Chromophobe renal cell carcinoma (RCC), which accounts for 4-6% 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 [ 1 ]. In this communication, the authors report two Japanese cases of chromophobe RCC together with the immunohistochemical findings. Renal cell carcinoma (RCC) is a morphologically and genetically heterogeneous malignancy (1, 2) arising from specialized cells in the nephron.The most common type is clear cell RCC (ccRCC), which represents 75-80% of RCC and derives its name from its clear cytoplasm on pathologic analysis. . While most Tissue of origin Proximal tubule Intercalated cell Collecting duct Principal cell Adenoma VHL-mutation-3p 7% Met-mutation +7, +17, -Y . Chromophobe renal cell carcinoma is a variant of renal cell carcinoma (RCC) that was first described in humans by Thoenes et al. Context.—. Chromophobe renal cell carcinoma showing large polygonal cells with prominent cell membranes, fairly dense eosinophilic granular cytoplasm, and perinuclear halos/clearing (hematoxylin and eosin, ×20 . 1. The incidence of renal cell carcinoma has increased by 35% from 1973 to 1991. Chromophobe Renal Cell Carcinoma Chromophobe RCC represents 5% of RCCs. Renal cell carcinoma (RCC) is a heterogeneous group of neoplasms derived from the renal tubular epithelial cells. Periphery of cell distinct, i.e. Physical examination in entirely normal. Publication types Case Reports Advertisement Kidney cancer is one of the 10 most common cancers in the United States with 90% being attributed to renal cell carcinoma. Routine urine test showed that urinary protein was ++ and white blood cells were . Histologic grade (Fuhrman nuclear grade): G2. . Genetics plays a role in kidney cancer with currently seven different renal cell carcinoma (RCC) genes having been identified (Linehan et al., 2011). Although tyrosine kinase inhibitors and immune check-point blockade treatments, alone or in combination, are giving promising results, failures are quite frequent due to intratumor heterogeneity and to the acquisition of drug resistance. We report the first case of chromophobe cell renal carcinoma in childhood. The prognosis of sarcomatoid chromophobe renal cell carcinoma is worse than classical chromophobe renal cell carcinoma. The reported size of the tumor ranges from 0.7 to 20 cm, with a mean reported size of 5.1 cm. CASE REPORT A 63-year-old female complained of painless gross hematuria for 3 months. An estimated 63,000 new renal tumors were diagnosed in 2013 with over . 4.1. J Urol. Gross and histologic examination of the nephrectomy specimen revealed a 9.3-cm renal tumor composed of solid trabecular sheets of polygonal epithelial cells with clear cytoplasm and distinct cell borders characteristic of a chromophobe renal cell carcinoma. Series studies have revealed that the incidence of CRCC is between 3.6% and 10.4% of all kidney neoplasms. Initially, diagnosis of chromophobe RCC appeared to be straightforward as a result of its characteristic light microscopic features. (1) Both are considered to derive from the intercalated cells of collecting ducts. Chromophobe renal cell carcinoma (CRCC) is a distinctive, but not rare, malignant renal neoplasm. (2) The two have a similar gross appearance (discrete mass, light-tan to brown on the cut surface, irregular fibrous bands or scar in the center). METHODS Three cases of CCRC were studied by analyzing the results of an operation, by light microscopy, immunohistochemistry, Hale's colloidal iron staining and electronmicroscopy (EMC). Routine surveillance for graft tumours is not routinely practiced and these tumours are commonly asymptomatic and incidentally . a pale halo surrounds the nucleus - key feature. The main differential diagnosis of renal oncocytoma is chromophobe renal cell carcinoma oncocytic variant, which like the renal oncocytoma has eosinophilic cytoplasm, but has perinuclear clearing and, typically, some degree of . Perinuclear clearing, i.e. . Chromophobe renal cell carcinoma (chRCC) is the third most common type of RCC with distinct biology compared to other kidney cancer subtypes. 1-9 Nevertheless, this variant may be misdiagnosed as either . Multilocular Cystic Renal Neoplasm of LMP : Microscopic. 4.5.1 General Features. Morphologic overlap between renal oncocytoma (RO) and chromophobe renal cell carcinoma (ChRCC) can pose diagnostic challenges, especially in biopsy samples in which tumor quantity may be limited, architectural features are not well represented, and gross examination is not possible. chRCC has a better prognosis . 1. 10 in addition to hoct and chromophobe rcc, affected patients are at risk of developing clear cell rcc, papillary rcc and angiomyolipoma, although the frequencies of each of these histological types … The incidence of renal cell carcinoma has increased by 35% from 1973 to 1991. RENAL CELL CARCINOMA. Gross hemorrhage, necrosis, cystic changes, and a central scar may be seen (Moch et al., 2016; Cheng et al., 2020). Chromophome renal cancer (ChRCC) is a subtype of RCC with a more favorable prognosis. Clear cell RCC. The spectrum of renal cell carcinoma subtypes is wide. The most common histology in secondary tumours was papillary renal cell . in 1985 1 and is estimated to comprise 2-5% of RCCs. Age group is 6th & 7th decades with male predominance. Chromophobe renal cell carcinoma is known to originate from the intercalated cells of the distal convoluted tubule. Gross— Chromophobe RCC is thought to arise from intercalated cells of collecting ducts ( Fig. chRCC may be detected as an incidental finding or less commonly may manifest with clinical symptoms. Routine urine test showed that urinary protein was ++ and white blood cells were . The case represents a typical sarcomatoid chromophobe renal cell carcinoma, which has the potential to behave aggressively and to metastasize. Gross findings of rhabdoid renal cell carcinoma. The 5-and 10-year survival rates of this cancer are reported 100 and 90%, respectively [ 2 ]. On gross inspection, . Primary renal lymphoma. The incidence of renal cell carcinoma (RCC) in renal allograft in transplant recipients is 0.22-0.25%. Eosinophilic cytoplasm is the most common finding of difficult-to-classify kidney tumors. It has been demonstrated that immunohistochemistry (IHC) for . cell membrane is easy to discern. 1-3 In China, the first CRCC case was described by our hospital in 1998; 4,5 there have since been only a few case reports of CRCC in China and very few instances of long-term follow-up. Renal clear cell carcinoma is well known to be the most common recipient of tumor-to-tumor metastasis in all tumors. Patients with chromophobe renal cell carcinoma have a clinical course similar to that of clear cell renal cell carcinoma.9 In our case, a 12 mm renal Oncol . The tumors were composed of sheets and cribriform glands formed by tumor cells with cloudy and reticular cytoplasm. GROSS PATHOLOGY. Key words: chromophobe, papillary, pathogenesis, renal cell PATHOLOGICAL FINDINGS carcinoma On gross examination the kidney contained a large, light The presence of concurrent subtypes of renal cell carcinoma brown and yellowish solid tumor occupying the lower pole, (RCC) is a very uncommon entity, because the majority of measuring . The tumor cells contained clear finely reticular ("chromophobe") and eosinophilic cytoplasm with perinuclear clearing. Chromophobe carcinoma is characterized by large polygonal cells with pale reticular cytoplasm characterize, and it does not exhibit 3p deletion. Accounts of 3% of visceral cancers in USA and 85% of renal cancers in adults. Cortez, MD Neal C. Dalrymple, MD Kedar N. Chintapalli, MD Renal cell carcinoma (RCC) is a cause of significant morbidity and mortality, with an estimated 35,000 new cases and 12,480 deaths in . T3b with gross extension into the renal vein or its segmental (muscle-containing) branches or the vena cava below the diaphragm, T3c with gross extension into . Ultrastructurally, the . for gross hematuria without urinary . As the disease progresses, symptoms may include: a lump in the abdomen. "Question ID","Question","Discussion","Answer" "20031036","Histology--Hematopoietic, NOS: When both the path and clinical diagnoses simultaneously reflect reportable . Immunohistochemical analysis of chromophobe renal cell carcinoma, renal oncocytoma, and clear cell carcinoma: an optimal and practical panel for differential . In gross appearance, the tumors are tan or mahogany brown, well circumscribed and contain a central scar. Publication types Case Reports English Abstract MeSH terms Tweets by @WebPathology. 6, 31 Compared with other subtypes of RCC, chromophobe RCC has distinct histologic, histochemical, ultrastructural, cytogenetic, and prognostic features. Smaller tumor nodule mainly present in the fat of renal pelvis. Microscopy— Chromophobe RCC is composed of cells with prominent cell membranes and pale eosinophilic cytoplasm, usually with a perinuclear halo around the nucleus. 1997; Bruder et al. 195(4 Pt 1): 847-851. On gross examination, the tumors ranged from 2 cm to 19 cm in greatest dimension (mean size = 7.3 cm). The tumor should be distinguished from the common clear cell RCC, because of the unique clinicopathological and molecular biological features. 1. 131,187-192 The majority of cases are found incidentally. Renal cell carcinomas (RCC) (historically also known as hypernephroma or Grawitz tumor) are primary malignant adenocarcinomas derived from the renal tubular epithelium and are the most common malignant renal tumor.They usually occur in 50-70-year old patients and macroscopic hematuria occurs in 60% of the cases. 2. Chromophobe renal cell carcinoma (CRCC) comprises 6% to 11% of all renal epithelial tumors and is the third most prevalent form of renal cell carcinoma (RCC) behind conventional RCC and papillary RCC. ChRCC is characterized by a distinct histology wherein the tumor cells are arranged in a solid and sheet-like pattern with delicate vascular septa. Chromophobe Renal Cell Carcinoma : Eosinophilic type. Keep reading to learn about the three most common subtypes, which account for more than 90 percent of all RCCs. Cystic Renal Cell Carcinoma (CRCC) is an uncommon subtype of renal cell carcinoma, occurring in 2.5% of cases. Multilocular Cystic Renal Neoplasm of LMP : Clinical & Gross. When RCC is in its early stages, patients may be symptom-free. ccRCC is characterized by alterations at chromosome 3p, affecting the von Hippel-Lindau locus (). 2004). A carcinoma arising from the renal parenchyma. The patient underwent left partial nephrectomy together with resection of neighboring small tumors. series of 697 renal cell carcinoma cases. The second cell type in chromophobe renal cell carcinoma is also characterized by an increased cytoplasmic eosinophilia or granularity, due . RCC occurrence shows a male predominance over women with a ratio of 1.5:1. . Adult renal tumors have been classified into various types by the World Health Organization ().Among them, renal oncocytoma and chromophobe renal cell carcinoma (RCC) originate from intercalated cells in the collecting duct and share some morphologic, histologic, electron microscopy findings, and immunohistochemical characteristics (2, 3).Although they have common pathologic features, renal . A carcinoma arising from the renal parenchyma. Chromophobe Renal Cell Carcinoma is the Most Common Nonclear Renal Cell Carcinoma in Young Women: Results from the SEER Database. nal cell carcinoma with the gross and . in our epidemiological study of the japanese population, the most frequent histological type was chromophobe rcc (43.6%), followed by hoct (34.5%). Symptoms of renal cell carcinoma. There is a strong correlation between cigarette smoking and the development of renal cell carcinoma. The clinical presentation includes : hematuria, flank pain and a palpable lumbar mass. A 60 yr old man presents with a feeling of fullness in his abdomen and 5kg weight loss over the past 6 months. Renal cell carcinoma (RCC) accounts for 3% of malignant tumors and is the seventh leading cause of cancer deaths in the USA. Although a wide range of histomorphologic prognostic parameters have been investigated for RCC, those that really matter to the patient are very limited. Sharply defined cell membranes (plant-like) Wrinkled irregular nuclei (raisinoid) Perinuclear halos (koilocytic) Terminology Chromophobe renal cell carcinoma (ChRCC), classic variant ICD coding ICD-10: C64 - malignant neoplasm of kidney, except renal pelvis Epidemiology Renal cell carcinoma (see the image below) is the most common type of kidney cancer in adults. Final Diagnosis: Sarcomatoid chromophobe renal cell carcinoma Symptoms: Painless gross hematuria Medication: — Clinical Procedure: — Specialty: Renal Surgery Objective: Rare disease Background: Sarcomatoid renal cell carcinoma is not a distinct histologic entity transformed from different subtypes of re-nal cell carcinoma. kidney cancer is one of the 10 most common cancers in the united states.1renal cell carcinoma accounts for 90% of all kidney cancers.2death attributed to renal cell carcinoma accounted for 2% of. Patient ages are similar . It has a better prognosis compared to conventional renal cell carcinoma , , , , and has distinct morphological, histochemical . Both oncocytoma and chromophobe renal cell carcinoma are noted to have mutations in mitochondrial genes (28-30). The clinical presentation includes : hematuria, flank pain and a palpable lumbar mass. Radical nephrectomy was performed. The rest of the kidney is unremarkable. . The mainstay of therapy for chRCC is surgical resection. Two types of tumor cells can be seen in varying . METHODS/RESULTS A 59-year-old woman referred a two-month history of constant right flank pain, and hematuria. It comprises a higher proportion of renal cancers in the Middle East. 1 In 1985, Thoenes et al. De novo clear cell, papillary, and chromophobe RCCs and RCCs with sarcomatoid differentiation originating in renal allograft have been reported. Answer Clear cell renal cell carcinoma (CCRCC) is typically a solitary tumor, with multifocality and bilaterality only occurring in 2-7% and 1-2% of sporadic cases, respectively. Chromophobe renal cell carcinoma (RCC) is a recently established subtype of RCC, which has rarely been reported in Japan. ChRCC accounts for less than 5% of all RCCs [5]. The second cell type in chromophobe renal cell carcinoma is also characterized by an increased cytoplasmic eosinophilia or granularity, due . Patients range in age from childhood to extreme old age, with a slight male preponderance. 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.RCC is the most common type of kidney cancer in adults, responsible for approximately 90-95% of cases. However, renal chromophobe cell carcinoma has not been reported to be a recipient. Chromophobe renal cell carcinoma (RCC), a newly established subtype of renal neoplasm, is composed of tumor cells with characteristically cloudy, weakly eosinophilic and reticular cytoplasm. Of these, papillary renal cell carcinoma, 12 of 21 (57%), was the most common type, followed by clear cell renal cell carcinoma, four of 21 (19%), clear cell papillary renal cell carcinoma (Figure 4), three of 21 (14%) and chromophobe renal cell carcinoma, two of 21 (10%). Sarcomatoid renal cell carcinoma: the chromophobe connection. Prehistory and epidemiology. clear cell, papillary, chromophobe, collecting duct carcinoma, medullary carcinoma, and unclassi- A 12-year-old boy presented gross hematuria following minor trauma. Currently it is recommended that chromophobe renal cell carcinoma not be graded with the WHO/ISUP system. Two discrete tumor nodules, 3.0 cm and 1.5 cm in maximum dimensions respectively (gross examination). RENAL CELL CARCINOMA AJM. This review compares well-recognized tumors such as granular clear cell carcinoma, papillary variants, chromophobe renal cell carcinoma, and oncocytoma and introduces newly described entities of hybrid oncocytic . Histopathology: Grossly the tumor tissue was . For example, loss of chromosome Y is not uncommon in a large number of non-renal tumors, as well as in many renal cell tumors, including clear cell and chromophobe renal cell carcinoma, and renal . . They may achieve a large size (up to 12 cm in diameter). The yellow expansile and nodular mass is the common gross morphology. OBJECTIVES We report herein the clinical, histological, and immunohistochemical features of a case of sarcomatoid chromophobe renal cell carcinoma. The most common type of RCC associated with HLRCC is type II papillary RCC although other types are seen. There are other types of tumors that can grow in the kidney, including sarcomas, lymphomas, transitional cell carcinoma . (2016). For example, clear cell RCC has a less favorable prognosis (stage for stage) than do papillary RCC and chromophobe RCC. Most ChRCC have good prognosis, but sarcomatoid change is a poor prognostic factor. They are called hypernephromas because of their gross yellow colour & resemblance of tumor cells with clear cells of adrenal cortex. a H&E section of the 11.7 cm right renal mass showing typical features of chromophobe renal cell carcinoma, including cells with prominent cell membranes (open arrows), abundant pale eosinophilic . RESULTS Doppler ultrasonic and CT features were not specific. Tumor-to-tumor metastasis is a rare phenomenon. In the most common type of RCC, called clear cell or conventional . The periphery of . However, chromophobe renal cell carcinoma characteristically exhibits multiple chromosomal losses, including commonly chromosomes Y, 1, 2, 6, 10, 13, 17, and 21 (29) and lesser rates of chromosomes 3, 5, 8, 9, 11, and 18 (30). Renal cancer is a health problem of major concern worldwide. Similarly, collecting duct carcinoma and renal medullary carcinoma are associated with poor prognosis and aggressive clinical behavior (Storkel et al. Clear cell renal carcinoma (referred to commonly as RCC) is the most common RCC with 70-80% of RCC's being of this type; papillary RCCs are the next most common type of RCC, followed by chromophobe Lack of necrosis is a very characteristic gross feature. Am J Surg Pathol. There is a strong correlation between cigarette smoking and the development of renal cell carcinoma. Chromophobe renal cell carcinoma (ChRCC), which originates from distal convoluted tubules and cortical collecting ducts, is a distinct subtype of renal cell carcinoma [ 3 ], which accounts for 4% to 6% of renal cell carcinoma subtypes [ 4 ]. Chromophobe renal cell carcinoma (RCC) is a rare neoplasm of the kidney that represents about 5% of RCCs. OBJECTIVE To study the diagnosis and treatment of chromophobe cell renal carcinoma (CCRC). described the first human renal carcinomas as an analogy to the cases in rodents showing similar morphology. Chromophobe renal cell carcinoma: histomorphologic characteristics and evaluation of conventional pathologic prognostic parameters in 145 cases. . Microscopy appearance. Introduction. One year later the boy was well. CRCC was first described by Thoenes et al. It is characterized by leiomyomas, mainly uterine or cutaneous, and renal cell carcinoma (RCC). . This malignant neoplasm of kidney is clinically diagnosed with an earlier stage and better prognosis than conventional clear-cell RCC [ 1 ]. Microscopic Classic Features - classic type (3 P's memory device): Pale cytoplasm, with wisps of eosinophilic material; the cells are not completely clear, they have "cobwebs". Chromophobe renal cell carcinoma and oncocytoma have certain features in common. . and weight loss and palpable mass (10%). All the excised tumors were diagnosed as chromophobe renal cell carcinoma on histological examination. Renal cell carcinoma is histologically heterogeneous and can be considered as a group of closely related but distinct tumour types that differ in histopathology and genetic findings. Histologically, the tumors were mainly composed of solid sheets, acini or tubules of malignant cells. An unusual mixed germ cell tumor of the testis consisting of rhabdomyosarcoma, mature teratoma and yolk sac tumor By Melita Balja and Eva Lovric Renal Metastases of Pancreatic Medullary Carcinoma: A Case Report It is important to recognize that sarcomatoid change of chromophobe renal cell carcinoma has the potential to behave aggressively and to metastasize. in 1985. 1997 Oct. 21 . Gross specimen of the kidney with renal cell carcinoma clear cell type. Lab studies show Hb is 8.2g/dl, haematocrit is 24% and MCV is 70, Urine analysis shows haematuria (+++), but no protein, glucose or leucocytes. (15% to 20%), and chromophobe . Morphology, cytogenetics, and immunohistochemical stains are discriminatory. Details are shown below: Not applicable . The same authors 3 years later described an eosinophilic variant of chromophobe RCC, which can easily be confused with renal oncocytoma or "eosinophilic" clear cell . On imaging, they have a variety of radiographic appearances, from solid and . Chromophobe RCC accounts for 4.9% of surgically excised renal epithelial neoplasms. Grossly, tumors are usually well circumscribed, with tan-brown to red cut surface. Figure 1: Gross and microscopic appearance of the renal mass: (A) . Here we present a case of sarcomatoid chromophobe renal cell carcinoma and review the clinicopathological characteristics of sarcomatoid chromophobe renal cell carcinoma. CASE REPORT:A 63-year-old female complained of painless gross hematuria for 3 months. Chromophobe RCC (chRCC) is the third most common subtype of RCC, accounting for 5% of cases. A careful gross analysis and description of tumor extension in a nephrectomy specimen is important and should guide blocking of tissue samples for histologic assessment.16 Careful documentation of . Publication types Case Reports Review 2 They noticed a 4.6% incidence of . The tumor does not show gender bias. Multilocular Cystic Renal Neoplasm of LMP : Molecular Genetics. Here we present a case of sarcomatoid chromophobe renal cell carcinoma and review the clinicopathological characteristics of sarcomatoid chromophobe renal cell carcinoma. Diagnosis: Chromophobe renal cell carcinoma. Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome is believed to result from an autosomal dominant mutation in the fumarate hydratase (FH) gene on chromosome 1. The chromophobe renal cell carcinoma consisted of compact epithelial cells arranged in a nested pattern, and these were mixed with extensive areas of sarcomatoid spindle cells with marked nuclear . Chromophobe renal cell carcinoma (chRCC) accounts for 5-10% of all RCC [4]. Chen X, Hu D, Fang L, Chen Y, et al. Renal cell carcinoma. Based on its morphological and electron‐microscopic characteristics, chromophobe renal cell carcinoma (RCC) was delineated among experimental kidney tumors nitrosomorpholine‐induced in rats. A case report and literature review. CDC is a subtype of RCC, which is considered to be derived from the principal cells of the collecting duct of Bellini, and it accounts for about 1 % of all renal tumors [ 1 ]. Chromophobe RCC (flickr.com). Female to male ratio is 2:1. While most Tissue of origin Proximal tubule Intercalated cell Collecting duct Principal cell Adenoma VHL-mutation-3p 7% Met-mutation +7, +17, -Y . Tulbah A, Kardar AH, Ali MA. He was diagnosed as having a left renal tumor 45-mm in diameter. [ 8, 39, 40] Tumor. On gross appearance, oncocytomas are usually well circumscribed and resemble normal renal parenchyma with tan or mahogany color on the cut surface, whereas RCC appears golden yellow. Gross image of a bivalved kidney showing renal cell carcinoma in the upper half. CRCC is very unlikely to metastasize (spread to other organs) and carries an excellent prognosis following surgery. 2. It arises from the intercalated cells of the collecting ducts [6]. blood in the urine . 3A ). There has been no change with the remaining tumors for 1.5 years postoperatively. Renal cell carcinoma (RCC) is the commonest neoplasm of the kidney. From our review of the international literature, around 150 cases have been reported since it was first documented by Campbel in 1868. Lack of necrosis is a very characteristic gross feature. The mean age of the patients is about 50 years (range, 13-83 years) with a male-to-female ratio of 2-3:1 [ 30 ]. 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