Metaplastic breast carcinoma with upper limb gangrene
Case presentation
A 22-year-old nulliparous Chinese woman with no significant family history of cancer presented to a private medical center with a painless 5 cm swelling in the left breast. The swelling, which was first noticed 3 months earlier, increased in size gradually. There was no associated nipple discharge or swelling elsewhere. Core biopsy of the swelling confirmed an infiltrating ductal carcinoma with triple negative receptor status for ER, PR, and HER2 receptors. Computed tomography (CT) of the thorax, abdomen, and pelvis was negative for distant metastasis. By then, 2 weeks had passed since she first presented with her complaints. Following discussion between the attending surgeons, the patient, and her family, the decision to proceed with neoadjuvant chemotherapy immediately, was made.
Two weeks into her neoadjuvant chemotherapy, she complained that the left breast mass had significantly increased in size from the initial 5 cm to 10 cm in diameter. She also developed a swelling in the left axilla that was clinically consistent with that of an axillary lymph node. Fine needle aspiration cytology of the left axillary lymph node was reported to be a metastatic carcinoma. A decision was made to proceed with left mastectomy and axillary clearance. Histopathology examination of the 14 cm by 12 cm left breast mass was conclusive for high-grade metaplastic breast carcinoma – spindle cell subtype, triple negative receptor status. All 12 excised axillary lymph nodes were positive for metastases. Six weeks following surgery, she underwent adjuvant chemotherapy. She defaulted follow-up soon after completion of chemotherapy and underwent Traditional Chinese Medication instead, despite advice regarding potential spread of the disease, and grave prognosis should that occur.
Four months later, she presented to a district hospital with complaints of pain over the left chest wall and inability to move her left upper limb. On examination, a large fungating mass with a necrotic patch was seen at her left chest wall, extending into the axilla. The tumor had infiltrated the anterior chest wall and was even seen over the posterior chest wall. The left upper limb was cold, pale, and mottled with patchy areas of necrosis. The brachial artery, radial artery, and ulnar artery were not palpable. Doppler signals were negative for all three arteries as well. Capillary refill time was significantly prolonged. There was no motor or sensory function in the left upper limb. A right breast lump was also noted during examination, measuring approximately 6 cm by 5 cm with enlarged ipsilateral axillary lymph nodes. Core biopsy of the right breast lump revealed similar histopathology and immunohistochemistry findings as the left breast lump, supporting the suspicion of a metastatic disease, rather than a separate entity.
CT of the thorax and abdomen was negative for distant metastases. CT angiogram was not performed, as the patient and family did not agree for her to undergo this invasive investigation. The patient was advised to have another round of palliative chemotherapy and debulking surgery, including a left forequarter amputation. She denied all modalities of intervention. She was given regular subcutaneous morphine and fentanyl patches for analgesia, and her wounds were dressed regularly. The left upper limb tissue gradually became waxy and soft. The flesh over the limb also began to spontaneously separate from the underlying bone, exposing parts of the humerus. The patient eventually requested amputation of the left upper limb, as it was becoming increasingly difficult for her family and nurses to take care of the truncal lesions with the rotting flesh and fragile, non-functioning limb getting in the way. The amputation at the site of the exposed left humerus was performed under intravenous sedation by an experienced orthopedic consultant. The patient succumbed 2 weeks later, slightly more than 6 months from the onset of the disease (Figures 1 and and2). The next of kin has provided written consent for publication of this case report and the accompanying images