Abstract
Abstract
Background:
As attending physicians in acute care hospitals consult palliative care services earlier for patients with a life-threatening illness, the role of the palliative care consultant (PCC) may expand, especially when there is diagnostic uncertainty.
Method:
The expanding role of the PCC and the complexities associated with earlier referral are illustrated by describing the case of a patient with an uncertain diagnosis.
Conclusion:
The diagnostic uncertainty that accompanies earlier palliative care consultation may hamper the PCC's ability to establish goals of care and the appropriateness of hospice palliative care unit admission. Attempts at resolving this diagnostic uncertainty may lead to an expanded role for the PCC, which ideally will occur in collaboration with the primary care team.
Introduction
In this context, we present the case of a 60-year-old female with widespread bony metastases, for whom bone biopsy revealed signet ring morphology. Extensive investigations failed to identify the location of the primary cancer. Although the palliative care team was consulted exclusively for symptom management and discharge planning, further attempts to identify the origin of the cancer were felt necessary to clarify treatment options, plan goals of care, and facilitate discharge. The search for the primary cancer had an unexpected outcome.
Case Description
A 60-year-old retired nurse presented with a 3-month history of pain (thoracic spine/pelvis) and weight loss. Her bone scan revealed metastatic disease throughout the axial skeleton. Thyroid ultrasound, computerized axial tomography scanning (head, chest, abdomen and pelvis), and colonoscopy failed to reveal the origin of the malignancy. Protein electrophoresis was normal. Bone (right iliac crest) biopsy revealed metastatic adenocarcinoma suggestive of gastric origin (signet ring morphology). Immunohistochemistry (CK 20 positive/CK 7 negative) suggested pancreatico-biliary or colon as other possible primary sites. Carcinoembryonic antigen (CEA) level was elevated (229 ng/ml) and CA 125 was normal.
Diagnostic esophagogastroduodenoscopy done at 4 months (after initial presentation) demonstrated a small gastric erosion in the antrum, not worrisome for malignancy by gross appearance. Biopsy from this lesion was reported as chronic active pangastritis, helicobacter associated. The patient had previously undergone helicobacter eradication therapy.
The patient subsequently spent time in Mexico where she underwent intravenous chelation therapy and was prescribed vitamins, minerals, and laetrile. At 5 months, she continued to ambulate well with only mild pain in the thoraco-lumbar region and occasional left hip/femur pain. At this time a 6.6-cm osteolytic lesion was discovered in the left acetabulum placing her at significant risk for fracture. Orthopedic surgery recommended pelvic reconstruction and hip replacement, but given the outlined risks and benefits of the procedure the patient declined.
At 8 months, she was seen at the regional cancer center. The need to address bone-related complications along with the patient's travels led to the delay in oncologic follow-up. At this time, empiric chemotherapy for cancer of unknown primary (CUP) was offered, but given the modest benefit predicted by the oncologist, she declined.
At 1 year, she suffered a subcapital fracture of her right hip and was admitted to the orthopedic surgical service of a large acute care hospital. It was felt that reconstructive intervention was not feasible due to poor bone stalk around the proximal femur. Surprisingly, the patient remained ambulatory. The inpatient palliative care consultation service was asked to see the patient to assist with symptom management and discharge planning. Opioid management was initiated for hip pain, which exhibited a significant incident component. She declined radiation therapy.
Although bone biopsy was suggestive of a gastrointestinal source, the PCC was concerned that the pathology (adenocarcinoma) and pattern of bone metastases (mixed osteolytic/osteoblastic) were potentially consistent with a subset of more treatable malignancies (e.g., breast, ovarian, colon cancer). 2 The oncologist had previously recommended repeat mammography, but the patient declined as she reported a normal study 1 year prior. The palliative care team struggled to define goals of care and assess the appropriateness of admission to a hospice palliative care unit without knowledge of the primary given that the median prognosis varies substantially among various malignancies. The PCC initiated investigations in this regard, but subsequently it was agreed that the patient was fit enough to return home following a period further rehabilitation at her local (rural) hospital. Although no longer officially involved in the case, the PCC continued to ponder the diagnostic dilemma and prepared to present the case to colleagues at palliative care rounds. While preparing to present this case, numerous clinical factors (see Discussion below) renewed the PCC's suspicion of gastric cancer. Review of the gastric biopsy was requested from pathology by the palliative care team. Upon review, signet ring cells were found underlying the normal gastric mucosa confirming the diagnosis of gastric cancer. The surgical pathology report was amended to “poorly differentiated adenocarcinoma signet ring type.”
Seventeen months postdiagnosis the patient was admitted to the hospital from her home with a decreased level of consciousness and right-sided weakness. Computed tomography scan of the head confirmed a large acute left-sided middle cerebral artery infarction. No intracranial brain metastases were seen. Chest x-ray demonstrated lymphangitic carcinomatosis and bilateral pleural effusions. During this admission she succumbed from disease-related complications.
A family meeting was held between the gastroenterologist and the patient's husband after the patient's death to inform them of the amended pathology report. The family expressed relief in the knowledge that the source of the malignancy had been discovered.
Discussion
Early consultation
Consensus guidelines and leaders in the field recommend that palliative care be introduced early in the course of a life-threatening illness, in conjunction with other therapies that are intended to prolong life, such as chemotherapy or radiation therapy. 3 Similarly, the experience of hospital-based palliative medicine physicians suggests that their skills are more broadly applicable than just for dying patients.1,3–9
We are observing an increasing trend toward this consultative model at our large civic teaching hospital (Edmonton, Alberta) where in 2010, 70% (375/536) of palliative care consultations were for patients without a previous terminal diagnosis, indicating they were early in the trajectory of their incurable illness. 10 Although likely of benefit to patients, consultation at this early stage may be accompanied by diagnostic uncertainty, blurring treatment options and rendering prognostication more difficult. This may lead to challenges in establishing goals of care and facilitating discharge planning. In these cases, the PCC may need to move beyond their traditional role in an attempt to resolve this uncertainty.
Expanded role for PCC
In the reported case, initial acute care palliative care consultation occurred 1 year after the patient's initial presentation with metastatic disease. Given the patient's presumed diagnosis of CUP, which predicts a median survival of 5 months, 11 one may have presumed that her needs would be met by providing symptom management, psychosocial support and referral to a hospice program. As the patient was not clearly in the terminal phase of her illness the PCC felt obliged to ensure that the patient's diagnosis did not belong to one of the clinically defined CUP subgroups for which more specific therapy is available and there is a more favorable prognosis. These subgroups include breast, ovary, and colon cancer along with testicular and prostate cancer in males. 12 The patient had previously declined empiric chemotherapy for CUP and we anticipated she would have questioned the need for oncologic reassessment. Still, the PCC maintained hope of discovering a preferential CUP subgroup. In the ideal scenario we would have identified the primary as an occult breast cancer and been able to offer systemic therapy with a predicted median survival potentially measured in years. To our disappointment, physical examination of the axilla did not reveal lymphadenopathy and bone biopsy was estrogen and progesterone receptor negative making breast cancer unlikely.
The clinical factors that renewed suspicion of a gastric primary included: the presence of a gastric lesion, previous helicobacter infection, signet ring pathology, and the characteristic bone metastasis pattern. 2 We communicated our suspicion to the gastroenterologist who postulated that the patient might have diffuse gastric cancer in which dispersed cells permeate the stomach wall, demonstrating extensive infiltration without associated ulceration. 13 As these tumors tend to infiltrate the submucosa and muscularis propria, superficial biopsy may be falsely negative. 14 In actual fact, this was not the case for our patient, as the original gastric biopsy did contain malignant signet ring cells. It is known that signet ring cells and inflammatory cells (macrophages) exhibit a histopathologic similarity and may be difficult to differentiate. 15 With confirmation of the primary malignancy as gastric cancer (although metastases to the gastrum remained a small possibility) the palliative care team felt that it would be most appropriate to focus on the patient's comfort and to provide support, without offering antineoplastic therapy for the primary cancer.
Role boundaries for the PCC
Treatment innovations for patients with advanced cancer have made the course of illness in some situations more unpredictable. The PCC is sometimes left to ascertain whether viable treatment options remain. This impacts goals of care in that the patient must be fully informed of all treatment options prior to foregoing interventional treatments and pursuing strictly comfort. As treatment options for patients with advanced cancer become more varied and complex, balancing their benefits and burdens becomes an increasing challenge.17,18
Once it was confirmed that our patient did not have a CUP from a more favorable subgroup (breast, ovary, colon), we took comfort in the knowledge that the empiric CUP chemotherapy regimen she was offered (but declined) was appropriate and was accurately portrayed as only having the potential of adding a few more months of life.
The lack of clarity in physician roles became evident once the misread biopsy was discovered. It was initially unclear which of the multiple involved services needed to communicate this to the family. As the gastroenterologist had originally obtained the endoscopic stomach biopsy, it became his role to communicate this change to the family.
Relationship with the attending physician/referring service
Any evolution of the PCC's role must occur within the context of the relationship between the attending physician and the PCC. Understandably, these relationships are as diverse as the patient's they serve. Lüthi and Cantin 19 outline the spectrum of this relationship by referring to the purely consultative model (simply answering a question) and the substitution model (taking over care). The ideal is said to be collaborative model balancing these two extremes. They further further point out that palliative care teams aims are pedagogical, supportive, and even probably diplomatic in acute settings where end of life issues need to be negotiated. 20
Beyond patient variability, multiple factors exist affecting the attending physician/PCC relationship (Table 1). The acute PCC must continually devote attention to these factors. In the reported case, the orthopedic service did not begrudge the PCC's efforts, which went beyond those requested, nor did the orthopedic team immediately request to have the patient discharged from a surgical bed. We feel this cooperation was enhanced by the collegial relationship previously developed between our services and the credibility afforded the well-established palliative care team.
PCC, palliative care consultant.
Conclusion
The described case report demonstrates issues that may arise from earlier palliative care consultation, especially when diagnostic uncertainty exists. This uncertainty can affect the PCC's ability to define treatment options, establish goals of care, and plan for the future. In an attempt to resolve this uncertainty the PCC's role boundaries may blur with that of other specialties. In order for the PCC's role to continue to evolve, a collaborative model between the attending health care professional and the PCC is essential.
Footnotes
Author Disclosure Statement
No competing financial interests exist.