Abstract
Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) is the rarest subtype of primary cutaneous lymphoma, accounting for approximately 2% of cutaneous lymphomas. The rarity of primary cutaneous PTCL-NOS means that there is a paucity of data regarding clinical and histopathological features and its clinical course. This malignancy is an aggressive and life-threatening hematological malignancy that often presents mimicking other less severe plaque-like skin conditions. Due to the nonspecific nature of these lesions, CD4-positive cutaneous T-cell lymphoma (CTCL) is often misdiagnosed as either mycosis fungoides or Sezary syndrome. We describe a patient who presented with a large tumoral mass in the right frontal area, with involvement of the right upper eyelid and the ocular globe, causing loss of vision greatly impacting the quality of life. Biopsy revealed primary cutaneous PTCL-NOS, treated successfully with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) plus etoposide combination chemotherapy. As elderly patients are indicated to receive attenuated doses of chemotherapy, CHOP-based regimens represent viable options.
Highlights
Peripheral T-cell lymphomas not otherwise specified (NOS) are a heterogeneous group of lymphomas that do not have well-defined criteria for being able to fit into a particular type of lymphoma [1,2,3,4]
Whendue shetopresented to our department, we performed second biopsy of the tumor and gave. When she presented to our department, we performed a second biopsy of the tumorpathology, and gave we the accurate diagnostic
Skin involvement of enteropathy-associated T-cell lymphoma and hepatosplenic T-cell lymphoma is unusual, and, in our case, they were clinically excluded. When it comes to interpreting immunohistochemistry (IHC) biomarkers staining for the diagnostic of PTCL-NOS, it is important to include as many as possible since these can only be interpreted as a cluster
Summary
Peripheral T-cell lymphomas not otherwise specified (NOS) are a heterogeneous group of lymphomas that do not have well-defined criteria for being able to fit into a particular type of lymphoma [1,2,3,4]. Cutaneous lymphomas exhibit various clinical, histological, immunophenotypic, and genetic features. They differ in prognosis and treatment from systemic lymphomas with similar histological features [5,6,7,8]. Many subtypes of cutaneous T-cell lymphomas were described, and the most common ones are mycosis fungoides and Sézary syndrome. PTCL-NOS is a diagnosis of exclusion, based on the presence of typical histopathological features of lymphoma, an aberrant T-cell immunophenotype, often with a loss of CD5 and CD7, and a clonal T-cell receptor (TCR) gene rearrangement, in the appropriate clinical context [15,16]. Primary cutaneous PTCL-NOS are clinically aggressive and heterogeneous, varying from localized to generalized plaques or nodules commonly associated with constitutional symptoms [17]. In the present manuscript we describe the successful clinical management of a PTCL-NOS of massive dimensions of the eye in a 72-year-old lady, with cardiac comorbidities
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
