Sarcomatoid carcinoma presenting as cancers of unknown primary: a clinicopathological portrait

Autor: Michael J. Overman, Ryan W. Huey, Aurelio Matamoros, Kanwal Pratap Singh Raghav, Jeannelyn S. Estrella, Lianchun Xiao, Gauri R. Varadhachary, Shalini Makawita
Jazyk: angličtina
Rok vydání: 2019
Předmět:
0301 basic medicine
Oncology
Male
Cancer Research
Sarcomatoid carcinoma
Kaplan-Meier Estimate
CUP
0302 clinical medicine
Carcinosarcoma
Surgical oncology
Neoplasms
Pathology
Molecular Targeted Therapy
Prospective Studies
Lymph node
Aged
80 and over
Univariate analysis
Unknown primary
High-Throughput Nucleotide Sequencing
Middle Aged
Prognosis
lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens
Chemotherapy regimen
Combined Modality Therapy
Immunohistochemistry
Survival Rate
medicine.anatomical_structure
Treatment Outcome
Docetaxel
030220 oncology & carcinogenesis
Female
medicine.drug
Research Article
Adult
medicine.medical_specialty
lcsh:RC254-282
03 medical and health sciences
Rare Diseases
Internal medicine
Genetics
medicine
Biomarkers
Tumor
Humans
Aged
Retrospective Studies
business.industry
Molecular
medicine.disease
Gemcitabine
Log-rank test
030104 developmental biology
Drug Resistance
Neoplasm
Neoplasms
Unknown Primary
business
Follow-Up Studies
Zdroj: BMC Cancer, Vol 19, Iss 1, Pp 1-10 (2019)
BMC Cancer
ISSN: 1471-2407
Popis: Background Sarcomatoid carcinoma of unknown primary (SCUP) is a rare entity of either poorly differentiated carcinoma with sarcoma-like differentiation or a true mixed lineage neoplasm. Limited data regarding clinicopathological profile and management exists. Methods We retrospectively reviewed the MD Anderson Cancer of Unknown Primary database and tumor registry to identify 48 SCUP patients between 2001 and 2017. Patient characteristics, pathology, molecular diagnostics, treatments, and outcomes were obtained. Kaplan-Meier method was used to estimate overall survival (OS) and compared using log rank test. Results Median age at diagnosis was 59 years (range 27–86). Majority of patients were female (58%) and presented with ≥3 metastatic sites (52%), commonly lymph node (50%), bone (42%), lung (27%), and liver (21%). First line treatment included chemotherapy (35%), surgery (27%), and radiation (24%). Gemcitabine and docetaxel (18%) was the most common chemotherapy regimen. Median OS for entire cohort was 11 months (95% CI: 5.6 to 16.4). Poor performance status (PS), > 1 metastatic site, elevated lactate dehydrogenase (LDH), and high neutrophil-to-lymphocyte ratio (NLR) were significantly associated with worse OS on univariate analyses. On multivariate analyses, poor PS (HR 8.7; 95%CI: 3.0–25.0; p p = 0.011) emerged as independent prognostic factors for OS. Conclusions SCUP is a rare presentation with an aggressive clinical course and limited survival. Diagnosis is difficult to make and requires careful review and synthesis of histology, immunohistochemistry, and molecular diagnostics. Chemotherapy resistance remains a challenge. Early mutational profiling is warranted, and clinical trial participation should be encouraged for this subset.
Databáze: OpenAIRE
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