Abdominal desmoid- course, unique genetic background, and severe outcomes in a large local series.

e23544 Background: Abdominal desmoid tumors are locally aggressive, non-metastatic tumors that develop mainly in Familial adenomatous polyposis (FAP) patients, within the mesentery or abdominal wall. Understanding and implications of treatment regimens are evolving. We aimed to assess course, treatment and outcomes of FAP and non-FAP abdominal desmoids and their related genetic alterations. Methods: Retrospective cohort study. Demographics, tumor characteristics, oncological and surgical history, complications, genetic-testing and mortality data were retrieved from two tertiary referral centers. Results: Sixty-two patients were identified (46 FAP, 16 non-FAP) with a median follow up of 72.4 months. Thirty-eight patients (61.3%) underwent surgical procedures: twelve urgent and 26 elective. Out of 33 tumor resections, 39.4% recurred. Hormonal therapy, COX-inhibitors, chemotherapy, imatinib and sorafenib were used in 35(56.4%), 30(48.4%), 18(29.1%), 7(11.3%) and 8(12.9%) of patients, respectively, with 2 years progression-free survival of 67.8%, 57.7%, 38.4%, 28.5%, respectively. Only 1/9 patients treated with sorafenib had disease progression after a median follow up of 6.8 months. Forty-one patients (66.1%) suffered complications: bowel obstruction (30.6%), hyperalimentation (14.5%), ureteral obstruction (12.9%), perforation (11.3%), abscess formation (3.2%) and spinal cord compression (3.2%). Two patients died. Non-FAP patients presented with three renal-cell carcinomas and one germ-cell tumor and carried pathogenic mutations in CHEK2, BLM, ERCC5, MSH6 and PALB2. Conclusions: Abdominal desmoids are mostly FAP-related and are associated with severe outcomes. We report a group of non-FAP abdominal desmoids that includes patients with additional cancer-related gene alterations. This interesting group should undergo genetic consultation and be further explored.

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Abdominal Desmoid: Course, Severe Outcomes, and Unique Genetic Background in a Large Local Series

Cancers ◽

10.3390/cancers13153673 ◽

2021 ◽

Vol 13 (15) ◽

pp. 3673

Author(s):

Gilad Ophir ◽

Shamai Sivan ◽

Strul Hana ◽

Rosner Guy ◽

Gluck Nathan ◽

...

Keyword(s):

Progression Free Survival ◽

Cord Compression ◽

Genetic Alterations ◽

Mortality Data ◽

Free Survival ◽

Cox Inhibitors ◽

Treatment Regimens ◽

Cancer Related Gene ◽

Surgical History ◽

Gene Alterations

Introduction: Abdominal desmoid tumors are locally aggressive tumors that develop in familial adenomatous polyposis (FAP) patients, within the mesentery or abdominal wall. The understanding and implications of the treatment regimens are evolving. Aim: To assess the course, treatment, and outcomes of FAP and non-FAP abdominal desmoids and their related genetic alterations. Methods: Retrospective cohort study. Demographics, tumor characteristics, oncological and surgical history, complications, genetic-testing, and mortality data were retrieved from two tertiary referral centers. Results: Sixty-two patients were identified (46 FAP and 16 non-FAP). Thirty-eight patients (61.3%) underwent surgical procedures (12 urgent and 26 elective). Out of 33 tumor resections, 39.4% recurred. Hormonal therapy, COX-inhibitors, chemotherapy, imatinib, and sorafenib were used in 35 (56.4%), 30 (48.4%), 18 (29.1%), 7 (11.3%), and 8 (12.9%) of patients, respectively, with a 2 year progression-free survival of 67.8%, 57.7%, 38.4%, and 28.5%, respectively. Forty-one patients (66.1%) suffered complications: bowel obstruction (30.6%), hyperalimentation (14.5%), ureteral obstruction (12.9%), perforation (11.3%), abscess formation (3.2%), and spinal cord compression (3.2%). Non-FAP patients carried pathogenic mutations in CHEK2, BLM, ERCC5, MSH6, and PALB2. Conclusions: Abdominal desmoids are mostly FAP-related and are associated with severe outcomes. We also report a group of non-FAP abdominal desmoids, which includes patients with additional cancer-related gene alterations. This interesting group should be further explored.

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Erdafitinib: A novel therapy for FGFR-mutated urothelial cancer

American Journal of Health-System Pharmacy ◽

10.1093/ajhp/zxz329 ◽

2020 ◽

Vol 77 (5) ◽

pp. 346-351 ◽

Cited By ~ 2

Author(s):

Kiera Roubal ◽

Zin W Myint ◽

Jill M Kolesar

Keyword(s):

Bladder Cancer ◽

Urothelial Cancer ◽

Growth Factor Receptor ◽

Progression Free Survival ◽

Clinical Effectiveness ◽

Genetic Alterations ◽

Novel Therapy ◽

Days Of Therapy ◽

Phase 2 Clinical Trial ◽

Gene Alterations

Abstract Purpose To provide an overview of fibroblast growth factor receptor (FGFR) gene alterations and the pharmacology, clinical effectiveness, dosage and administration, cost, and place in therapy of erdafitinib in bladder cancer. Summary Erdafitinib (Balversa, Janssen Pharmaceuticals) is a novel pan-FGFR inhibitor recently approved for the treatment of patients with advanced urothelial cancer with specific FGFR genetic alterations who have received at least one prior platinum-containing regimen. Erdafitinib binding to the FGFR2 and FGFR3 receptors inhibits FGF activity, resulting in cell death. Erdafitinib is available in tablet form, and the current recommended daily dosing is 8 mg, with dose escalation to 9 mg after 14 to 21 days of therapy if tolerated. A phase 2 clinical trial demonstrated that patients who received erdafitinib experienced on average 5.5 months of progression-free survival (95% confidence interval [CI], 4.2-6.0 months). In addition, 40% (95% CI, 31-50%) of patients responded to erdafitinib therapy. Patients receiving erdafitinib therapy should be monitored specifically for elevations in serum phosphate levels and changes in vision. Other adverse effects include anemia, thrombocytopenia, and electrolyte abnormalities. Conclusion Erdafitinib is the first small-molecule FGFR inhibitor approved for use in advanced bladder cancer.

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Genomic overview of right-sided and left-sided colorectal cancer using comprehensive genomic sequencing.

Journal of Clinical Oncology ◽

10.1200/jco.2017.35.15_suppl.e15101 ◽

2017 ◽

Vol 35 (15_suppl) ◽

pp. e15101-e15101

Author(s):

Yoshifumi Shimada ◽

Hitoshi Kameyama ◽

Masayuki Nagahashi ◽

Hiroshi Ichikawa ◽

Ryoma Yagi ◽

...

Keyword(s):

Colorectal Cancer ◽

Stage Iv ◽

Progression Free Survival ◽

Pi3k Pathway ◽

Genetic Alterations ◽

Genomic Sequencing ◽

Wild Type ◽

Mutant Type ◽

The Difference ◽

Gene Alterations

e15101 Background: Although the difference in right vs. left sidedness of colorectal cancer (CRC) in response to targeted therapy has been reported, we hypothesized that right-sided colorectal cancer (RCC) is more likely to have genetic alterations associated with resistance of anti-EGFR therapy. We tested this hypothesis using comprehensive genomic sequencing (CGS) on a set of samples from well-characterized CRC patients. Methods: Two-hundred-one primary colon cancer patients with either RCC or left-sided colorectal cancer (LCC) were analyzed. We investigated gene alterations using 415 gene panel, which includes the gene alterations associated with resistance of anti-EGFR therapy: TK receptors ( ERBB2, MET, EGFR, FGFR1, and PDGFRA), RAS pathway ( KRAS, NRAS, HRAS, BRAF, and MAPK2K1), and PI3K pathway ( PTEN and PIK3CA). We defined the patients who had no alterations in any of the genes as “all wild-type”, who are theoretically considered as responders of anti-EGFR therapy. Other patients with genetic alterations in resistance pathways were defined as “mutant-type”. Results: Fifty-six patients (28%) and 145 patients (72%) had RCC and LCC, respectively. Mutation of PIK3CA, BRAF, ACVR2A, MSH6, and CTNNB1 were significantly associated with RCC. Conversely, mutation of APC and TP53 were significantly associated with LCC. Regarding the gene alterations associated with anti-EGFR therapy, only 6 of 56 patients (11%) of RCC were “all wild-type”; in contrast to 41 of 145 patients (28%) of LCC that were “all wild-type” ( P = 0.009). In 45 Stage IV patients treated with anti-EGFR therapy, RCC showed significantly worse progression-free survival (PFS) than LCC ( P = 0.019), and “mutant-type” RCC showed worse PFS compared to the others ( P = 0.018). Conclusions: RCC is more likely to have the genetic alterations associated with resistance of anti-EGFR therapy compared to LCC. Primary tumor sidedness is a surrogate for the non-random distribution of molecular subtypes in CRC.

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Prognostic significance of circulating tumor cell (CTC) levels at various times during first- and second-line chemotherapy for metastatic breast cancer (MBC).

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.15_suppl.e21077 ◽

2012 ◽

Vol 30 (15_suppl) ◽

pp. e21077-e21077

Author(s):

Rafael Arteta-Bulos ◽

Paul J. E. Miller ◽

Rebecca S. Overbury ◽

Alison Schwartzberg ◽

Mark S. Walker ◽

...

Keyword(s):

Prognostic Significance ◽

Metastatic Breast ◽

Community Setting ◽

Progression Free Survival ◽

Circulating Tumor Cell ◽

Sensitivity Analyses ◽

Prognostic Information ◽

Treatment Regimens ◽

Oncology Practice

e21077 Background: CTCs have prognostic capability in the treatment of MBC based on a dichotomous level of ≥5/7.5 ml blood (Hi) vs. <5/7.5 ml blood (Lo) at baseline and first follow-up. The value of CTCs beyond baseline is not well established, and the implication of values that are reduced but remain high has not been explored. Methods: Chart review of patients (pts) on 1st or 2nd line treatment of MBC, with CTC determinations between 2/2008 and 9/2011, were retrospectively identified at a large community oncology practice. Pts with ≥2 CTCs or 1 CTC ≥5 were eligible. Demographics, treatment regimens and progression-free survival (PFS) were assessed. The Veridex Cell Search system was utilized for all CTC determinations. Qualifying baseline CTCs were those drawn -14 days to +30 days from start of treatment. Results: 221 eligible pts were identified, 71 with baseline CTCs in 1st line, and 84 in 2nd line. Median age was 59.5 (range 26-90), 40.7% were African-American, 17.6% HER2+, and 63%/49% ER/PR+. The median time to baseline CTC was 5 days in the 1st line and 1 day in the 2nd line. 1st line PFS was 8.0 vs. 5.8 mo among baseline Hi vs. Lo groups (p = 0.085). 2nd line PFS was 3.7 vs. 4.0 mo for Hi and Lo groups, respectively, (ns). Follow-up CTCs were obtained at clinical convenience. A 90 day landmark was used for analysis of PFS by four baseline/follow-up CTC pattern groups. 1st line PFS showed medians ranging from 6.0 mo (Hi/Hi) to 10.2 mo (Lo/Lo). In 1st line pts with Hi baseline CTCs (n=24), median PFS was 6.0 vs. 7.0 mo for Hi vs. Lo at follow-up (p = .64). Grouping pts with Hi baseline CTCs by reductions in absolute CTC count at follow-up (<33% vs. ≥33%) showed median PFS 3.5 mo for <33% reduction, 8.8 mo for ≥33% reduction (p = .0179). Sensitivity analyses with other cutoffs in reduction from 20-80% showed similar results. Conclusions: In the community setting, baseline and subsequent CTCs obtained at various times after baseline yield clinically useful prognostic information. Pts with positive baseline CTC levels experiencing a reduction of ≥33% exhibit significantly longer PFS, indicating that a simple high/low approach to the test can be modified to yield more prognostic value.

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A prospective study tracking longitudinal changes in genome-wide cell-free DNA (cfDNA) methylation to identify early nonresponders to cancer treatment.

Journal of Clinical Oncology ◽

10.1200/jco.2019.37.15_suppl.3042 ◽

2019 ◽

Vol 37 (15_suppl) ◽

pp. 3042-3042

Author(s):

Andrew A. Davis ◽

Wade Thomas Iams ◽

David Chan ◽

Michael S Oh ◽

Robert William Lentz ◽

...

Keyword(s):

Predictive Value ◽

Epigenetic Modification ◽

Progression Free Survival ◽

Response To Treatment ◽

Treatment Regimens ◽

Cancer Pathogenesis ◽

Before And After ◽

The One ◽

A Prospective Study

3042 Background: Methylation is an epigenetic modification linked to cancer pathogenesis. The aim was to determine if changes in cfDNA methylation patterns before and after initiation of treatment could predict non-response to treatment prior to routine imaging and clinical follow-up. Methods: We prospectively collected clinical data and blood from 28 patients with metastatic malignancies (13 lung, 11 breast, 4 other). Blood was drawn prior to start of a new treatment, after first cycle (median 30 days), and/or second cycle (median 57 days). We performed whole-genome (WG) bisulfite sequencing (median depth 18X) on plasma cfDNA to determine methylation levels. By tracking how methylation levels deviate from unaffected individuals, from baseline to subsequent timepoints, we classified patients as either progressors (greater deviance) or non-progressors. Treatment response at first follow-up imaging (FUI) was determined by RECIST 1.1. Study endpoints were agreement with first FUI and progression-free survival (PFS) by cfDNA classification. Results: The cohort consisted of 68% females and the median age was 70. Main treatment regimens were chemo- (N = 12), immuno- (6), endocrine (5), or targeted-therapy (5). PFS was significantly shorter (log-rank p = 8 x 10-7) in patients classified as progressors by cfDNA (N = 8; median: 62 days) compared to non-progressors (N = 20, median: 263 days). For patients classified as progressors, the cfDNA assay preceded imaging and clinical evaluation by a median of 34 days. 7 out of 8 patients classified as cfDNA progressors were later confirmed to progress at first follow-up evaluation (88% positive predictive value). The one patient who was classified as progressor based on cfDNA was stable based on FUI (day 93 of treatment) but was later confirmed as progression on day 128 by FUI. For the remaining patients, 18 of 20 did not progress (90% negative predictive value). Thus, sensitivity for the assay for identifying progression was 78% and specificity was 95%. Conclusions: Our results show that WG cfDNA methylation change is a novel signature with potential to identify patients whose treatment regimen is ineffective prior to imaging.

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A phase II, multicenter study of encorafenib/binimetinib followed by a rational triple-combination after progression in patients with advanced BRAF V600-mutated melanoma (LOGIC2).

Journal of Clinical Oncology ◽

10.1200/jco.2020.38.15_suppl.10022 ◽

2020 ◽

Vol 38 (15_suppl) ◽

pp. 10022-10022 ◽

Cited By ~ 3

Author(s):

Reinhard Dummer ◽

Shahneen Kaur Sandhu ◽

Wilson H. Miller ◽

Marcus O. Butler ◽

Christian U. Blank ◽

...

Keyword(s):

Objective Response ◽

Safety Data ◽

Progression Free Survival ◽

Genetic Alterations ◽

Tumor Evolution ◽

Free Survival ◽

Group A ◽

The Individual ◽

Bayesian Logistic Regression ◽

Gene Alterations

10022 Background: LOGIC2 evaluates the benefit of a 3rd agent added to encorafenib (enco)/binimetinib (bini), selected at progression based on the genetic tumor evolution. Methods: In part I/run-In, pts were treated with enco/bini until disease progression (as defined per RECIST v1.1). Foundation One NGS was applied on a baseline sample and on a PD sample. Based on the genetic evolution between the biopsy at inclusion (bxI) and at progression (bxPD) and clinical considerations, pts entered part II and received one of four 3rd agent additions to enco/bini combinations: A. LEE011 (CDK4/6 inhibitor), B. BKM120 (PI3K inhibitor), C. INC280 (c-Met inhibitor), or D. BGJ398 (FGFR inhibitor). An adaptive Bayesian logistic regression model (BLRM) guided by the escalation with overdose control (EWOC) principle was used to make dose escalation decisions. Assessments include objective response rate (ORR), disease control rate (DCR), progression-free survival (PFS), and safety. Data cutoff for this analysis was May 12, 2019. Data is as is. Part 1 of study is ongoing. Part 2 of study is closed to enrollment. Results: 58 pts enrolled into part II (group A=38; B=6; C=13; D=1). 29 pts were assigned to treatment based on bxPD results (Table). In groups A, B, and C, the confirmed ORR was 5.3%, 0%, and 0%, and the DCR was 26.3%, 16.7%, and 15.4%, with median PFS of 2.1, 1.6, and 2.2 months, respectively. Safety was consistent with known profiles of the individual agents. Conclusions: Triple therapy is feasible when a 3rd agent is added to enco/bini at progression based on genetic alterations, although activity observed was low. Further exploration to identify patterns of resistance susceptible to the addition of a 3rd agent is needed. Gene alterations for enrollment into part 2. Clinical trial information: NCT02159066. [Table: see text]

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Capecitabine, Bevacizumab, and Mitomycin in First-Line Treatment of Metastatic Colorectal Cancer: Results of the Australasian Gastrointestinal Trials Group Randomized Phase III MAX Study

Journal of Clinical Oncology ◽

10.1200/jco.2009.27.7723 ◽

2010 ◽

Vol 28 (19) ◽

pp. 3191-3198 ◽

Cited By ~ 270

Author(s):

Niall C. Tebbutt ◽

Kate Wilson ◽

Val J. Gebski ◽

Michelle M. Cummins ◽

Diana Zannino ◽

...

Keyword(s):

Colorectal Cancer ◽

Metastatic Colorectal Cancer ◽

Progression Free Survival ◽

Phase Iii ◽

Open Label ◽

Free Survival ◽

Treatment Regimens ◽

The United Kingdom

Purpose To determine whether adding bevacizumab, with or without mitomycin, to capecitabine monotherapy improves progression-free survival (PFS) in patients with metastatic colorectal cancer (mCRC) in an open-label, three-arm randomized trial. Patients and Methods Overall, 471 patients in Australia, New Zealand, and the United Kingdom with previously untreated, unresectable mCRC were randomly assigned to the following: capecitabine; capecitabine plus bevacizumab (CB); or capecitabine, bevacizumab, and mitomycin (CBM). We compared CB with capecitabine and CBM with capecitabine for progression-free survival (PFS). Secondary end points included overall survival (OS), toxicity, response rate (RR), and quality of life (QOL). Results Median PFS was 5.7 months for capecitabine, 8.5 months for CB, and 8.4 months for CBM (capecitabine v CB: hazard ratio [HR], 0.63; 95% CI, 0.50 to 0.79; P < .001; C v CBM: HR, 0.59; 95% CI, 0.47 to 0.75; P < .001). After a median follow-up of 31 months, median OS was 18.9 months for capecitabine and was 16.4 months for CBM; these data were not significantly different. Toxicity rates were acceptable, and all treatment regimens well tolerated. Bevacizumab toxicities were similar to those in previous studies. Measures of overall QOL were similar in all groups. Conclusion Adding bevacizumab to capecitabine, with or without mitomycin, significantly improves PFS without major additional toxicity or impairment of QOL.

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Progression-Free Survival in Third-Line Treatment of Relapsed/Refractory Multiple Myeloma Among Patients with Who Have Received Prior Bortezomib and Immunomodulatory Drugs

Blood ◽

10.1182/blood.v126.23.3311.3311 ◽

2015 ◽

Vol 126 (23) ◽

pp. 3311-3311

Author(s):

Sundar Jagannath ◽

Anuja Roy ◽

Jonathan K Kish ◽

Denise Globe ◽

Orsolya Lunacsek ◽

...

Keyword(s):

Progression Free Survival ◽

Standard Of Care ◽

Employment Equity ◽

Free Survival ◽

Treatment Regimens ◽

Risk Of Progression ◽

Study Population ◽

Equity Ownership ◽

Immunomodulatory Drug

Abstract Background: There are limited data on real-world treatment outcomes for novel agents used in the treatment of relapsed/refractory multiple myeloma (RRMM). These data are particularly important for patients who have already received bortezomib (BTZ) and an immunomodulatory drug (IMiD) given the lack of a standard of care in 3rd line and the costs of novel therapies including lenalidomide (LEN), carfilzomib (CFZ) and pomalidomide (POM). Methods: Data including patient and disease characteristics, treatment information and mortality data were collected from electronic medical records. RRMM patients diagnosed between 01/2007-08/2014 having received at least three treatment regimens (not including maintenance) were selected from the International Oncology Network (ION) database. Treatment regimens were aggregated into 8 mutually exclusive categories. Progression-free survival (PFS), based on physician reported date of progression, was calculated by treatment regimen overall and for the subgroup of patients who had prior BTZ and IMiD exposure. A multivariate proportional hazard model was used to estimate the risk of progression or death in the subgroup adjusting for age, gender, race, year of diagnosis, ISS stage, presence of cytogenetic abnormality, immunoglobulin type, receipt of stem-cell transplant (SCT), ECOG status and comorbidities at diagnosis. Results: There were 391 patients that met inclusion criteria. Mean age at diagnosis was 68 years, 46.9% being ISS III and 55.5% were classified as IgG. Median duration of follow-up was 36.1 months with 45% of patients being deceased by the end of follow-up. SCT information was available for 218 patients: 25.2% were deemed ineligible, 46.8% received an SCT and 28% of eligible patients had no evidence of receiving a SCT. BTZ and an IMiD were received by 239/391 (61.1%) patients prior to 3rd line. PFS in 3rd -line for the overall study population and the subgroup are presented in Table 1. For the subgroup, the median PFS in 3rd-line among regimens with at least 10 patient was: BTZ monotherapy ± non-IMiD - 7.3 months; LEN monotherapy or ± non-proteasome inhibitor (PI) - 4.9 months; BTZ + IMiD - 7.3 months; CFZ monotherapy ± IMiD - 3.7 months; and POM monotherapy ± PI - 5.8 months. Although not statistically significant, risk of progression or death after adjustment for baseline demographic and clinical characteristics was lower for BTZ + IMiD and other chemotherapies and higher for LEN monotherapy or ± non-PI, CFZ monotherapy ± IMiD and POM monotherapy ± PI compared to BTZ mono ± non-IMID (Table 1). Conclusions: These data are the first recent real-world treatment outcomes in RRMM. For BTZ-, LEN-, and POM-containing regimens, PFS was non-significantly shorter in 3rd-line for those patients with prior BTZ and IMiD treatment compared to the overall study population. For the subgroup, the results of the multivariate proportional hazards model showed no significant difference in the risk of progression or death in 3rd-line for patients with prior BTZ and IMiD therapy compared to other treatment regimens. As such, there remains a significant unmet need for novel treatment regimens which significantly improve PFS for the subgroup of patients who have received prior treatment with both BTZ and an IMiD. Table 1. Overall Patients with prior BTZ and IMiD¥ 3rd - Line Treatment Regimen N PFS (95% CI) N PFS (95% CI) HR p-value BTZ mono ± non-IMID 128 9.1 (7.1 - 10.9) 69 7.3 (4.2 - 10.8) REF - LEN mono ± non-PI 88 7.0 (4.7 - 10.6) 45 4.9 (3.0 - 8.6) 1.23 0.43 BTZ + IMID (thalidomide or lenalidomide) 59 9.4 (5.3 - 12.3) 39 7.3 (5.0 - 15.0) 0.84 0.55 THAL mono ± non-PI 12 8.5 (1.4 - 16.4) 7 9.8 (0.1 - 50.4) 0.94 0.89 MEL mono ± non-IMiD, non-PI 19 8.3 (3.4 - 13.2) 7 3.4 (0.6 - NR) 1.16 0.78 MEL + THAL 5 25.2 (0.7 29.4) 4 25.2 (0.7 - 29.4) * * CFZ mono ±IMID / Other † 37 3.6 (2.6 - 8.4) 34 3.7 (2.8 - 8.4) 1.13 0.68 POM mono ± PI / Other 17 9.6 (1.6-13.8) 15 5.8 (1.4 - 13.8) 1.18 0.68 Other Chemotherapies 26 5.6 (2.1 -13.3) 19 7.9 (1.9 - 14.0) 0.95 0.87 ¥ Steroids (dexamethasone or prednisone) may have been given in conjunction with any of the therapies per usual standard of care. † Includes patients treated with combination CFZ + POM. * Patients combined with "Other Chemotherapies" in model. Key: BTZ - bortezomib; CI - confidence interval; CFZ - carfilzomib; IMiD - immunomodulatory drug; LEN - lenalidomide; MEL - melphalan; NR - not reached; PI - proteasome inhibitor; POM - pomalidomide; THAL - thalidomide. Disclosures Jagannath: Janssen: Honoraria; Celgene: Honoraria; Merck: Honoraria; Bristol Myers Squibb: Honoraria; Novartis: Honoraria. Roy:Novartis Pharmaceuticals Corporation: Employment, Equity Ownership. Kish:Xcenda LLC: Employment. Globe:Novartis Pharmaceuticals Corporation: Employment, Equity Ownership. Lunacsek:Xcenda LLC: Employment. Eaddy:Xcenda LLc: Employment. Kuriakose:Novartis: Employment, Equity Ownership. Willey:Xcenda LLc: Employment. Siegel:Celgene Corporation: Consultancy, Speakers Bureau; Amgen: Speakers Bureau; Takeda: Speakers Bureau; Novartis: Speakers Bureau; Merck: Speakers Bureau.

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Spinal cord compression secondary to metastasis of malignant chondroid syringoma: case report

Journal of Neurosurgery Spine ◽

10.3171/2014.9.spine14360 ◽

2015 ◽

Vol 22 (3) ◽

pp. 310-313 ◽

Cited By ~ 3

Author(s):

Ricardo H. Menéndez ◽

Santiago G. Erice ◽

Carlos A. Bas ◽

Gabriel Casas ◽

Horacio S. Dillon

Keyword(s):

Spinal Cord ◽

Spinal Cord Compression ◽

Progression Free Survival ◽

Cord Compression ◽

Chondroid Syringoma ◽

History Of ◽

Epidural Metastasis ◽

Histological Results

The authors describe a case of spinal cord compression due to an epidural metastasis of malignant chondroid syringoma. Chondroid syringoma is a rare mixed tumor of the skin composed of both epithelial and mesenchymal elements. Although most are benign, malignant forms have been reported. Malignant chondroid syringoma may progress very slowly and the metastatic spread occurs late, appearing years after the original diagnosis. There is only one other report of spinal cord compression secondary to metastasis of malignant chondroid syringoma, which was finally diagnosed by microscopic examination of an autopsy specimen. This 63-year-old woman presented with a 4-week history of progressive paraparesis. Admission MRI of the thoracic spine showed an extradural mass arising from the posterior elements and left pedicle of T-9, which caused posterior compression of the spinal cord. Surgical decompression resulted in resolution of the neurological impairments. The histological results were consistent with metastasis of malignant chondroid syringoma. The patient underwent adjuvant radiotherapy and a favorable outcome was noted at the 2-year follow-up visit. This represents the first reported case of spinal cord compression from a metastasis of a malignant chondroid syringoma histologically confirmed in vivo. The authors' experience in this case suggests that resection followed by radiotherapy might be an acceptable means for achieving short-term, progression-free survival.

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