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Kidney tumor - adult malignancies

Adult renal cell carcinoma

Medullary carcinoma of kidney


Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 30 June 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Rare; <100 cases reported, first characterized in 1995 (Am J Surg Pathol 1995;19:1)
● Also called the seventh sickle cell nephropathy (others are unilateral hematuria, papillary necrosis, renal infarct, nephrotic syndrome, pyelonephritis, inability to concentrate urine)
Origin: arises from collecting duct system; may be due to regenerating renal papillary epithelium
● May be related to collective duct carcinoma based on similar histologic and immunoreactive features
● May be related to rhabdoid tumor, based on shared molecular/genetic alteration (loss of INI1)

Epidemiology
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● Majority of patients have been black
● Sickle cell trait or hemoglobin SC disease in almost all cases
● Average age 21-24 years, 75% male
● 75-89% occur in right kidney (Urology 2007;70:878)

Clinical description
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● Aggressive; usually advanced disease at presentation with metastases to lymph nodes, adrenal gland, peritoneum, perinodal retroperitoneum, liver, lungs or inferior vena cava
● Death in 4-6 months, resistant to radiotherapy and chemotherapy, although one 8 year old boy is free of disease 8 years after surgery (Mod Pathol 2007;20:914)

Case reports
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● 15 year old black girl with sickle cell trait, lymphadenopathy, pleural and pericardial effusions (Arch Pathol Lab Med 2003;127:e288)
● 20 year old pregnant black woman with sickle cell trait (Arch Pathol Lab Med 2002;126:627)
● 21 year old black man with sickle cell disease and 40 year old black man with HbSC disease (Arch Pathol Lab Med 2003;127:e135)
● 33 year old black man with sickle cell trait (Arch Pathol Lab Med 2000;124:1561)
● 37 year old black woman with tumor and presence of sickle cell trait discovered at autopsy (Am J Surg Pathol 1998;22:260)
● 42 year old white man without sickle hemoglobinopathy (Nat Rev Urol 2010;7:110)

Gross description
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● Ill-defined, firm, rubbery, tan-gray tumor in renal medulla and adjacent soft tissues; mean 7 cm
● Satellite nodules in cortex; hemorrhage and necrosis common

Gross images
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Well-circumscribed yellow-white tumor


Focally infiltrative

Lobulated tumor

Micro description
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● Tubular, solid, reticular, adenoid cystic or yolk sac-like patterns, but not tubulopapillary
● Tumor cells have hyperchromatic nuclei, prominent nucleoli, rhabdoid features
● Angiolymphatic invasion, desmoplastic stroma, infiltrative borders and intratumoral neutrophils are common
● Lymphocytes at rim
● Hemorrhagic and geographic necrosis, frequent mitotic figures
● Contains mucin

Micro images
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Patterns:
   
Reticular growth pattern


Tubular pattern with prominent desmoplasia


Microcystic pattern


Solid pattern


Adenoid cystic pattern


Collections of neutrophils


Sickled RBCs


Rhabdoid versus medullary tumor: H&E and INI1

Case reports:


15 year old black girl with sickle cell trait:
Figure 1: CT shows upper pole mass
Figure 2: FNA shows cohesive three dimensional tumor cell balls with prominent nucleoli
Figure 3: cell block shows cells with eosinophilic cytoplasm, macronucleoli, brisk mitotic activity
Figure 4: lymph node biopsy shows solid pattern of large anaplastic cells



20 year old pregnant black woman with sickle cell trait:
Figure 1: tumor extends into hilus and renal vein
Figure 2: sheets of intermediate to large cells with ill defined borders, moderate cytoplasm, vesicular nuclei, prominent nucleoli; also extensive necrosis, inflammation, mitotic figures
Figure 3: adjacent stroma shows spindle cells with vesicular nuclei, desmoplastic stroma, inflammation



21 year old black man with sickle cell disease


40 year old black man with HbSC

Cytology description
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● Cohesive groups of cells with vacuolated cytoplasm that often displace or indent the nuclei; nuclei often have irregular membranes, coarse or vesicular chromatin and prominent nucleoli (Cancer 2005;105:28)

Cytology images
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33 year old black man with sickle cell trait:
Figure 1: touch imprints show clusters of large malignant cells with well defined borders, hyperchromatic nuclei, irregular nuclear borders and prominent nucleoli
Figure 2: biopsy shows sheets and cords of large epithelioid cells, with abundant eosinophilic cytoplasm, enlarged hyperchromatic nuclei and prominent nucleoli
Figure 3: EM shows large tumor cells with streaming bundles of cytokeratin, varying rough endoplasmic reticulum,
mitochondria and lysosomes, and irregular nuclei


Various images

Positive stains
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● High molecular weight cytokeratin, variable vimentin and mucicarmine, occasional EMA and CEA

Negative stains
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● Loss of INI1 expression (Mod Pathol 2008;21:647), colloidal iron, PAS, desmin

Cytogenetics description
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● Associated with monosomy 11 (beta globin gene is at end of 11p)

Molecular description
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● Hypoxia-inducible factor and VHL abnormalities (Hum Pathol 2011;42:1979)
● ALK rearrangement with vinculin (VCN) fusion (Genes Chromosomes Cancer 2011;50:146)
● DNA topoisomerase II amplification (BJU Int 2010;1:62)

Differential diagnosis
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Collecting duct carcinoma: tubulopapillary pattern of irregular glands within desmoplastic stroma with neutrophils
High grade urothelial carcinoma
Rhabdoid tumor

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Medullary carcinoma of kidney


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