Breast cancer

- Commoner in the older age group

 Invasive ductal carcinomas are the most common type. Some may arise as a result of ductal
carcinoma in situ (DCIS). There are associated carcinomas of special type e.g. Tubular that may
carry better prognosis.
 The pathological assessment involves assessment of the tumour and lymph nodes, sentinel lymph
node biopsy is often used to minimise the morbidity of an axillary dissection.
 Treatment, typically this is either wide local excision or mastectomy. There are many sub types of
both of these that fall outside of the MRCS. Some key rules to bear in mind.
 Whatever operation is contemplated the final cosmetic outcome does have a bearing. A woman
with small breasts and a large tumour will tend to fare better with mastectomy, even if clear
pathological and clinical margins can be obtained. Conversely a women with larger breasts may
be able to undergo breast conserving surgery even with a relatively large primary lesion (NB
tumours >4cm used to attract recommendation for mastectomy). For screen detected and
impalpable tumour image guidance will be necessary.
 Reconstruction is always an option following any resectional procedure. However, its exact type
must be tailored to age and co-morbidities of the patient. The main operations in common use
include latissimus dorsi myocutaneous flap and sub pectoral implants. Women wishing to avoid a
prosthesis may be offered TRAM or DIEP flaps.

Surgical options
Mastectomy vs Wide local excision

Mastectomy Wide Local Excision

Multifocal tumour Solitary lesion
Central tumour Peripheral tumour
Large lesion in small breast Small lesion in large breast
DCIS >4cm DCIS <4cm
Patient Choice Patient choice

Central lesions may be managed using breast conserving surgery where an acceptable cosmetic result may
be obtained, this is rarely the case in small breasts

A compelling indication for mastectomy, a larger tumour that would be unsuitable for breast conserving
surgery

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 Image sourced from Wikipedia

Whatever surgical option is chosen the aim should be to have a local recurrence rate of 5% or less at 5
years [1].

Nottingham Prognostic Index

The Nottingham Prognostic Index can be used to give an indication of survival. In this system the tumour
size is weighted less heavily than other major prognostic parameters.

Calculation of NPI
Tumour Size x 0.2 + Lymph node score(From table below)+Grade score(From table below).

Score Lymph nodes involved Grade

1 0 1
2 1-3 2
3 >3 3

Prognosis

Score Percentage 5 year survival

2.0 to 2.4 93%
2.5 to 3.4 85%
3.5 to 5.4 70%
>5.4 50%

This data was originally published in 1992. It should be emphasised that other factors such as vascular
invasion and receptor status also impact on survival and are not included in this data and account for
varying prognoses often cited in the literature.

2
Breast cancer management
- Surgery is performed in most patients suffering from breast cancer.

 Chemotherapy may be used to downstage tumours and allow breast conserving surgery.
Hormonal therapy may also be used for the same purposes.
 Radiotherapy is given to most patients who have undergone breast conserving surgery (some
older patients receiving hormone treatment and who have small low grade tumours may safely
avoid DXT.
 Therapeutic mammoplasty is an option for some patients but requires symmetrizing surgery in
most cases.
 Patients who have undergone mastectomy may be offered a reconstructive procedure either in
conjunction with their primary resection or as a staged procedure at a later date.

Surgical options
Mastectomy vs Wide local excision

Mastectomy Wide Local Excision

Multifocal tumour Solitary lesion
Central tumour Peripheral tumour
Large lesion in small breast Small lesion in large breast
DCIS >4cm DCIS <4cm
Patient Choice Patient choice

Central lesions may be managed using breast conserving surgery, where an acceptable cosmetic result may
be obtained, this is rarely the case in small breasts

Axillary disease

 As a minimum, all patients with invasive breast cancer should have their axilla staged. In those
who do not have overt evidence of axillary nodal involvement this can be undertaken using
sentinel lymph node biopsy.
 Patients with a positive sentinel lymph node biopsy or who have imaging and cytological or
histological evidence of axillary nodal metastasis should undergo axillary node clearance or
axillary irradiation.
 Axillary node clearance is associated with the development of lymphoedema, increased risk of
cellulitis and frozen shoulder.
Thyroid disease
Patients may present with a number of different manifestations of thyroid disease. They can be broadly
sub classified according to whether they are euthyroid or have clinical signs of thyroid dysfunction. In
addition it needs to be established whether they have a mass or not.

Assessment

 History
 Examination including USS

3
  If a nodule is identified then it should be sampled ideally via an image guided fine needle
aspiration
 Radionucleotide scanning is of limited use

Thyroid Tumours

 Papillary carcinoma
 Follicular carcinoma
 Anaplastic carcinoma
 Medullary carcinoma
 Lymphoma's

Multinodular goitre

 One of the most common reasons for presentation

 Provided the patient is euthyroid and asymptomatic and no discrete nodules are seen, they can
be reassured.
 In those with compressive symptoms surgery is required and the best operation is a total
thyroidectomy.
 Sub total resections were practised in the past and simply result in recurrent disease that requires
a difficult revisional resection.

Endocrine dysfunction

 In general these patients are managed by physicians initially.

 Surgery may be offered alongside radio iodine for patients with Graves disease that fails with
medical management or in patients who would prefer not to be irradiated (e.g. pregnant women).
 Patients with hypothyroidism do not generally get offered a thyroidectomy. Sometimes people
inadvertently get offered resections during the early phase of Hashimotos thyroiditis, however,
with time the toxic phase passes and patients can simply be managed with thyroxine.

Complications following surgery

 Anatomical such as recurrent laryngeal nerve damage.

 Bleeding. Owing to the confined space haematoma's may rapidly lead to respiratory compromise
owing to laryngeal oedema.
 Damage to the parathyroid glands resulting in hypocalcaemia.
Hyperthyroidism
Causes of hyperthyroidism include:

 Diffuse toxic goitre (Graves Disease)

 Toxic nodular goitre

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  Toxic nodule
 Rare causes

Graves disease
Graves disease is characterised by a diffuse vascular goitre that appears at the same time as the clinical
manifestations of hyperthyroidism. It is commonest in younger females and may be associated with eye
signs. Thyrotoxic symptoms will predominate. Up to 50% of patients will have a familial history of
autoimmune disorders. The glandular hypertrophy and hyperplasia occur as a result of the thyroid
stimulating effects of the TSH receptor antibodies.

Toxic nodular goitre

In this disorder the goitre is present for a long period of time prior to the development of clinical
symptoms. In most goitres the nodules are inactive and in some cases it is the internodular tissue that is
responsible for the hyperthyroidism.

Toxic nodule
Overactive, autonomously functioning nodule. It may occur as part of generalised nodularity or be a true
toxic adenoma. The TSH levels are usually low as the autonomously functioning thyroid tissue will exert a
negative feedback effect.

Signs and symptoms

Symptoms Signs
Lethargy Tachycardia
Emotionally labile Agitation
Heat intolerance Hot, moist palms
Weight loss Exopthalmos
Excessive appetite Thyroid goitre and bruit
Palpitations Lid lag/retraction

Diagnosis
The most sensitive test for diagnosing hyperthyroidism is plasma T3 (which is raised). Note in
hypothyroidism the plasma T4 and TSH are the most sensitive tests. A TSH level of <0.5U/L suggests
hyperthyroidism. TSH receptor antibodies may be tested for in the diagnosis of Graves.

Treatment
First line treatment for Graves disease is usually medical and the block and replace regime is the favored
option. Carbimazole is administered at higher doses and thyroxine is administered orally. Patients are
maintained on this regime for between 6 and 12 months. Attempts are then made to wean off
medication. Where relapse then occurs the options are between ongoing medical therapy, radioiodine or
surgery.

Parathyroid glands and disorders of calcium metabolism

Hyperparathyroidism
Disease type Hormone profile Clinical features Cause
Primary  PTH (Elevated)  May be Most cases due to solitary
hyperparathyroidism  Ca2+ (Elevated) asymptomatic if mild adenoma (80%), multifocal
 Phosphate (Low)  Recurrent abdominal disease occurs in 10-15% and
 Urine calcium : pain (pancreatitis, renal parathyroid carcinoma in 1%

5
 Disease type Hormone profile Clinical features Cause
creatinine clearance colic) or less
ratio > 0.01  Changes to emotional
or cognitive state
Secondary  PTH (Elevated)  May have few Parathyroid gland hyperplasia
hyperparathyroidism  Ca2+ (Low or normal) symptoms occurs as a result of low
 Phosphate (Elevated)  Eventually may calcium, almost always in a
 Vitamin D levels (Low) develop bone disease, setting of chronic renal failure
osteitis fibrosa cystica
and soft tissue
calcifications
Tertiary  Ca (Normal or high)  Metastatic
2+
Occurs as a result of ongoing
hyperparathyroidism  PTH (Elevated) calcification hyperplasia of the parathyroid
 Phosphate levels  Bone pain and / or glands after correction of
(Decreased or Normal) fracture underlying renal disorder,
 Vitamin D (Normal or  Nephrolithiasis hyperplasia of all 4 glands is
decreased)  Pancreatitis usually the cause
 Alkaline phosphatase
(Elevated)

Differential diagnoses
It is important to consider the rare but relatively benign condition of benign familial hypocalciuric
hypercalcaemia, caused by an autosomal dominant genetic disorder. Diagnosis is usually made by genetic
testing and concordant biochemistry (urine calcium : creatinine clearance ratio <0.01-distinguished from
primary hyperparathyroidism).

Treatment

Primary hyperparathyroidism
Indications for surgery

 Elevated serum Calcium > 1mg/dL above normal

 Hypercalciuria > 400mg/day
 Creatinine clearance < 30% compared with normal
 Episode of life threatening hypercalcaemia
 Nephrolithiasis
 Age < 50 years
 Neuromuscular symptoms
 Reduction in bone mineral density of the femoral neck, lumbar spine, or distal radius of more than
2.5 standard deviations below peak bone mass (T score lower than -2.5)

Secondary hyperparathyroidism
Usually managed with medical therapy.

Indications for surgery in secondary (renal) hyperparathyroidism:

 Bone pain
 Persistent pruritus

6
  Soft tissue calcifications

Tertiary hyperparathyroidism
Allow 12 months to elapse following transplant as many cases will resolve
The presence of an autonomously functioning parathyroid gland may require surgery. If the culprit gland
can be identified then it should be excised. Otherwise total parathyroidectomy and re-implantation of part
of the gland may be required.

Phaeochromocytoma and adrenal lesions

Phaeochromocytoma
Neuroendocrine tumour of the chromaffin cells of the adrenal medulla. Hypertension and hyperglycaemia
are often found.

 10% of cases are bilateral.

 10% occur in children.
 11% are malignant (higher when tumour is located outside the adrenal).
 10% will not be hypertensive.

Familial cases are usually linked to the Multiple endocrine neoplasia syndromes (considered under its own
heading).

Most tumours are unilateral (often right sided) and smaller than 10cm.

Diagnosis
Urine analysis of vanillymandelic acid (VMA) is often used (false positives may occur e.g. in patients eating
vanilla ice cream!)

Blood testing for plasma metanephrine levels.

CT and MRI scanning are both used to localise the lesion.

Treatment
Patients require medical therapy first. An irreversible alpha adrenoreceptor blocker should be given,
although minority may prefer reversible blockade(1). Labetolol may be co-administered for cardiac
chronotropic control. Isolated beta blockade should not be considered as it will lead to unopposed alpha
activity.

These patients are often volume depleted and will often require moderate volumes of intra venous normal
saline perioperatively.

Once medically optimised the phaeochromocytoma should be removed. Most adrenalectomies can now
be performed using a laparoscopic approach(2). The adrenals are highly vascular structures and removal
can be complicated by catastrophic haemorrhage in the hands of the inexperienced. This is particularly
true of right sided resections where the IVC is perilously close. Should the IVC be damaged a laparotomy
will be necessary and the defect enclosed within a Satinsky style vascular clamp and the defect closed with
prolene sutures. Attempting to interfere with the IVC using any instruments other than vascular clamps
will result in vessel trauma and make a bad situation much worse.

7
Incidental adrenal lesions
Adrenal lesions may be identified on CT scanning performed for other reasons(3). Factors suggesting
benign disease on CT include(4):

 Size less than 3cm

 Homogeneous texture
 Lipid rich tissue
 Thin wall to lesion

All patients with incidental lesions should be managed jointly with an endocrinologist and full work up as
described above. Patients with functioning lesions or those with adverse radiological features (Particularly
size >3cm) should proceed to surgery.

Abberations of normal development and involution- breast

Fibroadenoma
Under the age of 25 years the breast is usually classified as undergoing development. Lobular units are
being formed and a dense stroma is formed within the breast tissue. This may result in the development
of fibroadenomas.
As a group, fibroadenomas account for 13% of all palpable breast lesions. However, in women aged 18-25
they constitute up to 60% of all palpable breast lesions. The are classified as juvenile, common and giant.
The former occur in early adolescence and the latter are characterised by a size greater than 4cm. In
young females with small fibroadenomas (less than 3cm on imaging) a policy of watchful waiting without
biopsy may be adopted. A size of greater than 4cm attracts a recommendation for core biopsy to exclude
a phyllodes tumour. The natural history of fibroadenomas is that 10% will increase in size, 30% regress
and the remainder stay the same. This does not apply during pregnancy and lactation when they may
increase in size substantially and subsequently sequester milk.
Some women may wish to have their fibroadenomas excised, they can usually be shelled out through a
circumareolar incision. Smaller lesions may be removed using a mammotome.

Breast cysts
Palpable cysts constitute 15% of all breast lumps. They occur most frequently in perimenopausal females
and are caused by distended and involuted lobules.
They may be readily apparent on clinical examination as soft, fluctuant swellings. It is important to exclude
the presence of an underlying mass lesion. On imaging they will usually show a 'halo appearance' on
mammography. Ultrasound will confirm the fluid filled nature of the cyst. Symptomatic cysts may be
aspirated and following aspiration the breast re-examined to ensure that the lump has gone.

Duct ectasia
As women progress through the menopause the breast ducts shorten and dilate. In some women this
may cause a cheese like nipple discharge and slit like retraction of the nipple. No specific treatment is
required.
Benign breast lesions

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 Lesion Features Treatment
Fibroadenoma  Develop from a whole lobule If >3cm surgical excision is usual,
 Mobile, firm breast lumps Phyllodes tumours should be widely
 12% of all breast masses excised (mastectomy if the lesion is
 Over a 2 year period up to 30% large)
will get smaller
 No increase in risk of malignancy
Breast cyst  7% of all Western females will Cysts should be aspirated, those which
present with a breast cyst are blood stained or persistently refill
 Usually presents as a smooth should be biopsied or excised
discrete lump (may be fluctuant)
 Small increased risk of breast
cancer (especially if younger)
Sclerosing adenosis,  Usually presents as a breast lump Lesions should be biopsied, excision is
(radial scars and complex or breast pain not mandatory
sclerosing lesions)  Causes mammographic changes
which may mimic carcinoma
 Cause distortion of the distal
lobular unit, without hyperplasia
(complex lesions will show
hyperplasia)
 Considered a disorder of
involution, no increase in
malignancy risk
Epithelial hyperplasia  Variable clinical presentation If no atypical features then conservative,
ranging from generalised those with atypical features require
lumpiness through to discrete lump either close monitoring or surgical
 Disorder consists of increased resection
cellularity of terminal lobular unit,
atypical features may be present
 Atypical features and family
history of breast cancer confers
greatly increased risk of
malignancy
Fat necrosis  Up to 40% cases usually have a Imaging and core biopsy
traumatic aetiology
 Physical features usually mimic
carcinoma
 Mass may increase in size initially
Duct papilloma  Usually present with nipple Microdochectomy
discharge
 Large papillomas may present with
a mass
 The discharge usually originates
from a single duct
 No increase risk of malignancy

Management of thyroid disease

Management of thyroid disease

9
 Lesion
Large multinodular goitre
Toxic nodule
Follicular lesion (THY 3f)
Papillary thyroid cancer
Follicular thyroid cancer
Anaplastic thyroid cancer
Medullary thyroid cancer
Lymphoma of the thyroid
Persistent refilling cysts
Graves disease with significant
eye signs
Graves disease without
significant eye signs
Management
Surgery for pressure symptoms. Total thyroidectomy is treatment of choice
Hemithyroidectomy
Hemithyroidectomy to establish diagnosis
Total thyroidectomy and central compartment nodal dissection (extended
lymphadenectomy as required)
Total thyroidectomy (usually completion as already had hemithyroidectomy)
Palliative radiotherapy
Total thyroidectomy and central compartment nodal dissection (screen for
other MEN tumours)
Consider core biopsy
Injection sclerotherapy and surgery if this fails
Total thyroidectomy
Patient choice radioiodine Vs surgery

Non malignant breast disease

Duct ectasia

 Mammary duct ectasia may be seen in up to 25% of normal female breasts

 Patients usually present with nipple discharge, which may be from single or multiple ducts
(usually present age >50 years)
 The discharge is often thick and green
 Duct ectasia is a normal variant of breast involution and is not the same condition as periductal
mastitis
 Mass develops behind nipple

Periductal mastitis

 Present at younger age than duct ectasia

 May present with features of inflammation, abscess or mammary duct fistula
 Strongly associated with smoking
 Usually treated with antibiotics, abscess will require drainage
 Mass develops around nipple

Intraductal papilloma

 Growth of papilloma in a single duct

 Usually presents with clear or blood stained discharge originating from a single duct
 No increase in risk of malignancy

10
Breast abscess

 Lactational mastitis is common

 Infection is usually with Staphylococcus aureus
 On examination there is usually a tender fluctuant mass
 Treatment is with antibiotics and ultrasound guided aspiration
 Overlying skin necrosis is an indication for surgical debridement, which may be complicated by
the development of a subsequent mammary duct fistula.

Tuberculosis

 Rare in western countries, usually secondary TB

 Affects women later in child bearing period
 Chronic breast or axillary sinus is present in up to 50% cases
 Diagnosis is by biopsy culture and histology
Thyroid function tests
The interpretation of thyroid function tests is usually straightforward:

Disorder TSH Free T4

Thyrotoxicosis (e.g. Graves' disease) Low High In T3 thyrotoxicosis the free T4 will be
normal
Primary hypothyroidism (primary atrophic High Low
hypothyroidism)
Secondary hypothyroidism Low Low Replacement steroid therapy is required
prior to thyroxine
Sick euthyroid syndrome* Low** Low Common in hospital inpatients
Poor compliance with thyroxine High Normal /
high
Steroid therapy Low Normal

*now referred to as non-thyroidal illness

**TSH may be normal in some cases

Thyroiditis
Sub acute thyroiditis
Subacute thyroiditis (also known as De Quervain's thyroiditis) is thought to occur following viral infection
and typically presents with hyperthyroidism

Features

11
  Hyperthyroidism
 Painful goitre
 Raised ESR
 Globally reduced uptake on iodine-131 scan

Management

 Usually self-limiting - most patients do not require treatment

 Thyroid pain may respond to aspirin or other NSAIDs
 In more severe cases steroids are used, particularly if hypothyroidism develops

Hashimotos thyroiditis
Hashimotos thyroiditis is an immunological disorder in which lymphocytes become sensitised to thyroidal
antigens. The three most important antibodies include; thyroglobulin, TPO and TSH-R. During the early
phase of Hashimotos the the thyroglobulin antibody is markedly elevated and then declines.

Features

 Goitre and either euthyroid or mild hypothyroidism

 Progressive hypothyroidism (and associated symptoms)

Management

 During the hyperthyroid phase of illness beta blockers may manage symptoms
 As hypothyroidism develops patients may require thyroxine

Blood testing in thyroid disease

Assay Usage
Thyroid peroxidase  Found in autoimmune disease affecting the thyroid (Hashimotos 100%)
(microsomal) antibodies and Graves (70%)
Antibodies to TSH receptor  Individuals with Graves disease (95%)
Thyroglobulin  Not useful for clinically distinguishing between different types of thyroid
disease, may be used as part of thyroid cancer follow up
Calcitonin  Released from the parafollicular cells
 Usually found in patients with medullary carcinoma of the thyroid

Complications following thyroid surgery

The major immediate risk following thyroidectomy is haemorrhage. A tension haematoma deep to the
cervical fascia is usually the result of reactionary haemorrhage from an arterial source. This results in the
development of laryngeal oedema and airway compromise. The treatment is by urgent wound
decompression of all layers prior to return to theatre for haemostasis. Sub cutnaeous haematomas and
seromas may accumulate under skin flaps and can often be managed conservatively or by simple
aspiration.

12
In patients with longstanding large goitres there is the risk of tracheomalacia. This is characterised by the
development of flacidity of the tracheal cartilage. This can result in airway compromise. In the normal
situation the tracheal diameter increases slightly during inspiration and narrows during expiration. In
tracheomalacia these processes are exaggerated and the trachea may collapse in expiration resulting in
stridor. In the immediate situation an endotracheal tube will need to be inserted.

Recurrent laryngeal nerve injury is recognised following thyroid surgery and may be unilateral or bilateral
depending upon the procedure performed. The risk is 1.8% at one month which declines to 0.5% at three
months following first time explorations. It is rare for nerve injury alone to result in airway compromise.
However, it may occur when nerve injury is associated with minor degrees of laryngeal oedema (such as
following intubation).

Hypoparathyroidism is a recognised complication following thyroid surgery and damage to the blood
supply to the parathyroid glands is probably the commonest cause. The incidence of permanent
hypoparathyroidism is in the region of 1 to 3%. It can present dramatically in the first 2-5 post operative
days. In the emergency setting treatment is with intravenous calcium gluconate. Oral calcium carbonate is
used in the longer term.

Gynaecomastia
Gynaecomastia describes an abnormal amount of breast tissue in males and is usually caused by an
increased oestrogen:androgen ratio. It is important to differentiate the causes of galactorrhoea (due to
the actions of prolactin on breast tissue) from those of gynaecomastia

Causes of gynaecomastia

 physiological: normal in puberty

 syndromes with androgen deficiency: Kallman's, Klinefelter's
 testicular failure: e.g. Mumps
 liver disease
 testicular cancer e.g. Seminoma secreting HCG
 ectopic tumour secretion
 hyperthyroidism
 haemodialysis
 drugs: see below

Drug causes of gynaecomastia

 spironolactone (most common drug cause)

 cimetidine
 digoxin

13
  cannabis
 finasteride
 oestrogens, anabolic steroids

Very rare drug causes of gynaecomastia

 tricyclics
 isoniazid
 calcium channel blockers
 heroin
 busulfan
 methyldopa

Treatment options

 identify and manage any overt underlying cause

 liposuction provides the best cosmetic outcome
Primary hyperparathyroidism
In exams, primary hyperparathyroidism is stereotypically seen in elderly females with an unquenchable
thirst and an inappropriately normal or raised parathyroid hormone level. It is most commonly due to a
solitary adenoma

Causes of primary hyperparathyroidism

 80%: solitary adenoma

 15%: hyperplasia
 4%: multiple adenoma
 1%: carcinoma

Features - 'bones, stones, abdominal groans and psychic moans'

 Polydipsia, polyuria
 Peptic ulceration/constipation/pancreatitis
 Bone pain/fracture
 Renal stones
 Depression
 Hypertension

Associations

 Hypertension
 Multiple endocrine neoplasia: MEN I and II

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Investigations

 Raised calcium, low phosphate

 PTH may be raised or normal
 Technetium-MIBI subtraction scan

Treatment

 Parathyroidectomy, if imaging suggests target gland then a focused approach may be used

Thyroglossal cysts
The commonest midline neck mass in children is a congenital cyst of the thyroglossal duct.
Embryologically, the cyst can arise at any point along the route of the thyroglossal duct, extending from
the foramen caecum to the thyroid gland.
They are typically located below the hyoid and moves both on swallowing and tongue protrusion. Whilst
most cysts are asymptomatic, associated infection may result in pain and swelling.
The usual treatment is excision of the cyst and track, this is a called a Sistrunk procedure.
Breast imaging
Breast imaging is undertaken with a combination of USS and mammography in most women presenting
with a palpable lump. In younger patients, the denser breasts encountered in this group may make
mammography less informative.

In women who present with breast implants with a palpable lump, the imaging of choice remains the
same. However, specialized imaging techniques may be needed to obtain optimal mammographic views.
Where there are specific concerns about a breast implant, rather than a lump, the imaging modality of
choice is MRI scanning.
MRI scanning may be beneficial in screening younger patients with a family history and also in patients
with lobular cancers who are being considered for breast conserving surgery.
Complications of breast surgery
Breast surgery may be associated with the following complications:

 Long thoracic nerve injury. This may occur during the axillary dissection and result in winging of
the scapula.
 Intercostobrachial nerve injury. These nerves traverse the axilla. When they are divided (which
they often are) the patient will notice an area of parasthesia in the armpit.
 Injury to the thoracodorsal trunk. This nerve and vessels supply latissimus dorsi. If they are
damaged the functional effects are not too serious, the greatest setback is that a latissimus dorsi
flap cannot be used for reconstruction purposes.
 Infections. Cellulitis of the chest wall and arm may be a major problem if axillary nodal clearance
is undertaken. Infections may run a protracted course and require polytherapy for treatment.

15
  Lymphoedema. Usually complicates axillary node clearance or irradiation. Treatment is with
manual lymphatic drainage and compression sleeves.
 Seroma. This is an accumulation of fluid at the site of surgery. The fluid is usually straw coloured
and may re-accumulate despite drainage. Most will resolve with time.
Familial breast cancer
The general population risk of breast cancer in the UK is 11%.
Women with a moderate risk of breast cancer have a risk of breast cancer between 17% and under 30%
Women with high risk of breast cancer have risks in excess of 30%.
All women who have a faulty BRCA1, BRCA2 or TP53 gene are at high risk.

Family history and risk

The following indicate high family history risk (and referral for genetics)
Female breast cancers only
1 first degree relative
Under 40
OR
2 first degree relatives
Any age
OR
1 first and 1 second-degree relative
Any age
OR
3 first degree relatives
Any age
OR
3 second-degree relatives
Any age

Drug treatment
Premenopausal women at high risk can be offered tamoxifen
Post menopausal women at high risk can be offered anastrazole.
Where drug treatment is given, the period of dosage should not exceed 5 years.

Surgery
Women at high risk of developing breast cancer can be offered a risk reducing bilateral mastectomy (with
reconstruction if wished). Drug treatments as outlined above can be used whilst patients are considering
surgical options.
Pancreatic endocrine tumours
Insulinoma

 Insulin producing tumours of the pancreatic β cells

 Incidence of 1 per 1,000,000 per year

16
  90% of lesions are benign
 Most tumours less than 2cm in size
 Between 5 and 10% have MEN type 1
 75% of patients with MEN 1 will develop pancreatic islet cell tumours

Typical features of insulinoma

Symptomatic hypoglycaemia during fasting
Concomitant blood glucose of less than 3mmol/L
Relief of hypoglycaemia by use of glucose

Testing
When neuroglycopenic symptoms occur blood is taken for serum insulin levels, serum glucose, C-peptide
and pro insulin concentrations. The plasma insulin concentration is >10 micro U/ml in patients with the
disorder.

Tumour localisation

 USS (25% accuracy), endoscopic USS better (75% accuracy)

 CT scanning (pancreatic protocol=40% accuracy)
 Malignant insulinomas are larger and diagnostic accuracy with MRI is nearly 100% in such cases
 Somatostatin receptor scintigraphy (50% accuracy)

Treatment
Since the majority of tumours are benign; the blind segmental resection of the pancreas (e.g. Whipples)
cannot be justified, this may be considered acceptable for malignant lesions. The best approach at
laparotomy is to corroborate pre operative imaging with intraoperative ultrasonography to identify the
lesion. Tumours may be close of the pancreatic duct and this must be appreciated by the operating
surgeon. The perioperative use of octreotide reduces the amount of pancreatic drainage, but not overall
complications.

Glucagonoma
Glucagonomas originate in the alpha 2 cells of the pancreas. Symptoms are generally related to hormone
overproduction and include diabetes, hypoaminoacidemia, normochromic and normocytic anemia, and
necrolytic migratory erythema (NME), which is the most characteristic clinical sign (as opposed to
symptom) of this pathology.

Diagnosis
Serum measurement of glucagon levels (a level of greater than 1000pg/ml is diagnostic)
Imaging with CT scanning of the chest/ abdomen and pelvis
Endoscopic USS may be used
Combined CT/MRI can accurately localise the lesion in 95% of cases

Treatment
In most cases, disease is metastatic at presentation and therapy with chemotherapy is undertaken,
doxorubicin and 5FU have both been used. The biological agent, sunitinib, has also been used. Where the
lesion is small, resection may be undertaken, this is usually similar to the resections performed for
adenocarcinoma.

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Somatostatinoma
Somatostatinomas are rare neuroendocrine tumors that arise from the pancreas or the gastrointestinal
tract and are characterized by excessive secretion of somatostatin hormone by tumor cells of D-cell origin.
They are frequently associated with a classic clinical pentad of diabetes mellitus, cholelithiasis, weight loss,
steatorrhea and diarrhea, and hypochlorhydria and achlorhydria.

They are rare tumours with an approximate incidence of 1 per 40 million. Over 90% are sporadic. Most are
malignant and 85% of patients will have metastatic disease at presentation.

The tumours are, however, slow growing so reasonable 5 year survival figures are seen even when
metastatic disease is present. In those resected without metastatic disease, 5 year survival figures of 100%
are reported.

Diagnosis is based on serum measurements of somatostatin. Imaging is with CT and MRI. Endoscopic USS
is also useful. As with all pancreatic malignancies, biopsy is not routinely performed unless the patient is
not going to have a definitive resection.

Chemotherapy is sometimes used as a treatment and 5FU and doxorubicin are typically the agents of
choice.

Gastrinomas
Gastrinomas are typically located near the head of the pancreas, they are tumours of borderline biological
behaviour but do progress over time. They are typically associated with Zollinger Ellison syndrome (think
ulcers refractory to PPI=check gastrin levels). They are usually treated with surgical excision.
Tamoxifen

 Synthetic partial oestrogen agonist, acts primarily by binding to the oestrogen receptor.
 Half life of 7 days, takes 4 weeks for drug to reach plasma steady state.
 Should usually be considered in patients with oestrogen receptor positive tumours (alternative
agents may be preferred in some groups).
 Although antagonistic with respects to breast tissue tamoxifen may serve as an agonist at other
sites. Therefore risk of endometrial cancer is increased, preservation of bone density and
decreased cardiovascular risks.
 Climateric side effects are common, 3% stop taking the drug because of these.
 Aromatase inhibitors are an alternative class of drugs, these work by blocking the peripheral
aromatization of androgens (post menopausal women produce oestrogens in this way). They may
treat cancers for which tamoxifen is no longer effective.

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