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An elusive diagnosis

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

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An elusive diagnosis

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  1. An elusive diagnosis

  2. 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

  3. 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

  4. 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

  5. Clinical presentation

  6. Ultrasound

  7. MLO view

  8. 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

  9. Repeat TBC • Biopsy Clinical punch biopsy of the edematous area with thickened skin in the areolar area (two 4mm biopies)

  10. Histopathological diagnosis punch biopsy x20 x5

  11. Follow-up • Palpable mass at the area of the punch biopsies • Clinical core biopsy

  12. Histopathological diagnosis Core biopsy x20 x10

  13. 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

  14. 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

  15. 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

  16. 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) • Race Higher among black women • Age • 49.5 americanindian • 54 Black asian pacific • 58 whites • Sex No major difference

  17. Risk factors • No association with • Menstrual history • Reproduction • Family history • Alcohol use • Higher BMI poses a risk for IBC for pre and postmenopausal women

  18. Clinical presentation

  19. Diagnosis • Haagensen criteria • Clinical symptoms • Imaging

  20. 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

  21. Differential diagnosis • Non-puerperal mastitis • Radiation dermatitis • Lymphoma • CCF

  22. Differential lymphoma mastitis

  23. Mammogram mastitis IBC

  24. 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

  25. Treatment Remains a challenge • Neo-adjuvant chemotherapy • Mastectomy +/- axilla • Additional chemotherapy? • Radiotherapy • Hormonal therapy for ER positive tumours

  26. 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)

  27. 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

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