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Invasive breast carcinoma - IARC

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lipoma {1645}, hibernoma {2425} and<br />

chondrolipoma {1774a}. Adenomas containing<br />

glandular <strong>breast</strong> tissue such as<br />

adenolipoma are considered as hamartomas<br />

by some and variants of lipoma by<br />

others.<br />

Granular cell tumour<br />

Definition<br />

A tumour of putative schwannian origin<br />

consisting of cells with eosinophlic granular<br />

cytoplasm.<br />

ICD-O code 9580/0<br />

Epidemiology<br />

Granular cell tumour (GCT) can occur in<br />

any site of the body. It is relatively uncommon<br />

in the <strong>breast</strong> {2114}. It occurs more<br />

often in females than in males {430} with<br />

a wide age range from 17-75 years {325,<br />

617,668,3090} GCT is a potential mimicker<br />

of <strong>breast</strong> cancer, clinically, radiologically<br />

and grossly {608,617,995,2549}.<br />

Clinical features<br />

GCT generally presents as a single, firm,<br />

painless mass in the <strong>breast</strong> parenchyma<br />

but may be superficial causing skin<br />

retraction and even nipple inversion,<br />

w h e reas location deep in the bre a s t<br />

p a renchyma may lead to secondary<br />

involvement of the pectoralis fascia.<br />

Rarely, patients have simultaneous GCTs<br />

occurring at multiple sites in the body,<br />

including the <strong>breast</strong> {1920,1922}.<br />

Imaging typically shows a dense mass<br />

with stellate margin.<br />

Macroscopy<br />

GCT appears as a well circumscribed or<br />

infiltrative firm mass of 2–3 cm or less<br />

with a white to yellow or tan cut surface.<br />

H i s t o p a t h o l o g y<br />

The histology is identical to that seen in<br />

GCT at other sites of the body. There is<br />

an infiltrating growth pattern, even in<br />

lesions, which appear circumscribed on<br />

g ross examination. The cellular component<br />

is composed of solid nests, clusters<br />

or cords of round to polygonal cells<br />

with coarsely granular, eosinophilic periodic<br />

acid-Schiff (PAS) positive (diastase<br />

resistant) cytoplasm. due to the pre s-<br />

ence of abundant intracytoplasmic<br />

lysosomes. Aw a reness that GCT can<br />

occur in the <strong>breast</strong> is essential.<br />

I m m u n o reactivity with S-100 is important<br />

in confirming the diagnosis of GCT;<br />

lack of positivity for cytokeratins<br />

excludes <strong>breast</strong> carc i n o m a .<br />

Prognosis and predictive factors<br />

The clinical behaviour of GCT is usually<br />

benign following complete surgical<br />

excision. Rare l y, lymph node metastases<br />

have been re p o rted {668}. In contrast,<br />

a malignant course should be<br />

expected in the extremely rare malignant<br />

mammary GCTs which show<br />

nuclear pleomorphism, mitoses and<br />

n e c rosis {468}.<br />

Benign peripheral<br />

nerve sheath tumours<br />

D e f i n i t i o n<br />

Benign peripheral nerve sheath<br />

tumours (BPNST) include three distinct<br />

lesions usually occurring in the peripheral<br />

nerves or soft tissues: schwannomas<br />

composed of diff e re n t i a t e d<br />

Schwann cells; neuro f i b romas consisting<br />

of a mixture of Schwann cells,<br />

perineurial like cells and fibroblasts and<br />

p e r i n e u romas composed of perineural<br />

cells.<br />

ICD-O codes<br />

Schwannoma 9560/0<br />

Neurofibroma 9540/0<br />

Epidemiology<br />

The <strong>breast</strong> is only rarely the primary site<br />

of BPNST. There are only a few case<br />

reports of schwannomas {881,953,1081}<br />

and neuro f i b romas {1223,1675,2645},<br />

but to our knowledge primary perineuroma<br />

of the <strong>breast</strong> has not been recorded.<br />

Since neurofibromas may be part of neurofibromatosis<br />

type I (NF1), follow-up is<br />

needed because of the potential for<br />

malignant degeneration. The occurrence<br />

of <strong>breast</strong> cancer in the context of <strong>breast</strong><br />

neurofibromas has been reported {1948}.<br />

The age of patients at diagnosis is wide,<br />

ranging from 15 to 80 years with a prevalence<br />

of females.<br />

Clinical features<br />

The lesions present as a painless nodule<br />

and the pathology and immunophenotype<br />

is identical to their counterparts at<br />

other sites of the body.<br />

Angiosarcoma<br />

Definition<br />

A malignant tumour composed of neoplastic<br />

elements with the morphological<br />

properties of endothelial cells.<br />

ICD-O code 9120/3<br />

Synonyms<br />

These tumours include lesions which<br />

were formerly termed haemangiosarcoma,<br />

haemangioblastoma, lymphangiosarcoma<br />

and metastasizing haemangioma.<br />

Lymphangiosarcomas probably<br />

exist as a specific sarcoma of lymphatic<br />

endothelium but, at present, there is no<br />

A<br />

B<br />

Fig. 1.140 Primary mammary angiosarcoma. A, B Grade I (well differentiated) angiosarcoma showing interanastomosing channels containing red blood cells. The<br />

endothelial nuclei are prominent and hyperchromatic, but mitotic figures are absent. C High grade (poorly differentiated) angiosarcoma. Solid aggregates of pleomorphic<br />

endothelial cells surround vascular channels.<br />

C<br />

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

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