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    Journal Club: Dr. Preethi.S DNB Resident Ent 31/03/23

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    Preethi
    Anaplastic thyroid carcinoma is a highly aggressive and rare form of thyroid cancer with a very poor prognosis. It accounts for 2-3% of thyroid cancers. Key characteristics include a median survival of 4 months, older age at diagnosis typically in the 6th-7th decade of life, and a female to male ratio of 2:1. Treatment involves surgery for debulking if possible followed by external beam radiation therapy. Chemotherapy may also be used but the cancer is generally not responsive to treatment and mortality is close to 100%.

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    Journal Club: Dr. Preethi.S DNB Resident Ent 31/03/23

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    Anaplastic thyroid carcinoma is a highly aggressive and rare form of thyroid cancer with a very poor prognosis. It accounts for 2-3% of thyroid cancers. Key characteristics include a median survival of 4 months, older age at diagnosis typically in the 6th-7th decade of life, and a female to male ratio of 2:1. Treatment involves surgery for debulking if possible followed by external beam radiation therapy. Chemotherapy may also be used but the cancer is generally not responsive to treatment and mortality is close to 100%.

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    Anaplastic thyroid carcinoma is a highly aggressive and rare form of thyroid cancer with a very poor prognosis. It accounts for 2-3% of thyroid cancers. Key characteristics include a median survival of 4 months, older age at diagnosis typically in the 6th-7th decade of life, and a female to male ratio of 2:1. Treatment involves surgery for debulking if possible followed by external beam radiation therapy. Chemotherapy may also be used but the cancer is generally not responsive to treatment and mortality is close to 100%.

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    41 views27 pages

    Journal Club: Dr. Preethi.S DNB Resident Ent 31/03/23

    Uploaded by

    Preethi
    Anaplastic thyroid carcinoma is a highly aggressive and rare form of thyroid cancer with a very poor prognosis. It accounts for 2-3% of thyroid cancers. Key characteristics include a median survival of 4 months, older age at diagnosis typically in the 6th-7th decade of life, and a female to male ratio of 2:1. Treatment involves surgery for debulking if possible followed by external beam radiation therapy. Chemotherapy may also be used but the cancer is generally not responsive to treatment and mortality is close to 100%.

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    JOURNAL CLUB

    DR. PREETHI.S
    DNB RESIDENT ENT
    31/03/23
    ANAPLASTIC THYROID
    CARCINOMA
    • Highly aggressive malignant tumor

    • Accounts for 2 to 3 percent of all thyroid gland


    neoplasms.

    • Very poor prognosis (mortality is close to 100 percent)

    • Median survival of 4 months and a 6-month OS of 35%.

    • Survival beyond 2 years is 12%


    ETIOLOGY
    • Low education level

    • Type B blood group

    • Goiter.

    Following characteristics tend to present with more complicated and


    adverse clinicopathological findings;

    1. older age

    2. male patient

    3. advanced locoregional disease

    4. distant metastases.
    EPIDEMIOLOGY

    • Elderly (sixth to seventh decades of life)

    • Mean age at diagnosis is 65 years

    • Female-to-male ratio is 2 to 1
    PATHOPHYSIOLOGY
    • Tp53 gene inactivation may play an important role
    in the progression from differentiated to
    undifferentiated carcinoma.

    • Thyroid-specific rearrangements RET/PTC and


    PAX8/PPARγ are rarely found in poorly
    differentiated or undifferentiated thyroid cancer -
    these genetic alterations do not predispose cells to
    dedifferentiation
    CYTOGENETICS
    • Gene mutation is commonly reported in ATC:

    • p53 (most common)

    • RAS

    • BRAF

    • β-catenin

    • PIK3CA

    • Axin

    • APC

    • PTEN
    HISTOPATHOLOGY
    Macroscopic Findings

    • Bulky mass (mean: 6 cm)

    • Homogeneous and variegated appearance

    • On cut section: light tan and fleshy with zones of necrosis and
    haemorrhage

    • Infiltrating, often into adjacent soft tissues and organs


    Microscopic Findings

    Broadly categorized into three patterns, which can occur


    singly or in any combination:

    • Sarcomatoid

    • Giant cell

    • Epithelial

    • Common to all three forms are necrosis, an elevated


    mitotic rate, and an infiltrative growth pattern. Vascular
    invasion is also often present.
    IMMUNOHISTOCHEMISTRY
    • Common thyroid-lineage markers such as TTF1
    and thyroglobulin are usually absent

    • PAX, also a thyroid-lineage marker, is retained in


    approximately half of all cases.

    • Positive cytokeratin expression -epithelial nature


    of anaplastic thyroid carcinoma
    CLINICAL FEATURES

    • Rapidly growing, painful, firm, low anterior neck


    mass usually fixed to the underlying structures

    • Hoarseness, dysphagia, dyspnea, and cough

    • Regional nodal metastases and vocal cord


    paralysis present in 40% and 30%, respectively.
    EVALUATION
    • USG

    • FNAC

    • CT

    • MRI

    • FDG-PET
    STAGING
    According to the International Union Against Cancer (UICC)—
    TNM staging and AJCC system:

    • All anaplastic thyroid carcinomas are


    considered stage IV

    • Stage IVA and IVB patients have intrathyroidal


    tumors (IVA) and extrathyroidal tumors (IVB) and no
    distant metastatic disease

    • Stage IVC patients have distant metastasis.


    PROGNOSTIC FACTORS
    The favorable prognostic indicators of anaplastic thyroid carcinoma are:

    • Younger age (less than or equal to 60 years old)

    • An absence of cervical or distant metastases

    • Small tumors (less than or equal to 5 to 7 cm)

    • Unilateral tumors

    • An absence of local invasion of the surrounding tissue or nodal


    involvement

    • An incidental finding of anaplastic thyroid carcinoma within a


    thyroidectomy specimen
    MANAGEMENT
    • Surgery- debulking, airway

    • Radiation!

    External-beam irradiation is effective in improving local control

    Adjuvant radiation therapy should be performed in all cases, including


    completely resected small-size incidental anaplastic thyroid carcinoma and
    anaplastic thyroid carcinoma with a differentiated component.

    • Chemotherapy!

    • Usually not responsive to I131 therapy.

    • Recommendation upon identification of a differentiated iodine-positive


    component.
    COMPLICATIONS
    • Local invasion occurs in almost 70% of patients as
    observed in large series: muscles (65%), trachea
    (46%), esophagus (44%), laryngeal nerve (27%),
    and larynx (13%). Lymph node metastases are a
    feature in almost 40% of patients.

    • Metastases occur in up to 75% of patients. They


    most frequently involve the lungs (80%), the brain
    (5 to 13%), and bones (6 to 15%).
    DIFFERENTIAL DIAGNOSIS
    • Metastatic disease to the thyroid

    • Primary thyroid lymphoma

    • Primary thyroid sarcoma

    • Poorly differentiated thyroid carcinoma

    • Squamous cell thyroid carcinoma

    • Medullary carcinoma
    • To avoid the development of anaplastic carcinoma,
    longstanding goiters, as well as benign nodules,
    should be followed carefully and considered for
    resection if they grow or do not respond to medical
    therapy.

    • Total thyroidectomy for malignant disease can


    prevent the development of anaplastic carcinoma.
    THANKYOU

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