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Invasive breast carcinoma - IARC

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Glycogen–rich, clear cell<br />

<strong>carcinoma</strong> (GRCC)<br />

Definition<br />

A <strong>carcinoma</strong> in which more than 90%<br />

of the neoplastic cells have abundant<br />

clear cytoplasm containing glycogen.<br />

ICD-O code 8315/3<br />

Synonyms<br />

Clear cell <strong>carcinoma</strong> 8310/3<br />

Glycogen-rich <strong>carcinoma</strong> 8315/3<br />

Epidemiology<br />

The frequency is from 1-3% of <strong>breast</strong> <strong>carcinoma</strong>s<br />

{880,1264}, with an age range<br />

of 41-78 years, median 57 years {2870}.<br />

Clinical features<br />

These tumours show similar presentation<br />

features to ductal NOS <strong>carcinoma</strong>.<br />

Macroscopy<br />

The clear cell glycogen-rich <strong>carcinoma</strong><br />

does not differ grossly from that of usual<br />

invasive or intraductal <strong>carcinoma</strong> {1165}.<br />

The neoplasm ranges from 1 to 8 cm in<br />

size {2422,2754,2870}.<br />

Histopathology<br />

A strict definition for clear cell glycogenrich<br />

is necessary for two re a s o n s .<br />

C a rcinomas in the <strong>breast</strong> with a clear cell<br />

appearance are uncommon and are due<br />

to an artefact produced by extraction of<br />

intracytoplasmic substances during tissue<br />

processing. However, as the substances<br />

that are extracted diff e r, they may<br />

be of diff e rent biological significance. In<br />

addition, intracytoplasmic glycogen has<br />

been observed without significant clear<br />

cell in 58% of <strong>breast</strong> <strong>carcinoma</strong> {880}.<br />

The lesions usually have the structural<br />

f e a t u res of intraductal and infiltrating<br />

ductal neoplasms but rarely those of lobular,<br />

medullary or tubular types have<br />

been noted. GRCCs has either circumscribed<br />

or infiltrative borders {880,165,<br />

2754,2870}.<br />

The in situ component, either in the<br />

p u re form or in association with most<br />

invasive cases has a compact solid,<br />

comedo or papillary growth pattern .<br />

The invasive tumour is generally composed<br />

of solid nests, rarely of tubular or<br />

p a p i l l a ry structures.<br />

The tumour cells tend to have sharply<br />

defined borders and polygonal contours.<br />

The clear or finely granular cytoplasm<br />

contains PAS positive diastase labile<br />

glycogen. The nuclei are hyperc h ro m a t i c ,<br />

with clumped chromatin and pro m i n e n t<br />

n u c l e o l i .<br />

Differential diagnosis<br />

To differentiate this tumour from other<br />

clear cell tumours, including lipid rich<br />

<strong>carcinoma</strong>, histiocytoid <strong>carcinoma</strong>, adenomyoepithelioma,<br />

clear cell hidradenoma<br />

and metastatic clear cell <strong>carcinoma</strong><br />

( p a rticularly of renal origin), enzyme<br />

c y t o c h e m i s t ry and immunohistochemistry<br />

are useful {702,1165,1549,2754}.<br />

Immunoprofile<br />

Hormone receptor status is similar to<br />

ductal NOS {880}.<br />

Prognosis and predictive factors<br />

Most reports suggest that GRCC is more<br />

aggressive than typical ductal <strong>carcinoma</strong><br />

{2313,2754}. The incidence of axillary<br />

lymph node invasion is significantly higher<br />

than in the other non-GRCC forms<br />

{1264}. The histologic grade is intermediate<br />

to high with a paucity of grade I<br />

tumours {1165}.<br />

Although follow up studies confirm that<br />

disease free and overall survival is significantly<br />

worse in GRCC, due to the low<br />

incidence, there are no multiparametric<br />

analyses to compare GRCC stage by<br />

stage with the other histological types of<br />

<strong>breast</strong> <strong>carcinoma</strong>.<br />

Sebaceous <strong>carcinoma</strong><br />

Definition<br />

A primary <strong>breast</strong> <strong>carcinoma</strong> of the skin<br />

adnexal type with sebaceous diff e re n t i a-<br />

tion. There should be no evidence of derivation<br />

from cuteneous adnexal sebaceous<br />

glands.<br />

ICD-O code 8410/3<br />

Epidemiology<br />

Only 4 examples of this rare mammary<br />

tumour have been observed {2876}. The<br />

women, three of whom were white, were<br />

aged 45-62 years {2876,3006}.<br />

Clinical features<br />

All the patients presented with a palpable<br />

mass.<br />

Macroscopy<br />

The tumours range in size from 7.5-20<br />

cm. The margins are sharply delineated,<br />

and the cut surface is solid and bright<br />

y e l l o w.<br />

A<br />

Fig. 1.62 Glycogen-rich <strong>carcinoma</strong>. A Cells with abundant clear cytoplasm and relatively uniform round nuclei grow in a solid pattern supported by branching<br />

vessels. B Note transition from typical ductal epithelial cells to clear cells in a duct adjacent to the invasive <strong>carcinoma</strong>.<br />

B<br />

46 Tumours of the <strong>breast</strong>

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