RESUMEN

Primary retroperitoneal mucinous cystadenocarcinomas (PRMCs) are extremely rare tumors with limited understanding of their pathogenesis and biological behavior. We describe a case of a 50-year-old female patient who underwent surgical treatment. The patient had a history of previous surgeries for mesenteric mucinous cystadenoma, without evidence of recurrence. During routine abdominal ultrasound a new tumor was found. An abdomen magnetic resonance imaging was done and confirmed the presence of a cystic lesion in the right iliac fossa. After discussion in multidisciplinary committee, surgical complete resection of the tumor, along with bilateral adnexectomy, was performed successfully. Histopathological examination revealed a mucinous adenocarcinoma adjacent to a mucinous cystadenoma. Immunohistochemical analysis supported the diagnosis of a primary retroperitoneal lesion. The patient had an uneventful recovery and has remained disease-free during the two-year postoperative follow-up. PRMCs are challenging to diagnose preoperatively due to nonspecific symptoms. Surgical excision is the mainstay of treatment. The long-term prognosis and optimal therapeutic strategies require further investigation.

Los cistoadenocarcinomas mucinosos primarios retroperitoneales (CMPR) son tumores extremadamente raros con una comprensión limitada de su patogénesis y comportamiento biológico. Describimos el caso de una mujer de 50 años sometida a tratamiento quirúrgico. La paciente tenía antecedentes de cirugías previas por cistodenoma mucinoso mesentérico, sin evidencia de recurrencia. Durante una ecografía abdominal de rutina se encontró un nuevo tumor. Se realizó una resonancia magnética abdomen que confirmó la presencia de una lesión quística en la fosa ilíaca derecha. Luego de discutir el caso en el comité multidisciplinario, se realizó con éxito la resección quirúrgica completa del tumor, junto con la anexectomía bilateral. El examen histopatológico reveló un adenocarcinoma mucinoso adyacente a un cistodenoma mucinoso. El análisis inmunohistoquímico apoyó el diagnóstico de lesión primaria retroperitoneal. La paciente tuvo una buena recuperación y permaneció libre de enfermedad durante dos años de seguimiento postoperatorio. Los CMPR son difíciles de diagnosticar debido a que presentan síntomas inespecíficos. La escisión quirúrgica es la base del tratamiento. El pronóstico a largo plazo y las estrategias terapéuticas óptimas requieren más investigación.

Asunto(s)

RESUMEN

Introduction: schwannomas are benign and common soft tissue tumors. They are usually asymptomatic and are discovered for other reasons. Materials: we present the case of an 82-year-old male patient with a recent diagnosis of moderately differentiated adenocarcinoma of the colon and a hypermetabolic periaortic nodule as an incidental finding. Results: percutaneous biopsy of the periaortic nodule confirmed the diagnosis of schwannoma. At one year of follow-up, growth of the schwannoma has been demonstrated. There are no signs of progression of his oncological disease. Conclusions: schwannomas are benign tumors, rarely found in the retroperitoneum and can be sources of false-positive positron emission tomography results.

Introducción: los schwannomas son tumores benignos y frecuentes de las partes blandas. Habitualmente son asintomáticos y son descubiertos por otros motivos. Materiales y métodos: presentamos el caso de un paciente masculino de 82 años con diagnóstico reciente de adenocarcinoma de colon moderadamente diferenciado y con un nódulo periaórtico hipermetabólico como hallazgo incidental. Resultados: la biopsia percutánea del nódulo periaórtico confirmó el diagnóstico de schwannoma. Al año de seguimiento, se ha demostrado crecimiento del schwannoma. No hay signos de progresión de su enfermedad oncológica. Conclusión: los schwannomas son tumores benignos, infrecuentes en el retroperitoneo y pueden ser fuentes de resultados falsos positivos en tomografía por emisión de positrones.

Asunto(s)

RESUMEN

For pheochromocytoma and paraganglioma (PPGL), the efficacy of percutaneous ablative therapies in achieving control of metastatic tumors measuring <3 cm had been demonstrated in only few reports, and intraoperative radiofrequency ablation (RFA) of locally invasive primary PPGLs has not been reported. We presented the case of a 31-year-old man who had a 9-cm functioning unresectable PPGL. He was treated with 13 cycles of cytotoxic chemotherapy without objective tumor response, according to the Response Evaluation Criteria in Solid Tumors (RECIST). Subsequently, magnetic resonance imaging revealed a 9.0 × 8.6 × 6.0-cm retroperitoneal mass that extended to the inferior portion of the inferior vena cava, the inferior mesenteric artery, and the infrarenal aorta. Biochemical evaluation demonstrated high level of plasma normetanephrine (20.2 nmol/L, normal range <0.9 nmol/L). Genetic investigation showed the germline pathogenic variant c.1591delC (p. Ser198Alafs*22) in the SDHB gene. I131-metaiodobenzylguanidine scintigraphy was negative and Ga68-dotatate PET-CT scan showed high tumor uptake without distant metastases. On open laparotomy, tumor debulking was not possible. Therefore, intraoperative RFA was performed by a highly experienced team of interventional radiologists. At 12 months after the RFA, the tumor volume decreased from 208 to 45 mL (78%), plasma normetanephrine decreased from 20.2 to 2.6 nmol/L (87%), and the doxazosin dose was reduced from 16 to 8 mg/day. To our best knowledge, this was the first report on intraoperative RFA that markedly reduced the size of a large primary unresectable PPGL, along with clinical and biochemical responses.

Asunto(s)

RESUMEN

Presentamos el caso de un paciente masculino de 57 años con un leiomiosarcoma retroperitoneal encontrado de manera incidental. Es una patología poco común, la incidencia anual es 2.7 casos por millón de personas. De igual manera, es una enfermedad de evolución silenciosa, por lo que pasa inadvertida hasta que la masa tumoral alcanza un gran tamaño. Los leiomiosarcomas se evidencian como extensas áreas de heterogeneidad y realce heterogéneo, ocasionado por la presencia de necrosis y focos hemorrágicos. Normalmente no hay presencia de calcificaciones y hay ausencia de tejido adiposo, de esta manera que una gran masa retroperitoneal mayor a 10 cm sin contenido graso y necrosis interna variable debe sugerir la posibilidad de un leiomiosarcoma. La eliminación quirúrgica completa es el tratamiento de elección, ésta se debe realizar con márgenes negativos amplios, factor que repercute en el pronóstico, ya que estos tumores tienden a alcanzar un tamaño muy grande y, a menudo, la extirpación amplia es imposible, lo que afecta la supervivencia del paciente. Este tipo de neoplasias tienen un mal pronóstico, pues se sabe que la supervivencia a los 5 años es de aproximadamente 15%, debido a su alta tendencia a la invasión local, metástasis o diagnóstico tardío.

We present the case of a 57-year-old male patient with a retroperitoneal leiomyosarcoma found incidentally. It is a rare disease, with an annual incidence of 2.7 cases per million people. Likewise, it is a disease with a silent evolution, so it goes unnoticed until the tumor mass reaches a large size. Leiomyosarcomas appear as extensive areas of heterogeneity and heterogeneous enhancement, caused by the presence of necrosis and hemorrhagic foci. Normally, there is no presence of calcifications and there is an absence of adipose tissue, so a large retroperitoneal mass greater than 10 cm with no fat content and variable internal necrosis should suggest the possibility of a leiomyosarcoma. Complete surgical removal is the treatment of choice, and should be performed with wide negative margins, a factor that affects the prognosis, since these tumors tend to reach a very large size and wide excision is often impossible, which affects the patient's survival. This type of neoplasm has a poor prognosis, since it is known that the 5-year survival rate is approximately 15%, due to its high tendency to local invasion, metastasis or late diagnosis.

Apresentamos o caso de um paciente do sexo masculino, 57 anos, comleiomiossarcoma retroperitoneal encontrado incidentalmente. É umapatologia rara, a incidência anual é de 2,7 casos por milhão de pessoas. Da mesma forma, é umadoença de evolução silenciosa, por issopassadespercebida até que a massa tumoral atinja umtamanho grande. Os leiomiossarcomasaparecem como extensas áreas de heterogeneidade e realce heterogêneo, causadas pela presença de necrose e focos hemorrágicos. Normalmente nãohápresença de calcificações e háausência de tecido adiposo, portantouma grande massa retroperitoneal maior que 10 cm semconteúdo de gordura e necrose interna variáveldeve indicar a possibilidade de leiomiossarcoma. A remoçãocirúrgica completa é o tratamento de escolha, este deve ser realizado com amplas margens negativas, fator que afeta o prognóstico, poisesses tumores tendem a atingir tamanhomuito grande e, muitasvezes, a remoção ampla é impossível, o que prejudica a sobrevida do paciente. Esse tipo de neoplasia apresentamauprognóstico, pois sabe-se que a sobrevida em 5 anos é de aproximadamente 15%, devido à sua alta tendência à invasão local, metástaseou diagnóstico tardio.

Asunto(s)

RESUMEN

Introducción: Los schwannomas suelen ser tumores benignos con origen en el sistema neural que sostiene la célula de Schwann. La mayoría presentan síntomas abdominales inespecíficos. Dado su escasa frecuencia, 1-5% de todos los tumores retroperitoneales en la población mundial, se presenta el siguiente caso. Pueden tener múltiples localizaciones, como el páncreas, tracto gastrointestinal y espacio retroperitoneal. Son pocos los casos que se han reportado siendo apenas notificados menos de 150 casos a la actualidad. Caso Clínico: Paciente femenina de 33 años con dolor abdominal inespecífico. Niega antecedentes relevantes y no presenta hallazgos positivos al examen físico. Estudios complementarios con tomografía y resonancia magnética nuclear de abdomen contrastados mostraron una masa retroperitoneal quística de 5x4 cm de localización suprarrenal derecha, que desplazaba la cava retrohepatica y que realza con medio contraste intravenoso. Debido a que el dolor era incapacitante se realizó resección quirúrgica. No se consideró biopsia preoperatoria, ante el riesgo de ruptura y diseminación tumoral. Se realizó escisión transperitoneal con resección completa, técnicamente compleja por su localización. El reporte histopato-lógico fue Schwannoma, sin tejido supra-adrenal, sugiriendo lesión yuxta-adrenal.

Introduction: Schwannomas are usually benign tumors originating in the neural system that supports the Schwann cell. Most have nonspecific abdominal symptoms. Given its low frequency, 1-5% of all retroperitoneal tumors in the world population, the following case is presented. They can have multiple locations, such as the pancreas, gastrointestinal tract and retroperitoneal space. Few cases have been reported, with less than 150 cases reported to date. Clinical Case: 33-year-old female patient with nonspecific abdominal pain. She denies relevant history and has no positive findings on physical examination. Complementary studies with contrast-enhanced tomography and magnetic resonance imaging of the abdomen showed a 5x4 cm cystic retroperitoneal mass located in the right suprarenal region, displacing the retrohepatic vena cava and enhancing with intravenous contrast medium. Because the pain was incapacitating, surgical resection was performed. A preoperative biopsy was not considered, given the risk of rupture and tumor dissemination. Transperitoneal excision was performed with complete resection, technically complex due to its location. The histopathological report was Schwannoma, without supra-adrenal tissue, suggesting a juxta-adrenal lesion.

Asunto(s)

RESUMEN

Retroperitoneal liposarcomas are very infrequent retroperitoneal malignant tumours. Most patients complain of palpable abdominal mass and only half present with abdominal pain. With haematogenous spread they can reach, mainly, lungs and liver, but only in 10% of cases. Here we report the presentation and surgical treatment of a giant massive retroperitoneal liposarcoma in a 53-year-old man.

Asunto(s)

RESUMEN

Retroperitoneal liposarcomas are very infrequent retroperitoneal malignant tumours. Most patients complain of palpable abdominal mass and only half present with abdominal pain. With haematogenous spread they can reach, mainly, lungs and liver, but only in 10% of cases. Here we report the presentation and surgical treatment of a giant massive retroperitoneal liposarcoma in a 53-year-old man.

Asunto(s)

Asunto(s)

Asunto(s)

RESUMEN

Retroperitoneal lipomas are extremely rare with few cases reported so far in the literature. They can reach different sizes and present with a variety of symptoms. The differential diagnosis is mainly with well-differentiated liposarcoma (WDLPS). We present a 34-year-old woman with a retroperitoneal lipoma herniating through the inguinal canal into the proximal thigh. The patient underwent complete oncological resection using a Karakousis's abdominoinguinal incision. Retroperitoneal lipomas are a very rare condition and sometimes require resections technically challenging. MDM2 amplification is critical for its differential diagnosis with WDLPS.

Asunto(s)

RESUMEN

Complete surgical resection of soft-tissue sarcomas (STSs) frequently requires complex multivisceral resections, which is the most important factor related to overall survival and local recurrence. Major vascular involvement is not rare in patients with primary retroperitoneal STSs. We present a 54-year-old woman with a retroperitoneal STS encasing the infrarenal abdominal aorta. The patient underwent complete oncological resection with vascular reconstruction using a bi-iliac vascular graft. Major blood vessel involvement is not a contraindication for STS surgery with a curative intent.

Asunto(s)

Asunto(s)

RESUMEN

OBJECTIVE: To report the case of a pregnant patient diagnosed with a primary retroperitoneal tumor and to conduct a review of the literature pertaining to the diagnosis, treatment and maternal-fetal prognosis of this disease condition during pregnancy. METHODS: A 19-year-old patient who presented with a retroperitoneal tumor identified on ultrasound. The results of the percutaneous biopsy showed a benign tumor. A healthy neonate was delivered by cesarean section. Surgical resection was performed four months later, and histopathology showed a mature cystic teratoma. A search was conduced in the Medline via PubMed, Lilacs, SciELO and ScienceDirect databases using the terms "pregnancy," "neoplasms," and "retroperitoneal neoplasms," including case reports and case series of retroperitoneal tumors during pregnancy. Diagnosis, treatment and maternal-fetal prognosis were identified. RESULTS: Overall, 1658 titles were identified. Of these, 34 case reports and 1 case series met the inclusion criteria. Of the cases, 62.9 % were benign. Diagnosis was made as an incidental finding in 63 % of cases, and 77 % of the masses were identified on ultrasound. Percutaneous biopsy was used in 8 % of cases, including the case reported here. Surgical treatment was used in 88 % of cases usually after delivery. Maternal mortality occurred in 8.5 % of cases. Fetal prognosis was good in 65 % of the pregnancies. CONCLUSIONS: Despite being frequently benign, retroperitoneal tumors during gestation have a reserved maternal and fetal prognosis in a substantial number of cases. There is a need to assess the risks and benefits of percutaneous biopsy.

TITULO: TUMOR RETROPERITONEAL PRIMARIO DURANTE EL EMBARAZO: REPORTE DE UN CASO Y REVISIÓN DE LA LITERATURA. OBJETIVO: Reportar el caso de una paciente gestante con diagnóstico de tumor retroperitoneal primario y hacer revisión de la literatura del diagnóstico, tratamiento y pronóstico materno-fetal de esta entidad durante el embarazo. METODOS: Gestante de 19 años que consulta por tumor retroperitoneal identificado por ecografía, se realizó biopsia percutánea con resultado de tumor benigno; parto por cesárea con recién nacido sano. A los 4 meses se realizó resección quirúrgica, la histopatología mostró un teratoma quístico maduro. Se realizó búsqueda en las bases de datos de: Medline vía PubMed, Lilacs, SciELO y ScienceDirect, con los términos: "embarazo", "neoplasias" y "neoplasias retroperitoneales", incluyendo reportes y series de caso de tumores retroperitoneales en el embarazo. Se identificó el diagnóstico, tratamiento y pronóstico materno-fetal. RESULTADOS: Se identificaron 1.658 títulos, de los cuales 34 reportes de casos y una serie de casos cumplieron con los criterios de inclusión. El 62,9 % eran tumores benignos. El diagnóstico se hizo como hallazgo incidental en el 63 % de los casos, el ultrasonido identificó la masa en el 77 %, la biopsia percutánea se utilizó en el 8 % de los casos incluyendo el reportado. El tratamiento quirúrgico fue utilizado en el 88 % de los casos, generalmente después del parto. Hubo mortalidad materna en el 8,5 % de los casos. El pronóstico fetal fue bueno en el 65 % de las gestaciones. CONCLUSIONES: Los tumores retroperitoneales en la gestación, a pesar de ser principalmente benignos, tienen un pronóstico materno y fetal reservado en un importante número de casos. Se requiere evaluar los beneficios y riesgo de la biopsia percutánea.

Asunto(s)

Asunto(s)

Asunto(s)

RESUMEN

Apresentamos um caso de doença relacionada à IgG4 devido às suas várias formas de apresentação clínica e dificuldades diagnósticas, objetivando acrescentar à literatura científica essa apresentação atípica. Trata-se de um homem de 65 anos com dor abdominal, febre e sintomas urinários. Exames laboratoriais inocentes para processo infeccioso agudo. Em tomografia computadorizada de abdome, evidenciou-se pseudotumor em região retroperitoneal, envolvendo ureter, veia e artéria ilíacas. Foi submetido à ressecção cirúrgica, tendo sido confirmado o diagnóstico de doença relacionada à IgG4 pela imuno-histoquímica. Iniciou tratamento com glicocorticoide com melhora clínica importante, seguindo em acompanhamento ambulatorial.

This is a case report of IgG4-related disease because of its wide variety of clinical presentations and difficulties in diagnosis, aiming at adding this atypical presentation to scientific literature. It is a case of a 65-year-old man with abdominal pain, fever and urinary symptoms. Laboratory tests were naive for acute infectious processes. In a computed tomography of the abdomen, retroperitoneal pseudotumor involving ureter, and iliac vein and artery was found. The patient underwent surgical resection, and the diagnosis of IgG4-related disease was confirmed by immunohistochemistry. The treatment was begun with glucocorticoids, with important clinical improvement. He was followed in outpatient settings.

Asunto(s)

RESUMEN

ABSTRACT Testicular germ cell tumor is the most common cancer in 20-to 35-years-old men. There are known risk factors such as undescended testicle(s) and history of testicular cancer. Most lesions are germ cell tumors with two main subtypes: seminomas and non-seminomatous germ cell tumors.

Asunto(s)

RESUMEN

Testicular germ cell tumor is the most common cancer in 20-to 35-years-old men. There are known risk factors such as undescended testicle(s) and history of testicular cancer. Most lesions are germ cell tumors with two main subtypes: seminomas and non-seminomatous germ cell tumors.

Asunto(s)

RESUMEN

ABSTRACT CONTEXT: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Adjuvant radiotherapy and/or chemotherapy are administered according to the patient's clinical characteristics. CASE REPORT: A 42-year-old female patient was operated to treat a retroperitoneal mass. The diagnosis was established as PEComa with benign behavior. Two years after the diagnosis, chest and abdominal computed tomography scans showed intra-abdominal recurrence and lymphangioleiomyomatosis in the lung. Treatment with everolimus was started. The disease stabilized in the third month of treatment, according to the response evaluation criteria in solid tumors. CONCLUSION: PEComas are tumors with unpredictable behavior. Therefore, these patients require long-term follow-up, even in cases of correct diagnosis and benign PEComa.

Asunto(s)

RESUMEN

CONTEXT: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Adjuvant radiotherapy and/or chemotherapy are administered according to the patient's clinical characteristics. CASE REPORT: A 42-year-old female patient was operated to treat a retroperitoneal mass. The diagnosis was established as PEComa with benign behavior. Two years after the diagnosis, chest and abdominal computed tomography scans showed intra-abdominal recurrence and lymphangioleiomyomatosis in the lung. Treatment with everolimus was started. The disease stabilized in the third month of treatment, according to the response evaluation criteria in solid tumors. CONCLUSION: PEComas are tumors with unpredictable behavior. Therefore, these patients require long-term follow-up, even in cases of correct diagnosis and benign PEComa.

Asunto(s)