Abstract:Primary renal extra-skeletal Ewing sarcoma is a rare neoplasm, often metastatic at diagnosis, and with a poor outcome. A multimodal approach is often the treatment of choice in this aggressive neoplasm. We present a case of primary renal extra-skeletal sarcoma in a 45-year-old woman who underwent tumor resection without clear margins. After no response to the first cycle of chemotherapy, we documented an early onset of local recurrence. The patient refused any other treatment and died four months after surgery. Show more
“…La quimioterapia se considera uno de los métodos más eficaces para tratar los tumores con metástasis. No obstante, se ha descrito que esta solo es efectiva durante los primeros ciclos, ya que los tumores desarrollan resistencia muy rápidamente (10)(11)(12)(13)(14)(15) . En el caso de los TNPP que aparecen desde tumores de células germi-nales, algunos autores recomiendan el uso de quimioterapia basada en bleomicina, etopósido y cisplatino (16) .…”
El tumor neuroectodérmico primitivo del ovario es un sarcoma de tejido blando de células redondas pequeñas, raro y agresivo, de origen neural que generalmente se asocia con una alta morbilidad y mortalidad. La inmunohistoquímica es un complemento útil en el diagnóstico diferencial. Se describe un caso de tumor neuroectodérmico primitivo del ovario en paciente nulípara de 21 años que refería dolor y aumento de la circunferencia abdominal. La ecografía mostró tumoración de contenido heterogéneo sólido-quística que aparentemente se originaba del anexo izquierdo. La resonancia magnética confirmó la presencia de tumoración que se extendía hacia la fosa iliaca izquierda sin afectación de órganos locales ni metástasis regionales o a distancia. Los marcadores tumorales estaban todos dentro del rango normal. Durante la laparotomía se observó tumoración de ovario izquierdo con ovario derecho normal. Se realizó salpingoforectomía izquierda debido al tamaño del tumor, resección en cuña de ovario derecho, linfadenectomía pélvica y omentectomía. El examen histopatológico reveló tumor compuesto por láminas de células redondas. Las células tumorales fueron positivas para cromogranina A, sinatrofisina, vimentina y enolasa específica de neuronas, lo que confirmó el diagnóstico de tumor neuroendocrino primitivo de ovario izquierdo, que se originaba de teratoma quístico inmaduro. La paciente rechazó la quimioterapia postoperatoria.
“…La quimioterapia se considera uno de los métodos más eficaces para tratar los tumores con metástasis. No obstante, se ha descrito que esta solo es efectiva durante los primeros ciclos, ya que los tumores desarrollan resistencia muy rápidamente (10)(11)(12)(13)(14)(15) . En el caso de los TNPP que aparecen desde tumores de células germi-nales, algunos autores recomiendan el uso de quimioterapia basada en bleomicina, etopósido y cisplatino (16) .…”
El tumor neuroectodérmico primitivo del ovario es un sarcoma de tejido blando de células redondas pequeñas, raro y agresivo, de origen neural que generalmente se asocia con una alta morbilidad y mortalidad. La inmunohistoquímica es un complemento útil en el diagnóstico diferencial. Se describe un caso de tumor neuroectodérmico primitivo del ovario en paciente nulípara de 21 años que refería dolor y aumento de la circunferencia abdominal. La ecografía mostró tumoración de contenido heterogéneo sólido-quística que aparentemente se originaba del anexo izquierdo. La resonancia magnética confirmó la presencia de tumoración que se extendía hacia la fosa iliaca izquierda sin afectación de órganos locales ni metástasis regionales o a distancia. Los marcadores tumorales estaban todos dentro del rango normal. Durante la laparotomía se observó tumoración de ovario izquierdo con ovario derecho normal. Se realizó salpingoforectomía izquierda debido al tamaño del tumor, resección en cuña de ovario derecho, linfadenectomía pélvica y omentectomía. El examen histopatológico reveló tumor compuesto por láminas de células redondas. Las células tumorales fueron positivas para cromogranina A, sinatrofisina, vimentina y enolasa específica de neuronas, lo que confirmó el diagnóstico de tumor neuroendocrino primitivo de ovario izquierdo, que se originaba de teratoma quístico inmaduro. La paciente rechazó la quimioterapia postoperatoria.
“…Ewing sarcoma is a poorly differentiated and highly malignant tumor that usually arises in bone and soft tissue [ 1 , 2 ]. The presence of small round blue cells is characteristic of this tumor as well as the rearrangement of the ESWR1 gene on chromosome 22 [ 3 ]. It is most commonly diagnosed in young adults and adolescents with a peak incidence at 15 years of age [ 1 ].…”
Section: Introductionmentioning
confidence: 99%
“…Diagnosis can be achieved through radiological modalities such as magnetic resonance imaging (MRI) and computed tomography (CT) scans which help identify tumor size, location, and extent of distant or local metastasis [ 3 ]. Histopathology coupled with immunohistochemistry (IHC) and cytogenic studies are also vital to make a definitive diagnosis of Ewing sarcoma [ 5 ].…”
Section: Introductionmentioning
confidence: 99%
“…However, ESK is associated with a poor prognosis with a survival of less than one year [ 7 ]. The poor prognosis is attributed to factors such as delayed diagnosis, large size, aggressive nature, and high risk of metastasis [ 3 ].…”
Primary Ewing sarcoma of the kidney (ESK) is a rare and aggressive entity, with a poor prognosis. It often presents as metastatic disease with the lungs being the most common site. In adults, the occurrence of these tumors is uncommon, with patients exhibiting non-specific symptoms such as weight loss, flank pain, hematuria, and an abdominal mass. The combination of these vague clinical symptoms and the rarity of these tumors often results in a delayed diagnosis, leading to poorer outcomes for these patients. We present a case of a 38-year-old female with metastatic ESK. The patient initially presented with abdominal pain, vomiting, and a four-day history of constipation. The diagnosis was confirmed through computed tomography scans, ultrasound-guided biopsy of the lesion, and fluorescence in situ hybridization that revealed translocation of the
EWS
gene on chromosome 22q12. She was managed with chemotherapy regimens and palliative care; however, the disease progressed and she passed away six months after her initial diagnosis.
“…Primary renal extraskeletal Ewing sarcoma is an another rare renal mesenchymal neoplasm, often metastatic at diagnosis, and with a poor outcome [ 10 ].…”
Renal leiomyosarcoma is a rare, aggressive tumor of the smooth muscles of the kidney. In our case, the tumor has special characteristics that made it highly challenging, as it involved major vessels and other adjacent vital structures. The rarity of the tumor type itself and the special challenging features we faced intraoperatively encouraged us to report the case including the management plan for R0 resection. Our patient is a forty-two years old previously healthy female, with vague nonspecific presenting complains, ended up with a major highly advanced surgery necessitating the need for vascular reconstruction of IVC. The surgery was performed by a multidisciplinary team of highly specialized surgeons in related fields. The surgery went well, and the outcome was promising. The patient was followed up for about four months later, with uneventful course.
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