The Kidney: A Comprehensive Guide to Pathologic Diagnosis and Management

Contents
Contributors
1: Embryology, Anatomy, and Histology of the Kidney
	Development of the Kidney
	Anatomy of the Kidney
		Overall Structure
		Adjacent Structures
		Fascial Contributions
		Arterial Supply, Venous Drainage, and Lymphatics
		Innervation
	Histology of the Kidney
		Renal Capsule
		Parenchyma
			Overview
			Cortex
			Medulla
		Renal Corpuscle
		Nephron
			Nephron Tubules
			Proximal Convoluted Tubule
			Loop of Henle
		Collecting Tubules and Ducts
	References
2: Polycystic Kidney Disease
	Autosomal Dominant Polycystic Kidney Disease
		Background
		Genetics/Pathogenesis
		Pathology
		Clinical Presentation
			Extrarenal Manifestations
		Diagnosis/Evaluation
		Sequelae
		Treatments
		Prognosis
	Autosomal Recessive Polycystic Kidney Disease
		Background
		Genetics/Pathogenesis
		Pathology
		Clinical Presentation
		Diagnosis/Evaluation
		Treatment/Prognosis
	References
3: Nonneoplastic Disease Presenting as a Renal Lesion
	Introduction
	Inflammatory and Infectious Lesions
		Focal (Localized) Pyelonephritis
		Renal Abscess
		Xanthogranulomatous Pyelonephritis (XGP)
		Renal Malakoplakia
	Cystic Lesions of the Kidney
		Autosomal Dominant Polycystic Kidney Disease (ADPKD)
		Autosomal Recessive Polycystic Kidney Disease (ARPKD)
		Acquired Cystic Kidney Disease (ACKD)
	Miscellaneous Lesions
		Renal-Adrenal Fusion
		Ectopic Adrenal Tissue
		Splenorenal Fusion
		Extramedullary Hematopoiesis (EMH)
	Conclusions
	References
4: Overview and Staging of Renal Neoplasms
	Classification of Renal Neoplasms
	Staging of Renal Cancers
		Introduction
		Historical Background
		Components of the TNM Staging System for Renal Cancer
			Organ-Confined Tumors
				Tumors 7 cm or Smaller (T1)
				Tumors Larger Than 7 cm (T2)
			Locally Aggressive Tumors
				Perinephric or Sinus Fat, Renal Vein, and Vena Caval Extension (T3)
				Invasion into Ipsilateral Adrenal Gland or Beyond Gerota’s Fascia (T4)
			Regional Lymph Nodes and Distant Metastasis
		Pathologist Handling of Kidney Resections for Staging Adequacy
		Validation Studies of the 2010 TNM Staging System
	References
5: Conventional Forms of Renal Neoplasia
	Introduction
	Renal Tumor Pathology and Grading
		Clear Cell Renal Cell Carcinoma
		Multilocular Cystic Clear Cell Renal Cell Neoplasm of Low Malignant Potential (Multilocular Cystic Renal Cell Carcinoma)
		Papillary Renal Cell Carcinoma
		Papillary Adenoma
		Chromophobe Renal Cell Carcinoma
		Oncocytoma
	Staging of Renal Cell Carcinoma
	Evaluation and Workup of Cortical Renal Tumors
	Management
		Role of Percutaneous Biopsy of Renal Masses
		Management of Localized Renal Masses (Stage I/Stage II)
		Management of Locally Advanced and Metastatic Disease (Stage III/Stage IV)
	Future Directions
	References
6: Familial Forms of Renal Cell Carcinoma and Associated Syndromes
	Chapter
	Von Hippel-Lindau
		Introduction
		Clinical Presentation
		Pathology
		Treatment
	Hereditary Papillary Renal Cell Carcinoma (HPRCC)
		Introduction
		Clinical Presentation
		Pathology
		Treatment
	Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC)
		Introduction
		Clinical Presentation
		Pathology
			Renal Cancer
		Leiomyomas of the Skin and Uterus
		Treatment
	Birt-Hogg-Dubé (BHD) Syndrome
		Introduction
		Clinical Presentation
		Pathology
		Skin and Other Lesions
		Treatment
	Tuberous Sclerosis (TS) Syndrome
		Introduction
		Clinical Presentation
		Pathology
		Treatment
	Succinate Dehydrogenase Mutation
	Cowden/PTEN Hamartoma Tumor Syndrome
	Microphthalmia Transcription (MiT) Family Translocations
	Microphthalmia-Associated Factor (MITF) Mutation
	BAP1 Mutation
	Chromosome 3 Translocation
	References
7: Translocation-Associated Carcinoma
	Introduction
	History and Epidemiology
	Genetics
	Diagnosis and Pathologic Analysis
	Treatment
	Surveillance
	Conclusion
	References
8: Collecting Duct Carcinoma and Renal Medullary Carcinoma
	Introduction
	Epidemiology, Clinical Features, and Radiographic Features of Collecting Duct and Renal Medullary Carcinoma
		Epidemiology and Clinical Features
		Radiographic Features
	Pathologic Characteristics of Collecting Duct and Renal Medullary Carcinoma
		Collecting Duct Carcinoma
			Gross Pathology
			Microscopic Pathology
			Immunohistochemistry
			Cytogenetic and Molecular Findings
			Differential Diagnosis
		Renal Medullary Carcinoma
			Gross Pathology
			Microscopic Pathology
			Immunohistochemistry
			Cytogenetic and Molecular Findings
			Differential Diagnosis
	Clinical Management of Patients with Collecting Duct and Renal Medullary Carcinoma
		Collecting Duct Carcinoma
			Surgical Management
			Systemic Therapy
				Cytotoxic Chemotherapy
				Immunotherapy
				Targeted Therapy
		Renal MedullaryCarcinoma
	References
9: Emerging and Recently Described Subtypes of Renal Carcinoma
	Mucinous Tubular and Spindle Cell Carcinoma (MTSCC)
		Introduction
		Clinical Presentation
		Pathology
		Prognosis and Clinical Management
	Clear Cell Papillary RCC
		Introduction
		Clinical Presentation
		Pathology
		Prognosis and Clinical Management
	Tubulocystic Carcinoma
		Introduction
		Clinical Presentation
		Pathology
		Prognosis and Clinical Management
	Acquired Cystic Disease-Associated RCC
		Introduction
		Clinical Presentation
		Pathology
		Prognosis and Clinical Management
	Neuroblastoma-Associated RCC
		Introduction
		Clinical Presentation
		Pathology
		Prognosis and Clinical Management
	Thyroid-Like Follicular Carcinoma of the Kidney (TLFCK)
		Introduction
		Clinical Presentation
		Pathology
		Prognosis and Clinical Management
	Succinate Dehydrogenase-Deficient RCC
		Introduction
		Clinical Presentation
		Pathology
		Prognosis and Clinical Management
	TCEB1-Mutated (Monosomy-8) Renal Cell Carcinoma
		Clinical Presentation
		Pathology
		Prognosis and Clinical Management
	Renal Carcinoid Tumor
		Introduction
		Clinical Presentation
		Pathology
		Prognosis and Clinical Management
	References
10: Non-epithelial Renal Neoplasms of the Adult Kidney
	Introduction
	Mesenchymal Neoplasms
	Benign Mesenchymal Tumors
		Angiomyolipoma (AML)
			Epithelioid Angiomyolipoma
		Leiomyoma
		Hemangioma
		Solitary Fibrous Tumor
		Juxtaglomerular Cell Tumor (JG Tumor)
		Renomedullary Interstitial Cell Tumor
	Malignant Mesenchymal Neoplasms Involving the Kidney
		Leiomyosarcoma
		Synovial Sarcoma
		Liposarcoma
	Hematopoietic Tumors
	Neuroendocrine Tumors
	References
11: Pediatric Renal Neoplasms
	Malignant Pediatric Renal Tumors
		Wilms Tumor
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
		Premalignant Neoplasms Associated with Wilms Tumor: Nephrogenic Rests and Nephroblastomatosis
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
		Clear Cell Sarcoma of the Kidney (CCSK)
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
		Rhabdoid Tumor of the Kidney
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
		Desmoplastic Small round Cell Tumor of the Kidney
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
		Anaplastic Sarcoma of the Kidney
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
	Indolent Renal Lesions and Malformations
		Congenital Mesoblastic Nephroma (CMN)
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
		Metanephric Stromal Tumor
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
		Metanephric Adenofibroma
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
		Multicystic Dysplastic Kidney
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
		Medullary Sponge Kidney
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
		Reflux Nephropathy
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
	Hematologic Malignancies Involving the Pediatric Kidney
		Renal Lymphoma and Leukemia
			Introduction
			Clinical Presentation
			Pathology
			Prognosis and Clinical Management
	References
12: Glomerular Disease
	Renal Anatomy
		The Nephron
		The Glomerulus
		The Proximal Tubule
		The Loop of Henle
		Distal Convoluted Tubule and Connecting Segment
		Collecting Duct
	Renal Biopsy
		Percutaneous Kidney Biopsy
		Open Kidney Biopsy
		Transvenous Kidney Biopsy
	Tissue Fixation and Preparation
		Light Microscopy
		Electron Microscopy
		Immunofluorescence or Immunoperoxidase
			Glomerular Syndromes and Mechanisms of Injury
			Structural Patterns of Injury
	Nephritic Syndromes
	Rapidly Progressive Glomerulonephritis
		Clinical Presentation
		Pathogenesis
		Treatment
		Anti-glomerular Basement Membrane Disease
		Pathogenesis
			Pathology
		Management
		Pauci-immune Glomerular Disease
		Pathogenesis
			Pathology
		Management
		Immune Complex Deposit Mediated Disease
		IgA Nephropathy
			Pathophysiology
			Pathology
			Management
		Postinfectious Glomerulonephritis
			Pathogenesis
			Pathology
			Management
		Lupus Nephritis
			Nephrotic Syndrome
	The Podocytopathies: Minimal Change Disease and Focal Segmental Glomerulosclerosis
		Minimal Change Disease
			Pathophysiology
			Pathology
			Management
			Focal Segmental Glomerulosclerosis
			Pathophysiology
			Pathology
			Management
	Membranous Nephropathy
		Pathophysiology
		Pathology
		Management
	References
13: Nonneoplastic Kidney Diseases in the Setting of a Renal Mass
	Introduction
	The Harmful Impact of Chronic Kidney Disease
	Renal Pathology
	Processing and Evaluation of the Nonneoplastic Kidney in Tumor Nephrectomy Specimens
	Algorithm
	Artifacts and Pitfalls (Tumor Compression)
	Diabetic Nephropathy
	Arterionephrosclerosis/Hypertensive Nephrosclerosis
	Focal Segmental Glomerulosclerosis
	Thrombotic Microangiopathy
	Summary
	References
Index