Pediatric Subcutaneous Panniculitis-like T-cell Lymphoma of the Orbit

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare and distinct subtype of peripheral T-cell lymphoma, representing <1% of all non-Hodgkin lymphomas. SPTCL usually arises in the fourth decade of life with multifocal involvement of the limbs and trunk. Orbital disease is uncommon. We present the youngest known case of orbital SPTCL in a 3-year-old child, where the diagnosis was initially confounded by a lower eyelid mass masquerading as preseptal cellulitis. MRI revealed a poorly defined anterior orbital mass. Immunophenotyping and histological analysis of an orbital biopsy specimen confirmed SPTCL, which was managed by the pediatric oncology team with multiagent chemotherapy. This case is unique due to the young age of presentation and primary orbital involvement. Nonresolving or atypical periorbital cellulitis needs to be investigated, as malignancy can mimic such conditions.

Eyelid involvement of primary cutaneous lymphomas: A clinical perspective

“…Sometimes it may cause erythematous nodules on the face and healing of the longstanding lesions may result in focal lipoatrophy 9,47,48 . Although rare it may involve periorbital tissue beginning as erythema or a mass on the eyelid masquerading as preseptal nonresolving cellulitis which has been reported in paediatric patients 49,50 . A case presenting with unilateral progressive eyelid swelling causing exophthalmos has been reported 51 .…”

“…9,47,48 Although rare it may involve periorbital tissue beginning as erythema or a mass on the eyelid masquerading as preseptal nonresolving cellulitis which has been reported in paediatric patients. 49,50 A case presenting with unilateral progressive eyelid swelling causing exophthalmos has been reported. 51 We observed a case with diffuse periorbital atrophy (marked tissue loss) after regression of the lesions of this area (Figure 7b) associated with typical panniculitis-like lesions on other sites of the body.…”