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Pediatric cystic nephroma is a rare, clinically benign, renal tumor. Pediatric renal cystic lesions are complex. Imaging findings and tumor appearance are often nonspecific, and careful pathological examination is necessary. We discuss diagnosis of pediatric cystic nephroma and how to differentiate it from multicystic dysplastic kidney and cystic partially differentiated nephroblastoma.
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El nefroma mesoblastico congénito es un tumor renal infrecuente, pero común en el periodo neonatal. De comportamiento benigno y pronóstico excelente en su variedad clásica pero en el subtipo celular puede presentar recurrencia local, invasión retroperitoneal y metástasis pulmonares y cerebrales. El tratamiento es la nefrectomía radical. Los factores pronósticos son la edad menor a tres meses y la resección quirúrgica completa. Paciente de 17 días nacida a las 35 semanas por polihidramnios, con diagnóstico prenatal de masa abdominal. Al examen físico masa en flanco derecho e hipertensión arterial (121/79 mm Hg, TAM 77mm Hg). Ecografía abdominal con riñón derecho aumentado de tamaño y voluminosa formación sólida, de 4,4 x 4,3 x 4,8 cm, heterogénea y vascularizada al Doppler en polo inferior. Tomografía de abdomen con formación nodular heterogénea de 5,0 x 4,1 x 5,1 cm en polo inferior del riñón derecho. Se realizó nefrectomía radical derecha sin complicaciones. Evolución en los últimos 20 meses satisfactoria y libre de enfermedad.
Congenital mesoblastic nephroma is an uncommon renal tumor, but frequent in neonates. Classical variety usually benign with excellent prognosis. Cellular cases may be recurrent with retroperitoneal invasion, brain and lung metastasis. Radical nephrectomy is the treatment of choice. Relevant prognostic factors are diagnosis before three months of age and complete resection. A 17 day old patient, borned at 35 weeks due to polyhydramnios, with prenatal diagnosis of abdominal mass. On examination, right flank abdominal mass and arterial hypertension (121/79 mm Hg, MAP 77 mm Hg). Abdominal ultrasound showed an enlarged right kidney with a voluminous solid heterogeneous mass, 4.4 x 4.3 x 4.8 cm in the lower pole, vascularized on Doppler scan. Computed tomography showed a big 5.0 x 4.1 x 5.1 cm, nodular, heterogeneous mass in the lower pole of the right kidney. Right radical nephrectomy was performed with an uneventful recovery. Follow up for the last 20 months satisfactory and free of disease.
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Nephroma, MesoblasticABSTRACT
Objective:To analyze the characteristics of prenatal MRI and postnatal CT images of fetal congenital mesoblastic nephroma (CMN).Methods:We retrospectively recruited three women with a singleton pregnancy suspected of having fetal CMN by prenatal imaging examination and confirmed by postpartum surgical resection and pathological examination at Guangzhou Women and Children Medical Center from August 2017 to April 2019. The prenatal MRI, postnatal CT, and pathological examination data were analyzed to summarize the image features of fetal CMN using descriptive statistical analysis.Results:(1) The total number of deliveries in the setting during the same period was 57 716, and the incidence of CMN was 0.005% (3/57 716). All the cases were male and born through cesarean section due to polyhydramnios for case 1 and 2 and sudden fetal distress, fetal pericardial effusion with enlarged mass in the third trimester for case 3. (2) Prenatal MRI examination found that the lesions in case 1 and 3 were located in the left kidney, and in the right kidney in case 2. The tumors in the three cases were all around with clear borders and hyperintense signal on T2-weighed imaging (T2WI). Iso-signal on T1WI was noted for case 1 and 2, a slightly hypointense signal on T1-weighed imaging (T1WI), and a hyperintense signal on diffused weighed imaging were noted for case 3. (3) Postnatal CT plain scan showed uniform density tumor in cases 1 and 2 with "packing sign" in CT enhancement. Non-uniform density, internal, larger and high-density bleeding tumor was found in case 3, and the CT enhancement manifested as nodular, non-uniform, and intratumoral bleeding. (4) Postoperative pathological examination revealed that cases 1 and 2 were classic types, and case 3 was cellular type.Conclusions:In this study, the prenatal MRI characteristic of CMN is a uniform signal, iso-signal on T1WI for classic type and non-uniform signal, and reduced signal on T1WI for cellular type. In classic type cases, postnatal CT enhancement showed a "packing sign." In contrast, in cellular type cases, the CT plain scan is manifested as non-uniform, intratumoral bleeding, and the enhancement pattern showed nodular and non-uniform enhancement. However, the results of this study is limited due to the small sample size and studies with larger sample size is needed in the future.
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Objective: To explore the clinicopathological features and the diagnostic value of the CT scan in multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) and cystic nephroma (CN). Methods: The clinical data of 12 patients with CN and 20 patients with MCRNLMP, confirmed by pathology at the Renmin Hospital of Wuhan University and Jingzhou Central Hospital from Janu-ary 2000 to March 2019, were retrospectively analyzed. The receiver operating characteristic (ROC) curves were used to analyze the feature of contrast-enhanced CT images of the tumors, and the immunophenotypes of the tumors were observed by immunohisto-chemistry. Results: There were statistically significant differences between MCRNLMP and CN in terms of thickness of the cyst wall and partition, number of soft-tissue enhancing masses, peak intensity of enhancement, and the Bosniak classification (P<0.05). Based on ROC curve analyses, when the thickness of the capsule wall and partition was greater than 2.25mm, the number of enhanced high-density lesions was greater than 1, and the peaking intensity of fortification was above the moderate level in the diagnosis of CRNLMP. The areas under the curve of the three indexes were 0.879, 0.800 and 0.838, which can be used as the best diagnostic criterion for MCRNLMP. Immunophenotyping revealed that MCRNLMP characteristically expressed the renal cell carcinoma (RCC) marker, and CN characteristically expressed the estrogen receptor(ER) and progesterone receptor(PR). Conclusions: The cyst wall and septal thickness, number of soft-tissue enhancing masses, and peak intensity of enhancement show a higher diagnostic value in differentiating MCRN-LMP and CN. The precise diagnosis relies on the pathological and immunohistochemical examination.
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To discuss clinicopathological features and molecular genetic change of congenital mesoblastic nephroma (CMN).Methods Nine cases diagnosed as CMN were analyzed retrospectively in this study.Histological features,immunohistochemical profiles and ETV6 gene rearrangement status were assessed.Results All patients were within two years of age and eight of them were within one year.The average diameter of tumors was 9.5 cm (3.2-15.0 cm).These series cases included 3 classic CMN,5 cellular CMN and 1 mixed CMN.Cystic degeneration was found in 5 cases,and cartilage islands were observed in 2 cases.Compared with classic CMN,tumor size was bigger,and hemorrhage,necrosis and mitotic figures were easily to see in cellular CMN.All the tumor cells were positive for vimentin and negative for WT-1 by immunohistochemistry.ETV6 gene rearrangement was detected in 5 cases (including 4 cellular CMN and 1 classic CMN).Three cellular CMN harbored ETV6 gene translocation,1 mixed CMN and 1 cellular CMN were negative for ETV6 gene translocation by FISH analysis.The follow up data were obtained in 7 cases and 2 cases were lost.All the 7 patients were alive without evidence of recurrence and metastasis from 5 to 46 months.Conclusion CMN is a rare infant renal tumor with unique clinicopathological characteristics.Most of cellular CMNs harbor ETV6 gene translocation.The prognosis of CMN is relative good and needs to be differentiated from other malignant renal tumors.
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Objective To discuss the diagnostic performance of MSCT in cystic nephroma(CN).Methods The MSCT findings of CN in 7 patients proved by operation and pathology were retrospectively reviewed.Results All of 7 cases were isolated cystic lesion, including 5 males and 2 females.MSCT scanning showed cystic tumor in the renal parenchyma with well-defined boundary.All cases showed regular cystic wall and septa with no attached cystic wall nodule.The cystic wall and septa appeared homogeneous enhancement on contrast-enhanced CT,while the cystic space showed inhomogeneous density and lack of enhancement.Conclusion The MSCT findings of CN may benefit the definite diagnosis.
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Objective Cystic nephroma ( CN ) is an unusual benign neoplasm with high misdiagnosis rate , and at present there is no general method on its treatment .This study aimed to analyze the diagnosis and treatment of CN based on clinical data of CN patients. Methods We retrospectively analyzed the clinical data on 25 patients (including 2 cases of male pediatric patients , aged 14 and 16 years old, and the remaining 23 cases were adults, 11 males and 12 females, aged 14-69[45.1 ±19.6]years) treated in our department of the First Hospital of Shijiazhuang from January 2003 to July 2015 .All patients underwent ultrasound , CT and MRI examination , as well as surgical resection . Results Among these 25 CN patients , there were 15 cases of partial nephrectomy , 5 ca-ses of nephrectomy , 2 cases of retroperitoneal laparoscopic cyst unroofing , 1 case of laparoscopic enucleation of the tumors with nephron-sparing surgery , 1 case of laparoscopic radical nephroureterectomy , and 1 case of retroperitoneal laparoscopic radical nephrectomy .All the patients were successfully followed up for 6 months to 132 months, 1 patient recurred 6 months after retroperitoneal laparoscopic cyst unroo-fing and underwent open partial nephrectomy .No recurrence and me-tastasis were found in the remaining patients . Conclusion Imaging examination is an important measurement for CN , and intraoperative frozen pathology contributes to pathological diagnosis .Since most CN cases are benign , CN patients with no symptom or small cysts can take follow up survey .The principle of the operation is complete resection of the tumor , and nephron-sparing surgery is the first choice . In addition , regular follow-up is necessary in case of recurrence and malignant potential .
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Abstract A male child born at 27 weeks, weighting 1305 g and presenting with a right-sided abdominal tumor. Computed tomography scan demonstrated the presence of a solid mass compressing the right kidney. Puncture biopsy revealed congenital mesoblastic nephroma. The patient underwent total right nephroureterectomy, and died on the second day after surgery.
Resumo Criança do gênero masculino, nascida com 27 semanas e 1.305 g de peso, apresentando tumor abdominal à direita. Tomografia computadorizada revelou formação sólida comprometendo o rim direito. Foi realizada biópsia por punção, cujo diagnóstico foi nefroma mesoblástico congênito. O paciente foi submetido a nefroureterectomia total direita, falecendo no segundo dia de pós-operatório.
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Congenital mesoblastic nephroma is a rare renal tumor that is diagnosed during pregnancy and is associated with polyhydramnios, prematurity, and neonatal hypertension. Differential diagnoses include Wilms tumor, adrenal neuroblastoma, and other abdominal tumors. We report a case of congenital mesoblastic nephroma detected by prenatal ultrasonography as a large fetal renal mass with polyhydramnios at 32 weeks of gestation. Ultrasonography showed a 6x6-cm complex, solid, hyperechoic, round mass in the right kidney. At 35 weeks of gestation, the patient was admitted with preterm premature rupture of membranes and the baby was delivered vaginally. Postnatal ultrasonography and computed tomography showed a heterogeneous solid mass on the right kidney. At the end of the first week of life, a right nephrectomy was performed and subsequent pathological examination confirmed a cellular variant of congenital mesoblastic nephroma with a high mitotic count. Postoperative adjuvant chemotherapy was administered. The newborn was discharged in good condition.
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Humans , Infant, Newborn , Pregnancy , Chemotherapy, Adjuvant , Diagnosis, Differential , Drug Therapy , Hypertension , Kidney , Membranes , Nephrectomy , Nephroma, Mesoblastic , Neuroblastoma , Polyhydramnios , Prenatal Diagnosis , Rupture , Ultrasonography , Ultrasonography, Prenatal , Wilms TumorABSTRACT
Congenital mesoblastic nephroma (CMN) is the most common renal tumor in the first year of life. Here, we present unique findings of cellular variant CMN seen on prenatal and postnatal MRI with diffusion-weighted imaging (DWI).The mass was well-visualized on prenatal MR DWI with diffusion restriction in the solid portions. After excision of the mass, follow-up whole body MRI with DWI helped identify local tumor recurrence with suspicious liver metastasis. This hepatic lesion also showed diffusion restriction.
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Adult , Female , Humans , Infant, Newborn , Pregnancy , Combined Modality Therapy , Diffusion Magnetic Resonance Imaging/methods , Kidney Neoplasms/congenital , Liver Neoplasms/secondary , Nephroma, Mesoblastic/congenitalABSTRACT
INTRODUÇÃO: Nefroma Mesoblástico Con-gênito é uma rara neoplasia renal pediátrica. Apresenta dois subtipos histológicos, clássico e celular, sendo o último de pior prognóstico e responsável por aproximadamente dois terços dos casos. Esse tumor ainda é um desafio diagnóstico aos patologistas devido à similaridade com outras neoplasias pediátricas renais mais frequentes. RELATO DO CASO: Criança do gênero feminino, 2 anos e 9 meses de idade, foi encaminhada a serviço médico com referência em oncologia apresentando massa renal à esquerda. Após nefrectomia, o estudo do espécime mostrou, macroscopicamente, extensa área tumoral granular, brancoacinzentada, ocupando aproximadamente todo o rim, invadindo seio renal, cápsula e gordura perirrenal, com áreas de hemorragia e necrose. Histologicamente, caracterizava-se pela presença de células fusiformes e mitoses, sem atipias celulares. O diagnóstico foi de Nefroma Mesoblástico Congênito subtipo celular e a paciente foi submetida a quimioterapia. Durante o primeiro ano de tratamento, houve recidiva do tumor, apresentando-se irressecável e sem resposta a nova quimioterapia. A paciente foi a óbito aos 4 anos de idade. DISCUSSÃO: O subtipo celular do nefroma mesoblástico tende a ser mais agressivo, apresentando uma taxa de sobrevivência de 85 por cento, comparada com 100 por cento para a variante clássica. Geralmente, a recorrência ocorre no primeiro ano de tratamento, principalmente quando o subtipo é o celular.
INTRODUCTION: Congenital Mesoblastic Nephroma (CMN) is a rare pediatric renal tumor. It comprises two histological subtypes, namely classic and cellular, with the second accounting for two thirds of all cases and being more often associated with poor prognosis. It remains a diagnostic challenge for pathologists due to its similarity with other more frequent pediatric kidney neoplasms. CASE REPORT: We describe the case of a 2-year- old girl who presented with a left renal mass. After nephrectomy, the specimen analysis showed, on gross examination, an extensive, granular and whitish tumor lesion occupying almost the entire kidney, invading the renal sinus, capsule and perirenal fat, with areas of hemorrhage and necrosis. Histologically, it was characterized by ovoid spindle cells, mitoses and no cell atypia, which led to a diagnosis of cellular mesoblastic nephroma. Adjuvant chemotherapy was carried out, but tumor recurrence occurred in the first year, presenting as an unresectable tumor that did not respond to adjuvant chemotherapy and the patient died at 4 years of age. DISCUSSION: The cellular variant tends to be more aggressive, with a survival rate of 85 percent versus 100 percent for the classic variant. Recurrence generally occurs in the first year, particularly with the cellular variant.
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Humans , Female , Child, Preschool , Kidney Neoplasms/congenital , Nephrectomy , Nephroma, Mesoblastic/diagnosis , Nephroma, Mesoblastic/mortality , Nephroma, Mesoblastic/therapy , Kidney Neoplasms/diagnosis , Kidney Neoplasms/mortality , Kidney Neoplasms/pathology , Kidney Neoplasms/therapy , Nephroma, Mesoblastic/congenital , Fatal Outcome , Kidney Neoplasms/diagnosis , Kidney Neoplasms/therapy , Nephroma, Mesoblastic/diagnosis , Nephroma, Mesoblastic/therapyABSTRACT
Cystic nephroma (CN) is a benign cystic neoplasm composed of mixed epithelial and stromal elements. Less than 200 cases have been reported. We had a patient, a 41-year-old woman, who had a huge typical CN. The patient was admitted for a right renal mass that was found incidentally. On laparaoscopic right nephrectomy, there was an encapsulated 7 cm multilocular cystic mass at the upper pole. Microscopically, the cystic wall was lined by a single layer of low cuboidal or hobnail epithelium without a solid area. The thin septa were composed of bland, ovarian type spindle cells. The main differential diagnoses were mixed epithelial and stromal tumor (MEST), low grade multilocular renal cell carcinoma, and tubulocystic carcinoma. The results of immunohistochemical staining were cytokeratin 7/19(+/+) and CD10(-) in lining epithelium, estrogen receptor/progesterone receptor(+/+) in stromal cells. After surgery, she was free of recurrence for 10 months. We report this rare case and compare it with other cystic renal tumors, especially MEST.
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Adult , Female , Humans , Carcinoma, Renal Cell , Diagnosis, Differential , Epithelium , Estrogens , Keratins , Kidney , Nephrectomy , Receptors, Estrogen , Recurrence , Stromal CellsABSTRACT
Objective To investigate the features of the mixed epithelial and stromal tumor of kidney (MESTK) and cystic nephroma (CN) on multi-detector CT (MDCT), in order to improve the accuracy of preoperative diagnosis and differential diagnosis. Methods Based on blind-retrospective review, CT features of 6 MESTK patients and 6 CN patients were reviewed retrospectively, and compared with pathological results postoperatively. Results All the lesions were single regular multi-cystic masses. Five patients with MESTK were diagnosed as Bosniak Ⅲ and 1 as Bosniak Ⅳ. In contrast, 6 of CN were all diagnosed as Bosniak Ⅱ. Six MESTK lesions were all consisted of irregular mixture of solid and cystic components, and the average thickness of septa was 0.93 cm. Six CN lesions were entirely cystic masses without solid components, and the average thickness of septa was 0.23 cm. After contrast enhancement, the solid components of MESTK and the cystic wall of CN presented mild-to-moderate delayed enhancement. Conclusion Some signs on multi-detector CT can help to differentiate MESTK and CN, especially the presence of solid components of the tumor.
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Although congenital renal tumors are rare, congenital mesoblastic nephroma (CMN) is the most common renal tumor in early infancy. It is non-metastatic, well differentiated, amenable to surgical removal, and carries a good prognosis. Polyhydramnios has been detected in most of the published cases of CMN. However, we experienced a rare case of fetal CMN associated with oligohydramnios. A 28-yr old woman at 34 weeks of gestation was referred to our hospital for oligohydramnios and a fetal abdominal mass. An ultrasonography revealed a huge, well-encapsulated mass arising from the right kidney. An emergency cesarean section was performed due to fetal distress. After birth, despite intensive neonatal care, the baby died because of renal failure, disseminated intravascular coagulopathy, pulmonary edema, together with other problems.
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Pregnancy , Infant, Newborn , Humans , Female , Adult , Oligohydramnios/diagnosis , Nephroma, Mesoblastic/diagnosis , Kidney Neoplasms/diagnosis , Fetal Diseases/etiology , Fatal Outcome , Cesarean SectionABSTRACT
The descriptive term "mixed epithelial and stromal tumor of the kidney" was recently proposed for a group of renal tumors characterized histologically by a mixture of stromal and epithelial proliferation. It is a rare benign neoplasm of the kidney which has been reported under various names such as adult type mesoblastic nephroma or others. We report a case of mixed epithelial and stromal tumor in a 47 yr old female patient presenting as a partly cystic and partly solid renal mass. Microscopically, the tumor exhibited spindle cell component in solid portion and epithelial proliferation around microcystic areas. Immunoreactive profiles and ultrastructural examination suggested myofibroblastic nature of the stromal cells. We believe this case exemplifies a unique adult renal tumor displaying both epithelial and stromal neoplastic component and has a few unusual features worthy of attention.
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Middle Aged , Humans , Female , Nephroma, Mesoblastic/pathology , Neoplasms, Glandular and Epithelial/pathology , Kidney Neoplasms/pathology , Diagnosis, DifferentialABSTRACT
A multilocular cyst of kidney is a rare pathological entity, which has been reported in the literature under several names. These various names reflect the controversy surrounding their nature. This tumor is traditionally regarded as benign in nature and a nephrectomy has to be performed because of the difficulty in its accurate diagnosis. Malignant recurrence of a multilocular cyst of kidney has an even rarer incidence, with only a few cases having been reported. We report a case of 50-year-old male, with an incidentally detected right renal cystic mass. This mass was pathologically confirmed as a multilocular cyst of kidney after a radical nephrectomy, which locally recurred as a malignant mesoblastic nephroma.
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Humans , Male , Middle Aged , Diagnosis , Incidence , Kidney Diseases, Cystic , Kidney , Nephrectomy , Nephroma, Mesoblastic , RecurrenceABSTRACT
Mesoblastic nephroma is a neoplasm of the kidney which is characterized by interlacing bundles of spindle mesenchymal cells. It is usually diagnosed during the first six months of life and is mostly benign. Incidence in adults is exceedingly rare. In most cases, only total excision is required without postoperative adjuvant therapy, and the rare cases of local recurrence have usually been related to incomplete removal. However, mesoblastic nephroma may behave aggressively, in contrast to a congenital mesoblastic nephroma. Several cases of metastatic mesoblastic nephroma have been previously described. We report herein a case of a 42-year-old woman with mesoblastic nephroma which recurred as a large metastatic lung mass seven years after the nephrectomy. The patient presented with chest wall discomfort for four days. Seven years previously, total nephrectomy had been performed because of a right renal tumor which had been diagnosed as a mesoblastic nephroma. There had been no evidence of recurrence for five years, after which she discontinued follow-up. On readmission two years later, chest X-ray and CT scan revealed a large lung mass in the left upper lobe. It was completely excised and the pathologic examination was identical with that of the original renal tumor. Synovial sarcoma was excluded because the fusion transcripts of the SYT-SSX fusion gene associated with the t(X;18) translocation were negative. The final diagnosis was a lung metastasis of mesoblastic nephroma and the patient remained free of disease for 7 months postoperatively.
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Adult , Female , Humans , Diagnosis , Follow-Up Studies , Incidence , Kidney , Lung , Neoplasm Metastasis , Nephrectomy , Nephroma, Mesoblastic , Recurrence , Sarcoma, Synovial , Thoracic Wall , Thorax , Tomography, X-Ray ComputedABSTRACT
Multilocular cystic nephroma is a rare disease, noninherited benign renal neoplasm occurring in both children and adults. It is necessary to make a differential diagnosis from all renal diseases with a cystic component, such as Wilms tumor, armatoma or polycystic dysplastic kidney in childhood. There are about only 200 case reports in the world since Walter Edmunds had described it first. We report a case of multilocular cystic nephroma presented with painless abdominal mass, treated with nephrectomy and confirmed with pathology.
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Adult , Child , Humans , Diagnosis, Differential , Kidney , Kidney Neoplasms , Nephrectomy , Pathology , Rare Diseases , Wilms TumorABSTRACT
Mesoblastic nephroma is a benign neoplasm of the kidney, which is usually diagnosed during the first six months of life. Incidence in adults is exceedingly rare. We report herein a case of mesoblastic nephroma that occurred in the upper pole of the right kidney of a 39-year-old woman. It was a round mass measuring 3X3X2 cm in dimension. Microscopically, it consisted of uniform spindle cells with foci of hyalinization and dystrophic calcification. Tubular structures were entrapped in the tumor. Upon immunohistochemical staining, the spindle cells were found to be positive for smooth muscle actin, desmin, and vimentin. The epithelial cells of the entrapped tubules were positive for cytokeratin. On electron microscopic examination, the spindle cells demonstrated smooth muscle differentiation identified by indented nuclei, microfilaments beneath the cell membrane, dense bodies, and basal lamina-like materials.