Primary hypothalamic lymphoma with clinical findings mimicking pituitary apoplexy: a case report.

BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a rare but well-known extra-nodal lymphoma, which usually presents with non-Hodgkin B-cell lymphomas. PCNSL is generally located around the ventricle and is often detected as multiple lesions. It is rarely seen in the area of the hypothalamus. CASE PRESENTATION: We report the case of a 48-year-old Caucasian woman with progressive short-term memory deterioration, headache, mental confusion, diabetes insipidus (DI) and hypopituitarism. Early findings were suggestive of a pituitary apoplexy. The results of tests performed during the initial admission at the tertiary health center revealed hypernatremia, hypopituitarism and DI. Intravenous hydrocortisone treatment was initiated for the secondary adrenal insufficiency, and 75 mcg/day of levothyroxine was started for the secondary hypothyroidism on the fourth day following hydrocortisone treatment. A daily dose of 120 mg desmopressin melt tablet was started twice a day for polyuria/polydipsia after the patient's volume status was balanced. A brain magnetic resonance imaging scan revealed a mass lesion in the hypothalamic area, which was surrounded by marked edema. Anti-edema treatment was initially started considering the suggestion by our neurosurgery team. The patient's clinical and laboratory findings improved after the initiation of the anti-edema therapy. Afterwards, a biopsy was performed, which diagnosed a malignant diffuse large B-cell lymphoma. Subsequently, intravenous high-dose methotrexate-based therapy was started; however, after the second cycle of chemotherapy, the patient died due to sepsis. CONCLUSION: In this report, we present a case of hypopituitarism that developed due to the mass effect of hypothalamic lymphoma with clinical findings of pituitary apoplexy. Intracranial masses may cause obvious endocrinological findings related to hypopituitarism, while vague findings may also be observed due to partial failure. Therefore, it is important to perform a comprehensive endocrinological examination at the time of diagnosis in patients with intracranial masses.

Coagulative necrotic pituitary adenoma apoplexy: A retrospective study of 21 cases from a large pituitary center in China.

PURPOSE: Coagulative necrotic pituitary apoplexy (CNPA) is a clinical entity with unique intraoperative and histopathological manifestations. We aimed to improve the knowledge of this rare disease through the largest case series published to date. METHODS: A retrospective review of 21 CNPA patients was performed from among 5095 patients who underwent surgery for pituitary adenomas at a single institution between January 2009 and June 2017. The demographic, clinical, endocrine, neuroimaging, intraoperative, and histopathological findings, management and prognosis were summarized. RESULTS: Headache was the most common symptom that was observed in 21 patients, followed by visual disturbances (17/21, 81.0%), nausea and vomiting (16/21, 76.2%), electrolyte disturbance (13/21, 61.9%), and oculomotor palsies (10/21, 47.6%). Hypopituitarism with at least one anterior pituitary deficiency, especially panhypopituitarism (10/21, 47.6%), was present in 81.0% of patients. Most patients (81.0%) showed typical MRI appearances. All 21 patients underwent transsphenoidal surgery (TSS), and 16 patients had total tumor resection demonstrated by postoperative MRI. Cottage cheese-like necrosis was observed in 16 patients (76.2%) intraoperatively. Histopathology showed large areas of pink, acellular, coagulative necrotic areas in the central zone, and a pseudocapsule in the border zone. After follow-up for 4.3 ± 2.3 years, only 28.6% of patients still suffered from corticotropic deficiency, and 9.5% of patients had gonadotropic deficiency. These patients were administered the appropriate corresponding hormones for life. CONCLUSIONS: CNPA can be correctly diagnosed preoperatively by typical clinical and MRI characteristics. Early surgery combined with hyperbaric oxygen therapy early postoperatively usually yields satisfactory endocrine and neuro-ophthalmic outcomes.

An unsuspected complication with immune checkpoint blockade: a case report.

BACKGROUND: Immunotherapy treatment with immune-checkpoint blockade has become a new paradigm in cancer treatment. Despite its efficacy, it has also given rise to a new class of adverse events, immune-related adverse events, which may affect any organ, including the thyroid and the pituitary. CASE PRESENTATION: We present a case of a 77-year-old Caucasian man with metastatic renal cell carcinoma on immunotherapy treatment who was admitted to our hospital with a severe persistent headache of sudden onset. He had been on corticosteroid therapy for 10 days for suspected immune-related thyroiditis. The patient had tachycardia and mild diarrhea, and his thyroid function tests were compatible with subclinical hyperthyroidism with a suppressed thyroid-stimulating hormone level of 0.01 µIU/ml (0.4-4.5), a raised free T4 level of 2.17 ng/dl (0.7-1.9), and a free T3 level of 4.66 pg/ml (2.27-5). Computed tomography and magnetic resonance imaging revealed an enlargement of the pituitary gland compatible with macroadenoma. In the face of a probable immune-related hypophysitis, high-dose corticosteroid treatment was started. A posterior hormonal evaluation revealed secondary hypothyroidism with a suppressed thyroid-stimulating hormone level of 0.11 µIU/ml (0.4-4.5) and low thyroid hormones, a normal free T4 level of 1.02 ng/dl (0.7-1.9), and a low free T3 level of 1.53 pg/ml (2.27-5). These new findings suggested central hypothyroidism possibly due to pituitary apoplexy as a complication of the macroadenoma. Therefore, levothyroxine substitution was started along with the previously started corticosteroid therapy. The patient's headache and asthenia gradually resolved, and after a few days, he was released from the hospital with levothyroxine substitution and corticosteroid tapering. CONCLUSIONS: This case emphasizes the importance of the differential diagnosis when dealing with patients on immune checkpoint inhibitors because other non-immune-related events may present. Our patient was finally diagnosed with immune-related hyperthyroidism and a concurrent pituitary macroadenoma. This case also highlights the importance of a prompt start of corticosteroid therapy once immune-related adverse events such as hypophysitis are suspected, because otherwise the outcome would be fatal.

Unilateral Tuberothalamic Artery Ischemia Caused by Pituitary Apoplexy.

BACKGROUND AND IMPORTANCE: Pituitary apoplexy (PA) occasionally occurs in patients with pituitary adenoma and may cause severe functional deficits. Headache, pituitary insufficiency, visual impairment, and cranial nerve palsies are the most frequent symptoms in patients with PA. Secondary cerebral ischemia develops in only a limited number of PA patients. Two pathogenic mechanisms were previously proposed. One states that ischemia may be due to major vessel encasement or to vessel compression, as a result of extended tumor growth. The second states that cerebral vasospasm following PA may cause ischemia. We present another mechanism. After PA, a sudden increase in suprasellar tumor volume can lead to compression of perforating arteries causing hypoperfusion and subsequent focal ischemia of the thalamus, basal ganglia, and internal capsule. CLINICAL PRESENTATION: We present the case of a 75-year-old woman who, after having PA, developed cerebral ischemia in the territory of the left anterior thalamus and internal capsule that is primarily supplied by the tuberothalamic artery. Computed tomography and magnetic resonance imaging are used to describe how mechanical compression of the tuberothalamic artery caused this rare phenomenon. The recent literature, vascular anatomy, and pathophysiologic aspects of PA are discussed. CONCLUSION: PA can lead to compression of perforating arteries, for example, the tuberothalamic artery supplying the thalamus or lenticulostriate region, and thus cause hypoperfusion and subsequent focal cerebral ischemia. This may occur when perforating cerebral arteries are affected and compressed by the sudden increase in tumor volume due to hemorrhage or tumor swelling.

Pituitary apoplexy and rivaroxaban.

Pituitary apoplexy (PA), defined by the occurrence of a massive hemorrhagic necrotic rearrangement within a pituitary adenoma, is rare. Its occurrence can be associated with certain risk factors, including anticoagulation. We report the first case of PA with rivaroxaban which is one of the new oral anticoagulants: a 73 year-old patient presenting with severe headache and visual field deterioration. Surgery was performed. Radiotherapy treatment was decided three months after surgery because of tumor residue.

Sudden death from pituitary apoplexy in a patient presenting with an isolated sixth cranial nerve palsy.

A 68-year-old diabetic, hypertensive man presented with a left sixth cranial nerve palsy. MRI demonstrated an inhomogeneous sellar mass encroaching on the left cavernous sinus. Two days later, a left third cranial nerve palsy developed. Within 24 hours, the patient went into cardiac arrest and died. An autopsy showed hemorrhage within a pituitary macroadenoma ("pituitary apoplexy"). Pituitary apoplexy should be considered a cause of acute isolated sixth cranial nerve palsy and may represent a life-threatening emergency that can be averted with emergent hormonal replacement and hypophysectomy.

Vascular complications of transsphenoidal surgery.

Vascular complication of transsphenoidal surgery can lead to mortality and serious morbidity. In a series of 3,061 transsphenoidal operations for pituitary disease, 24 such complications were encountered, seven of which were fatal. The anatomic substrate for such complications is discussed, along with technical aspects of surgery and other methods for the avoidance of vascular complications.

[MRI findings and endocrinological dysfunction in hemorrhagic pituitary adenoma].

Magnetic resonance image (MRI) findings, intraoperative macroscopic findings and endocrinological functions were reported in 13 cases of hemorrhagic pituitary adenoma (HPA) according to clinical severity. The cases were divided into 3 groups: (1) classical pituitary apoplexy (PA) (n = 2), (2) subacute PA (n = 4), (3) asymptomatic HPA (n = 7). Based on MRI intensity and intraoperative findings, there were 7 cases with hemorrhagic PA and 5 with necrotic cyst formation. MRI intensities predicted the cyst contents, either hemorrhagic or xanthochromic, more accurately than CT findings. In addition, two classical cases of the PA group disclosed niveau formation on MRI, but MRI intensity in the first case differed from that in the second case. Classical PA of the first case occurred during the pregnancy. MRI intensity in the case 7 months after the onset disclosed high intensity of the upper part and normointensity of the lower part. T1 weighted image and proton image showed homogeneous intensity. On the contrast, PA of the second case showed water-like intensity on the upper part and methemoglobin-like intensity on the lower part. These different MRI intensities suggest different etiologies of niveau formation. MRI findings in the first case may indicate the chronic stage of massive intratumoral hemorrhage but the mechanism may be the same in both cases. From MRI intensity and clinical course, the cause of niveau formation in the second case is similar to that found in the literature. That is, hemorrhage was thought to have occurred in the pre-existing cyst cavity.(ABSTRACT TRUNCATED AT 250 WORDS)