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My Pecoma Study

Malignant Pecoma: How To Deal With A Rare Disease?

PEC tumoris a genre of tumors that are hereditary, occurring congenitally in people with suppressed TSC1 and TSC2 genes which cause the disease tuberous sclerosis. They are formed on the soft tissue organs like stomach, lungs, intestines, reproductive organs and tracks etc.

Tumor doesnít necessarily mean cancer
While most PEComas are harmlessly benign, allowing people to live their whole life without springing into action. However, about 0.05% of all tuberous sclerosis patients show a growth of malignant pecoma at a certain age.

Even in malignancy, there is hope
Malignant pecoma can occur on any part of the body, as scientists havenít yet figured what triggers malignancy at such later years and whether the environmental factors play a role or one. The onset of sclerosis can suppress the immune system, which is perceived to be one of the factors triggering malignancy in PEC tumors. Like most cancers, it is aggressive and can only be neutralised by imputative surgery and chemotherapy with radiation to explain the margins.

Typical behaviour of a malignant become
Most pecomas present themselves as a heterogeneous mass, exhibiting different levels of differentiation and variability, ranging from soft-tissue tumors to sclerosing masses with lipid-rich PEComas.

They have been observed to grow at many locations within a body like the mesentery arteries, tracts, liver, heart, pancreas, lungs, and rectum and in rare cases, in bones.

The degree of malignancy is much varied in the observed cases, but that holds true for mesenchymal sarcomas. A malignant pecoma becomes malignant based on the size, location and the mitotic rate of the cell division.

Resection is the safest option available in modern medicines, but it also is not entirely valid as if the margins are not clear or the scans do not reveal a pocket of cancerous cells, it can recur. Almost 50% of all cancer patients who have undergone complete remission have been diagnosed with another form of cancer within five years of remission.

There is a new method that has shown promise in stopping the growth of malignant tumors without irradiation. This method uses chemical inhibitors to block the pathways in which motor signalling to and from the tumor takes place, actually cutting off its supply lines and starving it to be resected.

by: My Pecoma Study

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