Expression of GPNMB in renal eosinophilic tumors and its value in differential diagnosis / 中华病理学杂志

Objective: To investigate the expression of glycoprotein non metastatic melanoma protein B (GPNMB) in renal eosinophilic tumors and to compare the value of GPNMB with CK20, CK7 and CD117 in the differential diagnosis of renal eosinophilic tumors. Methods: Traditional renal tumor eosinophil subtypes, including 22 cases of renal clear cell carcinoma eosinophil subtype (e-ccRCC), 19 cases of renal papillary cell carcinoma eosinophil subtype (e-papRCC), 17 cases of renal chromophobe cell carcinoma eosinophil subtype (e-chRCC), 12 cases of renal oncocytoma (RO) and emerging renal tumor types with eosinophil characteristics [3 cases of eosinophilic solid cystic renal cell carcinoma (ESC RCC), 3 cases of renal low-grade eosinophil tumor (LOT), 4 cases of fumarate hydratase-deficient renal cell carcinoma (FH-dRCC) and 5 cases of renal epithelioid angiomyolipoma (E-AML)], were collected at the Affiliated Drum Tower Hospital of Nanjing University Medical School from January 2017 to March 2022. The expression of GPNMB, CK20, CK7 and CD117 was detected by immunohistochemistry and statistically analyzed. Results: GPNMB was expressed in all emerging renal tumor types with eosinophil characteristics (ESC RCC, LOT, FH-dRCC) and E-AML, while the expression rates in traditional renal eosinophil subtypes e-papRCC, e-chRCC, e-ccRCC and RO were very low or zero (1/19, 1/17, 0/22 and 0/12, respectively); the expression rate of CK7 in LOT (3/3), e-chRCC (15/17), e-ccRCC (4/22), e-papRCC (2/19), ESC RCC (0/3), RO (4/12), E-AML(1/5), and FH-dRCC (2/4) variedly; the expression of CK20 was different in ESC RCC (3/3), LOT(3/3), e-chRCC(1/17), RO(9/12), e-papRCC(4/19), FH-dRCC(1/4), e-ccRCC(0/22) and E-AML(0/5), and so did that of CD117 in e-ccRCC(2/22), e-papRCC(1/19), e-chRCC(16/17), RO(10/12), ESC RCC(0/3), LOT(1/3), E-AML(2/5) and FH-dRCC(1/4). GPNMB had 100% sensitivity and 97.1% specificity in distinguishing E-AML and emerging renal tumor types (such as ESC RCC, LOT, FH-dRCC) from traditional renal tumor types (such as e-ccRCC, e-papRCC, e-chRCC, RO),respectively. Compared with CK7, CK20 and CD117 antibodies, GPNMB was more effective in the differential diagnosis (P<0.05). Conclusion: As a new renal tumor marker, GPNMB can effectively distinguish E-AML and emerging renal tumor types with eosinophil characteristics such as ESC RCC, LOT, FH-dRCC from traditional renal tumor eosinophil subtypes such as e-ccRCC, e-papRCC, e-chRCC and RO, which is helpful for the differential diagnosis of renal eosinophilic tumors.

Causes of False-Positive Results in 68Ga-Labeled Fibroblast Activation Protein Inhibitor PET/CT Imaging / 中国医学科学院学报

Objective To investigate the causes of false-positive results in the 68Ga-labeled fibroblast activation protein inhibitor (68Ga-FAPI-04) PET/CT imaging. Methods The imaging data of 547 patients undergoing 68Ga-FAPI-04 PET/CT examination in the Department of Nuclear Medicine of the Affiliated Hospital of Southwest Medical University from September 2020 to May 2021 were retrospectively collected.Two experienced nuclear medicine diagnostic physicians analyzed the clinical data,relevant imaging examinations,laboratory examinations,pathological results and follow-up results of the patients with false-positive results. Results The 68Ga-FAPI-04 PET/CT imaging of 547 patients showed false-positive results in 99 (18.1%) patients,including 56 males and 43 females.The postoperative pathological examination confirmed false-positive results in 13 patients,including 1 patient of thyroiditis,2 patients of pulmonary tuberculosis,1 patient of bone tuberculosis,2 patients of pulmonary inflammatory pseudotumor,1 patient of pulmonary sarcoidosis,1 patient of pulmonary benign fibroma,1 patient of organic pneumonia,2 patients of renal angiomyolipoma,1 patient of mass pancreatitis,and 1 patient of pancreatic mucinous cystadenoma.The medical history,relevant imaging examination,and long-term follow-up confirmed false-positive results in 86 patients.Specifically,the false-positive uptake in the neck,chest,abdomen,bone joint,and skin occurred in 8 (9.3%),13 (15.1%),5 (5.8%),57 (66.3%),and 3 (3.5%) patients,respectively.Inflammation-related uptake appeared in 83 (83.8%) patients with false-positive imaging results,of which arthritis (23 patients) and osteophyte (29 patients) were the most common.Sixteen (16.2%) patients showed the false-positive uptake related to fibroblasts. Conclusion 68Ga-FAPI-04 PET/CT imaging will show non-malignant tumor false-positive results,which are mainly associated with inflammation and fibroblasts.

Angiomiolipoma hepático epitelioide: reporte de un caso / Hepatic epithelioid angiomyolipoma: Case Report

Angiomyolipomas (AML) are mesenchymal tumors belonging to the group of perivascular epithelioid cell tumors, which, rarely, can display a malignant behavior. They are composed of adipose tissue, vessels, and muscle tissue in different proportions, and constitute a differential diagnosis for other focal liver lesions. We report a 34-year-old woman in whom a focal hepatic lesion was discovered incidentally. The pathology report of an ultrasound guided biopsy informed an epithelioid angiomyolipoma, a rare variant of these lesions. During ten years of imaging follow, the size and features of the lesion has not changed. The patient rejected a surgical excision.

Renal angiomyolipoma with inferior vena cava and right atrial embolism: A case report and literature review / 中南大学学报(医学版)

Renal angiomyolipoma (AML) with renal vein, inferior vena cava (IVC), and right atrial embolism is a rare solid tumor, whose etiology and pathogenesis are still unclear. Moreover, it is often misdiagnosed. One patient with renal AML complicated with renal vein, IVC, and right atrial embolism was admitted to the Second Xiangya Hospital of Central South University, who was a 35-year-old female, without any previous medical history, presented with right low back pain for more than 3 years. Computed tomography (CT) scan showed irregular lobulated fatty density mass in the right kidney, renal vein, IVC, and right atrium. The contrast-enhanced scan showed no enhancement of fat components at each phase and mild enhancement of solid components. Radical resection of the right kidney and removal of tumor thrombus were performed, and there was no recurrence 1 year after the operation. It is rare for renal AML to grow along the renal vein, IVC, and extend to the right atrium. Imaging examination is extremely important, and the CT findings of this case are characteristic, but the diagnosis eventually depends on pathological and immunohistochemical examinations.

Sporadic Renal Angiomyolipoma: Can We Adopt a Uniform Management Protocol / Angiomiolipoma renal esporádico: ¿Podemos adoptar un protocolo de tratamiento estándar?

Renal angiomyolipomas (AMLs), formerly known as PEComas (tumors showing perivascular epithelioid cell differentiation) are common benign renal masses composed of a varying ratio of fat, blood vessels, and smooth muscles. They are largely asymptomatic and diagnosed incidentally on imaging. The adipose tissue content is the factor that gives AMLs their characteristic appearance on imaging and makes them easily identifiable. However, the fat-poor or fat-invisible varieties, which are difficult to differentiate radiologically from renal cell carcinomas (RCCs), present a diagnostic challenge. It is thus essential to establish the diagnosis and identify the atypical and hereditary cases as they require more intense surveillance and management due to their potential for malignant transformation. Multiple management options are available, ranging from conservative approach to embolization and to the more radical option of nephrectomy. While the indications for intervention are relatively clear and aimed at a rather small cohort, the protocol for follow-up of the remainder of the cohort forming the majority of cases is not well established. The surveillance and discharge policies therefore vary between institutions and even between individual practitioners. We have reviewed the literature to establish an optimum management pathway focusing on the typical AMLs.

Los angiomiolipomas renales (AML), antes conocidos como PEComas (tumores que muestran epitelioides perivasculares) son masas renales benignas frecuentes compuestas por una proporción variable de grasa, vasos sanguíneos y músculos lisos. Suelen ser asintomáticos y se diagnostican de forma incidental en las pruebas de imagen. El contenido de tejido adiposo es el factor que confiere a los AML su aspecto característico en las imágenes y los hace fácilmente identificables. Sin embargo, las variedades pobres en grasa o invisibles, que son difíciles de diferenciar radiológicamente de los carcinomas de células renales (CCR), suponen un reto diagnóstico. Por lo tanto, es esencial establecer el diagnóstico e identificar los casos atípicos y hereditarios, ya que requieren una vigilancia y un tratamiento más intensos debido a su potencial de malignización. debido a su potencial de transformación maligna. Existen múltiples opciones de tratamiento, que van desde el enfoque conservador hasta la embolización y la opción más radical de la nefrectomía. Si bien las indicaciones para la intervención son relativamente claras y están dirigidas a una cohorte bastante pequeña, el protocolo para el seguimiento del resto de la cohorte que forma la mayoría de los casos no está bien establecido. Por lo tanto, las políticas de vigilancia y alta varían entre instituciones e incluso entre profesionales individuales. Hemos revisado la literatura para establecer una ruta de manejo óptima centrada en los AML típicos.

Primary application of Gerota's fascia suspension device in retroperitoneal laparoscopic partial nephrectom / 北京大学学报(医学版)

OBJECTIVE@#To evaluate the value of Gerota's fascia suspension device in retroperitoneal laparoscopic partial nephrectomy, and to share the operation experience.@*METHODS@#From October 2018 to December 2020, 6 cases of tumor located in the ventral side of the kidney were selected, including 3 males and 3 females, with 3 cases on the right side and 3 cases on the left side, aged 38-60 years, with an average of 52 years. The body mass index (BMI) was 18.3-30.2 kg/m2, with an average of 22.9 kg/m2. One patient with diabetes mellitus, three patients with renal cysts, and two patients underwent cholecystectomy before. All the patients were found by physical examinations. The course of disease was 7 days to 20 years, with a median time of 1 month. The tumor was in the ventral side of the kidney, 2 cases located in the upper pole, 1 case in the lower pole and 3 cases near the renal hilum. The tumor size was 1.2-7.8 cm, with an average of 4.5 cm. The R.E.N.A.L score was 7 in 1 case, 8 in 3 cases and 9 in 2 cases. After the preoperative examination completed, retroperitoneal laparoscopic partial nephrectomy (Gerota's fascia suspension device) was performed.@*RESULTS@#All the operations were successfully completed. The operation time ranged from 139 to 193 min, with an average of 172 min. The renal artery occlusion time was 7-43 min, with an average of 19 min, only one case was more than 30 min. The blood loss ranged from 10 to 500 mL, with an average of 128 mL. The postoperative hospital stay ranged from 4 to 13 days, with an average of 6.5 days. Postoperative pathology revealed 4 cases of renal angiomyolipoma and 2 cases of renal clear cell carcinoma. The patients were followed up for 2-27 months, with an average of 17 months, without recurrence.@*CONCLUSION@#In the operation of retroperitoneal laparoscopic partial nephrectomy, Gerota's fascia suspension device is beneficial to expose the ventral surgical field, and it is convenient for the surgeon to operate with both hands. This technique is an effective method to deal with the ventral renal tumor, which is worthy of promotion.

Renal angiomyolipoma with epithelial cyst

Angiomyolipoma with epithelial cysts (AMLEC) is a recently described entity and is an uncommon subtype of kidney angiomyolipomas. AMLEC is a benign entity but usually masquerades a renal cell carcinoma on imaging examination. AMLEC has a distinct histological and immunohistochemical staining pattern, which helps in the pathological diagnosis. We present a rare case of AMLEC in a 26-year-old female, which was provisionally diagnosed as renal cell carcinoma on radiology. We also summarize the differential diagnosis of this rare variant, its characteristic features, and a review of the literature.

Síndrome de Wünderlich asociado a hemodiálisis / Wünderlich syndrome associated with hemodialysis

INTRODUCCIÓN. El Síndrome de Wunderlich es un hallazgo infrecuente en la práctica urológica, se caracteriza por dolor lumboabdominal intenso, inestabilidad hemodinámica y masa palpable en flanco ipsilateral (Triada de Lenk). En la mayoría de casos, la etiología es de origen tumoral (Angiomiolipoma); así como discrasias sanguíneas y enfermedad renal quística adquirida, considerada como factor de riesgo para que ocurra hemorragia espontánea en pacientes con hemodiálisis a largo plazo. La tomografía computarizada es el examen de imagen estándar para el diagnóstico; el tratamiento va desde el manejo conservador hasta nefrectomía de emergencia. CASO CLÍNICO. Paciente masculino de 29 años con antecedentes de Hidrocefalia y Enfermedad Renal Crónica estadio IV, en tratamiento con Hemodiálisis. Acudió por dolor abdominal y descenso de valores de he-moglobina. RESULTADOS. En Urotomografía simple/contrastada se reportó hematoma retroperitoneal, por lo que se realizó una nefrectomía simple, se encontró un hematoma más trauma renal grado V. El paciente falleció en el posquirúrgico. DISCUSIÓN. Se han reportado algunos casos de Síndrome de Wunderlich relacionados a hemodiálisis que se acompañaron de dolor abdominal intenso, y se sospechó de esta patología. CONCLU-SIÓN. El diagnóstico temprano y tratamiento oportuno en el Síndrome de Wünderlich fue crucial para el manejo médico del paciente.

INTRODUCTION. Wunderlich Syndrome is an infrequent finding in urological practi-ce, it ́s characterized by intense lumbo-abdominal pain, hemodynamic instability and palpable mass in the ipsilateral flank (Lenk's Triad). In most cases, the etiology is of tumor origin (Angiomyolipoma); as well as blood dyscrasias and acquired cystic kidney disease, considered a risk factor for spontaneous bleeding in patients with long-term hemodialysis. Computed tomography is the standard imaging test for diag-nosis; Treatment ranges from conservative management to emergency nephrectomy. CLINICAL CASE. 29-year-old male patient with a history of Stage IV Hydrocephalus and Chronic Kidney Disease, undergoing treatment with hemodialysis. He came for abdominal pain and decreased hemoglobin values. RESULTS. A simple / contrasted urotomography revealed retroperitoneal hematoma, for which a simple nephrectomy was performed, a hematoma plus grade V renal trauma was found. The patient died postoperatively. DISCUSSION. Some cases of Wunderlich Syndrome related to he-modialysis that were accompanied by severe abdominal pain have been reported, and this pathology was suspected. CONCLUSION. Early diagnosis and timely treat-ment in Wünderlich Syndrome was crucial for the medical management of the patient.

Angiomiolipoma renal con invasión de vena renal y vena cava inferior. Presentación de un caso y revisión de la literatura / Renal Angiomyolipoma with Extension to Renal and Cava Veins. A Case Report and Revision of the Literature

Introducción El angiomiolipoma es un tumor benigno cuya presentación con invasión del seno renal y formación de trombo venoso tumoral es muy infrecuente, habiéndose descrito aproximadamente 50 casos desde que en 1982 Kutcher describiera el primero. Reporte de caso Presentamos un caso angiomiolipoma renal con afectación venosa diagnosticado mediante TAC a partir del hallazgo de trombo venoso en ecografía y resonancia en una paciente de 43 años embarazada y asintomática que presentó elevación de transminasas en una analítica. Ante la posibilidad de que se tratara de una variante epitelioide y/o clásica con potencial de malignización, se decidió tratamiento mediante nefrectomía y trombectomía abierta, sin incidencias intra ni postoperatorias. La paciente se encuentra asintomática tras 6 meses de seguimiento. La histología fue compatible con angiomiolipoma clásico con trombo venoso. Conclusión La invasión de vena renal por un angiomiolipoma es extremadamente rara. Aunque infrecuente, habría que considerar la posibilidad de malignización para no demorar su tratamiento

Introduction Angiomyolipoma is a benign kidney tumour that rarely affects the renal sinus or the renal or caval vein. Kutcher made the first case report in 1982, and since then there have been described approximately 50 cases. Case Report We present a case of angiomyolipoma with venous thrombus in a 43 years-old asymptomatic pregnant woman who presented elevation of transaminases in a blood test, with an ultrasonography that showed a venous thrombus without any renal mass, being diagnosed after giving birth by scanner. Having in account the possibility of malignancy of the mass, we performed surgical treatment by open radical nephrectomy and thrombectomy, without intraoperative nor postoperative incidences. The patient remains asymptomatic 6 months after surgery. The histology was compatible with classic angiomyolipoma with venous thrombus. Conclussion Angiomyolipoma causing venous invasion is extremely rare, but we should take into consideration its possibility of malignancy in order to treat it as soon as possible

Angiomyolipoma of the broad ligament

Angiomyolipoma is a benign mesenchymal neoplasm of the renal parenchyma, accounting for 1% of all renal parenchymal tumors. However, this entity may rarely occur in extrarenal sites. Extrarenal angiomyolipoma has been documented in various sites of the body, but angiomyolipoma of the broad ligament was reported in only two cases. We report the reputed third case of angiomyolipoma of the broad ligament in a 33-year-old female, who presented clinically with abdominal distension. With a working diagnosis of low-grade neoplasm, an en-bloc excision of the left broad ligament mass was performed. Based on histopathology and immunohistochemistry, a diagnosis of the classical variant of angiomyolipoma of the left broad ligament was made. The post-operative period was uneventful with no recurrence after 6 months of follow-up.

Epithelioid angiomyolipoma with tumor thrombus in IVC and right atrium

Epithelioid angiomyolipoma is an uncommon subtype of renal angiomyolipoma associated with potentially malignant behavior and is considered a distinct entity by the World Health Organization classification of renal tumors. We present a case of an epithelioid variant of angiomyolipoma with extension into the renal vein, inferior vena cava reaching up to the right atrium. Pre-operatively, a diagnosis of renal cell carcinoma was considered based on imaging findings. Intra-operatively due to extensive adhesions, surgical resection was not performed and only tissue sampling was performed for histopathology. Microscopic examination revealed short fascicles of spindle cells and perivascular epithelioid cells. A differential diagnosis of renal cell carcinoma with sarcomatoid differentiation was considered. The immunohistochemical profile showed tumor cells that express Melan-A and smooth muscle actin, while they were negative for pan-cytokeratin, PAX8, CK7, CD117 and CD34. Therefore a diagnosis of epithelioid angiomyolipoma was rendered. The presence of intravascular thrombi on radiological investigation and carcinoma-like growth pattern on light microscopy may compound an erroneous diagnosis of renal cell carcinoma. Hence, it is prudent for the urologist to consider differential diagnosis other than renal cell carcinoma when confronted with a renal neoplasm presenting with intravascular thrombi. In these cases, a core biopsy should be planned pre-operatively and diagnosis should be made with aid of appropriate immunohistochemical markers.

Renal angiomyolipoma

SUMMARY INTRODUCTION Angiomyolipoma is one of the most common benign solid renal tumors. We investigated the characteristics of renal angiomyolipomas and the clinical outcomes of patients in the last thirteen years. METHODS The medical records of the patients who underwent nephrectomy were reviewed retrospectively from July 2005 to May 2018. The laboratory data, radiology, and pathology reports were recorded. Patients diagnosed with angiomyolipoma were included in the study. RESULTS A total of 28 patients were included in the study, eight of them male. The mean age of the patients was 55.89+14.49 years. The patients were treated with open and laparoscopic techniques. Partial nephrectomy was performed in 12 patients(42.85%). After pathological examination, 23 patients were diagnosed as fat rich, four patients as fat poor, and one as epithelioid angiomyolipoma. There were no recurrences in the follow-up 91.21+48.31 months. CONCLUSION Angiomyolipoma is a rare renal tumor in daily urology practice. Clinicians must be aware of its complications and manage patients well.

RESUMO OBJETIVO O angiomiolipoma é um dos tumores renais benignos sólidos mais comuns. Investigamos as características dos angiomiolipomas renais e os desfechos clínicos dos pacientes nos últimos treze anos. MÉTODOS Os prontuários dos pacientes, para os quais a nefrectomia foi realizada, foram revisados retrospectivamente de 2008 a 2018. Os dados laboratoriais, relatórios de radiologia e patologia foram registrados. Os pacientes diagnosticados como angiomiolipoma foram incluídos no estudo. RESULTADOS Vinte e oito pacientes foram incluídos no estudo, oito deles do sexo masculino. A média de idade dos pacientes foi de 55,89 + 14,49 anos. Os pacientes foram tratados com técnicas abertas e laparoscópicas. Nefrectomia parcial foi realizada em 12 pacientes (42,85%). Depois de exame patológico, 23 pacientes foram diagnosticados como ricos em gordura, quatro pacientes como gordurosos e um paciente como angiomiolipoma epitelioide. Nenhum paciente teve recorrências no seguimento. CONCLUSÕES O angiomiolipoma é um tumor renal raro na prática urológica diária. Os médicos devem estar cientes das complicações e gerenciar bem os pacientes.

Masas renales pequeñas: predictores de malignidad en una serie de 10 años / Small renal masses: analysis of 152 cases

Background: Small renal masses (SRM) are defined as complex organ-confined solid or cystic lesions < 4 cm. Up to 20% of these can be benign. A conservative management with active surveillance can be done in some patients. However, it is difficult to identify patients with a higher risk of malignancy. Aim: To characterize the clinical, radiological and histopathological aspects of patients with SRM, analyzing predictive factors for tumor aggressiveness. Material and Methods: Retrospective analysis of a cohort of patients undergoing partial or total nephrectomy for renal tumors between 2006 and 2016. All tumors of 4 cm or less were included. Four histological groups were defined: benign, favorable, intermediate and unfavorable. Two categories of risk were also defined: low and high. Preoperative clinical and radiological variables of these patients were analyzed. Results: Data of 152 patients were analyzed. Six percent had a benign histology, and the majority was of intermediate risk (74%). According to histological type, clear cell carcinoma was the most common type (74%). Three percent were benign angiomyolipomas. No malignancy predictive variable was identified. Conclusions: In these patients, the percentage of benign SRM was low. No variable that could predict the presence of a benign or malignant lesion in the definitive biopsy was identified.

Embolization of ruptured renal angiomyolipoma in puerpera / Embolização de angiomiolipoma renal roto em puérpera

Abstract Angiomyolipomas (AMLs) are rare benign tumors derived from mesenchymal tissue and composed of varying degrees of adipose tissue, muscle and blood vessels. Renal AMLs (RAMLs) are the result of a sporadic event, and, in most of cases, the diagnosis is usually incidental, but hemorrhage and shock may be present. During pregnancy, the size of AMLs may increase and they may rupture, probably due to the high expression of hormone receptors, and the increase in maternal circulation and abdominal pressure. The authors present a case of a woman with ruptured RAML submitted to urgent endovascular treatment four days after giving birth by cesarean section.

Resumo Angiomiolipomas (AMLs) são tumores benignos raros derivados do tecido mesenquimal, compostos em graus variados de tecido adiposo, muscular e de vasos sanguíneos. Os AMLs renais (AMLRs) resultam de um evento esporádico e, na maioria dos casos, o diagnóstico costuma ser fortuito, mas quadros de hemorragia e choque podem estar presentes. Durante a gestação, os AMLs podem aumentar de tamanho e romper, provavelmente pela altaexpressãodereceptoreshormonais,epeloaumentodacirculaçãomaternaedapressão abdominal. Os autores apresentam um caso de uma paciente com AMLR roto submetida a tratamento endovascular de urgência no quarto dia pós-operatório de uma cesariana.

Complejo linfangioleiomiomatosis-esclerosis tuberosa, ¿contraindicación para el trasplante pulmonar? Presentación de un caso / Lymphangioleiomyomatosis-Tuberous Sclerosis Complex. Contraindication For Lung Transplantation? A Case Report

El complejo linfangioleiomiomatosis (LAM)-esclerosis tuberosa (ET) es una enfermedad rara, con compromiso multisistémico que afecta principalmente pulmón y cerebro. Se presenta el caso de una paciente de 25 años con LAM pulmonar que ingresa a la institución para protocolo de trasplante pulmonar. Durante los estudios de extensión se documenta compromiso extrapulmonar sistémico del complejo LAM-ET. A partir de este caso, se realizó una revisión de la literatura de las manifestaciones radiológicas sistémicas de la enfermedad y las recomendaciones de diagnóstico y manejo de estos pacientes; en especial, las indicaciones y contraindicaciones del trasplante pulmonar.

The lymphangioleiomyomatosis (LAM) - Tuberous Sclerosis (TS) Complex is a rare disease with multisystem involvement affecting mainly lung and brain. We present the case of a 25-year-old female patient with pulmonary LAM. During the work-up studies for the lung transplantation protocol, the systemic extrapulmonary involvement of the LAM-TS complex is documented. From this case, a literature review of the systemic radiological manifestations of the disease and of the diagnosis and management recommendations of these patients was made. Special focus was made on the indications and contraindications of lung transplantation.

Minimal fat renalangiomyolipoma with multiple lymph nodes enlargement and postoperative refractory lymphatic fistula: a case report and literature review / 北京大学学报(医学版)

Renal angiomyolipoma (AML) is a common benign tumor in the urinary system, mainly composed of adipose tissue, blood vessels and muscle tissue. Renal AML is sporadic in most of patients, while a few are associated with tuberous sclerosis. Classical renal AML occurs predominantly in middle-aged females. Most cases are found incidentally during imaging examinations. The fat content makes AML have unique imaging characteristics and is easy to be identified with other renal tumors. However, the amount of fat varies in each tumor. AML that contains only microscopically detectable fat and whose amount of intratumoral fat may be too small to be identified on unenhanced computed tomography (CT) images is termed minimal fat or fat-poor renal AML, which appears as a high density shadow in the renal parenchyma on unenhanced CT images. Thus, it can be difficult to distinguish it from renal cell carcinoma (RCC) on imaging. Since the imaging findings are atypical, the diagnosis depends on pathological results. In addition, a few of AML can mimic malignant neoplasms. Recent studies suggested that AML might involve to peri-renal or renal sinus fat, regional lymphatics and other visceral organs, as well as inferior vena cava, which further makes the diagnosis more difficult. However, there is currently no reports about involvement of regional limphatics in minimal fat renal AML. In this article, we report a 27-year-old female patient without family history of tuberous sclerosis, who came to visit the hematologist because a high density shadow near the left kidney was found during CT scan which was accompanied by neck, armpits, groin, abdominal cavity and retroperitoneal lymph nodes enlargement. She was suspected of lymphoma in the beginning and transferred to Department of Urology to perform laparoscopic left renal mass and retroperitoneal lymph node excision and pathological examination for a definitive diagnosis. Finally, pathologic results revealed AML. Postoperative continuous lymphatic fistula developed and the retroperitoneal drainage of chylous fluid was 100-200 mL per day, lasting for 12 weeks. The fistula was successfully closed after conservative treatment including fasting and rehydration. This article summaries and discusses the diagnosis and treatment of renal AML with lymph nodes enlargement and the management of postoperative refractory lymphatic fistula by reviewing the related cases and literature.

Comparison of safety and effectiveness between retroperitoneal laparoscopic tumor aspiration and laparoscopic partial nephrectomy in the treatment of renal angiomyolipoma / 北京大学学报(医学版)

OBJECTIVE@#To compare the safety and treatment effectiveness of retroperitoneal laparoscopic tumor aspiration and laparoscopic partial nephrectomy (LPN) in the treatment of renal angiomyolipoma (RAML).@*METHODS@#We retrospectively reviewed the clinical data of patients with pathologically confirmed RAML who received operation between August 2010 and August 2016 in the Department of Urology, Peking University First Hospital. Among them, a series of 121 patients were included in this trial according to the inclusion criteria, of which 74 cases could be collected and followed-up effectively. Based on the detailed surgical route, the 74 patients were divided into groups A and B: group A, which underwent retroperitoneal laparoscopic tumor aspiration, included 43 cases; group B, which received retroperitoneal LPN, included 31 cases. Patient demographics, intraoperative variables and postoperative outcomes were reported and compared between the groups.@*RESULTS@#No statistical difference was detected in both groups before the treatment. Intraoperatively, the mean estimated blood loss was 48.7 mL in group A and 102.9 mL in group B, and the mean operative time was 70.1 min (21.2 min of warm ischemia time included) in group A and 103.6 min (28.5 min of warm ischemia time included) in group B, which were both statistically different. In group A, no complications occurred and yet 2 complications of transfusion and 1 complication of urine leakage were discovered in group B, although all finally recovered only with conservative treatment. A statistical difference was observed in the complication rates. Post-operatively, the mean serum creatinine level was 1.13 mg/dL in group A, and the level was 1.08 mg/dL in group B, in which no evident difference was detected. In a mean 52.6-months' follow-up, a recurrence of 3 cases in group A (7.0%) and a recurrence of 2 cases in group B (6.5%) were reported. No evident difference was also detected between the groups in the tumor recurrence rates.@*CONCLUSION@#Due to the improvements in the intraoperative blood loss and operative time, retroperitoneal laparoscopic tumor aspiration may be provided with more potential advantages in the safety, also with equal efficacy of lower tumor recurrence rates when compared with the traditional retroperitoneal LPN in the treatment of RAML.

Angiomyolipoma of the Liver without a Fat Component, Mimicking a Hepatocellular Carcinoma / 대한소화기학회지

Angiomyolipoma (AML) is a rare benign mesenchymal tumor in the liver, which is composed of blood vessels, smooth muscle, and adipose cells. The proportion of each component varies, making a diagnosis difficult. This paper reports a case of AML in the liver without adipose tissue, mimicking a hepatocellular carcinoma (HCC), which was diagnosed by a surgical tissue biopsy. A 65-year-old woman was admitted for an evaluation of a hepatic mass that had been detected by ultrasonography. The serologic markers of viral hepatitis B and C were negative. The liver function tests and alpha fetoprotein level were within the normal limits. Magnetic resonance imaging revealed a 1.9 cm sized mass in segment 6 of the liver with early arterial enhancement and washout on the delayed phase accompanied by a rim-like enhancement, which is similar to the imaging findings of HCC. A frozen section examination during surgery indicated a hepatocellular neoplasm and suggested the possibility of HCC. On the other hand, the final pathologic diagnosis was epithelioid myoid type of AML with no adipose tissue component. The tumor cells were positive for human melanocyte B-45 and negative for cytokeratin and hepatocyte paraffin 1. This paper reports a very rare case of AML without adipose tissue in the liver mimicking HCC that was diagnosed by a surgical tissue biopsy.