Introduction. Chordomas are rare malignancies of bone origin that occur in the axial skeleton, typically the skull base and lumbar/sacral regions. Although often classified as low-grade neoplasms, ...its locally infiltrative behavior may result in significant morbidity and mortality. Optimal surgical resection may be curative, but up to 50% of the cases relapse within 5 years, and currently there are no systemic treatments approved in this setting. A large proportion of these tumors express stem-cell factor receptor (c-KIT) and platelet-derived growth factor receptors (PDGFRs), providing a rationale for the use of tyrosine-kinase inhibitors (TKIs). Case report. A 27-year-old male presented with recurrent chordoma of the lumbar spine 4 years after initial diagnosis. Salvage therapies in the interval included repeat resections and radiation therapy. He ultimately developed multifocal recurrence not amenable to complete excision or reirradiation. A comprehensive genomic profiling assay was performed and revealed nondrugable alterations. Decision was made to proceed with systemic treatment with pazopanib 800 mg/day, resulting in tumor reduction (−23.1% reduction in size) and prolonged disease control. Conclusion. For this patient with a multiple recurrent chordoma and limited treatment options, pazopanib resulted in sustained clinical benefit following initial tumor reduction.
Eosinophilic solid and cystic renal cell carcinoma (ESC RCC) is a recently described entity with distinct clinical, pathologic, and molecular features. However, the radiological aspects of ESC RCC ...have not been characterized. In this report, we describe the imaging findings of 2 ESC RCCs. We found 2 distinct imaging patterns that varied depending on histopathologic features (solid or cystic predominance). In conclusion, it is important to know the imaging characteristics and pathologic correlation of this novel neoplasm to increase its recognition and to improve the decision-making process.
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Background: Despite optimal management of NMIBC with transurethral resection of bladder tumor (TURBT) and intravesical BCG therapy, a significant proportion of patients (pts) will ...eventually present with disease recurrence or progression. To date, there is no validated predictive biomarker to guide patient selection for the most appropriate therapy in this setting. Methods: We retrospectively identified pts with high-risk NMIBC treated with TURBT, repeat TUR and intravesical BCG (≥ 6 instillations) from 2009 to 2016. Patients were classified as BCG-responsive (BCG-R) and BCG-unresponsive (BCG-UR) based on the International Bladder Cancer Group criteria. Whole exome sequencing was performed using archival FFPE tumor tissue from pre-BCG TURBT samples. Association of genomic variables and outcomes was evaluated by Cox proportional hazards survival analysis and likelihood ratio tests. Results: Thirty-five patients were included (BCG-R = 17, BCG-UR = 18). Median follow-up was 46 months for BCG-R and 52 months for BCG-UR pts. The majority of pts was male (91.4%), former smoker (60%), and presented with high-grade urothelial carcinoma (85.7%) and/or T1 staging (71.4%). Median time for relapse and progression was 10.5 and 19 months, respectively, in the BCG-UR group. In this cohort, TMB was significantly different in BCG-R and BCG-UR groups, with a median TMB of 5.53 +- 4.60 and 3.17 +- 1.82 mutations/Mb, respectively ( P= 0.045). TMB was also associated with relapse-free survival (RFS), with a median RFS of 38 and 15 months in high versus low TMB groups, respectively ( P= 0.0092). Intratumoral genetic heterogeneity assessed by mutant-allele tumor heterogeneity (MATH) was not statistically different between the groups, with a median MATH score of 31.8 and 21.9 for BCG-R and BCG-UR ( P= 0.14), respectively. On multivariate analysis, age and TMB were independently associated with RFS. Conclusions: In this exploratory biomarker study, high TMB was associated with benefit from immunotherapy with BCG for NMIBC. The identification of predictive biomarkers in this setting is an important unmet need and integrative analysis of TMB with other potential predictive biomarkers should be assessed in larger datasets.
Abstract
Background
Endometrial stromal sarcomas (ESSs) are the second most common uterine sarcomas. Although ESSs are often indolent, they have metastatic potential. To the best of our knowledge, ...there are only three reports of brain metastasis, and the present report is the first to describe a late skull metastasis of an ESS.
Case Report
We describe the case of a 51-year-old woman who presented abnormal vaginal bleeding 14 years ago; she was diagnosed with an uterine mass and submitted to a hysterectomy. One year ago she presented ESS lung metastasis followed by a left parietal calvarial metastasis. The optimal treatment for metastatic ESS is controversial, but the use of progesterone and aromatase inhibitors is advisable.
To evaluate transrectal ultrasound, amplitude Doppler ultrasound, conventional T2-weighted magnetic resonance imaging, spectroscopy and dynamic contrast-enhanced magnetic resonance imaging in ...localizing and locally staging low-risk prostate cancer.
Prostate cancer has been diagnosed at earlier stages and the most accepted classification for low-risk prostate cancer is based on clinical stage T1c or T2a, Gleason score ≤6, and prostate-specific antigen (PSA) ≤10 ng/ml.
From 2005 to 2006, magnetic resonance imaging was performed in 42 patients, and transrectal ultrasound in 26 of these patients. Seven patients were excluded from the study. Mean patient age was 64.94 years and mean serum PSA was 6.05 ng/ml. The examinations were analyzed for tumor identification and location in prostate sextants, detection of extracapsular extension, and seminal vesicle invasion, using surgical pathology findings as the gold standard.
Sixteen patients (45.7%) had pathologically proven organ-confined disease, 11 (31.4%) had positive surgical margin, 8 (28.9%) had extracapsular extension, and 3 (8.6%) presented with extracapsular extension and seminal vesicle invasion.
Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy values for localizing low-risk prostate cancer were 53.1%, 48.3%, 63.4%, 37.8% and 51.3% for transrectal ultrasound; 70.4%, 36.2%, 65.1%, 42.0% and 57.7% for amplitude Doppler ultrasound; 71.5%, 58.9%, 76.6%, 52.4% and 67.1% for magnetic resonance imaging; 70.4%, 58.7%, 78.4%, 48.2% and 66.7% for magnetic resonance spectroscopy; 67.2%, 65.7%, 79.3%, 50.6% and 66.7% for dynamic contrast-enhanced magnetic resonance imaging, respectively.
Sensitivity, specificity, PPV, NPV and accuracy values for detecting extracapsular extension were 33.3%, 92%, 14.3%, 97.2% and 89.7% for transrectal ultrasound and 50.0%, 77.6%, 13.7%, 95.6% and 75.7% for magnetic resonance imaging, respectively. For detecting seminal vesicle invasion, these values were 66.7%, 85.7%, 22.2%, 97.7% and 84.6% for transrectal ultrasound and 40.0%, 83.1%, 15.4%, 94.7% and 80.0% for magnetic resonance imaging.
Although preliminary, our results suggest that imaging modalities have limited usefulness in localizing and locally staging clinically low-risk prostate cancer.
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Background: Partial nephrectomy (PN) is the standard of care in the management of cT1a tumors, while radical nephrectomy (RN) is indicated in more advanced tumors. Recent studies ...provided evidence that PN could be performed in patients with tumors greater that 7 cm with complication rates and oncological outcomes comparable with those undergoing RN. This study compares the recurrence-free survival (RFS), overall (OS) and cancer-specific survival (CSS) of PN and RN in patients with non-metastatic pathological T3a renal cell carcinoma (RCC) with perirenal fat invasion only. Methods: We reviewed 1202 patients undergoing RN (n = 653) and PN (n = 549), at a oncological referral center, from January 2003 to June 2016. Of all patients, we identified 25 RN and 41 PN pT3a tumors with exclusively perirenal fat invasion. None had nodal or distant metastasis at pretreatment clinical staging. Patients characteristics were compared with Mann-Whitney U test and Student t-test for categorical and numeric variables with normal distribution, respectively. Both groups were compared for RFS, OS and CSS with a Kaplan-Meier survival analysis. Results: All patients included had pT3a stage with isolated perirenal fat invasion. Groups undergoing RN and PN were not significantly different regarding Charlson Comorbidity Index (Median 3 for RN vs 4 for PN, p = 0.24) or Age (Mean 65.3 for RN vs 62.0 for PN, p = 0.99). Patients undergoing RN had bigger tumors (7.9 cm vs 4.6, p < 0.001) and higher Fuhrman grade (p = 0.01). Median follow-up was 36 months for RN and 34 months for PN. At the end of follow-up, recurrence was seen in 3 patients undergoing RN (12%) and 2 undergoing PN (5%), p = 0.36. Mortality was similar across groups (16% for RN vs 15% for PN, p = 0.99) as well as Cancer-specific mortality (4% for RN vs. 5% for PN, p = 0.99). At the end of follow-up, RFS was 80% (20/25) for RN and 82% (34/41) for PN. Conclusions: In our data, renal cell carcinoma with T3 stage due to perirenal fat invasion exclusively had similar outcomes when treated with Radical or Partial Nephrectomy. OS as well as RFS were comparable for both surgical modalities, suggesting that, although RN is currently the gold standard for this staging, PN may provide similar oncologic results.
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Background: The role of extended pelvic lymph node dissection (ePLND) in treating prostate cancer (PCa) patients remains controversial, mainly by the lack of RCTs. Methods: ...Patients with D'Amico intermediate or high risk PCa, absence of bone metastasis and no previous treatment were prospectively computer randomised to undergo extended or limited PLND (1:1) during radical prostatectomy. Limited PLND (lPLND) included the obturator chain bilaterally; ePLND involved bilaterally chains: obturator, external-, internal-, common-iliac and pre-sacral. Surgical specimens and each chain were analyzed separately, according to College of American Pathologists. All patients signed a free and informed consent and local ethics committee approved the study. The primary endpoint was biochemical recurrence-free survival, analysed in the intention-to-treat population. This trial is registered with ClinicalTrials.gov, number NCT01812902. Results: Since May 2012 until August 2016, 291 patients were randomly assigned, 145 to ePLND and 146 to lPLND. Preoperative data were comparable between groups. Median follow-up was 35.2 months. EPLND increased significantly operative time (54 minutes), estimated blood loss (100 mL), length of hospital stays (1 day) p≤0.001, transfusion rate p = 0.05 and postoperative complications according to Clavien scale p = 0.03. There was no difference in Pathologic Gleason grade, T stage or positive surgical margin. On ePLND and lPLND groups, 59.3% and 61.7% were staged ≥ pT3a, respectively. EPLND and lPLND yielded median (mean) 17 (19.8) and 3 (4.1) nodes, respectively (p < 0.001). EPLND showed 6.3 times more lymph node metastases (p < 0.001) and only it was able to show positive nodes in intermediate risk. There were no difference in biochemical recurrence (PSA ≥ 0.2 ng/mL) using Kaplan-Meyer method (p = 0.4), Radiotherapy, Androgen Deprivation Therapy, bone metastases or death. Conclusions: Extended lymphadenectomy in intermediate- and high-risk prostate cancer patients is associated with better tumor staging, increased morbidity and no oncological benefits in this initial short follow-up time. Clinical trial information: NCT01812902.
Abstract
Introduction
Pigmented villonodular synovitis (PVNS) is a benign but aggressive lesion arising from sinovia. The temporomandibular joint (TMJ) is hardly ever involved.
Methods
We describe ...a case of PVNS arising in the left TMJ involving infratemporal fossa soft tissue and the skull base; we also present the reconstruction.
Results
A 37-year-old woman had progressive mandibular swelling for 6 months. Computed tomography of the skull revealed an osteolytic lesion in the left TMJ, involving the upper mandible, condyle, and glenoid fossa and extending to the infratemporal fossa and fossa media through a defect in temporal bone. Surgical management included a left pterional craniotomy to reach the temporal skull base and resect the intracranial tumor and a facial approach with partial left mandibulectomy and resection of left condyle, glenoid fossa, and tumor removal in infratemporal fossa. Mandible function was restored with prosthetic reconstruction of the condyle. She progressively started to eat solid foods after 3 months, becoming increasingly functional and asymptomatic. At 30 months' follow-up, she had no sign of tumoral recurrence and showed asymptomatic and normal TMJ function.
Conclusion
PVNS should be considered in the differential diagnosis of bone neoplasms affecting young patients. In such cases, radical excision is mandatory and TMJ prosthesis for local reconstruction may be used to preserve functionality.
Background: Pituicytomas are considered World Health Organization Grade I malignancies. Until September 2017, a total of 81 cases of pituicytomas were diagnosed and described in literature. We ...present such a case in which histopathology shows an epithelioid pattern, a rare variant of pituicytoma. As far as we know, this is only the second such case described in the literature.
Case Description: A 61-year-old male patient presented with complaints of progressive decrease in visual acuity for about 7 months, worse on the left side. Laboratory and endocrinological investigation returned normal values. Magnetic resonance imaging revealed a mixed solid-cystic lesion, measuring about 3.1 × 2.2 × 2.9 cm. The lesion presented with intermediate signal intensity in T1 and T2 sequences and showed avid postcontrast enhancement. The patient underwent resection through a left pterional approach. Pathology revealed a glial neoplasm with an epithelioid pattern and moderate cellularity with rounded-elongated cell nuclei and with a broad eosinophilic cytoplasm. Absence of cellular pleomorphism, any mitotic figures, or areas of necrosis was noted.
Conclusion: The epithelioid variant of pituicytomas differs from the commonly encountered forms of this tumor which typically present in a fascicular pattern. Microsurgical resection is the treatment of choice. However, in many cases, subtotal resection was performed because of a considerable risk for neurovascular injuries.
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