Rhabdoid Variant of Clear Cell Renal Cell Carcinoma– a Case Report (original) (raw)
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Annals of Diagnostic Pathology, 2011
Renal cell carcinoma (RCC) with rhabdoid morphology (RCC-RM) is a recently described variant of RCC, which has an aggressive biologic behavior and poor prognosis, akin to sarcomatoid RCC. The current World Health Organization classification of RCC does not include the rhabdoid phenotype as a distinct histologic entity. The aim of this study is to investigate whether RCC-RM represents a dedifferentiation of a classifiable-type World Health Organization RCC or a carcinosarcoma with muscle differentiation. We reviewed 168 cases of RCC obtained between 2003 and 2008. From these cases, 10 (6%) were found to have areas of classic rhabdoid morphology. Immunohistochemistry for cytokeratin, epithelial membrane antigen, desmin, CD10, and CD117 was performed in each case using the labeled streptavidin-biotin method. Rhabdoid differentiation was identified in association with conventional-type RCC (9) and with unclassifiable-type RCC with spindle cell morphology (1). In all cases, both the rhabdoid and nonrhabdoid tumoral areas were positive for cytokeratin and epithelial membrane antigen and negative for desmin. Cytokeratin positivity in the rhabdoid areas was focal. In cases associated with conventional-type RCC, CD10 was positive in both the rhabdoid and nonrhabdoid foci. CD117 was negative in these tumors. The unclassifiable-type RCC with spindle cell morphology was negative for both CD10 and CD117. The similar immunophenotype between the rhabdoid and nonrhabdoid tumoral foci supports the origin of the rhabdoid cells from the classifiabletype RCC. Areas of rhabdoid morphology do not represent muscle metaplastic differentiation. Renal cell carcinoma with rhabdoid morphology may represent a dedifferentiation of a classifiable-type RCC, similar to that of sarcomatoid differentiation. The recognition of RCC-RM is important as it allows for the inclusion of these high-grade malignancies into a category associated with poor prognosis despite lacking the spindle cell component classically identified as sarcomatoid change.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2015
Rhabdoid histology in clear-cell renal cell carcinoma is associated with a poor prognosis. The prognosis of patients with clear-cell renal cell carcinoma may also be influenced by molecular alterations. The aim of this study was to evaluate the association between histologic features and salient molecular changes in rhabdoid clear-cell renal cell carcinoma. We macrodissected the rhabdoid and clear-cell epithelioid components from 12 cases of rhabdoid clear-cell renal cell carcinoma. We assessed cancer-related mutations from eight cases using a clinical next-generation exome-sequencing platform. The transcriptome of rhabdoid clear-cell renal cell carcinoma (n=8) and non-rhabdoid clear-cell renal cell carcinoma (n=37) was assessed by RNA-seq and gene expression microarray. VHL (63%) showed identical mutations in all regions from the same tumor. BAP1 (38%) and PBRM1 (13%) mutations were identified in the rhabdoid but not in the epithelioid component and were mutually exclusive in 3/3 c...
Malignant rhabdoid tumour of kidney - a rare aggressive tumour
Journal of Clinical and Scientific Research, 2016
Malignant rhabdoid tumour of kidney is a rare highly aggressive neoplasm of childhood. We present the case of a 18- months old girl presenting with decreased appetite, abdominal distention of 20 days duration and 3 episodes of haematuria. The patient underwent left radical nephrectomy and histopathological examination of the excised specimen confirmed the diagnosis of malignant rhabdoid tumour of the kidney. This case highlights the need to consider malignant rhabdoid tumour of the kidney of possibility young children in presenting with a renal mass.
Chromophobe renal cell carcinoma with rhabdoid differentiation in an adult
Wiener klinische Wochenschrift, 2012
Your article is protected by copyright and all rights are held exclusively by Springer-Verlag Wien. This e-offprint is for personal use only and shall not be self-archived in electronic repositories. If you wish to selfarchive your work, please use the accepted author's version for posting to your own website or your institution's repository. You may further deposit the accepted author's version on a funder's repository at a funder's request, provided it is not made publicly available until 12 months after publication. case report Chromophobe renal cell carcinoma with rhabdoid differentiation in an adult 419 1 3 Chromophobes renales Zellkarzinom mit rhabdoider Differenzierung bei einem Erwachsenen Zusammenfassung In der Literatur wurde bisher über Fälle von Nierenkrebs mit rhabdoider Differenzierung normalerweise im Zusammenhang mit Klarzellkarzinomen und Pappilarkarzinomen berichtet. In der zugänglichen Literatur findet man nur einen Bericht von chromophobem Karzinom mit rhabdoider Differenzierung. Wir berichten über den Fall eines 47 Jahre alten männlichen Patienten, der einen routinemäßigen Checkup durchführte. Eine Computer-Tomographie ergab eine große heterogene Masse in der linken Niere, multiple noduläre Veränderungen in beiden Lungenflügeln und multiple vergrößerte Lymphknoten. Mikroskopische Untersuchungen der linken Niere zeigten, dass 65 % des Nierentumors aus rhabdoiden Bereichen bestanden und der Rest des Tumors eosinophile Bereiche aufwies, die mit einem chromophoben renalen Zellkarzinom konsistent waren. Die endgültige Diagnose ergab Chromophobes renales Zellkarzinom mit rhabdoider Differenzierung. Bei Erwachsenen zeigt diese Art von Tumoren eine erhöhte Aggressivität, erfordert aggressivere Behandlung und weist schlechte Erfolgsaussichten auf. Daher sollte rhabdoide Differenzierung im Zusam-menhang mit dieser Art von Karzinomen erkannt und im pathohistologischen Bericht festgestellt werden.
Clinical and imaging features of rhabdoid tumor of the kidney
Urologic Radiology, 1991
Rhabdoid tumor of the kidney (RTK) is a rare, highly malignant neoplasm of childhood. The clinical profile of this neoplasm differs from that of Wilms’ tumor. We present two cases of RTK. In both our cases, large bulky masses with poorly defined margins and calcifications were demonstrated. The clinical and imaging findings are compared with other childhood renal neoplasms.
Aggressive uncommon pediatric renal tumor: Rhabdoid tumor of kidney
Indian Journal of Pathology and Oncology
Rhabdoid tumor of kidney is one of the uncommon pediatric renal neoplasms with highly aggressive clinical course. We are presenting a case of 1 year child with abdominal distension and hematuria. On Contrast enhanced computed tomography (CECT) a heterogenous mass in lower pole of kidney was detected. Histopathological and immunohistochemical study after radical nephrectomy revealed Malignant rhabdoid tumor of kidney. It has a tendency to metastasize early and it is associated with synchronous presence of other malignancies. No definite effective treatment for this tumor is available till now. This further necessitates the early diagnosis and prompt management which can be life saving for the patient. We are presenting this case to emphasize on its rarity and aggressive clinical behaviour.
Originally misdiagnosed rhabdoid tumour of the kidney. A case report and differential diagnosis
Polish journal of pathology : official journal of the Polish Society of Pathologists, 2011
Rhabdoid tumour of the kidney (RTK) is considered to be one of the most aggressive neoplasms of early life. The histogenesis of RTK still remains a matter of controversy. Immunohistochemistry usually shows diffuse reactivity for vimentin, focal reactivity to the epithelial marker, variable expression of mesenchymal and neuroectodermal markers, and loss of INI1 protein staining. Expression of the Wilms' tumour protein (WT1) was described in the RTK cases. We would like to present a case of rhabdoid tumour of the kidney in Latvia, which caused diagnostic difficulties of a 27-month-old girl, and a short review of literature.
Treatment of adult rhabdoid renal cell carcinoma with sorafenib
Canadian Urological Association journal = Journal de l'Association des urologues du Canada, 2008
Renal cell carcinoma (RCC) with rhabdoid features is an uncommon and highly aggressive malignancy. We report a case of adult clear-cell RCC with extensive rhabdoid features treated with the tyrosine kinase inhibitor sorafenib. A review of the literature summarizes important aspects of this malignancy. We discuss clinical and histological findings as well as the patient's response to sorafenib therapy.
Radiology–Pathology Conference: rhabdoid tumor of the kidney
Clinical Imaging, 2006
Rhabdoid tumor of the kidney (RTK) is a rare pediatric malignancy. Its imaging features by ultrasound and CT have been well described; however, to our knowledge, there are only a handful of case reports that describe the MR appearance of rhabdoid tumor. We report the MR appearance of a RTK in an 18-month-old boy, review the literature on its imaging features, and discuss the differential diagnosis of solid renal tumors in children. D