discuss the CLASSIFICATION OF KIDNEY TUMOURS.pptx

1. CLASSIFICATION OF
KIDNEY TUMOURS.
Histopathology
Dr Haruna Usman Kamba.

2. Outline.
 Introduction
 Epidemiology.
 Clinical features
 WHO Classification of kidney tumours
 Classification of RCC
 Conclusion
 References.

3. Introduction.
 In the last two decades, there has been a considerable rise in the
number of renal masses incidentally detected.
 Although the majority of these masses have distinct clinical and
radiological features, a minority share similar patterns leading to
diagnostic uncertainty.
 A clear understating of the evaluation, current management
approaches, and awareness of the evolving evidence pertaining to
renal masses is essential.
 With improvement in imaging modalities and pathological assessment
techniques, a more precise diagnosis can be made.
 The understanding of the molecular basis of many of these lesions is
paramount.
 Translational research has led to the introduction of various novel
targeted therapies in an attempt to improve oncological outcomes.

4. Epedemiology.
 Renal tumours constitute 1-2 %of all malignancies,
 RCC = 90% of all renal malignancies, making it the most
common solid renal lesion.
 RCC accounts for up to 2.5% of all adult malignancies and
accounts for 1–2 per 100 000 of all cancer‐ specific
mortality worldwide.
 It is most common in the sixth and seventh decades of
life.
 RCCs have a preponderance for male gender with a male
to female ratio of 3:2
 Age /SEX/RACE/GENETIC/LIFE STYLE

5. Clinical features.
 Asymptomatic, incidentally detected 50%
 Localised or locally advanced disease
 Visible haematuria
 Flank or loin pain
 Palpable abdominal mass
 Vena caval obstruction: bilateral leg oedema,
 irreducible or acute (left‐sided) varicocele

6. Systemic or metastatic
disease
 Bone pain
 Coughing or haemoptysis
 Lymph node enlargement
 Weight loss, cachexia,
night sweats, fatigue
 Amyloid deposits
 Pyrexia of unknown origin
Paraneoplastic syndrome
(ectopic hormone
production):
 Hypertension (renin secretion, or
atrioventricular
 fistula, or renal artery
compression)
 Polycythaemia (erythropoietin
section)
 Anaemia (haematuria/chronic
disease)
 Hypoglycaemia (insulin secretion)
 Hypocalcaemia (para‐thyroid
hormone‐like secretion

7.  Cushing’s syndrome (adrenocorticotropic hormone
 secretion)
 Stauffer syndrome (hepatic dysfunction)
(unknown cause resolves in 60–70% after
nephrectomy)

8. Classification of kidney tumours.

9. WHO (2016)
Renal cell tumors
 Clear cell renal cell carcinoma
 Multilocular cystic renal neoplasm of
low malignant potential
 Papillary renal cell carcinoma
 Hereditary leiomyomatosis and renal
cell carcinoma associated renal cell
carcinoma
 Chromophobe renal cell carcinoma
 Collecting duct carcinoma
 Renal medullary carcinoma
 MiT family translocation renal cell
carcinomas
 Succinate dehydrogenase deficient
renal cell carcinoma
 Mucinous tubular and spindle cell
carcinoma
Metanephric tumors
 Metanephric adenoma
 Metanephric adenofibroma
 Metanephric stromal tumor
Nephroblastic and cystic tumors
occurring mainly in children
 Nephrogenic rests
 Nephroblastoma
 Cystic partially
differentiated nephroblastoma
 Pediatric cystic nephroma

10. Mesenchymal tumors
Mesenchymal tumors
occurring mainly in
children
 Clear cell sarcoma
 Rhabdoid tumor
 Congenital
mesoblastic nephroma
 Ossifying renal tumor
of infancy
Mesenchymal tumors occurring
mainly in adults
 Leiomyosarcoma (including renal
vein leiomyosarcoma)
 Angiosarcoma
 Rhabdomyosarcoma
 Osteosarcoma
 Synovial sarcoma
 Ewing sarcoma
 Angiomyolipoma
 Epithelioid angiomyolipoma
 Leiomyoma
 Hemangioma
 Lymphangioma
 Hemangioblastoma
 Renomedullary interstitial cell
tumor
 Schwannoma

11. • Mixed epithelial and stromal tumor
family
• Adult cystic nephroma
• Mixed epithelial and stromal tumor
•
Neuroendocrine tumors
• Well differentiated neuroendocrine
tumor
• Large cell neuroendocrine carcinoma
• Small cell neuroendocrine carcinoma
• Paraganglioma
• Renal hematopoietic neoplasms
Germ cell tumors
Metastatic tumors
Tumor-like lesions
Xanthogranulomatous pyelonephritis
• IgG4 related disease

12. Described entities not currently in WHO
 Thyroid-like follicular carcinoma of kidney
 Renal cell carcinoma with smooth muscle stroma
 Renal cell carcinoma with anaplastic lymphoma kinase (ALK)
translocation
 Renal cell carcinoma occurring in patients with prior neuroblastoma
 Eosinophilic, solid and cystic renal cell carcinoma
 Biphasic squamoid alveolar renal cell carcinoma
 Chromophobe renal cell carcinoma with neuroendocrine and
neuroendocrine-like features

13. Classification of renal cell carcinomas
 Classification of renal cell carcinomas has become more challenging.
 The 2016 WHO classification included 14 different subtypes and 4 emerging entities.
 Nomenclature is based on:
cytoplasmic appearance
architecture
combination of morphologies
anatomic location
underlying disease
familial syndromes, and specific genetic alterations.
 Immunohistochemistry is useful in selected cases while it can be insufficient in entities that
require molecular confirmation of a specific gene alteration.

14. 1.Tumours with predominant clear cells
Clear cell RCC - 70%, 95% sporadic, VHL associated, clear/
granular cytoplasm. Architexture –acinar, nested, alveolar, tubular or
solid/ cords.
 Most common genetic mutationis deletion on short arm of chromosome 3 and
inactivation of VHL gene.
 No specific markers. CA- IX, RCC Ag, CD10 +ve, CK7 –ve

15.  Chromophobe RCC with prominent “clear” cells.
 typically shows sheets of cells separated by incomplete septations.
 The presence of cells with wrinkled nuclei, perinuclear halos, and prominent cell
membranes.
 immunochemistry may be helpful to distinguish clear cell RCC (carbonic anhydrase IX
strong, diffuse and with a membranous pattern; CD10, RCC antigen, and vimentin
positive) and chromophobe RCC (c-KIT and cytokeratin 7 positive).
 Multilocular cystic renal neoplasm of low malignant potential

16. Mesenchymal tumors
 Angiomyolipomas comprise 0.7–2.0% of all renal tumors
 and about 20% of them are associated with tuberous sclerosis.
 As a consequence, germline mutations of TSC1 gene and the TSC2 gene are
recommended.
 The pathological diagnosis is usually straightforward due to characteristic
combination of bland morphologies of adipose tissue, smooth muscle tissue
and large vessels with thickened walls.
 The clinical behavior is benign in the absence of epithelioid morphology.
 The diagnosis of epithelioid angiomyolipomas requires more than 80% of
epithelioid cells. Epithelioid angiomyolipoma has metastatic potential may also
be associated with tuberous sclerosis.
 Metastatic behavior is predicted by larger size (> 7 cm), carcinoma-like atypia,
involvement of perinephric tissues, renal vein invasion and necrosis.
 Those aggressive epithelioid angiomyolipoma may mimic high-grade

17. Emerging and new entities
 Low-grade oncocytic tumor of the kidney
 shares with oncocytomas - predominant solid and nested
growth pattern and the oncocytic cytoplasm.
 However, they show a distinct immunophenotype of c-
KIT/CD117 negative and cytokeratin 7 positive.
 microscopic picture is of solid sheets and compact nests,
with gradual transition to trabecular areas, sharply delineated
edematous stromal areas with loose cell growth.

18.  High-grade oncocytic tumor of the kidney
 Eosinophilic solid and cystic renal cell carcinoma
(ESC RCC)
 Anaplastic Lymphoma Kinase (ALK) rearrangements-
associated renal cell carcinoma (ALK RCC)
 Renal cell carcinoma with angio - leiomyomatous
stroma (RCCLMS)
 Unclassified renal cell carcinoma

24. Conclusion.
 Renal tumours are considerably increasing in the number , most of which are
incidentally detected.
 Although the majority of these tumours have distinct clinical and radiological
features, some have similar patterns leading to diagnostic uncertainty.
 Therefore, a clear understating of the evaluation, current management
approaches, and awareness of the evolving evidence pertaining to renal
masses is essential.
 Recent improvement in imaging modalities and pathological assessment
techniques and understanding of the molecular basis of kidney tumours has
helped in more precise diagnosis and subsequent newer classifications.

25. References.
 Andeen NK, Tretiakova M. WHO classification.
PathologyOutlines.com
website.https://www.pathologyoutlines.com/topic/kidne
ytumorWHOclass.html. Accessed April 19th, 2022.
 Adam J, Couturier J, Molinié V, Vieillefond A, Sibony M
(2011) Clear-cell papillary renal cell carcinoma: 24 cases
of a distinct low-grade renal tumour and a comparative
genomic hybridization array study of seven cases.
Histopathology 58:1064–1071

26. Thank
you