Kidney tumor - adult malignancies
Adult renal cell carcinoma
Medullary carcinoma

Author: Sean Williamson, M.D. (see Authors page)

Revised: 30 January 2017, last major update June 2012

Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

PubMed Search: Medullary carcinoma [title] kidney

Cite this page: Medullary carcinoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/kidneytumormalignantmedullarycar.html. Accessed April 23rd, 2017.
Definition / General
  • 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
  • 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 Features
  • 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
Gross Description
  • Ill defined, firm, rubbery, tanish 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 yellowish white tumor

Focally infiltrative

Lobulated tumor

Micro Description
  • 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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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
  • 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
Negative Stains
Molecular / Cytogenetics Description
Molecular:
Cytogenetics
  • Associated with monosomy 11 (beta globin gene is at end of 11p)
Differential Diagnosis