Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Renal medullary carcinoma
C.J. Davis
Definition
A rapidly growing tumour of the renal
medulla associated almost exclusively
with sickle cell trait.
ICD-O code 8319/3
eosinophilic with clear nuclei and usually
with prominent nucleoli. The sheets of
cells can have squamoid or rhabdoid
quality. Neutrophils are often admixed
with the tumour and the advancing margins
often bounded by lymphocytes.
Oedematous or collagenous stroma
forms a considerable bulk of many
Epidemiology
This is a rare tumour. Over a period of 22
years the Armed Forces Institute of
Pathology had collected only 34 cases
{562} and over the next 5 years only 15
more had been described {1304}.
Clinical features
Signs and symptoms
With few exceptions these are seen in
young people with sickle cell trait
between ages 10 and 40 (mean age 22
years) and chiefly in males by 2:1. The
common symptoms are gross haematuria
and flank or abdominal pain. Weight
loss and palpable mass are also common.
Metastatic deposits such as cervical
nodes or brain tumour may be the initial
evidence of disease {2119}.
Fig. 1.42 Renal medullary carcinoma. Infiltrating
tumour expanding renal contour.
Fig. 1.43 Renal medullary carcinoma. Infiltrating
tumour with perinephric extension at lower right.
Imaging
In the clinical setting of a young person
with sickle cell trait it is often possible to
anticipate the correct diagnosis with
imaging studies {557,1304}. Centrally
located tumours with an infiltrative
growth pattern, invading renal sinus, are
typical. Caliectasis without pelviectasis
and tumour encasing the pelvis are also
described.
Macroscopy
These are poorly circumscribed tumours
arising centrally in the kidney. Size
ranges from 4 to 12 cm with a mean of 7
cm. Most show much haemorrhage and
necrosis {562}.
Histopathology
Most cases have poorly differentiated
areas consisting of sheets of cells. A
reticular growth pattern and a more compact
adenoid cystic morphology are the
common features. The cells are
B
Fig. 1.44 Renal medullary carcinoma. A Adenoid cystic morphology. B Adenoid cystic area admixed with
neutrophils. Note lymphocytes at advancing margin. C Poorly differentiated area. Note sickled red cells at
lower left.
C
Renal medullary carcinoma
35