Testis & paratestis
Epididymal tumors
Papillary cystadenoma
Author: Swapnil U. Rane, M.D.
Last author update: 1 December 2014
Last staff update: 20 October 2022
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed Search: Papillary cystadenoma epididymis
Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Clinical features | Laboratory | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Differential diagnosisCite this page: Rane S. Papillary cystadenoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testisepidpapcyst.html. Accessed December 20th, 2024.
Definition / general
- Benign tumor of epididymis characterized by prominent papillary architecture
- Often associated with von Hippel-Lindau disease (Hum Pathol 1995;26:1341)
- First described by Sherrick (Cancer 1956;9:403)
Terminology
- Also called papillary cystadenofibroma
Epidemiology
- Second most common benign neoplasm of epididymis after adenomatoid tumor
- Sporadic or familial
- Unilateral or bilateral (40%)
- Mean age 36 years
- Associated with von Hippel-Lindau (VHL) disease; 65% with bilateral tumors have VHL vs. 18 - 20% with unilateral lesions (Arch Pathol Lab Med 2010;134:630)
Sites
- In male GU, occurs in epididymis (develops within efferent ductules of head of epididymis)
- In female GU, occurs in broad ligament, peritoneum; also fallopian tube and uterus
Clinical features
- Benign, usually asymptomatic
- If symptomatic, typically is slow growing swelling in scrotum, with only rare pain or tenderness
- Rarely discovered during infertility workup (Arch Pathol Lab Med 2010;134:630)
Laboratory
- No specific laboratory features
- Negative for germ cell tumor markers
Case reports
- 46 year old man (Int J Clin Exp Pathol 2011;4:629)
- Vascular endothelial growth factor in von Hippel-Lindau syndrome - associated papillary cystadenoma of epididymis (Hum Pathol 1998;29:1322)
- 3 cases (Arch Pathol Lab Med 1990;114:672)
- Solid variant (Histopathology 2015;67:138)
Treatment
- Surgical excision; does not recur
- If bilateral, patient should be investigated for VHL syndrome
Clinical images
Images hosted on other servers:
Complex echo pattern
Gross description
- Well circumscribed, 1 - 5 cm, gray, brown, yellow tumor nodule
- Cut surface may show papillary fronds or may be cystic with areas of hemorrhage
Gross images
Images hosted on other servers:
Epididymal tumor
Microcystic with hemorrhage
Microscopic (histologic) description
- Papillary in foldings project into cystic spaces covered by single to double layered cuboidal / columnar cells
- Tubules common, colloid type cystic material common
- Tumor cells have lightly eosinophilic to clear cytoplasm
- May see dilated efferent ductules
- May have clear cells resembling metastastic renal cell carcinoma but no mitoses, no necrosis, no pleomorphism
- Papillary cystadenofibroma: prominent stroma
Microscopic (histologic) images
Images hosted on other servers:
Cystic space with papillary excrescences
Cilia
Case with clear cells
CK7
CD10
Cytology description
- Simple and complex papillary clusters
- Tumor cells are monomorphic, with moderate to abundant cytoplasm, well defined cytoplasmic borders; round to oval nuclei, finely dispersed chromatin, inconspicuous nucleoli
- Vacuolations may be present
- No features of malignancy - i.e. no mitoses, no necrosis, no atypia
- Usually no psammoma bodies, but see Cytopathology 2014;25:279, Acta Cytol 2004;48:467
Positive stains
- AE1 / AE3, CK7, alpha-1-antitrypsin, alpha-1-antichymotrypsin, CAM5.2, EMA, vimentin, vascular endothelial growth factor
- Lectin
- PAX8, carbonic anhydrase IX
- Variable CEA (Am J Surg Pathol 2014;38:713)
Negative stains
- Useful negative stains include AMACR and RCC antigen, to differentiate from clear cell papillary renal cell carcinoma
- CD10 is usually negative but focal staining may be noted (Am J Surg Pathol 2005;29:520)
Differential diagnosis
- Metastatic renal cell carcinoma
- Other features of malignancy
- CD10, RCC antigen are strongly positive
- Lectin and CK7 are negative
- Serous borderline tumors and serous adenocarcinoma of paratestis: atypia, mitotic activity, stratification and detached cell clusters
