An eight-year-old male patient was admitted to the hospital for recurrent fever for fifteen months. His personal history, past history, and family history were not special. He had no history of tuberculosis exposure.
The child had a fever of 40 ℃, accompanied by cough, chest pain, abdominal distension, vomiting, night sweat, and emaciation fifteen months ago. The patient was admitted to the local hospital nearly once or twice a month for recurrent fever, and symptoms were alleviated temporarily after antibiotic treatments. The laboratory test revealed a rise of white blood cells (WBC, as high as 18.05 × 10 ^ 9/L), eosinophils (as high as 14.5%), C-reactive protein (CRP, up to 187 mg/L), and erythrocyte sedimentation rate (ESR, up to 115 mm/h). Thoracoabdominal enhanced computer tomography (CT) revealed multiple enlargements of lymph nodes in the thorax, abdomen, and armpit and a bit of bilateral pleural effusion. Histopathologic features of inguinal lymph nodes displayed reactive hyperplasia, and bone marrow aspiration demonstrated increased eosinophils and a few cells of unknown origin. The greater omentum was discovered to be covered by massive miliary nodules by laparoscopic examination, and the histopathologic characteristics of the mesentery and greater omentum indicated massive proliferation of fibrous tissue, combined with many eosinophils and lymphocytes infiltration. The patient was diagnosed with intraperitoneal tuberculosis, and the symptoms did not improve after treatment with isoniazid and rifampicin for five months.
Physical examinations were recorded. The patient was anemia and emaciation. The scar of the bacillus Calmette Guerin vaccine existed on the right upper arm. Enlarged lymph nodes of the bilateral side of the neck, armpit, and groin were palpable, with the maximum one at the size of 1.0 centimeter (cm) × 1.0 cm. The abdomen was slightly distended, and a mass of about 5 cm × 5 cm can be touched at the umbilicus. The other examinations were normal.
The laboratory tests revealed the elevation of leukocyte (maximum: 24.5 × 10 ^ 9/L), eosinophils (maximum: 31%), platelet (maximum: 856 × 10 ^ 9/L), C-reactive protein (maximum 106 mg/L), and decrease of hemoglobin (minimum: 69 g/L). Tests for tuberculosis yielded normal results, and radionuclide images of the bones were normal. Thoracoabdominal enhanced CT revealed bilateral pulmonary inflammation, pleural effusion, and pleural thickening. Many enlarged lymph nodes were found in the mediastinum, bilateral hilar, axilla, and abdominal cavity, with partial necrosis and obvious enhancement. The intestinal wall of the abdominal cavity was thickened and swollen, and there was a little fluid in the abdominal cavity and pelvis (Fig. 1).
The patient was diagnosed with a fever with enlarged lymph nodes of unknown origin. Disseminated tuberculosis and lymphoma were considered.
The children received isoniazid (H), rifampicin (R), pyrazinamide (Z), ethambutol (E) (HRZE) for anti-tuberculosis treatment, and meropenem for antibacterial. One week later, the child's temperature gradually returned to normal, and abdominal distension and appetite ameliorated. During the following four months of receiving HRZE therapy, the patient remained afebrile for one month, with weight increase, improvement of abdominal distention and vomiting, and relief of lesions on the thoracoabdominal CT. When the regiment was adapted to HR, symptoms including fever, abdominal distension, and appetite loss aggravated. The superficial lymph nodes of the whole body were enlarged, and several masses were formed in the abdomen, with a maximum of about 3 cm × 4 cm.
Exploratory laparotomy revealed many miliary nodules attached to the abdominal wall, greater omentum, and intestinal wall. Histologic fetures of abdominal mass, stained by hematoxylin and eosin, represented that spindle-shaped cells proliferated in patches or nodules with variable fibrosis and hyaline degeneration, and mitosis was difficult to find. There was obvious infiltration of inflammatory cells, mainly eosinophils, as well as lymphocytes, plasma cells, and histiocytes in the background. Immunohistochemical staining of abdominal mass displayed that the spindle cells were negative for EMA, Des, s-100 protein, CD34, aK, CD117, DOG-1, and EBER1/2-ish. The Ki-67/mib-1 proliferation index was 20% and S were partially positive. The FISH test did not detect the ALK gene and ROSI gene translocation. After six times of fierce discussions directed by pathologists and hematologists, the patient was eventually diagnosed as an inflammatory myofibroblastic tumor. The patient was ultimately transferred to the department of hematology and oncology for chemotherapy and recovered well after regular chemotherapy.