A case of primary unilateral adrenal Burkitt-like large cell lymphoma presenting as adrenal insufficiency.

Primary adrenal lymphoma is extraordinarily rare, in comparison with secondary adrenal involvement by non-Hodgkin lymphoma. Although higher-resolution imaging techniques have enhanced detection of adrenal masses, biopsy or excision is often needed for definitive diagnosis. Percutaneous computed tomography-guided fine needle aspiration has great diagnostic value in the workup of adrenal masses, but is limited by sampling error and artifacts. Primary adrenal lymphoma most commonly manifests with diffuse large B-cell morphology. Burkitt-like large cell lymphoma morphology has been previously reported only once, to our knowledge. We report an 80-year-old man presenting with unilateral primary adrenal lymphoma showing Burkitt-like morphology and adrenal insufficiency. Fine needle aspiration yielded a dispersed population of monomorphic, medium to large cells suggestive of lymphoma. Although dispersed cell populations cytologically favor lymphoma, metastatic poorly differentiated carcinoma and adrenal cortical carcinoma can manifest similarly. Integrated histological, immunohistochemical, and flow cytometric immunophenotyping would provide an accurate and definitive diagnosis. We review the literature and discuss important issues with regard to diagnosis.

Extra-adrenal myelolipoma in the renal hilum: a case report and review of the literature.

Myelolipoma most commonly arises in the adrenal gland. Extra-adrenal myelolipomas are rare; to our knowledge, approximately 37 previous cases have been reported. We report a myelolipoma presenting as a localized mass in perirenal adipose tissue juxtaposed to the renal hilum in a 65-year-old Caucasian man who presented with back pain, weight loss, hematuria, and flank pain. The most likely diagnostic considerations were pyelonephritis or primary renal malignancy. However, histology revealed mainly mature adipose tissue along with multiple scattered islands of hematopoietic precursor cells. Representation of all the three hematopoietic cell lineages (granulocytic, erythroid, and megakaryocytic) was present. Perirenal masses such as morphologically identified myelolipomas are rarely, if ever, considered in differential diagnosis. The purpose of this report is to elicit consideration of extra-adrenal myelolipoma when formulating a differential diagnosis for perirenal and retroperitoneal tumors. Although primary and secondary malignant retroperitoneal tumors are much more common and aggressive neoplasms, establishing the correct diagnosis has important therapeutic and prognostic implications.

Acute lymphoblastic leukemic transformation in a patient with chronic idiopathic myelofibrosis and paroxysmal nocturnal hemoglobinuria: a case report and review of the literature.

Leukemic transformation of chronic idiopathic myelofibrosis (CIMF) to acute lymphoblastic leukemia (ALL) is rare. We report a case of a patient with CIMF who developed paroxysmal nocturnal hemoglobinuria (PNH) 2 years after initial presentation. His disease eventually transformed to ALL of precursor B-cell type. In that CIMF and PNH are clonal stem cell disorders with different pathogeneses, there may be an association between them. However, leukemic transformation is a rare sequel of both disorders. Coexistence of CIMF and PNH and subsequent transformation to ALL have, to our knowledge, never been previously reported in the world literature. The simultaneous presentation of CIMF and PNH, complicated by the rare sequela of leukemic transformation, raises important issues with regard to diagnosis and treatment.