An 11-Year-Old Girl With Facial Swelling and Leg Pain

Jeffrey A. Toretsky, MD; Stefan Zöllner, MD

Disclosures

October 08, 2019

Physical Examination and Work-up

Upon admission, the patient appears well nourished (between the 50th and 75th percentile for age) and has a mild fever (temperature, 100.8°F [38.2°C]), a heart rate of 120 beats/min, and blood pressure of 104/63 mm Hg. Intraoral examination reveals a hard, fixed, expansive mass approximately 3.5 cm in diameter, with slight evidence of dental caries on the right side of the mandible. An example similar to the patient in this case is shown in Figure 1.

Figure 1.

The overlying mucosa is centrally erythematous, without ulceration. The patient has enlarged tonsils with no exudates. Further examination reveals slight numbness of the skin over the swelling. The right eardrum is injected and bulging. The left eardrum is unremarkable. The cranial nerves are otherwise without deficits.

The pupils are symmetric and normally reactive, and the conjunctivae are pale. No cervical, axillary, or inguinal lymph nodes are palpated. Mild systolic murmur is noted on cardiac auscultation, with slight basal pulmonary crackles. Upper and lower extremities are bilaterally normal, apart from a slightly pressure-sensitive hematoma in resorption on the left tibia.

Blood examination shows the following:

  • White blood cell count: 9630 cells/µL

  • Hemoglobin level: 8.2 g/dL

  • Hematocrit: 35%

  • Mean corpuscular volume: 75 fL

  • Mean cell hemoglobin level: 29 pg

  • Platelet count: 360,000 cells/µL

  • C-reactive protein level: 5.4 mg/L

  • Erythrocyte sedimentation rate: 25 mm/h

  • Alanine aminotransferase level: 265 U/L

  • Aspartate aminotransferase level: 253 U/L

  • Alkaline phosphatase level: 349 U/L

  • Lactate dehydrogenase level: 1095 U/L

  • Uric acid level: 3.2 mg/dL

Maxillofacial CT with contrast reveals an enhancing lytic mass measuring 3.6 cm × 2.7 cm × 4.3 cm in the right mandible, with multiple surrounding lytic areas.

The patient undergoes an incisional biopsy, and the material is sent to the pathologist for histopathologic analysis. Microscopically, the specimen shows solid sheets of small round cells, exhibiting scant cytoplasm and round and oval hyperchromatic nuclei. Neither mitotic figures nor necrotic areas are seen. Intracytoplasmic glycogen is demonstrated on periodic acid-Schiff (PAS) staining.

Immunohistochemistry results are as follows:

  • Cytokeratin: negative

  • Desmin: negative

  • S-100: positive

  • Vimentin: positive

  • Leukocyte common antigen: negative

  • CD99: positive

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