Your Diagnosis, Please
A previously healthy 12-year-old hispanic male presented to the emergency department with moderately severe left-sided back and right-sided shoulder pain, which prevented him from sleeping. During the previous 6 months, he had intermittent left upper quadrant (LUQ) abdominal pain, and in the past 2 weeks, he developed decreased activity, pallor, decreased appetite, low-grade fevers, nighttime cough, night sweats and a 20-pound weight loss. The patient denied hemoptysis, rash or rhinorrhea.
The patient lived with his parents and 2 younger siblings. They reported travelling together to Mexico 2 years earlier, but the patient had experienced no illnesses during or immediately after the trip. He denied any exposure to sick contacts, pets or animals or trauma. He had no known tuberculosis (TB) exposure. On physical examination, the patient appeared well, alert and in no acute distress. His weight was 45 kg (50th percentile), a significant drop from his premorbid weight of 54 kg. His temperature was 37.5°C, heart rate was 98 beats/min, blood pressure was 99/66 mm Hg, respiratory rate was 26 breaths/min and oxygen saturation was 100% in ambient air. He had good oral hygiene and no evidence of dental or periodontal disease. He had a <1 cm lymph node in the right posterior cervical chain, but there was no other palpable lymphadenopathy. He had diminished breath sounds in the left lower lung fields, but otherwise his lungs were clear to auscultation. Cardiac examination was normal. He had tenderness to palpation at the LUQ, left flank and left costovertebral angle. His spleen tip was palpable, but he had no hepatomegaly, and no other palpable masses. There was no vertebral tenderness. His physical examination was otherwise normal.
Laboratory results included a white blood cell count of 15,500/mm3 with 69% neutrophils, 22% lymphocytes and 8% monocytes; hemoglobin of 10.6 g/dL and platelet count of 515,000. A metabolic panel was notable for an elevated total protein of 10.5 g/dL with a low normal albumin (3.9 g/dL), normal lactate dehydrogenase and uric acid, nonreactive HIV1 and HIV2 antibodies, negative interferon-gamma release assay for Mycobacterium tuberculosis and sterile blood culture. Tuberculin skin testing was negative.
Chest radiograph revealed an ill-defined opacity of the left lower lung field and possible left lower lobe infiltrate. The patient was given a dose of intravenous ceftriaxone and admitted to the hospital for presumed pneumonia. The following day, a computed tomographic scan of the chest revealed a heterogeneous mass that extended from the left lower lobe across the diaphragm and into the LUQ of the abdomen with invasion of the paraspinous and intercostal muscles, as well as an enlarged lymph node in the left hilum. A magnetic resonance imaging of the chest and abdomen also revealed multiple nodules in the liver, suggestive of possible metastatic disease.
The fever and pain abated, but without clear evidence of a bacterial infection within 3 days, ceftriaxone treatment was discontinued.
Open exploratory surgery revealed a retroperitoneal mass in the LUQ that originated from or was invading in the left chest. The pathology from the left retroperitoneal mass revealed fibrosis and chronic inflammatory infiltrates and was interpreted to be more consistent with infection than with tumor. Gram-stained smear revealed few polymorphonuclear leukocytes and rare Gram-positive bacillary forms. TB polymerase chain reaction (PCR), stains for acid fast bacilli (AFB), fungal and bacterial cultures and cultures for acid-fast organisms were all negative.
A second, open surgical debulking of the tumor was performed 12 days later, and additional studies confirmed the diagnosis.