Sarcoma: A Diagnosis of Patience

An ESUN Article by Peter J. Buecker, MD

Identification of an enlarging mass in one’s body can be quite alarming. Typically, such a discovery provokes a great deal of anxiety. Patients with sarcoma, however, usually do not feel ill and may have little or no pain, and thus do not consider the fact that this mass could represent a very deadly disease. In fact, it is often the case that these lesions present at relatively advanced stages after some symptomatology (e.g. nerve dysfunction or severe pain) has developed, or worse yet, after an unplanned excision has occurred. This problem of delayed diagnosis is compounded by the fact that the first line of contact is most often a care provider with very little experience with sarcomas, and the diagnosis may not be considered initially. By the time a given patient reaches a tertiary sarcoma specialist, the tumor has often been noticeable for several months, and may have been present for even longer.

As with many medical conditions, the diagnosis, though perhaps suggested by history and physical examination findings, requires appropriate evaluation and testing to be rendered accurately. This often involves the collusion of multiple professionals from a variety of disciplines (e.g. medical oncologists, radiation oncologists, oncologic surgeons, radiologists, and pathologists). By the time the tumor has been imaged (usually with either X-Ray, MRI or both), staging studies have been performed (i.e. chest CT, bone scan, laboratory studies) and a biopsy has been attained for definitive diagnosis, several more days or, less frequently, weeks may have passed. This can be very frustrating for everyone involved, particularly the patient and his/her support group. Feelings of anxiety and even helplessness can be overwhelming during this period of "limbo."

By the time the patient presents to the appropriate specialists, a great deal of stress has already occurred, and is magnified when the diagnosis of cancer is made. Often the patient’s wish is to have the tumor removed as soon as possible in an effort to "get the cancer out." While this sentiment is understandable, with sarcoma, surgery usually follows after an even longer period of time for the administration of preoperative adjutants such as chemotherapy and radiation.

As a surgeon specializing in the care of sarcoma patients, this process can be frustrating as well. It is difficult to tell an individual in the above situation that surgery may not occur for another 6-12 weeks. Though many variations exist between cancer centers in the approach to these diseases, typically some form of adjuvant therapy, such as radiation and/or chemotherapy, accompanies surgical care for sarcomas.

Treatment of bone sarcomas in most centers involves up to 3 months of chemotherapy prior to surgery (neoadjuvant or induction chemotherapy), with completion of the course after an appropriate period of recovery from surgery has occurred. In all, this process can take up to a year, assuming no other complications or need for further procedures occur. With regards to soft tissue sarcomas, typically a course of radiation therapy (often about 5 weeks’ worth) with or without chemotherapy preceeds surgical resection. While the decision to give radiation before, after or before and after surgery remains somewhat controversial (or may change on a case-by-case basis), it is certainly an integral part of the treatment plan for these tumors. Logistically, it adds a component of complexity for the patient, requiring daily treatments for about 5 weeks. Given the length and complexity of treatment for bone and soft tissue sarcomas, it is no wonder that patients often become disillusioned and frequently depressed during the process. It is important for patients and their families to understand that this is a very normal and natural response. Commonly, centers where sarcoma patients are treated have resources and/or personnel available to help patients and their families cope with such accompanying psychological difficulties.

Once the course of treatment has been completed, a necessary schedule of follow up begins. Usually, this means visits with some type of imaging and/or other ancillary testing (cardiac testing, laboratory exams, etc.) every three months for 2-3 years, every 6 months until 5 years after treatment, then annually. Many permutations of this certainly exist, and schedules are often varied for a particular patient, predicated on many potential factors, most frequently the presence of metastasis (disease spread). As opposed to most types of carcinoma, there is really no time when "cure" is felt to have been definitively achieved. Sarcoma is a lifelong diagnosis, and should be treated as such. Having said this, most recurrences or metastases will be discovered within the first two to five years after treatment. Once the patient has cleared the five-year mark, the risk of recurrence diminishes greatly, but still certainly exists.

It is critical that patients and caregivers alike understand that there is no "quick fix" with regards to treatment of sarcoma. The process of evaluation, diagnosis, treatment and follow up is long and arduous, and requires a significant commitment from all involved. With current treatment protocols, many more are surviving sarcomas than ever before, and research is ongoing. Prognoses continue to improve. With these advances and the relatively long lifespan of many of these patients, the importance of long-term follow up and recognition of distant treatment sequelae becomes even greater.

One of the greatest challenges facing those committed to improving care of patients with sarcoma is that of education. Most people are familiar with standards of carcinoma care (i.e. regular mammograms and self-exams for breast cancer screening and annual exams for prostate and colorectal cancer screening). In comparison, very few people have even heard of sarcoma, and there certainly are no currently known effective screening or prevention programs. There is also a conspicuous lack of knowledge about these rare cancers among health care providers, leading to not only the aforementioned delays in diagnosis, but also to unplanned resections. This occurs when a "lump" is felt to be "probably benign" or "just a lipoma" and is excised. Only on microscopic investigation is the diagnosis of sarcoma made. Unplanned excision may not only obviate the possibility of limb-saving tumor resection, but may actually endanger the life of the patient. Persistent and tireless dedication to the cause of awareness is the call for all affected by sarcoma. This includes not only providers, nurses, and cancer centers, but also patients, their friends and family members. Only through heightened awareness among health care workers and the public at large will unfortunate delays in diagnosis and the frequency of unplanned excisions diminish.