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# | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | |||
| Image = Medullary thyroid carcinoma - 2 - high mag.jpg | |||
| Width = | |||
| Caption = Medullary thyroid carcinoma. [[H&E stain]]. | |||
| Synonyms = | |||
| Micro = nuclei with neuroendocrine features (round nuclei with salt-and-pepper chromatin), +/-[[amyloid]] deposits (fluffy appearing acellular eosinophilic material), +/-[[C-cell hyperplasia]] | |||
| Subtypes = | |||
| LMDDx = | |||
| Stains = congo red +ve (amyloid deposits) | |||
| IHC = calcitonin +ve, [[CEA]] +ve, chromogranin A +ve, synaptophysin +ve, thyroglobulin -ve (usually) | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = usu. well-circumscribed, white, gray or yellow, gritty, firm | |||
| Grossing = | |||
| Staging = [[thyroid cancer staging]] | |||
| Site = [[thyroid gland]] | |||
| Assdx = [[C-cell hyperplasia]] | |||
| Syndromes = [[multiple endocrine neoplasia IIa]], [[multiple endocrine neoplasia IIb]] | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = +/-serum calcitonin elevated | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = poor | |||
| Other = | |||
| ClinDDx = | |||
| Tx = | |||
}} | |||
'''Medullary thyroid carcinoma''', abbreviated '''MTC''', is an uncommon epithelial [[malignancy]] of the thyroid gland that may be syndromic. | |||
==General== | |||
Medical school memory device - 3 M's: | |||
*[[amyloid|aMyloid]]. | |||
*Median node dissection done. | |||
*[[MEN IIa syndrome]]/[[MEN IIb syndrome]]. | |||
**Medullary thyroid carcinoma. | |||
**[[Pheochromocytoma]]. | |||
**[[Parathyroid adenoma]]. | |||
Epidemiology: | |||
*Very rare. | |||
*Poor prognosis. | |||
*May be genetic (MEN IIa/b syndrome). | |||
*Arises from C cells (which produce calcitonin). | |||
Sporadic tumours | |||
*~80% | |||
*Slightly older age at presentation (~45) | |||
*Tend to be solitary | |||
Syndromic tumours - typically:<ref name=pmid21455198>{{Cite journal | last1 = Nosé | first1 = V. | title = Familial thyroid cancer: a review. | journal = Mod Pathol | volume = 24 Suppl 2 | issue = | pages = S19-33 | month = Apr | year = 2011 | doi = 10.1038/modpathol.2010.147 | PMID = 21455198 |URL = http://www.nature.com/modpathol/journal/v24/n2s/full/modpathol2010147a.html }}</ref> | |||
*Present in 30s or 40s. | |||
*+/-Multifocal. | |||
*+/-Bilateral. | |||
*[[C-cell hyperplasia]]. | |||
Serology: | |||
*Serum calcitonin classically elevated.<ref name=pmid24470736>{{Cite journal | last1 = Vainas | first1 = I. | last2 = Marthopoulos | first2 = A. | last3 = Chrisoulidou | first3 = A. | last4 = Raptou | first4 = K. | last5 = Tziomalos | first5 = K. | last6 = Pazaitou-Panayiotou | first6 = K. | title = Calcitonin stimulation tests for the early diagnosis and follow-up of patients with C cell disease: a descriptive analysis. | journal = Hippokratia | volume = 17 | issue = 3 | pages = 246-51 | month = Jul | year = 2013 | doi = | PMID = 24470736 }}</ref> | |||
*CEA may also be elevated. | |||
==Gross== | |||
Features:<ref name=pmid21455198/> | |||
*Usu. well-circumscribed. | |||
*White, gray or yellow. | |||
*Gritty. | |||
*Firm. | |||
Image: | |||
*[http://www.nature.com/modpathol/journal/v24/n2s/fig_tab/modpathol2010147f2.html MTC (nature.com)]. | |||
==Microscopic== | |||
Architecture - various | |||
*Nested with delicate vascular septa | |||
*Trabecular | |||
*Tubular/glandular | |||
*Pseudo-papillary | |||
Cells | |||
*Polygonal to spindle to small cells | |||
*Amphophilic, somewhat granular cytoplasm | |||
*Cells may have a more bizarre appearance | |||
*Cells may appear to be 'falling apart'due to interstitial oedema. | |||
Stroma | |||
*+/-[[Amyloid]] deposits - fluffy appearing acellular eosinophilic material in the cytoplasm. | |||
*Stroma is vascular and can show haemorrhage, hyalinised collagen, oedema or metaplastic bone | |||
*Coarse calcification | |||
*True psammoma bodies may be present | |||
Nuclei | |||
*Nuclei with "neuroendocrine features". | |||
**Small, round nuclei. | |||
**Coarse chromatin (''salt and pepper nuclei''). | |||
Surrounding Thyroid | |||
*+/-[[C-cell hyperplasia]] - seen with familial forms of MTC. | |||
**C cells (AKA ''parafollicular cell''): abundant cytoplasm - clear/pale. | |||
Note: | |||
*The amyloid is formed from ''calcitonin''.<ref name=pmid15459123>{{Cite journal | last1 = Khurana | first1 = R. | last2 = Agarwal | first2 = A. | last3 = Bajpai | first3 = VK. | last4 = Verma | first4 = N. | last5 = Sharma | first5 = AK. | last6 = Gupta | first6 = RP. | last7 = Madhusudan | first7 = KP. | title = Unraveling the amyloid associated with human medullary thyroid carcinoma. | journal = Endocrinology | volume = 145 | issue = 12 | pages = 5465-70 | month = Dec | year = 2004 | doi = 10.1210/en.2004-0780 | PMID = 15459123 }}</ref> | |||
DDx: | |||
*Other thyroid tumours: | |||
**[[Anaplastic thyroid carcinoma]]. | |||
**[[Papillary thyroid carcinoma]]. | |||
**[[Hurthle cell carcinoma]] | |||
***The oncocytic variant of medullary carcinoma can be confused with [[Hurthle cell carcinoma]]. Clues to suggest medullary carcinoma: | |||
****Cytoplasm is amphophilic as opposed to eosinophilic | |||
****Nests of tumour cells separated by fibrous septa | |||
*[[C-cell hyperplasia]]. | |||
**Invasive medullary carcinoma shows fibrosis around tumor cells and stains more weakly for calcitonin. | |||
*Other neuroendocrine tumours - primary or metastatic: | |||
**[[Paraganglioma]] - negative for keratin, calcitonin and [[CEA]]. | |||
**[[Carcinoid]] - negative for calcitonin. | |||
*Metastatic [[melanoma]]. | |||
**Pigment. | |||
**Melanoma markers positive, calcitonin and CEA negative. | |||
===Images=== | |||
www: | |||
*[http://www.anatomyatlases.org/MicroscopicAnatomy/Images/Plate287.jpg Parafollicular cells (anatomyatlases.org)]. | |||
<gallery> | |||
Image:Medullary_thyroid_carcinoma_-_low_mag.jpg | MTC - low mag. (WC) | |||
Image:Medullary_thyroid_carcinoma_-_high_mag.jpg | MTC - high mag. (WC) | |||
Image:Medullary_thyroid_carcinoma_-_2_-_high_mag.jpg | MTC and amyloid - high mag. (WC) | |||
Image:Thyroid MedullaryCarcinoma 216 PA.JPG|Thyroid - Medullary carcinoma - low power (SKB) | |||
Image:Thyroid MedullaryCarcinoma 217 PA.JPG|Thyroid - Medullary carcinoma - low power (SKB) | |||
Image:Thyroid MedullaryCarcinoma Amyloid RBWH.JPG|Thyroid - Medullary carcinoma - amyloid (SKB) | |||
Image:Thyroid MedullaryCarcinoma Amyloid MP3 PA.JPG|Thyroid - Medullary carcinoma - amyloid - medium power (SKB) | |||
Image:Thyroid MedullaryCarcinoma Amyloid MP2 PA.JPG|Thyroid - Medullary carcinoma - amyloid - medium power (SKB) | |||
Image:Thyroid MedullaryCarcinoma Amyloid MP4 PA.JPG|Thyroid - Medullary carcinoma - amyloid - medium power (SKB) | |||
Image:Thyroid MedullaryCarcinoma Amyloid MP PA.JPG|Thyroid - Medullary carcinoma - amyloid - medium power (SKB) | |||
Image:Thyroid MedullaryCarcinoma Amyloid HP PA.JPG|Thyroid - Medullary carcinoma - amyloid - high power (SKB) | |||
Image:Thyroid MedullaryCarcinoma Comedonecrosis LP2 CTR.jpg|Thyroid - Medullary carcinoma - comedonecrosis - low power (SKB) | |||
Image:Thyroid MedullaryCarcinoma Comedonecrosis LP CTR.jpg|Thyroid - Medullary carcinoma - comedonecrosis - medium power (SKB) | |||
Image:Thyroid MedullaryCarcinoma Comedonecrosis HP CTR.jpg|Thyroid - Medullary carcinoma - comedonecrosis - high power (SKB) | |||
Image:Thyroid MedullaryCarcinoma SpindleCell LP PA.JPG|Thyroid - Medullary carcinoma - Spindle cell variant - low power (SKB) | |||
Image:Thyroid MedullaryCarcinoma SpindleCell MP PA.JPG|Thyroid - Medullary carcinoma - Spindle cell variant - medium power (SKB) | |||
Image:Thyroid MedullaryCarcinoma SpindleCell LP2 PA.JPG|Thyroid - Medullary carcinoma - Spindle cell variant - low power (SKB) | |||
Image:Thyroid MedullaryCarcinoma SpindleCell HP PA.JPG|Thyroid - Medullary carcinoma - Spindle cell variant - high power (SKB) | |||
Image:Thyroid MedullaryCarcinoma SmallCell HP PA.JPG|Thyroid - Medullary carcinoma - Small cell variant - high power (SKB) | |||
Image:Thyroid MedullaryCarcinoma SmallCellVariant MP CTR.jpg|Thyroid - Medullary carcinoma - small cell variant - medium power (SKB) | |||
Image:Thyroid MedullaryCarcinoma SmallCellVariant HP CTR (3).jpg|Thyroid - Medullary carcinoma - small cell variant - high power (SKB) | |||
Image:Thyroid MedullaryCarcinoma MP CTR (2).jpg|Thyroid - Medullary carcinoma - medium power (SKB) | |||
Image:Thyroid MedullaryCarcinoma HP2 CTR.jpg|Thyroid - Medullary carcinoma - high power (SKB) | |||
</gallery> | |||
==Stains== | |||
*Congo-red +ve (amyloid present) - mnemonic: ''CRAP'' -- congo red amyloid protein. | |||
==IHC== | |||
Features:<ref>URL: [http://pathologyoutlines.com/thyroid.html#medullary http://pathologyoutlines.com/thyroid.html#medullary]. Accessed on: 17 January 2011.</ref> | |||
*[[Calcitonin]] +ve - it arises from C cells (which produce calcitonin). | |||
*Neuroendocrine markers. | |||
**[[Chromogranin A]]. | |||
**[[Synaptophysin]]. | |||
*[[CEA]] +ve (often better staining than calcitonin).<ref>SB. 7 January 2010.</ref> | |||
*Thyroglobulin usu. -ve.<ref name=pmid8454270>{{Cite journal | last1 = de Micco | first1 = C. | last2 = Chapel | first2 = F. | last3 = Dor | first3 = AM. | last4 = Garcia | first4 = S. | last5 = Ruf | first5 = J. | last6 = Carayon | first6 = P. | last7 = Henry | first7 = JF. | last8 = Lebreuil | first8 = G. | title = Thyroglobulin in medullary thyroid carcinoma: immunohistochemical study with polyclonal and monoclonal antibodies. | journal = Hum Pathol | volume = 24 | issue = 3 | pages = 256-62 | month = Mar | year = 1993 | doi = | PMID = 8454270 }}</ref> | |||
*TTF-1 +ve | |||
==EM== | |||
*Neurosecretory granules. | |||
**Feature seen in neuroendocrine tumours. | |||
Images: [http://pathhsw5m54.ucsf.edu/case7/image77.html Neurosecretory granules (ucsf.edu)]. | |||
==Sign out== | |||
<pre> | |||
Lesion, Liver, Core Biopsy: | |||
- METASTATIC MEDULLARY THYROID CARCINOMA, see comment. | |||
Comment: | |||
Stains/IHC confirm the morphologic findings; the tumour stains as follows: | |||
POSITIVE: calcitonin, CEAp, synaptophysin, CD56, TTF-1 (focal, moderate), congo red (confirms the presence of amyloid). | |||
NEGATIVE: thyroglobulin, CDX2. | |||
</pre> | |||
===Micro=== | |||
The sections show cells of intermediate size without apparent nucleoli, moderate eosinophilic cytoplasm, arranged in nests, focally associated with amorphous acellular cotton candy-like material. | |||
The cotton candy-like material has a light apple-green appearance when polarized. | |||
==See also== | |||
*[[Thyroid gland]]. | |||
*[[Medullary carcinoma]]. | |||
==References== | |||
{{reflist|2}} | |||
[[Category:Thyroid gland]] | |||
[[Category:Diagnosis]] |
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