was noted at C1/C2 on the right while inferiorly extension to the right sterno-clavicular joint was noted. The trachea was compressed and displaced to the left (Figure 2). A serum TSH was measured at 2.28µIU/mL and in conjunction with CT radiological findings, recurrence of Thyroid Papillary Carcinoma was suggested. She was subsequently referred to an ENT surgeon in Toowoomba (Queensland, Australia) for debulking of the tumour mass.
Figure 1
Figure 2
On the 23 rd of March, 2010 she proceeded to surgery and underwent debulking of recurrent tumour. The operation was a two stage procedure with debulking of the right neck initially. Intraoperatively a mass measuring 1352 grams with tumour measuring 205 X 175 X 170mm multinodular with cystic areas and areas of haemorrhage was removed from the right neck. The tumour also appeared slightly pigmented. Histology confirmed metastatic papillary carcinoma. Left neck debulking was performed subsequently on the 31 st of March with tumour size measuring 110 X 80 X 65 millimetres and histology confirming massive papillary carcinoma with a malignant sinus to skin and areas of haemorrhage and necrosis within the mass.
Following surgical debulking she was referred to Royal Brisbane and Women’s Hospital (Brisbane, Queensland) on the 28 th of June 2010 where she received I131 ablation therapy for residual disease. She was recommenced on thyroxine and discharged on the 1 st of July 2010 with residual disease as evidenced by CT and a thyroglobulin of 3710µg/L.
Discussion
Papillary carcinoma of the thyroid is by far and away the most common form of thyroid cancer with 80% of patients with thyroid cancer having papillary carcinoma and a staggering 90% of new cases of thyroid cancer within the differentiated thyroid cancer family being attributable to this type4,5,6. The remaining 10% of differentiated thyroid cancer is of the follicular type5. Although prevalence is high, papillary thyroid cancer is associated with good outcomes and generally the tumour is indolent with low distal metastatic potential with a high rate of cure3,7. If appropriate treatment is implemented from the outset, long-term survival exceeds 90%7 and mortality rates reported in two studies as 2% and 7% respectively according to 10-year relative survival3.
Although favourable prognostically, recurrence of papillary carcinoma is not uncommon. In one study my Toniato
Clearly there is some consensus regarding risk factors for recurrence and overall survival for papillary carcinoma, however what is uncertain is the role these factors play in predicting hugely recurrent disease as reported in this case. One reason for this is the lack of adequate definitions of recurrence based on size or extent. Here we use the term hugely recurrent as a means to justify the enormity of recurrence in this lady. However, there is no current guideline or definition in place to adequately stratify the size of recurrence. Furthermore there have been few reported cases of hugely recurrent papillary thyroid disease. Tomisawa
Given the inadequacy of studies predicting factors for hugely recurrent papillary disease we can only hypothesise in this case as to reasons for such extensive recurrence. The currently accepted American Thyroid Association (ATA) guidelines for the initial treatment of differentiated thyroid cancer is the removal of all evidence of gross disease in the neck while minimising treatment and disease related morbidity10. Furthermore if any remnant disease exists the standard of care is the use of radioactive iodine (RAI) ablation therapy for residual disease. Intraoperative findings, histopathology description, RAI scan results and postoperative thyroglobulin are important elements of information during initial therapy which helps to stage disease and subsequently facilitates risk stratification and dictates further management and follow up. This information was unavailable regarding our case and whether she had received RAI is also uncertain after her first and second operations. Inadequate follow up appears to have played a significant role in the development of hugely recurrent disease as presented in this case. The second recurrence was not assessed until 2009, some two years since the patient first detected recurrent lumps. This was due to a perceived failure of surgery. This highlights the importance of appropriate patient education regarding the extent and nature of disease, particularly in patients who have had previous recurrent disease.
Access to health care is an issue which plaques a number of developing nations. The ministry of health in Fiji underwent some major health care reforms between 1999 – 2003. Although changes to the health care structure have been made, the public health care system in Fiji is heavily dependent on general taxation. There is also a growing demand for and cost of health care and unfortunately limitations to resources11. As a result for many individuals, particularly those living in smaller peripheral communities, access to health care is still an issue. Inadequate follow up coupled with issues surrounding access to healthcare may have played a role in the extent of recurrence reported in this patient.