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approach to medullar thyroid ca

  1. 1. + How To Approach MTC Sara Al-Ghanem
  2. 2. + 2 Objectives  Introduction  Pathology  Classification & clinical pic.  Approach to MTC
  3. 3. + History ( from the patient ) 3  33 yo female pt ,G7P4+2  Medically free  No sp  No hx of allergy  She was will till 1 year ago , when she started to suffer from neck pain in the RT side .  While she was eating her daughter accidently noted that she has a neck swelling on rt side .  The swelling increased in size with time  There was no skin changes or discharge coming from it  No other swelling in the body
  4. 4. + 4  She has symptoms suggesting hypothyroidism in the form of : cold intolerance, wt gain & malaise  also she is having compressive symptoms , like dysphagia ,hoarseness  No hx of aspiration or respiratory distress   No hx of neck radiation in childhood FHx: her aunt has a thyroid problem ? She did know the dx & no surgery was done for her
  5. 5. + 5 Hospital course  Initially she was dx with lymphadenitis in PHC & received a course of Ab . But she did not improve.  US was arranged & upon the finding , they FNA was requested  FNA showed hashimoto thyroididtis  after that she was referred to GS , & they did excisional LN biopsy for her .  Finally we received the pt in ENT clinic as a case of met medullary Ca in RT cervical LN
  6. 6. + 6 ENT Plan  The patient was admitted in the hospital on Tuesday under care of Dr.Al-Saab for further evaluation & multidisciplinary assessment with other teams before surgery
  7. 7. + 7 On EX By inspection of the neck:  1- there is goiter more prominent in RT side  2- scar in RT side of neck measuring about 3 cm ( in level II) By palpation :  There swelling is located in center of the neck  moving with deglutition  More prominent in RT side ( 3*2.5 cm ) , left side ( 2*2)  Firm, not fixed to underlying skin  With no substernal extension  Bil cerviacl lymphadenopathy ( level II)
  8. 8. + 8 Labs  Calcitonin  Calcium  Metanephrine ?
  9. 9. + 9 Imaging  CT of chest & neck ?  It was done yesterday but we are waiting the report
  10. 10. + 10 Surgery Plan ? That what I am looking for 
  11. 11. + 11 Introduction Medullary thyroid cancer (MTC) is a neuroendocrine tumor of the parafollicular or C cells of the thyroid gland
  12. 12. + 12 Gross pathology  the tumors of MTC are typically slow-growing, and gray and firm in appearance. Familial forms are usually multi-focal and bilateral.
  13. 13. + 13 Microscopic features ( histology )  Histologically, MTC is characterized by uniform polygonal cells with central nuclei and finely granular eosinophilic cytoplasm.  The finding of stromal amyloid is a distinguishing feature of MTC, but is present in only one-third of cases.  C-cell hyperplasia is a precursor to malignant transformation, and is most commonly seen in familial MTC  The presence of amyloid is a diagnostic finding, but immunohistochemistry for calcitonin is more commonly used as a diagnostic tumor marker.  These tumors also stain positively for CEA and calcitonin gene– related peptide.
  15. 15. + 15  MTC frequently metastasizes to regional lymph nodes, and these metastases are often apparent at the time of diagnosis. The frequency has been reported to be over 50% in patients who present with palpable, established primary tumors. Spread is most frequent to the central compartment (Level VI), followed by the ipsilateral jugular chain of nodes (Levels II through V) and the contralateral cervical nodes.  Spread is also seen to the upper and anterior mediastinum  Hematogenous spread may occur to the lungs, liver, bones, brain, and soft tissues .
  16. 16. + Medullary thyroid carcinoma (MTC) comprises 5– 10% of all thyroid cancers. 16
  17. 17. MTC is a sporadic malignancy in the + majority of cases (75%), with the remainder comprising three familial syndromes: MEN 2A, MEN 2B, and familial non-MEN MTC (FMTC). MEN 2A, the most common syndrome (up to 80% of hereditary cases) 17
  18. 18. + 18 Sporadic MTC  Sporadic MTC accounts for about 80 %  The typical age of presentation is in the fifth or sixth decade .  there may be a slight female preponderance  The most common presentation of sporadic MTC is that of a solitary thyroid nodule  In most patients, the disease has already metastasized at the time of diagnosis. 50%of patients have clinically detectable CLN involvement 15% have symptoms of upper aerodigestive tract compression. 5% have distant metastatic
  19. 19. + 19 Sporadic MTC  Systemic symptoms may occur due to hormonal secretion by the tumor.  Tumor secretion of calcitonin, calcitonin-gene related peptide, or other substances can cause :  diarrhea  or facial flushing in patients with advanced disease.  In addition, occasional tumors secrete corticotropin (ACTH), causing ectopic Cushing's syndrome.
  20. 20. + 20 Inherited MTC  MEN2 is subclassified into three distinct syndromes, each of which is transmitted in an autosomal dominant fashion and is associated with MTC: MEN2A MEN2B and familial medullary thyroid cancer (FMTC).  These syndromes result from different mutations in the RET proto-oncogene.
  21. 21. + 21 MEN2A  the most common syndrome (up to 80% of hereditary cases). M = multi-focal, bilateral MTC 100% penetrance P = pheochromocytoma  42% penetrance H = hyperpar- athyroidism  10 – 30% penetrance  Less commonly, this syndrome includes a skin condition, lichen planus amyloidosis, and/or Hirschsprung disease. 2 MPH or 2 Miles Per Hour
  22. 22. + 22 MEN 2B MTC (also with 100% penetrance), Marfanoid habitus. Mucosal neuromas,ganglioneuromatosis of the gastrointestinal tract, and megacolon. ( variable presence ) Pheochromocytoma (40% penetrance) MMMP (Think: 3M Plastics)
  23. 23. 23 +
  24. 24. + FMTC is characterized by development of MTC alone . 24
  25. 25. the clinical presentation and + manifestations of MEN2-associated MTC are similar to those of sporadic MTC. The most common presentation is that of a solitary thyroid nodule or cervical lymphadenopathy. 25
  26. 26. + Hx & PE FNA biopsy aided by immunocyto- chemical staining for calcitonin. Serum calcitonin and CEA Radiologic evaluation genetic testing for a hereditary syndrome. Testing for coexisting tumors STAGING 26
  27. 27. + Immunocytologic staining for calcitonin is positive (brown staining which is best seen at the arrow). 27
  28. 28. ultrasonography of the neck is indicated to look for cervical lymph node involvement. For patients with local lymph node metastases on ultrasound or with preoperative serum basal calcitonin >400 pg/mL : +  indicating high risk of local or distant metastatic disease  additional imaging is required to assess for metastatic disease:  chest CT, neck CT, three-phase contrast-enhanced liver CT or contrastenhanced liver MRI Radiologic evaluation 28
  29. 29. + genetic testing for a hereditary syndrome  All patients presenting with clinically evident MTC should have genetic testing to evaluate for a hereditary syndrome.  Approximately 6–10% of patients who present with MTC with no family history will be found to have a germline RET mutation.  In patients at-risk for familial MTC, the availability and accuracy of genetic screening for RET mutations has replaced yearly screening calcitonin measurement as the primary diagnostic tool because identification of a mutation allows diagnosis and treatment prior to the C-cell proliferation (either through hyperplasia or cancer) necessary to elevate calcitonin levels 29
  30. 30. + genetic testing for a hereditary syndrome  In patients at-risk for MEN 2B, genetic testing should be done immediately after birth, because MTC is usually already established by that time and early thyroidectomy is necessary to increase chance for cure.  In patients at-risk for FMTC or MEN 2A, genetic screening may be done in early childhood (before age 5 or 6), to allow planning for preventative thyroidectomy. 30
  31. 31. Stage I —  MTCs that are less than 2 cm in diameter  without evidence of disease outside of the thyroid gland Stage II —  Any tumor between 2 and 4 cm  without evidence of extrathyroidal disease Stage III —  Any tumor greater than 4 cm,  or level VI nodal metastases  or microscopic extrathyroidal invasion regardless of tumor size Stage IV —  Any distant metastases,  or lymph node involvement outside of level VI,  or gross soft tissue extension + 31
  32. 32. + 32 SURGICAL STRATEGY FOR MTC  the operative procedure recommended is a total thyroidectomy with a central compartment lymphadenectomy  and parathyroid auto- transplantation,  and ipsilateral functional neck dissection (Levels II – V)  Bilateral neck dissections may be considered.  Radioactive iodine, external beam radiation therapy (EBRT), and conventional chemotherapy have not been effective.
  33. 33. + Points to Remember 33
  34. 34.  Medullary thyroid cancer (MTC) is a neuroendocrine tumor of the parafollicular or C cells of the thyroid gland  A characteristic feature of this tumor is the production of calcitonin. Most cases are sporadic  For patients diagnosed with MTC on the basis of cytologic evaluation of a thyroid nodule, evaluation should include measurement of serum calcitonin, carcinoembryonic antigen , US of the neck, genetic testing for germline RET mutations, and biochemical evaluation for coexisting tumors, especially pheochromocytoma +  Genetic analysis, along with imaging and calcitonin testing aid in optimizing surgical resection in these cases. 34
  35. 35. + 35 References Current approaches to medullary thyroid carcinoma, sporadic and familial.
  36. 36. + 36