Askin Tumor is a primitive peripheral neuro ectodermal tumor ( PNET ), and is a type of malignant tumor that develops from bone and muscle tissue in the chest. Although Askin Tumors occur in muscle tissue and breastbone, Askin's Tumors initially arise from primitive nerve tissue. In addition to the neural network, Askin tumors can also appear on other tissues such as the waist or hip. Most Askin tumors occur in children and young adults, especially among the white race (Caucasian). Like Ewing's sarcoma which is also derived from primitive nerve tissue, Askin's tumor is a rare disease. And to diagnose its existence, it needs to be done a complex examination and involving several specialist doctors.
Symptoms of Askin Tumor
Symptoms that generally appear in patients with Askin tumors are:
- Cough for long periods.
- Chest pain, especially in the right chest.
- Weight loss.
- Hard to breathe.
- The emergence of Horner's syndrome, in which the patient has diminished the pupil, relaxes the upper eyelid followed by the removal of the lower eyelid, and expels less sweat or no sweat at all on the face.
- The emergence of regional lymphadenopathy.
- The presence of fluid in the lungs ( pleural effusion ).
- Erosion on the ribs.
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Causes of Askin Tumors
Like Ewing's sarcoma, the cause of Askin's tumor has not been accurately known. It is thought that the cause of DNA mutations in certain tissues, especially bone tissue. Although most people with Askin tumors are children, adolescents, and young adults, it is possible to occur also in infants, middle-aged, and elderly. Askin Tumors are thought to be more common in men than in women.
Diagnosis of Askin Tumor
Askin Tumor is a rare disease so that the method of diagnosis to determine its existence is quite complex. Some checks that can be done to diagnose Askin tumor are:
- CT scan. This scan can be useful for seeing lumps or tumors in the body In Askin tumors, lumps are present in the lower ribs. CT scans will present a more accurate picture if aided with contrast materials such as iodine or other diagnostic methods such as MRI.
- X-rays Examination of radiographs with X-rays can help the CT scan to see if there is an Askin tumor on the ribs or not.
- Isotope bone scan . The bone scan serves to determine whether the Askin tumor has spread to other bones or not.
- MRI. This scan can help scan the CT scan to show a tumor clump in the rib tissue.
- Bone and lung biopsy. A biopsy is performed to take a sample of bone and lung tissue, to be examined microscopically.
Askin Tumor Treatment
The purpose of treatment of Askin tumor is to destroy the tumor and prevent metastasis. In general, treatment given to Askin tumors includes:
- Chemotherapy. Chemotherapy given to patients with Askin tumors is done in several cycles, in the hope that tumor cells can be completely lost. The type of chemotherapy given may be either adjuvant chemotherapy (after surgical removal of the tumor), as well as neoadjuvant (before surgical removal of the tumor). Askin's tumor, as well as Ewing's sarcoma, has a high probability of recurrence (recurrence). Therefore, chemotherapy should be given systemically, although the diagnosis indicates that the tumor is present only in certain organs. The most dangerous facet of this chemotherapy is bone marrow damage. To overcome this, bone marrow transplant can be done from self-sufferers themselves, although the results are still in doubt. To treat Askin tumors through chemotherapy, the anticancer drugs given are generally a combination of several drugs. The combination of drugs given can be composed of 4-5 anticancer drugs. Some types of drugs that can be given and combined at the time of chemotherapy are:
- Actinomycin D.
- Resection of tumor tissue. Surgical removal of tumor tissue is performed to remove primary Askin tumors, especially on the bone. In patients with Askin tumors that have spread, can be given chemotherapy first before surgical removal of tumors performed. The goal is to shrink the size of the tumor, so removal of the tumor tissue can be easier and the results more effective. Through surgery, Askin tumor sufferers can experience a good recovery and life expectancy in 5 years post-treatment is quite high. However, please note that although surgical resection of tumors performed well, there is still the possibility of tumors reappear. In addition, resection surgery is rather difficult to treat a tumor that has spread (metastasis).
- Radiotherapy Radiotherapy may be given to patients with Askin tumors, especially before surgical removal of tumors in which tumors can not be treated with chemotherapy. Radiotherapy prareseksi (before surgery) aims to shrink the tumor so that the organ function to be resected can be kept as optimal as possible. In addition, radiotherapy may also be given to patients who have undergone surgical resection with the aim of damaging the non-elevated tumor tissue, and may also be given to patients who are most likely to have tumor recurrence.