Paget disease of Breast

Q) True regarding Paget's disease of the breast
A. Seen in 5-10% Carcinoma  breast
B. 50-60% associated with underlying mass
C. Treated by MRM always

d) Radiotherapy is the treatment of choice

Ans b 50% have underlying mass 

Paget disease accounts for 1% or less of breast malignancies. It is characterized clinically by nipple erythema and irritation with
associated pruritus and may progress to crusting and ulceration. ( Sabiston page 860)

Paget disease is a condition of the nipple that is commonly associated with an underlying breast cancer  More than 95% of patients with Paget disease have an underlying breast carcinoma. Paget disease may be accompanied by a palpable mass in slightly more than 50% of Epidermal layer of skin is involved. Clinically, dermatitis occurs that may appear eczematoid and moist or dry and psoriatic.

Treatment of Paget disease

(i) mastectomy with axillary staging 

(ii) wide local excision of the nipple and areola to achieve clear margins, axillary staging, and radiation therapy. 

MEN 2A

Q)  MEN 2A also known as
A. Sipple syndrome
B. Wermer syndrome

C) Werner syndrome

Ans a

Sipple and Steiner described the association of thyroid cancer with pheochromocytoma and hyperparathyroidism, respectively

Bailey says MTC combined with phaeochromocytoma alone is called Sipple’s syndrome (page 856)

MEN2A is characterized by MTC,  pheochromocytoma (50%) and hyperparathyroidism (25%).

Associated with mutations in codon 634 in the RET proto-oncogene.

Wermer-  MEN 1 is characterised by the triad of tumours in the anterior pituitary gland, mostly presenting as prolactinomas
or non-functioning tumours, hyperplasia of the parathyroids causing primary hyperparathyroidism (pHPT) and pancreaticoduodenal endocrine tumours (PETs)

Patients with MEN 2B do not develop pHPT

Axilla management in CA breast

Q) 47 year old premenopausal lady with a 3X 3cm left breast lump with IDC grade III, TNBC.

On examination, there is a  single subcentimetric mobile soft mobile ipsilateral  axillary LN palpable.

Usg nodes no loss of hilum.  Management of axilla? a) SlND

b) ALND

c) Radiotherapy only

d) No treatment

Ans a

Selective lymph node dissection

ACOSOG Z0011 trial 0  (stages I and II) in patients who undergo breast conservation therapy, axillary lymph node dissection does not improve locoregional controlor survival.

This trial has demonstrated the safety of limiting axillary surgery to the SLNB without performing formal axillary dissection for sentinel node positivity.

This  avoids of the  morbidity of the axillary dissection.

If nodes positive the patient should receive adjuvant chemotherapy and radiation therapy.