Endodermal sinus tumor

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Endodermal sinus tumor
Other names Yolk sac tumor (YST)
Mixed germ cell tumour - high mag.jpg
Micrograph showing the yolk sac component of a mixed germ cell tumour. H&E stain.
Specialty Oncology   OOjs UI icon edit-ltr-progressive.svg

Endodermal sinus tumor (EST) is a member of the germ cell tumor group of cancers. [1] It is the most common testicular tumor in children under three, [2] and is also known as infantile embryonal carcinoma. This age group has a very good prognosis. In contrast to the pure form typical of infants, adult endodermal sinus tumors are often found in combination with other kinds of germ cell tumor, particularly teratoma and embryonal carcinoma. While pure teratoma is usually benign, endodermal sinus tumor is malignant.

Contents

Cause

Causes for this cancer are poorly understood.[ citation needed ]

Diagnosis

The histology of EST is variable, but usually includes malignant endodermal cells. These cells secrete alpha-fetoprotein (AFP), which can be detected in tumor tissue, serum, cerebrospinal fluid, urine and, in the rare case of fetal EST, in amniotic fluid. When there is incongruence between biopsy and AFP test results for EST, the result indicating presence of EST dictates treatment. [3] This is because EST often occurs as small "malignant foci" within a larger tumor, usually teratoma, and biopsy is a sampling method; biopsy of the tumor may reveal only teratoma, whereas elevated AFP reveals that EST is also present. GATA-4, a transcription factor, also may be useful in the diagnosis of EST. [4]

Diagnosis of EST in pregnant women and in infants is complicated by the extremely high levels of AFP in those two groups. Tumor surveillance by monitoring AFP requires accurate correction for gestational age in pregnant women, and age in infants. In pregnant women, this can be achieved simply by testing maternal serum AFP rather than tumor marker AFP. In infants, the tumor marker test is used, but must be interpreted using a reference table or graph of normal AFP in infants.[ medical citation needed ]

Pathology

EST can have a multitude of morphologic patterns including: reticular, endodermal sinus-like, microcystic, papillary, solid, glandular, alveolar, polyvesicular vitelline, enteric and hepatoid.[ medical citation needed ]

Schiller–Duval bodies on histology are pathognomonic and seen in the context of the endodermal sinus-like pattern. Rarely, it can be found in the vagina. [6] [7]

Treatment

Most treatments involve some combination of surgery and chemotherapy. Treatment with cisplatin, etoposide, and bleomycin has been described. [8] Before modern chemotherapy, this type of neoplasm was highly lethal, but the prognosis has significantly improved since then.[ citation needed ] When endodermal sinus tumors are treated promptly with surgery and chemotherapy, fatal outcomes are exceedingly rare. [9]

See also

Related Research Articles

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<span class="mw-page-title-main">Testicular cancer</span> Medical condition

Testicular cancer is cancer that develops in the testicles, a part of the male reproductive system. Symptoms may include a lump in the testicle or swelling or pain in the scrotum. Treatment may result in infertility.

<span class="mw-page-title-main">Ovarian cancer</span> Cancer originating in or on the ovary

Ovarian cancer is a cancerous tumor of an ovary. It may originate from the ovary itself or more commonly from communicating nearby structures such as fallopian tubes or the inner lining of the abdomen. The ovary is made up of three different cell types including epithelial cells, germ cells, and stromal cells. When these cells become abnormal, they have the ability to divide and form tumors. These cells can also invade or spread to other parts of the body. When this process begins, there may be no or only vague symptoms. Symptoms become more noticeable as the cancer progresses. These symptoms may include bloating, vaginal bleeding, pelvic pain, abdominal swelling, constipation, and loss of appetite, among others. Common areas to which the cancer may spread include the lining of the abdomen, lymph nodes, lungs, and liver.

<span class="mw-page-title-main">Sex cord–gonadal stromal tumour</span> Medical condition

Sex cord–gonadal stromal tumour is a group of tumours derived from the stromal component of the ovary and testis, which comprises the granulosa, thecal cells and fibrocytes. In contrast, the epithelial cells originate from the outer epithelial lining surrounding the gonad while the germ cell tumors arise from the precursor cells of the gametes, hence the name germ cell. In humans, this group accounts for 8% of ovarian cancers and under 5% of testicular cancers. Their diagnosis is histological: only a biopsy of the tumour can make an exact diagnosis. They are often suspected of being malignant prior to operation, being solid ovarian tumours that tend to occur most commonly in post menopausal women.

<span class="mw-page-title-main">Germ cell tumor</span> Medical condition

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<span class="mw-page-title-main">Polyembryoma</span> Medical condition

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<span class="mw-page-title-main">Immature teratoma</span> Medical condition

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<span class="mw-page-title-main">Schiller–Duval body</span> Pathognomic of Yolk sac tumor ovary

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<span class="mw-page-title-main">Scrotal ultrasound</span> Medical ultrasound examination of the scrotum.

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<span class="mw-page-title-main">Extracranial germ cell tumor</span> Type of tumor

An extracranial germ cell tumor (EGCT) occurs in the abnormal growth of germ cells in the gonads and the areas other than the brain via tissue, lymphatic system, or circulatory system. The tumor can be benign or malignant (cancerous) by its growth rate. According to the National Cancer Institute and St. Jude Children's Research Hospital, the chance of children who are under 15 years old having EGCTs is 3%, in comparison to adolescents, a possibility of 14% with aged 15 to 19 can have EGCTs. There is no obvious cut point in between children and adolescents. However, common cut points in researches are 11 years old and 15 years old.

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References

  1. "Endodermal Sinus Tumor" . Retrieved 2018-10-10.
  2. Hari, Anil; Grossfeld, Gary; Hricak, Hedvig (2002-01-01), Bragg, David G.; Rubin, Philip; Hricak, Hedvig (eds.), "Chapter 29 - Tumors of the Scrotum", Oncologic Imaging, Oxford: Elsevier, pp. 603–628, doi:10.1016/b0-72-167494-1/50032-3, ISBN   978-0-7216-7494-0 , retrieved 2020-10-21
  3. Luther N, Edgar MA, Dunkel IJ, Souweidane MM (Aug 2006). "Correlation of endoscopic biopsy with tumor marker status in primary intracranial germ cell tumors". Journal of Neuro-Oncology. 79 (1): 45–50. doi:10.1007/s11060-005-9110-0. PMID   16598424. S2CID   19124218.
  4. Siltanen S, Anttonen M, Heikkilä P, Narita N, Laitinen M, Ritvos O, Wilson DB, Heikinheimo M (Dec 1999). "Transcription Factor GATA-4 Is Expressed in Pediatric Yolk Sac Tumors". American Journal of Pathology. 155 (6): 1823–9. doi:10.1016/S0002-9440(10)65500-9. PMC   1866939 . PMID   10595911. Archived from the original on 2009-03-16.
  5. Fischerova D, Indrielle-Kelly T, Burgetova A, Bennett RJ, Gregova M, Dundr P; et al. (2022). "Yolk Sac Tumor of the Omentum: A Case Report and Literature Review". Diagnostics. 12 (2): 304. doi: 10.3390/diagnostics12020304 . PMC   8871053 . PMID   35204394.{{cite journal}}: CS1 maint: multiple names: authors list (link)
    - "This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/)."
  6. Bhatt MD, Braga LH, Stein N, Terry J, Portwine C (July 2015). "Vaginal Yolk Sac Tumor in an Infant: A Case Report and Literature Review of the Last 30 Years". Journal of Pediatric Hematology/Oncology. 37 (5): e336–40. doi:10.1097/MPH.0000000000000325. PMID   25851552. S2CID   7605939.
  7. Coran, Arnold G.; Caldamone, Anthony; Adzick, N. Scott; Krummel, Thomas M.; Laberge, Jean-Martin; Shamberger, Robert (2012-01-25). Pediatric Surgery E-Book. Elsevier Health Sciences. ISBN   978-0323091619.
  8. Motegi M, Takakura S, Takano H, Tanaka T, Ochiai K (February 2007). "Adjuvant chemotherapy in a pregnant woman with endodermal sinus tumor of the ovary". Obstetrics and Gynecology. 109 (2 Pt2): 537–40. doi:10.1097/01.AOG.0000245450.62758.47. PMID   17267887. S2CID   24159507.
  9. Prepubertal Testicular and Paratesticular Tumors at eMedicine