Pseudotumor retroperitoneal como apresentação de doença relacionada à IgG 4: relato de caso / Retroperitoneal pseudotumor as a presentation of IgG4-related disease: a case report

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.

Spontaneous gas in a retroperitoneal mass: check the testis!

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.

Recurrence of retroperitoneal localized perivascular epithelioid cell tumor two years after initial diagnosis: case report

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.

Schwannomas mediastinal y retroperitoneal metacrónicos / Metachronous mediastinal and retroperitoneal schwannomas

Los schwannomas, también llamados neurilemomas, son tumores originados en la vaina de los nervios periféricos. El 45% ocurren en cabeza y cuello, solo el 9% en mediastino y el 0,7-2,7% en retroperitoneo. La multicentricidad es extremadamente rara. Presentamos el caso de un paciente de 30 años que consultó por dolor torácico derecho asociado a derrame pleural, al que se le diagnosticó un schwannoma mediastinal posterior, el cual fue resecado en forma completa por cirugía videoasistida. A los 3 años, vuelve a consultar por dolor en flanco derecho y, al estudiarlo, se constata un nuevo schwannoma de localización retroperitoneal, que se resecó por vía abierta dada su posición retrocava. Describimos la metodología de estudio de esta neoplasia y los hallazgos histopatológicos que demostraron su benignidad. Conclusión: es una patología muy infrecuente pero con excelente pronóstico posoperatorio si la resección quirúrgica es completa.

Schwannomas, also known as neurilemmoma, are neurogenic tumors that arise from the peripheral nerve sheaths. Forty-five percent of schwannomas occur in the head and neck, 9% in the mediastinum and 0.7-2.7% in the retroperitoneum. Multiple shwannomas are extremely rare. We report the case of a 30-year old male patient with chest pain in the right hemithorax associated with pleural effusion due to schwannoma of the posterior mediastinum that was completely resected with video-assisted thoracoscopy. Three years later, he presented pain on the right lumbar region due to a retroperitoneal schwannoma behind the vena cava that was completely removed with open surgery. We describe the tests used to evaluate this tumor and the histopathological findings confirming its benign nature. Conclusion: Schwannoma is a rare condition with excellent postoperative outcome after complete surgical resection.

Linfangioma quístico retroperitoneal: Un tumor infrecuente en la edad adulta / Retroperitoneal cystic lymphangioma: A rare tumor in adulthood

Los linfangiomas son tumores benignos raros del sistema linfático, más comunes en la población pediátrica. La localización intraabdominal es muy infrecuente: tan solo el 1% de los linfangiomas aparece en el retroperitoneo. Se describe una tumoración quística infrecuente tanto por su localización como por la edad de presentación. Los linfangiomas retroperitoneales son tumores raros y su etiopatogenia es incierta. El diagnóstico se realiza mediante estudios de imagen; las técnicas de elección son la ultrasonografía (USG), la resonancia magnética (RM) o la tomografía computarizada (TC). A pesar de que se trata de tumores de naturaleza benigna, la exéresis quirúrgica completa es el tratamiento de elección para prevenir complicaciones como la sobreinfección, la rotura o el sangrado. Nuestro caso resulta infrecuente tanto por la localización retroperitoneal del tumor como por la tardía edad de presentación.

Background: lymphangiomas are rare benign tumors of the lymphatic system, being more common in the pediatric population. Intra-abdominal localization is very rare; only 1 % of lymphangiomas appear in the retroperitoneum. We report a case of a rare tumor because of its location and the elderly age of presentation. Retroperitoneal lymphangioma is a rare tumor with an unertain pathogenesis. Diagnosis is usually confirmed by imaging studies , e.g., US, MRI or CT. Although they are benign tumors, complete surgical resection is the treatment of choice. With this treatment, complications like infection, perforation or bleeding are prevented. Our case is unusual because of the retroperitoneal location of the tumor and the late age of presentation.

Extensive renal sinus lipomatosis in xanthogranulomatous pyelonephritis simulating liposarcoma

ABSTRACT Renal replacement lipomatosis is a condition characterized by varying degrees of renal parenchymal atrophy and perirenal fibrofatty proliferation secondary to chronic inflammation such as xanthogranulomatous pyelonephritis. In severe cases, imaging findings can be misdiagnosed as retroperitoneal liposarcoma.

Liposarcoma retroperitoneal gigante: a propósito de un caso / Giant retroperitoneal liposarcoma:a case report

Paciente masculino de 48 años con antecedente de hipertensión, que consulta por dolor abdominal difuso continuo, anorexia y aumento progresivo del perímetro abdominal. Al examen por TC se observa importante formación sólida heterogénea de densidad predominantemente grasa, que ocupa la casi totalidad del abdomen con desplazamiento de estructuras del abdomen superior; luego de la administración de medio de contraste formación presenta además componente de densidad de partes blandas de aspecto vegetante con realce heterogéneo que infiltra el retroperitoneo. Luego de su recesión completa, se concluye con el diagnostico de liposarcoma.

48 years old male patient with a history of hypertension, that consultation by diffuse abdominal pain continued, anorexia and progressive increase in the abdominal circumference. To review by TC is important training solid heterogeneous density predominantly fat that occupies almost the whole of the abdomen with displacement of structures in the upper abdomen, after the administration of contrast medium training is also a component of soft tissue density of vegetative aspect with heterogeneous enhancement that infiltrates the retroperitoneum, after his complete recession, it is concluded with the diagnosis of liposarcoma.

Tumor fibroso solitario y síndrome Doege-Potter / Solitary fibrous tumor and Doege-Potter syndrome

El tumor fibroso solitario es raro. Si bien su localización más frecuente es la pleura, han sido informados algunos casos de ubicación retroperitoneal. Es difícil diferenciarlo imagenológicamente de otras neoplasias, por lo que casi siempre el diagnóstico es histológico. Dado que los tumores fibrosos solitarios tienen presentaciones clínicas muy diversas, una mejor compresión de la ubicación y sus características imagenológicas ayudaría a abreviar la lista de diagnósticos diferenciales. Comunicamos un tumor fibroso solitario de localización retroperitoneal cuyo diagnóstico fue confirmado por examen histológico.

The solitary fibrous tumor is uncommon. Even though it frequently locates in the pleura, a few cases have been reported in the retroperitoneum. Differentiation from other neoplasms is difficult, and therefore the diagnosis is always attained through histological examination. Although solitary fibrous tumors have variable clinical behaviors, a better comprehension of the location and its imaging characteristics would help to decrease the list of differential diagnoses. We report a solitary fibrous tumor located in the retroperitoneum whose diagnosis was confirmed by histological examination.

Liposarcoma retroperitoneal complicado: a propósito de un caso / Complicated retroperitoneal liposarcoma: a case report

Resumen Las neoplasias retroperitoneales suponen un reto para el profesional sanitario, tanto en el diagnóstico como en el tratamiento. Caso clínico: Varón de 56 años diabético tipo 2. Acude por edema en extremidad inferior derecha y parestesias. Se realiza ecografía doppler con resultados de trombosis venosa profunda y conglomerado adenopático. En TAC abdominal se observa gran masa retroperitoneal que engloba paquete vasculonervioso iliaco derecho. Se realiza vaciamiento ganglionar con resultado inicial de hamartoma. Se practica cirugía de resección de la masa y ligadura de la vena iliaca externa trombosada preservando la arteria iliaca. Anatomía patológica intraoperatoria sugestiva de sarcoma y definitiva de liposarcoma. Reingresa por shock hipovolémico y rotura de la arteria iliaca derecha. Se realiza ligadura y bypass femoro-femoral izquierdo-derecho. Al alta es derivado a oncología, falleciendo a los 6 meses. Discusión: El sarcoma es el tumor primario más frecuente a nivel retroperitoneal (15%). De difícil diagnóstico y tardío tratamiento, suelen ser asintomáticos hasta que debutan como una masa y clínica de compresión local o afectación de estructuras vasculonerviosas. El TAC es la prueba diagnóstica de elección. El tratamiento del tumor localizado es la cirugía; la radioterapia y quimioterapia se reservan para tumores irresecables o de alto grado.

Abstract Retroperitoneal neoplasias may be a diagnostic and therapeutic challenge for the clinician. Case report: A 56 year old male with prior history of type 2 diabetes consults with right inferior limb edema and paresthesias. He was diagnosed of deep vein thrombosis and lymph node conglomerate by ultrasound. A CT scan was performed, observing great retro peritoneal mass enclosing vascular and nervous structures. An initial histological diagnosis of hamartoma was made. Surgery was scheduled to remove the tumour, ligation of thrombosed external iliac vein and the external iliac artery was preserved. Intraoperative histological findings suggested sarcoma, definite studies showed presence of high grade liposarcoma. Two weeks after the intervention, the patient presents in hypovolemic shock due to rupture of the right external iliac artery. Emergent ligation of the artery and femorofemoral bypass was performed, with adequate postoperatory recovery. The patient was discharged and continued adjuvant oncology treatment and was exitus six months later. Discussion: Sarcomas are the most frequent primary retroperitoneal tumours. They are difficult to diagnose and often have untimely treatment. Sarcomas tend to be asymptomatic, or present with local compression symptoms, affecting vascular and nervous structures. CT scan is the gold standard for diagnostic imaging. Treatment requires a multidisciplinary approach, surgical resection as the main therapy; radio and chemotherapy represents a solution for irresectable or high grade malignancies.

Leiomioma retroperitoneal: reporte de un caso / Retroperitoneal leiomyoma: report of a case

Introducción: El mioma retroperitoneal es un tumor de músculo liso con baja incidencia, de etiología variada, siendo lo más frecuente debido a una larga exposición a estrógenos. Presentación del caso: Mujer de 52 años, con antecedentes de diabetes mellitus tipo 1 e hipotiroidismo, consultó por irregularidad menstrual de 1 año de evolución. Al examen físico destacó una masa abdominal palpable y al tacto rectal abombamiento del fondo de saco posterior. Una ecografía transvaginal mostró una lesión sólida retro-uterina de 90x67x79mm por lo que se planteó como hipótesis diagnóstica un tumor pélvico, sin poder descartar origen anexial. Exámenes de laboratorio resultaron normales con marcadores tumorales negativos. Durante el intraoperatorio se identificaron anexos sanos y una masa retroperitoneal por lo que se decidió realizar tumerectomía y anexectomía izquierda por riesgo de bridas. La biopsia resultó compatible con neurilemoma, pero mediante inmunohistoquímica se confirmó diagnóstico de leiomioma. Cursó postoperatorio en buenas condiciones. Discusión: El leiomioma retroperitoneal es un diagnóstico diferencial de tumor retroperitoneal, a pesar de su baja incidencia y menor frecuencia que una tumoración maligna. Sus presentaciones clínicas más frecuentes son como masa abdominal palpable, distensión abdominal o asintomática. Actualmente ningún examen de laboratorio o imagenológico, ha demostrado una alta precisión para discriminar entre un tumor maligno y uno benigno, por lo cual, su confirmación sigue dependiendo de la biopsia excisional. La probabilidad de recurrencia es baja, sin embargo, si esta se presenta, puede deberse a un tumor maligno, por lo que es recomendable un adecuado seguimiento clínico e imagenológico de estas pacientes.

Introduction: The retroperitoneal myoma is a smooth muscle tumor with a low incidence, of varied etiology, being the most frequent due to a long estrogen exposure. Case report: Woman, 52 years old, with a history of diabetes mellitus type 1 and hypothyroidism who consulted for menstrual irregularity of 1 year of evolution. The physical examination revealed a palpable abdominal mass and rectal touch of the posterior recumbent sac. A transvaginal ultrasound showed a solid retro-uterine lesion of 90x67x79mm, so it was diagnosed a pelvic tumor, without being able to rule out adnexal origin. Laboratory tests were normal with negative tumor markers. During the intraoperative period, were identified healthy appendages and a retroperitoneal mass, so it was decided to perform tumerectomy and left annexectomy for the risk of flanges. The biopsy was compatible with neurilemoma, but by means of immunohistochemistry a diagnosis of leiomyoma was confirmed. Came postoperative in good condition. Discussion: Retroperitoneal leiomyoma is a differential diagnosis of retroperitoneal tumor, despite its low incidence and less frequent than a malignant tumor. Its most frequent clinical presentations are palpable abdominal mass, abdominal distension or asymptomatic. At present, no laboratory or imaging examination has shown a high precision to discriminate between a malignant tumor and a benign one, so that its confirmation still depends on the excisional biopsy. The probability of recurrence is low, however, if it is present, it may be due to a malignant tumor, which is why it is advisable to have adequate clinical and imaging follow-up of these patients

Retroperitoneal Hygroma

ABSTRACT :We present a 46-year-old white male with a retroperitoneal hygroma protruding from the right flank

Thoraco-abdominal mature teratoma in a left diaphragmatic hernia.

This case describes a contiguous mediastinal and retroperitoneal mature teratoma in a congenital diaphragmatic defect, a combination that is hitherto unreported in literature. It substantiates embryological chronology of events during the cephalad migration of the primordial germ cells through the developing diaphragm, prior to its closure.

[Clinical manifestation of retroperitoneal tuberculosis as psuedotumor in an 18-year-old female]

The case was an 18-year-old female with progressive crampy abdominal pain, nausea and vomiting, initiated from 4 months ago, Who had 4-5 Kg weight loss in this period. In physical examination, an ill-defined mass with mild tenderness in periumblical region palpation was detected. In upper GI endoscopy, bulging folds with a necrotic mass in D2- D3 were detected. In small bowel series study, cut off of barium in D2-D3 portions was seen. For better evaluation spiral CT was performedin which a large mass with extension to small bowel in retroperitoneum was seen. In Pathologic study, classification granuloma compatible with tuberculosis was reported. This finding was confirmed with Ziel Neelsen staining and PCR

Pararenal Leiomyosarcoma of the Inferior Vena Cava

A complete surgical resection is the only proven therapeutic modality that prolongs the survival in patients with leiomyosarcoma of the inferior vena cava (IVC). Reconstruction of the IVC is not always necessary but is often required to facilitate venous drainage of the kidney for the tumors at the pararenal area of the IVC. Controversy exists in postoperative adjuvant therapy. Recently, we experienced four cases of pararenal leiomyosarcoma of the IVC, of which treatment consisted of a complete resection of the tumor, ringed polytetrafluoroethylene (PTFE) graft interposition, and bilateral renal vein reconstructions in all patients. Postoperative radiation therapy was instituted in 3 of 4 patients. One patient who did not receive the postoperative radiation therapy was treated with adjuvant chemotherapy. The kidneys were preserved in all patients and no deep vein thrombosis (DVT) or venous insufficiency of the lower extremity veins developed. Distant metastasis to the lung was noted in one patient at 18 months after surgery, who was not received the postoperative radiation therapy but chemotherapy. In conclusion, a complete resection of the tumor, IVC reconstruction, and bilateral renal vein reconstruction followed by adjuvant radiation therapy is recommended for the treatment of pararenal leiomyosarcoma of the IVC.

Retroperitoneal Malignant Mesenchymoma: A Case of Mesenchymal Mixed Tumor with Osteosarcoma, Leiomyosarcoma, Liposarcoma and Fibrosarcoma

Malignant mesenchymoma is an interesting but very rare tumor in which malignant differentiation has occurred twice or more. We report a case of retroperitoneal malignant mesenchymoma consisting of osteosarcoma, leiomyosarcoma, liposarcoma and fibrosarcoma. Abdominal CT showed a large retroperitoneal mass with two separate and distinct parts, namely an area of prominent calcification and one of clearly enhancing solid components. The mass contained histologically distinct tumorous components with no histologic admixure at the interfaces. The densely calcified nodule corresponded to osteosarcoma, and the noncalcified clearly enhancing nodules to leiomyosarcoma, liposarcoma and fibrosarcoma.