An elusive diagnosis

History

• P/C:39 yr female, presented with symptoms right breast

Pain Swelling Redness Edematous, thickened skin

• HOPC & Past history

No masses, no nipple discharge, no previous h/o similar complaint Non-smoker, non-diabetic, no family history of breast or any cancer 6 children, no breast feeding No previous h/o benign breast disease

Examination & Management

• Examination finding

Erythema Swelling Edematous skin right breast No masses, no nipple discharge, no lymphadenopathy

• WBC 7.7, normal haemoatology/biochemistry• Treated with intravenous antibiotics (staphylococcus and

anaerobic cover) with good clinical response• Follow-up in breast clinic

Follow-up and TBC

• Mastitis not fully settled• Persistent edematous and thickened skin in the retro-

areolar area• Referral to triple assessment clinic

Mammogram Ultrasound Image guided retro-areolar area biopsy

Clinical presentation

Ultrasound

MLO view

Repeat TBC Further follow-up Persistent pain right breast, symptoms not settling

• Clinical examination

Thickened skin in the areolar area with nipple inversion No masses, no area to be biopsied

• Haematological investigation

ESR, CRP, Immunoglobulin profile (plasma cell mastitis)

• Radiological assessment

Mammogram ultrasound

Repeat TBC

• Biopsy

Clinical punch biopsy of the edematous area with thickened skin in the areolar area (two 4mm biopies)

Histopathological diagnosis

x5 x20

punch biopsy

Follow-up

• Palpable mass at the area of the punch biopsies

• Clinical core biopsy

Histopathological diagnosis

x10 x20

Core biopsy

Inflammatory breast cancer

• Composite clinico-pathological entity characterized by diffuse edema (peau d’orange) and erythema of the breast, over the majority of the breast and often without an underlying mass

History

• First described by Sir Charles Bell (1814)• Known as Wokman’s syndrome in pregnant women• Taylor/Meltzer differentiated IBC from LABC (secondary

IBC) in 1938• Thomas Bryant in 1887 describe the pathology

Tumour invasion of the dermal lymphatic vessels

Classification

• Clinical findings onlyNo evidence of pathological plugging of the lymphatics

• Pathology onlyClinical findings not present

• Clinico-pathologicalBoth findings are present

• AJCC (TNM) T4d

Stage IIIB or IV

Epidemiology • Geographical USA : 1% new cases in females, 0.59% in males Europe: Spain 2.9% (series 1977-1993) France : France 5.4% (series 1955-1961) In our unit: 0.02% (2008, 3/149 cases)

• RaceHigher among black women

• Age 49.5 american indian 54 Black asian pacific 58 whites

• SexNo major difference

Risk factors

• No association with Menstrual history

Reproduction Family history Alcohol use

• Higher BMI poses a risk for IBC for pre and postmenopausal women

Clinical presentation

Diagnosis

• Haagensen criteria• Clinical symptoms

• Imaging

Diagnosis

• Haagensen criteria• Rapid enlargement of the breast• Generalized induration in the presence or absence of mass• Edema of the skin of the breast• Erythema involving more than 1/3 rd of the breast• Biopsy proven carcinoma (DLI is present in about 50-75% of cases although not a pre-

requisite for diagnosis)

• Clinical symptoms• Ache and heaviness before swelling and erythema• Skin changes can be very early• Erythema and edema intensify as disease progresses

• Imaging• Mammogram• Ultrasound• MRI

Differential diagnosis

• Non-puerperal mastitis• Radiation dermatitis

• Lymphoma• CCF

Differential

mastitis lymphoma

Mammogram

IBC mastitis

Tumour characteristics

• IBC is a distinct and aggressive disease entity• Tumour size: unknown in 82.5%• Nodal status positive• Grade II/III• Receptor status

ER/PR negative in 56-83% HER-2 positive higher portion than normal

E-cadherin positive p53 is a marker for survival (30-69%) inversely

Treatment

Remains a challenge

Neo-adjuvant chemotherapy Mastectomy +/- axilla Additional chemotherapy? Radiotherapy Hormonal therapy for ER positive tumours

Clinical outcome

• Median overall survival with multimodal therapy is less than four years

CPR at mastectomy indicates better DFS and OS

• Worse for black race• No difference between clinical sub-types

• Overall at 5 years ER + 48.5% (91% all breast cancer) ER - 25.3%(77% all breast cancer)

Summary

• IBC is a pathological diagnosis• Aggressive disease with variable clinical presentation• Differential is essential and imaging may be helpful• Treatment and outcome remain a challenge