A 13-year-old girl with recurrent inguinal lymphadenopathy.

PEDIATRIC ANNALS 43:4 | APRIL 2014 Editor’s note: Each month, this department features a discussion of an unusual diagnosis in areas including genetics, radiology, or dermatology. A description and images are presented, followed by the diagnosis and an explanation of how the diagnosis was determined. As always, your comments are welcome via email at pedann@Healio.com. For diagnosis, see page 137 A13-year-old girl was referred to our hospital for diagnostic evaluation of recurrent inguinal lymphadenopathy. Eleven months before, she had been admitted to another institution with non-specific right inguinal lymphadenopathy associated with bi-monthly intermittent mild fever and hypochromic microcytic anemia, elevation of C-reactive protein (CRP), and hypergammaglobulinemia at blood screen. After excluding infectious causes by blood exams, diagnostic lymph nodal excisional biopsy was performed to exclude malignant etiologies. Histological examination showed nonspecific lymphoid hyperplasia, and the girl was discharged with the diagnosis of non-specific lymphadenopathy and irondeficiency anemia, with prescription of iron supplements. During the following months, follow-up blood exams showed persistence of anemia. At admission to our institution, the physical examination was unremarkable except for the presence of a mass in the inner upper aspect of the right thigh. An accurate history revealed that the mass had reappeared soon after the biopsy; the lesion was below the biopsy area and had enlarged slowly during the course of 10 months. It did not show any inflammatory signs (calor, dolor, rubor, or loss of function); had a solid, woody consistency; and was not movable. During the hospital stay, initial diagnostic workup confirmed persistence of microcytic anemia — interpreted as anemia of chronic disease — elevation of CRP and erythrocyte sedimentation rate (ESR) and hypergammaglobulinemia (Table 1). Considering the elevated inflammatory markers, antibiotic therapy was started. Nevertheless, all of the extensive microbiological tests performed both on blood and on bone marrow were negative. Indeed, an underlying infectious disease was excluded (Table 1). Auto-inflammatory causes and autoimmune diseases responsible for recurrent lymphadenopathy were also excluded because serum auto-antibodies screening results were negative (Table 1). An inguinal ultrasound scan revealed the presence of a 4 × 3 × 4 cm oval lesion in the internal upper side of the right thigh. The edges of the mass appeared to be well-defined, and it was predominantly Case Challenge