Endocrine Abstracts

Introduction

Hematological neoplastic mass lesions of the sellar region are rare.

Aim of the study

To analyze a case series of patients with hematological malignancies affecting sellar region.

Patients and methods

A retrospective study of 1166 patients with sellar lesions diagnosed at Department of Neuroendocrinology over the 16-year period (2005-2020).The demographics, clinical presentation, laboratory features, radiological findings, histological diagnosis, course of treatment and outcomes are described.

Results

We identified five cases (0.4%) of sellar region lesions attributed to a hematological malignancy (all females, mean age 55.2±3.4years). The variety of hematological pathologies included: one patient with multiple myeloma (MM), one patient had acute myeloid leukemia (AML), while three other patients had lymphoma (intravascular large B cell lymphoma (IVL, n=1) or high-grade B cell non Hodgkin lymphoma (NHL, n=2/in one patient in leukemic phase). Three patients (except IVL and AML) presented with symptoms and signs suggestive for cavernous sinus infiltration (cranial nerve palsies and diplopia), one patient with NHL and large mass presented with visual field defects and one patient (AML) presented with diabetes insipidus (DI). Typically the duration of neurological and endocrine symptoms and signs was short. All patients were in poor general condition, with malaise, half of them with pronounced sweating and vomiting. All patients had elevated sedimentation rate and altered blood count (anemia, thrombocytopenia (n=4), thrombocytosis (n=1) and rapid progressive leukocytosis (n=1), while patients with lymphoma had elevated lactate dehydrogenase (LDH). On magnetic resonance, sellar mass was demonstrated in three patients while patient with IVL had empty sella. Patient with AML had loss of posterior lobe T1W hyperintensity. Two patients (IVL and NHL) presented with multiple anterior pituitary deficiencies and one patient (AML) had isolated DI. Hyperprolactinemia due to pituitary stalk compression occurred in 3 out of 5 patients (excluding IVL and AML). Two patients were operated by transsphenoidal approach (NHL and MM). All patients were treated with chemotherapy. Three patients had lethal outcome during treatment. Patient with IVL achieved long term remission with partial reversal of hypopituitarism. One patient with NHL is now treated with immunochemotherapy.

Conclusion

Suspicion of hematological malignancy in sellar region should be raised 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 to induce disease remission and partial or full recovery of pituitary function.