In November last year, Pratiksha Mane, 48, underwent surgery for Ewing's Sarcoma, a rare form of breast cancer. She was diagnosed with cancer in her right breast, which was conserved post sugery by doctors at Mumbai's Jaslok Hospital & Research Centre.
As per the American Academy of Orthopaedic Surgeons, Ewing's Sarcoma also known as PNET (primitive neuroectodermal tumour) is a rare malignant tumour that usually begins growing in a bone. It occurs primarily in children and young adults, often appearing during the teen years. This is also a reason why doctors treating Mane considered the case to be significant, given that this cancer was now seen in someone who is close to 50 years of age and that too in a soft trissue, which is rare.
Mane first approached the hospital's outpatient department last year - a month before she underwent the surgery - with a large, painless lump in the right upper quadrant of her breast. It was a large non-metastatic tumour which could be removed by surgery. A PET CT confirmed the disease to be localised to the right breast only. Following a comprehensive evaluation and consultation with a multidisciplinary team of experts, Mane proceeded with breast conservation surgery, which was performed in a single sitting and Mane was discharged within a week. She completed "6 fractions of external beam radiotherapy out of planned 25 fractions", said Dr Sharmila Agarwal, who added that the patient may require chemotherapy in the future.
In a paper titled 'Ewing’s Sarcoma of the Breast in a Young Woman...', researchers from Italy describe the case report of a 23-year-old patient with a growing breast lump, who required an accurate and challenging diagnostic estimation and which ultimately resulted in a peripheral primitive neuroectodermal tumour (pPNET). Through this case description and a brief narrative review of the literature, the researchers aimed to highlight "the rarity of Ewing's Sarcoma located in the breast". They referred to ES as being the third most common primary malignant bone tumour. Just like Mane, this patient, too, had no family history of cancer.
In a telephonic conversation with THE WEEK, surgical oncologist Dr Satish Rao spoke about the difficulty in identifying Ewing's Sarcoma. "Clinically, when I looked at the tumour, I could not make out whether it was breast cancer or Ewing's Sarcoma, even on the PET scan. Both of them appeared the same. It was only after conducting the biopsy, histopathology and immunochemistry that I was able to get a diagnosis. Given that it was a rare tumour, I approached two of my colleagues, both of who confirmed that it was indeed an Ewing's Sarcoma of the breast," Rao said.
It presented itself as a swelling in the breast, and everything else, including the symptoms experienced by the patient were exactly the same as that of a patient of breast cancer. Mane agreed, saying she felt a "hard ball kind of swollen lump in her right breast that was very painful".
She is currently with her son and his family at Pune. "Who knows what tomorrow brings, I'm not thinking about the tumour anymore. I will take each day as it comes," she told THE WEEK over the phone.
Rao said that nobody has really studied this rare tumour. "What has been widely written about is the stage 4 of Ewing's Sarcoma. But our case was unique because we diagnosed it early, that is at Stage 3, when it hadn't yet spread across the body and it was possible for us to operate, save the breast and do breast reconstruction with tumour free margins,” said Rao, for whom it was the first such case of Ewing's Sarcoma in the past 25 years of practice.