The sellar region as presenting theater for hematologic malignancies—A 17-year single-center experience

Abstract

Hematological neoplastic mass lesions of the sellar region are rare. We identified five cases of hematological malignancy with first presentation in the sellar region from our departmental database of 1,405 patients (0.36%) with sellar lesions diagnosed over the 17-year period (2005-2021). All patients were females (mean age 55.2 ± 3.4 years). One patient had multiple myeloma (MM), one patient had acute myeloid leukemia (AML), while three other patients had lymphoma (intravascular lymphoma (IVL, n = 1) or non-Hodgkin's lymphoma (NHL, n = 2). Most patients presented with ophthalmoplegia, and one patient with diabetes insipidus (DI), with short duration of symptoms (median 30 days). All patients had an elevated erythrocyte sedimentation rate and altered blood count, while patients with lymphoma had elevated lactate dehydrogenase (LDH). Sellar mass was demonstrated in three patients while the patient with IVL had an empty sella and in the AML patient posterior lobe T1W hyperintensity was lost. Two patients (IVL and NHL) presented with multiple anterior pituitary deficiencies and one patient (AML) had DI. All patients were treated with chemotherapy. Two patients responded well to treatment (one had reversed hypopituitarism), while three patients died. Differential diagnosis of sellar-parasellar pathology should include suspicion of hematological malignancy, particularly in patients with short duration of nonspecific symptoms, neurological signs (ophthalmoplegia), blood count alterations and LDH elevation, pituitary dysfunction and imaging features atypical for pituitary adenoma. Early diagnosis is crucial for timely initiation of hematological treatment aimed at inducing disease remission and partial or full recovery of pituitary function.

Pathology of sellar tumors: A contemporary diagnostic approach

“…Nevertheless, they should be considered as a primary manifestation of the disease, although being exceedingly rare. Multiple myeloma, lymphomas (both secondary and primary), as well as acute and chronic leukemia were described in literature (46). Such cases should be carefully distinguished from more frequent hypophysitis and correlated with endocrinological and hematologic findings before establishing the final diagnosis by an experienced hematopathologist (47).…”

Chronic lymphocytic leukemia/small lymphocytic lymphoma arising in the pituitary gland: illustrative case

“…This coupled with the complexity of the hematological disease typology has led to a wide variety of diagnostic and therapeutic modalities for a solid tumor of the hematological system occurring in the pituitary gland. [4][5][6] In this report, we describe a 60-year-old female who was examined for an accidental scalp injury, which revealed an abnormal mass in the sellar region. The mass was surgically removed and pathologically confirmed as non-Hodgkin's small B-cell lymphoma.…”