Abstract
Background: Familial adenomatous polyposis (FAP) is an autosomal dominant condition caused by germline mutation in the tumor suppressor gene APC. FAP occurs in 1 to 10.000 individuals, and ...is characterized by hundreds to thousands of colonic adenomatous polyps with a high risk of developing into colorectal cancer. Extracolonic manifestations can be malignant or benign. The major causes of morbidity and mortality in patients with FAP are abdominal desmoid tumors, with incidences ranging between 7% and 17%. Adrenal incidentaloma are frequently discovered in these patients, generally as benign lesions when they undergo abdominal CT-scan in the course of surveillance. Adrenal lesions in FAP ranged from 7.4% to 16%. We described an unusual patient with FAP, associated with autonomous cortisol production due to bilateral adrenal tumors and the development of aggressive desmoid tumor after unilateral adrenalectomy. Clinical Case: A 33-years-old female FAP-patient presented with abdominal pain, weight gain (10kgs), humor instability, paroxysmal of chest pain, dizziness and tremors. The abdominal MRI showed a heterogeneous, left adrenal mass (9.0 x 7.9 x 6.7cm), suspected for malignant tumor, and right adrenal mass with 3.6 x 1.8 cm suggestive of adenoma. Abdominal CT and PETCTFDG revealed on the left adrenal lesion with 33UH and maxSUV 3.9 and a right adrenal lesion 13UH and maxSUV 3.1. Serum hormone levels were as follows: cortisol after DST (1mg-dexamethasone) 4.8 ug/Dl, ACTH 8,8pg/Ml with no other abnormal hormone secretion detected. Patient underwent left adrenalectomy. Histological analysis revealed Weiss 1, modified Weiss 2 and Ki67 <1% compatible with adenoma. On follow-up, abdominal MRI revealed a 4.3cm-solid-homogeneous mass at the surgical incision, suspected of malignance. The mass progressively enlarged to 6.3 cm in diameter. Histological analysis of the biopsy identified a desmoid tumor. The contralateral adrenal tumor maintained stable during the follow-up; however, it began to produce cortisol autonomous secretion as observed on DST. The patient developed metabolic syndrome and did not present classical Cushing’ syndrome. Contralateral adrenalectomy was contraindicated because of concern of emergence of a new desmoid tumor. Discussion: FAP-associated with adrenal tumors can produce mineralocorticoids, corticosteroids, or both. Although adrenal cortical tumors have been reported frequently in FAP patients, the presence of bilateral commitment tumors on adrenal glands is extremely rare. Conclusion: We reported a woman with FAP and bilateral adrenal tumors with non-synchronic cortisol secretion associated with an aggressive desmoid tumor developing after the adrenalectomy. The patient is taking an adrenal inhibitor of steroidogenesis to control cortisol secretion and to provide clinical improvement.
Abstract
Dermal sarcomas represent a group or rare malignancies of mesenchymal origin. Although surgical excision with wide margins can be curative, in the advanced/metastatic setting, treatment ...options are limited and the benefit from anthracycline-based chemotherapy or targeted agents is usually short-lived. Tumor mutational burden and PD-L1 expression scores can be used as predictive biomarker for response to immunotherapy in some metastatic cancers. The role of immune-checkpoint blockade for sarcoma patients remains investigational. Here we present three cases of dermal sarcomas with high TMB and PD-L1 expression and responses to anti-PD1 agents in two of them.
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e12513
Background: Temozolomide (TMZ) both concurrently and after radiation therapy constitutes the standard of care for newly diagnosed GBM PTS. BEV is FDA approved option for ...recurrent high-grade gliomas PTS based on the results phase II trials. Reports of the use of BEV in these setting have been limited to USA and European academic centers thus far. Methods: We conducted a cross sectional retrospective study of 39 consecutive PTS with histologically confirmed GBM that received BEV in the second or third line setting. The main objective of this analysis was to assess the efficacy and safety of BEV given “off protocol” in an unselected cohort of PTS treated at a Brazilian teaching hospital cancer center and to compare our results to those reported by North American and European academic centers. PTS received first-line treatment with TMZ plus radiotherapy and most received maintenance TMZ and received Bev-based therapy at the time of disease progression. Main endpoints were the evaluation of progression-free survival (PFS), overall survival (OS) and safety. Results: Between 2007 and 2011, 39 PTS with recurrent GBM that received BEV in second (92%) or third-line (8%) were identified. Seven PTS progressed during concomitant RT and TMZ, and 40 during or after TMZ maintenance. 72% were male; median age: 56 years (range 22-79). Most patients (89,7%) received BEV in combination with irinotecan, and the median number of cycles, regardless of combination, was 14. Reasons for BEV discontinuation were disease progression in 79,5% and toxicity in 4 patients. There was one treatment related death due to thrombocytopenia and bleeding. Twenty-four PTS (61,5%) achieved an objective response with BEV. Median PFS of the patients that received BEV in either second or third line was 7.5 months and median OS was 22.6 months. No bowel perforations or any unexpected toxicities occurred. Conclusions: This constitutes the first report of recurrent GBM PTS treated with Bev in the Southern Hemisphere. In this unselected “real life” cohort of PTS with recurrent GBM we have been able to reproduce and confirm the efficacy and safety of this agent used in the treatment of GBM in the second and third line setting.
There has been a rapid increase in the volume of genomic data gathered from different cancers, this has helped to develop new tumor classifications as well as to select better tailored therapies for ...the patients. Some of the genomic markers identified are also prognostic and predictive factors. Additionally, many technologies have been used to investigate these alterations, each with different benefits and caveats. The Genomics Committee from the Sociedade Brasileira de Oncologia Clínica (SBOC) put together a group of specialists, from different regions of Brazil that work both in the private and public scenario, to gather and organize the information regarding the utility of somatic mutation testing in solid tumors. This special article summarizes their recommendations on how to better incorporate this information into clinical practice.
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2038
Background: Pilocytic astrocytoma (PA) accounts for up to 25% of all brain tumors in children. A 75% progression-free survival rate at 5 years and overall survival rates of 95.8% ...at 10 years have been reported after a complete resection. However, the incidence in adults is low and the outcome less well characterized. We conducted a retrospective analysis of adult PAs identified in order to review the clinical characteristics and outcome of this neoplasm in this age group. Methods: A descriptive, cross-sectional study was undertaken in patients over 16 y.o., diagnosed with PA between January 1990 and December 2010 at our center. Clinical characteristics, date and extent of surgery, tumor location, postoperative treatment, complications and recurrences were collected from hospital records. Results: From May, 1990 to December, 2010, 18 eligible adult patients (pts) with the diagnosis of PA were identified. Median age was 25,5 (16-52) years, 72,2% female and 83,3% had an ECOG performance status ≤ 2. The most frequent tumor sites were the cerebellum (44,4%), followed by the cerebral hemispheres (38%). All patients underwent surgery as primary treatment, 72% had a complete macroscopic resection (CMR) and 28% had a partial resection (PR). Two patients received postoperative radiotherapy: one following a PR and the other after tumor relapse following a CMR. Two patients relapsed five months and 44 months after initial complete gross resection. Salvage surgery achieved CMR in both. With a median follow-up of 89 months, all patients are alive, except for one who died in the postoperative period due to fungal meningitis. Of note, none of 5 pts undergoing a partial resection progressed and 3 are alive for more than 10 years. Conclusions: As it occurs in the pediatric population, PA in adults seems to carry a similarly favorable prognosis. It is conceivable that after initial surgical resection a watch and wait type of approach is appropriate, even following partial resection. The role of upfront radiotherapy is uncertain and it should probably be left for progressive tumors.
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e16046
Background: Since the results of the RTOG 9111 trial, cisplatin based chemoradiation (CRT) has been the standard of care for LP in advanced laryngeal SCC. Recently, the role of ...taxanes in managing head and neck SCC has been studied. In 2002 we reported the interim results of a phase II clinical study designed to test the efficacy of P and C concurrent with RT for organ preservation in advanced L and HP SCC. Here we report the long term survival and LP rates. Methods: Eligible patients had untreated advanced L and HP SCC, stage T3N0 or higher and suitable for radical total laryngectomy. Treatment consisted of weekly P (30 mg/m
2
) and C (20 mg/m
2
) concurrent to RT up to 7040 cGy in 180 cGy/day fractions. Response evaluation was performed at 5040 cGy and at 4 weeks after completing RT. Salvage surgery was planned for patients not responding at 5040 cGy, residual tumor at the end of RT or at the time of local recurrence. Neck dissection was planned for clinically positive neck (cN1-3). Results: Between 06/1999 and 10/2001, 48 patients were enrolled in a single institution (35 L; 13 HP), 40 male and 8 female with a 58-year median age (39-74). The majority had T3 (64%) N1-3 (52%) disease and 38% needed tracheostomy prior to treatment. Grade (G) 3 and 4 mucositis was noted in 27% of patients, G 3-4 odynophagia in 50%, G 3-4 radiodermatitis in 35% and G 3-4 leucopenia in 13%, with no treatment related death. Two patients needed salvage surgery, one after 5040 cGy and one after 7040 cGy. The complete response rate to treatment was 95%. At a median follow up of 66 months the LP rate at 2 and 5 years were 88% and 54%, respectively. Recurrence free survival (RFS) was 51% at 2 and 47% at 5 years and overall survival (OS) was 81% and 52% at 2 and 5 years, respectively. Conclusions: The finding of similar LP rate and survival compared to the recent reports of high dose cisplatin CRT and neoadjuvant 3 drug CT followed by CRT in an advanced disease population suggests a role for platinum plus taxane as a radiosensitizer regimen in this scenario with acceptable toxicity, but further evaluation in a direct comparative trial with the standard regimen is needed.
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