Pituitary neuroendocrine tumor
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History
Risk factors
Pituitary tumors have very few known risk factors, and these are related to genetics. There are no known environmental or lifestyle-related risk factors for pituitary tumors. Though science has suggested that people who are overweight or obese might be at increased risk.
Youn et al. discovered that a 3'untranslated region (3'UTR) variant, rs181031884 of CDKN2B (Asian-specific variant), had significant association with the risk of pituitary neuroendocrine tumor (PA) (Odds ratio = 0.58, P = 0.00003). Also, rs181031884 appeared as an independent causal variant among the significant variants in CDKN2A and CDKN2B, and showed dose-dependent effects on PA.
Although further studies are needed to verify the impact of this variant on pituitary neuroendocrine tumor susceptibility, the results may help to understand CDKN2B polymorphism and the risk of pituitary neuroendocrine tumor 1).
Epidemiology
Classification
Natural History
Genetic syndromes
Pathogenesis
Clinical features
Diagnosis
Treatment
Outcome
Case series
Case reports
A 45-year-old woman who suffered from limb edema for 2 months. Dong et al. focused on tumor recurrence and other common potential diseases based on the pituitary neuroendocrine tumor history. However, none of the examinations showed any abnormality. Later, her continuous complaints about the family relationship and depressed mood came into sight, and a psychiatry consultation was arranged. Following that, she was diagnosed with major depressive disorder. After several days of Melitracen and tandospirone treatment, the patient's limb edema dramatically subsided. This is the first case of limb edema associated with depression. This highlights the importance of awareness of mental illness for non-psychiatrists, especially in patients with severe somatic symptoms, but with negative results 2).
Recurrence
Pituitary neuroendocrine tumor General University Hospital of Alicante Cases
A 39-year-old woman reports visual loss (blurred vision) in both eyes for 2 months, the left worse than the right. Refers to headache (twice a week) that subsides with paracetamol In the last 15 days, he has woken up at night several days due to headaches.
Migraines under control by Neurology
Antiphospholipid syndrome antibody/hypercoagulability in follow-up by Hematology
APA is positive at low titer.
Bilateral superior external quadrantanopia
Treatment with Zolmitriptan
Computed tomography
Suprasellar mass is observed that occupies and expands the sella turcica, difficult to define, which produces a break in the continuity of the floor of the sella turcica and a complete occupation of the sphenoid sinus, which also presents an expansion of the same and thinning of its bony walls. It has maximum measurements of 3.5 x 2.8 x 1.2 cm (craniocaudal by transverse by anteroposterior). These findings seem compatible with an aggressive pituitary neuroendocrine tumor
The mass ascends through the sellar diaphragm and compresses the optic chiasm, and in the lateral areas, it completely encompasses both intracavernous carotid arteries and both cavernous sinuses bilaterally.
Brain MRI
A large sellar lesion with a marked isointense suprasellar extension on T1 and heterogeneous on T2 with multiple hyperintense foci on T2, especially the cystic-necrotic suprasellar portion in relation to macroadenoma. The lesion measures approximately 4 cm in diameter craniocaudal, 3.9 anteroposterior, and 4.2 cm transverse. It extends to both cavernous sinuses and surrounds the right internal carotid artery for more than 180° and the left for approximately 180°. It causes a mass effect on the optic chiasm, displacing it superiorly. The pituitary stalk seems to be located anterior to the lesion with a slight right lateralization, although it is difficult to locate it due to the large size of the lesion.
HORMONES
FREE T4 1.7 ng/dL
FSH 5.3 U/L
LH 3.4 U/L
PROLACTIN 8.3ng/mL
ESTRADIOL 32.0 pg/mL
CORTISOL MORNING 10.9 µg/dL
IGF-1 231 ng/mL
Under general anesthesia, orotracheal intubation, and antibiotic prophylaxis with cefazolin 2 gr IV. Supine position with neutral head resting on a donut-type pillow. Preoperative topical intranasal oxymetazoline was applied with lectins.
nasal phase: Right middle turbinate resection. Preparation of a nasoseptal flap with mucosa from the right septum. It is left lodged in the right choana. Posterior septostomy and communication of both nostrils. In the ostium, a tumor is visualized that completely occupies the sphenoid sinus. Wide anterior sphenoidotomy with the help of a laminotome and cutting burr. Profuse bleeding throughout the nasal phase comes from the tumor. Part of the left paramedian septum that was encompassed by the tumor was removed.
Excision phase: Excision of the tumor part contained in the sphenoid sinus until the bony limits of the sella turcica were visualized. With the help of neuronavigation and Doppler, both ICAs were located. Clivus partly eroded. In the most inferior and posterior part, a bone area corresponding to the posterior clinoid is observed, which is moth-eaten and loose, encompassed by a tumor. Intracapsular excision of the tumor is started by way of debulking, and sending tumor samples for AP analysis. The tumor shows a friable consistency and a purplish color compatible with a pituitary neuroendocrine tumor. Central excision until visualizing gradual descent of sellar and arachnoid diagrams in the sellar cavity with contained low-flow fistula. Exeresis in the posterior region until observing the dura mater of the posterior fossa. Excision of the lateral walls and part of the cavernous sinus. Hemostasis with Floseal.
Reconstruction phase: Tachosil is placed covering the arachnoid in the area of the contained fistula. A nasoseptal flap is placed in contact with the bone defect around the sellar opening. The flap is fixed with surgicel and tissucol. Rapid -Rhino binasal tires are left. The free mucosa of the middle turbinate is left covering the part of the septum from which the flap has been removed.