Ovary
Sex cord stromal tumors
Signet ring stromal tumor


Topic Completed: 12 September 2019

Minor changes: 11 March 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed search: Ovarian signet ring stromal tumor

Aarti Sharma, M.D.
Jennifer Bennett, M.D.
Page views in 2019: 1,553
Page views in 2020 to date: 3,899
Cite this page: Sharma A, Bennett J. Signet ring stromal tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ovarytumorsignetringstromal.html. Accessed September 20th, 2020.
Definition / general
  • Rare, benign ovarian stromal tumor with signet ring cells
Essential features
  • Primary ovarian stromal neoplasm comprised of various proportions of signet ring and spindle cells
  • Cytoplasmic vacuoles in signet ring cells are negative for lipid, mucin and glycogen
  • Diffusely positive for vimentin, variably express inhibin A, calretinin and actin - alpha smooth muscle but typically are negative for cytokeratin and EMA
Terminology
  • Signet ring stromal cell tumor (SRSCT)
  • Signet ring stromal tumor (SRST)
ICD coding
  • ICD-10: D27.9 - benign neoplasm¬†of unspecified¬†ovary
  • ICD-O: 8590 / 1, C56 - sex cord gonadal stromal tumor, NOS
Epidemiology
  • < 20 cases reported to date
  • No age predilection (range: 21 - 83 years; mean: 54 years; median: 53 years)
Sites
  • Ovary
Pathophysiology
Etiology
  • Unknown
Clinical features
  • Nonspecific including abdominopelvic pain, abnormal uterine bleeding or incidental finding on imaging
Laboratory
Radiology description
  • No defining features to distinguish from other ovarian neoplasms on imaging
  • Solid or cystic, may appear complex
Prognostic factors
  • No reports of metastasis or recurrence
Case reports
Treatment
  • Surgical excision (oophorectomy)
Gross description
  • Most are unilateral
  • Typically well circumscribed and unencapsulated
  • Yellow to tan and soft to firm cut surface
    • May show white fibromatous areas
    • Variable hemorrhage, necrosis and cystic spaces
  • Range from 1.1 to 13 cm (mean: 6.8 cm; median: 5.5 cm)
Gross images

Case #184

Yellowish with areas of hemorrhage



Images hosted on other servers:

White-gray ovarian mass

Microscopic (histologic) description
Microscopic (histologic) images

Case #184

Diffuse growth

Signet ring cells

Vimentin


AFIP images

Diffuse growth of cells with eccentric nuclei

Positive stains
Negative stains
Electron microscopy description
Molecular / cytogenetics description
Sample pathology report
  • Right ovary, oophorectomy:
    • Signet ring stromal tumor (5.0 cm)
Differential diagnosis
Board review style question #1
Which of the following is true regarding the ovarian lesion pictured below?



  1. It arises from ovarian stromal cells
  2. It has a poor prognosis
  3. It is typically bilateral
  4. Treatment involves local excision with adjuvant chemotherapy
  5. Tumor cells are strongly positive for cytokeratin
Board review answer #1
A. It arises from ovarian stromal cells (this is a signet ring stromal tumor)

Reference: Signet ring stromal tumor

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Board review style question #2
A 32 year old woman without significant past medical history presents with abdominal pain. Workup reveals a 2 cm right adnexal mass on ultrasound and she undergoes salpingo-oophorectomy. Histologic examination of the ovarian tumor reveals sheets of cells with crescent shaped nuclei and intracytoplasmic vacuoles. The vacuoles are negative for mucicarmine and PAS, while the cells are negative for CAM5.2 and inhibin A. What is the likely diagnosis?

  1. Adult granulosa cell tumor with signet ring cell change
  2. Krukenberg tumor
  3. Microcystic stromal tumor
  4. Signet ring stromal tumor
  5. Thecoma with signet ring cell transformation
Board review answer #2
D. Signet ring stromal tumor

Reference: Signet ring stromal tumor

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