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| LMDDx = | | LMDDx = | ||
| Stains = congo red +ve (amyloid deposits) | | Stains = congo red +ve (amyloid deposits) | ||
| IHC = calcitonin +ve, CEA +ve, chromogranin A +ve, synaptophysin +ve, thyroglobulin -ve (usually) | | IHC = calcitonin +ve, [[CEA]] +ve, chromogranin A +ve, synaptophysin +ve, thyroglobulin -ve (usually) | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = | ||
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| Gross = usu. well-circumscribed, white, gray or yellow, gritty, firm | | Gross = usu. well-circumscribed, white, gray or yellow, gritty, firm | ||
| Grossing = | | Grossing = | ||
| Staging = [[thyroid cancer staging]] | |||
| Site = [[thyroid gland]] | | Site = [[thyroid gland]] | ||
| Assdx = [[C-cell hyperplasia]] | | Assdx = [[C-cell hyperplasia]] | ||
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*May be genetic (MEN IIa/b syndrome). | *May be genetic (MEN IIa/b syndrome). | ||
*Arises from C cells (which produce calcitonin). | *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> | 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> | ||
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Serology: | 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> | *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== | ==Gross== | ||
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==Microscopic== | ==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". | *Nuclei with "neuroendocrine features". | ||
**Small, round nuclei. | **Small, round nuclei. | ||
**Coarse chromatin (''salt and pepper nuclei''). | **Coarse chromatin (''salt and pepper nuclei''). | ||
Surrounding Thyroid | |||
*+/-[[C-cell hyperplasia]] - seen with familial forms of MTC. | *+/-[[C-cell hyperplasia]] - seen with familial forms of MTC. | ||
**C cells (AKA ''parafollicular cell''): abundant cytoplasm - clear/pale. | **C cells (AKA ''parafollicular cell''): abundant cytoplasm - clear/pale. | ||
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DDx: | DDx: | ||
*[[Anaplastic thyroid carcinoma]]. | *Other thyroid tumours: | ||
*[[Papillary thyroid carcinoma]]. | **[[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=== | ===Images=== | ||
www: | www: | ||
*[http://www.anatomyatlases.org/MicroscopicAnatomy/Images/Plate287.jpg Parafollicular cells (anatomyatlases.org)]. | *[http://www.anatomyatlases.org/MicroscopicAnatomy/Images/Plate287.jpg Parafollicular cells (anatomyatlases.org)]. | ||
<gallery> | <gallery> | ||
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Image:Thyroid MedullaryCarcinoma SpindleCell HP PA.JPG|Thyroid - Medullary carcinoma - Spindle cell variant - high 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 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> | </gallery> | ||
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**[[Chromogranin A]]. | **[[Chromogranin A]]. | ||
**[[Synaptophysin]]. | **[[Synaptophysin]]. | ||
*CEA +ve (often better staining than calcitonin).<ref>SB. 7 January 2010.</ref> | *[[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> | *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== | ==EM== | ||
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Images: [http://pathhsw5m54.ucsf.edu/case7/image77.html Neurosecretory granules (ucsf.edu)]. | 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== | ==See also== |
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