Doege-Potter syndrome associated to metastatic solitary fibrous tumor

Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge

Doege-Potter syndrome associated to metastatic solitary fibrous tumor

Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge

Phase II, non-comparative, prospective, randomized study of treatment with induction chemotherapy with cisplatin and gemcitabine followed by chemoradiation or definitive chemoradiation in invasive locally advanced carcinomas of uterine cervix

INTRODUÇÃO: O câncer cervical é o quarto mais comum no mundo e a quarta causa de morte por câncer em mulheres. A Quimiorradioterapia (QRT), com cisplatina, é o tratamento padrão para as pacientes com Câncer Cervical Localmente Avançado (CCLA). No entanto, 40% das pacientes apresentam recorrência da doença. A quimioterapia adjuvante, com gencitabina e cisplatina, aumenta a sobrevida global nesse grupo, com o aumento da toxicidade. O papel da Quimioterapia Neoadjuvante (QTN) no tratamento padrão com QRT permanece incerto. Conduziu-se um estudo clínico, a fim de investigar se a QTN, com cisplatina e gencitabina, seguida do tratamento QRT padrão, poderia melhorar os desfechos no CCLA. PACIENTES E MÉTODOS: Nesse estudo de fase II, as pacientes com CCLA (FIGO IIB-IVA) foram randomizadas para três ciclos de QTN, com cisplatina 50 mg/m² D1 e gencitabina 1000 mg/m2 D1 e D8, seguidos do tratamento de QRT padrão, com cisplatina semanal na dose de 40 mg/m2/semana por seis semanas, associado à radioterapia pélvica, seguida de Braquiterapia (BQT), braço experimental, ou ao tratamento de QRT padrão, seguido de BQT, braço padrão. OBJETIVOS: O objetivo primário foi avaliar a Sobrevida Livre de Progressão (SLP) em três anos. Os objetivos secundários foram analisar a Taxa de Resposta (TR), o controle locorregional, em três anos; a Sobrevida Global (SG), em três anos; e a segurança e a Qualidade de Vida (QV). RESULTADOS: Entre julho de 2012 e julho de 2017, 107 pacientes foram randomizadas. As características das pacientes foram semelhantes entre os grupos. A mediana de idade foi 48 anos. A maioria das pacientes apresentava carcinoma de células escamosas (87,8%) e estadiamento FIGO IIB (43,9%) ou IIIB (44,8%). Após um seguimento mediano de 31,7 meses, no grupo QTN, a SLP em 3 anos foi 40,9% em comparação a 60,4% no grupo QRT (HR, 1,91; IC 95%, 1,06 - 3,45; P = 0,030). Em três anos, no grupo QTN, a SG foi 60,7% versus 86,8% no grupo QRT (HR, 3,55; IC 95%, 1,42 - 8,91; P = 0,007). A taxa de resposta completa foi 56,3%, no grupo QTN e 80,3%, no grupo QRT (P = 0,008). A taxa de controle locoregional, em três anos, foi 63,4% no grupo QTN e 69.9%, no grupo QRT (HR, 1,56; IC 95%, 0,75-3,26; P = 0,227). As toxicidades foram semelhantes em ambos os grupos de tratamento, exceto a hipomagnesemia e a neuropatia que acorreram com maior frequência no grupo QTN. Na maioria das escalas de QV, não foram observadas diferenças significativas entre os grupos. O grupo QTN apresentou escores menores na escala de sintomas de menopausa (P = 0,034) e maiores em função sexual/vaginal (P = 0,012), em comparação à QRT padrão. CONCLUSÃO: No CCLA, a incorporação de QTN, com cisplatina e gencitabina ao tratamento padrão de QRT, foi inferior ao tratamento padrão com QRT em termos de SLP, SG e TR. A toxicidade e a QV foram semelhantes entre os grupos de tratamentoBACKGROUND: Cervical cancer is the fourth most common cancer worldwide and the fourth most frequent cause of cancer death in women. Although chemoradiation with cisplatin (CRT) remains the standard treatment for patients with locally advanced cervical cancer (LACC), 40% of patients present disease recurrence. Adjuvant chemotherapy with gemcitabine plus cisplatin improves overall survival, with high toxicity. The role of the addition of neoadjuvant chemotherapy (NAC) to definitive CRT is still a matter of debate. We conducted a trial to investigate if NAC with cisplatin and gemcitabine followed by CRT would improve outcomes. PATIENTS AND METHODS: In this phase II trial, LACC patients (FIGO IIB-IVA) were randomized to 3 cycles of NAC with cisplatin 50 mg/m2 D1 and gemcitabine 1000mg/m2 D1 and D8 followed by standard CRT with weekly cisplatin 40mg/m2/week for 6 weeks plus pelvic radiotherapy followed by brachytherapy (BCT) or to the standard CRT and BCT alone. OBJECTIVES: The primary endpoint was 3-year progression-free survival (PFS). Secondary endpoints were response rate (RR), 3-year locoregional control, 3-year overall survival (OS), safety and quality of life (QOL). RESULTS: Between July 2012 and July 2017, 107 patients were randomized. Patients characteristics were similar between groups. Median age was 48 years. The majority of the patients had squamous cell carcinoma (87.8%), and FIGO stage IIB (43.9%) or IIIB (44.8%). After a median follow-up of 31.7 months, NAC was associated with an inferior PFS, with 3-year PFS rates of 40.9% compared with 60.4% in the CRT arm (HR, 1.91; 95% CI, 1.06-3.45; P = .030). NAC was also associated with a lower OS (3-year OS, 60.7% vs 86.8%; (HR, 3.55; 95% CI, 1.42 - 8.91; P = .007). After treatment completion, complete response rates were 56.3% in the NAC arm and 80.3% in the CRT arm (P = .008). Three-year locoregional control rates were 63.4% in NAC arm and 69.9% in CRT arm (HR, 1.56; 95% CI, 0.75-3.26; P = 0.227). Toxicities were similar in both arms, with the exception of hypomagnesemia and neuropathy being more common with NAC. Comparing study arms, NAC showed statistically significant lower scores in menopausal symptoms (P = .034) and higher score in sexual/vaginal functioning (P=.012). For other scales, no difference between study arms was seen. CONCLUSION: This study has shown the addition of NAC consisting of cisplatin and gemcitabine to standard CRT is inferior to CRT alone for the treatment of LACC in PFS, OS and RR. Toxicity and QOL were similar in both arm

Adjuvant Carboplatin and Paclitaxel Chemotherapy Followed by Radiotherapy in High-Risk Endometrial Cancer: A Retrospective Analysis

Purpose: The best adjuvant treatment in high-risk endometrial cancer remains unclear. Although adjuvant chemotherapy seems to improve overall survival (OS) in locally advanced disease, the role of adding radiotherapy is not certain. We evaluated the outcomes of patients with high-risk endometrial cancer treated with adjuvant chemotherapy followed by radiotherapy. Patients and Methods: We performed a retrospective analysis of patients with high-risk endometrial cancer (endometrioid histology stages III to IVA or carcinosarcoma, clear cell, or serous histology stages I to IVA) treated with adjuvant carboplatin and paclitaxel, followed by radiotherapy, from 2010 to 2017 at a Brazilian cancer center. The Kaplan-Meier method was used for survival analysis, and prognostic factors were analyzed using the Cox proportional hazards model. Results: One hundred forty-six consecutive patients were evaluated. The OS rates were 86.2% at 3 years and 75.4% at 5 years. OS was significantly affected by pelvic lymphadenectomy (P = .001) and positive peritoneal cytology (P < .001). Three- and 5-year disease-free survival (DFS) rates were 78.3% and 69.5%, respectively. The initial site of recurrence was limited to the pelvis in 4.1% of patients, within the abdomen in 1.3%, and extra-abdominal in 11.6%. Patients with grade 1 or 2 endometrioid carcinoma had better prognosis than patients with endometrioid carcinoma grade 3 or nonendometrioid histology (3-year DFS, 93.67% v 68.5%, respectively; P = .0017). Conclusion: Adjuvant carboplatin and paclitaxel, followed by radiotherapy, is effective in high-risk endometrial cancer and associated with low rates of pelvic recurrence, which might be explained by the addition of radiotherapy. The high-risk group is heterogeneous, and the benefit of adjuvant treatment in patients with grade 1 or 2 endometrioid carcinoma is less clear

Key issues in the management of cervical cancer: consensus recommendations by a Brazilian expert panel

Objective: We report the results of a panel of Brazilian experts and provide recommendations for the management of these patients. Material and Methods: The panel convened composed by 28 local opinion leaders, addressed 59 multiple-choice questions taking into account the published scientific literature and their own clinical experience. The level of agreement among panel members was qualified as (1) consensus, when at least 75% of the voting panel members; (2) majority vote (50%-74.9%); or (3) less than majority vote. Results: There was at least majority vote for eight of 10 questions on staging and follow-up; for 14 of 23 questions on the treatment of early-stage disease; for 12 of 14 questions related to the treatment of locally-advanced disease; and for seven of the 12 questions related to the treatment of recurrent/metastatic disease. Conclusion: The current recommendations may help practitioners from Brazil and other countries to improve the care they provide to patients