Primary retroperitoneal extraovarian granulosa cell tumor

Extraovarian granulosa cell tumors (GCTs) develop from ectopic gonadal tissue situated along the embryonal route of the genital ridge. Primary retroperitoneal tumors are extremely rare, with an incidence of 02% -06% and 80-85% probability of malignancy. Only eight such case reports have been published previously. We herein, report a rare case of extraovarian retroperitoneal GCT in a 55-year-old woman who presented with intermittent left lumbar region pain of one-year duration. She had a history of hysterectomy and bilateral salpingo-oophorectomy 8 years ago for uterine leiomyoma. Laparotomy revealed a retroperitoneal mass measuring 8cm x 10cm x 20cm in size, solid cystic with areas of necrosis and hemorrhage. The gross features, classical histopathology, and positive immunostaining of the retroperitoneal mass with inhibin, calretinin, PR, WT1 and immunonegativity for EMA were characteristic of adult-type GCT. Excluding any previous history of primary ovarian GCT in this patient, a de-novo retroperitoneal diagnosis was established.

Retroperitoneal liposarcoma in a nonagenarian

Retroperitoneal liposarcomas are rare tumors arising from the soft tissue of the retroperitoneum and are of mesenchymal cell origin. They can reach a large size prior to causing symptoms and generally have a poor prognosis. We present the case of a 93-year-old lady presenting with a large retroperitoneal liposarcoma at the site of a previous colonic anastomosis for the adenocarcinoma treatment. It caused minimal symptoms initially, but surgical resection was undertaken when the tumor was found to be growing significantly in size. However, due to the tumor's location and its invasion into surrounding structures, the resection was not feasible and subsequently abandoned. A retroperitoneal liposarcoma arising from the site of a previous colonic resection has not been previously described. A review of the diagnosis and current management of these lesions is also given.

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.

Biopsia percutánea core con aguja gruesa guiada por tomografía computada en lesiones retroperitoneales: Experiencia de 10 años / CT-guided core biopsy for retroperitoneal lesions. Experience in 136 procedures

Background: CT-guided core biopsy is a widely used diagnostic technique for retroperitoneal lesions. Aim: To evaluate the diagnostic yield and safety of this procedure. Material and Methods: Review of medical records of 136 patients aged 57 ± 16 years (55% males) subjected to core biopsies performed between 2006 and 2016. Procedure images, biopsy reports and patients' medical charts were reviewed. Diagnostic yield was calculated in those patients whose final diagnosis was confirmed using strict criteria for malignancy. Results: A final diagnosis was confirmed in 122/136 patients. Of these, 110 had malignant lesions. The sensitivity and global accuracy of the procedure for malignancy were 93%. In only 4 of 13 benign lesions (31%), a specific diagnosis was obtained with the biopsy. Only minor complications were reported (6 small, self-contained hematomas). There were no major complications. Conclusions: CT-guided core biopsy of retroperitoneal lesions is a safe procedure, with an excellent diagnostic yield.

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.

Tumor miofibroblástico inflamatorio: presentación variable de una misma patología / Inflammatory myofibroblastic tumor: variable presentation of the same pathology

INTRODUCCIÓN: El tumor miofibroblástico inflamatorio (TMI) es una neoplasia benigna infrecuente, de comportamiento clínico impredecible. OBJETIVOS: describir 3 casos de TMI diagnosticados entre marzo 2014 y enero 2018 en Hospital Clinico San Borja Arriaran, y realizar una revisión actualizada de la literatura. CASO 1: Adolescente de género masculino de 14 años de edad, hospitalizado por dolor abdominal, diagnosticado de invaginación yeyunoyeyunal secundaria a un tumor de pared intestinal. La histología fue compatible con un tumor miofibroblástico inflamatorio. CASO 2: Adolescente de género femenino, edad 12 años, hospitalizada por neumonía y dolor lumbar en estudio asociado a pérdida de peso. Se diagnosticó una masa retroperitoneal que comprometía el músculo psoas derecho, músculos paravertebrales, vértebras, riñón derecho y diafragma ipsilateral. Se efectuó biopsia por punción cuyo resultado fue compatible con un tumor miofibroblástico inflamatorio. CASO 3: Preadolescente de género femenino de 11 años de edad, hospitalizada para estudio de infección del tracto urinario a repetición. Se identificó un tumor vesical y la biopsia mostró ser compatible con tumor miofibroblástico inflamatorio. CONCLUSIÓN: Debido al comportamiento variable del tumor miofibroblástico inflamatorio, el manejo de este dependerá de la localización, la expresión del anaplasic like lymphoma (ALK), el comportamiento del tumor y la posibilidad de resección.

INTRODUCTION: The inflammatory myofibroblastic tumor is an infrequent benign neoplasm with unpredictable cli nical behavior. OBJECTIVES: to describe three clinical cases at the San Borja Arriarán Clinical Hospital between March 2014 and January 2018 and to carry out an updated review of the literature. CASE 1: 14-year-old male adolescent, hospitalized due to abdominal pain, diagnosed with jejunojejunal intus susception secondary to an intestinal wall tumor. The histology was compatible with an inflamma tory myofibroblastic tumor. CASE 2: 12-year-old female adolescent, hospitalized due to pneumonia and low-back pain under study associated with weight loss. A retroperitoneal mass was diagnosed involving the right psoas muscle, paravertebral muscles, vertebrae, right kidney, and ipsilateral dia phragm. A puncture biopsy was performed and the result was compatible with an inflammatory myofibroblastic tumor. CASE 3: 11-year-old female pre-adolescent, hospitalized to study recurrent urinary tract infection. A bladder tumor was identified, and the biopsy showed compatibility with inflammatory myofibroblastic tumor. CONCLUSION: Due to the variable behavior of the inflammatory myofibroblastic tumor, its management will depend on the location, expression of the anaplastic lymphoma kinase (ALK), tumor behavior, and the resection possibility.

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.

Lipoblastoma retroperitoneal gigante: Caso dínico / Giant retroperitoneal lipoblastoma: Case report

El lipoblastoma forma parte de los tumores derivados del tejido adiposo. Es una neoplasia benigna que se puede presentar de forma localizada o difusa, que afecta a niños en la primera infancia y que, generalmente, se localiza en el tronco y las extremidades. Estos tumores son poco frecuentes en la edad pediátrica, pero cuando uno de ellos se presenta, constituye un desafío que se debe resolver rápidamente por el riesgo de malignidad que implican otras lesiones neoplásicas, de similares características. El diagnóstico preoperatorio es dificultoso, ya que los métodos por imágenes no aportan información específica que permita diferenciarlos de otros tumores, como los liposarcomas. El diagnóstico definitivo es anatomopalógico y, en casos dudosos, es necesaria la confirmación citogenética. Se presenta el caso de un niño de 14 meses de edad con diagnóstico de lipoblastoma de localización retroperitoneal de gran tamaño.

Lipoblastoma is part of tumors derived from adipose tissue. It is a benign neoplasm that can be localized or diffuse, affecting children in early childhood and usually located in the trunk and extremities. These tumors are uncommon in the pediatric age, but when one of them is present, it is a challenge that we must resolve quickly due to the risk of malignancy that involves other similar neoplastic lesions. The preoperative diagnosis is difficult, because the imaging methods do not provide specific information that allows us to differentiate them from other tumors, such as liposarcomas. The definitive diagnosis is anatomopathological and, in doubtful cases, cytogenetic confirmation is necessary. We present the case of a 14-month-old boy with diagnosis of large sized lipoblastoma of retroperitoneal localization.

Lipoblastoma retroperitoneal en un lactante: Caso clínico / Retroperitoneal lipoblastoma in an infant: A case report

Introducción El lipoblastoma es una neoplasia benigna del tejido adiposo, de presentación infrecuente y casi exclusiva en niños menores de 3 años. Usualmente se presenta en las extremidades como una masa indolora de crecimiento progresivo, estableciéndose su diagnóstico definitivo mediante análisis histológico y citogenético. Objetivo Presentar un caso clínico de lipoblastoma de ubicación inhabitual en una lactante y revisar la literatura al respecto. Caso clínico Lactante mayor de 16 meses, con aumento de volumen abdominal de 6 meses de evolución, asociado a una ingesta alimentaria disminuida, sin otros síntomas. El estudio de imagen reveló una imagen de aspecto lipoideo que comprometía casi la totalidad de la cavidad abdominal, muy sugerente de lipoblastoma, realizándose la resección de un tumor retroperitoneal de 18 cm de diámetro que rechazaba los órganos vecinos. El análisis histológico fue suficiente para confirmar el diagnóstico. En el seguimiento no hubo recidiva. Conclusión Tomando en cuenta la baja frecuencia de esta afección y su inusual presentación, se reporta el caso de esta paciente, para considerarlo dentro del diagnóstico diferencial de masa abdominal en un lactante.

Introduction Lipoblastoma is a benign neoplasia of the adipose tissue. It is a rare conditionand almost exclusively presents in children under 3 years old. It usually occurs in extremities as a painless volume increase of progressive growth, with the definitive diagnosis being established by pathological and cytogenetic analysis. The treatment of choice is complete resection, and follow-up period of up to five years is recommended due to a recurrence of up to 25%. Objective To present an unusual location of this uncommon condition in an infant, and review the related literature. Case report A sixteen-month child with an increase in abdominal growth of six-months progression, associated with a decreased food intake, and with no other symptoms. The imaging study revealed a lipoid-like image compromising almost the entire abdominal cavity, very suggestive of lipoblastoma. A resection was performed on an 18 cm diameter retroperitoneal tumour that rejected the adjacent organs. Histological analysis was enough to confirm diagnosis without the need for cytogenetic analysis. The follow-up showed no recurrence of the disease. Conclusion Given the rarity of this disease and its unusual presentation, we communicate this clinical case, in order to be considered in the differential diagnosis of abdominal mass in chilhood.

Occurrence of colonic liposarcoma after retroperitoneal liposarcoma

No abstract available.

Case Report: giant retroperitoneal presacral ancient schwannoma

Ancient schwannoma, is a rare variant of schwannoma with characterization of degenerative changes and diffuse hypercellularity. Retroperitoneal presacral form is often found incidentally, because they present with vague symptoms or symptomless. Schwannoma occurring in this area occasionally presents with enormous dimensions, known as a giant schwannoma. The tumor removal is a surgical challenge due to the difficult approach and abundant vascularity. In this report we describe a 61 year old female presented to ER with vaginal bleeding and lower abdominal pain. The case diagnosed on clinical, CT and MRI findings to be a fibroma of the left ovary. Exploration by the gynecology team revealed a huge retroperitoneal presacral tumor compressing the left external iliac vessels and displacing the left ureter; they took a biopsy and closed the abdomen. Histopathological result was benign schwannoma. The patient were referred to our hospital [Al Hada Armed Forces Hospital, Taif, Saudi Arabia] to be managed from postoperative DVT when her family asked our department of surgery for further management and signed a high risk consent. We explored the case after insertion of IVC filter and ureteric catheter. A 20x20 cm mass was thoroughly dissected and resected with part of sacrum. The final histopathological result was benign nerve sheath tumor with features consistent with degenerated [ancient] schwannoma and the tumor was completely resected. The patient was discharged from the hospital without complications and follow up for three years revealed no recurrence. The clinical, radiological, and pathological features of this disease are discussed in this report. To conclude, retroperitoneal giant ancient schwannomas are a rare variant of the benign schwannoma and often present as unrecognized slow growing masses. Keep in mind potentially severe bleeding and neurological deficit risk of surgical intervention without away from oncologic principle. Therefore, careful preoperative evaluations and postoperative monitoring are necessary

Retroperitoneal sclerosing PEComa with melanin pigmentation and granulomatous inflammation-A rare association within an uncommon tumor.

PEComa, defined as a perivascular epithelioid cell tumor, displays a wide clinicopathological spectrum. Lately, a sclerosing PEComa has been identified as its distinct variant, but with limited documentation, in view of its rarity. Herein, we describe an uncommon case of a 53-year-old lady, who was referred to us with pain abdomen. Radiological imaging disclosed a well-defined, hypodense retroperitoneal mass. The excised tumor was a round, encapsulated soft tissue mass measuring 7 cm with a tan-brown cut surface. Microscopy showed uniform, epithelioid cells with clear cytoplasm, focal melanin pigmentation and mild nuclear atypia, arranged in sheets and nests around capillary-sized vessels in a dense sclerotic stroma. Additionally, co-existing epithelioid granulomas were noted. On immunohistochemistry (IHC), tumor cells were diffusely positive for HMB45; focally for desmin and smooth muscle actin (SMA), while negative for EMA, CD10, S100-P, Melan A, CD34, AMACR and CK MNF116. This case reinforces sclerosing PEComa as an uncommon, but a distinct clinicopathological entity and exemplifies diagnostic challenge associated with it; necessitating application of IHC markers for its correct identification. Presence of melanin pigment and granulomatous inflammation in the present tumor constitute as novel histopathological findings in a sclerosing PEComa.

Benign giant retroperitoneal Schwannoma: a case report

Retroperitoneal Schwannoma, or Neurilemmoma, is a rare tumor that originates in the neural sheath from Schwann cells and accounts for only a small percentage of retroperitoneal tumors. Presentation is typically varied and non-specific, ranging from abdominal pain, abdominal mass or an incidental finding, and the diagnosis is quite often difficult, being confirmed by pathological study afterwards. We report a case of benign retroperitoneal Schwannoma which was incidentally noticed by the patient himself as an abdominal mass. Preoperative imaging studies were not helpful for reaching correct diagnosis. Surgical exploration and complete excision of tumor was successful. The histological diagnosis of the tumor was reported as benign Ancient Schwannoma. Postoperative period was uneventful and the patient was discharged with a partial femoral nerve injury characterized by mild antero-lateral thigh and leg anesthesia, and quadriceps muscle palsy: Medical Research Council Rating Score [MRC] - grade 2 muscle power. At ten weeks, his quadriceps weakness improved to MRC grade 3, and the anesthesia, although still present, is slightly improved

decade of clinical experience with extra-adrenal paragangliomas of retroperitoneum: report of 67 cases and a literature review

The purpose was to highlight the diagnosis and treatment of extra-adrenal para-gangliomas, which often causes catecholamine hypersecretion and hypertension. 67 cases of extra-adrenal paraganglioma of retroperitoneum proven pathologically from 1999 to 2009 were reviewed and studied after operation. Endocrine secretion examinations, B-US, CT, MRI, 131-MIBG, octreotide and hands microcirculation inspection were used to diagnose the disease. All patients underwent successful surgical resection of the tumors, which proved to be paragangliomas. They were from 3 cm to 25 cm in size. Almost all of them were diffusely positive for cgA, syn, NSE and s-100 by immunohitochemical staining. There were nine cases assayed malignant paraganglioma by the follow-up. 131-MIBG and octreotide have high sensitivity and accuracy in diagosing extra-adrenal paraganglioma. Surgical treatment should be carried out on the basis of correct drug preparation of a-receptor blocker, such as prazosin and phenoxybenzamine. Complete surgical excision is the treatment of choice for extra-adrenal paragangliomas as well as recurrent or metastatic disease, which could be resected laparoscopically. Intimate lifelong follow-up is necessary and important

Primary retroperitoneal synovial sarcoma in CT and MRI

Synovial sarcomas are most commonly localized in the extermities, especially the lower thigh and knee areas. Retroperitoneal synovial sarcoma is very rare. We decribe the radiological findings of an adult retroperitoneal synovial sarcoma

El signo de la aorta flotante / The floating aorta sign

El signo de la aorta flotante es causado por voluminosos conglomerados ganglionares que rodean y desplazan hacia anteriora la aorta abdominal, separándola de la columna lumbar, sin evidencias de compresión o estenosis aórtica. De esta manera la aorta parece flotar en el espesor de una masa de partes blandas. Se observa característicamente en linfomas. El diagnóstico diferencial incluye metástasis de carcinoma testicular, sarcomas retroperitoneales primarios, adenitis tuberculosa y fibrosis retroperitoneal. Aunque este signo puede observarse en estudios ultrasonográficos, es mejor demostrado y evaluado con tomografía computada y resonancia magnética.

The floating aorta sign is caused by an enlarged retroperitoneal lymph node mass which surrounds and displaces the abdominal aorta anteriorly, separating it from the lumbar spine without evidence of compression or aortic stenosis. Thus, the aorta seems to be floating inside a soft tissue mass. This radiologic sign is typically observed in lymphomas. The differential diagnosis includes testicular cancer metastases, primary retroperitoneal sarcomas, tuberculosis adenitis, and retroperitoneal fibrosis. Although this sign can be seen on ultrasound studies, it is best demonstrated and assessed through computed tomography and magnetic resonance imaging sequences.

The Differential Imaging Features of Fat-Containing Tumors in the Peritoneal Cavity and Retroperitoneum: the Radiologic-Pathologic Correlation

There are a variety of fat-containing lesions that can arise in the intraperitoneal cavity and retroperitoneal space. Some of these fat-containing lesions, such as liposarcoma and retroperitoneal teratoma, have to be resected, although resection can be deferred for others, such as adrenal adenoma, myelolipoma, angiomyolipoma, ovarian teratoma, and lipoma, until the lesions become large or symptomatic. The third group tumors (i.e., mesenteric panniculitis and pseudolipoma of Glisson's capsule) require medical treatment or no treatment at all. Identifying factors such as whether the fat is macroscopic or microscopic within the lesion, the origin of the lesions, and the presence of combined calcification is important for narrowing the differential diagnosis. The development and wide-spread use of modern imaging modalities make identification of these factors easier so narrowing the differential diagnosis is possible. At the same time, lesions that do not require immediate treatment are being incidentally found at an increasing rate with these same imaging techniques. Thus, the questions about the treatment methods have become increasingly important. Classifying lesions in terms of the necessity of performing surgical treatment can provide important information to clinicians, and this is the one of a radiologist's key responsibilities.

Neuroblastoma en el adulto: informe de un caso / Neuroblastoma in the adult: case report

Introducción: El neuroblastoma es una neoplasia común en la infancia, pero extremadamente rara en el adulto. Se origina del sistema nervioso simpático y su localización más común es abdominal. Su estadificación y tratamiento se han estandarizado en niños y adultos, aunque el pronóstico es muy distinto debido a un comportamiento más agresivo y menor sobrevida en los segundos. Caso clínico: Hombre de 31 años de edad evaluado por dolor abdominal inespecífico y constipación, a quien se le diagnosticó gran neuroblastoma retroperitoneal estadio III. Al no tolerar quimioterapia se realizó cirugía. Se presenta la evaluación, manejo y seguimiento, así como una revisión de la literatura. Conclusiones: El neuroblastoma en el adulto es una enfermedad poco común que cursa con una evolución inicial insidiosa y la presentación suele ser en estadios avanzados. A diferencia del comportamiento en la infancia, en el adulto es más agresivo y con menor sobrevida a pesar de realizar el mismo tratamiento.

BACKGROUND: Neuroblastoma is a common malignancy in infancy but extremely rare in adults. These tumors, commonly found in the abdomen, originate in the sympathetic nervous system. Staging and management are standardized in children and adults, although their prognosis is very different, being more aggressive and with a poorer outcome in the adult. CLINICAL CASE: We present the case of a 31-year-old male with non-specific abdominal pain and constipation. After several studies, a stage III giant retroperitoneal neuroblastoma was diagnosed. We discuss here the evaluation, management and follow-up of the patient. A literature review is presented as well. CONCLUSIONS: Adult neuroblastoma is an unusual disease with an insidious presentation and is usually diagnosed in advanced stages. Unlike its behavior in young patients, in the adult it is more aggressive and with a poor prognosis.