Ovary tumor
Other tumors not specific to ovary
Solid pseudopapillary neoplasm


Topic Completed: 14 December 2018

Revised: 14 February 2019

Copyright: (c) 2018, PathologyOutlines.com, Inc.

PubMed Search: Solid pseudopapillary neoplasm ovarian
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Cite this page: Singh K. Solid pseudopapillary neoplasm. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ovarytumorspn.html. Accessed May 21st, 2019.
Definition / general
Essential features
  • Rare tumor of reproductive age group; 10 reported cases
  • Possible origin from genital ridge related cells, presumed epithelial / neuroendocrine nature
  • Solid nests with pseudopapillary architecture, cysts with colloid-like material, papillae with myxoid stroma and tumor cell nuclei oriented away from the papillae
  • Abnormal nuclear β catenin immunohistochemical staining and E-cadherin (negative NCH-38 clone)
  • Limited cases with followup, presumed to be of low malignant potential, like pancreatic solid pseudopapillary neoplasm
Terminology
ICD coding
  • ICD-10: C56.9 - Malignant neoplasm of unspecified ovary
Epidemiology
Sites
  • Ovary
Clinical features
  • Abdominal mass, fullness, swelling or pain (6 cases)
  • Pelvic pain (2 cases)
  • Incidental radiologic finding (2 cases)
  • Postmenopausal bleeding (1 case)
Diagnosis
  • Exclude metastasis of solid pseudopapillary neoplasm from extraovarian site
Radiology description
Radiology images

Images hosted on other servers:

Solid pseudopapillary neoplasm

Prognostic factors
  • Unknown due to limited number of cases
  • Only patient who died of disease was stage IV
  • 6 patients had no evidence of disease at followup, including a stage IIIC case at 18 months followup
Case reports
Treatment
  • Surgery is mainstay of treatment
Gross description
  • Circumscribed ovarian / adnexal mass with solid and cystic hemorrhagic cut surface
Microscopic (histologic) description
  • Well circumscribed tumor
  • Tumor cell sheets, nests and bands surrounded by fibrous septa
  • Thin delicate capillary sized vessels forming myxohyaline pseudopapillary cores; nuclei located away from the papillary core
  • Moderate amount of pale eosinophilic cytoplasm with central / eccentric round to oval nuclei with uniform chromatin
  • Inconspicuous mitoses, usually no necrosis, no significant nuclear atypia
  • Abundant foamy cytoplasm, paranuclear vacuoles, nuclear grooves and intra / extracellular PAS+ diastase resistant eosinophilic globules can be present
  • Microcysts with colloid-like material
  • Infarction can be present
Microscopic (histologic) images

Contributed by Kamaljeet Singh, M.D.

Circumscribed mass

Nests with fibrous septa

Pseudopapillae

Intracytoplasmic vacuoles


β catenin

c-kit

CAM5.2

CD10

CD99


Synaptophysin

Vimentin

WT1

E-cadherin

Ki67

Positive stains
Negative stains
Electron microscopy description
  • Tumor cells with numerous pleomorphic mitochondria interspersed among short strands of rough endoplasmic reticulum (Ann Diagn Pathol 2012;16:498)
Molecular / cytogenetics description
Differential diagnosis
Board review question #1
The most characteristic immunohistochemical staining pattern of solid pseudopapillary neoplasm of the ovary is

  1. c-kit-
  2. Consistent keratin expression
  3. Consistent positive expression of both chromogranin and synaptophysin
  4. Nuclear β catenin- and E-cadherin-
  5. S100+ and HMB45+
Board review answer #1
D. Nuclear β catenin- and E-cadherin-

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Board review question #2
The image below is taken from an H&E stained section of a 3.0 cm circumscribed hemorrhagic left ovarian lesion of a 48 year old woman. Plasma inhibin levels were normal. Tumor cells expressed CAM5.2, vimentin and synaptophysin and were negative for S100, inhibin, FOXL2, E-cadherin and chromogranin. Genetic analysis showed c.98C > G point mutation in CTNNB1 exon 3. The diagnosis is



  1. Ependymoma
  2. Granulosa cell tumor
  3. Microcystic stromal tumor
  4. Solid pseudopapillary neoplasm
  5. Strumal carcinoid
Board review answer #2
D. Solid pseudopapillary neoplasm

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