Case report
A 5-year-old previously healthy boy presented to our dermatology
department with a 2-month history of skin tumor on his scalp associated
with cervical nodes. On physical examination, there was a 4 cm,
dome-shaped, firm, tender, erythematous tumor of the left parietal
region of the scalp (figure 1). It was associated with multiple,
bilateral, asymmetric cervical nodes. Blood cell count showed normal
values for blood cell count and platelet count. Skin biopsy of the scalp
lesion showed a diffuse infiltrate of atypical lymphoid cells in the
entire dermis (figure 2A). The neoplastic lymphoid cells appeared
medium-sized with inconspicuous nucleoli, scant cytoplasm, and a high
mitotic rate (figure 2B). A grenz zone was present below a
normal-appearing epidermis. Immunohistochemical (IHC) studies revealed
that neoplastic cells expressed terminal deoxynucleotidyl transferase
(TdT) (figure 2C), CD10, CD34, CD79a, and Bcl2, but were negative for
CD3, and CD20. Peripheral blood and bone marrow aspiration smear,
cerebrospinal fluid analysis revealed no evidence of any neoplastic
populations. Whole-body computed tomography revealed involvement of the
cervical, axillary and mesenteric lymph nodes associated with
hepatomegaly. Based on these findings a diagnosis of primary cutaneous
precursor B-cell lymphoblastic lymphoma was made. The patient was
treated with polychemotherapy GFAL B2001 protocol including 6 courses of
vincristine, oncovin, methotrexate, and prednisone. He showed partial
remission after a 14-month follow-up.