Ovary

Sex cord stromal tumors

Pure stromal tumors

Luteinized thecoma associated with sclerosing peritonitis


Editorial Board Member: Lucy Ma, M.D.
Deputy Editor-in-Chief: Gulisa Turashvili, M.D., Ph.D.
Rayan Sibira, M.D.
Mahmoud A. Khalifa, M.D., Ph.D.

Last author update: 30 January 2024
Last staff update: 30 January 2024

Copyright: 2023-2024, PathologyOutlines.com, Inc.

PubMed Search: Luteinized thecoma associated with sclerosing peritonitis

Rayan Sibira, M.D.
Mahmoud A. Khalifa, M.D., Ph.D.
Cite this page: Sibira R, Khalifa M. Luteinized thecoma associated with sclerosing peritonitis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/ovaryluteinizedthecomasclerperi.html. Accessed December 25th, 2024.
Definition / general
  • Uncommon syndrome characterized by presence of ovarian (mostly bilateral) stromal tumor(s) resembling theca cells and peritoneal fibrotic lesions
Essential features
  • Benign tumor
  • Usually bilateral (unilateral cases have been reported)
  • Ovarian theca cell proliferation involving the cortex with or without mass formation
  • Associated abnormal proliferation of fibroblasts and myofibroblasts in the peritoneum that results in an irreversible fibrosis
  • Leads to incarceration and strangulation of the abdominal organs, which is eventually fatal
Terminology
  • Luteinized thecomatosis associated with sclerosing (encapsulating) peritonitis
ICD coding
  • ICD-O: 8601/0 - thecoma, luteinized
  • ICD-10
    • D27 - benign neoplasm of ovary
    • K65.8 - other peritonitis
    • K65.9 - peritonitis, nonspecific
  • ICD-11
    • 2F32.Y & XH0Z30 - other specified benign neoplasm of ovary & thecoma, luteinized
    • DC50.Z - peritonitis, unspecified
Epidemiology
Sites
Pathophysiology
  • Still unclear; debatable whether this is a neoplasm or nonneoplastic proliferation
  • Suggested that neoplastic theca cells are able to produce substances that, when released into the peritoneal cavity, stimulate fibroblastic proliferation
  • Has also been suggested that luteinized theca cells secrete steroid hormones (estrogen and progesterone) that would target submesothelial fibroblasts characterized by hormonal sensitivity
  • Among the substances produced are cytokines, in particular transforming growth factor beta (TGFβ), which induce a fibrotic reaction within the peritoneum and, rarely, even in distant anatomical sites
  • References: Expert Rev Anticancer Ther 2021;21:23
Etiology
  • Unknown
Clinical features
Diagnosis
  • Histologic examination
Laboratory
Radiology description
  • Magnetic resonance imaging features
    • Cystic, solid adnexal region mass
    • Cystic component gives a homogeneous iso or hyperintense signal
    • Solid component shows moderate enhancement
  • Computed tomography (CT) features
    • Thickened contrast material enhanced abnormal peritoneal membrane, dilation of bowel loops with clump appearance
    • Thickening or calcification of bowel walls, with or without loculated ascites
  • References: J Ovarian Res 2013;6:58, Radiographics 2019;39:62
Radiology images

Contributed by Rajesh Thampy, M.D.
Sclerosing encapsulating peritonitis, abdominopelvic CT Sclerosing encapsulating peritonitis, abdominopelvic CT

Sclerosing encapsulating peritonitis, abdominopelvic CT



Images hosted on other servers:
Luteinized thecoma

Luteinized thecoma

Prognostic factors
  • Ovarian luteinized thecoma itself is a benign tumor without risk of recurrence or metastasis
  • However, associated peritoneal fibrosis may result in bowel incarceration and strangulation, causing potentially life threatening bowel obstruction
  • References: Expert Rev Anticancer Ther 2021;21:23, Pathology 2018;50:5
Case reports
Treatment
  • Surgical management
    • Bilateral salpingo-oophorectomy if localized to ovaries
    • Oophorectomy or unilateral salpingo-oophorectomy if unilateral and preserved fertility is desired
    • Total hysterectomy with bilateral salpingo-oophorectomy is indicated in postmenopausal patients
    • Omentectomy in extensive disease
    • Peritoneal biopsy in case of peritoneal nodules only
    • Appendectomy or small bowel resection in case of involvement
  • Medical management (single or combined therapy)
    • Cyclophosphamide, thalidomide, leuprorelin, tamoxifen, goserelin and corticosteroids
  • References: Expert Rev Anticancer Ther 2021;21:23, Gynecol Oncol Rep 2019;28:44
Gross description
  • Usually bilateral
  • Dimensions range from 2 - 31 cm
  • Outer surface is brown, red, purple, pink or gray
  • Cut surface is solid, yellow, pink or gray, very often characterized by cystic hemorrhagic or sometimes soft lobulated, cerebriform or polypoid cut surface
  • Peritoneum with brown to white fibrous adhesions
  • Omentum with lobulation, nodularities, indurations or thickening
  • Thick, white adherent membrane encasing the small bowel (partially or complete)
  • Reference: Am J Surg Pathol 2008;32:1273
Frozen section description
  • Sheets of bland spindle cell proliferation circumferentially involve the entire cortex, with sparing of the medulla in smaller lesions (Am J Surg Pathol 2008;32:1273)
  • Luteinized cells are arranged in small nests or singly with small to moderate amounts of clear or eosinophilic cytoplasm (Am J Surg Pathol 2008;32:1273)
  • Mitotic activity is typically prominent (Am J Surg Pathol 2008;32:1273)
  • Edema with extravasated erythrocytes, prominent small vessels and microcystic areas
  • Peritoneal / omental lesions have proliferation of bland spindled cells with variably fibrillar cytoplasm
  • While it is unlikely peritoneal samples are submitted for frozen section examination along with the ovarian tumor, it is very helpful to inquire from the surgeon about the presence of peritoneal lesions or seek this information from the patient's chart as this helps guide diagnosis
Frozen section images

Contributed by Rayan Sibira, M.D. and Mahmoud A. Khalifa, M.D., Ph.D.
Spindle cells with entrapped luteinized cells

Spindle cells with entrapped luteinized cells

Luteinized cells and mitoses Luteinized cells and mitoses

Luteinized cells and mitoses

Peritoneal lesions

Peritoneal lesions

Microscopic (histologic) description
  • Highly cellular
  • Sheets of spindle cell proliferation circumferentially involves the entire cortex with sparing of the medulla
  • Proliferation arranged haphazardly in small fascicles or short cords and frequently includes pre-existing ovarian structures
  • Less cellular areas show edema with characteristic microcystic pattern
  • Larger lesions show prominent cellular lobules or vaguely nodular cellular aggregates, often highlighted by hemorrhage, separated by less cellular regions with varying amounts of edema which could give a microcystic appearance
  • Edematous areas contain extravasated erythrocytes and prominent small vessels
  • High power, the cells are rounded, fusiform or stellate and cytoplasm volume ranged from scant to moderate with pale or eosinophilic color
  • Ill defined aggregates of luteinized cells with rounded nuclei, eosinophilic to pale cytoplasm, in the background of bland spindle cells
  • Brisk mitotic activity may be seen
  • Sex cord-like differentiation in rare cases
  • Entrapped normal ovarian structures, usually follicles, rarely corpora albicans (Am J Surg Pathol 2008;32:1273)
  • Variable amount of hemorrhage, ischemic type necrosis and in rare cases fibrin thrombi in large vessels (Am J Surg Pathol 2008;32:1273)
  • Peritoneal lesions composed of a proliferation of fibroblastic spindled cells
  • Prominent omental lobulation in which the fibrosis expands between the septa of lobules with or without mild chronic inflammatory lymphocytic infiltrate
Microscopic (histologic) images

Contributed by Rayan Sibira, M.D. and Mahmoud A. Khalifa, M.D., Ph.D.
Spindled and luteinized cells Spindled and luteinized cells

Spindled and luteinized cells

Luteinized cells Luteinized cells

Luteinized cells

Luteinized cells with scarred mitosis

Luteinized cells with scarred mitosis

Sex cord-like differentiation

Sex cord-like differentiation


Peritoneal lesions Peritoneal lesions

Peritoneal lesions

Reticulin stain

Reticulin stain

Calretinin IHC stain

Calretinin IHC stain

Beta catenin IHC stain

Beta catenin IHC stain

Virtual slides

Images hosted on other servers:
LTSP, ovary, frozen section

LTSP, ovary, frozen section

LTSP, ovary, frozen section

LTSP, peritoneal lesions, frozen section

LTSP, ovary, frozen section

LTSP, ovary, permanent section

LTSP, ovary, frozen section

LTSP, peritoneal lesions, permanent section

Positive stains
Molecular / cytogenetics description
  • MGAT5B::NCOA3 fusion gene recently reported in luteinized thecoma associated with sclerosing peritonitis (LTSP), identified in the luteinized cells (Medicine (Baltimore) 2023;102:e33911)
  • Negative for FOXL2 mutation
Molecular / cytogenetics images

Images hosted on other servers:
MGAT5B::NCOA3 fusion gene

MGAT5B::NCOA3 fusion gene

Sample pathology report
  • Right ovary and fallopian tube, right salpingo-oophorectomy:
    • Ovarian luteinized thecoma, encased with fibrosis
    • Fibrosis involving the surface of the ovary
    • Unremarkable right fallopian tube
    • Negative for malignancy

  • Anterior pelvic peritoneum, excision:
    • Peritoneum with fibrosis and mesothelial cell hyperplasia (see comment)
    • Negative for malignancy
    • Comment: The given picture of peritoneal sclerosis (fibrosis) with hyperplasia of the submesothelial mesenchymal cells can result from a variety of stimuli. Given the finding of luteinized thecoma of the ovary, this favors the diagnosis of luteinized thecoma associated with sclerosing peritonitis (LTSP). LTSP is an uncommon syndrome characterized by the presence of a benign ovarian stromal tumor resembling theca cells and peritoneal fibrotic lesions. In some cases, peritoneal lesions lead to irreversible fibrosis with the incarceration and strangulation of abdominal organs, eventually proving fatal.
    • Other causes of peritoneal sclerosis include chronic ambulatory peritoneal dialysis, the use of peritoneovenous shunt, bacterial or mycobacterial infection, sarcoidosis, carcinoid syndrome, familial Mediterranean fever and exposure to fibrogenic foreign materials. Idiopathic sclerosing peritonitis is diagnosed when all known causes are excluded, with supportive intraoperative and imaging findings.
Differential diagnosis
Board review style question #1

A 37 year old woman presents with severe abdominal pain and distention. Laboratory investigations revealed a negative beta hCG and slightly elevated serum CA125. Radiologic images showed thick soft tissue of peritoneal sclerosis encasing the small bowel loops. Exploratory laparotomy reveals a 12.5 cm right ovarian mass and multiple peritoneal nodules, which are resected. Which of the following statements is true regarding the ovarian mass?

  1. Associated with an unfavorable outcome in the small bowel
  2. Associated with hormonal manifestations
  3. Bilateral manifestation indicates metastasis of contralateral ovarian tumor
  4. Peritoneal nodules are metastasized from the ovarian mass
Board review style answer #1
A. Associated with an unfavorable outcome in the small bowel. Axial abdominopelvic computed tomography (CT) shows peritoneal sclerosis encasing the small bowel loops with a characteristic clumped appearance of small bowel loops within a cocoon. The histopathology picture shows nodules of spindled and luteinized cells. Luteinized thecoma associated with sclerosing peritonitis (LTSP) may result in bowel incarceration and strangulation, causing potentially life threatening bowel obstruction. Answers C and D are incorrect because the ovarian tumor is benign and there is no recurrence or metastasis of the ovarian tumor after excision. Answer B is incorrect because this ovarian tumor usually is not hormonally active.

Comment Here

Reference: Luteinized thecoma associated with sclerosing peritonitis
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