性腺芽腫
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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/03/03 14:33:30」(JST)
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| Gonadoblastoma |
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Micrograph of a gonadoblastoma. H&E stain.
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| Classification and external resources |
| ICD-O: |
M9073/1 |
| DiseasesDB |
34299 |
| MeSH |
D018238 |
A gonadoblastoma is a complex neoplasm composed of a mixture of gonadal elements,[1] such as large primordial germ cells, immature Sertoli cells or granulosa cells of the sex cord, and gonadal stromal cells. Most gonadoblastomas are benign.[2]
Contents
- 1 Associations
- 2 Classification
- 3 Treatment
- 4 References
- 5 External links
Associations
Gonadoblastoma is most often associated with an abnormal chromosomal karyotype, gonadal dysgenesis, or the presence of a Y chromosome in over 90% of cases. Gonadoblastoma has been found in association with androgen insensitivity syndrome, mixed gonadal dysgenesis and Turner syndrome, especially in the presence of Y chromosome-bearing cells.[3][4]
Classification
Main article: Germ cell tumor
Gonadoblastomas can contain elements of both germ cells and gonadal stroma.[5]
Formerly, gonadoblastoma was sometimes regarded as a subset of dysgerminoma. In modern literature, it is sometimes considered to progress to dysgerminoma.[6]
Treatment
Standard treatment would include surgical exploration via laparotomy. Laparoscopy may be an option if the surgeon is particularly skilled in removing ovarian neoplasms via laparoscopy intact. If the diagnosis of gonadoblastoma is certain, a bilateral salpingo-oophorectomy (BSO) should be performed to remove both the primary tumor and the dysgenic contralateral ovary. If uninvolved, the uterus should be left intact. Modern reproductive endocrinology technology allows patients post BSO to achieve pregnancy via in-vitro fertilization (IVF) with a donor egg.
References
- ^ Cools M, Stoop H, Kersemaekers AM, et al. (June 2006). "Gonadoblastoma arising in undifferentiated gonadal tissue within dysgenetic gonads". J. Clin. Endocrinol. Metab. 91 (6): 2404–13. doi:10.1210/jc.2005-2554. PMID 16608895.
- ^ Shahrzad Ehdaivand, Nalini Gupta "Gonadoblastoma", PathologyOutlines.com, 2 July 2014
- ^ Maggio MC, Liotta A, De Grazia E, Cimador M, Di Pace R, Corsello G (2007). "Polycystic ovary and gonadoblastoma in Turner's syndrome". Minerva Pediatr. 59 (4): 397–401. PMID 17947845.
- ^ Bianco B, Lipay MV, Melaragno MI, Guedes AD, Verreschi IT; Lipay; Melaragno; Guedes; Verreschi (2006). "Detection of hidden Y mosaicism in Turner's syndrome: importance in the prevention of gonadoblastoma". J. Pediatr. Endocrinol. Metab. 19 (9): 1113–7. doi:10.1515/JPEM.2006.19.9.1113. PMID 17128558.
- ^ Kumar, Vinay; Fausto, Nelso; Abbas, Abul (2009) Robbins & Cotran Pathologic Basis of Disease (8th ed.). Saunders. Chapter 21. ISBN 1-4160-3121-9.
- ^ Cooper C, Cooper M, Carter J, Russell P (2007). "Gonadoblastoma progressing to dysgerminoma in a 55-year-old woman with normal karyotype". Pathology 39 (2): 284–5. doi:10.1080/00313020701230708. PMID 17454768.
External links
- www.health.am: Gonadoblastoma
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Germ cell tumors (ICD-O 9060–9119) (C45–C49/D17–D21, 171/214–215)
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| Germinomatous |
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| Nongerminomatous |
- Embryonal carcinoma
- Endodermal sinus tumor/Yolk sac tumor
- Teratoma: Fetus in fetu
- Dermoid cyst
- Struma ovarii
- Strumal carcinoid
- Trophoblastic neoplasm: Gestational trophoblastic disease
- Choriocarcinoma
- Placental site trophoblastic tumor
- Polyembryoma
- Gonadoblastoma
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Tumors: female urogenital neoplasia (C51–C58/D25–D28, 179–184/218–221)
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| Adnexa |
| Ovaries |
Glandular and epithelial/
surface epithelial-
stromal tumor |
| CMS: |
- Ovarian serous cystadenoma
- Mucinous cystadenoma
- Cystadenocarcinoma
- Papillary serous cystadenocarcinoma
- Krukenberg tumor
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- Endometrioid tumor
- Clear-cell ovarian carcinoma
- Brenner tumour
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| Sex cord-gonadal stromal |
- Leydig cell tumour
- Sertoli cell tumour
- Sertoli-Leydig cell tumour
- Thecoma
- Granulosa cell tumour
- Luteoma
- Sex cord tumour with annular tubules
- Steroid cell tumor (NOS)
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| Germ cell |
- Dysgerminoma
- Nongerminomatous
- Embryonal carcinoma
- Endodermal sinus tumor
- Gonadoblastoma
- Teratoma/Struma ovarii
- Choriocarcinoma
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| Fibroma |
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| Fallopian tube |
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| Uterus |
| Myometrium |
- Uterine fibroids/leiomyoma
- Leiomyosarcoma
- Adenomyoma
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| Endometrium |
- Endometrioid tumor
- Uterine papillary serous carcinoma
- Clear cell carcinoma
- Endometrial intraepithelial neoplasia
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| Cervix |
- Cervical intraepithelial neoplasia
- SCC
- Glassy cell carcinoma
- Villoglandular adenocarcinoma
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| Placenta |
- Choriocarcinoma
- Gestational trophoblastic disease
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| General |
- Uterine sarcoma
- Mixed Müllerian tumor
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| Vagina |
- SCC
- Botryoid rhabdomyosarcoma
- Clear cell adenocarcinoma of the vagina
- Vaginal intraepithelial neoplasia
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| Vulva |
- SCC
- Melanoma
- Papillary hidradenoma
- Extramammary Paget's disease
- Vulvar intraepithelial neoplasia
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Index of the female reproductive system
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| Description |
- Anatomy
- Physiology
- Development
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| Disease |
- Congenital
- Neoplasms and cancer
- Other
- Symptoms and signs
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| Treatment |
- Procedures
- Drugs
- infection
- estrogens
- progestogens
- Assisted reproduction
- Birth control
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- Tumors: male urogenital neoplasia (C60–C63/D29, 185–187/222)
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| Internal |
| Testicles |
Sex cord-
gonadal stromal |
- Sertoli-Leydig cell tumour
- Sertoli cell tumour
- Leydig cell tumour
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| Germ cell |
| G |
- Seminoma
- Spermatocytic seminoma
- Intratubular germ cell neoplasia
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| NG |
- Embryonal carcinoma
- Endodermal sinus tumor
- Gonadoblastoma
- Teratoma
- Choriocarcinoma
- Embryoma
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| Prostate |
- Adenocarcinoma
- High-grade prostatic intraepithelial neoplasia
- Small-cell carcinoma
- Transitional cell carcinoma
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| External |
| Penis |
- Carcinoma
- Extramammary Paget's disease
- Bowen's disease
- Bowenoid papulosis
- Erythroplasia of Queyrat
- Hirsuties coronae glandis
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Index of the male reproductive system
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| Description |
- Anatomy
- Physiology
- Development
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| Disease |
- Congenital
- Neoplasms and cancer
- gonadal
- Other
- Symptoms and signs
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| Treatment |
- Procedures
- Drugs
- androgens
- benign prostatic hypertrophy
- erectile dysfunction and premature ejaculation
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UpToDate Contents
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English Journal
- Gonadal malignancy in 13 consecutive collected patients with disorders of sex development (DSD) from Semarang (Indonesia).
- Juniarto AZ, Setyawati BA, Miranti IP, Santosa A, Hersmus R, Stoop H, Cools M, Oosterhuis JW, Drop SL, Faradz SM, Looijenga LH.SourceDivision of Human Genetics Center for Biomedical Research, Faculty of Medicine Diponegoro University (FMDU), Semarang, Central Java, Indonesia.
- Journal of clinical pathology.J Clin Pathol.2012 Dec 25. [Epub ahead of print]
- AIMS: Caucasian patients with disorders of sex development (DSD) are at a high risk of developing germ cell cancer (GCC). GCC is prominent in young adults in Western countries, while the incidence is significantly lower in Asia. So far, the risk of GCC in Asian DSD patients is unknown.METHODS AND RE
- PMID 23268320
- SRY mutation analysis by next generation (deep) sequencing in a cohort of chromosomal Disorders of Sex Development (DSD) patients with a mosaic karyotype.
- Hersmus R, Stoop H, Turbitt E, Oosterhuis JW, Drop SL, Sinclair AH, White SJ, Looijenga LH.SourceDepartment of Pathology, Erasmus MC, University Medical Center Rotterdam, Josephine Nefkens Institute, Daniel den Hoed Cancer Center, Rotterdam, The Netherlands. l.looijenga@erasmusmc.nl.
- BMC medical genetics.BMC Med Genet.2012 Nov 16;13:108. doi: 10.1186/1471-2350-13-108.
- ABSTRACT:BACKGROUND: The presence of the Y-chromosome or Y chromosome-derived material is seen in 4-60% of Turner syndrome patients (Chromosomal Disorders of Sex Development (DSD)). DSD patients with specific Y-chromosomal material in their karyotype, the GonadoBlastoma on the Y-chromosome (GBY) reg
- PMID 23157850
Japanese Journal
- 13.Gonadoblastomaを生じていた性分化異常の2例(一般演題,第40回日本小児外科学会北陸地方会)
- 桑原 強,安井 良僚,押切 貴博,増山 宏明,河野 美幸,伊川 廣道,伊藤 順庸,犀川 太
- 日本小児外科学会雑誌 47(1), 142, 2011-02-20
- NAID 110008506530
- 腹腔鏡下に性腺を摘出した性染色体異常 (モザイク) の2例
- 福原 健,万代 昌紀,三木 通保,藤原 浩,小西 郁生
- 日本産科婦人科内視鏡學會雜誌 = The journal of the Japan Endoscopy Society of Obstetrics and Gynecology 25(1), 272-276, 2009-09-01
- … A laparoscopic bilateral gonadectomy was performed to avoid the risk of gonadoblastoma or dysgerminoma. …
- NAID 10026403517
Related Links
- Calcification and hyalinization can ultimately lead to changes referred to as "burnt-out" gonadoblastoma in which tumor cells can be sparse or even completely absent, and the pattern of calcification or the presence of Leydig-like cells ...
- In 1953, Scully first described a unique gonadal neoplasm that strongly resembled a normally developing gonad and subsequently named the neoplasm gonadoblastoma. Gonadoblastoma is a rare benign tumor that has ...
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