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Anaplastic Thyroid Cancer (ATC)

Anaplastic thyroid cancers are undifferentiated tumors of the thyroid follicular epithelium

Prevalence

1-9 / 1 000 000

331-2,979

US Estimated

513-4,622

Europe Estimated

Age of Onset

ageofonset adult https://raremedicalnews.com/wp-content/uploads/2023/10/Depositphotos_195515734_L-e1696429163116.jpg

ICD-10

C73

Inheritance

This condition does not appear to have a clear pattern of inheritance.

Rare View

A rare and highly aggressive form of thyroid cancer with a poor prognosis, characterized by rapid growth and early metastasis. Treatment typically involves a combination of surgery, radiation, and chemotherapy, though outcomes remain poor.

rv anaplastic thyroid cancer atc https://raremedicalnews.com/wp-content/uploads/2023/10/Depositphotos_195515734_L-e1696429163116.jpg

5 Facts you should know

FACT

1

Anaplastic carcinoma of the thyroid (ATC) is the most aggressive thyroid gland malignancy

FACT

2

Although ATC accounts for less than 2% of all thyroid cancers, it causes up to 40% of deaths from thyroid cancer

FACT

3

The overall 5-year survival rate is reportedly less than 10%, and most patients do not live longer than a few months after diagnosis

FACT

4

Patients with ATC typically present with a rapidly growing neck mass

FACT

5

The female-to-male ratio is approximately 3:1

Anaplastic Thyroid Cancer (ATC) is also known as...

Anaplastic Thyroid Cancer (ATC) is also known as:

 
  • Thyroid cancer, anaplastic
  • Thyroid carcinoma, anaplastic
  • Anaplastic thyroid carcinoma
 
 

What’s your Rare IQ?

Which of the following is the least likely sites of to find metastatic disease in anaplastic thyroid cancer?

Common signs & symptoms

Rapidly Growing Neck Mass

A rapidly growing lump or swelling in the neck is often one of the first noticeable symptoms

Difficulty Swallowing (Dysphagia)

As the tumor grows, it may press on the esophagus, leading to difficulty swallowing or a feeling of choking

 

Hoarseness or Voice Changes

Pressure on the recurrent laryngeal nerve, which controls the vocal cords, can cause hoarseness or changes in voice

Difficulty Breathing (Dyspnea)

Large tumors may compress the trachea (windpipe), leading to difficulty breathing

Swollen Lymph Nodes

Enlarged lymph nodes in the neck may be present, indicating potential spread of the cancer

Pain

Some individuals may experience pain in the neck or throat region, especially if the tumor has spread to nearby tissues or structures

Weight Loss

Unexplained weight loss may occur due to the metabolic effects of the cancer

Fatigue

Generalized weakness or fatigue can be a symptom, though it is not specific to anaplastic thyroid cancer

Current treatments

Current treatments for anaplastic thyroid cancer typically involve a combination of surgery, radiation therapy, chemotherapy, targeted therapy, and immunotherapy. Here's an overview of these treatment modalities:

Surgery

Surgery is often the initial treatment for anaplastic thyroid cancer, aiming to remove as much of the tumor as possible. However, due to the aggressive nature of this cancer, complete surgical removal may not always be feasible

Radiation Therapy

Radiation therapy, either external beam radiation or internal radiation (brachytherapy), is commonly used after surgery to kill remaining cancer cells or as a primary treatment for unresectable tumors. It may also be used palliatively to relieve symptoms such as pain or difficulty swallowing

Chemotherapy

Chemotherapy drugs, such as doxorubicin, cisplatin, and paclitaxel, may be used alone or in combination to help shrink tumors, slow cancer growth, or relieve symptoms. Chemotherapy is often used in advanced or metastatic cases

Targeted Therapy

Targeted therapies, such as tyrosine kinase inhibitors (e.g., lenvatinib, sorafenib) and BRAF inhibitors (e.g., dabrafenib, vemurafenib), may be prescribed for tumors with specific genetic mutations. These drugs work by targeting specific molecules involved in cancer cell growth and proliferation

Immunotherapy

Immunotherapy, particularly immune checkpoint inhibitors like pembrolizumab or nivolumab, is being investigated as a treatment option for anaplastic thyroid cancer. These drugs help the immune system recognize and attack cancer cells

Clinical Trials

Participation in clinical trials may offer access to experimental treatments and novel therapies that are not yet widely available. Clinical trials are crucial for advancing the understanding and treatment of anaplastic thyroid cancer

Top Clinical Trials

TitleDescriptionPhasesStatusInterventionsMore Information
A Multi-Center Single-Arm Phase 2 Trial Of Zanzalintinib In Combination With Cemiplimab In BRAF Wild-Type Anaplastic Thyroid Cancer: The ZEPHYR TrialThe goal of the trial is to improve this OS by 4 months (to 9.9 months) using the zanzalintinib + cemiplimab treatment combination. Given an accrual period of 24 months and a maximum follow-up time of 36 months, at the significance level of 0.1, to achieve the power of 0.8, the sample size needed is...Phase 2Not yet recruitingDrug: Cemiplimab
Drug: Zanzalintinib
More Info
A Study of Avutometinib and Defactinib in People With Thyroid CancerThe researchers are doing this study to find out if the combination of avutometinib and defactinib is an effective treatment for RAF dimer-driven radioiodine-refractory differentiated thyroid cancer or anaplastic thyroid cancer. The researchers will also test whether avutometinib and defactinib is a...Phase 2RecruitingDrug: Avutometinib
Drug: Defactinib
More Info
IMMUNORARE5: A National Platform of 5 Academic Phase II Trials Coordinated by Lyon University Hospital to Assess the Safety and the Efficacy of the IMMUNOtherapy With Domvanalimab + Zimberelimab Combination in Patients With Advanced RARE CancersImmune checkpoint inhibitors (ICI) have revolutionized the management of advanced cancers. However, most rare cancers have been excluded from this progress due to the lack of clinical trials involving these diseases. After the standard first-line treatment, there are no other validated treatments fo...Phase 2RecruitingDrug: DOMVANALIMAB + ZIMBERELIMAB
Drug: DOMVANALIMAB + ZIMBERELIMAB + FOLFOX-4
More Info
Study of the Rechallenge Concept in Patients With BRAF-positive Anaplastic Thyroid Cancer After Progression on Anti-BRAF TherapyThis pilot phase 2 study evaluates the effectiveness and safety of the Rechallenge concept in patients with BRAF-positive anaplastic thyroid cancer after progression on anti-BRAF therapy. Patients with BRAF-positive anaplastic thyroid cancer who were previously treated with dabrafenib and trametinib...Phase 2RecruitingDrug: Dabrafenib + TrametinibMore Info
Basket Study of Entrectinib (RXDX-101) for the Treatment of Patients With Solid Tumors Harboring NTRK 1/2/3 (Trk A/B/C), ROS1, or ALK Gene Rearrangements (Fusions)This is an open-label, multicenter, global Phase 2 basket study of entrectinib (RXDX-101) for the treatment of patients with solid tumors that harbor an NTRK1/2/3, ROS1, or ALK gene fusion. Patients will be assigned to different baskets according to tumor type and gene fusion.Phase 2ActiveDrug: EntrectinibMore Info
Nivolumab and Ipilimumab in Treating Patients With Rare TumorsThis phase II trial studies nivolumab and ipilimumab in treating patients with rare tumors. Immunotherapy with monoclonal antibodies, such as nivolumab and ipilimumab, may help the body's immune system attack the cancer, and may interfere with the ability of tumor cells to grow and spread.

This tri...
Phase 2ActiveProcedure: Biospecimen Collection
Procedure: Computed Tomography
Procedure: Echocardiography Test
Biological: Ipilimumab
Procedure: Magnetic Resonance Imaging
Biological: Nivolumab
More Info
Trametinib in Combination With Paclitaxel in the Treatment of Anaplastic Thyroid CancerThe purpose of this study is to test the safety and tolerability of this treatment combination of paclitaxel and trametinib. Additionally, this study aims to to find out what effects the combination of paclitaxel and trametinib has on the shrinkage and growth of anaplastic thyroid cancer.Early Phase 1ActiveDrug: Trametinib
Drug: Paclitaxel
More Info
DETERMINE Trial Treatment Arm 07: Dabrafenib in Combination With Trametinib in Adult, Paediatric and Teenage/Young Adult Patients With BRAF V600 Mutation-Positive Cancers.This clinical trial is looking at two drugs called dabrafenib and trametinib. Dabrafenib and trametinib are approved as standard of care treatment for adult patients with melanoma (a type of skin cancer) or lung cancer and in children with glioma (a type of brain tumour). This means they have gone t...Phase 2, Phase 3Not yet recruitingDrug: Dabrafenib
Drug: Trametinib
More Info
Rare Disease Patient Registry & Natural History Study - Coordination of Rare Diseases at SanfordCoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help a...RecruitingMore Info
NEO- and Adjuvant Targeted Therapy in Braf-mutated Anaplastic Cancer of the Thyroid (NEO-ATACT Study)Anaplastic thyroid cancer (ATC) is an almost invariable lethal cancer in humans.

Most patients present with a rapid progressive mass in the neck with progressive complaints like dyspnoea, dysphagia or pain. The risk of suffocation is the main reason for rapid surgical intervention, but we know from...
Phase 2RecruitingDrug: dabrafenib/trametinibMore Info