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 only No evidence of pathological plugging of the lymphatics Pathology only Clinical findings not present Clinico-pathological Both 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 )  France :France 5.4% (series )  In our unit: 0.02% (2008, 3/149 cases) Race Higher among black women Age  49.5 american indian  54 Black asian pacific  58 whites Sex No 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