Primary Cutaneous T-Cell Lymphoma Involving the Cheek: An Infant Case with a Unique Clinicopathologic Feature

Abstract

We report a clinicopathologic feature of primary cutaneous T-cell lymphoma (CTCL) in a five-year-old boy with increasing swelling of his cheek since two years of age. Histologically, an infiltrate of atypical lymphoid cells with mature T-cell phenotype and clonality was prominent from the dermis to the subcutaneous tissue of the cheek. Although little effect was seen with aggressive multidrug-combined chemotherapy, therapy with interferon-alpha and steroids achieved a prolonged remission. This patient may provide important clues to understanding the clinicopathologic feature of rare primary CTCL in young children.

Aggressive subcutaneous panniculitis-like T-cell lymphoma with hemophagocytosis in two children (subcutaneous panniculitis-like T-cell lymphoma)

“…This underscores the importance of continued follow-up of patients given the diagnosis of CHP and the need to perform repeated biopsies if warranted. 14 15/F CHOP, cyclosporine In remission at 9 mo 38 15 3/M Multidrug chemotherapy # Alive at 8 y 49 16 3/M Multidrug chemotherapy** Alive at 2 y 49 CHOP, Cyclophosphamide, doxorubicin, vincristine, and prednisolone; DIC, disseminated intravascular coagulation; F, female; HPS, hemophagocytic syndrome; M, male. *Cyclophosphamide, Adriamycin, vincristine, prednisolone, and etoposide (CHOP-E).…”

“…It typically presents with subcutaneous nodules and plaques. 2,5,[12][13][14][15][16][17] Systemic symptoms are common, particularly fever and malaise. Patients may initially present with CHP, a form of panniculitis characterized histologically by lobular panniculitis with proliferation of benign-appearing cytophagic histiocytes.…”

Paniculitis como manifestación de síndrome febril prolongado en pediatría: Caso clínico

“…In the immunohistochemistry, two subtypes are described: the alpha/beta with an indolent course and favorable prognosis and the gamma/delta, which has an aggressive course and a greater association with hematopagocytic syndrome. The estimated survival of the first is 80% and the second 10% to 5 years 21 . We reviewed 9 pediatric cases described in the literature [17][18][19][20][21][22][23] , and we found some similar features with the present clinical case, as the presence of erythematous-violaceous non-ulcerated few sensitive nodules in the anterior abdominal wall, dorsum and extremities, nodules of insidious installation and evolution in outbreaks.…”

“…The estimated survival of the first is 80% and the second 10% to 5 years 21 . We reviewed 9 pediatric cases described in the literature [17][18][19][20][21][22][23] , and we found some similar features with the present clinical case, as the presence of erythematous-violaceous non-ulcerated few sensitive nodules in the anterior abdominal wall, dorsum and extremities, nodules of insidious installation and evolution in outbreaks. In 6 of the 9 cases there was concomitance with PFS and 7 of them, kept their weight 17,18,22,23 .…”

“…Table 1 shows the classification of panniculitis ac- nocturnal diaphoresis and weight loss) and possible hematological alterations 20,21 . Histologically, atypical lymphocytes are commonly seen forming a ring-like around necrotic adipocytes as a loop 13,[17][18][19][20][21] . In the immunohistochemistry, two subtypes are described: the alpha/beta with an indolent course and favorable prognosis and the gamma/delta, which has an aggressive course and a greater association with hematopagocytic syndrome.…”