The Challenges of Treating Rare Sarcomas: A Focus on Bone Tumors in Children

The number of people who join the ranks of cancer has not stopped increasing for years. In Spain alone, estimates are that in 2023 there will be close to 280,000 new cases, and half are concentrated in five types (colorectal, lung, breast, prostate and bladder); the rest is distributed in 19 defined locations and under the name “others” another 18,000 affected by minority tumorsas those who suffer from less than one percent of the population.

The price of this exclusivity is high: the Therapeutic options are much scarcer than for the most common tumors, due, above all, to the fact that there is less research on its origin, causes, treatments, etc. The best strategy to turn this reality around is to make them visible, to make them known. Among these rare cancers are sarcomasof which today is their International Day, a name that includes 150 varieties that are included in two big categories: soft tissue sarcomas (fat, muscle, tissue that connects different parts of the body) and bone sarcomas, which mainly affect children and adolescents.

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All are rare, with a variable prognosis – those of the fat (liposarcomas) are usually cured with surgery-, but they conform to the universal principle that the smaller they are detected, the more chances there are of surpassing them. However, the reality is more complex, and on many occasions the tumor does not manifest itself, growing secretly until its enormous volume reveals its presence.

The tumor can grow behind the intestine, infiltrate large blood vessels, and displace other organs, such as the kidneys.

That’s what they often do. sarcomas retroperitonealeswhich develop behind the intestine and can have a volume like that of a basketball, “infiltrating large blood vessels and displacing other organs, such as the kidneys”, explain the doctors of the Quenet Torrent Institute (QTI), in Barcelona, ​​focused on complex oncological surgery.

How are they discovered?

Almost half of soft tissue sarcomas (40%) start in the abdomen. “The feeling of weight gainand asymmetrical increase in abdominal girth or bleeding in stool” are warning signs for the doctor, says François Quénet, an oncological surgeon at QTI. Although they can occur at any age, They most often appear between the ages of 40 and 60.which leads some affected people to think that “they are getting fatter because of age and not because of a tumor.”

Retroperitoneal sarcoma, originating in fatty tissue (liposarcoma), which occupies the entire abdominal cavity, has displaced the left kidney upwards and rubs against the aorta artery. (Photo courtesy of QTI)

And although the alarms go off, the diagnosis is not easy and is established through biopsies performed by radiologists and pathologists specialized in sarcomas. “We need an accurate diagnosis to develop a personalized therapeutic strategysince this is the key to the success of the surgery”, emphasizes the expert.

Which is the treatment?

As long as it can be done, you have to operatebecause it’s him only curative treatmentsince therapies directed against these tumors have not yet been developed and there has not been a great evolution of other cancer treatments, such as chemotherapy”.

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This therapeutic limitation forces program to the millimeter intervention, because in many cases the life of the patient depends on it. And it is not a matter of removing the tumor only, in some that infiltrate large blood vessels you also have to replace them with polyester prosthesisa technique in which François Quénet has extensive experience, as exposed by a study published in Journal of Vascular Surgery .

What is the operation about?

To remove retroperitoneal sarcomas, the team at the Catalan center performs compartment surgerywhich consists of opening the abdomen to remove the entire tumor, wrapped in the normal healthy tissue that surrounds it, along with other organs of the compartment it occupies. It’s an operation almost blind and without directly touching the tumor due to the high risk of it spreading through the abdominal cavity. How do surgeons know where the tumor is? “For the previous imaging studies to surgery”, clears Quénet.

A team of QTI surgeons operating on a patient.

If the tumor is very aggressive, it may be necessary to remove the kidney, spleen, or part of the colon. As highlighted by the oncological surgeon Juan Jose Torrentco-founder of QTI, “the first objective is protect the patient from recurrence and the second objective is to protect him from too important an intervention so that he does not lose many organs, so that he has a Good life quality. We are looking for a balance between both objectives, which are contrary”.

What about after surgery?

After leaving the operating room, the patient remains in the ICU for one or two days and admitted to the hospital for a week. Already at his home, Torrent emphasizes that “patients who have had their kidneys removed drink water frequently to stay well hydratedespecially in the summer months, and thus be able to preserve good renal function”.

After operation, some sarcomas require adjuvant chemotherapy or radiation therapy, but the response rate is low.

Surgeons claim that the quality of life of patients it is generally very good. But don’t hide the possibility of tumor relapse, “especially in the most aggressive ones”, a risk that is reduced with a good surgical technique.

As to complementary treatmentsin some sarcomas radiotherapy or chemotherapy is administered, although “the response rate is low”. For this reason, “surgery is the main treatment.”

Risk factor’s

The doctors deny that the lifestyle or obesity increase the risk of developing such a tumor. “They are tumors in which we have not yet discovered risk factors, with the exception of some inherited genetic syndromeswhere they may be associated with any of these sarcomas”, they conclude.

The number of people who join the ranks of cancer has not stopped increasing for years. In Spain alone, estimates are that in 2023 there will be close to 280,000 new cases, and half are concentrated in five types (colorectal, lung, breast, prostate and bladder); the rest is distributed in 19 defined locations and under the name “others” another 18,000 affected by minority tumorsas those who suffer from less than one percent of the population.

2023-07-13 07:12:41
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