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Undifferentiated Thyroid
Carcinoma
Done By :- Abu-Alhassn haedar
Supervised By:- Dr.Qahtan
Anaplastic carcinoma
• This is one of the most aggressive malignancies in humans.
• Median survival 6 months.
• % of thyroid cancer 1–2%.
• The disease is characterized by rapid growth, visceral invasion
and distant metastases.
• The peak incidence occurs in the 6th to7th decade of life.
Women comprise 55% to 77% of patients.
• Risk factors for thyroid cancer (Female gender, exposure to
ionizing radiation, and family history of thyroid cancer).
clinical features
• Rapidly growing mass. A dull aching pain in the neck is quite common
• Local invasion may involve, RLN and causes hoarseness of the voice , the
trachea causing dyspnea and stridor , and involvement of esophagus
causing dysphagia .
• Invasion of the cervical sympathetic nerves may cause Horner's syndrome
(contraction of the pupil ,enophthalmos , narrowing of the palpebral fissure
and loss of sweating of face and neck .)
• Direct tissue invasion and systemic metastasis to lung, bone and brain
(Pulmonary metastases are common )
Note : Anaplastic CA can presents commonly with cervical invasion and
pulmonary metastases at the same time , unlike the papillary in which
pulmonary metastases is less common .
•Physical
examination
1. General examination
• Patients often breathe with difficulty and
have stridor .
• There is often wasting and anemia.
• There may be evidence of skeletal
metastases even pathological
fractures.
• In advanced cases the liver may be
enlarged.
2. Thyroid
examination
• Diffuse thyroid enlargement and
the mass is hard and solid.
• The mass in the neck has no
definable shape.
• The overlying skin often has a
red-blue tinge because the
underlying infiltration interferes
with its venous drainage.
• 50% may have enlarged cervical
Diagnosis
• The diagnosis is usually suspected on clinical
examination and confirmed by FNAB or core
biopsy.
• FNAB has been shown to be accurate in
90% of cases.
• CT and MRI are useful in defining the local
extent of disease and identifying distant
metastases
High risk findings in US for
malignancy
1. Micro-calcification
2. Hypo-echoic nodule / Solid
3. Irregular margin
4. Intra-nodular vascularity
5. More tall then wide
Staging
TNM CLASSIFICATION:
• All anaplastic carcinoma is stage IV
• Stage IV contains 3 stages:
IV A ; Tumor is limited to the thyroid and surgically
resectable.
IV B : Tumor extending beyond thyroid and
considered surgically irresectable.
IV C: Tumor presented with distant metastasis.
TREATMENT
1) Surgery
2) Radiotherapy
3) Chemotherapy
• Initial surgical resection or debulking may be
considered if preoperative imaging suggests
feasibility.
• If not, chemotherapy and external beam radiation
may be performed as first-line therapy, possibly
followed by surgery in responsive cases.
• Treatment is generally palliative (given its
aggressive nature) and may include early
tracheostomy for airway protection.
Medullary carcinoma
• These are tumors of the para-follicular (C cells) derived from the
neural crest.
• 25% Inherited (Germline mutation RET oncogene).
• High levels of serum calcitonin and carcinoembryonic antigen are
produced by many medullary tumors, which should be tested for in
suspected cases.
• Calcitonin levels fall after resection and rise again with recurrence,
making it a valuable tumor marker in the follow-up of patients with
this disease.
• Some tumors are familial and account for 10–20% of all cases.
• The majority of patients are between the ages of 50 and 70 years
when the tumor is sporadic in nature
• When the condition occurs in young adults (20–30 years),
often with a family history, it may be a manifestation of the
multiple endocrine neoplasia syndrome (MEN) types 2a or
2b. In these cases, the medullary thyroid carcinoma may
be accompanied by associated conditions such as:
Medullary Thyroid Cancer Occurs in
Four Clinical Settings
1- Sporadic
• 80% of all cases of medullary thyroid cancer.
• Typically unilateral
• No associated endocrinopathies
• Peak onset 40 - 60.
• Females predominance: 3:2 ratio.
• One third will present with intractable diarrhea.
2-MEN 2-A (Multiple Endocrine Neoplasia 2 A).
1. bilateral medullary carcinoma
2. pheochromocytoma
3. hyperparathyroidism.
• This syndrome is inherited as autosomal dominant. Because
of this, males and females are equally affected.
• Peak incidence of medullary carcinoma in these patients is in
the 30's.
3-MEN 2 B This syndrome has
medullary carcinoma
• Inheritance is autosomal dominant as in MEN IIA (m=f)
• Pheochromocytomas must be detected prior to any operation.
• The idea here is to remove the pheochromocytoma first to remove the
risk of severe hypertensive episodes while the thyroid or parathyroid
is being operated on.
4-Inherited medullary carcinoma without associated
endocrinopathies.
• This form of medullary carcinoma is the least aggressive.
• Like other types of thyroid cancers, the peak incidence is
between the ages of 40 and 50.
Presentation
• The common presentation is a firm, smooth and distinct
lump in the neck(most commonly in single lobe ).
• It is indistinguishable from any other form of thyroid
solitary nodule.
• The symptoms which should make you think of medullary
tumor, apart from a lump in the neck and the presence of
the above lesions in the patient or their family, are diarrhea
and flushing. Diarrhea occurs in one-third of the patients.
The fluidity of the stool and the frequency of defecation
are both increased.
• Diarrhea is a feature in 30% of cases and this
may be due to 5-hydroxytryptamine or
prostaglandins produced by the tumor cells.
• Involvement of lymph nodes occurs in 50–60% of
cases of medullary carcinoma and blood-borne
metastases are common.
Diagnosis
1. Family history.
2. ↑Serum Calcitonin
3. ↑CEA
4. FNAB.
• tumors are not TSH dependent and do not
take up radioactive iodine.
•Family members at risk should firstly
be screened for(RET mutation) in
their blood. when recognized as a
carrier of the mutation, should be
screened by neck ultrasound and
calcitonin.
Treatment
• Standard treatment is total thyroidectomy and
clearance of involved anterior cervical and superior
mediastinal lymph nodes. Without metastases,
operation is often curative but when nodes are
involved, 10-year survival falls to about 50%.
• External Beam Radiation for unresectable residual or
recurrent tumor.
• No effective Chemotherapy.
FAMILIAL MTC (RET mutation +ve)
 Prophylactic Thyroidectomy:
 Before age of 6 yrs for MEN2A
 Before age of 1 yr for MEN2B
• Pheochromocytoma when associated should be operated
first
• During follow-up, calcitonin levels are monitored and raised
levels indicate tumor recurrence. Surgical re-exploration of
the neck may be undertaken to remove involved nodes.
Malignant lymphoma
• Thyroid lymphomas are rare and usually arise in pre-
existing autoimmune (Hashimoto’s) thyroiditis. Most are
non- Hodgkin lymphomas. Diagnosis can only be made
histologically, by core needle biopsy or open biopsy, as
FNA is inadequate.
• The prognosis is good, particularly if there is no
involvement of cervical lymph nodes.
• Presentation – rapid growing goiter + diffuse pain
• Median age is 7th decade.
•Treatment is with radiotherapy, and survival
depends on whether spread has extended beyond the
thyroid capsule. For lesions within the capsule, 5-year
survival is 85%, falling to 40% when local spread has
occurred.
• Response to irradiation is dramatic and radical
surgery is unnecessary once the diagnosis is
established by biopsy.
• advanced disease is managed by a chemotherapy

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Undifferentiated thyroid carcinoma

  • 1. Undifferentiated Thyroid Carcinoma Done By :- Abu-Alhassn haedar Supervised By:- Dr.Qahtan
  • 2. Anaplastic carcinoma • This is one of the most aggressive malignancies in humans. • Median survival 6 months. • % of thyroid cancer 1–2%. • The disease is characterized by rapid growth, visceral invasion and distant metastases. • The peak incidence occurs in the 6th to7th decade of life. Women comprise 55% to 77% of patients. • Risk factors for thyroid cancer (Female gender, exposure to ionizing radiation, and family history of thyroid cancer).
  • 3. clinical features • Rapidly growing mass. A dull aching pain in the neck is quite common • Local invasion may involve, RLN and causes hoarseness of the voice , the trachea causing dyspnea and stridor , and involvement of esophagus causing dysphagia . • Invasion of the cervical sympathetic nerves may cause Horner's syndrome (contraction of the pupil ,enophthalmos , narrowing of the palpebral fissure and loss of sweating of face and neck .) • Direct tissue invasion and systemic metastasis to lung, bone and brain (Pulmonary metastases are common ) Note : Anaplastic CA can presents commonly with cervical invasion and pulmonary metastases at the same time , unlike the papillary in which pulmonary metastases is less common .
  • 4. •Physical examination 1. General examination • Patients often breathe with difficulty and have stridor . • There is often wasting and anemia. • There may be evidence of skeletal metastases even pathological fractures. • In advanced cases the liver may be enlarged.
  • 5. 2. Thyroid examination • Diffuse thyroid enlargement and the mass is hard and solid. • The mass in the neck has no definable shape. • The overlying skin often has a red-blue tinge because the underlying infiltration interferes with its venous drainage. • 50% may have enlarged cervical
  • 6. Diagnosis • The diagnosis is usually suspected on clinical examination and confirmed by FNAB or core biopsy. • FNAB has been shown to be accurate in 90% of cases. • CT and MRI are useful in defining the local extent of disease and identifying distant metastases
  • 7. High risk findings in US for malignancy 1. Micro-calcification 2. Hypo-echoic nodule / Solid 3. Irregular margin 4. Intra-nodular vascularity 5. More tall then wide
  • 8. Staging TNM CLASSIFICATION: • All anaplastic carcinoma is stage IV • Stage IV contains 3 stages: IV A ; Tumor is limited to the thyroid and surgically resectable. IV B : Tumor extending beyond thyroid and considered surgically irresectable. IV C: Tumor presented with distant metastasis.
  • 10. • Initial surgical resection or debulking may be considered if preoperative imaging suggests feasibility. • If not, chemotherapy and external beam radiation may be performed as first-line therapy, possibly followed by surgery in responsive cases. • Treatment is generally palliative (given its aggressive nature) and may include early tracheostomy for airway protection.
  • 11.
  • 12. Medullary carcinoma • These are tumors of the para-follicular (C cells) derived from the neural crest. • 25% Inherited (Germline mutation RET oncogene). • High levels of serum calcitonin and carcinoembryonic antigen are produced by many medullary tumors, which should be tested for in suspected cases. • Calcitonin levels fall after resection and rise again with recurrence, making it a valuable tumor marker in the follow-up of patients with this disease. • Some tumors are familial and account for 10–20% of all cases. • The majority of patients are between the ages of 50 and 70 years when the tumor is sporadic in nature
  • 13. • When the condition occurs in young adults (20–30 years), often with a family history, it may be a manifestation of the multiple endocrine neoplasia syndrome (MEN) types 2a or 2b. In these cases, the medullary thyroid carcinoma may be accompanied by associated conditions such as:
  • 14. Medullary Thyroid Cancer Occurs in Four Clinical Settings 1- Sporadic • 80% of all cases of medullary thyroid cancer. • Typically unilateral • No associated endocrinopathies • Peak onset 40 - 60. • Females predominance: 3:2 ratio. • One third will present with intractable diarrhea.
  • 15. 2-MEN 2-A (Multiple Endocrine Neoplasia 2 A). 1. bilateral medullary carcinoma 2. pheochromocytoma 3. hyperparathyroidism. • This syndrome is inherited as autosomal dominant. Because of this, males and females are equally affected. • Peak incidence of medullary carcinoma in these patients is in the 30's.
  • 16. 3-MEN 2 B This syndrome has medullary carcinoma • Inheritance is autosomal dominant as in MEN IIA (m=f) • Pheochromocytomas must be detected prior to any operation. • The idea here is to remove the pheochromocytoma first to remove the risk of severe hypertensive episodes while the thyroid or parathyroid is being operated on.
  • 17. 4-Inherited medullary carcinoma without associated endocrinopathies. • This form of medullary carcinoma is the least aggressive. • Like other types of thyroid cancers, the peak incidence is between the ages of 40 and 50.
  • 18. Presentation • The common presentation is a firm, smooth and distinct lump in the neck(most commonly in single lobe ). • It is indistinguishable from any other form of thyroid solitary nodule. • The symptoms which should make you think of medullary tumor, apart from a lump in the neck and the presence of the above lesions in the patient or their family, are diarrhea and flushing. Diarrhea occurs in one-third of the patients. The fluidity of the stool and the frequency of defecation are both increased.
  • 19. • Diarrhea is a feature in 30% of cases and this may be due to 5-hydroxytryptamine or prostaglandins produced by the tumor cells. • Involvement of lymph nodes occurs in 50–60% of cases of medullary carcinoma and blood-borne metastases are common.
  • 20. Diagnosis 1. Family history. 2. ↑Serum Calcitonin 3. ↑CEA 4. FNAB. • tumors are not TSH dependent and do not take up radioactive iodine.
  • 21. •Family members at risk should firstly be screened for(RET mutation) in their blood. when recognized as a carrier of the mutation, should be screened by neck ultrasound and calcitonin.
  • 22. Treatment • Standard treatment is total thyroidectomy and clearance of involved anterior cervical and superior mediastinal lymph nodes. Without metastases, operation is often curative but when nodes are involved, 10-year survival falls to about 50%. • External Beam Radiation for unresectable residual or recurrent tumor. • No effective Chemotherapy.
  • 23. FAMILIAL MTC (RET mutation +ve)  Prophylactic Thyroidectomy:  Before age of 6 yrs for MEN2A  Before age of 1 yr for MEN2B • Pheochromocytoma when associated should be operated first • During follow-up, calcitonin levels are monitored and raised levels indicate tumor recurrence. Surgical re-exploration of the neck may be undertaken to remove involved nodes.
  • 24. Malignant lymphoma • Thyroid lymphomas are rare and usually arise in pre- existing autoimmune (Hashimoto’s) thyroiditis. Most are non- Hodgkin lymphomas. Diagnosis can only be made histologically, by core needle biopsy or open biopsy, as FNA is inadequate. • The prognosis is good, particularly if there is no involvement of cervical lymph nodes. • Presentation – rapid growing goiter + diffuse pain • Median age is 7th decade.
  • 25. •Treatment is with radiotherapy, and survival depends on whether spread has extended beyond the thyroid capsule. For lesions within the capsule, 5-year survival is 85%, falling to 40% when local spread has occurred. • Response to irradiation is dramatic and radical surgery is unnecessary once the diagnosis is established by biopsy. • advanced disease is managed by a chemotherapy