Anaplastic thyroid carcinoma with unusual long-term survival: a case report

Abstract

Background Anaplastic thyroid carcinoma is a rare, rapidly progressive, and highly aggressive tumor. It has a global annual incidence of 1–2 per million people. It mostly affects older adults and women. The median survival duration after diagnosis does not exceed 6–8 months. Case presentation A 60-year-old female patient of mixed race (Honduran) presented to the local medical service with dysphonia that had started approximately 2 months earlier, accompanied by orthopnea that had started 1 month earlier. On physical examination, a soft mass was palpated within the anterior neck region; it was approximately 4 cm in diameter, painless, and mobile on swallowing, and had irregular margins. Ultrasound and computed tomography of the neck were performed. Subsequently, fine needle aspiration biopsy was performed. The histological diagnosis was anaplastic thyroid carcinoma (stage IVB). She underwent total thyroidectomy and chemotherapy. She is currently in her fifth year of remission after diagnosis and remains under oncologic surveillance. Discussion Anaplastic thyroid carcinoma demonstrates a lethal behavior. Approximately 18% survive for more than a year after diagnosis, and 0–10% survive for 5 years. Different pretherapeutic prognostic factors may affect survival, including age < 70 years, the absence of distant metastases, and complete local resection. Conclusion Conventional treatment improves the quality of life of the patient, but the results are not encouraging for the medium and long term. Only a few patients manage to exceed the average life expectancy of 3–6 months, despite undergoing the currently available therapeutic regimen.

Update on current diagnosis and management of anaplastic thyroid carcinoma

“…US can be used to detect heterogeneous echogenicity, abnormal shape, calcifications, increased vascularity, diffuse infiltration of adjacent tissues and regional lymph node involvement. US plain or preferably high resolution is the first step of exploration[ 18 , 19 ], followed preferably by core needle biopsy[ 21 ]. After establishing the diagnosis of the primary tumor of anaplastic thyroid carcinoma, staging imaging is necessary for local tumor extension assessment and revealing distant metastases.…”

“…CT can be used to detect heterogeneous tumor appearance, necrosis, calcifications, hypervascularity, and possible infiltration of the trachea and esophagus. CT is preferred over MRI[ 18 , 19 ]. Additionally, fiberoptic laryngoscopy for vocal cord evaluation, bronchoscopy and esophagoscopy are necessary prerequisites[ 20 , 43 ].…”

“…It may range from palliative debulking intervention to more radical resections, including total or near total thyroidectomy and extended lymphadenectomy, including the central and lateral lymph node level either unilaterally or bilaterally[ 6 , 8 , 36 , 53 , 61 ]. The above curative surgery may be performed in some patients with earlier disease and may provide, accompanied by adjuvant chemotherapy, occasional long survival over 5 years[ 18 , 19 ]. By multivariate analysis in a systematic review and meta-analysis, surgery and radiotherapy were found to be independent factors predicting increased overall survival[ 36 ].…”

“…Despite, the conflicting aspects for the above mentioned, it could be possibly explained by the higher expression of PD-L1 in papillary than in anaplastic carcinoma and response to immunotherapy[ 59 ] and anti-BRAF targeted therapy[ 24 ]. A long-term survival reaching above 5 years has been reported in isolated cases after surgical excision and adjuvant chemotherapy[ 18 , 19 ].…”

“…Early diagnosis based mainly on ultrasound (US) and core needle biopsy is crucial[ 17 - 21 ]. The following staging after the initial diagnosis of anaplastic carcinoma is of great importance and can be achieved by computed tomography (CT), magnetic resonance imaging (MRI)[ 18 , 19 ], and preferably positron emission tomography/CT (PET-CT)[ 17 , 22 , 23 ]. Modern molecular testing by revealing implicated genes, basically the BRAF gene ( BRAF-V600E and BRAF wild type), and other molecules[ 24 - 27 ] can contribute to more accurate diagnosis but most importantly determine molecular pathways indicating novel treatment strategies by targeted biological factors, i.e., anti-BRAF, anti-vascular endothelial growth factor (VEGF)-A or anti-epidermal growth factor receptor (EGFR) agents[ 24 ].…”