Sarcoma PART – 1- Etiology, Diagnosis

(All the articles published in past are available at www.shyamhemoncclinic.com/blog/)
Question: Thank you Chiragbhai for explaining important points related to Upper Urinary Tract Tumors. Most of the points are essentially same as for bladder cancer. 1. Surgery is the mainstay of treatment. 2. Most common histology is Transitional Cell Carcinoma. Risk factors are same as bladder cancer. field cancerization leading to multiple cancers in the tract are also common. Renal pelvis is also included in this group of cancers. 3. Due to field cancerization, whole ureter is removed, along with ipsilateral kidney and bladder cuff. Same as in kidney cancers, kidney sparing approach is feasible in some cases. 4. Perioperative chemotherapy, preferably before surgery, is preferred in most patients. postoperative may be difficult in many patients due to impaired renal function, since use of cisplatin is important.
Now that we have completed Genitourinary cancers as well, what is the next topic for discussion? All common cancers have been covered so far, including Breast, Head and Neck, Lung, Colon, Rectum, Stomach, Esophagus, Pancreas, Liver, Biliary tract, Anal Canal, Leukemias (both Acute and Chronic), Lymphomas (both Hodgkin and Non Hodgkin), Myeloma, Myeloproliferative neoplasms, Myelodysplastic syndromes, Aplastic anemia, Pancytopenia, Thrombocytopenia, Eosinophilia, Bone Marrow/Stem Cell Transplant in general and with regard to most common indications, Cervix, Ovary, Uterus, Prostate, Kidney, Bladder, Testis, and few other uncommon sites within the above discussed organ sites. That is about 28 broad areas covered.
I am glad you have kept all the articles on your clinic website, in the blog, for any interested reader who wants to go back and refer.
Ans: Thank you. Yes it has been an extensive work. We still have few more areas to cover, much less common in terms of percentage, but nevertheless very important. One such area is Sarcoma. These are tumors of connective tissue. They constitute about 1 percent of all cancers, but they are important since they have distinct behavior, good cure rates are possible, and a number of these are seen in children and young adults.
Very little is known about etiololgy, risk factors. In most cases, there is none. For some like angiosarcoma, vinyl chloride is a chemical with known association. Many chemicals have been discussed and analyzed, but not with strong evidence e.g. pesticides.
¬- Radiation therapy is a known risk factor, very small but definite. Over about 10-15 years post radiotherapy, sarcoma (soft tissue or bone) may develop within radiation field. Higher the dose, bigger the risk. This is one of the reasons why large radiation fields and large doses have been replaced by smaller fields and doses for malignancies like Hodgkin lymphoma.
¬- Among infections, HHV 8 and HIV is known to be associated with Kaposi sarcoma.

• Among genetic conditions, neurofibromatosis, Li Fraumeni syndrome, and other rare syndromes are associated with various types of sarcomas.
• Paget’s disease of bone predisposes to osteosarcoma.
  A frequent observation by patients is history of Trauma before sarcoma. This has not been proven to be a risk factor, but more likely reason for their attention drawing to the site of tumor.
  Que: How do these tumors present and how are they diagnosed?
  Ans: There are about 100 subtypes of sarcomas. Two major categories are SOFT TISSUE SARCOMA and BONE SARCOMA. Soft tissue sarcomas generally present as a painless swelling, frequently very large, but can be small too. In some sites, they may be painful or cause pressure on nearby organ with resultant symptoms. Retroperitoneal sarcomas frequently present with abdominal pain or some gastrointestinal symptoms.
  Bone sarcomas more commonly present with pain and swelling. Some have constitutional symptoms. Some present with a pathological fracture i.e. fracture without injury or with a minor injury. Joint effusions also may be seen.
  Diagnosis is mainly by imaging studies and biopsy. For extremities, MRI is more commonly used. For abdomen or chest or other sites, CT scan is more commonly used. PET-CT has role only in very limited cases, and should not be used as a routine.
  Biopsy should preferably be done by a surgeon conversant with principles of treating sarcomas. Biopsy done improperly may lead to need for larger surgery or may make surgery difficult.
  June 14th 2019. Dr. Chirag A. Shah; M.D. Oncology/Hematology (USA), 079 26754001. Diplomate American Board of Oncology and Hematology. Ahmedabad. drchiragashah@gmail.com