Tags

Type your tag names separated by a space and hit enter

Sarcomatoid carcinoma presenting as cancers of unknown primary: a clinicopathological portrait.
BMC Cancer 2019; 19(1):965BC

Abstract

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 < 0.001) and high NLR (HR 3.4; 95%CI: 1.3-8.8; 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.

Authors+Show Affiliations

Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.Biostatistics, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.Anatomical Pathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA. kpraghav@mdanderson.org.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31623602

Citation

Huey, Ryan W., et al. "Sarcomatoid Carcinoma Presenting as Cancers of Unknown Primary: a Clinicopathological Portrait." BMC Cancer, vol. 19, no. 1, 2019, p. 965.
Huey RW, Makawita S, Xiao L, et al. Sarcomatoid carcinoma presenting as cancers of unknown primary: a clinicopathological portrait. BMC Cancer. 2019;19(1):965.
Huey, R. W., Makawita, S., Xiao, L., Matamoros, A., Estrella, J. S., Overman, M. J., ... Raghav, K. (2019). Sarcomatoid carcinoma presenting as cancers of unknown primary: a clinicopathological portrait. BMC Cancer, 19(1), p. 965. doi:10.1186/s12885-019-6155-6.
Huey RW, et al. Sarcomatoid Carcinoma Presenting as Cancers of Unknown Primary: a Clinicopathological Portrait. BMC Cancer. 2019 Oct 17;19(1):965. PubMed PMID: 31623602.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Sarcomatoid carcinoma presenting as cancers of unknown primary: a clinicopathological portrait. AU - Huey,Ryan W, AU - Makawita,Shalini, AU - Xiao,Lianchun, AU - Matamoros,Aurelio, AU - Estrella,Jeannelyn S, AU - Overman,Michael J, AU - Varadhachary,Gauri R, AU - Raghav,Kanwal, Y1 - 2019/10/17/ PY - 2019/02/27/received PY - 2019/09/12/accepted PY - 2019/10/19/entrez PY - 2019/10/19/pubmed PY - 2019/10/19/medline KW - CUP KW - Immunohistochemistry KW - Molecular KW - Neoplasms KW - Pathology KW - Prognosis KW - Sarcomatoid carcinoma KW - Unknown primary SP - 965 EP - 965 JF - BMC cancer JO - BMC Cancer VL - 19 IS - 1 N2 - 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 < 0.001) and high NLR (HR 3.4; 95%CI: 1.3-8.8; 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. SN - 1471-2407 UR - https://www.unboundmedicine.com/medline/citation/31623602/Sarcomatoid_carcinoma_presenting_as_cancers_of_unknown_primary:_a_clinicopathological_portrait L2 - https://bmccancer.biomedcentral.com/articles/10.1186/s12885-019-6155-6 DB - PRIME DP - Unbound Medicine ER -