関: androblastoma、Sertoli-Leydig cell tumor

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/12/21 00:26:26」(JST)

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Sertoli–Leydig cell tumour is a group of tumours composed of variable proportions of Sertoli cells, Leydig cells, and in the case of intermediate and poorly differentiated neoplasms, primitive gonadal stroma and sometimes heterologous elements.^[1]

Sertoli–Leydig cell tumour is a member of the sex cord-stromal tumour group^[2] of ovarian and testicular cancers. The tumour is rare, comprising less than 1% of testicular tumours.^[1] While the tumour can occur at any age, it occurs most often in young adults. Recent studies have shown that many cases of Sertoli–Leydig cell tumor of the ovary are caused by germline mutations in the DICER1 gene.^[3]^[4] These hereditary cases tend to be younger, often have a multinodular thyroid goiter and there may be a personal or family history of other rare tumors such as pleuropulmonary blastoma, Wilms tumor and cervical rhabdomyosarcoma.

Closely related terms include arrhenoblastoma^[5] and androblastoma.^[6] Both terms are classified under Sertoli–Leydig cell tumour in MeSH.

Classification

The tumour is subdivided into many different subtypes. The most typical is composed of tubules lined by Sertoli cells and interstitial clusters of Leydig cells.

Presentation

Due to excess testosterone secreted by the tumour, one-third of female patients present with a recent history of progressive masculinization. Masculinization is preceded by anovulation, oligomenorrhoea, amenorrhoea and defeminization. Additional signs include acne and hirsutism, voice deepening, clitoromegaly, temporal hair recession, and an increase in musculature. Serum testosterone level is high.

Diagnosis

Presence of an ovarian tumour plus hormonal disturbances suggests a Sertoli–Leydig cell tumour. However, hormonal disturbance is present in only 2/3 of cases. A conclusive diagnosis is made via histology, as part of a pathology report made during or after surgery. See also Sex cord-stromal tumour.

Treatment

The usual treatment is surgery. The surgery usually is a fertility-sparing unilateral salpingo-oophorectomy. For malignant tumours, the surgery may be radical and usually is followed by adjuvant chemotherapy, sometimes by radiation therapy. In all cases, initial treatment is followed by surveillance. Because in many cases Sertoli–Leydig cell tumour does not produce elevated tumour markers,^[7] the focus of surveillance is on repeated physical examination and imaging. Given that many cases of Sertoli–Leydig cell tumor of the ovary are hereditary, referral to a clinical genetics service should be considered.

The prognosis is generally good as the tumour tends to grow slowly and usually is benign: 25% are malignant.^{[citation needed]} For malignant tumours with undifferentiated histology, prognosis is poor.^[7]

Additional images

High magnification micrograph of a Leydig cell tumour. H&E stain.
High magnification micrograph of a Sertoli cell tumour. H&E stain.

References

^ ^a ^b WHO, 2003^{[verification needed]}
^ Sachdeva, Poonam; Arora, Raksha; Dubey, Chandan; Sukhija, Astha; Daga, Mridula; Kumar Singh, Deepak (2008). "Sertoli–Leydig cell tumor: A rare ovarian neoplasm. Case report and review of literature". Gynecological Endocrinology 24 (4): 230–4. doi:10.1080/09513590801953465. PMID 18382911.
^ Frio, Thomas Rio; Bahubeshi, Amin; Kanellopoulou, Chryssa; Hamel, Nancy; Niedziela, Marek; Sabbaghian, Nelly; Pouchet, Carly; Gilbert, Lucy; O'Brien, Paul K. (2011). "DICER1 Mutations in Familial Multinodular Goiter With and Without Ovarian Sertoli-Leydig Cell Tumors". JAMA 305 (1): 68–77. doi:10.1001/jama.2010.1910. PMC 3406486. PMID 21205968.
^ Slade, Ingrid; Bacchelli, Chiara; Davies, Helen; Murray, Anne; Abbaszadeh, Fatemeh; Hanks, Sandra; Barfoot, Rita; Burke, Amos; Chisholm, Julia (2011). "DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome". Journal of Medical Genetics 48 (4): 273–8. doi:10.1136/jmg.2010.083790. PMID 21266384.
^ "arrhenoblastoma" at Dorland's Medical Dictionary
^ "androblastoma" at Dorland's Medical Dictionary
^ ^a ^b Lenhard, Miriam; Kuemper, Caroline; Ditsch, Nina; Diebold, Joachim; Stieber, Petra; Friese, Klaus; Burges, Alexander (2007). "Use of novel serum markers in clinical follow-up of Sertoli–Leydig cell tumours". Clinical Chemistry and Laboratory Medicine 45 (5): 657–61. doi:10.1515/CCLM.2007.120. PMID 17484630.

UpToDate Contents

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1. 卵巣の性索間質腫瘍：セルトリ・間質細胞腫瘍 sex cord stromal tumors of the ovary sertoli stromal cell tumors
2. 卵巣の性索間質腫瘍の概要 overview of sex cord stromal tumors of the ovary
3. 男性型多毛症の病因と原因 pathogenesis and causes of hirsutism
4. 妊娠時のアンドロゲン過剰症の原因 causes of gestational hyperandrogenism
5. 思春期早発症の定義、原因、および評価 definition etiology and evaluation of precocious puberty

English Journal

Judicious DICER1 testing and surveillance imaging facilitates early diagnosis and cure of pleuropulmonary blastoma.

Schultz KA1, Harris A, Williams GM, Baldinger S, Doros L, Valusek P, Frazier AL, Dehner LP, Messinger Y, Hill DA.
Pediatric blood & cancer.Pediatr Blood Cancer.2014 May 13. doi: 10.1002/pbc.25092. [Epub ahead of print]
Pleuropulmonary blastoma (PPB) and Sertoli-Leydig cell tumor (SLCT) are both associated with germline mutations in DICER1. In this brief report, a maternal history of SLCT led to identification of a deleterious DICER1 mutation in the patient and her asymptomatic infant. Radiographic screening reveal
PMID 24821309

Clinicopathologic features of renomedullary interstitial cell tumor presenting as the main solid renal mass.

Bazzi WM1, Huang H2, Al-Ahmadie H2, Russo P3.
Urology.Urology.2014 May;83(5):1104-6. doi: 10.1016/j.urology.2014.01.008. Epub 2014 Mar 15.
OBJECTIVE: To review the clinical, pathologic, and radiographic features of renomedullary interstitial cell tumor (RMICT). This is a rare benign renal tumor formerly known as medullary fibroma and is indistinguishable from other renal cortical tumors by imaging.METHODS: After institutional review bo
PMID 24642074

A novel DICER1 mutation identified in a female with ovarian Sertoli-Leydig cell tumor and multinodular goiter: a case report.

Rossing M, Gerdes AM, Juul A, Rechnitzer C, Rudnicki M, Nielsen FC, Vo Hansen T1.
Journal of medical case reports.J Med Case Rep.2014 Apr 3;8(1):112. doi: 10.1186/1752-1947-8-112.
INTRODUCTION: Germ-line mutations in the micro-ribonucleic acid processing gene DICER1 have been shown to predispose to a subset of benign tumors susceptible to malignant transformation, including ovarian Sertoli-Leydig cell tumor, nontoxic multinodular goiter, multilocular cystic nephroma and pleur
PMID 24708902

Japanese Journal

Arrhenoblastoma--とくに電顕的所見を中心としたHistogenesis

野沢志朗 [他]
臨床婦人科産科 37(2), p149-157, 1983-02
NAID 40003798030

Arrhenoblastomaの1症例--術後妊娠分娩し,10年予後の判明した症例

五十嵐俊彦 [他]
産科と婦人科 49(8), p1352-1357, 1982-08
NAID 40001458961

卵巣男化腫瘍の形態学ならびに内分泌学的研究

高倉賢二 [他],岡村均,麻生武志,竹中章,松岡賢光,神崎秀陽,鈴木瞭,西村敏雄
日本産科婦人科學會雜誌 33(7), 961-968, 1981-07-01
19歳の未婚婦に男性化をもたらした卵巣男化腫瘍につき, 形態学的・内分泌学的に検討を加えた.形態学的には, 腫瘍はMeyer分類の第2型の中間型に属し, 酵素組織化学的に腫瘍中のライディヒ細胞に3β-HSDおよびG-6-PDH活性が認められ, 電顕的にもこれらの細胞がステロイド産生細胞の形態学的特徴を備えていることが証明された.内分泌学的には, 各種ステロイドの内, testosteroneとan …
NAID 110002210718

Related Pictures

★リンクテーブル★

リンク元	「セルトリ・ライディッヒ細胞腫」「androblastoma」「男化腫瘍」「男性化細胞腫」

「セルトリ・ライディッヒ細胞腫」

Sertoli–Leydig cell tumour
	Classification and external resources
	Micrograph of a Sertoli–Leydig cell tumour. The Leydig cells have abundant eosinophilic or light pink cytoplasm. The Sertoli cells have a pale/clear cytoplasm. H&E stain.
ICD-9	183.0, 256.1
ICD-O:	8630-8631/0
MedlinePlus	001172
MeSH	D018310

　　[★]

英: Sertoli-Leydig cell tumor
同: Sertoli-Leydig細胞腫、男性化細胞腫 arrhenoblastoma
関: androblastoma、arrhenoblastoma

精巣特異的な細胞が認められる卵巣腫瘍

「androblastoma」

　　[★]

セルトリ間質細胞腫瘍、アンドロブラストーマ

関: arrhenoblastoma、Sertoli-Leydig cell tumor

「男化腫瘍」

　　[★]

英: arrhenoblastoma
関: セルトリ・ライディッヒ細胞腫、セルトリ間質細胞腫瘍

「男性化細胞腫」

　　[★]

英: arrhenoblastoma
関: セルトリ・ライディッヒ細胞腫

[WHO2003-1] WHO, 2003^{[verification needed]}

[pmid18382911-2] Sachdeva, Poonam; Arora, Raksha; Dubey, Chandan; Sukhija, Astha; Daga, Mridula; Kumar Singh, Deepak (2008). "Sertoli–Leydig cell tumor: A rare ovarian neoplasm. Case report and review of literature". Gynecological Endocrinology 24 (4): 230–4. doi:10.1080/09513590801953465. PMID 18382911.

[3] Frio, Thomas Rio; Bahubeshi, Amin; Kanellopoulou, Chryssa; Hamel, Nancy; Niedziela, Marek; Sabbaghian, Nelly; Pouchet, Carly; Gilbert, Lucy; O'Brien, Paul K. (2011). "DICER1 Mutations in Familial Multinodular Goiter With and Without Ovarian Sertoli-Leydig Cell Tumors". JAMA 305 (1): 68–77. doi:10.1001/jama.2010.1910. PMC 3406486. PMID 21205968.

[4] Slade, Ingrid; Bacchelli, Chiara; Davies, Helen; Murray, Anne; Abbaszadeh, Fatemeh; Hanks, Sandra; Barfoot, Rita; Burke, Amos; Chisholm, Julia (2011). "DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome". Journal of Medical Genetics 48 (4): 273–8. doi:10.1136/jmg.2010.083790. PMID 21266384.

[5] "arrhenoblastoma" at Dorland's Medical Dictionary

[6] "androblastoma" at Dorland's Medical Dictionary

[pmid17484630-7] Lenhard, Miriam; Kuemper, Caroline; Ditsch, Nina; Diebold, Joachim; Stieber, Petra; Friese, Klaus; Burges, Alexander (2007). "Use of novel serum markers in clinical follow-up of Sertoli–Leydig cell tumours". Clinical Chemistry and Laboratory Medicine 45 (5): 657–61. doi:10.1515/CCLM.2007.120. PMID 17484630.

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案内

案内

arrhenoblastoma