Lesiones metastásicas a glándula tiroides. Una serie de casos / Metastatic lesions to the thyroid gland. Case series

Las lesiones metastásicas representan hasta un 3 % de los tumores malignos de la glándula tiroides. La mayoría de los casos se originan de tumores de células renales y de pulmón. El abordaje diagnóstico implica una alta sospecha clínica en pacientes con primarios conocidos, sin embargo, puede ser la manifestación inicial de una enfermedad maligna extensa no diagnosticada hasta en un 20 % a 40 % de los pacientes. La biopsia por aguja fina ha demostrado buen rendimiento para el diagnóstico de los nódulos metastásicos. El pronóstico y la opción del tratamiento quirúrgico dependen del control local del primario y del estado de la enfermedad sistémica asociada, por lo tanto, debe ser individualizado. Por lo general, hasta un 80 % de los pacientes con compromiso de la tiroides tienen enfermedad metastásica multiorgánica, y la intención del tratamiento quirúrgico es con fines paliativos para prevenir las complicaciones derivadas de la extensión local de la enfermedad a las estructuras del tracto aerodigestivo superior en el cuello. Se presenta a continuación, una serie de seis casos de pacientes con lesiones metastásicas a glándula tiroides con primarios en riñón, mama y de melanomas

Metastatic lesions represent up to 3% of malignant tumors of the thyroid gland. Most cases originate from lung and renal cell tumors. The diagnostic approach implies a high clinical suspicion in patients with known primaries, however, it can be the initial manifestation of an extensive undiagnosed malignant disease in up to 20% to 40% of patients. Fine-needle biopsy has shown good performance for the diagnosis of metastatic nodules. The prognosis and the option of surgical treatment depend on the local control of the primary condition and the state of the associated systemic disease, therefore it must be individualized. In general, up to 80% of patients with thyroid involvement have multi-organ metastatic disease and surgical treatment is intended to be palliative to prevent complications resulting from local extension of the disease to structures of the upper aerodigestive tract in the neck. A case series of six patients with metastatic lesions to the thyroid gland with primaries in the kidney, breast and melanomas is presented below

Metástasis oral de carcinoma de células renales. A propósito de un caso y revisión de la literatura / Oral metastasis of renal cell carcinoma. A Case report and literature review / Metástase oral de carcinoma de células renais. Relato de um caso e revisão da literatura

Los tumores metastásicos en cavidad oral son pocos frecuentes, representan el 1% de las neoplasias malignas orales, son relativamente más frecuentes en los maxilares, respecto a aquellos localizados en los tejidos blandos orales. Se describe el caso de una paciente de 75 años de edad, con antecedentes de carcinoma renal de células claras, que consulta por la aparición repentina de una lesión tumoral en reborde alveolar superior izquierdo. Se realizó la biopsia exéresis con diagnóstico presuntivo de tumor metastásico o posible lesión reactiva. El informe anátomo - patológico confirma el diagnóstico de metástasis de un carcinoma renal. Las metástasis orales tienen un pronóstico generalmente malo, compromete la sobrevida, por lo que es importante realizar un exhaustivo estudio del paciente y considerar sus antecedentes, ya que en ocasiones son diagnosticadas tardíamentete.

Metastatic tumors to the oral cavity are rare, representing only 1% of malignant oral neoplasms. These metastatic tumors occur more frequently in the jaws than in soft oral tissues. This article describes the case of a 75-year-old patient with a history of clear cell renal carcinoma who seeks care because of the sudden appearance of a tumor lesion in the upper left alveolar ridge. An excision biopsy was performed with a presumptive diagnosis of a metastatic tumor or potential reactive lesion. The pathology report confirmed the diagnosis of renal cell carcinoma metastasis. Oral metastatic tumors usually present a bad prognosis with a low survival rate. It is important to examine patients thoroughly and consider their medical records, as they are sometimes diagnosed late.

Os tumores metastásicos na cavidade oral são raros, representam 1% das neoplasias malignas orais, sendo relativamente mais frequentes nos ossos maxilares enquanto aos localizados nos tecidos moles orais. Descreve-se o caso de uma mulher de 75 anos com história de carcinoma renal de células claras, que consultou com queixa de lesão tumoral localizada na crista alveolar superior esquerda. Uma biópsia foi feita com diagnóstico clínico presuntivo de tumor metastático ou possível lesão reativa. O laudo anátomopatológico confirmou o diagnóstico de metástase de carcinoma renal. As metástases orais geralmente têm um prognóstico ruim, com baixa sobrevivência, portanto é importante fazer um estudo exaustivo do paciente e tomar em conta sua história, já que às vezes o diagnóstico é tardio.

Eosinophilic vacuolated tumor of the kidney: clinicopathological and molecular characteristics / 中华病理学杂志

Objective: To study the clinicopathological features, immunophenotype, molecular changes, differential diagnosis and prognosis of eosinophilic vacuolated tumor (EVT) of the kidney. Methods: Four cases were collected retrospectively from 2014 to 2020 at Ningbo Diagnostic Pathology Center. The clinicopathologic features and immunophenotypic profile were studied by light microscopy and immunohistochemistry. Targeted next-generation sequencing (NGS) panel was used to detect cancer-associated mutation. Follow-up and literature review were also performed. Results: Among the 4 patients studied,2 were males and 2 were females. The age of the patients ranged from 44 to 63 years (the mean age: 51 years).Tumor size ranged from 1.5 to 4.2 cm (mean: 2.3 cm). Microscopically, tumors were well-circumscribed, unencapsulated. Thick-walled vessels and entrapped renal tubules were found within or at the periphery of the tumors. The tumors were predominantly composed of nest pattern, and focal tubular pattern. The tumor cells exhibited abundant, eosinophilic, granular cytoplasm and conspicuous, large nucleoli. Prominent intracytoplasmic vacuoles were seen. These cytoplasmic vacuoles varied in size and frequently coalesced into a large space. Loose fibromatous or hyaline stroma was focally noted. Immunohistochemically, the tumor cells in all cases exhibited a CD117+/CK7-phenotype. All cases were positive for CD10 and p504s. MTOR, S6 and cathepsin K were positive in 4 cases. TFE3, CA9, Melan A and HMB45 were negative in all cases. SDHB retained expression. NGS demonstrated MTOR mutations in all cases, and TSC2 mutation in 2 cases. Conclusions: EVT is a rarely oncocytic renal tumor with unique morphology, immunohistochemical phenotype, molecular profile and an indolent behavior. Recognition of the characteristics of this novel but rare entity will allow for better classification of renal tumors.

Vertebral metastasis of renal carcinoma at diagnosis: mechanical and clinical evaluation: surgical management / Metástasis vertebral de carcinoma renal en el momento del diagnóstico: evaluación mecánica y clínica: manejo quirúrgico

Renal cell carcinoma accounts for 2-3% of all malignant neoplasms. Metastatic disease of the spine is common and 50% of bone metastases are already present at the time of primary diagnosis. Bone metastases from renal cell carcinoma are difficult to manage, especially vertebral localization.A 48-year-old woman was diagnosed with renal cell carcinoma in the context of low back pain. The patient presented two skeleton metastases at diagnosis (T11 and 5th rib). The patient received neoadjuvant treatment with cabozantinib, followed by removal of the renal tumor. Radiotherapy was administered for the lumbar lesion. In spite of the radiotherapy treatment, increased low back pain limiting mobility and ambulation. MRI showed an occupation of the spinal canal, without neurological lesion. The SINS scale revealed a score of 14 (vertebral instability). The patient's prognosis was greater than 12 months according to the Tokuhashi score. Based on clinical and mechanical criteria, surgical treatment of the vertebral lesion was decided. T11 vertebrectomy was performed, the reconstruction was made with an expandable cage, and T8 a L2 posterior spinal arthrodesis. A partial resection of the fifth rib was performed in order to remove the whole macroscopic tumor. After 3 months, she was diagnosed with a local infection, treated by irrigation, debridement and antibiotherapy, with good evolution. At 1-year follow-up, she has no low back pain or functional limitation. Follow-up chest-abdomen-pelvis computed CT scan showed absence of disease progression, furthermore, the vertebral arthrodesis shows fusion signs. At the time of this report, there are no clinical or radiological data of infection

El carcinoma de células renales representa el 2-3% de todas las neoplasias malignas. La enfermedad metastásica de la columna vertebral es frecuente y el 50% de las metástasis óseas ya están presentes en el momento del diagnóstico. Las metástasis óseas del carcinoma de células renales son difíciles de manejar, especialmente en localización vertebral.Una mujer de 48 años fue diagnosticada de carcinoma de células renales en el contexto de un dolor lumbar. La paciente presentaba dos metástasis óseas en el momento del diagnóstico (T11 y 5ª costilla). Inicialmente recibió tratamiento neoadyuvante con cabozantinib, seguido de la extirpación quirúrgica del tumor renal. Se administró radioterapia para la lesión lumbar. A pesar del tratamiento radioterápico, aumentó el dolor lumbar con limitación para la movilidad y la deambulación. La RM mostró una ocupación del canal espinal, sin lesión neurológica. La escala SINS reveló una puntuación de 14 (inestabilidad vertebral). El pronóstico de la paciente era superior a 12 meses según la puntuación de Tokuhashi. Basándose en criterios clínicos y mecánicos, se decidió el tratamiento quirúrgico de la lesión vertebral. Se realizó una vertebrectomía de T11, para la reconstrucción se usó una caja extensible, junto con una artrodesis vertebral T8-L2. Se realizó una resección parcial de la quinta costilla para eliminar todo el tumor macroscópico. A los 3 meses de la cirugía la paciente fue diagnosticada de infección local, tratada mediante irrigación, desbridamiento y antibioterapia, con buena evolución. Al año de seguimiento, no presenta dolor lumbar ni limitación funcional. La tomografía computarizada de tórax-abdomen-pelvis de seguimiento mostró ausencia de progresión de la enfermedad, además, la artrodesis vertebral muestra signos de fusión. En el momento de este informe, no hay datos clínicos ni radiológicos de infección.

A comparison of oncologic and functional outcomes in patients with pt3a renal cell carcinoma treated with partial and radical nephrectomy

ABSTRACT Hypothesis: Partial Nephrectomy is oncological safe in patients with pT3a RCC. Purpose: To compare the oncological and functional outcomes of patients with pT3a RCC scheduled for PN and RN. Materials and Methods: We retrospectively reviewed patients with pT3a N0 M0 RCC who underwent partial or radical nephrectomy from 2005 to 2016. Perioperative characteristics, including estimated glomerular filtration rate, tumor size, pathological histology, and RENAL nephrometry score, were compared between patients scheduled for partial or radical nephrectomy. We used multivariable Cox proportional hazards regression models to compare overall survival, cancer-specific survival, and recurrence-free survival between planned procedure type. Results: Of the 589 patients, 369 (63%) and 220 (37%) were scheduled for radical and partial nephrectomy, respectively; 26 (12%) of the scheduled partial nephrectomy cases were intraoperatively converted to radical nephrectomy. After adjusting for tumor size and histology, there were no statistically significant differences in overall survival (hazard ratio 0.66; 95% CI, 0.38-1.13), cancer-specific survival (hazard ratio 0.53; 95% CI, 0.16-1.75), or recurrence-free survival (hazard ratio 0.66; 95% CI, 0.34-1.28) between patients scheduled for partial or radical nephrectomy. Fewer patients scheduled for partial nephrectomy had estimated glomerular filtration rate reductions 3 to 9 months after surgery than patients scheduled for radical nephrectomy. Conclusion: We found no evidence that patients scheduled to undergo partial nephrectomy had poorer oncologic outcomes than patients scheduled to undergo radical nephrectomy. In select patients with pT3a renal cell carcinoma in whom partial nephrectomy is deemed feasible by the surgeon, partial nephrectomy should not be discouraged.

Epistaxis: the cause found beyond the nose

Renal cell carcinoma (RCC) is a malignant disease that is often diagnosed at a metastatic stage. The head and neck represent up to 3% of the metastatic RCC, and the paranasal sinus area is one of the least involved sites. Here, we introduce the case of a 74-year-old female patient who presented with a history of traumatic nasal bleed. A cranial computed tomography scan and magnetic resonance imaging showed a fronto-ethmoidal mass with pachymeningeal involvement. A nasal biopsy from the paranasal sinuses was taken. On histopathological examination, metastatic clear cell carcinoma was the main hypothesis, which later was confirmed to be RCC on immunohistochemistry. On further radiological examination, an exophytic mass was depicted in the kidney's upper and middle pole. The patient had no renal complaints and was asymptomatic. Fronto-ethmoidal sinus is a rare site for metastatic RCC, especially in cases where the patient is asymptomatic. Early detection by keeping RCC metastasis as the differential diagnosis in such cases can lead to early treatment and improve the overall survival of the patient.

Bellini duct carcinoma

The modern era has brought an appreciation that renal cell carcinoma (RCC) includes diverse subtypes derived from the various parts of the nephron, each with its distinctive genetic basis and tumor biology. Carcinoma of the collecting ducts of Bellini (CDC) is a rare subtype of RCC, with a predictably poor prognosis. This rare subtype represents less than 1% of all kidney carcinomas. It derives from presumably numerous chromosomal losses. It is of chief importance to differentiate CDC from other types of renal cell cancer. Typically, it is characterized by a firm, centrally located tumor with infiltrative borders. Regarding the histopathologic characteristics, we can find complex, highly infiltrative cords with inflamed (desmoplastic) stroma, with high-grade nuclei and mitoses. Most reported cases of CDC had been high grade, advanced stage, and unresponsive to conventional therapies. This rare form of disease highlights the importance of multidisciplinary teams in the management of cancer patients.

Metastasis from tongue squamous cell carcinoma to the kidney

Metastasis to the kidney from other primary sites is extremely rare. Previous studies reported the lung as the most common primary site. Distant metastasis from the tongue to the kidney is exceedingly rare. Herein, we describe a case of metastatic squamous cell carcinoma to the kidney in a 71-year-old male with a detailed discussion of differentiating it from potential mimickers. The patient underwent a total glossectomy and bilateral cervical lymph node dissection. A diagnosis of well-differentiated squamous cell carcinoma of the tongue was rendered and the tumor was staged pT3 pN3b. Within two years of initial presentation, the patient developed widely metastatic disease, including pulmonary nodules, renal masses, left adrenal mass, and pancreatic mass. Accurate diagnosis of a secondary involvement of the kidney by a metastatic tumor requires the appropriate correlation of clinical and imaging findings as well as morphologic and immunohistochemical clues.

How far is too far? Exploring the indications for robotic partial nephrectomy in a highly complex kidney tumor

ABSTRACT Purpose: The conservative management of localized renal masses has been recently widened to cT2 tumors showing encouraging functional and oncological outcomes (1). This video aims to report the conservative management of a highly complex renal tumor treated with robotic pure enucleation in our center, specifically focusing on preoperative work-up, video-reported surgical steps and perioperative outcomes. Materials and Methods: A 63 year-old lady underwent CT scan revealing a single 75 x 68mm, mainly endophytic, right renal mass dislocating the vascular pedicle (cT3a). Two renal arteries and two veins were identified. PADUA, RENAL and simplified SPARE scores were 14a, 12a and 12 respectively. Since the contralateral kidney was hypotrophic, the indication for nephron-sparing approach was considered absolute. Preoperative surgical planning included the employment of 3D-virtual models (2). Results: Operative time was 150 minutes and warm ischemia time was 25 minutes. No major complication occurred. Histopathological analysis revealed a cromophobe renal cell carcinoma with extension to perirenal fat tissue (pT3a). Resection technique was classified as pure enucleation since Surface-Intermediate-Base (SIB) score was 0-0-0 (3, 4). At seven-months follow-up no signs of local or systemic recurrence were recorded. Postoperative CT-scan revealed optimal parenchymal volume preservation with last creatinine blood level of 1.16mg/dL. Conclusion: This video highlights how, in experienced hands, robotic partial nephrectomy represents a feasible, effective treatment option for surgical management of highly complex renal tumors. The employment of intraoperative ultrasonography and 3D-virtual models allowed to accurately tailor surgical approach, improving the perception of tumor anatomy and its vascularization and maximizing perioperative outcomes.

Diagnostic accuracy of contrast-enhanced ultrasound for detecting bland thrombus from inferior vena cava tumor thrombus in patients with renal cell carcinoma

ABSTRACT Purpose: To evaluate the role of contrast-enhanced ultrasound (CEUS) in differentiating bland thrombus from tumor thrombus of the inferior vena cava (IVC) in patients with renal cell carcinoma (RCC). Materials and Methods: We retrospectively investigated 30 consecutive patients who underwent robot-assisted radical nephrectomy with IVC thrombectomy and had pathologically confirmed RCC. All patients underwent US and CEUS examination. Two off-line readers observed and recorded thrombus imaging information and enhancement patterns. Sensitivity, specificity, accuracy, positive predictive value and negative predictive value for bland thrombus were assessed. Results: Of the 30 patients, no adverse events occurred during administration of the contrast agent. Early enhancement of the mass within the IVC lumen on CEUS was an indicator of tumor thrombus. Bland thrombus showed no intraluminal flow on CEUS. There were eight (26.7%) patients with bland thrombus, including three level II, two level III, and three level IV. There were three cases with cephalic bland thrombus and five cases with caudal bland thrombus. Three caudal bland thrombi extended to the iliac vein and underwent surgical IVC interruption. Based on no intraluminal flow, for bland thrombus, CEUS had 87.5% sensitivity, 100% specificity, 96.7% accuracy, 100% positive predictive value and 95.6% negative predictive value. Conclusion: Our study demonstrates the potential of CEUS in the differentiation of bland and tumor thrombus of the IVC in patients with RCC. Since CEUS is an effective, inexpensive, and non-invasive method, it could be a reliable tool in the evaluation of IVC thrombus in patients with RCC.

Computed tomography imaging characteristics of clear cell papillary renal cell carcinoma

ABSTRACT Purpose: Clear cell papillary (CCP) renal cell carcinoma (RCC) is a new subtype of RCC that was formally recognized by the International Society of Urological Pathology Vancouver Classification of Renal Neoplasia in 2013. Subsequently, CCP RCC was added to the 2016 World Health Organization Classification of Tumors of the Urinary System and Male Genital Organs. In this study, we retrospectively investigated the computed tomography (CT) findings of pathologically diagnosed CCP RCC. Materials and Methods: This study included 12 patients pathologically diagnosed with CCP RCC at our institution between 2015 and 2017. We reviewed the patient's CT data and analyzed the characteristics. Results: Nine solid masses and 3 cystic masses with a mean tumor size of 22.7±9.2mm were included. Solid masses exhibited slight hyper-density on unenhanced CT with a mean value of 34±6 Hounsfield units (HU), good enhancement in the corticomedullary phase with a mean of 195±34HU, and washout in the nephrogenic phase with a mean of 133±29HU. The walls of cystic masses enhanced gradually during the corticomedullary and nephrogenic phases. Solid and cystic masses were preoperatively diagnosed as clear cell RCC and cystic RCC, respectively. Conclusions: The CT imaging characteristics of CCP RCCs could be categorized into either the solid or cystic type. These masses were diagnosed radiologically as clear cell RCC and cystic RCC, respectively.

Evaluating the clinical significance of SHMT2 and its co-expressed gene in human kidney cancer

BACKGROUND: Kidney cancer is one of the most common cancers in the world. It is necessary to clarify its underlying mechanism and find its prognostic biomarkers. Current studies showed that SHMT2 may be participated in several kinds of cancer. METHODS: Our studies investigated the expression of SHMT2 in kidney cancer by Oncomine, Human Protein Atlas database and ULCAN database. Meanwhile, we found its co-expression gene by cBioPortal online tool and validated their relationship in A498 and ACHN cells by cell transfection, western blot and qRT-PCR. Besides these, we also explored their prognostic values via the Kaplan-Meier plotter database in different types of kidney cancer patients. RESULTS: SHMT2 was found to be increased in 7 kidney cancer datasets, compared to normal renal tissues. For the cancer stages, ages and races, there existed significant difference in the expression of SHMT2 among different groups by mining of the UALCAN database. High SHMT2 expression is associated with poor overall survival in patients with kidney cancer. Among all co-expressed genes, NDUFA4L2 and SHMT2 had a high co-expression efficient. SHMT2 overexpression led to the increased expression of NDUFA4L2 at both mRNA and protein levels. Like SHMT2, overexpressed NDUFA4L2 also was associated with worse overall survival in patients with kidney cancer. CONCLUSION: Based on above results, overexpressed SHMT2 and its co-expressed gene NDUFA4L2 were all correlated with the prognosis in kidney cancer. The present study might be benefit for better understanding the clinical significance of SHMT2 and provided a potential therapeutic target for kidney cancer in future.

Metanephric Adenoma with cystic changes- An uncommon presentation of a rare tumor in a young adult

Metanephric adenoma (MA) is a rare benign neoplasm of the kidney that is usually asymptomatic and incidentally diagnosed. MA usually present as a solid mass; however, a cystic presentation has been reported. The main differential diagnosis of MA is the epithelial predominant Wilms tumor (e-WT) and the solid variant of papillary renal cell carcinoma (pRCC). The presence of the BRAF gene mutation has recently been reported in 85% of MA, and less than 10% of cases of MA do not express this specific gene mutation. Herein we report a 22-year-old man who presented with back pain and abdominal discomfort with a renal mass on the computed tomographic scan. The diagnosis of metanephric adenoma was confirmed histopathologically. In our case, the tumor presented as a solid and cystic mass hence mimicking a papillary renal cell carcinoma. The VE1 protein, which correlates with BRAF gene mutation, did not show any significant expression. We want to highlight that MA can present as a cystic lesion that should be taken into account to avoid unnecessary radical nephrectomy. Also, we demonstrated that a subset of MA might not harbor the BRAF gene and, they are classified as the BRAF wild type MA.

Metástasis bilateral de húmero, en paciente con cáncer renal, tratado con inmunoterapia y cirugía: reporte de un caso / Bilateral Humeral metastases, in a patient with renal cancer, treated with immunotherapy and surgery: case report

El carcinoma de células renales es el 7mo cáncer en frecuencia a nivel mundial con más de 300.000 casos nuevos al año y es la 3era malignidad genitourinaria más frecuente. El sitio más común de metástasis es el pulmón mientras que el esqueleto ocupa el segundo lugar con una frecuencia que varía entre un 20% a un 35%. Se ha reportado una sobrevida de 12 meses en promedio luego de la aparición de metástasis óseas. Reporte del caso de un paciente con diagnóstico de cáncer renal de células claras y metástasis óseas (en columna, pelvis y ambos húmeros), que evoluciona con fractura de ambos brazos. El paciente es aceptado en un ensayo clínico de tratamiento con inmunoterapia y además se somete a una reducción y osteosíntesis bilateral de húmero con clavo endomedular, logrando la consolidación de ambas fracturas y, por ende, la regresión de la enfermedad.

Renal cell carcinoma is ranked 7th in frequent cancer worldwide with more than 3000.000 new cases per year, as well as it's ranked 3rd in frequent genitourinary malignancy. The most common area of metastases is lung followed by skeleton in second place. The frequency of skeleton metastases varies from 20% to 35%. A survival average time of 12 months is generally observed after the appearance of bone metastases. This case report is about a patient diagnosed with clear cell renal cancer and bone metastases (in the spine, pelvis and both humerus) that evolves with fracture of both arms. The patient undergoes a clinical trial with immunotherapy and also a reduction and osteosynthesis of both arms with intramedullary nail, achieving consolidation of both fractures and regression of the disease.

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.

Computed Tomography Imaging Features of IgG4-related Nephropathy / 中国医学科学院学报

Objective To investigate the computed tomography(CT)imaging features of IgG4-related kidney disease(IgG4-RKD).Methods The clinicopathological and imaging data of 36 IgG4-RKD patients(including 26 cases of renal parenchyma,10 cases of renal pelvis,24 cases of double kidney or multiple lesions,and 12 cases of single focus)were retrospectively analyzed.Results IgG4-RKD had specific clinicopathological and imaging features.Although the kidney or renal pelvis was involved,there were no common clinical manifestations of malignant tumors such as hydronephrosis and hematuria.The boundary was clear and complete,and it had typical characteristics of continuous progressive enhancement.The peak value was mostly in the solid and excretory phase,and there were no imaging manifestations such as liquefaction,necrosis,cystic degeneration,and calcification.Even if the renal pelvis was obviously involved and the focus was large,there was no invasion of the peripheral renal parenchyma,the shape of the renal pelvis still existed,the wall was smooth,and there was no enlarged peripheral lymph nodes.Conclusions CT images of IgG4-RKD are similar to those of renal malignant tumors.Based on clinicopathological features,CT findings,and blood IgG4-related tests,a definite diagnosis can be made and unnecessary operation can be avoided.

Intra-tumor genetic heterogeneity in Wilms tumor samples

SUMMARY Childhood renal tumors account for ~7% of all childhood cancers, and most cases are embryonic Wilms' tumors (WT). Children with WT are usually treated by either COG or SIOP. The later treats the children using preoperative chemotherapy, but both have around 90% of overall survival in five years. WT is a genetically heterogeneous group with a low prevalence of known somatic alterations. Only around 30% of the cases present mutation in known genes, and there is a relatively high degree of intra-tumor genetic heterogeneity (ITGH). Besides potentially having an impact on the clinical outcome of patients, ITGH may interfere with the search for molecular markers that are prospectively being tested by COG and SIOP. In this review, we present the proposal of the current UMBRELLA SIOP Study 2017/Brazilian Renal Tumor Group that requires the multi-sampling collection of each tumor to better evaluate possible molecular markers, as well as to understand WT biology

RESUMO Os tumores renais pediátricos correspondem a aproximadamente 7% de todos os tumores infantis, sendo o mais frequente o tumor de Wilms (TW). Crianças com TW são geralmente tratadas seguindo dois distintos protocolos terapêuticos (COG ou SIOP), sendo que no último, os pacientes recebem tratamento quimioterápico pré-operatório. Ambos apresentam sobrevida global em cinco anos em torno de 90%. TW é geneticamente heterogêneo, apresentando baixa prevalência de alterações somáticas conhecidas, com cerca de 30% dos casos apresentando mutações em genes conhecidos e um alto grau de heterogeneidade genética intratumoral (HGIT). Além de potencialmente ter um impacto sobre o desfecho clínico dos pacientes, a HGIT pode interferir na busca de marcadores moleculares que estão sendo testados prospectivamente pelos grupos COG e Siop. Nesta revisão, apresentamos a proposta do atual estudo Umbrella Siop 2017/Grupo de Tumores Renais Brasileiros (GTRB), que orienta a coleta de três diferentes regiões do tumor para melhor avaliar possíveis marcadores moleculares, bem como para compreender a biologia do TW.

Right atrium tumor extension through the inferior vena cava. considerations about nine cases operated under cardiopulmonary bypass

Abstract Introduction: Adrenocortical and renal cell carcinomas rarely invade the right atrium (RA). These neoplasms need surgical treatment, are very aggressive and have poor prognostic and surgical outcomes. Case series: We present a retrospective cohort of nine cases of RA invasion through the inferior vena cava (four adrenocortical carcinomas and five renal cell carcinomas). Over 13 years (2002-2014), nine patients were operated in collaboration with the team of urologists. Surgery was possible in all patients with different degrees of technical difficulty. All patients were operated considering the imaging examinations with the aid of CPB. In all reported cases (renal or suprarenal), the decision to use CPB with deep hypothermic circulatory arrest (DHCA) on surgical strategy was decided by the team of urological and cardiac surgeons. Conclusion: Data retrospectively collected from patients of public hospitals reaffirm: 1) Low incidence with small published series; 2) The selected cases did not represent the whole historical casuistry of the hospital, since they were selected after the adoption of electronic documentation; 3) Demographic data and references reported in the literature were presented as tables to avoid wordiness; 4) The series highlights the propensity to invade the venous system; 5) Possible surgical treatment with the aid of CPB in collaboration with the urology team; 6) CPB with DHCA is a safe and reliable option; 7) Poor prognosis with disappointing late results, even considering the adverse effects of CPB on cancer prognosis are expected but not confirmed.

Thyroid-like follicular carcinoma of the kidney presenting on a ten year-old prepubertal girl

ABSTRACT The very rare thyroid-like carcinoma of the kidney (TLCK) is microscopically similar to thyroid follicular cell carcinoma (TFCC). Differential diagnosis with secondary thyroid tumors depends on non-reactivity to immunohistochemical (IHC) markers for TFCC (thyroglobulin - TG and TTF1). We herein describe the fourth Pediatric case in literature and extensively review the subject. Only 29 cases were published to the moment. Most cases were asymptomatic and incidentally detected. Most tumors are hyperechoic and hyperdense with low grade heterogenous enhancement on CT and MRI. Most patients were treated with radical nephrectomy, but partial nephrectomy was used in some cases, apparently with the same results. Metastases are uncommon and apparently do not change prognosis, but follow-ups are limited. Up to the moment, TLCK presents as a low grade malignancy that may be treated exclusively with surgery and frequently with partial kidney renal preservation. A preoperative percutaneous biopsy is a common procedure to investigate atypical tumors in childhood and adult tumors. To recognize the possibility of TLCK is fundamental to avoid unnecessary thyroidectomies in those patients, supposing a primary thyroid tumor.

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.