Psychological Adaptation , Coping , and Distress in Adult-Onset Soft Tissue Sarcomas Segall

A diagnosis of soft-tissue sarcoma (STS) poses a potential threat to life and psychological wellbeing. This article reviews the evidence about psychological adaptation, coping, and distress in cancer patients in general, and STS patients in particular. While the majority of cancer and STS patients cope reasonably well with their illness and treatment, many experience transient symptoms of depression, anxiety, and posttraumatic stress, and a minority develop clinical disorders. Risk and prevalence for depressive, anxiety, and posttraumatic symptoms and disorders in cancer and STS patients are reviewed, and the contribution of neuroticism, dispositional optimism, and monitoring/blunting to psychological adaptation in cancer patients is explored. The efficacy of various problem-focused and emotion-focused coping strategies is reviewed with special attention to denial and religious/spiritual coping. Issues specific to quality of life in patients with rare cancers and STS are addressed. The article concludes with a set of generalizations and recommendations for patients, providers, and researchers. INTRODUCTION Soft tissue sarcomas (STSs) are a diverse group of rare malignant mesenchymal tumors that account for approximately 1% of all cancers in an adult population (1,2). While STSs can occur at any age, the median age of patients at time of diagnosis is forty-eight years, with 40% of STSs occurring after the age of fifty-five. STSs can emerge anywhere in the body, but they most frequently occur in the extremities, viscera, trunk, and retroperitoneum (1). The primary treatment is wide surgical resection, often supplemented by adjuvant radiotherapy (3, 4). The role of adjuvant chemotherapy is less clearly established. The lungs are the most frequent site of metastases should they occur. If metastasis is limited, pulmonary metastectomy with curative intent is the primary treatment (2,5). If metastasis is extensive, prognosis is guarded (6), and treatment is primarily palliative. There are over seventy histologic subtypes of STS, and while the above generalizations are true for the majority of subtypes, they are not necessarily true for all. For example, gastrointestinal stromal tumors (GISTs) are unresponsive to chemotherapy but respond to other biological agents, and when GISTs metastasize, the liver is the most likely site. An STS diagnosis is an alarming event that presents patients with a significant threat to life and well-being. Patients have to confront, perhaps for the first time, existential issues of personal vulnerability and mortality. Patients must find ways to master initial emotions of alarm and distress and to cope with potentially overwhelming amounts of novel, complex and confusing medical information. Some patients try to learn as much as they can about their illness and treatment, whereas others place all their faith in the hands of experts. Patients may have to learn how to cope with invasive and arduous therapies and manage interruptions in their roles as parents, workers, and community members. They may need to navigate issues concerning finance, career, and medical insurance. They may need to sort out issues related to body image, identity, dependency, and social stigma. Patients may struggle with fatigue, nausea, pain, mobility Psychological Adaptation, Coping, and Distress in Adult-Onset Soft Tissue Sarcomas Segall, DuHamel & Paul A Resource from The Liddy Shriver Sarcoma Initiative P a g e | 2 limitations, hair loss, neuropathy, and other medical complications. The introduction to STS patienthood is a baptism by fire: It may take all of one's adaptive resources to cope with this unwanted and unexpected intrusion into one's life with its attendant disruption of one's life plans. How well one adjusts to an STS diagnosis depends on a multitude of factors including: a) the complications encountered in establishing the diagnosis including diagnostic delays, misdiagnosis, and ineffective treatments based on misdiagnosis, b) the type, grade, stage, size, and site of the tumor, c) whether one is recently diagnosed or is experiencing a recurrence or metastasis, and one’s d) age at time of diagnosis, e) understanding of diagnosis f) illness burden from comorbid conditions, g) degree of functional and role impairment due to illness and treatment, h) degree of physical disfigurement, i) burden of iatrogenic symptomatology and morbidity, j) relationship with the treatment team, k) coping strategies, l) personality structure, m) premorbid susceptibility to mental illness, n) expectancies and beliefs about cancer, and o) degree of support from family and friends. The experience of sarcoma patienthood varies depending on the unique constellation of these factors that each patient encounters and endures. Imagine a best-case scenario: Patient A had a small, superficial, well-differentiated sarcoma located in an extremity. He is fortunate to have good health insurance and is diagnosed and treated at a sarcoma center. Treatment is completed after a single successful surgical resection with clear margins. This patient has no prior history (or family history) of psychosis, depression, or anxiety disorder. His personality is naturally outgoing and optimistic. He has no other medical illnesses. He understands that his prognosis is good and has supportive family and friends. He has a good relationship with his treatment team which takes the time to carefully explain the diagnosis, prognosis and treatment. His treatment turns out to be minimally disruptive to his physical, social and occupational functioning. Compare this ideal scenario with a worst-case scenario: Patient B had a large undifferentiated retroperitoneal lesion that has recurred and metastasized. He has undergone multiple extensive surgeries, radiation therapy, and multiple courses of palliative chemotherapy marked by barely acceptable levels of toxicity. This patient has a past history of recurrent major depressive disorder and has several comorbid medical conditions including diabetes and coronary artery disease. He has strong psychological tendencies to ruminate and dwell on his problems and tends towards pessimism, defeatism and withdrawal. He is being treated by a local community oncologist who is not a sarcoma specialist and who is emotionally unavailable and only minimally communicative. The patient's sarcoma and the morbidity associated with illness and treatment have forced him into early retirement, and he barely gets by on social security disability. His Medicare eligibility has not yet kicked-in and he cannot afford COBRA benefits, so he is currently without health insurance. His wife has divorced him and he lives alone. His recurrent cancer, poor long-term prognosis, insecure financial status, and depressed demeanor have made him unattractive to others as either a friend or a potential romantic partner, and he feels isolated and abandoned. It takes little imagination to predict that patients A and B are going to have vastly different psychological adjustments to their illness. Most patients will be neither as lucky as Patient A nor as unlucky as Patient B, and will experience conditions that lie somewhere between these two extremes. The remainder of this article summarizes what psychologists know about psychological distress, coping, and adaptation in cancer patients in general, and STS patients in particular. Unfortunately, most research on psychological adaptation to cancer has been done with patients with frequently diagnosed cancers, especially breast cancer. Studies specific to patients with STSs are comparatively rare and tend to focus on comparing quality of life outcomes after different treatments. Quality of life measures provide useful information about STS patients’ physical functioning, activities of daily living, and role functioning, but do not offer as much indepth information about their psychological struggles and their adaptive and maladaptive coping strategies as one might wish. Psychological Adaptation, Coping, and Distress in Adult-Onset Soft Tissue Sarcomas Segall, DuHamel & Paul A Resource from The Liddy Shriver Sarcoma Initiative P a g e | 3 The following sections combine what is known about psychological adaptation to cancer in general, supplemented by more limited information about what is known specifically about adaptation to adult-onset STS. Each section starts with what is known about cancer in general, and concludes with what is known about STS in specific. In each and every case in which comparisons between cancer patients in general and STS patients can be made, the data about psychological adaptation in STS patients does not look significantly different from the data from patients with other types of cancer. For the most part, the psychological issues that STS patients struggle with and the strategies they employ to cope with those issues are not all that different from the issues and strategies that all cancer patients struggle with. There are certain issues, however, which may be unique to patients with rare cancers in general, and STS patients in specific, which we discuss just prior to our conclusions and recommendations. RESEARCH ON PSYCHOLOGICAL DISTRESS IN CANCER PATIENTS AND SURVIVORS Depression Depressive disorders comprise a group of clinical syndromes marked by a constellation of affective, cognitive, neurovegetative, and behavioral signs and symptoms. These signs and symptoms include a depressed mood, lack of energy, lack of motivation, diminished libido, insomnia or hypersomnia, poor appetite, anhedonia, and pessimistic assessments about one's own worth, abilities and future. In severe cases, suicidal and/or delusional thinking may be present. The depressive disorders range from mild (e.g. Adjustment Disorder with Depressed Mood) to severe (e.g., Major Depression with Psychotic Features). One can have individual or multiple symptoms of depression without meeting the criteria for a formal diag