Difference between revisions of "Renal mucinous tubular and spindle cell carcinoma"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      = Renal mucinous tubular and spindle cell carcinoma -- high mag.jpg
| Image      = Renal mucinous tubular and spindle cell carcinoma - 2 -- high mag.jpg
| Width      =
| Width      =
| Caption    = Renal mucinous tubular and spindle cell carcinoma. [[H&E stain]].
| Caption    = Renal mucinous tubular and spindle cell carcinoma. [[H&E stain]].
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| Micro      = mucin (foamy macrophages) - may be scant, spindle cells, small tubules - lined by cuboidal epithelium
| Micro      = mucin (foamy macrophages) - may be scant, spindle cells, small tubules - lined by cuboidal epithelium
| Subtypes  =
| Subtypes  =
| LMDDx      =
| LMDDx      = sarcomatoid [[papillary renal cell carcinoma]], [[papillary renal cell carcinoma]], [[metanephric adenoma]]
| Stains    =
| Stains    =
| IHC        = CK7 +ve, AMACR +ve, CD10 -ve, AE1/AE3 +ve
| IHC        = CK7 +ve, AMACR +ve, CD10 -ve, AE1/AE3 +ve
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| Prevalence = rare
| Prevalence = rare
| Bloodwork  =
| Bloodwork  =
| Rads      =
| Rads      = features overlap with [[papillary renal cell carcinoma]]
| Endoscopy  =
| Endoscopy  =
| Prognosis  = good
| Prognosis  = good
| Other      =
| Other      =
| ClinDDx    =
| ClinDDx    = other [[kidney tumours]]
| Tx        = surgical excision
| Tx        = surgical excision
}}
}}
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==General==
==General==
*Rare.<ref name=pmid19494850>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | title = Uncommon and recently described renal carcinomas. | journal = Mod Pathol | volume = 22 Suppl 2 | issue =  | pages = S2-S23 | month = Jun | year = 2009 | doi = 10.1038/modpathol.2009.70 | PMID = 19494850 }}</ref>
*Rare<ref name=pmid19494850>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | title = Uncommon and recently described renal carcinomas. | journal = Mod Pathol | volume = 22 Suppl 2 | issue =  | pages = S2-S23 | month = Jun | year = 2009 | doi = 10.1038/modpathol.2009.70 | PMID = 19494850 }}</ref> - largest reported series (published in 2015) has 19 cases.<ref name=pmid25395040/>  
*Accepted by WHO in 2004 as a separate entity.<ref name=pmid19805959>{{cite journal |author=Geramizadeh B, Salehipour M, Moradi A |title=Mucinous tubular and spindle cell carcinoma of kidney: a rare case report and review of the literature |journal=Indian J Pathol Microbiol |volume=52 |issue=4 |pages=514–6 |year=2009 |pmid=19805959 |doi=10.4103/0377-4929.56144 |url=}}</ref>
*Accepted by WHO in 2004 as a separate entity.<ref name=pmid19805959>{{cite journal |author=Geramizadeh B, Salehipour M, Moradi A |title=Mucinous tubular and spindle cell carcinoma of kidney: a rare case report and review of the literature |journal=Indian J Pathol Microbiol |volume=52 |issue=4 |pages=514–6 |year=2009 |pmid=19805959 |doi=10.4103/0377-4929.56144 |url=}}</ref>
*Relatively good prognosis.
*Relatively good prognosis.
**In a series of 19 patients: one died, one developed mets, one recurred (post-ablation) and was salvaged by surgery, one observed... remaining 15 cured by surgery or ablation.<ref name=pmid25395040>{{Cite journal  | last1 = Kenney | first1 = PA. | last2 = Vikram | first2 = R. | last3 = Prasad | first3 = SR. | last4 = Tamboli | first4 = P. | last5 = Matin | first5 = SF. | last6 = Wood | first6 = CG. | last7 = Karam | first7 = JA. | title = Mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney: a detailed study of radiological, pathological and clinical outcomes. | journal = BJU Int | volume = 116 | issue = 1 | pages = 85-92 | month = Jul | year = 2015 | doi = 10.1111/bju.12992 | PMID = 25395040 }}</ref>


==Gross==
==Gross==
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*Greyish-yellow or tan-yellow.
*Greyish-yellow or tan-yellow.
*Well-circumscribed.
*Well-circumscribed.
Note:
*Imaging characteristics overlap with [[papillary renal cell carcinoma]].<ref name=pmid25395040/>


==Microscopic==
==Microscopic==
Features:<ref name=pmid19066686>{{cite journal |author=Trabelsi A, Stita W, Yacoubi MT, Rammeh S, Hmissa S, Korbi S |title=Renal mucinous tubular and spindle cell carcinoma |journal=Can Urol Assoc J |volume=2 |issue=6 |pages=635–6 |year=2008 |month=December |pmid=19066686 |pmc=2593603 |doi= |url=}}</ref>
Features:<ref name=pmid19066686>{{cite journal |author=Trabelsi A, Stita W, Yacoubi MT, Rammeh S, Hmissa S, Korbi S |title=Renal mucinous tubular and spindle cell carcinoma |journal=Can Urol Assoc J |volume=2 |issue=6 |pages=635–6 |year=2008 |month=December |pmid=19066686 |pmc=2593603 |doi= |url=}}</ref>
*Mucin (foamy macrophages) - may be scant.
*Mucin - may be scant.
**Often within foamy macrophages.
*Spindle cells - '''key feature'''.
*Spindle cells - '''key feature'''.
**Often seen only focally.
**Often seen only focally.
Line 56: Line 61:
*Sarcomatoid [[papillary renal cell carcinoma]]<ref name=pmid18941398>{{cite journal |author=Dhillon J, Amin MB, Selbs E, Turi GK, Paner GP, Reuter VE |title=Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change |journal=Am. J. Surg. Pathol. |volume=33 |issue=1 |pages=44–9 |year=2009 |month=January |pmid=18941398 |doi=10.1097/PAS.0b013e3181829ed5 |url=}}</ref> (papillary renal cell carcinoma with [[sarcomatoid RCC|sarcomatoid differentiation]]).
*Sarcomatoid [[papillary renal cell carcinoma]]<ref name=pmid18941398>{{cite journal |author=Dhillon J, Amin MB, Selbs E, Turi GK, Paner GP, Reuter VE |title=Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change |journal=Am. J. Surg. Pathol. |volume=33 |issue=1 |pages=44–9 |year=2009 |month=January |pmid=18941398 |doi=10.1097/PAS.0b013e3181829ed5 |url=}}</ref> (papillary renal cell carcinoma with [[sarcomatoid RCC|sarcomatoid differentiation]]).
*[[Papillary renal cell carcinoma]].
*[[Papillary renal cell carcinoma]].
*[[Metanephric adenoma]].


===Grading===
===Grading===
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===Images===
===Images===
====Case 1====
<gallery>
Image: Renal mucinous tubular and spindle cell carcinoma - 2 -- very low mag.jpg | RMTSCC - very low mag.
Image: Renal mucinous tubular and spindle cell carcinoma - 2 -- low mag.jpg | RMTSCC - low mag.
Image: Renal mucinous tubular and spindle cell carcinoma - 2 -- intermed mag.jpg | RMTSCC - intermed. mag.
Image: Renal mucinous tubular and spindle cell carcinoma - 2 -- high mag.jpg | RMTSCC - high mag.
Image: Renal mucinous tubular and spindle cell carcinoma - 2 -- very high mag.jpg | RMTSCC - very high mag.
Image: Renal mucinous tubular and spindle cell carcinoma - 2 alt -- very high mag.jpg | RMTSCC - very high mag.
</gallery>
====Case 2====
<gallery>
<gallery>
Image: Renal mucinous tubular and spindle cell carcinoma -- low mag.jpg | RMTSCC - low mag.
Image: Renal mucinous tubular and spindle cell carcinoma -- low mag.jpg | RMTSCC - low mag.
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Others:
Others:
*PAX-8 +ve.
*[[PAX8]] +ve.
*CAM5.2 +ve.
*[[CAM5.2]] +ve.
*p63 -ve.
*[[p63]] -ve.
*CK20 -ve.<ref name=pmid17058608>{{Cite journal  | last1 = Arias | first1 = LF. | last2 = Blanco | first2 = J. | last3 = Hernández | first3 = S. | last4 = Bocardo | first4 = G. | last5 = González | first5 = L. | title = [Immunohistochemical profile and clinical features of mucinous tubular and spindle renal cell carcinoma]. | journal = Actas Urol Esp | volume = 30 | issue = 7 | pages = 649-54 | month =  | year =  | doi =  | PMID = 17058608 }}</ref>
*[[CK20]] -ve.<ref name=pmid17058608>{{Cite journal  | last1 = Arias | first1 = LF. | last2 = Blanco | first2 = J. | last3 = Hernández | first3 = S. | last4 = Bocardo | first4 = G. | last5 = González | first5 = L. | title = [Immunohistochemical profile and clinical features of mucinous tubular and spindle renal cell carcinoma]. | journal = Actas Urol Esp | volume = 30 | issue = 7 | pages = 649-54 | month =  | year =  | doi =  | PMID = 17058608 }}</ref>
*[[CD57]] +ve.<ref name=pmid18459456>{{Cite journal  | last1 = Okoń | first1 = K. | last2 = Klimkowska | first2 = A. | last3 = Pawelec | first3 = A. | last4 = Dobrowolski | first4 = Z. | last5 = Kohla | first5 = Z. | last6 = Stachura | first6 = J. | title = Immunophenotype and cytogenetics of mucinous tubular and spindle cell carcinoma of the kidney. | journal = Pol J Pathol | volume = 58 | issue = 4 | pages = 227-33 | month =  | year = 2007 | doi =  | PMID = 18459456 }}</ref><ref name=pmid14989744>{{Cite journal  | last1 = Kuroda | first1 = N. | last2 = Nakamura | first2 = S. | last3 = Miyazaki | first3 = E. | last4 = Hayashi | first4 = Y. | last5 = Taguchi | first5 = T. | last6 = Hiroi | first6 = M. | last7 = Yamasaki | first7 = Y. | last8 = Shuin | first8 = T. | last9 = Enzan | first9 = H. | title = Low-grade tubular-mucinous renal neoplasm with neuroendocrine differentiation: a histological, immunohistochemical and ultrastructural study. | journal = Pathol Int | volume = 54 | issue = 3 | pages = 201-7 | month = Mar | year = 2004 | doi = 10.1111/j.1440-1827.2004.01608.x | PMID = 14989744 }}</ref>
*CK19 +ve.<ref name=pmid14989744/>


Note:
Note:
*Immunophenotype overlaps with [[papillary renal cell carcinoma]].<ref name=pmid{{Cite journal  | last1 = Paner | first1 = GP. | last2 = Srigley | first2 = JR. | last3 = Radhakrishnan | first3 = A. | last4 = Cohen | first4 = C. | last5 = Skinnider | first5 = BF. | last6 = Tickoo | first6 = SK. | last7 = Young | first7 = AN. | last8 = Amin | first8 = MB. | title = Immunohistochemical analysis of mucinous tubular and spindle cell carcinoma and papillary renal cell carcinoma of the kidney: significant immunophenotypic overlap warrants diagnostic caution. | journal = Am J Surg Pathol | volume = 30 | issue = 1 | pages = 13-9 | month = Jan | year = 2006 | doi =  | PMID = 16330937 }}>{{Cite journal  | last1 = Paner | first1 = GP. | last2 = Srigley | first2 = JR. | last3 = Radhakrishnan | first3 = A. | last4 = Cohen | first4 = C. | last5 = Skinnider | first5 = BF. | last6 = Tickoo | first6 = SK. | last7 = Young | first7 = AN. | last8 = Amin | first8 = MB. | title = Immunohistochemical analysis of mucinous tubular and spindle cell carcinoma and papillary renal cell carcinoma of the kidney: significant immunophenotypic overlap warrants diagnostic caution. | journal = Am J Surg Pathol | volume = 30 | issue = 1 | pages = 13-9 | month = Jan | year = 2006 | doi =  | PMID = 16330937 }}</ref>
*Immunophenotype overlaps with [[papillary renal cell carcinoma]].<ref name=pmid16330937>{{Cite journal  | last1 = Paner | first1 = GP. | last2 = Srigley | first2 = JR. | last3 = Radhakrishnan | first3 = A. | last4 = Cohen | first4 = C. | last5 = Skinnider | first5 = BF. | last6 = Tickoo | first6 = SK. | last7 = Young | first7 = AN. | last8 = Amin | first8 = MB. | title = Immunohistochemical analysis of mucinous tubular and spindle cell carcinoma and papillary renal cell carcinoma of the kidney: significant immunophenotypic overlap warrants diagnostic caution. | journal = Am J Surg Pathol | volume = 30 | issue = 1 | pages = 13-9 | month = Jan | year = 2006 | doi =  | PMID = 16330937 }}</ref>


==Sign out==
==Sign out==

Latest revision as of 20:08, 20 December 2019

Renal mucinous tubular and spindle cell carcinoma
Diagnosis in short

Renal mucinous tubular and spindle cell carcinoma. H&E stain.
LM mucin (foamy macrophages) - may be scant, spindle cells, small tubules - lined by cuboidal epithelium
LM DDx sarcomatoid papillary renal cell carcinoma, papillary renal cell carcinoma, metanephric adenoma
IHC CK7 +ve, AMACR +ve, CD10 -ve, AE1/AE3 +ve
Gross solid, greyish-yellow or tan-yellow, well-circumscribed
Grossing notes partial nephrectomy grossing, total nephrectomy for tumour grossing
Site kidney - see kidney tumours
Prevalence rare
Radiology features overlap with papillary renal cell carcinoma
Prognosis good
Clin. DDx other kidney tumours
Treatment surgical excision

Renal mucinous tubular and spindle cell carcinoma, abbreviated RMTSCC or MTSCC, is a rare malignant epithelial neoplasm of the kidney.

It is also known as renal mucinous tubular spindle cell carcinoma and mucinous tubular and spindle cell carcinoma of the kidney.[1]

General

  • Rare[2] - largest reported series (published in 2015) has 19 cases.[3]
  • Accepted by WHO in 2004 as a separate entity.[4]
  • Relatively good prognosis.
    • In a series of 19 patients: one died, one developed mets, one recurred (post-ablation) and was salvaged by surgery, one observed... remaining 15 cured by surgery or ablation.[3]

Gross

Features:[5]

  • Solid.
  • Greyish-yellow or tan-yellow.
  • Well-circumscribed.

Note:

Microscopic

Features:[6]

  • Mucin - may be scant.
    • Often within foamy macrophages.
  • Spindle cells - key feature.
    • Often seen only focally.
  • Small tubules - lined by cuboidal epithelium.[5]

DDx:

Grading

Images

Case 1

  • RMTSCC - very low mag.

  • RMTSCC - low mag.

  • RMTSCC - intermed. mag.

  • RMTSCC - high mag.

  • RMTSCC - very high mag.

  • RMTSCC - very high mag.

Case 2

  • RMTSCC - low mag.

  • RMTSCC - intermed. mag.

  • RMTSCC - low mag.

  • RMTSCC - very high mag.

www

Stains

IHC

Features:[13]

  • CK7 +ve.
  • AMACR +ve.
  • CD10 -ve.
    • 15% +ve (vs. 80% +ve in PaRCC).[14]
    • 11% +ve (vs. 80% in PaRCC).[15]
  • AE1/AE3 +ve.

Others:

Note:

Sign out

Left Kidney, Partial Nephrectomy:
- RENAL MUCINOUS TUBULAR AND SPINDLE CELL CARCINOMA (RMTSCC).
-- Margins clear.
-- ISUP nucleolar grade 2.
-- TNM stage: pT1a pNx.
-- Please see tumour summary.

Comment:
The stains are as follows:
POSITIVE: CK7, AMACR, AE1/AE3, mucicarmine.
NEGATIVE: WT-1, CD56, CD57, CD10, CD68.

Notes:

  • The (bland) spindle areas are not sarcomatoid as per the synoptic report.

Micro

The tumour consists of small cells with mild pleomorphism and mild-to-moderate cytoplasm. A tubular architecture is predominant and mucin is present. Focal spindled areas are also seen.

See also

References

  1. ↑ Brandal, P.; Lie, AK.; Bassarova, A.; Svindland, A.; Risberg, B.; Danielsen, H.; Heim, S. (Feb 2006). "Genomic aberrations in mucinous tubular and spindle cell renal cell carcinomas.". Mod Pathol 19 (2): 186-94. doi:10.1038/modpathol.3800499. PMID 16258504.
  2. ↑ Srigley, JR.; Delahunt, B. (Jun 2009). "Uncommon and recently described renal carcinomas.". Mod Pathol 22 Suppl 2: S2-S23. doi:10.1038/modpathol.2009.70. PMID 19494850.
  3. ↑ 3.0 3.1 3.2 Kenney, PA.; Vikram, R.; Prasad, SR.; Tamboli, P.; Matin, SF.; Wood, CG.; Karam, JA. (Jul 2015). "Mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney: a detailed study of radiological, pathological and clinical outcomes.". BJU Int 116 (1): 85-92. doi:10.1111/bju.12992. PMID 25395040.
  4. ↑ Geramizadeh B, Salehipour M, Moradi A (2009). "Mucinous tubular and spindle cell carcinoma of kidney: a rare case report and review of the literature". Indian J Pathol Microbiol 52 (4): 514–6. doi:10.4103/0377-4929.56144. PMID 19805959.
  5. ↑ 5.0 5.1 5.2 Wu, XR.; Chen, YH.; Sha, JJ.; Zhao, L.; Huang, JW.; Bo, JJ.; Liu, DM.; Huang, YR. (Dec 2013). "Renal mucinous tubular and spindle cell carcinoma: a report of 8 cases and review of the literature.". Diagn Pathol 8 (1): 206. doi:10.1186/1746-1596-8-206. PMID 24330589.
  6. ↑ Trabelsi A, Stita W, Yacoubi MT, Rammeh S, Hmissa S, Korbi S (December 2008). "Renal mucinous tubular and spindle cell carcinoma". Can Urol Assoc J 2 (6): 635–6. PMC 2593603. PMID 19066686. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2593603/.
  7. ↑ Dhillon J, Amin MB, Selbs E, Turi GK, Paner GP, Reuter VE (January 2009). "Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change". Am. J. Surg. Pathol. 33 (1): 44–9. doi:10.1097/PAS.0b013e3181829ed5. PMID 18941398.
  8. ↑ Gross, M. (Apr 1989). "Vertical dimension--Part II.". Refuat Hashinayim 7 (2): 8-18. PMID 2637792.
  9. ↑ Kuroda, N.; Hes, O.; Michal, M.; Nemcova, J.; Gal, V.; Yamaguchi, T.; Kawada, T.; Imamura, Y. et al. (Dec 2008). "Mucinous tubular and spindle cell carcinoma with Fuhrman nuclear grade 3: a histological, immunohistochemical, ultrastructural and FISH study.". Histol Histopathol 23 (12): 1517-23. PMID 18830937.
  10. ↑ Kuroda, N.; Hes, O.; Michal, M.; Nemcova, J.; Gal, V.; Yamaguchi, T.; Kawada, T.; Imamura, Y. et al. (Dec 2008). "Mucinous tubular and spindle cell carcinoma with Fuhrman nuclear grade 3: a histological, immunohistochemical, ultrastructural and FISH study.". Histol Histopathol 23 (12): 1517-23. PMID 18830937.
  11. ↑ URL: http://www.cttr.org/cms/?p=1406. Accessed on: 28 February 2014.
  12. ↑ Hussain, M.; Ud Din, N.; Azam, M.; Loya, A.. "Mucinous tubular and spindle cell carcinoma of kidney: a clinicopathologic study of six cases.". Indian J Pathol Microbiol 55 (4): 439-42. doi:10.4103/0377-4929.107776. PMID 23455776.
  13. ↑ Ferlicot S, Allory Y, Compérat E, et al. (December 2005). "Mucinous tubular and spindle cell carcinoma: a report of 15 cases and a review of the literature". Virchows Arch. 447 (6): 978–83. doi:10.1007/s00428-005-0036-x. PMID 16231179.
  14. ↑ 14.0 14.1 Paner, GP.; Srigley, JR.; Radhakrishnan, A.; Cohen, C.; Skinnider, BF.; Tickoo, SK.; Young, AN.; Amin, MB. (Jan 2006). "Immunohistochemical analysis of mucinous tubular and spindle cell carcinoma and papillary renal cell carcinoma of the kidney: significant immunophenotypic overlap warrants diagnostic caution.". Am J Surg Pathol 30 (1): 13-9. PMID 16330937.
  15. ↑ Sarsik, B.; Sımşır, A.; Karaarslan, S.; Sen, S. (May 2011). "Mucinous tubular and spindle cell carcinoma of kidney and problems in diagnosis.". Turk Patoloji Derg 27 (2): 116-26. doi:10.5146/tjpath.2011.01059. PMID 21630197.
  16. ↑ Arias, LF.; Blanco, J.; Hernández, S.; Bocardo, G.; González, L.. "[Immunohistochemical profile and clinical features of mucinous tubular and spindle renal cell carcinoma].". Actas Urol Esp 30 (7): 649-54. PMID 17058608.
  17. ↑ Okoń, K.; Klimkowska, A.; Pawelec, A.; Dobrowolski, Z.; Kohla, Z.; Stachura, J. (2007). "Immunophenotype and cytogenetics of mucinous tubular and spindle cell carcinoma of the kidney.". Pol J Pathol 58 (4): 227-33. PMID 18459456.
  18. ↑ 18.0 18.1 Kuroda, N.; Nakamura, S.; Miyazaki, E.; Hayashi, Y.; Taguchi, T.; Hiroi, M.; Yamasaki, Y.; Shuin, T. et al. (Mar 2004). "Low-grade tubular-mucinous renal neoplasm with neuroendocrine differentiation: a histological, immunohistochemical and ultrastructural study.". Pathol Int 54 (3): 201-7. doi:10.1111/j.1440-1827.2004.01608.x. PMID 14989744.
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