Small Cell Carcinoma of the Ovary: A Rare Tumor With a Poor Prognosis

Nasioudis, Dimitrios; Chapman‐Davis, Eloise; Frey, M.K.; Caputo, Thomas A.; Holcomb, Kevin

doi:10.1097/igc.0000000000001243

Cited by 21 publications

(33 citation statements)

References 25 publications

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“…Serum CA125 was slightly elevated in 15 SSCOHT patients with available data 9. A recent study from the National Cancer Data Base (NCDB) between 2004 and 2014 showed a high frequency of elevated CA125 (271/337, 83.9%) 24. CA125 can potentially be applied as a clinical indicator for SCCOHT although we feel like that the serum level in SCCOHT does not elevate as obviously as that in ovarian epithelial carcinomas.…”

Section: An Overview Of Scchotmentioning

confidence: 81%

“…A combined investigation on 293 SCCOHT patients 10 showed that patients at 40 years or older had a worse outcome than younger patients, but there was no significant survival difference between patients with and without germline SMARCA4 mutations. The 5-year overall survival rate in the NCDB cohort was 24.1% for patients with cancer- directed surgery, and only 18% and 12.3% in patients with stage III and IV respectively 24. Intriguingly, Ghazi A, et al 25 reported a SCCOHT patient that developed after two months of ovarian stimulation for in vitro fertilization and died one month after her initial symptoms.…”

Section: Clinical Behavior and Treatment Of Sccohtmentioning

confidence: 99%

“…Recent data have indicated that a multi-modality treatment approach consisting of surgery, high dose multi-agent chemotherapy with possible stem cell transplantation and radiotherapy is an attractive treatment option for the SCCOHT patients 9-11, 24, 31, 32. Qin Q, et al 32 reported that a young girl with stage IIIc SCCOHT had an 8-year disease free survival after a combined treatment of cytoreductive surgery, high-dose consolidative chemotherapy, autologous hematopoietic stem cell transplantation and pelvic radiotherapy.…”

Section: Clinical Behavior and Treatment Of Sccohtmentioning

confidence: 99%

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An In-Depth Look at Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT): Clinical Implications from Recent Molecular Findings

Lü¹,

Shi²

2019

J. Cancer

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Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a highly aggressive cancer in young women. The histogenesis remains unclear although a potential origin of germ cells has been suggested recently. The high throughput next generation sequencing techniques have facilitated the identification of inactivating SMARCA4 mutations as the driver of SCCOHT. These findings may greatly impact on the prevention, diagnosis, molecular classification and treatment of SCCOHTs. The SMARCA4 mutations, typically associated with dual loss of BRG1 and BRM expression, are highly sensitive and specific for the diagnosis of SCCOHT. Germline mutations of SMARCA4 support familial SCCOHT with a critical requirement of genetic counseling and possible prophylactic surgery for carriers. SCCOHT, malignant atypical teratoid/rhabdoid tumors, thoracic sarcomas and some undifferentiated carcinomas harbor rhabdoid morphology and mutations in the SMARC genes, generating an emerging molecular classification of SMARC-mutated tumors. A multi-modality treatment approach consisting of surgery and high dose multi-agent chemotherapy in atypical teratoid/rhabdoid tumors may have potential benefits for SCCOHT patients. Preliminary studies have implicated that the inhibitors targeting EZH2 and the receptor tyrosine kinase, and anti-PD-L1 immunotherapy might be potentially effective for SCCOHT patients. These recent advances on molecular genetics, diagnosis and treatment of SCCOHT address the necessity of multiple institutional collaboration work among oncologist, pathologist, genomic scientist, geneticist, molecular biologist, and pharmacologist.

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Section: An Overview Of Scchotmentioning

confidence: 81%

Section: Clinical Behavior and Treatment Of Sccohtmentioning

confidence: 99%

Section: Clinical Behavior and Treatment Of Sccohtmentioning

confidence: 99%

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An In-Depth Look at Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT): Clinical Implications from Recent Molecular Findings

Lü¹,

Shi²

2019

J. Cancer

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“…6 Approximately one half of patients are diagnosed with advanced-stage disease. 3,[7][8][9] Prognosis is poor; one study reported a mean overall survival (OS) declining from 35 months for stage I disease to 3.3 months for stage IV disease, with 75% of all patients experiencing disease relapse. 4 SCCOHT is associated with germline and somatic SMARCA4 mutations, with biallelic inactivation occurring in 25% to 100% of patients in small case series.…”

Section: Sccoht: Pathogenesis and Historical Treatmentmentioning

confidence: 99%

“…Multiagent chemotherapy regimens vary widely, generally including a platinum agent with taxanes, etoposide, vinblastine, cyclophosphamide, doxorubicin, or bleomycin; external beam radiation may be useful in certain circumstances. [4][5][6]8,[16][17][18] Highdose chemotherapy with autologous stem cell rescue may improve OS. In a prospective SCCOHT trial of 10 patients treated with the PAVEP regimen (ie, cisplatin, doxorubicin, etoposide, and cyclophosphamide), followed by consolidative chemotherapy with autoSCT, seven were long-term survivors.…”

Section: Sccoht: Pathogenesis and Historical Treatmentmentioning

confidence: 99%

Combined CDK4/6 and PD-1 Inhibition in Refractory SMARCA4-Deficient Small-Cell Carcinoma of the Ovary, Hypercalcemic Type

Lee

Esselen

Kolin

et al. 2020

JCO Precision Oncology

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The patient provided written informed consent allowing discussion of her case and inclusion of pertinent images. AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The following represents disclosure information provided by authors of this manuscript. All relationships are considered compensated unless otherwise noted. Relationships are self-held unless noted. I = Immediate Family Member, Inst = My Institution. Relationships may not relate to the subject matter of this manuscript. For more information about ASCO's conflict of interest policy, please refer to www.asco.org/rwc or ascopubs.org/po/author-center. Open Payments is a public database containing information reported by companies about payments made to US-licensed physicians (Open Payments). Larissa J. Lee Research Funding: AstraZeneca (Inst) Patents, Royalties, Other Intellectual Property: Methods and Systems for Quantitative Monitoring of In Vivo Tumor Oxygenation; shared patent filing and intellectual property held by Brigham and Women'

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Primary neuroendocrine tumors of the ovary: Management and outcomes

Pang

2021

Cancer Medicine

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Background There is currently no recognized first‐line treatment strategy for ovarian neuroendocrine tumors (NETs). Furthermore, because of the low incidence of ovarian NETs, no studies have reported prognostic statistics derived from large samples. This retrospective study aimed to investigate the clinical behavior of ovarian NETs. Methods The Surveillance, Epidemiology, and End Results database was used to identify women diagnosed with ovarian NETs from 2004 to 2015. Overall survival (OS), cancer‐specific survival (CSS), and independent prognostic factors for ovarian NETs were evaluated. The effects of different treatments on prognosis were also compared, as were OS and CSS rates for histological subtypes. Results The 5‐year OS rates were 83.3%, 30.0%, 20.3%, and 9.8% for patients in stages I (n = 159), II (n = 23), III (n = 101), and IV (n = 148), respectively. The 5‐year CSS rates were 85.6%, 41.7%, 21.2%, and 9.8% for patients in stages I–IV, respectively. Age, American Joint Committee on Cancer (AJCC) stage, lymph node metastasis, treatment, and histological type were related to poor OS and CSS. In the early stage, the 5‐year OS and CSS rates were 97.03% and 96.90%, respectively. For patients in the advanced stage receiving comprehensive treatment (surgery + chemotherapy + radiotherapy), the 5‐year OS and CSS rates were 72.9% and 70.00%, respectively. When comparing low‐ and high‐grade neuroendocrine carcinoma, 5‐year OS rates were 93.96% and 7.01%, 5‐year CSS rates were 97.44% and 7.31%, 10‐year OS rates were 93.56% and 2.34%, and 10‐year CSS rates were 97.44% and 4.88%, respectively. Conclusion Age, AJCC stage, treatment, and histological type are independent prognostic factors of ovarian NETs. OS and CSS are relatively good for early‐stage cases treated with surgery alone, whereas more comprehensive treatment is required to improve OS and CSS in the advanced stage.

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Small Cell Carcinoma of the Ovary: A Rare Tumor With a Poor Prognosis

Abstract: Small cell carcinomas of the ovary are unilateral tumors primarily arising in premenopausal women. Multimodal treatment with cancer-directed surgery and CT results in a modest increase of a generally poor survival.

Cited by 21 publications

References 25 publications

An In-Depth Look at Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT): Clinical Implications from Recent Molecular Findings

An In-Depth Look at Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT): Clinical Implications from Recent Molecular Findings

Combined CDK4/6 and PD-1 Inhibition in Refractory SMARCA4-Deficient Small-Cell Carcinoma of the Ovary, Hypercalcemic Type

Primary neuroendocrine tumors of the ovary: Management and outcomes

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