View source for Medicine:Leydig cell tumour

{{Infobox medical condition (new)
| name            = Leydig cell tumour
| synonyms        = Testicular interstitial cell tumour
| image           = Histopathology of leydig cell tumor of the ovary, high mag, annotated.png
| caption         = Histopathology of a Leydig cell tumor, high magnification, H&E stain, showing typical features.<ref>{{cite web|url=https://www.pathologyoutlines.com/topic/testisleydig.html|title=Testis & epididymis - Sex cord-stromal tumors - Leydig cell tumor|website=PathologyOutlines|author=Zhengshan Chen, M.D., Ph.D., Manju Aron, M.D.}} Topic Completed: 4 March 2021. Minor changes: 12 April 2021.</ref>
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'''Leydig cell tumour''', also '''Leydig cell tumor''' (US spelling), '''(testicular) interstitial cell tumour''' and '''(testicular) interstitial cell tumor''' (US spelling), is a member of the sex cord-stromal tumour group<ref name="pmid18382911">{{cite journal |vauthors=Sachdeva P, Arora R, Dubey C, Sukhija A, Daga M, Singh DK |title=Sertoli-Leydig cell tumor: a rare ovarian neoplasm. Case report and review of literature |journal=Gynecol. Endocrinol. |volume=24 |issue=4 |pages=230–4 |date=April 2008 |pmid=18382911 |doi=10.1080/09513590801953465 |s2cid=42384623 }}</ref> of [[Medicine:Ovarian cancer|ovarian]] and [[Medicine:Testicular cancer|testicular cancer]]s. It arises from [[Biology:Leydig cell|Leydig cell]]s. While the tumour can occur at any age, it occurs most often in young adults.

A [[Medicine:Sertoli–Leydig cell tumour|Sertoli–Leydig cell tumour]] is a combination of a Leydig cell tumour and a [[Medicine:Sertoli cell tumour|Sertoli cell tumour]] from [[Biology:Sertoli cell|Sertoli cell]]s.

==Presentation==
The majority of Leydig cell tumors are found in males, usually at 5–10 years of age or in middle adulthood (30–60 years). Children typically present with [[Medicine:Precocious puberty|precocious puberty]].{{cn|date=May 2021}}

Due to excess [[Chemistry:Testosterone|testosterone]] secreted by the tumour, one-third of female patients present with a recent history of progressive masculinization. Masculinization is preceded by [[Medicine:Anovulation|anovulation]], oligomenorrhea, [[Medicine:Amenorrhea|amenorrhea]] and ''[[Biology:Defeminization|defeminization]]''. Additional signs include acne and hirsutism, voice deepening, clitoromegaly, temporal hair recession, and an increase in musculature. Serum testosterone level is high.{{citation needed|date=January 2017}}

In men, testicular swelling is the most common presenting feature. Other symptoms depend on age and the type of tumour. If it is secreting [[Biology:Androgen|androgen]]s the tumour is usually asymptomatic, but can cause [[Medicine:Precocious puberty|precocious puberty]] in pre-pubertal boys. If the tumour secretes [[Biology:Estrogen|oestrogens]] it can cause feminisation in young boys. In adults, this causes a number of problems including [[Medicine:Gynecomastia|gynaecomastia]], erectile dysfunction, infertility, feminine hair distribution, gonadogenital atrophy, and a loss of libido.<ref name="emedicine.medscape.com">{{Cite journal|date=2016-10-27|title=Leydig Cell Tumors: Practice Essentials, Background, Pathophysiology|url=http://emedicine.medscape.com/article/437020-overview}}</ref>

==Cause==
Animal studies a suggest possible link with C8 (C<sub>8</sub>HF<sub>15</sub>O<sub>2</sub>, [[Chemistry:Perfluorooctanoic acid|perfluorooctanoic acid]]). <ref>{{cite journal |last1=Biegel |first1=L. B. |last2=Liu |first2=R. C. |last3=Hurtt |first3=M. E. |last4=Cook |first4=J. C. |title=Effects of ammonium perfluorooctanoate on Leydig cell function: in vitro, in vivo, and ex vivo studies |issue=1 |journal=Toxicology and Applied Pharmacology |volume=134 |pages=18–25 |doi=10.1006/taap.1995.1164 |pmid=7676454 |date=1995}}</ref>

==Diagnosis==
Presence of an ovarian tumour plus hormonal disturbances suggests a Leydig cell tumour, [[Medicine:Granulosa cell tumour|granulosa cell tumour]] or thecoma.  However, hormonal disturbances, in Leydig tumours, is present in only 2/3 of cases.  Testicular Leydig cell tumours can be detected sonographically, ultrasound examinations may be ordered in the event of a palpable scrotal lump, however  incidental identification of these lesions is also possible.<ref>{{cite journal|last1=Reddan|first1=Tristan|last2=Powell|first2=Jennifer|last3=Long|first3=Gillian|title=Ultrasound of a prepubertal Leydig cell tumour of the testis|journal=Sonography|volume=4|issue=3|pages=125–128|date=2017|doi=10.1002/sono.12111|s2cid=79812660|url=https://eprints.qut.edu.au/106388/2/106388.pdf}}</ref>

[[File:Leydig cell tumour1.jpg|thumb|Low magnification [[Micrograph|micrograph]] of a '''Leydig cell tumour'''. H&E stain.]]

A conclusive diagnosis is made via [[Biology:Histology|histology]], as part of a pathology report made during or after surgery.  [[Biology:Reinke crystals|Reinke crystals]] are classically found in these tumours and help confirm the diagnosis, although they are seen in less than half of all Leydig cell tumours. Immunohistochemical markers of Leydig cell tumours include inhibin-alpha, calretinin, and melan-A.<ref name="pmid12409718">{{cite journal |vauthors=Ulbright TM, Srigley JR, Hatzianastassiou DK, Young RH |title=Leydig cell tumors of the testis with unusual features: adipose differentiation, calcification with ossification, and spindle-shaped tumor cells |journal=Am. J. Surg. Pathol. |volume=26 |issue=11 |pages=1424–33 |date=November 2002 |pmid=12409718 |doi= 10.1097/00000478-200211000-00004|s2cid=25993642 }}</ref>

==Treatment==
The usual chemotherapy regimen has limited efficacy in tumours of this type, although [[Chemistry:Imatinib|imatinib]] has shown some promise.<ref>{{cite journal | last1=Basciani | first1=Sabrina | last2=Brama | first2=Marina | last3=Mariani | first3=Stefania | last4=De Luca | first4=Gabriele | last5=Arizzi | first5=Mario | last6=Vesci | first6=Loredana | last7=Pisano | first7=Claudio | last8=Dolci | first8=Susanna | last9=Spera | first9=Giovanni | last10=Gnessi | first10=Lucio | title=Imatinib Mesylate Inhibits Leydig Cell Tumor Growth: Evidence for ''In vitro'' and ''In vivo'' Activity | journal=Cancer Research | volume=65 | issue=5 | date=2005-03-01 | doi=10.1158/0008-5472.can-04-2181 | doi-access=free | pmid=15753388 | pages=1897–1903}}</ref> There is no current role for radiotherapy.<ref>{{Cite journal|date=2016-10-27|title=Leydig Cell Tumors Treatment & Management: Medical Care, Surgical Care|url=http://emedicine.medscape.com/article/437020-treatment#d8}}</ref>

The usual treatment is surgery.  The surgery for females 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 Leydig cell tumour does not produce elevated tumour markers,<ref name="pmid17484630">{{cite journal |vauthors = Lenhard M, Kuemper C, Ditsch N, Diebold J, Stieber P, Friese K, Burges A |title    = Use of novel serum markers in clinical follow-up of Sertoli-Leydig cell tumours |journal  = Clin. Chem. Lab. Med. |volume   = 45 |issue    = 5 |pages    = 657–61 |year     = 2007 |pmid     = 17484630 |doi      = 10.1515/CCLM.2007.120 |s2cid = 12883618 |url    = http://nbn-resolving.de/urn:nbn:de:bvb:19-epub-17779-0 }}</ref> the focus of surveillance is on repeated physical examination and imaging.

In males, a radical inguinal orchiectomy is typically performed. However, testes-sparing surgery can be used to maintain fertility in children and young adults. This approach involves an inguinal or scrotal incision and ultrasound guidance if the tumour is non-palpable. This can be done because the tumour is typically unifocal, not associated with precancerous lesions, and is unlikely to recur.<ref>{{Cite journal|vauthors=Ferretti L, Sargos P, Gross-Goupil M, Izard V, Wallerand H, Huyghe E, Rigot JM, Durand X, Benoit G, Ferriere JM, Droupy S |year=2014|title=Testicular-sparing surgery for bilateral or monorchide testicular tumours: a multicenter study of long-term oncological and functional results.|url=http://reference.medscape.com/medline/abstract/24180380|journal=BJU Int|volume=114|issue=6|pages=860–4|doi=10.1111/bju.12549|pmid=24180380|s2cid=24924124|doi-access=}}</ref>

The [[Medicine:Prognosis|prognosis]] is generally good as the tumour tends to grow slowly and usually is benign: 10% are malignant.<ref name="emedicine.medscape.com"/><ref name="pmid17284120">{{cite journal |vauthors=Al-Agha OM, Axiotis CA |title=An in-depth look at Leydig cell tumor of the testis |journal=Arch. Pathol. Lab. Med. |volume=131 |issue=2 |pages=311–7 |date=February 2007 |pmid=17284120 |doi= 10.5858/2007-131-311-AILALC|url=http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=131&page=311}}</ref>  For malignant tumours with undifferentiated histology, prognosis is poor.<ref name="pmid17484630" />

==Additional images==
<gallery mode="packed" widths="360px" heights="220">
Image:Leydig cell tumour2.jpg|Intermediate magnification [[Micrograph|micrograph]] of a Leydig cell tumour. H&E stain.
Image:Leydig cell tumour3.jpg|High magnification [[Micrograph|micrograph]] of a Leydig cell tumour. H&E stain.
File:Gross pathology of a Leydig cell tumor of ovary.jpg|Typical gross pathology of a Leydig cell tumor (in this case of the ovary): A well circumscribed, solid homogeneous mass with golden brown to brownish green cut surface.<ref>{{cite web|url=https://www.pathologyoutlines.com/topic/testisleydig.html|title=Testis & epididymis - Sex cord-stromal tumors - Leydig cell tumor|website=PathologyOutlines|author=Zhengshan Chen, M.D., Ph.D., Manju Aron, M.D.}} Topic Completed: 4 March 2021. Minor changes: 12 April 2021.</ref>
</gallery>

== See also ==
* Androgen-dependent syndromes
* [[Medicine:Sertoli cell tumour|Sertoli cell tumour]]
* [[Medicine:Sertoli–Leydig cell tumour|Sertoli–Leydig cell tumour]]

==References==
{{Reflist}}
== External links ==
{{Medical resources
|  DiseasesDB     = 7445
|  ICD10          =
|  ICD9           = {{ICD9|183.0}}, {{ICD9|256.1}}
|  ICDO           = 8631
|  OMIM           =
|  MedlinePlus    = 000409
|  eMedicineSubj  =
|  eMedicineTopic =
|  MeshID         = D007984
}}

{{DEFAULTSORT:Leydig Cell Tumour}}
[[Category:Rare cancers]]

{{Sourceattribution|Leydig cell tumour}}