Rare cancer that made girl, 3, to bleed from genitals as if she had been defiled
What you need to know:
- The cancer is characterised by rapid division of cells gone rogue
- It normally affects girls below eight years of age, especially at the age of three
- The bleeding sheds off in clumps that look like grapes
- It can compromise fertility if it progresses to a certain level
- Surgery and chemotherapy are among the treatment procedures
What is your name, dear?” I asked the little girl lying on her back on the ward bed, holding her doll tightly.
“Peppy,” she replied. “And my doll is Baby.”
She stole my heart with the bright smile on her face. Despite lying in a ward full of sick children, Peppy was not bothered. She told me her mum had gone to get her a biscuit.
I was a medical student who was excited to finally get to my fourth year and interact with patients fully. After an extremely satisfying rotation in paediatrics, I felt like I had finally found my niche. Only to be rudely interrupted by industrial action. Our lecturers went on strike for four months.
Trying to be proactive, I sought a temporary placement in the national hospital and the doctor who was willing to take me under his wing was a paediatric surgeon. I quickly learnt that administering medicine to a child was vastly different from giving the same to a person requiring surgery.
I had a lot to learn. I also learnt that the real Dr Jekylls and Mr Hydes existed right in the paediatric surgical unit! This ward had a reputation: the specialists were extremely gracious and sweet to the babies and their caregivers, but when they turned around to address the upcoming specialists in training, it was like facing the executioner! Rotating in the unit was baptism by fire back then.
I met Peppy on my first day in the ward as I waited for the ward round to begin. During the rounds, I listened to the doctors who were studying for their Master’s specialisation (residents) make presentations of the various patients in the ward to the senior specialists. The conditions were extremely different and I was definitely way in over my head.
When we got to Peppy’s bedside, my curiosity peaked again. I listened to the resident explain that Peppy had been admitted two days prior. She was almost three and her mother brought her to the emergency department because she suddenly started bleeding vaginally.
The first suspicion was that Peppy had been sexually assaulted and was hence referred to the department for evaluation under anaesthesia.
The examination was done the same night and the diagnosis quickly changed. Peppy was diagnosed to have an extremely rare childhood tumour — sarcoma botryoides.
The surgeons took a specimen to the lab and were awaiting confirmation of the diagnosis before planning definitive care.
The best I could do that day was write the name down and go back to my books as a good student should. Sarcoma botryoides is a tumour of muscles, specifically skeletal muscles. These are muscles that control our voluntary functions such as walking, lifting, smiling and chewing.
You have to intentionally command them to execute an action. This is unlike smooth muscles which are not within our control, such as muscles of the intestines which move food along the gut without your control.
In the case of sarcoma botryoides, the tumour cells aren’t coming out of existing muscle cells but are pockets of extremely immature muscle cells resembling the early development of the foetus in the womb, before they fully mature into clear muscle cells. Because of this immaturity, they rapidly divide and are generally a form of high-grade cancer.
The tumour is common in girls below eight, most especially around age three. It tends to occur in the vagina and as it gets big, has a tendency to bleed and shed off in clumps that look like grapes. The vaginal bleeding is what got everyone so worried, thinking that little Peppy may have been violated.
Peppy stayed with us in the ward for quite a while. When her biopsy results confirmed the diagnosis, she was taken to surgery by a multidisciplinary team comprising gynaecologists, paediatric surgeons and plastic surgeons.
The surgery took four hours but the little champ pulled through beautifully. It is astounding to see such a tiny, frail person covered in green sterile drapes, surrounded by a multitude of adults, all working in such tiny spaces, to dig out the threatening tumour.
Her mother sat by her bedside in the post-anaesthesia care unit, tears silently rolling down her cheeks, grateful her little one had pulled through. It had been a difficult, emotional journey for the family. Peppy went home a week later and was eventually transferred to the paediatric oncology unit for follow-up chemotherapy.
Thankfully, though aggressive, many children with sarcoma botryoides do well when diagnosed and treated early.
However, when recurrence happens, or in extensive tumours, the prognosis isn’t too good. There may be a need for radiation therapy, which compromises fertility for these young ones, a complication they may have to struggle with when they reach reproductive age.
It has been almost two decades. Peppy must be 21 now, hopefully living a full life and not having to worry about her future and her desire to have babies. May she truly get to hold a real “Baby” in her arms when she is ready!
Dr Bosire is an obstetrician/ gynaecologist