RESUMEN
Background: Spontaneous remission (SR) is defined as the complete or partial disappearance of a diagnosed malignant disease in the absence of known active medical treatment. The role of the immune system is thought to be important, but has not yet been elucidated. On this matter, there are studies that suggest that the abscopal effect (AE), which is defined as the remission of untreated lesions beyond the irradiated area, may be explained by the activation of a systemic immune response against the tumor. Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of soft tissue sarcoma that is characterized by a slow evolution, with local recurrences and late metastases. The treatment is based on surgery, leaving a minimal role to chemotherapy (ChT) and radiotherapy (RT) for metastatic unresectable disease, and no cases of SR have been reported in the literature so far. Case Description: We present the case of a patient with a lung metastatic recurrence of SEF, diagnosed and treated with surgery 8 years before. After progression to pazopanib and other ChT drugs, because of the chest pain associated with a pleural mass invading the second costal arch, the patient received antalgic local RT treatment. Months later, and without any further treatment, a partial remission of all the tumoral lesions was presented, and she is alive 25 years after the first diagnosis. Conclusions: As far as reported in the literature, this is the first case of SR in SEF. Among the possible causes of this SR, we think that the most plausible is that palliative treatment with RT of the pleural mass induced an AE, leading to a reduction of all tumoral lesions, even those outside the irradiated region.
RESUMEN
Endometrial stromal sarcoma (ESS) is an uncommon mesenchymal tumor that accounts for less than 1% of all primary uterine malignancies and extrauterine endometrial stromal sarcoma (EESS) is even rarer. We report the case of a 75-year-old woman with an abdominal tumor and multiple peritoneal implants. Histological analysis of the surgical specimens showed bland cellularity resembling normal endometrial stroma. The diagnosis of a low-grade EESS was confirmed by immunophenotypic findings and demonstration of JAZF1 translocation. After extensive sampling, no evidence of endometriosis was found. Our case showed atypical aggressive behavior and we discuss the possible influence of the high mitotic count (8/10 HPFs) in some areas of the tumor, the multifocality of the abdominal implants and the postmenopausal status of the patient. The unusual clinical presentation and extrauterine location of such a rare tumor were challenging implying a wide range of differential diagnosis. The correlation of morphological, immunohistochemical and molecular findings was necessary to arrive at the correct diagnosis.
Asunto(s)
Neoplasias Endometriales/patología , Tumores Estromáticos Endometriales/patología , Neoplasias Peritoneales/patología , Anciano , Proteínas Co-Represoras/genética , Proteínas de Unión al ADN/genética , Neoplasias Endometriales/genética , Tumores Estromáticos Endometriales/genética , Femenino , Humanos , Índice Mitótico , Neoplasias Peritoneales/genética , Translocación GenéticaRESUMEN
Pancreatic cancer is a dismal disease with an increasing incidence. Despite the majority of patients are not candidates for curative surgery, a subgroup of patients classified as borderline resectable pancreatic cancer can be selected in whom a sequential strategy of neoadjuvant therapy followed by surgery can provide better outcomes. Multidisciplinary approach and surgical pancreatic expertise are essential for successfully treating these patients. However, the lack of consensual definitions and therapies make the results of studies very difficult to interpret and hard to be implemented in some settings. In this article, we review the challenges of borderline resectable pancreatic cancer, the complexity of its management and controversies and point out where further research and international cooperation for a consensus strategy is urgently needed.