Specialist of clinical oncology and nuclear medicine, National oncology center/Aden, Yemen
Published Date: 18/11/2020
*Corresponding author: Amani Saleh Hadi Saeed, Specialist of clinical oncology and nuclear medicine, National oncology center/Aden, Yemen
Medullary carcinoma (MBC) of breast is rare variant of invasive ductal carcinoma of breast. represent < 5% of all invasive breast carcinomas. This breast cancer named because the tumor resembles the part of the brain known as the medulla. It can be difficult to diagnose and may be missed on conventional imaging as the finding may overlap with benign lesions i.e. fibroadenoma. These tumors tend to occur in younger women, with average age reported to range from 42 to 52 years although these tumors show aggressive pathological features they are often associated with a more favorable outcome. They are almost invariably negative for hormone receptors as well as Her-2/neu (Triple negative phenotype). It is also said to be particularly common in carriers of BRCA1/2 mutations. The prognosis for medullary carcinoma is better than the ordinary invasive ductal carcinoma. We report similar case in 60-year-old female with cytological, histological and immunohistochemical analysis of MBC.
Keywords: Medullary breast carcinoma, basal phenotype; immunohistochemistry, fibroadenoma
Medullary carcinoma is rare and distinct subgroup of breast carcinomas accounting for less than 5% of all invasive breast cancers . The 2012World Health Organization (WHO) up dated the classification of medullary carcinoma under an umbrella term ''carcinomas with medullary feature'', which includes atypical Medullary carcinoma and invasive carcinoma of no special type with medullary features . The diagnosis of medullary carcinoma is usually defined by histologic diagnostic criteria proposed by Ridolfi . Histologically medullary carcinoma demonstrates syncytial growth pattern of poorly differentiated tumor cells with a high mitotic rate. prominent lymphocytic infiltrates with circumscribed microscopic appearance of desmoplastic inflammatory reaction involving mainly the periphery was also diffusely present throughout the substance of the tumor-this is another characteristic feature, which may account for its clinical and biological behavior. Recent publications of breast cancer classification base on gene expression profile analyses indicate that medullary breast carcinoma can be consider part of basal -like carcinoma spectrum made up of estrogen receptor ER negative, progestogen receptor PR negative Her-2/neu negative (triple negative phenotype) . However, there are also publication in which one my find that some MBC and atypical MBC are ER, PR and or. Her-2 positive, indicating the heterogeneity of this type of breast carcinomas [5,6]. The prognosis of MBC is still under dispute. Some studies have reported that survival rates do not differ from those of IDC [3,7]. Most published study have reported lower incidence of axillary lymph node involvement in patients with medullary carcinoma (19% to 46%) than those with atypical medullary carcinomas (30% to 52%) or invasive ductal carcinomas (29% to 65%) [8,9]. Some authors have shown that patients with medullary carcinoma of the breast with positive lymph nodes have 10year overall survival lower than those with N0 nodal disease (58.8% versus 97.1%) . The 5-year survival rate for medullary carcinoma is approximately 78%. Death secondary to this disease is only 10% though. The 20year disease free survival for stage I and II patients are approximately 95% and 61% respectively .
A60-year old female presented to breast surgeon with lump in the left breast since 6months. On physical examination it had hard mass pain less and fixed to skin with palpable axillary lymph node. She had non-family history of breast carcinoma, no history of nipple discharge or trauma. The clinical and Ultrasonography diagnoses were given as fibroadenoma. Sonommamogrphy for both breasts done show: both breast having normal parenchyma echopattern, the palpable mass in left breast is hypoechoic well-defined visualized mass about 15x11x11mm in size (suspicious mass for FNA).no focal breast mass or cystic lesion detected at right breast, normal nipple a retro- areolar areas. Both axillary regions scanned there is three lymph nodes at left axillary having normal center echogenic center with oval shape, they are 24x9mm and 11x5mm in size, the third lymph node is hypoechoic rounded shape (suspicious lymph node LN) of about (6x6 mm) in size. no evidence of significant axillary LN in right side. All other routine investigation was within normal limits.
6/11/2019 FNAB from left breast mass +left axillary LN was cohesive sheet of ductal epithelial cell showing moderate atypia mixed with myoepithelial cells lie against proteinaceous back ground, the smears contain heterogenous populations of lymphocytes dispersed as single cell are numerically dominated by small round lymphocytes. patient underwent Excisional biopsy for left breast mass 23/11/2019. Specimen was received for histopathological examination and IHC status. Excisional specimen of an irregular pieces of breast tissue 3.5x2x1.5 cm, gross measurement of mass 1.8x1.5 cm) and 1cm distance from close surgical margin. Cut section showed will circumscribed mass. Histopathology analysis of the mas was suggestive of medullary carcinoma. There was no evidence of vascular or capsular invasion. Microscopic grade III (with SBR score :9out 9). Immunohistochemistry of tumor was negative for ER, PR and Her-2/neu. we recommended for modified radical mastectomy with axillary clearance26/12/2019. A mastectomy specimen measure 18x12x5 cm, compose of entire breast parenchyma, the underlying and surrounding adipose tissue with normal nipple and normal surrounding skin, the overlying skin is 17x15 cm. serial section show unremarkable breast tissue with cavity 3x2.5 cm.
No residual tumor seen grossly. Left axillary clearance: several pieces of fibro-fatty tissue, in aggregate:8x8cm contain ten (10) loos lymph node the largest is 1x1x0.5 cm was recovered from axillary tail are all fee of metastasis 0/10. Histopathology was medullary carcinoma, TNM stage (T1N0M0 stage IIA, based on American joint committee on cancer staging system). All surgical margin and base of the specimen were free of dysplastic or malignant cell infiltration. Post-operative chemotherapy with Adriamycin and cyclophosphamide and 5FU complete 6 cycles, no evidence bases for radiotherapy in this case because N0 and surgical margin free. patient put on fellow up till writing this case she has no evidence of local tumor or recurrence or any suspicious distant metastatic lesion .
According to WHO over 1.2 million women are diagnosis with breast cancer annually worldwide. Infiltrating ductal carcinoma is abroad entity which comprise of tumor that exhibit one or more characteristics of specific types of breast cancers. There is evidence of tubular, papillary, medullary or mucinous differentiation microscopically . It has a predilection for women of younger age group and in a study by Rosen  it was found to constitute 11% of all breast malignancies among aged35 and younger. In gross appearance these lesions are well-circumscribed, soft and tan -brown to grey tumors that bulge above the cut surface of the specimen. A multinodular appearance may be appreciated in some spectrum (ER. PR and HER-2negative with high proliferative MIB-1) and apoptotic activity (P53) . The prognosis of medullary carcinoma is better than invasive ductal carcinoma but like another invasive ductal carcinoma it depends on the tumor stage. Based on several studies done previously, medullary carcinoma of the breast is usually described as an irregular shaped mass with micro- lobulation and least frequently with posterior acoustic shadowing sonographically . Calcification is usually not present on mammographic imaging as our case report, so sonographic features of medullary carcinoma and other subtypes group do not differ substantially. Treatment is based on disease stage, and in early stages conservative breast surgy, followed by chemotherapy and radiotherapy strongly recommended .
Radiotherapy and chemotherapy were common adjuvant therapies for invasive breast cancers. however, it was often suggested that MBC had good prognosis and therefore may be not benefit from systemic. There was a study found chemotherapy would improve 5- and 10-year overall survival (OS). However, the P value was 0.08, which might not be solid .
Medullary breast carcinoma is an uncommon type of infiltrative breast carcinoma that usually affects women around 50years old. It usually misdiagnosed clinically and grossly with fibroadenoma hence we should keep in mind this type of carcinoma in differential diagnosis of fibroadenoma.