Germ Cell Tumors of the Female Genital Tract

Euscher, Elizabeth D.

doi:10.1016/j.path.2019.01.005

Cited by 88 publications

(104 citation statements)

References 107 publications

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“…Additional negative stains included thyroid markers (thyroglobulin, TTF-1), inhibin, α-feto protein (AFP), chromogranin, GFAP, SF-1, S-100, and OCT3/4. Yolk sac tumor was considered given glypican-3 positivity but thought to be unlikely given the minimal cytokeratin staining and negative AFP (Euscher 2019). In addition, positive glypican-3 staining can be seen in immature neural elements (Zynger et al 2008).…”

Section: Clinical Presentationmentioning

confidence: 99%

DICER1-associated metastatic abdominopelvic primitive neuroectodermal tumor with an EWSR1 rearrangement in a 16-yr-old female

Pancaldi

Peng

Rhee

et al. 2020

Cold Spring Harb Mol Case Stud

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We report a case of a DICER1-associated EWSR1-rearranged malignant primitive neuroectodermal tumor (PNET) arising in a patient with DICER1 tumor predisposition syndrome. A 16-yr-old female with a history of multinodular goiter presented with a widely metastatic abdominal small round blue cell tumor with neuroectodermal differentiation. EWSR1 gene rearrangement was identified in the tumor by fluorescence in situ hybridization (FISH). Genetic analysis revealed biallelic pathogenic DICER1 variation. The patient was treated with an aggressive course of chemotherapy, surgery, and radiation with complete pathologic response. We believe this case to represent a new expression of the DICER1 tumor predisposition syndrome, an entity caused by deleterious germline mutations in the DICER1 gene, encoding a ribonuclease active in the processing of miRNA. Patients with germline mutations in DICER1 develop a diverse group of benign and malignant tumors. Some of these tumors have been noted to have immature neuroepithelium as a component, including the ciliary body medulloepithelioma and the recently described DICER1-associated presacral malignant teratoid neoplasm. To our knowledge, abdominal sarcomas that resemble PNET histology with an EWSR1 rearrangement have not previously been described as a classical expression of the DICER1 syndrome phenotype.

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Section: Clinical Presentationmentioning

confidence: 99%

DICER1-associated metastatic abdominopelvic primitive neuroectodermal tumor with an EWSR1 rearrangement in a 16-yr-old female

Pancaldi

Peng

Rhee

et al. 2020

Cold Spring Harb Mol Case Stud

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“…Teratoma contains the different tissues from the three germ cell layers, the endoderm, mesoderm, and ectoderm [3]. The ovarian carcinoid is a rare but second most common monodermal teratoma after struma ovarii [5]. They are usually seen in perimenopausal or early postmenopausal females [5].…”

Section: Discussionmentioning

confidence: 99%

Ovarian Carcinoid Misinterpreted as Endometrioid Adenocarcinoma in Mature Cystic Teratoma

Tewari

Nigam

Kumar

et al. 2020

Cureus

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“…Moreover, two more exclusion criteria were added to exclude some possible GC patients. First, due to hematogenous spread of GC, primary lesions in the uterine cavity may spontaneously regress, but metastasis to the lungs may still occur [17][18][19]. On the other hand, since lung is not a common site of germ cell tumors, patients with solitary lesions in the lungs but no lesions in other organs were most likely to be GC metastasis patients and were excluded accordingly.…”

Section: Discussionmentioning

confidence: 99%

Clinical features of a Chinese female nongestational choriocarcinoma cohort: a retrospective study of 37 patients

Shao

Xiang

Jiang

et al. 2020

Preprint

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Background Choriocarcinoma is a rare malignant neoplasm, which is classified as either gestational choriocarcinoma or nongestational choriocarcinoma. The purpose of this study was to examine the clinical characteristics of Chinese female nongestational choriocarcinoma patients and discuss our experience in treating this rare disease.Results We conducted a single-center retrospective study on a sample of 37 nongestational choriocarcinoma patients who were diagnosed and treated at Peking Union Medical College Hospital from March 1982 to March 2020. Their demographic, clinical, laboratory, and therapeutic data were collected. Detailed information was available for all 37 individuals in our sample. The primary lesions included 34 in the ovaries, 2 in the pituitary and 1 in the stomach. The median age of onset was 22 years, and the median follow-up period spanned 41 months. The lungs (40.5%) were the most commonly observed metastatic site. All subjects were treated with surgery and multidrug chemotherapies, and a median of 4.0 courses was required to achieve complete remission. The overall complete response rate, relapse rate, and 3-year and 5-year survival rates were 81.1%, 16.7%, 80.0%, and 75.5%, respectively.Conclusions Nongestational choriocarcinoma can be managed well using surgery and multidrug chemotherapies, but the overall outcome of nongestational choriocarcinoma is still worse than that of gestational choriocarcinoma. Mixed nongestational choriocarcinoma seems to have similar therapeutic outcomes as pure tumors.

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Germ Cell Tumors of the Female Genital Tract

Cited by 88 publications

References 107 publications

DICER1-associated metastatic abdominopelvic primitive neuroectodermal tumor with an EWSR1 rearrangement in a 16-yr-old female

DICER1-associated metastatic abdominopelvic primitive neuroectodermal tumor with an EWSR1 rearrangement in a 16-yr-old female

Ovarian Carcinoid Misinterpreted as Endometrioid Adenocarcinoma in Mature Cystic Teratoma

Clinical features of a Chinese female nongestational choriocarcinoma cohort: a retrospective study of 37 patients

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