Gonadoblastoma. A gonadal tumor related to the dysgerminoma (Seminoma) and capable of sex-hormone production

Scully, Robert E.

doi:10.1002/1097-0142(195305)6:3<455::aid-cncr2820060303>3.0.co;2-u

Cited by 240 publications

(78 citation statements)

References 11 publications

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“…Nevertheless, because of the increased risk of gonadoblastoma in Turner syndrome patients with Y-chromosome material [11], the possibility of "low-level hidden" mosaicism for a Y-chromosome-positive cell line in the gonads, the variable age of expression [10], the high malignancy potential of gonado blastoma and the necessity of timely referral for gonadec tomy, analysis of SRY should be offered to all Turner syndrome patients.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with the SRY gene require special attention since the presence of a Y chromosome correlates with a 10-20% risk of developing gonadoblastoma or dys germinoma later in life [10,11]. Gonadoblastoma is an in situ malignancy of the dysgenetic gonad with variable age of appearance and considerable malignancy potential, and dysgerminomas and other malignant germ cell tumors can arise within the gonadoblastoma [10,11]. The critical region for development of gonadoblastoma has been tentatively localized to a 1-2 Mb region near the centromere of the Y chromosome [12].…”

Section: Introductionmentioning

confidence: 99%

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Analysis of the SRY Gene in Turner Syndrome Patients from the Republic of Macedonia

Papazovska-Cherepnalkovski¹,

Kočeva²,

Kočova³

2008

Balkan Journal of Medical Genetics

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Turner syndrome is characterized by short stature, gonadal dysgenesis and a variety of somatic features and major organ malformations. About 50% of the patients have a 45,X karyotype, while the remainder have structurally abnormal sex chromosomes or mosaicism including mos 45,X/46XY. Those with Y chromosomal material are at increased risk for developing gonadoblastoma or dys germinoma later in life. The SRY gene on Yp has a major role in sexual differentiation, being the primary testicular determinant. Detection of the SRY gene in Turner syndrome patients has important clinical and therapeutic implications.We performed a genetic study of 40 Turner syndrome patients for cytogenetics (G banding) and polymerase chain reaction (PCR) for the SRY gene using XES7/XES2 and SRY 1F/SRY 2R primer sets. Cytogenetics identified a 45,X karyotype in 50%, isochromosomes in 25%, proximal long or short arm deletions and markers in 7.5% and mos 45,X/46XY in 5% of the patients. The SRY gene was detected in blood leucocytes of only two of the patients with the mos 45,X/46XY karyotype. Our low incidence of SRY-gene positive results in Turner syndrome patients contrasts with reported studies using more sensitive techniques that have detected a higher percentage. The increased risk of gonadoblastoma and the necessity of timely referral for gonadectomy require that analysis of the SRY gene should be offered to all Turner syndrome patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Analysis of the SRY Gene in Turner Syndrome Patients from the Republic of Macedonia

Papazovska-Cherepnalkovski¹,

Kočeva²,

Kočova³

2008

Balkan Journal of Medical Genetics

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“…29,30 That work began a career-long interest in testicular and ovarian pathology. Other noteworthy contributions by him to this area have included, as noted earlier, the first English language paper on spermatocytic seminoma 20 and descriptions of the large cell calcifying Sertoli cell tumor, 31 juvenile granulosa cell tumor, 32 and gonadoblastoma, 28 the last heralding a lifelong interest in the pathology of intersex as well as the gonads in general. He ultimately reviewed his extended experience with gonadoblastoma many years later in one of the great descriptive clinical and pathologic studies of any area of pathology.…”

Section: The Testismentioning

confidence: 99%

“…26 The article of Dixon and Moore was preceded by an anonymous one page essay on the history of testicular tumors, 27 possibly by Dixon or Moore or perhaps the editor of Cancer at that time, Dr Fred W Stewart. The article of Dixon and Moore was followed by Dr Robert E Scully's paper on the unique gonadal lesion, the gonadoblastoma, 28 making that an illustrious issue of Cancer. The great tradition of testicular pathology at the AFIP has continued and from 1948 until his recent death it was under the leadership of Dr Fathollah K Mostofi ( Figure 5) (see below).…”

Section: The Testismentioning

confidence: 99%

“…129 His early focus from the publishing viewpoint, as previously noted, was testicular tumors and the distinctive lesion of intersex, the gonadoblastoma. 28 His attention, however, soon turned to the female gonad. A lifelong interest in functioning tumors of the ovary was stimulated by his collaboration with a gynecologist, Dr John McLean Morris, on the book 'Endocrine Pathology of the Ovary,' published in 1958.…”

Section: The Ovarymentioning

confidence: 99%

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A brief history of the pathology of the gonads

Young

2005

Mod Pathol

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Our understanding of gonadal pathology has reached its current state as a result of the contributions of numerous outstanding investigators. Knowledge of testicular tumor pathology dates back to the great British workers Percival Pott and Sir Astley Cooper but the single greatest early stride was made with the description in 1906 by the French urologist Maurice Chevassu of the seminoma. The seminal 1946 paper of Nathan B Friedman and Robert A Moore, which segregated out as a distinct entity embryonal carcinoma, is, however, the foundation for the current classification of testicular tumors. In that year Pierre Masson described the distinctive neoplasm, the spermatocytic seminoma. The 1950s saw the publication of an important paper by Frank J Dixon and Dr Moore and they also wrote the first series fascicle on testicular tumors. In this same timeframe, and thereafter, Robert E Scully made significant contributions to testicular pathology, writing the first English language paper on spermatocytic seminoma, describing several subtypes of sex cord tumor, and also the distinctive lesion of intersex, the gonadoblastoma, as well as playing a major role in 1980 in formulating the current classification of premalignant lesions of the testis. The current classification of testicular tumors was arrived at in the early 1970s when the World Health Organization, under the leadership of Dr FK Mostofi, who himself made notable contributions to testicular pathology, devised what is fundamentally the current classification of neoplasms of the male gonad. Although comments on ovarian pathology were made by such legendary figures of earlier times as Giovanni Battista Morgagni and Matthew Baillie, it is only in the mid to later years of the 19th century that contributions, mostly in Europe, began to move knowledge of ovarian pathology to its current state. Thomas Hodgkin, Richard Bright, and Sir James Paget all wrote extensively on ovarian neoplasms. In 1870, Heinrich Waldeyer, and later in that century, another German, Hermann Johannes Pfannenstiel wrote important papers on the surface epithelial tumors. The latter was likely the first to refer to neoplasms now known as of 'borderline malignancy' and also wrote on pseudomyxoma peritonei and other topics. Their work was followed by that of Robert Meyer who made monumental contributions to gynecological pathology, including recognizing the Brenner tumor as a distinctive neoplasm and proposing the first classification of Sertoli-Leydig cell tumors (arrhenoblastomas). He also coined the term 'disgerminoma' (soon changed to dysgerminoma) for the ovarian tumor that had been described in detail by the French investigator Marcel Chenot 5 years after Chevassu had mentioned the tumor in his paper describing the seminoma. During the Meyer era other significant contributions were made by, among others, Howard C Taylor writing on the borderline tumors and John A Sampson writing on endometriosis and tumors, associated with it. In the second-half of the 20th century major contributions were made b...

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An Embryologically Derived Classification of Ovarian Tumors

Bassis¹

1960

JAMA

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Gonadoblastoma. A gonadal tumor related to the dysgerminoma (Seminoma) and capable of sex-hormone production

Cited by 240 publications

References 11 publications

Analysis of the SRY Gene in Turner Syndrome Patients from the Republic of Macedonia

Analysis of the SRY Gene in Turner Syndrome Patients from the Republic of Macedonia

A brief history of the pathology of the gonads

An Embryologically Derived Classification of Ovarian Tumors

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