A Woman With Severe Diffuse Joint Pains

AUTHORS:
John Chapin, MD; Hani Hojjati, MD; and Bryan J. Schneider, MD
Weill Cornell Medical College, New York Presbyterian Hospital, New York
 

CITATION:
Chapin J, Hojjati H, Schneider BJ. A woman with severe diffuse joint pains. Consultant. 2013;53(6):430-431.

A 55-year-old African American woman reported several months of back pain, bilateral joint stiffness, and pain in her fingers and wrists. Swelling in the ankles and shins had also developed. She was treated with high doses of oral opioids to control her joint pain. She denied any previous medical problems and reported a heavy smoking history (more than 100 pack years); she had quit 4 years earlier.

PHYSICAL EXAMINATION

Her heart rate was 107 beats per minute; respiration rate was 15 breaths per minute; and blood pressure and temperature were normal. Oxygen saturation was 100% on room air. She was thin, and results of the lung examination were unremarkable. Examination of her fingernails revealed clubbing (Figure 1). The lower extremities were tender to mild palpation over the anterior tibia bilaterally and exhibited warmth and non-pitting edema. The neurologic exam was negative for focal deficits, and muscle strength was limited only by pain.

Figure 1. Nail and finger changes consistent with digital clubbing are evident in this patient (A and B). These changes do not resolve after tumor resection and are thought to be permanent.

LABORATORY, IMAGING, AND BIOPSY RESULTS

Laboratory studies demonstrated mild anemia (hemoglobin, 11.3 g/dL; normal range, 11.7 to 15.3 g/dL) and mildly elevated alkaline phosphatase (129 IU/L; normal range, 32 to 91 IU/L). Testing for rheumatoid factor was borderline positive (20 IU/mL; normal range, 0 to 19 IU/mL), antinuclear antibody was negative, and erythrocyte sedimentation rate (ESR) was elevated at 65 mm/hr (normal range, 0 to 30 mm/hr).

A CT scan of the chest revealed a 2.5 cm nodule in the upper lobe of the left lung. Positron emission tomography (PET) confirmed a 2.2 × 2.5 cm mass in the left upper lobe with a standard uptake value (SUV) of 8.2, and an adjacent smaller mass measuring 2 × 1.8 cm with an SUV of 2.9 (Figure 2A and 2B).

Figure 2A. A PET/CT scan performed with the initial staging workup demonstrated a 2.2 × 2.5 cm left upper lobe nodule with a standard uptake value of 8.2 (arrow).

Figure 2B. No FDG avid lesions were identified on the post-surgical PET/CT scan.

The patient underwent fine-needle aspiration of the larger left upper lobe mass, and pathology results were consistent with adenocarcinoma of the lung, positive for TTF-1 and CK7, negative for CK20, synaptophysin, chromogranin, and estrogen-receptor by immunohistochemistry. There was no evidence of disease outside of the left upper lobe based on mediastinoscopy with negative biopsies of the pleura and two mediastinal lymph nodes. She underwent left upper lobe resection. Pathology revealed adenocarcinoma of the lung in several foci but confined to the left upper lobe (Figures 3A, 3B, and 3C).

Figure 3A. Gross and microscopic pathology from the lobectomy are shown here. Resected adenocarcinoma lung nodule from left upper lobe

Figure 3B. Gross and microscopic pathology from the lobectomy are shown here. Hematoxylin and eosin staining of lung tissue from wedge specimen (acinar-subtype), revealed adenocarcinoma of the lung

Figure 3C. Gross and microscopic pathology from the lobectomy are shown here. Pathology from lobectomy specimen demonstrated adenocarcinoma with mixed subtypes (50% acinar, 40% bronchioalveolar, and 10% micropapillary variant).

OUTCOME OF THIS CASE

Six weeks after the resection, the patient reported significant symptomatic improvement of her joint pain. She no longer had arthralgias, lower extremity swelling, or back pain, and she subsequently discontinued all pain medication. Her ESR decreased into the normal range (23 mm/hr) and repeat scans revealed no evidence of recurrent disease. She elected not to receive adjuvant chemotherapy.

DISCUSSION

Hypertrophic pulmonary osteoarthropathy (HPO) is a paraneoplastic syndrome that occurs in approximately 5% to 12% of patients with non–small-cell lung cancer (NSCLC).¹ Patients with this syndrome typically present with multiple symptoms that may include digital clubbing, arthralgias, arthritis, and periostitis of long bones. HPO may occur with any histologic subtype of NSCLC and has been reported with other thoracic malignancies such as mesotheliomas and small cell lung cancer. HPO has rarely been associated with other malignancies including cancers of the colon, kidney, nasopharynx, esophagus, stomach, pancreas, and breast. Melanoma and Hodgkin’s lymphoma have also been reported with this syndrome.² While HPO may be associated with advanced stage disease, it does not necessarily herald metastasis to bones and, more importantly, may be a sign of potentially curable cancer.

The pathophysiology of HPO is incompletely understood. It has been hypothesized that circulating megakaryocytes accumulate in affected areas and release activated platelets. These cells secrete vascular-derived endothelial growth factor and platelet-derived growth factor that stimulate angiogenesis and osteoblast reproduction.^3,4 Bone scintigraphy and plain films may suggest HPO but, in the setting of malignancy it is important to rule out distant metastatic disease with imaging modalities such as PET. Although pain control may be accomplished with narcotics and bisphosphonates, treatment of the underlying malignancy often leads to complete resolution of discomfort. Surgical resection, palliative chemotherapy, and epidermal growth factor receptor inhibitors have all been reported to reduce the need for pain medication.^6,7

Dr Chapin and Dr Schneider are with the division of hematology-medical oncology, department of medicine, Weill Cornell Medical College of Cornell University, New York Presbyterian Hospital, New York. Dr Hojjati is with the department of pathology, Weill Cornell Medical College, New York Presbyterian Hospital.

The authors report no external sources of funding for this report and no conflicts of interest.

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