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Guillain-Barré Syndrome
Definition (1)
o Post-infectious, immune-mediated disease targeting peripheral nerves
o A.K.A. acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
Aetiology (1,2)
o Respiratory and gastrointestinal infections
Campylobacter jejuni
Cytomegalovirus (CMV) most common and severe
Epstein-Barr virus (EBV)
Mycoplasma pneumoniae
varicella-zoster virus
human immunodeficiency virus (HIV)
Clinical features (1,2,3)
o Presents 1-3 weeks following a relatively benign respiratory or gastrointestinal
illness
o Signs & symptoms:
Low back pain is a frequent early feature
Classically presents with symmetrical ascending weakness and/or distal
numbness (predominantly motor) accompanied by absent or depressed
deep tendon reflexes
Weakness and sensory loss progresses proximally, over several days to 6
weeks
Cranial nerve involvement is observed in 45-75%, most common in cranial
nerve VII (mimicking Bell’s palsy)
In 20%, weakness in respiratory muscles occurs, sometimes progressing to
complete paralysis
Pain is present in 89% of patients and dysaesthetic symptoms (burning,
tingling, or shock-like sensations) are observed in approximately 50% of
patients
Investigations to differentiate from other conditions (1,2)
established on clinical grounds
confirmed by nerve conduction studies
increased latency
decreased conduction velocity
normal amplitude
CSF protein is often raised to 1–3 g/L
Ddx:
CIDP: duration of symptoms greater than 8 weeks
Acute myelopathy (e.g. from compression, transverse myelitis,
vascular injury): MRI of spine, myelography
Myopathy: Asymmetrical, decreased amplitude on nerve conduction
studies with normal latency and conduction velocity
Botulism: nausea and vomiting, dry mouth and throat, followed by
CN paralysis
Metabolic neuropathies
o DM: predominantly sensory neuropathy
Yau Jun Wern (Year 3)
References:
1. Kumar and Clark’s Clinical Medicine (8th edition): Immune-mediated neuropathies, page
1145
2. https://emedicine.medscape.com/article/315632-overview
3. https://www-uptodate-com.ezproxy.lib.monash.edu.au/contents/guillain-barre-syndrome-
in-adults-clinical-features-and-
diagnosis?search=guillain%20barre&source=search_result&selectedTitle=1~150&usage_typ
e=default&display_rank=1
Morphology:
Tumour cells produce abundant quantities of extracellular mucin,
which dissects into the surrounding stroma
Present as well-circumscribe masses and can be mistaken for
fibroadenomas
Tumous are usually soft and gelatinous
Most are ER+/PR+/HER2-
o Tubular carcinoma
Rarely present as palpable masses
Usually detected as irregular mammographic densities
Morphology :
Well-formed tubules with low-grade nuclei
Lymph node metastases rare, prognosis excellent
Virtually all are ER+/PR+/HER2-
o Common features of invasive cancers
Tend to become adherent and fixed to pectoral muscles or deep fascia of
chest wall and the overlying skin, causing retraction or dimpling of skin or
nipple
Involvement of lymphatics may result in localised lymphedema, causing skin
thickening around exaggerated hair follicles, giving peau d’orange
appearance
o Treatment:
Excision (Wide local excision, hook-needle localisation, mastectomy)
Removal of axilla lymph node (sentinel node biopsy, axillary dissection)
Radiotherapy
Adjuvant therapy (cytotoxic, hormonal, Herceptin)
o Prognostic factors
Predicts disease recurrence and death
Patient-related factors: age, menopausal status, comorbidities
Tumour-related factors: LN staging, tumour size, grade, histological type,
lymphvascular invasion
o Predictive factors
Predicts benefit from adjuvant therapies
ER/PR/HER2 both prognostic and predictive
Stromal malignancies
Phyllodes tumour
o Morphology:
Biphasic epithelial and mesenchymal neoplasm of breast characterised by
variably cellular stroma and benign double-layered epithelial cells which
form ‘leaf-like’ structures
Stromal component can show heterologous differentiation, resembling
fibroblasts and myofibroblasts
o Most follow benign course, <10% locally invasive
Metaplastic carcinoma
o A group of neoplasms characterised by differentiation of neoplastic epithelium into
squamous cells and/or mesenchymal-looking elements
Yau Jun Wern (Year 3)
o
Includes squamous cell carcinoma, spindle cell carcinoma, low-grade
adenosquamous carcinoma, metaplastic carcinoma with mesenchymal component
o Lymph node metastasis significantly less than invasive carcinoma
o Distant metastasis can be found without lymph node metastasis
o May have lower response to adjuvant chemotherapy and worse prognosis
Sarcoma
o Rare
o Angiosarcoma is most common primary sarcoma
Occurs in 3 scenarios:
Sporadic/primary
After local radiation treatment
Chronic lymphedema following mastectomy Stewart-Treves
syndrome
Clinical presentation: ill-defined, haemorrhagic lesion
Morphology:
Well-differentiated: resembles vessels with bland cells
Poorly-differentiated: sheets of malignant cells with no vascular
differentiation
Diagnosis: Either discernable vascular channels or immunostains
demonstration vascular differentiation
Treatment: Wide surgical excision (with deep resection margin)
Metastasise to lungs, skin, bone and liver. Axillary nodes involvement rare
Lymphoma
Metastasis to breast
References:
1. Lecture notes
2. Robbins Basic Pathology (9th ed): Female Genital System and Breast, page 710