Precocious Puberty With Masculinization Due to Terato-Chorio-Gonadoblastoma

Drobnjak, P; Damjanov, Ivan; Grizelj, V.; Kalafatić, Z; Longhino, N

doi:10.1097/00006254-197206000-00026

Cited by 2 publications

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“…Sporadic patients in the literature have had one or sometimes even both endocrine findings, precocious puberty being most common. [38][39][40][41][42] This was almost invariably seen in patients whose tumor had syncytiotrophoblastic giant cells, as a component of choriocarcinoma but more often within embryonal carcinoma or dysgerminoma. 43 Tumors from both patients in our series presenting with precocious puberty had syncytiotrophoblastic giant cells, one as part of choriocarcinoma and the other as part of a nonchoriocarcinomatous component.…”

Section: Discussionmentioning

confidence: 99%

Malignant Mixed Germ Cell Tumors of the Ovary

Safdar

Stall

Young

2020

American Journal of Surgical Pathology

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One hundred malignant mixed germ cell tumors of the ovary that occurred in patients 3 to 55 years (mean: 20 y) of age are described. The clinical presentation was usually that of any highly malignant tumor of the ovary (abdominal pain and distension), but rarely (3 cases) endocrine manifestations were present. The tumors were usually unilateral (96%), ranged from 4 to 38 cm (mean: 16 cm), and were uniformly solid or, more often, solid and cystic; occasionally the typical appearance of dysgerminoma could be appreciated. The most common tumor type was yolk sac tumor (91%), followed by dysgerminoma (61%), immature teratoma (58%), embryonal carcinoma (38%), and choriocarcinoma (11%). A variety of admixtures were encountered; dysgerminoma and yolk sac tumor was the most common combination (25% of the tumors) with the 2 components often being sharply demarcated. Immature teratoma and yolk sac tumor was the next most common pairing (20%) followed by yolk sac tumor and embryonal carcinoma, with or without immature teratoma (16%). Tumors with a choriocarcinoma component had the most varied combinations of tumor types. Embryoid bodies were seen in 21% of the tumors, most often as fragmented forms arranged in a nodular manner with yolk sac tumor and/or embryonal carcinoma; uncommonly they occurred singly or in clusters. Numerous confluent well-formed embryoid bodies (polyembryoma) were prominent in 2 tumors. Three tumors had a focal diffuse embryoma pattern. The specific tumor types showed the known diverse spectrum of microscopic appearances, but the frequent haphazard arrangement of 2 or more subtypes often resulted in complex morphology. Overgrowth of another neoplastic component, most often primitive neuroectodermal tumor, occurred in 10% of the tumors further complicating the histologic picture. This is the largest series of ovarian malignant mixed germ cell tumors reported and details their characteristics including associations of their subtypes and the frequent apparent role of embryoid bodies in giving rise to yolk sac tumor and embryonal carcinoma components.

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Section: Discussionmentioning

confidence: 99%

Malignant Mixed Germ Cell Tumors of the Ovary

Safdar

Stall

Young

2020

American Journal of Surgical Pathology

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“…Alternatively, it might also be possible that the Sertoli or granulosa-like cells secreted steroid hor mones and if this is true, it would explain why the patient had not become virilized. Steroid, probably es trogen, secretion by granulosa-like cells was also noted in a case of gonadoblastoma associated with precocious puberty [6], Pure gonadoblastoma is clinically benign, probably an in situ germ cell cancer [2], However, since it is asso ciated with dysgerminoma or other malignant germ cell neoplasms in 50-60% of the cases [1,2,25] and almost entirely occurs in dysgenetic gonads and may cause vir ilization, there is clear consensus that the dysgenetic gonads should be excised as soon as the tumor is diag nosed. Unlike phenotypic females with dysgenetic go nads associated with a karyotype containing Y chromo some warranting gonadectomy, those patients with gon adoblastoma who do not possess Y chromosome, as our patient, present diagnostic difficulties.…”

Section: Discussionmentioning

confidence: 78%

“…Two patients had ovotestis [9,14] and 1 hypoplastic ovaries [12]. Gonadoblastoma was associated with ataxia-telangiectasia in 1 [8], with Fra ser's syndrome in 1 [18], and with precocious puberty in 1 patient [6], However, in the latter case the tumor was a mixture of choriocarcinoma, gonadoblastoma and tera toma. Somatic anomalies of streak gonad syndrome [19] were common in chromosomally abnormal individuals.…”

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confidence: 99%

45, X Streak Gonad Syndrome Associated with Bilateral ‘Burnt Out’ Gonadoblastoma

Bösze¹,

Magyar²,

Tóth³

et al. 1989

Gynecol Obstet Invest

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A 28-year-old woman with well-developed female secondary sexual characteristics, secondary amenorrhea, short stature and a few somatic anomalies of streak gonad syndrome who had bilateral ‘burnt out’ gonadoblastoma associated with 45, X karyotype is reported. Although the exact source of steroid production in the present case could not be defined, it seems probable that the tumor once secreted steroid hormones which were responsible for the development of the female secondary sexual characteristics. At the time of diagnosis there was no evidence of hormonal activity of the tumor. Cases of gonadoblastoma without Y chromosome are reviewed. The diagnostic difficulties encountered in patients with gonadoblastoma not associated with Y chromosome are discussed.

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Precocious Puberty With Masculinization Due to Terato-Chorio-Gonadoblastoma

Cited by 2 publications

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Malignant Mixed Germ Cell Tumors of the Ovary

Malignant Mixed Germ Cell Tumors of the Ovary

45, X Streak Gonad Syndrome Associated with Bilateral ‘Burnt Out’ Gonadoblastoma

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Precocious Puberty With Masculinization Due to Terato-Chorio-Gonadoblastoma

Cited by 2 publications

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Malignant Mixed Germ Cell Tumors of the Ovary

Malignant Mixed Germ Cell Tumors of the Ovary

45, X Streak Gonad Syndrome Associated with Bilateral &lsquo;Burnt Out&rsquo; Gonadoblastoma

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45, X Streak Gonad Syndrome Associated with Bilateral ‘Burnt Out’ Gonadoblastoma