Primary Adrenal Hodgkin Lymphoma: A Rare Disease Manifestation

Primary adrenal lymphoma (PAL) is a rare form of lymphoma with fewer than 200 cases reported.1 The most common subtypes are diffuse large B-cell lymphoma and peripheral T-cell lymphoma, which account for approximately 78 per cent and 7 per cent of all PAL, respectively.1 To our knowledge, there are only four cases of primary adrenal Hodgkin lymphoma (HL) reported in the literature before the case presented here.2 HL accounts for 11 per cent of all lymphoma within the United States, with approximately 9000 new cases each year.3 HL most commonly involves regional LNs; however, it can involve extranodal sites, particularly the spleen, liver, bone marrow, and lungs.3 Rarely does HL involve the adrenal glands, either as a primary location of tumor manifestation or as a site of local invasion.1 We present a unique case of unilateral primary adrenal nodular sclerosis subtype classical HL (NSCHL) that was diagnosed by histopathology after adrenalectomy and nephrectomy of what was initially thought to be an adrenocortical carcinoma (ACC). Early-stage HL patients receiving combinedmodality treatment, typically with ABVD followed by radiation therapy, obtain good disease control with the potential for cure.3 Although the standard treatment of HL involves consolidative radiation after two cycles of ABVD chemotherapy, our patient's combined-modality therapy consisted of surgery followed by ABVD because of the initial suspicion of ACC. Because of the complete surgical resection, radiation was not deemed necessary. The patient we present here was initially treated as an ACC patient, for which the standard is complete tumor resection.4 When pathology confirmed the tumor as primary adrenal HL stage Ia, the treatment was altered to the early-stage HL standard of care by following surgical management with ABVD chemotherapy. [...]we present the case of a patient with a unique primary adrenal NSCHL that was managed with complete surgical resection and chemotherapy.