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Invasive breast carcinoma - IARC

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Diagnostic procedures<br />

Elevated urine 5-hydro x y i n d o l e a c e t i c<br />

acid (5-HIAA) and serum serotonin levels<br />

are found in patients with carcinoid syndrome<br />

{631,2388}.<br />

Macroscopy<br />

Primary ovarian carcinoids are unilateral<br />

and present as a firm tan nodule (less<br />

than 5 cm) protruding into a typical dermoid<br />

cyst (32-60% of tumours) or are<br />

predominantly solid with small cysts. The<br />

sectioned surface is firm, homogeneous<br />

and tan to yellow.<br />

Histopathology<br />

Insular carcinoid accounts for 26-53% of<br />

cases {631,2743}) and resembles midgut<br />

derivative carcinoids. It is composed of<br />

nests of round cells with uniform nuclei<br />

and abundant eosinophilic cytoplasm<br />

enclosing small red argentaffin granules<br />

at the periphery of the nests. Acinus formation<br />

and a cribriform pattern with luminal<br />

eosinophilic secretion are present<br />

{2388}.<br />

Trabecular carcinoid accounts for 23-<br />

29% of cases {631,2743} and resembles<br />

hindgut or foregut derivative carcinoids.<br />

It exhibits wavy and anastomosing ribbons<br />

composed of columnar cells with<br />

the long axes of the cells parallel to one<br />

another and oblong nuclei with prominent<br />

nucleoli. The abundant cytoplasm is<br />

finely granular with red-orange argyrophilic<br />

granules at both poles of the<br />

nucleus {2392}.<br />

Mucinous carcinoid accounts for only<br />

1.5% of cases {2743} and resembles<br />

goblet cell carcinoids arising in the<br />

appendix. The well differentiated mucinous<br />

carcinoid is composed of numerous<br />

small glands lined by columnar or<br />

cuboidal cells, some of which contain<br />

intracytoplasmic mucin or have a goblet<br />

cell appearance, whilst others disclose<br />

Fig. 2.107 Carcinoid arising in a teratoma. Nests and cords of carcinoid cells proliferate next to a chrondroid<br />

nodule.<br />

o r a n g e - red neuroendocrine granules.<br />

Individual tumour cells may contain both<br />

mucin and neuroendocrine granules.<br />

Glands may be floating within pools of<br />

mucin that also dissect the surrounding<br />

fibrous stroma with isolated signet-ring<br />

cells infiltrating the stroma. Atypical<br />

mucinous carcinoid demonstrates<br />

crowded glands or a cribriform pattern.<br />

Carcinoma arising in mucinous carcinoid<br />

exhibits large islands of tumour cells or<br />

closely packed glands with high grade<br />

nuclei, numerous mitoses and necrosis<br />

{166}.<br />

Strumal carcinoid accounts for 26-44%<br />

of cases {631,2743} and is composed of<br />

a variable proportion of thyroid tissue<br />

and carcinoid, the latter mostly having a<br />

trabecular arc h i t e c t u re. The neuro e n-<br />

docrine cells invade progressively the<br />

strumal component, replacing the follicular<br />

lining cells. Glands or cysts lined by<br />

columnar epithelium with goblet cells<br />

may be found {2390}.<br />

Carcinoids with mixed patterns (essentially<br />

insular and trabecular), are classified<br />

according to the pattern that predominates<br />

{2388}.<br />

Immunoprofile<br />

C a rcinoids are immunoreactive to at least<br />

one of the neuroendocrine markers (chromogranin,<br />

synaptophysin, Leu-7) and<br />

various peptide hormones such as panc<br />

reatic polypeptide, gastrin, vasoactive<br />

intestinal peptides and glucagon {166}.<br />

Differential diagnosis<br />

Metastatic gastrointestinal carcinoid to<br />

the ovary should be ruled out specifically<br />

when extraovarian disease is detected.<br />

Bilateral and multinodular ovarian<br />

involvement, the absence of other teratomatous<br />

components and the persistence<br />

of the carcinoid syndrome after<br />

oophorectomy favour the diagnosis of<br />

metastasis {166,2391}.<br />

Prognosis and predictive features<br />

Almost all primary trabecular and strumal<br />

carcinoids occur in women with stage I<br />

disease and have an excellent outcome.<br />

The overall survival of patients with insular<br />

carcinoid is 95% at 5 years and 88%<br />

at 10 years {2388}.<br />

P r i m a ry ovarian mucinous carcinoid, like<br />

those in the appendix, has a more agg<br />

ressive behaviour with extraovarian<br />

s p read and lymph node metastases. The<br />

p resence of frank <strong>carcinoma</strong> within the<br />

tumour is an important prognostic factor<br />

{166}.<br />

Fig. 2.106 Struma ovarii. The struma resembles a thyroid<br />

microfollicular adenoma with dystrophic nuclei.<br />

Neuroectodermal tumours<br />

Definition<br />

Tumours composed almost exclusively of<br />

neuroectodermal tissue, closely resem-<br />

Germ cell tumours 173

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