Papillary cystadenoma of the epididymis

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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:

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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.