Testis & paratestis

Germ cell tumors

Choriocarcinoma


Editorial Board Member: Bonnie Choy, M.D.
Deputy Editor-in-Chief: Maria Tretiakova, M.D., Ph.D.
Alyssa Thompson, B.S.
Debra L. Zynger, M.D.

Last author update: 12 July 2022
Last staff update: 12 July 2022

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PubMed Search: Choriocarcinoma testis

Alyssa Thompson, B.S.
Debra L. Zynger, M.D.
Page views in 2021: 9,334
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Cite this page: Thompson A, Zynger DL. Choriocarcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testischorio.html. Accessed August 13th, 2022.
Definition / general
  • Choriocarcinoma is a malignant nonseminomatous germ cell tumor (GCT) composed of trophoblastic cells with associated hemorrhage
Essential features
  • Tumor that arises from trophoblastic tissue and consists of syncytiotrophoblasts, cytotrophoblasts and intermediate trophoblasts with associated hemorrhage
  • Increased serum beta hCG
  • Early hematogenous metastasis
  • Mixed germ cell tumor with choriocarcinoma component has better outcome than pure choriocarcinoma
Terminology
  • Nonseminomatous germ cell tumor (NSGCT)
  • Trophoblastic malignant teratoma, chorionepithelioma (not recommended)
ICD coding
  • ICD-O:
    • 9100/3 - choriocarcinoma, NOS
    • 9101/3 - choriocarcinoma combined with other germ cell elements
  • ICD-10:
    • C62.90 - malignant neoplasm of unspecified testis, unspecified whether descended or undescended
  • ICD-11:
    • 2C80.2 - germ cell tumor of testis
    • XH3WM1 - choriocarcinoma combined with other germ cell elements
    • XH8PK7 - choriocarcinoma, NOS
Epidemiology
Sites
Pathophysiology
  • Unknown
Etiology
  • No specific known cause
  • Precursor lesion: germ cell neoplasia in situ (GCNIS)
  • Testicular GCT risk factors include cryptorchidism and a personal or family history of testicular cancer (Am Fam Physician 2018;97:261)
  • Testicular GCT are likely the result of genetic and environmental factors during fetal development and subsequent changes in premalignant germ cells following puberty (Expert Rev Anticancer Ther 2020;20:75)
Clinical features
  • Presentation with elevated serum beta hCG, scrotal mass with or without pain, possible hyperthyroidism and gynecomastia (Curr Oncol Rep 2015;17:2, Am Fam Physician 2018;97:261)
  • Can present with hemoptysis, seizures, confusion (Radiol Case Rep 2021;16:923, Curr Oncol Rep 2015;17:2)
  • Patients with pure or predominant choriocarcinoma have distant metastasis at presentation (100% in 1 study), usually to the lungs (67%), liver (60%) and less frequently brain (20%) (Am J Surg Pathol 2014;38:111)
  • Cases with minimal choriocarcinoma component (≤ 5%) have regional lymph node metastases in 10% and distant metastases in 7% at presentation (Am J Surg Pathol 2018;42:1113)
  • Choriocarcinoma syndrome is a rare, life threatening complication in patients with substantially increased serum beta hCG (usually > 50,000 IU/L) due to high choriocarcinoma tumor burden at metastatic sites, often following chemotherapy induction, yielding fatal bleeding and pulmonary hemorrhage leading to acute respiratory failure (Cureus 2021;13:e14286)
Diagnosis
Laboratory
Radiology description
Radiology images

Images hosted on other servers:

CT with testicular mass

Testis ultrasound with calcification

Chest Xray with nodules

Chest Xray in choriocarcinoma syndrome


Chest CT with cannonball metastasis

MRI of brain

Abdominal CT with retroperitoneal mass

Abdomen CT with liver metastasis

Prognostic factors
Case reports
Treatment
Gross description
  • Central hemorrhage and necrosis, surrounded by viable tumor
  • Tumor size has a wide range:
Gross images

Contributed by Debra L. Zynger, M.D. and Andres Matoso, M.D.
Mixed GCT with predominately choriocarcinoma Mixed GCT with predominately choriocarcinoma

Mixed GCT with predominate choriocarcinoma

Mixed GCT with minor component of choriocarcinoma Mixed GCT with minor component of choriocarcinoma Mixed GCT with minor component of choriocarcinoma

Mixed GCT with minor component of choriocarcinoma

Large hemorrhagic tumor

Frozen section description
  • Intraoperative definitive diagnosis of the type of testicular GCT is not needed because it does not impact intraoperative management
  • Determination of benign versus malignant testicular tumor for small masses via frozen section is highly accurate (Eur Urol 2003;44:458)
Microscopic (histologic) description
  • Solid nests and sheets of syncytial cells (syncytiotrophoblasts) and mononucleated trophoblasts (cytotrophoblasts and intermediate trophoblasts)
  • Syncytiotrophoblasts:
    • Large cells
    • Abundant, dense, eosinophilic cytoplasm
    • Indistinct cell border
    • Multinucleated
    • Pleomorphic, hyperchromatic nuclei which can have a degenerated, smudged appearance
    • Grow over mononucleated cells and can form a thin compressed rim or a thicker cap
  • Mononucleated trophoblasts (cytotrophoblasts and intermediate trophoblasts):
    • Medium sized cells
    • Pale, eosinophilic cytoplasm
    • Distinct cell border
    • Mitotically active
  • Hemorrhage, cyst formation and necrosis are common (Am J Surg Pathol 2014;38:111)
  • Lymphovascular invasion is often present in tumors with pure or predominant choriocarcinoma (Am J Surg Pathol 2014;38:111)
  • Germ cell neoplasia in situ may be present
  • Remaining nonneoplastic testicular parenchyma may show findings seen in other germ cell tumors, including atrophy, maturation arrest, Leydig cell hyperplasia, microlithiasis, angiopathy, Sertoli cell only tubules and Sertoli cell nodules (Am J Surg Pathol 2006;30:1260)
Microscopic (histologic) images

Contributed by Debra L. Zynger, M.D.
2 cell types 2 cell types

2 cell types

Hemorrhage

Hemorrhage

Syncytiotrophoblasts

Syncytiotrophoblasts

Syncytiotrophoblast rim Syncytiotrophoblast rim

Syncytiotrophoblast rim


Mononucleated trophoblasts Mononucleated trophoblasts Mononucleated trophoblasts

Mononucleated trophoblasts


Choriocarcinoma with IHC

Choriocarcinoma with IHC

bhCG

Beta hCG

Inhibin

Inhibin

CK7

CK7

GATA3

GATA3

OCT 3/4

OCT 3/4

Cytology description
  • Dual cell population composed of mononucleated trophoblasts and syncytiotrophoblasts (Ann Saudi Med 1997;17:432)
  • Mononucleated trophoblasts:
    • Intermediate size, round
    • Single round nuclei, coarse chromatin and visible single nucleoli
    • Clear to slightly basophilic cytoplasm with few small vacuoles (Ann Saudi Med 1997;17:432)
  • Syncytiotrophoblasts:
    • Large cells
    • Multiple, pleomorphic nuclei
    • Abundant cytoplasm with variable numbers of small vacuoles (Ann Saudi Med 1997;17:432)
  • Background of necrosis and hemorrhage which may limit cellularity of specimen (Ann Saudi Med 1997;17:432)
Positive stains
Molecular / cytogenetics description
Sample pathology report
  • Right testis, radical orchiectomy:
    • Mixed germ cell tumor, teratoma (35%), seminoma (20%), embryonal carcinoma (20%), yolk sac tumor (20%) and choriocarcinoma (5%) types (see synoptic report)
Differential diagnosis
Board review style question #1

Which testicular germ cell tumor component is classically associated with hemorrhage?

  1. Choriocarcinoma
  2. Embryonal carcinoma
  3. Seminoma
  4. Teratoma
  5. Yolk sac tumor
Board review style answer #1
A. Choriocarcinoma

Comment Here

Reference: Choriocarcinoma
Board review style question #2

Which testicular germ cell tumor component is shown in the image?

  1. Choriocarcinoma
  2. Embryonal carcinoma
  3. Seminoma
  4. Teratoma
  5. Yolk sac tumor
Board review style answer #2
A. Choriocarcinoma

Comment Here

Reference: Choriocarcinoma
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