Difference between revisions of "Papillary cystadenoma of the epididymis"

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'''Papillary cystadenoma of the epididymis''' is a rare benign epithelial tumour of the epididymis.<ref name=pmid23708309>{{Cite journal  | last1 = Toutziaris | first1 = C. | last2 = Kampantais | first2 = S. | last3 = Perdikis | first3 = I. | last4 = Gourvas | first4 = V. | last5 = Laskaridis | first5 = L. | last6 = Gkagkalidis | first6 = K. | last7 = Lakis | first7 = S. | last8 = Ioannidis | first8 = S. | title = Papillary cystadenoma of epididymis: Is there a need for further investigation in unilateral cases? | journal = Int J Surg Case Rep | volume = 4 | issue = 7 | pages = 616-8 | month =  | year = 2013 | doi = 10.1016/j.ijscr.2013.03.038 | PMID = 23708309 }}</ref>
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Papillary_cystadenoma_of_epididymis_--_high_mag.jpg
| Width      =
| Caption    = Papillary cystadenoma of epididymis. [[H&E stain]].
| Synonyms  =
| Micro      = cystic spaces with papillae lined by clear cells with moderate cytoplasm
| Subtypes  =
| LMDDx      = serous borderline tumour of the paratestis, [[adenomatoid tumour]], metastatic [[clear cell carcinoma]] (esp. [[clear cell renal cell carcinoma]])
| Stains    =
| IHC        = AE1/AE3 +ve, EMA +ve, CD10 -ve
| EM        =
| Molecular  =
| IF        =
| Gross      = solid and/or cystic mass, well-circumscribed
| Grossing  =
| Site      = [[epididymis]]
| Assdx      =
| Syndromes  = [[von Hippel-Lindau disease]]
| Clinicalhx =
| Signs      =
| Symptoms  = +/-scrotal swelling
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = benign
| Other      =
| ClinDDx    = testicular masses - esp. [[germ cell tumours]], [[paratesticular lesion]]s
| Tx        = excision
}}
'''Papillary cystadenoma of the epididymis''' is a rare benign epithelial tumour of the [[epididymis]].<ref name=pmid23708309>{{Cite journal  | last1 = Toutziaris | first1 = C. | last2 = Kampantais | first2 = S. | last3 = Perdikis | first3 = I. | last4 = Gourvas | first4 = V. | last5 = Laskaridis | first5 = L. | last6 = Gkagkalidis | first6 = K. | last7 = Lakis | first7 = S. | last8 = Ioannidis | first8 = S. | title = Papillary cystadenoma of epididymis: Is there a need for further investigation in unilateral cases? | journal = Int J Surg Case Rep | volume = 4 | issue = 7 | pages = 616-8 | month =  | year = 2013 | doi = 10.1016/j.ijscr.2013.03.038 | PMID = 23708309 }}</ref>
 
''Papillary cystadenoma'' redirects here.


==General==
==General==
*May be associated with [[von Hippel-Lindau disease]].<ref name=pmid23708309/>
*May be associated with [[von Hippel-Lindau disease]].<ref name=pmid23708309/>  
**One third of reported cases associated with vHL.<ref name=pmid20367315/>
**Classically bilateral involvement.<ref name=pmid20367315/>
*Large age range (16-76 years old).<ref name=pmid20367315/>
*Typically young adults (median age 30 years old).<ref name=pmid20367315/>
 
Clinical:<ref name=pmid20367315/>
*Slow growing scrotal mass or incidental finding.
 
==Gross==
*Well-circumscribed mass.
*Solid and/or cystic.


==Microscopic==
==Microscopic==
Features:
Features:<ref name=pmid20367315/>
*Clear cells.
*Clear cells with moderate cytoplasm and bland nuclear features (no [[nuclear atypia]]).
**Often arranged in nests.
*Cystic spaces (often microscopic and macroscopic).
*Papillary structures - may be rare.


DDx:
DDx:
Line 14: Line 61:


===Images===
===Images===
====Case====
<gallery>
Image: Papillary cystadenoma of epididymis -- very low mag.jpg | PCE - very low mag.
Image: Papillary cystadenoma of epididymis -- low mag.jpg | PCE - low mag.
Image: Papillary cystadenoma of epididymis - alt -- low mag.jpg | PCE - low mag.
Image: Papillary cystadenoma of epididymis -- intermed mag.jpg | PCE - intermed. mag.
Image: Papillary cystadenoma of epididymis -- high mag.jpg | PCE - high mag.
Image: Papillary cystadenoma of epididymis -- very low high.jpg | PCE - very high mag.
</gallery>
====www====
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3679418/figure/fig0020/ PC (nih.gov)].<ref name=pmid23708309/>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3679418/figure/fig0020/ PC (nih.gov)].<ref name=pmid23708309/>
*[http://www.archivesofpathology.org/action/showFullPopup?id=i1543-2165-134-4-630-f01&doi=10.1043%2F1543-2165-134.4.630 PC (archivesofpathology.org)].<ref name=pmid20367315>{{Cite journal  | last1 = Odrzywolski | first1 = KJ. | last2 = Mukhopadhyay | first2 = S. | title = Papillary cystadenoma of the epididymis. | journal = Arch Pathol Lab Med | volume = 134 | issue = 4 | pages = 630-3 | month = Apr | year = 2010 | doi = 10.1043/1543-2165-134.4.630 | PMID = 20367315 }}</ref>
==IHC==
Features:<ref name=pmid20367315>{{Cite journal  | last1 = Odrzywolski | first1 = KJ. | last2 = Mukhopadhyay | first2 = S. | title = Papillary cystadenoma of the epididymis. | journal = Arch Pathol Lab Med | volume = 134 | issue = 4 | pages = 630-3 | month = Apr | year = 2010 | doi = 10.1043/1543-2165-134.4.630 | PMID = 20367315 }}</ref>
*AE1/AE3 (7 of 7 cases).
*EMA +ve (9 of 9 cases).
*CK7 +v3 (3 of 3 cases).
*PAX8 +ve (6 of 7 cases<ref name=pmid24441657>{{Cite journal  | last1 = Cox | first1 = R. | last2 = Vang | first2 = R. | last3 = Epstein | first3 = JI. | title = Papillary cystadenoma of the epididymis and broad ligament: morphologic and immunohistochemical overlap with clear cell papillary renal cell carcinoma. | journal = Am J Surg Pathol | volume = 38 | issue = 5 | pages = 713-8 | month = May | year = 2014 | doi = 10.1097/PAS.0000000000000152 | PMID = 24441657 }}</ref>)
Others:<ref name=pmid20367315/>
*Vimentin +ve/-ve (3 of 6 cases +ve).
*CD10 -ve (0 +ve of 5 cases).
*CK20 -ve (0 +ve of 4 cases).
*S100 -ve (1 +ve of 4 cases).
*RCC -ve (0 +ve of 7 cases<ref name=pmid24441657/>).
*AMACR -ve (2 weakly +ve of 7 cases<ref name=pmid24441657/>).
Note:
*''Papillary cystadenoma of the epithymis'' has an immunoprofile that is nearly identical to [[clear cell papillary renal cell carcinoma]].<ref name=pmid24441657/>
==Sign out==
<pre>
Right Epididymis, Mass, Excision:
- Papillary cystadenoma of the epididymis, see comment
Comment:
The tumour stains as follows:
POSITIVE: AE1/AE3, vimentin
NEGATIVE: CD10, calretinin, CD68, S100, OCT3/4
Papillary cystadenoma of the epididymis is a benign uncommon
tumour. Approximately 60 cases are reported in the literature.
It is associated with von Hippel-Lindau disease in approximately
1/3 of cases.
</pre>


==See also==
==See also==

Latest revision as of 18:16, 31 May 2016

Papillary cystadenoma of the epididymis
Diagnosis in short

Papillary cystadenoma of epididymis. H&E stain.

LM cystic spaces with papillae lined by clear cells with moderate cytoplasm
LM DDx serous borderline tumour of the paratestis, adenomatoid tumour, metastatic clear cell carcinoma (esp. clear cell renal cell carcinoma)
IHC AE1/AE3 +ve, EMA +ve, CD10 -ve
Gross solid and/or cystic mass, well-circumscribed
Site epididymis

Syndromes von Hippel-Lindau disease

Symptoms +/-scrotal swelling
Prevalence rare
Prognosis benign
Clin. DDx testicular masses - esp. germ cell tumours, paratesticular lesions
Treatment excision

Papillary cystadenoma of the epididymis is a rare benign epithelial tumour of the epididymis.[1]

Papillary cystadenoma redirects here.

General

  • May be associated with von Hippel-Lindau disease.[1]
    • One third of reported cases associated with vHL.[2]
    • Classically bilateral involvement.[2]
  • Large age range (16-76 years old).[2]
  • Typically young adults (median age 30 years old).[2]

Clinical:[2]

  • Slow growing scrotal mass or incidental finding.

Gross

  • Well-circumscribed mass.
  • Solid and/or cystic.

Microscopic

Features:[2]

  • Clear cells with moderate cytoplasm and bland nuclear features (no nuclear atypia).
    • Often arranged in nests.
  • Cystic spaces (often microscopic and macroscopic).
  • Papillary structures - may be rare.

DDx:

Images

Case

www

IHC

Features:[2]

  • AE1/AE3 (7 of 7 cases).
  • EMA +ve (9 of 9 cases).
  • CK7 +v3 (3 of 3 cases).
  • PAX8 +ve (6 of 7 cases[3])

Others:[2]

  • Vimentin +ve/-ve (3 of 6 cases +ve).
  • CD10 -ve (0 +ve of 5 cases).
  • CK20 -ve (0 +ve of 4 cases).
  • S100 -ve (1 +ve of 4 cases).
  • RCC -ve (0 +ve of 7 cases[3]).
  • AMACR -ve (2 weakly +ve of 7 cases[3]).

Note:

Sign out

Right Epididymis, Mass, Excision:
- Papillary cystadenoma of the epididymis, see comment

Comment:
The tumour stains as follows:
POSITIVE: AE1/AE3, vimentin
NEGATIVE: CD10, calretinin, CD68, S100, OCT3/4

Papillary cystadenoma of the epididymis is a benign uncommon
tumour. Approximately 60 cases are reported in the literature.
It is associated with von Hippel-Lindau disease in approximately 
1/3 of cases. 

See also

References

  1. 1.0 1.1 1.2 Toutziaris, C.; Kampantais, S.; Perdikis, I.; Gourvas, V.; Laskaridis, L.; Gkagkalidis, K.; Lakis, S.; Ioannidis, S. (2013). "Papillary cystadenoma of epididymis: Is there a need for further investigation in unilateral cases?". Int J Surg Case Rep 4 (7): 616-8. doi:10.1016/j.ijscr.2013.03.038. PMID 23708309.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 Odrzywolski, KJ.; Mukhopadhyay, S. (Apr 2010). "Papillary cystadenoma of the epididymis.". Arch Pathol Lab Med 134 (4): 630-3. doi:10.1043/1543-2165-134.4.630. PMID 20367315.
  3. 3.0 3.1 3.2 3.3 Cox, R.; Vang, R.; Epstein, JI. (May 2014). "Papillary cystadenoma of the epididymis and broad ligament: morphologic and immunohistochemical overlap with clear cell papillary renal cell carcinoma.". Am J Surg Pathol 38 (5): 713-8. doi:10.1097/PAS.0000000000000152. PMID 24441657.