Sertoli Cell Tumor Causing Precocious Puberty in a Girl with Peutz-Jeghers Syndrome

Zung, Amnon; Shoham, Zeev; Open, Magda; Altman, Y; Dgani, R.; Zadik, Zvi

doi:10.1006/gyno.1998.5063

Cited by 35 publications

(19 citation statements)

References 13 publications

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“…These tumours typically occur in women of reproductive age, and only about ten cases have been reported in children, with the youngest being 2 years of age; the most common clinical presentation in children is isosexual pseudoprecocity [4,7,8]. In most cases, SCT are well-differentiated and confined to the ovary, where they act in a benign fashion [4].…”

Section: Discussionmentioning

confidence: 99%

“…One striking feature of SCT is its association with PJS [4,7,8]. PJS (MIM# 175200) is an autosomal-dominant inherited syndrome characterized by hamartomatous polyposis of the gastrointestinal tract and mucocutaneous pigmentation [3].…”

Section: Abbreviationsmentioning

confidence: 99%

“…It is caused by disorders of the gonad or adrenal gland or by an autonomous secretion of gonadotrophins by a tumour. Ovarian Sertoli cell tumours (SCT) are a rare cause of GIPP in girls [4,8].…”

Section: Abbreviationsmentioning

confidence: 99%

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Germline mutation in the STK11 gene in a girl with an ovarian Sertoli cell tumour

Massa

Roggen

Renard³

2006

Eur J Pediatr

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Section: Discussionmentioning

confidence: 99%

Section: Abbreviationsmentioning

confidence: 99%

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Germline mutation in the STK11 gene in a girl with an ovarian Sertoli cell tumour

Massa

Roggen

Renard³

2006

Eur J Pediatr

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“…Patients can present with abdominal pain, swelling or menstrual irregularities, although not infrequently, they are asymptomatic. Most Sertoli cell tumors are non-functioning; however 30% of patients may exhibit hyperestrogenic manifestations, and lipid-rich variants have been reported to cause isosexual pseudoprecocity in children [238,239]. Less commonly, these tumors may cause androgenic manifestations.…”

Section: Sertoli Cell Tumor Prevalence and Clinical Presentationmentioning

confidence: 99%

Diagnostic Pathology of Ovarian Tumors

Soslow¹,

Tornos²

2011

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“…Among them, the left lesion had some features suggestive of oxyphilic CST. A SCT variant associated with PJS is the lipid-rich type (71,72) . These tumors have a malignant potential, as indicated by at least one such report (73) .…”

Section: Ovarian Tumors In Pjsmentioning

confidence: 99%

Ovarian tumors associated with multiple endocrine neoplasias and related syndromes (Carney complex, Peutz–Jeghers syndrome, von Hippel-Lindau disease, Cowden's disease)

Papageorgiou,

Stratakis

2002

Int J Gynecol Cancer

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Despite the relatively high prevalence of ovarian cancer (1% of American women will develop this disease in their lifetime) and recent developments in its molecular genetic understanding (several proto-oncogenes, such asAKT2andcKRAS, and tumor suppressor genes, such asBRCA1andBRCA2, have been implicated), little is known about the presence of ovarian tumors and cancer in women already diagnosed with other familial multiple tumor syndromes.In this review, we focus on the possible association of ovarian tumors with multiple endocrine neoplasias (MENs) and their related syndromes, such as Carney complex (CNC), Peutz–Jeghers syndrome (PJS), von Hippel-Lindau disease (VHLD), and Cowden's disease (CD). These conditions recently have been molecularly elucidated, and some of the genes responsible for them (includingSTK11/LKB1andPTEN, the genes responsible for PJS and CD, respectively) have already been investigated in series of sporadic ovarian lesions, mostly carcinomas. A brief description of each disease is followed by a literature search for affected patients with ovarian tumors; we review our own experience with CNC patients and ovarian tumors.An association between PJS and CNC and ovarian neoplasms seems likely; carcinoids of the ovary may occur in patients with MEN 1. Only few patients with CD and VHLD have any ovarian pathology, butPTEN, the CD gene has been investigated in sporadic ovarian tumors.The aim of the present report is to alert clinicians who care for patients with MENs, CNC, PJS, VHLD, CD, and other syndromes for possible associations between various types of ovarian tumors and these conditions.

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Sertoli Cell Tumor Causing Precocious Puberty in a Girl with Peutz-Jeghers Syndrome

Cited by 35 publications

References 13 publications

Germline mutation in the STK11 gene in a girl with an ovarian Sertoli cell tumour

Germline mutation in the STK11 gene in a girl with an ovarian Sertoli cell tumour

Diagnostic Pathology of Ovarian Tumors

Ovarian tumors associated with multiple endocrine neoplasias and related syndromes (Carney complex, Peutz–Jeghers syndrome, von Hippel-Lindau disease, Cowden's disease)

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