A 73-year-old man with delirium was admitted to our hospital complaining of left sternoclavicular joint pain. He had a history of diabetes mellitus, dyslipidemia, and stent placement for right renal arterial stenosis in 2011. In early August 2013, he experienced pain around the left sternoclavicular joint, and approximately the same time, he was unable to use either the television remote control or his car seatbelt. Although he visited his local physician several times, his symptoms did not improve and blood tests revealed no significant abnormalities except for an elevated CRP level. In mid-August, he developed delirium, and came to our hospital for detailed examination.
Physical examination findings on admission included: height, 160 cm; weight, 63 kg; body temperature, 36.8 C; blood pressure, 150/89 mmHg; and heart rate, 100 beats/min. Pressure pain was observed in his chest at the left and right sternoclavicular joints, sternum, and near the right fourth and fifth ribs. Examination of the head, abdomen, back, and limbs revealed no abnormalities, and no lymph node swelling was noted. Neurologically, the patient presented with slightly reduced consciousness; his Glasgow Coma Scale score was 14 (E4V4M6); however he scored 9 of a possible 30 on Hasegawa's Revised Dementia Scale. No neck stiffness was seen, and no cranial nerve, motor system, or sensory system abnormalities were observed. No symptoms of Parkinsonism, such as muscle rigidity and tremors, were noted.
Laboratory testing performed on admission revealed the following: white blood cell count, 7.4x103/muL (reference range: 4.5-7.7x103/muL); CRP, 20.04 mg/dL (reference range: 0.0-0.3 mg/dL); red blood cell count, 3.13x106/muL (reference range: 4.32-5.07x106/muL); hemoglobin, 8.3 g/dL (reference range: 13.5-15.7 g/dL); serum iron, 33 mug/dL (reference range: 100-150 mug/dL); ferritin, 1,276.6 ng/mL (reference range: 18.6-261 ng/mL); blood glucose, 254 mg/dL (reference range: 70-109 mg/dL); and hemoglobin A1c, 8.6% (reference range: 4.6-6.2%). Electrolytes, vitamins, renal function, and hepatic function were normal. Blood coagulation testing revealed fibrin degradation products, 5.5 mug/mL (reference range: 0-10 mug/mL), and D-dimer, 1.2 mug/mL (reference range: 0.0-1.0 mug/mL). Serum immunological testing revealed no specific abnormalities. Levels of tumor markers revealed that soluble interleukin-2 receptor (sIL-2R) level was 1,350 IU/mL (reference range: 124-466 IU/mL), and no other abnormalities. As indicated, blood test results showed elevated levels of CRP, ferritin, sIL-2R, fibrin degradation products, and D-dimer. Examination of cerebrospinal fluid revealed a nucleated cell count of 0/muL (reference range: 0-5/muL); protein, 83 mg/dL (reference range: 10-40 mg/dL); glucose, 102 mg/dL (reference range: >one half of blood sugar); anti-N-methyl-D-aspartate antibody was negative. Results of polymerase chain reaction testing for herpes simplex virus deoxyribonucleic acid (DNA) and cytodiagnosis were negative. Magnetic resonance imaging (MRI) of the head revealed no particular abnormalities.
Following admission, the patient often developed severe delirium at night and complained of pain in the left and right sternoclavicular joints, sternum, and right fourth and fifth ribs. MRI of the mediastinum revealed a low-intensity signal in the sternum on T1-weighted imaging (T1WI) and a high-intensity signal on T2-weighted imaging (T2WI) and fat-suppressed T2WI (Fig. 1). A low-intensity signal was observed in the right pedicle of the upper thoracic spine on T1WI, and a tumor shadow with a high-intensity signal was seen on T2WI. Gallium scintigraphy revealed abnormal uptake in the sternum (Fig. 2). Further blood test results showed an elevated CRP level; however, there were no signs of infection, such as fever. Results of blood, urine, and cerebrospinal fluid cultures were also negative. The primary tumor could not be identified by MRI or scintigraphy findings. However, a metastatic or multifocal bone tumor was suspected. After two weeks of testing to identify a primary tumor lesion, the patient developed vomiting and abdominal pain and subsequently died.
An autopsy was performed approximately 2 hours after the patient's death. Macroscopic pathological findings revealed necrosis extending from the duodenum to the ileum. No macroscopic lesions were observed in the ribs or vertebrae, and no macroscopic masses were observed in the lungs, esophagus, stomach, intestinal tract, liver, spleen, pancreas, bladder, prostate, testicles, or kidneys. Calcification was seen in the coronary arteries, but no valve vegetation was observed. No macroscopic masses were observed in the aorta from the chest to the abdomen. Spindle tumor cells were observed microscopically in the abdominal aortic intima. We could not find tumor cells in lymphatic vessels. Immunohistochemical staining of tumor cells were positive for vimentin (Fig. 3), which suggests that the tumor cells were derived from mesenchymal cells. Among other tested markers, AE1/AE3, S-100 protein, CD56, and desmin were negative; alphaSMA (smooth muscle actin) and HHF-35 (muscle-specific actin) were almost negative; and Ki-67 was positive for 16% cells. Spindle tumor cells were observed in the superior mesenteric artery (Fig. 4). Proliferation of spindle tumor cells was observed in the ribs, thoracic vertebrae, sternal medullary cavity near the sternoclavicular joint, and peritracheal lymph nodes (Fig. 5). No tumor cells were seen from the ascending aorta to the thoracic aorta, pulmonary artery, or renal artery stent. In the neuropathological examination, no tumor cells were seen in the brain. Several Lewy bodies were observed throughout the entire cerebral cortex and brain stem. However, there was not severe neuronal loss in the cerebral cortex and was not apparent gliosis. No perivascular lymphocytic infiltration and no cerebellar degeneration were seen. Based on these findings, we diagnosed abdominal aortic intimal sarcoma with multifocal bone metastasis and peritracheal lymph node metastasis.