Review Article

Anaplastic Thyroid Cancer: A Review of Epidemiology, Pathogenesis, and Treatment

Table 1

Chart review and retrospective studies.

AuthorsYearSiteNo. of ptsStudy detailsResults

Swaak-Kragten et al. [30]2009Netherlands75Chart Review
1972–2003
When treated with Doxorubicin and radiation, the median survival was 3 months. 1 yr OS was 9%. Locoregional control was significantly higher in patients who had undergone R0/R1 resection or chemoradiation, with best results for patients who underwent both (complete remission in 89%).

Vrbic et al. [31]2009Serbia16Chart Review 1997–2007Radiation was combined with doxorubicin 60 mg/m2 and cisplatin 40 mg/m2 every 3 weeks. Overall response rate was of 25% (95% CI: 7–55). Mean patient OS was 12.33 months (95% CI: 9.09–15.56) and median OS 11.0 months (95% CI: 8.56–13.44).

Yau et al. [32]2008Hong Kong50Chart review
1996–2006
Median survival was 97 days. On univariate analysis, age ≤ 65 ( ), absence of metastatic disease at presentation ( ), surgical resection ( ), and postoperative radiotherapy was associated with longer survival. Cytotoxic chemotherapy was not associated with longer survival.

Lim et al. [33]2007USA37Chart review
1984–2006
A median radiation of dose 5760 cGy, >4500 cGy in 32 (87%) was administered through hyperfractionated or once-daily schedules. Median number of treatments received 6, >4 in 24 (65%). 2-year outcomes: locoregional control 25%; progression free survival 8%; overall survival 18%. 6 patients remained alive at the time of last followup with survival durations of 4, 11, 12, 57, 59, and 141 months, respectively.

Lee et al. [34]2006Korea15Chart review 1988–2003The mean overall survival time of the 15 patients was 237 days (range, 28–717 days). The 6, 12, 18, and 24-month survival rates were 33%, 26%, 13%, and 0%.

Wang et al. [35]2006Canada47Chart review
1983–2004
The 6-month local progression-free success rate was 95% ( ) with radical radiotherapy > 40 Gy compared to 64% with palliative radiotherapy at <40 Gy. Survival was 11 months with radical and 3 months with palliative ( ). The median overall survival time in patients with twice-daily fractionation (13 months) was 3 months longer than patients treated with once-daily fractionation (10 months), but the difference was not statistically significant ( ).

Veness et al. [36]2004Australia18Chart review 1979–2002 Median survival was 6.2 months.

Haigh et al. [6]2001Canada33Chart reviewIn patients treated with potentially curative resection, median survival was 43 months and was 3 months with palliative resection ( ). The median survival of 3.3 months with only chemotherapy and irradiation and palliative resection ( ).

McIver et al. [27]2001USA134Chart review
1949–1999
Extent of resection or completeness of resection did not affect survival. ( ). Postoperative radiotherapy improved median survival (5 versus 3 months) but was not significant ( ).

Besic et al. [37]2001Slovenia162Chart review
1972–1998
82 patients with distant metastasis at presentation were excluded. Patients were divided into primary surgery ( ) or primary chemotherapy and/or radiation therapy. One-year survival was similar in both groups ( ).

Sugino et al. [38]2002Japan40Chart review 1989–1999The one-year survival rate for surgery was 60%. The survival rate without surgery was 21%. Surgery and chemotherapy were both used in some patients. One-year survival rates for patients with small focus of anaplastic thyroid cancer with well-differentiated thyroid cancer were 73%.
Heron et al. [39]2002USA32Chart review 1952–1999Patients were divided into two groups. Group 1 patients between 1952–80 ( ) and Group 2 between 1981–1999 ( ). Group 1 patients received once daily radiation and Group 2 patients twice-daily radiation with chemotherapy of doxorubicin, pacitaxel, vincristine, or cisplatin There was a combination of therapies in both groups, with the above generalizations. 2-year survival was 44% in group 1 and 52% in group 2. Progression-free survival was 53% group 1 and 38% group 2. The authors concluded that hyperfractionated radiation with chemotherapy is associated with better survival but not progression-free survival.

Nilsson et al. [18]1998Sweden81Chart review
1971–1997
Eight patients (10%) survived more than 2 years and were treated with combinations of chemotherapy, radiotherapy, and surgery.

Tennevall et al. [40]1994Sweden33Chart review
1984–1992
Combination of hyperfractionated radiotherapy, doxorubicin, and debulking surgery. Preoperative radiation up to 30 Gy and post operative radiation up to 46 Gy. 20 mg doxorubicin per week. 48% had no local recurrence and, 24% died due to local failure. In 4 patients survival exceeded 2 yrs. Local control better with accelerated radiation therapy.

Venkatesh et al. [24]1990USA121Chart reviewMedian survival was months.  35% patients had well-differentiated thyroid cancer as well.

Junor et al. [10]1992UK91Chart review 1961–1986Surgery and radiation were used. Total or partial thyroidectomy increased survival. 80% responded to radiation therapy.

Levendag et al. [41]1993Holland51Chart review 1970–1986Local control achieved a median survival of 7.5 months and local residual disease 1.6 months.

Kim and Leeper [12]1987USA41Chart review
1979–1986
Only reporting Group 2 with anaplastic thyroid cancer. Weekly doxorubicin 10 mg/m2 prior to hyperfractionated radiotherapy with 160 cGy twice daily to total 57–60 cGy in 40 days. Initial complete remission rate was 84%. Local tumor control at 2 years was 68% with combined therapy. Median survival was 1 year.