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Case Reports and Images in Surgery

 
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Rapid growth and local advance of an aggressive papillary thyroid cancer with anaplastic transformation in an elderly woman
Houssam Khodor Abtar1, Hiba El Hage Chehade2, Ali Chami3, Mustapha Mneimne4
1MD, Makassed General Hospital, Department of surgery, Beirut, Lebanon
2MD, MRCS, Makassed General Hospital, Department of surgery, Beirut, Lebanon
3MD Makassed General Hospital, Department of surgery, Lebanon
4MD, MBA, Makassed General Hospital, Department of Surgery, Beirut, Lebanon

Article ID: 100045Z12HA2017
doi:10.5348/Z12-2017-45-CL-10

Address correspondence to:
Houssam Khodor Abtar
Makassed General Hospital
P.O. Box: 11-6301 Riad EI-Solh
11072210, Beirut, Lebanon

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How to cite this article
Abtar HK, Chehade HEH, Chami A, Mneimne M. Rapid growth and local advance of an aggressive papillary thyroid cancer with anaplastic transformation in an elderly woman. J Case Rep Images Surg 2017;2:38–40.


Case Report

A 71-year-old female was presented to our surgical department complaining of painful, erythematous and ulcerated neck mass causing bleeding (Figure 1). She also complained of shortness of breath and generalized fatigue. Her medical history included osteoporosis, hypertension and dyslipidemia. The history dates back to five years ago when she had noticed to have a cervical nodule during routine examination for elective cholecystectomy. Chest radiograph at that time noticed a well-defined nodule about 4x4 cm (Figure 2). That pathological findings was ignored by the patient till recently when that mass started to grow up, ulcerate and bleed. Patient underwent incisional biopsy of the mass which turned to be metastatic papillary carcinoma in cervical lymph node with extensive neoplastic infiltration of skin and sub-cutaneous tissue by anaplastic tumor, giant cell type. A whole body computed tomography scan showed multiple metastatic lung nodules. The rest of the body was free of metastasis. Multimodal therapeutic approaches were started including debulking surgery (R2 resection), radiation to a biologically equivalent dose and adjuvant chemotherapy with doxorubicin and docetaxel. Despite supportive treatment her condition kept deteriorating and presented later with advanced necrotic neck tumor that causing active bleeding (Figure 3). At this moment the treatment discontinued as per family request and had just curettage to alleviate the bleeding process. After several weeks the tumor progressed rapidly to reach a huge size (Figure 4) and patient started to have dysphonia, dysphagia, and dyspnea. Unfortunately, patient subsequently died.


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Figure 1: Right sided ulcerated neck mass causing erythema of the skin.



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Figure 2: Chest X-ray showed well-defined cervical nodule 40.8 ml x 43.2 mm causing tracheal deviation.


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Figure 3: Advanced necrotic bleeding tumor extended outside the skin and involving the surrounding tissue.



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Figure 4: Very ugly looking 15x15 cm tumor with areas of necrosis.



Discussion

Anaplastic thyroid cancer (ATC) accounts for a small percentage of thyroid cancers (1.6%) and ranks among the most aggressive and lethal solid tumors of all human malignancies with a disease-speci?c mortality approaching 100% [1]. It accounts, however, for 14–39% of thyroid cancer deaths [2]. The mean survival rate ranges from 4–12 months [1].

Approximately half of patients at the time of diagnosis with ATC have evidence of distant metastatic disease, which is represents a poor clinical prognostic factor that predicts rapid disease progression and death [3]. Another 25% will develop distant spread during the course of their disease [4].

It is well known that ATC does not arise de novo but rather considered part of the natural history of untreated papillary thyroid carcinoma (PTC) or follicular carcinoma and Hürthle cell tumors [1]. However, some authors like Sam M et al., evaluate the change in the cancer expression profile that occurs during the transformation of DTC into ATC. They identified a panel of three upregulated markers (_-catenin [CTNNB1], topoisomerase II-_ [TOPO-II], and vascular endothelial growth factor [VEGF]) and two downregulated markers (thyroglobulin [TG], E-cadherin [E-CAD]) seem to most consistently differentiate ATC from DTC [4].

There is no de?nitely effective therapy exists for ATC even with aggressive multimodal approach. A research Consortium of Japan (ATCCJ) analyzed data from a large cohort of 677 ATC patients to determine prognostic factors and treatment outcomes for ATC. They found that only 15% of patients achieved long-term survival exceeding one year after diagnosis and concluded that complete resection is considered the cornerstone for longer survival, along with adjuvant radiochemotherapy [2].

A recent review reported success of combining external beam radiotherapy EBRT (multiple small radiation doses that allow more than one radiation treatment a day) with taxanes in anaplastic thyroid cancer. The local control rate was 60%, but the two-year survival was only 9%. In only 24% was death attributable to local failure, reflecting the high rate of distant metastatic disease that is unresponsive to chemotherapy [5].


Conclusion

In conclusion, we have described a case of anaplastic transformation of papillary carcinoma with lung metastasis and we emphasized the aggressiveness and rapid behavior of this tumor (it is not unusual for tumor volume to double over a week of observation). Almost all affected individuals eventually die of the disease because of its rarity and rapidly fatal clinical course ATC has been difficult to study and despite aggressive treatment.

Keywords: Anaplastic tumor, Fatal outcome, Local advance


References
  1. Wiseman SM, Loree TR, Rigual NR, et al. Anaplastic transformation of thyroid cancer: Review of clinical, pathologic, and molecular evidence provides new insights into disease biology and future therapy. Head Neck 2003 Aug;25(8):662–70.   [CrossRef]   [Pubmed]    Back to citation no. 1
  2. Sugitani I, Miyauchi A, Sugino K, Okamoto T, Yoshida A, Suzuki S. Prognostic factors and treatment outcomes for anaplastic thyroid carcinoma: ATC Research Consortium of Japan cohort study of 677 patients. World J Surg 2012 Jun;36(6):1247–54.   [CrossRef]   [Pubmed]    Back to citation no. 2
  3. Khairy G. Anaplastic transformation of differentiated thyroid carcinoma. Int J Health Sci (Qassim) 2009 Jan;3(1):93–6.   [Pubmed]    Back to citation no. 3
  4. Wiseman SM, Griffith OL, Gown A, Walker B, Jones SJ. Immunophenotyping of thyroid tumors identifies molecular markers altered during transformation of differentiated into anaplastic carcinoma. Am J Surg 2011 May;201(5):580–6.   [CrossRef]   [Pubmed]    Back to citation no. 4
  5. Brierley JD. Update on external beam radiation therapy in thyroid cancer. J Clin Endocrinol Metab 2011 Aug;96(8):2289–95.   [CrossRef]   [Pubmed]    Back to citation no. 5
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Author Contributions
Houssam Abtar – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Hiba Hassan El Hage Chehade – Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Ali Chami – Acquisition of data, Analysis and interpretation of data, Drafting the article, revising it critically for important intellectual content, Final approval of the version to be published
Mustafa Mneimne – Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2017 Houssam Abtar et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.



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