Nasopharyngeal B-cell lymphoma with pan-hypopituitarism and oculomotor nerve palsy: a case report and review of the literature

Abstract

Background Primary nasopharyngeal lymphoma (NPL) is a very rare tumor of Waldeyer ring (WR) lymphoid tissue. It is challenging to differentiate lymphoma infiltration of pituitary from a pituitary adenoma, meningioma infiltration, and other sellar lesions to plan a suitable treatment strategy. We presented for the first time a unique case of NPL with an unusual presentation of oculomotor nerve palsy associated with pan-pituitary involvement in a diabetic patient. Case presentation A 64-year old diabetic woman with no previous history of malignancy presented with intermittent diplopia for about the last nine months. Severe headache, left eye ptosis and hypoglycemic episodes were added to her symptoms after a while. Further complaints include generalized weakness, loss of appetite, generalized musculoskeletal pain, and 6–7 kg weight loss within six months. Her family history was unremarkable. Physical examinations of eyes indicated left eye 3rd, 4th, and 6th nerve palsy. But, she was not anisocoric, and the pupillary reflexes were normal on both eyes. No lymphadenopathy, organomegaly and other abnormalities were found. Magnetic resonance imaging (MRI) showed a heterogeneous enhancement in the seller and suprasellar regions, enlargement of the stalk, parasellar dural enhancement and thickening of the sphenoid sinus without bone erosion. Also, both cavernous sinuses were infiltrated and both internal carotid arteries were encased by the neoplastic lesion. It suggested an infiltrative neoplastic lesion which compressed the cranial nerves. Pituitary hormone levels assessment indicated a pan-hypopituitarism. Following nasopharyngeal mucosal biopsy, the immunohistochemistry (IHC) findings revealed a low-grade non-Hodgkin’s B-cell lymphoma. Systemic workup, including cerebrospinal fluid (CSF) studies, bone marrow aspiration, chest and abdominopelvic high-resolution computed tomography (HRCT) indicated no other involvement by the lymphoma. After chemotherapy courses, central adrenal insufficiency, partial central diabetes incipidious (CDI) and central hypothyroidism have been resolved. To our best knowledge, we found 17 cases of NPL with cranial nerve palsy, 1 case of NPL with pan-hypopituitarism and no NPL case with both cranial nerve palsy and pituitary dysfunction. Conclusions The incidence of cranial neuropathy in patients with diabetes should not merely be attributed to diabetic neuropathy without further evaluation.

Hypopituitarism and cranial nerve involvement mimicking Tolosa-Hunt syndrome as the initially presenting feature of diffuse large B-cell lymphoma: a case report

“…One feature of our case is the simultaneous presentation of both hemi-cranial nerve palsies and hypopituitarism with compression of the pituitary gland by a mass from sphenoidal sinus though osteolytic lesion of the sphenoidal bone. Zahedi et al reported a case of B-cell lymphoma at nasopharyngeal site in which there was presentation of both pan-hypopituitarism and oculomotor nerve palsy [ 3 ]. They also reviewed 17 cases of nasopharyngeal B-cell lymphoma, but none of the cases of nasopharyngeal B-cell lymphoma presented cranial nerve palsies nor hypopituitarism [ 3 ].…”

“…Zahedi et al reported a case of B-cell lymphoma at nasopharyngeal site in which there was presentation of both pan-hypopituitarism and oculomotor nerve palsy [ 3 ]. They also reviewed 17 cases of nasopharyngeal B-cell lymphoma, but none of the cases of nasopharyngeal B-cell lymphoma presented cranial nerve palsies nor hypopituitarism [ 3 ]. Similarly, Wajima et al reviewed 15 cases of sphenoidal lymphoma [ 5 ]; 73.3% patients initially presented with ptosis or diplopia caused by II, III, IV, or VI palsy, but not hypopituitarism.…”

“…Anatomically, since sphenoidal bone composes sella and cavernous sinus, it is a critical structure to protect pituitary gland and upper proximal cranial nerves running through the cavernous sinus [ 11 , 12 ]. Only one report has shown a case with both hypopituitarism and cranial nerve palsy by lymphoma at the paranasal site without definite bone erosion [ 3 ]. Based on the anatomical features, it was difficult to explain both symptoms being caused by one lesion from the nasopharyngeal site [ 3 ].…”

“…Therefore, to understand the patient’s clinical state, it is radiologically important to investigate how these two lesions could be simultaneously involved. As for lymphoma originating from extra pituitary gland, only one case of nasopharyngeal lesion has been reported; there was both oculomotor nerve palsy and pituitary dysfunction, but no bone erosion [ 3 ].…”

Diabetic Oculomotor Nerve Palsy Displaying Enhancement of the Oculomotor Nerve in the Orbit and Cavernous Sinus on MRI

“…Microvascular ischemia is regarded as the most common cause [2], and it is closely related to diabetes [3]. In addition, differences have been reported in the incidence rates of other causes of cranial nerve palsy in diabetic patients [4, 5]. Hence, diabetic ONP should be distinguished from other etiologies of isolated ONP.…”