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

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Renal mucinous tubular and spindle cell carcinoma (view source)

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-'''Renal mucinous tubular and spindle cell carcinoma''', abbreviated '''RMTSCC''', is a rare malignant epithelial neoplasm of the [[kidney]].
+{{ Infobox diagnosis
+| Name       = {{PAGENAME}}
+| Image      = Renal mucinous tubular and spindle cell carcinoma - 2 -- high mag.jpg
+| Width      =
+| Caption    = Renal mucinous tubular and spindle cell carcinoma. [[H&E stain]].
+| Synonyms   =
+| Micro      = mucin (foamy macrophages) - may be scant, spindle cells, small tubules - lined by cuboidal epithelium
+| Subtypes   =
+| LMDDx      = sarcomatoid [[papillary renal cell carcinoma]], [[papillary renal cell carcinoma]], [[metanephric adenoma]]
+| Stains     =
+| IHC        = CK7 +ve, AMACR +ve, CD10 -ve, AE1/AE3 +ve
+| EM         =
+| Molecular  =
+| IF         =
+| Gross      = solid, greyish-yellow or tan-yellow, well-circumscribed
+| Grossing   = [[partial nephrectomy grossing]], [[total nephrectomy for tumour grossing]]
+| Site       = [[kidney]] - see ''[[kidney tumours]]''
+| Assdx      =
+| Syndromes  =
+| Clinicalhx =
+| Signs      =
+| Symptoms   =
+| Prevalence = rare
+| Bloodwork  =
+| Rads       = features overlap with [[papillary renal cell carcinoma]]
+| Endoscopy  =
+| Prognosis  = good
+| Other      =
+| ClinDDx    = other [[kidney tumours]]
+| Tx         = 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
@@ Line 5: / Line 36: @@
 ==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>
 *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==
+Features:<ref name=pmid24330589/>
+*Solid.
+*Greyish-yellow or tan-yellow.
+*Well-circumscribed.
+Note:
+*Imaging characteristics overlap with [[papillary renal cell carcinoma]].<ref name=pmid25395040/>
 ==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>
-*Mucin (foamy macrophages) - may be scant.
+*Mucin - may be scant.
+**Often within foamy macrophages.
 *Spindle cells - '''key feature'''.
+**Often seen only focally.
+*Small tubules - lined by cuboidal epithelium.<ref name=pmid24330589/>
 DDx:
-*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>
+*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]]).
-*[[Renal mucinous tubular and spindle cell carcinoma]].
+*[[Papillary renal cell carcinoma]].
+*[[Metanephric adenoma]].
+===Grading===
+*Typically [[ISUP nucleolar grade|ISUP nucleolar (Fuhrman) grade]] 2.<ref name=pmid>{{Cite journal  | last1 = Gross | first1 = M. | title = Vertical dimension--Part II. | journal = Refuat Hashinayim | volume = 7 | issue = 2 | pages = 8-18 | month = Apr | year = 1989 | doi =  | PMID = 2637792 }}</ref>
+**Fuhrman grade 3 tumours are described.<ref name=pmid18830937>{{Cite journal  | last1 = Kuroda | first1 = N. | last2 = Hes | first2 = O. | last3 = Michal | first3 = M. | last4 = Nemcova | first4 = J. | last5 = Gal | first5 = V. | last6 = Yamaguchi | first6 = T. | last7 = Kawada | first7 = T. | last8 = Imamura | first8 = Y. | last9 = Hayashi | first9 = Y. | title = Mucinous tubular and spindle cell carcinoma with Fuhrman nuclear grade 3: a histological, immunohistochemical, ultrastructural and FISH study. | journal = Histol Histopathol | volume = 23 | issue = 12 | pages = 1517-23 | month = Dec | year = 2008 | doi =  | PMID = 18830937 }}</ref><ref>{{Cite journal  | last1 = Kuroda | first1 = N. | last2 = Hes | first2 = O. | last3 = Michal | first3 = M. | last4 = Nemcova | first4 = J. | last5 = Gal | first5 = V. | last6 = Yamaguchi | first6 = T. | last7 = Kawada | first7 = T. | last8 = Imamura | first8 = Y. | last9 = Hayashi | first9 = Y. | title = Mucinous tubular and spindle cell carcinoma with Fuhrman nuclear grade 3: a histological, immunohistochemical, ultrastructural and FISH study. | journal = Histol Histopathol | volume = 23 | issue = 12 | pages = 1517-23 | month = Dec | year = 2008 | doi =  | PMID = 18830937 }}</ref>
 ===Images===
-www:
+====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>
+Image: Renal mucinous tubular and spindle cell carcinoma -- low mag.jpg | RMTSCC - low mag.
+Image: Renal mucinous tubular and spindle cell carcinoma -- intermed mag.jpg | RMTSCC - intermed. mag.
+Image: Renal mucinous tubular and spindle cell carcinoma -- high mag.jpg | RMTSCC - low mag.
+Image: Renal mucinous tubular and spindle cell carcinoma -- very high mag.jpg | RMTSCC - very high mag.
+</gallery>
+====www====
 *[http://moon.ouhsc.edu/kfung/jty1/OPAQ/PathQuiz-Answer/K1A001-PQ01-Ans.htm RMTSCC (ouhsc.edu)].
 *[http://www.cttr.org/large/02146b.jpg RMTSCC (cttr.org)].<ref>URL: [http://www.cttr.org/cms/?p=1406 http://www.cttr.org/cms/?p=1406]. Accessed on: 28 February 2014.</ref>
+*[http://www.diagnosticpathology.org/content/8/1/206/figure/F2 RMTSCC (diagnosticpathology.org)].<ref name=pmid24330589>{{Cite journal  | last1 = Wu | first1 = XR. | last2 = Chen | first2 = YH. | last3 = Sha | first3 = JJ. | last4 = Zhao | first4 = L. | last5 = Huang | first5 = JW. | last6 = Bo | first6 = JJ. | last7 = Liu | first7 = DM. | last8 = Huang | first8 = YR. | title = Renal mucinous tubular and spindle cell carcinoma: a report of 8 cases and review of the literature. | journal = Diagn Pathol | volume = 8 | issue = 1 | pages = 206 | month = Dec | year = 2013 | doi = 10.1186/1746-1596-8-206 | PMID = 24330589 }}</ref>
+*[http://www.ijpmonline.org/viewimage.asp?img=IndianJPatholMicrobiol_2012_55_4_439_107776_f3.jpg RMTSCC (ijpmonline.org)].<ref>{{Cite journal  | last1 = Hussain | first1 = M. | last2 = Ud Din | first2 = N. | last3 = Azam | first3 = M. | last4 = Loya | first4 = A. | title = Mucinous tubular and spindle cell carcinoma of kidney: a clinicopathologic study of six cases. | journal = Indian J Pathol Microbiol | volume = 55 | issue = 4 | pages = 439-42 | month =  | year =  | doi = 10.4103/0377-4929.107776 | PMID = 23455776 }}</ref>
+==Stains==
+*[[Mucicarmine stain|Mucicarmine]] - mucin +ve.
+*[[Alcian blue stain|Alcian blue]] pH 2.5 - mucin +ve.
 ==IHC==
 Features:<ref name=pmid16231179>{{cite journal |author=Ferlicot S, Allory Y, Compérat E, ''et al.'' |title=Mucinous tubular and spindle cell carcinoma: a report of 15 cases and a review of the literature |journal=Virchows Arch. |volume=447 |issue=6 |pages=978–83 |year=2005 |month=December |pmid=16231179 |doi=10.1007/s00428-005-0036-x |url=}}</ref>
+*CK7 +ve.
+*AMACR +ve.
 *CD10 -ve.
+**15% +ve (vs. 80% +ve in [[PaRCC]]).<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>
+**11% +ve (vs. 80% in PaRCC).<ref>{{Cite journal  | last1 = Sarsik | first1 = B. | last2 = Sımşır | first2 = A. | last3 = Karaarslan | first3 = S. | last4 = Sen | first4 = S. | title = Mucinous tubular and spindle cell carcinoma of kidney and problems in diagnosis. | journal = Turk Patoloji Derg | volume = 27 | issue = 2 | pages = 116-26 | month = May | year = 2011 | doi = 10.5146/tjpath.2011.01059 | PMID = 21630197 }}</ref>
 *AE1/AE3 +ve.
-*AMACR +ve.
-*CK7 +ve.
+Others:
+*[[PAX8]] +ve.
+*[[CAM5.2]] +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>
+*[[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:
+*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==
+<pre>
+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.
+</pre>
+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==
@@ Line 37: / Line 142: @@
 [[Category:Diagnosis]]
-[[Category:Renal tumours]]
+[[Category:Kidney tumours]]

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

Renal mucinous tubular and spindle cell carcinoma (view source)

Revision as of 20:08, 20 December 2019

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