Primary Pure Signet Ring Cell Carcinoma Of The Breast – Case Report Of An Unusual Tumor With A Short Review Of Literature

摘要

Primary signet ring cell carcinoma (SRCC) of the breast are uncommon tumors with unique clinicopathologic features and aggressive nature. Histogenetically this tumor is distinct from both infiltrating ductal and lobular carcinomas. It is characterized by the presence of >20% signet ring cells (SRC). Pure SRCC breast is very rare and diagnosed when >90% of the tumor shows SRC. We report a case of primary pure SRCC breast in a 78 year old post menopausal woman with a short review of literature. Introduction Primary signet ring cell carcinoma (SRCC) of the breast was first described as a pathologically distinct entity in 1976 by Steinbrecher and Silverberg1. Recently, the 2003 World Health Organization (WHO) classification has placed this tumor as an independent entity under ‘mucinous carcinomas and other tumors with abundant mucin’2. Primary SRCC can originate from both invasive lobular and ductal carcinoma, although it usually originates from the lobular epithelium2.SRCC breast is clinically distinct and usually presents as locally advanced tumor with axillary lymph node, distant organ metastases and occasionally with an unusual metastatic pattern involving serosal surfaces of organs such as stomach, duodenum, endometrium, cervix and pelvic floor3. Histologically, SRCC is defined by the presence of at least 20% of the malignant cells appearing as signet ring form in the tumor1,2. Case Report A 78 year old post menopausal woman presented with a history of painless lump in the left breast from 6 years with a gradual increase in size since 3 months. Patient also complained of neck and back pain since 2 months. She gave history of fracture right femur 5 years back. Local examination showed skin discoloration and 2 x2 cm lump in left upper outer quadrant. There was no discharge per nipple or enlarged lymph nodes. Chest X-ray, gastrointestinal endoscopy and ultrasound abdomen were normal. CA-125 was within normal limits.Lumpectomy was done and sent for histopathological examination. Cut section of the lump showed an infiltrating grey white growth measuring 2x 1.5x 1 cms ( Fig. 1).