Exacerbated Renal and Hematologic Toxicities to Ifosfamide and Doxorubicin-Based Chemotherapy in a Patient with Retroperitoneal Liposarcoma Harboring a Germline Mutation in the WRN Gene.

Werner's syndrome is caused by the inactivation of both WRN alleles and is characterized by premature aging and increased risk of neoplasms, especially those of mesenchymal origins, such as sarcomas. Given the characteristic genomic instability, patients with this syndrome are more susceptible to develop toxicities when exposed to cytotoxic agents, such as alkylators and anthracyclines. The impact of the monoallelic WRN mutation on treatment-associated toxicities is poorly understood. Here, we report a patient with locally advanced dedifferentiated liposarcoma of the retroperitoneum harboring a heterozygous germline inactivation mutation in the WRN gene, who was treated with a classic regimen of ifosfamide and doxorubicin and developed exacerbated and prolonged hematological and renal toxicities.

18F-FDG PETCT and 68Ga-DOTA PETCT mismatch with in vivo histopathological characterization of low-grade neuroendocrine pancreatic tumor.

BACKGROUND: Pancreatic neuroendocrine tumor (PNET) is a subgroup of neuroendocrine tumor (NET) that has unique biology and natural history. The histological classification has a major role in the management of this pathology, but in recent years Gallium 68 dotatate (68Ga-DOTA) scanning is at the center of a discussion about how these imaging technologies can modify clinical management of neuroendocrine tumors and how their results are correlated to Ki67 index. METHOD: We hereby describe a case of a patient that investigated an unspecific stable pancreatic nodule suspected of high-grade NET after evaluation with 68Ga-DOTATOC positron emission tomography-computed tomography (PETCT) and 18F-Fluorodeoxyglucose (18F-FDG) PETCT. RESULTS: The images corroborate the hypothesis of high-grade NET based on the standard uptake value (SUV) described in both image exams (16.4 in 18FDG PETCT and 9.2 in 68Ga-DOTATOC PETCT). After surgery, the histopathological analyses revealed a localized grade 2 well-differentiated NET, Ki-67 of 4.7, glucose transport proteins 1 (GLUT1) negative by immunohistochemistry, evidencing a rare case of mismatch between the functional image and the in vivo characterization of the neoplasm. CONCLUSION: Functional imaging of neuroendocrine tumors with different modalities of PETCT is a well-described strategy for evaluating PNET and can dictate conducts in some cases. However, histopathological analysis is crucial to confirm the grade and prognosis related to this disease.

Response to anti-PD1 immunotherapy in patients with metastatic cutaneous sarcoma: case reports and literature review.

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.

Neoplasia mucinosa papilar intraductal: relato de casos / Intraductal papillary mucinous neoplasm: case reports

A Neoplasia Mucinosa Papilar Intraductal (NMPI) consiste em uma proliferação do epitélio ductal pancreático, frequentemente de aspecto papilar, com hipersecreção e retenção de mucina, e consequentemente dilatação cística do ducto pancreátrico principal e/ou ductos secundários. Desde sua primeira descrição, tem se observado um aumento frequente no diagnóstico de NMPI, assim como das lesões císticas pancreáticas, e conforme estas lesões se tornaram achados mais frequentes, é de extrema importância sua diferenciação, devido algumas apresentarem importante potencial maligno. A NMPI, na grande maioria das vezes, é assintomática ou apresenta sintomas inespecíficos, representando achados incidentais em exames de imagem por sinais e sintomas não relacionados ao pâncreas. O tratamento depende do tipo de NMPI. Enquanto a NMPI ducto principal sempre requer cirurgia, na NMPI ramos secundários pode-se optar por seguimento clínico. Relataremos dois casos de NMPI ramos secundários optado por tratamento clínico, sendo realizada ainda uma revisão da literatura sobre a caracterização da doença, aspectos do seu diagnóstico e tratamento (AU)