A Gastrointestinal Manifestation of Primary Cutaneous Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma: A Case Report

Abstract

Cutaneous T-cell lymphomas (CTCL) are the most frequently encountered form of primary cutaneous lymphoma (PCL). Although this group of diseases primarily affects the skin, extracutaneous manifestations are reported. Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma (pcAECTCL) is a rare form of CTCL that usually presents with an aggressive course and poor prognosis. Given the scarcity of cases, the disease is poorly understood. There have been reports of cases of extracutaneous manifestations of the disease, with more aggressive courses associated with an increased propensity for systemic spread. The most common extracutaneous manifestations of pcAECTCL include those affecting the lungs, central nervous system, testis, and oral mucosa, which can cause progressive dysphagia. We describe a patient with a chronic relapsing/remitting rash diagnosed as pcAECTCL. A positron emission tomography (PET) scan revealed diffuse uptake within the gastric region. Initial esophagogastroduodenoscopy (EGD) showed multiple ulcerations and erosions in the stomach with concern for extracutaneous spread of the patient's pcAECTCL. However, no definitive histopathological or flow immunophenotypic evidence of metastasis was found on the initial or subsequent repeat EGD.

Keywords: aggressive epidermotropic cytotoxic t cell lymphoma; cutaneous t cell lymphoma; duodenitis; gastric erosion; gastric ulcer; gastritis; t cell lymphoma.

Figure 1. Initial PET/CT

(a) CT scan axial view at the level of the abdominal viscera showing increased uptake in the gastric region, (b) F18 FDG PET fused axial imaging showing increased diffuse appearing uptake throughout the gastrum with a max SUV of 7.2. Given this increased uptake in the setting of the patient’s abdominal pain and active malignancy, there was a concern for this being representative of an extracutaneous manifestation of his pcAECTCL in his GI tract. PET/CT: positron emission tomography/computed tomography; F18 FDG PET: fludeoxyglucose-F18 positron emission tomography; SUV: standardized uptake value; pcAECTCL: primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma

Figure 2. Initial EGD with biopsy

(a-b) Multiple ulcerations and erosions are seen in the gastric body, (c-d) Multiple ulcerations and erosions are also shown in the fundus. Ulcerations and erosions are denoted by white arrows indicating disruptions in the normal pink-appearing gastric mucosa instead of showing red, white, or dark/black lesions with an erythematous rim. Biopsies of these lesions were taken with cold forceps; subsequent histology was negative for lymphoproliferative disorder. EGD: esophagogastroduodenoscopy

Figure 3. Histopathology of the gastric biopsy

(a) Gastric biopsy with Hematoxylin & Eosin (H&E) staining, obtained from the initial esophagogastroduodenoscopy (EGD). Arrows highlight viable atypical lymphocytes, which are concerning for an underlying lymphoproliferative disorder, but histopathology was negative for definitive signs to confirm the diagnosis. (b) Gastric biopsy with CD3 immunohistochemistry (IHC) staining, obtained from repeat EGD. The IHC highlights numerous T-cells present in the ulcerated tissue.

Figure 4. Repeat EGD with biopsy

(a-b) Mild-moderate gastritis, characterized by congestion and erythema denoted by white arrows, in the gastric body with ulcers largely resolved from prior and otherwise showing healthy light pink gastric mucosa, (c-d) Mild duodenitis represented by erythematous mucosa, again denoted by white arrows, without active bleeding or stigmata of bleeding found in the second portion of the duodenum. Multiple biopsies were taken with cold forceps for histology and flow cytometry from the stomach and second portion of the duodenum. No evidence of a lymphoproliferative disorder was found on flow cytometry or histopathology and Helicobacter pylori testing was negative. EGD: esophagogastroduodenoscopy