Conservative management of bilateral Sertoli cell tumors of the testicle in association with the Carney complex: a case report

Brown, Bazil; A, Ram; Clayton, Peter E.; Humphrey, G. M. E.

doi:10.1016/j.jpedsurg.2007.06.008

Cited by 27 publications

(11 citation statements)

References 12 publications

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“…Leydig cell tumors represent the most common histotype; they appear between 5 and 10 years of age or in pubertal boys and are bilateral in 10% of the cases [15,16]; hormonal activity is observed in 20% of patients and is characterized by symptoms of isosexual pseudoprecocity because of androgenic hormone production [17,18]. Sertoli cell tumors are more commonly diagnosed before 1 year of age and are considered to be hormonally silent, but occasionally, they can produce gynecomastia because of estrogenic secretion; they may occur in association with Peutz-Jeghers syndrome and Carney complex [19]. Juvenile granulosa cell tumors arise predominantly in neonates and infants [12,20,21] and may be associated with ambiguous genitalia or abnormal sex chromosomes [22].…”

Section: Discussionmentioning

confidence: 99%

Sex cord-stromal tumors of the testis in children. A clinicopathologic report from the Italian TREP project

Cecchetto¹,

Alaggio²,

Bisogno³

et al. 2010

Journal of Pediatric Surgery

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Section: Discussionmentioning

confidence: 99%

Sex cord-stromal tumors of the testis in children. A clinicopathologic report from the Italian TREP project

Cecchetto¹,

Alaggio²,

Bisogno³

et al. 2010

Journal of Pediatric Surgery

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“…Lesions may be bilateral, palpable and multifocal with some risk of malignancy and are associated with reduced fertility 22, 24, 51 . LCCSCT are detected by ultrasonography as multiple and bilateral testicular microcalcifications that distinguishes them from germ cell and other testicular tumors 2, 26 .…”

Section: Clinical Featuresmentioning

confidence: 99%

Carney complex: an update

Correa¹,

Salpea²,

Stratakis³

2015

European Journal of Endocrinology

339

348

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Carney Complex (CNC) is a rare autosomal dominant syndrome, characterized by pigmented lesions of the skin and mucosa, cardiac, cutaneous and other myxomas, and multiple endocrine tumors. The disease is caused by inactivating mutations or large deletions of the PRKAR1A gene located at 17q22–24 coding for the regulatory subunit type I alpha of protein kinase A (PKA) gene. Most recently, components of the complex have been associated with defects of other PKA subunits, such as the catalytic subunits PRKACA (adrenal hyperplasia) and PRKACB (pigmented spots, myxomas, pituitary adenomas). In this report, we review CNC, its clinical features, diagnosis, treatment, and molecular etiology including PRKAR1A mutations and the newest on PRKACA and PRKACB defects especially as they pertain to adrenal tumors and Cushing’s syndrome.

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“…Large cell calcifying Sertoli cell tumours may be bilateral and multifocal and are a complex management issue given a potential risk of malignancy and reduced fertility observed in men with CNC [1, 17]. Psammatomatous melanotic schwanomma in the gastrointestinal system and paraspinal sympathetic chain may be metastatic and have been reported in 33 patients with CNC.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous signs are important in the diagnosis of the rare neoplasia syndrome Carney complex

et al. 2009

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We describe a 15-year-old boy who presented with a stroke. Brain MRI imaging showed thalamic and multiple cerebral infarcts. An echocardiogram revealed multiple atrial masses, which were resected. Histological examination confirmed multiple atrial myxomas. Further clinical examination of the patient revealed subtle buccal and peri-oral lentigenes. The diagnosis of Carney complex was made clinically. The patient was subsequently diagnosed with testicular seminomas and a cutaneous angiomyxoma. Genetic investigation revealed a pathological mutation in the PRKAR1A gene. We review the reported manifestations and presentations of Carney complex, along with current diagnostic guidelines. We emphasise the importance of recognising the cutaneous manifestations of this rare autosomal dominantly inherited neoplasia syndrome.

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Conservative management of bilateral Sertoli cell tumors of the testicle in association with the Carney complex: a case report

Cited by 27 publications

References 12 publications

Sex cord-stromal tumors of the testis in children. A clinicopathologic report from the Italian TREP project

Sex cord-stromal tumors of the testis in children. A clinicopathologic report from the Italian TREP project

Carney complex: an update

Cutaneous signs are important in the diagnosis of the rare neoplasia syndrome Carney complex

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