Chromophobe cell renal carcinoma, described first histologically by the german pathologist thoenes 40,77 and subsequently in other studies, 78,80 was described in 1995 in the fna cytology literature. Chromophobe renal cell carcinoma with osseous metaplasia. Fourteenth international kidney cancer symposium miami, florida, usanovember 67, 2015. The following manuscript will endeavor to provide an overview of this uncommon entity including pathology, epidemiology, genetics, clinical aspects, and current and future treatment options. Chromophobe renal cell carcinoma chrcc is a subtype of rcc.
The cancer genome atlas chromophobe renal cell carcinoma. Resection of bulky chromophobe renal cell carcinoma. Aug 19, 2019 chromophobe renal cell carcinoma chrcc, which originates from distal convoluted tubules and cortical collecting ducts, is a distinct subtype of renal cell carcinoma, which accounts for 4% to 6% of renal cell carcinoma subtypes. My first post op cat scan was clear, the second 3 months later showed a 2 cm. Chromophobe renal cell carcinoma rcc, which accounts for 46% of all rccs, is a rare rcc subtype whose cells are thought to differentiate toward the type b intercalated cells of the cortical collecting duct. Metastatic chromophobe renal cell carcinoma clinical. Resection of bulky chromophobe renal cell carcinoma resolved. The pathological examination showed chromophobe renal cell carcinoma. Renal cell carcinoma rcc is a disease composed of multiple histological subtypes. Chromophobe renal cell carcinoma rcc is a rare neoplasm of the kidney that represents about 5% of rccs. This is one of the most problematic distinctions between renal neoplasms and may frequently be impossible.
Chromophobe is a type of renal cell carcinoma rcc, or kidney cancer. Renal cell carcinoma european association of urology. Skinnider, md from the department of pathology, vancouver general hospital and the university of british columbia, vancouver, british columbia, canada. Renal cell carcinoma rcc is a type of kidney cancer. However, crcc with sarcomatoid change crcc s is associated with poor clinical outcomes, with most cases of sarcomatoid rccs being diagnosed at tnm stages iii and iv, and with a median survival time. Chromophobe renal cell carcinoma rcc, which accounts for 46% of all rccs, is a rare rcc subtype whose cells are thought to differentiate toward the type. Chromophobe renal cell carcinoma is a rare type of kidney cancer that forms in the cells lining the small tubules in the kidney. The somatic genomic landscape of chromophobe renal cell carcinoma.
A comparison of colloidal iron staining and electron microscopy brian f. I am still having pains after eating but it is a different type of pain mainly gas and digestive most likely recovering from surgery also the location of the pain is different after surgery. Jul 01, 2015 chromophobe renal cell carcinoma is a rare subtype of the most common form of kidney cancer called renal cell carcinoma rcc. Pdf brain metastasis from chromophobe renal cell carcinoma. Rcc is the most common type of kidney cancer in adults, responsible for approximately 9095% of cases. Irregular nuclei, prominent nucleoli, and nuclear pleomorphism are inherently present in chromophobe rcc. Typical radiological features include a welldefined homogeneous mass and a hypovascular soft tissue enhancement. It is important to identify this entity because it has significantly better prognosis than the clear cell conventional and papillary renal cell carcinomas. It is characterized by a significant number of deletions in many chromosomes, as well as the loss of entire. The most prevalent form, clear cell rcc ccrcc accounts for 65% to 70% of the cases, whereas a heterogeneous group of nonccrcc subtypes1, 2, 3 is responsible for the remainder of the cases chromophobe rcc chrcc is the second most common form of nonccrcc after. Apoptosis in chromophobe renal cell carcinoma and renal oncocytoma.
Palliative tace for hepatic metastases of chromophobe. Renal cell carcinoma rcc is a heterogeneous disease with histologic types that include clear cell rcc, papillary rcc, chromophobe rcc, and renal oncocytomas kovacs et al. The latter commonly shows focal cytoplasmic hci reactivity, but microvesicles have not been previously reported. In the current study, the authors report their experience with crcc over the last 14 years. These two systems use 5 or 6 factors which, when combined, put people into low, intermediate, and highrisk groups. One study examined 6 chromophobe renal cell carcinomas. Differentiation of oncocytoma from chromophobe renal cell. Chromophobe renal cell carcinoma crcc is the third most common histological subtype of rcc and carries a better prognosis than conventional rcc. A novel tumor grading scheme for chromophobe renal cell. Chromophobe renal cell carcinoma rcc is a histologic subtype of rcc that portends a favorable prognosis. In people with bhd syndrome, chromophobe kidney cancer is often slowgrowing, and does not appear to metastasise. Chromophobe renal cell carcinoma with neuroendocrine. Chromophobe renal cell carcinoma rcc is the third most common histologic subtype of rcc after the clear cell and papillary subtypes, comprising approximately 68% of all renal tumors and approximately 410% of all cases of rcc.
Sarcomatoid chromophobe renal cell carcinoma with heterologous sarcomatoid elements. Prognosis of chrcc is better than in other types of rcc. We developed a database that contained 1,870 patients who were diagnosed with renal cell carcinoma rcc and who underwent surgery in our hospital between 2002 and 2010. Eighty six percent of chrccs cases are diagnosed in stage 1 or 2. The somatic genomic landscape of chromophobe renal cell. After the resection, the platelet count was maintained within the normal range without any treatment. We report a case of chromophobe renal cell carcinoma which showed neuroendocrine differentiation. Renal cell carcinoma rcc accounts for 23% of all adult malignancies and is the seventh and twelfth most common malignancy in men and women, respectively. Pdf giant chromophobe renal cell carcinoma sadi turkan. There is limited information on how quickly chromophobe renal cell carcinoma develops. An incidence of chrcc is similar in both men and woman. Despite extensive courses of various systemic targeted chemotherapies, progressive disease was noted on ct and mri and the patient suffered from persistent abdominal pain associated with his metastatic lesions. Chromophobe renal cell carcinoma is a rare subtype of renal cell carcinoma which accounts for 4% to 6% of renal cell carcinoma subtypes.
To our knowledge, this study is the largest radiology series of chromophobe rccs performed with a consistent ct technique. To discuss the pathological and clinical characteristics, treatments and prognosis of chromophobe renal cell carcinoma crcc. Pdf apoptosis in chromophobe renal cell carcinoma and renal. Chromophobe renal cell carcinoma crcc is often associated with a favorable prognosis.
Chromophobe renal cell carcinoma rcc is characterized by diffuse cytoplasmic staining with hale colloidal iron hci and the presence of numerous microvesicles. Chromophobe renal cell carcinoma presenting as a cystic renal. Pathology outlines chromophobe renal cell carcinoma. Chromophobe renal cell carcinoma is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. Differential diagnosis chromophobe renal cell carcinoma. Unlike clear cell renal cell carcinoma, chromophobe is classically caix negative, vimentin negative, ck7 and ckit cd117 positive. These small tubules help filter waste from the blood, making urine. Chromophobe renal cell carcinoma chrcc, which originates from distal convoluted tubules and cortical collecting ducts, is a distinct subtype of renal cell carcinoma, which accounts for 4% to 6% of renal cell carcinoma subtypes. Rcc is a heterogeneous disease comprised of different histological variants including clear cell. Mri features of renal oncocytoma and chromophobe renal. The updated who histological classification offers a subdivision of renal cell carcinoma rcc by both their morphological and cytogenetic features into clear cell, granular cell, chromophobe cell, spindle cell, cystassociated, papillary and collectingduct carcinomas. My urologist thinks all of mass was contained in kidney. This means that chromophobe renal cell carcinoma, or a subtype of chromophobe renal cell carcinoma, affects less than 200,000 people in the us population. Eighty six percent of chrccs cases are diagnosed in stage 1 or.
Chromophobe renal cell carcinoma genetic and rare diseases. Chromophobe renal cell carcinoma chrcc is a subtype of renal cell carcinoma rcc, representing. Chrcc is one of the renal cell carcinomas, a heterogenous group of cancers arising from the kidney nephron. Seeding metastasis of chromophobe renal cell carcinoma after. Chromophobe renal cell carcinoma chrcc is a subtype of renal cell carcinoma rcc. Chrcc is a rare tumor type accounting for approximately 5% of rcc cases 4. Chromophobe renal cell carcinoma symptoms, diagnosis. Chromophobe renal cell carcinoma chrcc is a distinct disease. Brain metastasis from chromophobe renal cell carcinoma. Chromophobe renal cell carcinoma crcc is a rare variant of renal carcinomas arising from the intercalated cells of the distal renal tubule and representing 5% among all renal tumors. Chromophobe renal cell carcinoma chrcc represents 5% of cancers arising from the kidney nephron due in part to its relative rarity, this disease has been understudied at the molecular level comprehensively profiled by tcga, as the first of its rare tumor projects. Treatment outcomes are poorly characterized in patients with metastatic chromophobe renal cell cancer.
Renal cell carcinoma rcc is the most common neoplasm of the kidney representing on average over 90% of all renal malignancies that occur in adults in both sexes. Multiphase mdct enhancement patterns and morphologic features differentiation of subtypes of renal cell carcinoma on helical ct scans jeong kon kim, tae kyoung kim, han jong ahn, chung soo kim, kyurae kim, kyoungsik cho. Initial treatment is most commonly either partial or complete removal of the affected. Chromophobe renal cell carcinoma rcc is the third most common subtype, accounting for about 5% of all rccs. A 70yearold gentleman with history of chromophobe renal cell carcinoma which was treated surgically and followed by adjuvant immunotherapy, presented 5 years later with history of. Jun 21, 2012 chromophobe renal cell carcinoma is a rare variant and accounts for 5% of all cases. Initial treatment is most commonly either partial or. Immunohistochemical analysis of chromophobe renal cell. Chromophobe renal cell carcinoma chrcc is a rare subtype of renal cell carcinoma rcc mainly diagnosed in the sixth decade of life. The ability to achieve confident accurate diagnosis of these renal tumors via nonsurgical means remain elusive until new specific molecular biomarkers are discovered.
Five and 10year disease free survival dfs for chrcc was 83. Efficacy of sunitinib and sorafenib in metastatic papillary. Nuclei tend to be irregular and wrinkled, and cells are sometimes binucleated asterisks. Chromophobe renal cell carcinoma is a rare subtype of the most common form of kidney cancer called renal cell carcinoma rcc. Chromophobe renal cell carcinoma in an 18yearold female. Chromophobe renal cell carcinoma or oncocytoma biomedicine. Renal cell carcinoma rcc is a kidney cancer that originates in the lining of the proximal convoluted tubule, a part of the very small tubes in the kidney that transport primary urine. The tumor was noted to extend into the perirenal fat and to. Useful markers for differential diagnosis of oncocytoma, chromophobe renal cell carcinoma and conventional renal cell carcinoma. The purpose of the study was to evaluate clinical features and prognostic factors in a large single institutional cohort of chromophobe renal cell carcinoma chrcc patients for identification of tumors with the highest metastatic potential. It is controversial whether the fuhrman nuclear grade of chromophobe rcc has prognostic utility. We consider a diagnosis of oncocytic renal neoplasm with a comment that chromophobe carcinoma cannot be completely excluded to be preferable to overdiagnosing a likely benign neoplasm as chromophobe carcinoma. We describe the case of a man with chromophobe renal cell carcinoma chrcc and numerous metastatic lesions restricted to the liver.
It is subdivided into different histopathological types, where clear cell renal carcinoma is being the most common, followed by papillary and chromophobe renal cell carcinoma 1. Outcomes of metastatic chromophobe renal cell carcinoma. Printable chromophobe renal cell carcinoma surgical. Chromophobe renal cell carcinoma presenting as a cystic. Herein we report a case of chromophobe cell renal carcinoma in a 67yearold male patient who. The classic autosomal recessive genetic correlation with chromophobe renal cell carcinoma is birthoggedube syndrome which is associated with a mutation of the hogg gene on chromosome 3p. Clinical diagnostic dilemma and difficult histopathological differentiation of renal oncocytoma from chromophobe renal cell carcinoma still persist. Oct 07, 2009 chromophobe renal cell carcinoma chrcc is a subtype of renal cell carcinoma rcc. The patient underwent a left nephrectomy and a diagnosis of chromophobe renal cell carcinoma was made. Chromophobe renal cell carcinoma usually metastasizes to the liver, lungs, lymph nodes and rarely to the brain 2. These tubules help filter waste from the blood, making urine. Chromophobe cell renal carcinoma is a recently established subtype of renal cell carcinoma. Adenoma, chromophobe carcinoma, renal cell kidney diseases, cystic.
Chromophobe renal cell carcinoma chrcc is a distinct subtype of renal cell carcinoma, possibly originating from the the distal nephron. The report indicates renal cell carcinoma chromophobe type 4. These tumors are macroscopically larger when compared with other forms and are commonly diagnosed at an early stage. When symptoms and signs appear they include constant back pain, fatigue, anemia, weight loss, intermittent fevers, a lump on the lower back side, and blood in the urine. The type of cancer is multifocal chromophobe renal cell carcinoma.
Chromophobe renal cell carcinoma constitutes up to 5% of all cases of kidney cancer. Should genetic testing for birthoggdube syndrome bhds be considered in light of his cancer history and other findings. Most chrcc have good prognosis, but sarcomatoid change is a poor prognostic factor. Abstract renal cell carcinoma is the most common neoplasm of the kidney. Chromophobe renal cell carcinoma is a rare variant and accounts for 5% of all cases. Choueiri, anne plantade, paul elson, sylvie negrier, alain ravaud, stephane oudard, ming zhou. Despite advances in our understanding of the biology of chromophobe renal cell carcinoma chrcc and renal oncocytoma ro, the differential diagnosis among these tumors remains one of. We studied cancerspecifics outcomes of 109 patients with metastatic chromophobe renal cell carcinoma with and without. The current case is the first report of chromophobe renal cell carcinoma causative of severe idiopathic thrombocytopenic purpura.
Squamous differentiation in a sarcomatoid chromophobe renal cell carcinoma. The most prevalent form, clear cell rcc ccrcc accounts for 65% to 70% of the cases, whereas a heterogeneous group of nonccrcc subtypes1, 2, 3 is responsible for the remainder of the cases chromophobe rcc chrcc is the second most common form of nonccrcc after papillary rcc and accounts for. Chromophobe renal cell carcinoma was described by thoenes et al. However, to the authors knowledge, only few clinical data are available regarding this variant of tumor. In addition, he has a lung hamartoma and bladder lipoma. Despite significant advances in renal cell carcinoma therapeutics in the past decade, no standard treatment has been identified for advanced. It is rare in the pediatric population and only 2% of rcc occur in patients younger than 20 years old. Mri features of renal oncocytoma and chromophobe renal cell. Molecular classification of renal cell carcinoma and its implication in future clinical practice. Pdf apoptosis in chromophobe renal cell carcinoma and.
Jul 01, 2015 my husband is a survivor of chromophobe renal cell carcinoma rcc. Chromophobe renal cell carcinoma is a rare variant and accounts for 5% of all. My husband is a survivor of chromophobe renal cell carcinoma rcc. Home marchapril 2000 volume 5 issue 2 chromophobe renal cell carcinoma. Chromophobe renal cell carcinoma chrrcc is a rare subtype of renal cell carcinoma, which arises from the distal regions of the kidney, as opposed clear cell rcc ccrcc, which arises from the proximal kidney. Renal oncocytoma is the second most common benign renal neoplasm after angiomyolipoma, comprising 37% of all renal tumors. It is often difficult to determine how quickly a tumor has developed, because each individual tumor is unique and the speed of growth can vary among tumors of the same type. Quirogagarza g, khurana h, shen s, ayala ag, ro jy. The two systems that are commonly used are the memorial sloan kettering cancer center mskcc criteria and the international metastatic renal cell carcinoma database consortium imdc criteria. This type of cancer forms in the cells lining the small tubules in the kidney. Chromophobe renal cell carcinomas crcc comprise about 5% of all renal neoplasms. Chromophobe rcc is seen in a number of diseases, including birthoggdube syndrome. Renal oncocytoma and chromophobe renal cell carcinoma.
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