Kidney tumor - cysts, children, adult benign
Childhood neoplasms
Congenital mesoblastic nephroma

Author: Mandolin Ziadie, M.D. (see Authors page)

Revised: 13 January 2017, last major update December 2011

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PubMed Search: Congenital mesoblastic nephroma [title] kidney

Cite this page: Congenital mesoblastic nephroma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/kidneytumormesoblastic.html. Accessed December 15th, 2017.
Definition / general
  • Congenital myofibroblastic tumor that resembles infantile fibromatosis / leiomyoma (classic) or fibrosarcoma (cellular)
  • Also called fetal mesenchymal / leiomyomatous hamartoma
  • First described in 1967 (Pediatrics 1967;40:272)
Epidemiology
  • Most common pediatric renal tumor in infancy
  • 5% of all pediatric renal tumors (Cancer 2006;106:2275)
  • Age at presentation varies with type (classic, 16 days; cellular, 5 months; mixed, 2 months)
  • Rarely occurs in children older than 2 years of age
  • Frequency: 66% cellular, 24% classic and 10% mixed
Diagrams / tables

Images hosted on PathOut server:

Age distribution curve

Clinical features
  • May present in utero with fetal hydrops / polyhydramnios
  • Excellent prognosis (5 year survival of 96%, Pediatr Blood Cancer 2008;50:1130)
  • 5% - 10% recur or metastasize (usually cellular type), usually by age 1 year to lung, brain or rarely bone
  • Poor prognostic factors include: cellular variant (Pediatr Surg Int 2007;23:205) and advanced stage or sinus / vascular involvement (margins and sinus / vascular involvement should be reported)
Case reports
Treatment
  • Nephrectomy with wide margins
  • Chemotherapy if resection is incomplete in infants 3 months or older, or if tumor ruptures during surgery
Gross description
  • Unilateral
  • Extensive sampling of margin should be performed
  • Classic: mean 5 cm, variably circumscribed, white / yellow whorled mass near hilum, involving over half of kidney with indistinct tumor kidney interface; resembles leiomyoma with whorled cut surface; may be cystic; may involve renal sinus; hemorrhage and necrosis uncommon
  • Cellular: mean 9 cm, necrosis and large cystic areas and hemorrhage
  • Mixed: mean 10 cm, cysts, hemorrhage and necrosis in cellular areas
Gross images

Images hosted on PathOut server:

Case of the week #57:

Tan fleshy masses
with hemorrhage,
necrosis and cystic
degeneration



Images hosted on other servers:

Tan fleshy masses
with hemorrhage,
necrosis and cystic
degeneration

Rubbery solid
homogenous
tumor invades the
renal parenchyma

Microscopic (histologic) description
  • Classic: resembles infantile fibromatosis or leiomyoma with fascicles and whorls of bland spindled myofibroblasts and thin collagen fibers; tumor surrounds tubules and glomeruli, has irregular borders; chondroid metaplasia / dysplasia of the entrapped tubules is common; mitoses are rare; necrosis / desmoplasia are not present
  • Cellular: resembles infantile fibrosarcoma with a sheet-like proliferation of plump, atypical spindle cells with abundant cytoplasm, vesicular nuclei and nucleoli; frequent mitotic figures (25 - 30 / 10 HPF) and necrosis; the tumor has a pushing border
  • Mixed: tumors with a combination of the above features
Microscopic (histologic) images
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Images hosted on PathOut server:

Prominent interlacing bundles

Tumor interdigitates with renal parenchyma

Perirenal invasion
with angiomatous
vascular proliferation


Embryonal metaplasia of Bowman capsule and entrapped tubules; #2 accompanied by epithelial hyperplasia

Extension into renal sinus
and along structures medial
to the kidney is an important
feature of most lesions



Cellular pattern:

Cellular and classic appearance

Mesoblastic Nephroma



Case of the Week #57:

Left to right: well circumscribed tumor; medium power; high power

Left to right: infiltrative margin; glomerular entrapment; angiomatoid area



Images hosted on other servers:

Resembles fibromatosis

Mitotic figures

Cellular areas have mitotic
figures, cystic degeneration,
hemorrhage and necrosis



Mixed pattern:

Mixed congenital mesoblastic nephroma



Cases resembling other tumors:

Left mesoblastic nephroma; right consistent with infantile fibrosarcoma

Cellular meso-
blastic nephroma
mimicking rhabdoid
tumor of kidney

Cellular congenital
mesoblastic nephroma
(CMN) mimicking
clear cell sarcoma

Virtual slides

Images hosted on other servers:

Mesoblastic nephroma

Cytology description
Positive stains
Negative stains
Molecular / cytogenetics description
Molecular / cytogenetics images

Images hosted on other servers:

Ideogram and FISH

Gel for fusion transcript

Differential diagnosis
  • Adult mixed epithelial and stromal tumor: mean age in 50s, usually women, similar morphology and staining pattern, also positive for estrogen and progesterone receptors
  • Clear cell sarcoma: similar age but has clear cells and chicken wire vasculature, tumor cells isolate single nephrons, fine nuclear chromatin and low mitotic rate; negative for smooth muscle markers
  • Metanephric stromal tumor: older age, nodular low power pattern, onion skin cuffing around entrapped renal tubules, heterologous differentiation and vascular changes, CD34+
  • Rhabdoid tumor: more invasive margins, usually epithelioid cells with cytoplasmic inclusions and prominent nucleoli, usually presents with metastases, INI1-
  • Wilms tumor: older age, also has blastema and nephrogenic rests; previously treated tumors may have well differentiated spindle cell stroma
Additional references