Prolactinomas

Introduction

Pituitary tumors account for approximately 14% of primary intracranial tumors, and their overall prevalence in the general population is estimated at 17%. Among pituitary tumors, the range of functional pituitary adenomas is 46% to 64% in several series, in which prolactinomas account for 32% to 51% of pituitary adenomas, or 69% to 80% of clinically functional pituitary adenomas. Female patients typically present earlier with smaller tumors, in which their symptoms include amenorrhea, galactorrhea, headache, infertility, and/or mass effect, whereas male patients present later with larger tumor sizes and have symptoms of visual loss (~40%), premature ejaculation, erective dysfunction, hypogonadism, and symptoms of mass effect. The conventional management is the use of dopamine agonists (bromocriptine, cabergoline); however, this is not always feasible because of patient’s symptoms, social situations, and economic status. ^, In this chapter, we present a case of a patient with a prolactin-secreting pituitary macroadenoma.

Example case

Chief complaint: visual loss

History of present illness

A 24-year-old, right-handed man with severe mental retardation and Down syndrome and lymphoma presented with acute vision loss. He is nonverbal at baseline, but family noted that he was walking into walls and did not appear to be able to see anything. He was seen in the emergency room, and imaging revealed a sellar mass ( Fig. 61.1 ).

Medications: None.
Allergies: No known drug allergies.
Past medical and surgical history: Down syndrome, lymphoma (controlled).
Family history: No history of intracranial malignancies.
Social history: Not independent with activities of daily living, no smoking or alcohol.
Physical examination: Awake, alert, nonverbal; Cranial nerves III to XII intact but unable to detect threat in either eye; Moves all extremities with good strength.
Pituitary labs: Prolactin 560 (with dilution), insulin-like growth factor-1, cortisol, thyroid stimulating hormone/T4 all within normal limits.

	Carlos E. Briceno, MD, Paitilla Medical Center, Panama City, Panama	William T. Curry, MD, Massachusetts General Hospital, Boston, MA, United States	Nasser M. F. El-Ghandour, MD, Cairo University, Cairo, Egypt	Theodore H. Schwartz, MD, Cornell University, New York, NY, United States
Preoperative
Additional tests requested	CT head to evaluate paranasal sinus MRA Endocrinology evaluation Ophthalmology evaluation	CT and CT angiogram	CT head to evaluate paranasal sinus	CT head Macroprolactin test
Surgical approach selected	Endoscopic transnasal transsphenoidal	Endoscopic transnasal transsphenoidal	Endoscopic transnasal transsphenoidal	Endoscopic transnasal transsphenoidal
Other teams involved during surgery	ENT	ENT	ENT	ENT
Anatomic corridor	Transnasal transsphenoidal	Transnasal transsphenoidal	Transnasal transsphenoidal	Transnasal transsphenoidal
Goal of surgery	Decompress optic apparatus, maintain normal endocrine function	Decompress optic apparatus	Decompress optic apparatus	Decompress optic apparatus, remove as much tumor as possible with preservation of gland
Offer surgery if no vision deficit	No	Yes	No	No
Perioperative
Positioning	Supine neutral with pins and 20 degree head elevation	Supine with pins	Supine neutral with pins	Supine neutral with pins
Surgical equipment	Surgical navigation Endoscopes Doppler	Surgical navigation Endoscopes	Surgical navigation Fluoroscopy Endoscopes	Endoscopes Doppler
Medications	Steroids	Steroids	Steroids	Steroids
Anatomic considerations	Sphenopalatine artery, ICA, optic nerves and chiasm, pituitary stalk, pituitary gland, cavernous sinuses	ICA, pituitary stalk, optic nerves and chiasm	Sphenoid sinus, sella, optic apparatus, ICA, cavernous sinus, cranial nerves III–VI, diaphragma sellae, pituitary gland, infundibulum	ICA, sphenoid septations
Complications feared with approach chosen	Brain retraction injury, CSF leak, meningitis, worsening visual deficit, panhypopituitarism, ICA or cavernous sinus injury, diabetes insipidus, electrolyte abnormalities, cranial neuropathy, hydrocephalus	Injury of pituitary gland, CSF leak	Injury of pituitary gland, CSF leak	Injury of pituitary gland
Intraoperative
Anesthesia	General	General	General	General
Skin incision	None	None	None	None
Bone opening	Sphenoid ostia, sphenoid rostrum, anterior sphenoid sinus wall, sellar face	+/– right middle turbinate, anterior sphenoid sinus wall, sellar face	Sphenoid rostrum, anterior sphenoid sinus wall, sellar face	Sphenoid rostrum, anterior sphenoid sinus wall, posterior ethmoids, sellar face
Brain exposure	Sella	Sella	Sella	Sella
Method of resection	Pack both nostrils with oxymetazoline, prep with antibiotics and antiseptic solutions, compress inferior/middle/superior turbinates laterally in right nostril, coagulate mucosa over rostrum of sphenoid sinus, nasal septum out fractured, submucosal dissection along contralateral rostrum to visualize contralateral sphenoid ostia for binostril approach, harvest nasoseptal mucosa and hide in nasopharynx, open both sphenoid ostia with Kerrison rongeurs first inferiorly, remove rostrum and anterior wall of sphenoid sinus, skeletonize walls of sphenoid sinus, identify landmarks (clivus, sellar floor, carotid prominences, optic prominences), use microdoppler to confirm ICA, open dura, piecemeal resection in the midline, debulk inferior part and remove until encounter posterior sella, resect tumor laterally off cavernous sinus walls, remove superior portion extracapsular if possible, inspection with 30-degree scope for residual, use abdominal fat and fascia for dural reconstruction, bone from vomer and nasoseptal flap to reconstruct sella, dural sealant, avoid packing nostrils	+/– removal of right middle turbinate, identify and expand sphenoid ostia to enter sphenoid sinus, remove sphenoid face, posterior nasal septectomy preserving sphenopalatine artery, remove intersinus septations, identify sellar anatomy, remove sellar face from carotid to carotid up to tuberculum, open dura, observe for diaphragm, no need to be aggressive in cavernous sinuses because expect medical therapy to be effective, seal dural defect with free mucosa, +/– abdominal fat graft	Antiseptic nasal wash, lateralization of bilateral middle turbinates, C-shaped incisions in bilateral septal mucosa, elevation of nasoseptal flaps, removal of sphenoid rostrum, enlargement of sphenoid ostia and removal of anterior sphenoid sinus wall, removal of sellar face after confirmation with lateral fluoroscopy, needle to confirm not carotid before dural opening, tumor debulking in piecemeal with ring curettes, Valsalva to cause descent of tumor, inspection with angled endoscopes. If CSF leak, then harvest fat, fascia lata or dural substitute, and fibrin glue, with lumbar drain for 3 days.	ENT opening, removal of anterior sphenoid wall/rostrum/posterior ethmoids, compare sphenoid septations to preoperative imaging to understand location of ICA and location of normal gland, sella opened from medial cavernous to cavernous sinus and from superior intercavernous sinus to floor, Doppler to identify ICAs, dura opened cruciate fashion, debulking at center and inferior of tumor and then laterally until medial wall of cavernous sinus identified, roof of the tumor attempted at en bloc, harvest fat for CSF leak, gelfoam for cavernous sinus bleeding
Complication avoidance	Preserve sphenopalatine artery and nasoseptal flap, identify key anatomic landmarks, microdoppler to confirm ICA, remove superior portion extracapsular, abdominal fat and fascia for dural reconstruction, use bone to reconstruct sella	Preserve sphenopalatine artery and nasoseptal flap, large sellar face opening, observe for diaphragm, conservative approach in cavernous sinus	Avoid removal of middle turbinates, large sphenoid opening, fluoroscopy to identify sellar anatomy, needle to confirm absence of vascular structures, harvesting of nasoseptal flaps	Study sphenoid septations to identify ICA location, study gland location, debulk center and inferior portion of tumor first, en bloc resection of superior component, abdominal fat for CSF leaks
Postoperative
Admission	ICU	Floor	ICU	Intermediate care
Postoperative complications feared	CSF leak, worsening visual deficit, panhypopituitarism, carotid or cavernous sinus injury, diabetes insipidus, electrolyte abnormality, cranial neuropathy, hydrocephalus	Recurrent hemorrhage, diabetes insipidus, CSF leak	Diabetes insipidus, panhypopituitarism, CSF leak	Diabetes insipidus, panhypopituitarism
Follow-up testing	Prolactin levels until <20, serum sodium/urea/creatinine postoperative and daily, a.m. cortisol MRI within 48 hours after surgery	Serum sodium, urine output and osmolarity (serum sodium every 3 days for several weeks), a.m. cortisol MRI prior to discharge	Prolactin at 2 and 6 weeks, then every 3 months after surgery Pituitary hormonal analysis 1 day after surgery Lumbar drain for 3 days if CSF leak MRI within 24 hours after surgery	Prolactin daily until discharge MRI within 48 hours after surgery Serum sodium and urine output every 6 hours initially
Follow-up visits	2 weeks after surgery	ENT 1 week after surgery Endocrinology 30 days after surgery Neurosurgery 6 weeks after surgery 7 days after surgery with ENT and endocrinology	2 weeks, 6 weeks, 3 months after surgery	Endocrinology 1 week after surgery ENT 1 week after surgery Neurosurgery 3 months after surgery
Adjuvant therapies recommended	Reoperation or radiation therapy if treatment failure	Defer to endocrinology	Craniotomy for residual	Defer to endocrinology