A 30 yr old gentleman, previously fit and well, presented to the ENT Surgeons for an endonasal biopsy, and was diagnosed with an olfactory neuroblastoma. He later presented with nausea and vomiting and his clinical condition deteriorated leading to a diagnosis of a macroprolactinoma. His initial prolactin level was > 847458 mu/L.Once the tumour was excised, the patient needed cranio cervical fixation for instability of the base of the skull. From being unable to see, hear, speak, walk and eat, to achieving all but hearing, we describe to you this patients miraculous journey over 8 months.
This case highlights the importance of neurological rehabilitation and shows the effectiveness of the multi disciplinary team.
macroprolactinoma, tumour, rehabilitation
Prolactinomas form one of the most common hormone – secreting pituitary tumours. The size of the tumour classifies them into microprolactinomas (<10 mm in diameter) and macroprolactinoma (>10 mm in diameter).
Prolactinomas are derived from the lactotroph cells of the adenohypophysis, and are histologically benign neoplasms accounting for almost 30% of all pituitary adenomas.
Treatment modalities include: Pharmacological, surgical and radiotherpay. Pharmacological therapy with Dopamine agonists has been very successful in a majority of the patients, with surgery being implicated in those with massive tumours which are invasive in nature.
We present an interesting case report of a patient who presented with nasal symptoms, diagnosed to have an olfactory neuroblastoma, got very ill, leading to an eventual diagnosis of a macroprolactinoma, requiring surgery. Despite a very hard struggle, our patient recovered very well and with a multi disciplinary approach, we managed to improve his quality of life to almost his pre morbid state.
Multiple case reports have been published with macroprolactinomas and their uncomplicated journey, but our patient had a good outcome after an extremely complicated and frustrating journey. Reports of a successful outcome after a complicated course of events following a diagnosis of macroprolactinoma has not been very well reported.
A 30 yr old gentleman, was seen by the ENT surgeons for an endonasal biopsy of a mass, after having presented with nasal symptoms. 5 days post biopsy, the patient presented to the Emergency Department with nausea and vomiting. An MRI of his brain revealed a macroprolactinoma. His prolactin level on admission was raised at >847458 mu/L. Cabergoline was started.
The very next day, he developed a lower motor neuron type facial palsy and tinnitus, associated with severe hearing loss and fasciculation of tongue. He was found to have problems with swallowing.
A CT Scan done at this stage showed a very large tumour centered on the sphenoid bone but
bulging into the posterior fossa and middle fossae and extending inferiorly into the ethmoid sinuses and nasal cavity. He was started on Dexamethasone. He rapidly deteriorated with proptosis and signs of brainstem dysfunction, at which point he was taken to the Neurological Intensive Care Unit, and a decision for a right pteronial craniotomy for decompression was made.
The patient needed an orogastric tube for feeding and a tracheostomy to keep the airway open. He was noted have a CSF leak through his right nostril which was treated conservatively. A repeat MRI showed the mass to be distorting the pons and a right frontal collection. A Lumbar puncture performed at the time excluded meningitis or infection. He had a couple of episodes of shoulder twitching which was diagnosed as post traumatic epilepsy and the patient was commenced on anti epileptic medications.
The patient had multiple medical problems post surgery and infections were treated with high doses of antibiotics. At this point he could not see, hear, speak or mobilize. The therapists communicated with him by writing on his hand. He managed to improve slowly with speech and was able to answer questions when the therapists wrote on his hand.
He had a PEG (Percutaneous endoscopic gastrostomy) tube inserted in the meantime and the dieticians and the speech and language therapists worked very hard to ensure his nutrition was maintained and his speech improved. His nutritional requirement was met with his gastrostomy feeding regimes.
A CT of his Cervical Spine showed extensive tumour infiltration, which had largely destroyed the right occipital condyle with loss of the normal cortical outline. There was very extensive infiltration of the left occipital condyle also, but with preservation of the wafer thin cortex and a normal outline.
A few months post his first surgery, he had a craniocervical fixation carried out to ensure stability of his base of skull and cervical spine. He failed his tracheostomy removal and was instructed to stay on it. He was trained to use his voice and talk with his tracheostomy.
He was referred to the rehabilitation team, when he was transferred to a medical ward. He developed MRSA (Methicillin resistant Staphylococcus aureus) colonization in his Tracheostomy and PEG sites and needed isolation. He was advised physiotherapy and occupational therapy to assess and improve his strength and muscle bulk. He was very soon transferred to the rehabilitation ward, where he began to mobilize.
The patient mobilized with a stick and moderate assistance initially, but as the intensive rehabilitation plan unfolded, he did not take long to walk independently. He was constantly seen by the dieticians and the speech and language therapists, who trained him to set his PEG Feeds and encouraged oral intake as able. He was seen and assessed by occupational therapy and was enrolled onto the work rehabilitation service to enable him to return to his work as a teacher. He was assessed in the kitchen to make meals which he did successfully. He was also seen by a clinical psychologist to assess his mood and adjust his anti depressants accordingly. The Epilepsy nurse specialist was involved to give him further information on his anti epileptic medication and advise him of side effects. Discharge prolactin level was 588 mu/L.
The patient made remarkable recovery. Having been acutely unwell for almost 5 months, he made rapid recovery with intensive rehabilitation in less than 2 months. He revisited the Neurological intensive therapy unit on his recovery which not only amazed the medical and therapy staff that looked after him previously, but also made him recognize the extent of recovery he had made. He was able to see though his visual acuity was slightly decreased, was able to talk and respond to people by lip reading, walked independently with no aids or assistance, able to cook for himself and manage his life as a whole.
After his 8 month ordeal in fighting for his life, our patient had a graded discharge to his apartment. He had numerous follow up appointments with all the teams who looked after him. He managed well on discharge and had no further complications. He was followed up in the outpatient clinic with no new concerns.
Macroprolactinomas despite being a common endocrine tumour can present with unexpected symptoms. Symptoms may range from headaches due to a compressive mass, to menstrual irregularity in women, and features of hypogonadism in men. Problems with visual acuity is seen commonly in both sexes.
Pituitary tumours rarely present with nasal symptoms. A prolactinoma must be considered in the differential for nasopharyngeal tumours.
Invasive macroprolactinomas can commonly be associated with epilepsy, particularly of temporal lobe origin. It is essential to enquire about epileptic symptoms, as the seizure disorder may have been undiagnosed/untreated for years.
Investigations primarily include an MRI Scan of the brain, along with measurement of the other pituitary hormones. Formal visual field testing would be beneficial, especially in the case of large tumours, which may lie close to the optic chiasma.
Standard treatment revolves around medical management with replacing hormones that are deficient. Surgical and radiotherapy options are also used in some patients.
Macroprolactinomas, unlike other symptomatic pituitary tumours, are typically treated with primary medical therapy; however, in the case of CSF leak, surgery is indicated.
For those complicated patients who show neurological involvement, it is absolutely essential that they are involved in an intensive neurological rehabilitation programme as with our patient.
Rehabilitation medicine encompasses a multi disciplinary approach to patient care with the goal of achieving premorbid status. The British Society of Rehabilitation Medicine describes rehabilitation with 2 definitions:
- Conceptual definition: A process of active change by which a person who has become disabled acquires the knowledge and skills needed for optimal physical, psychological and social function.
- Service definition: The use of all means to minimise the impact of disabling conditions and to assist disabled people to achieve their desired level of autonomy and participation in society.
Neurological rehabilitation is available in selected centres throughout the UK and appropriate referrals should be made on recognition of suitable patients. Patients are assessed by Rehabilitation physicians, and accepted to an intensive rehabilitation unit, where the multi disciplinary teams work hard in achieving goals. There is evidence to show that early referral and early rehabilitation has better outcomes in some patients [1-8].
Prolactinomas are benign, rarely malignant tumours of the lactotrophic adenohypophyseal cells. It has a lower prevalence in men compared to women. It runs an uncomplicated course in majority of patients.
Macroprolactinomas presenting with nasal symptoms are extremely rare, but must be remembered in the differential diagnosis. Serum Prolactin levels help tremendously in the diagnosis, even before imaging.
MRI scans of the brain remain the best imaging modality though CT Scans could be used to look for bony destruction or erosion.
Treatment modalities include medical, surgical and radiotherapy. The most important aspect of management includes the rehabilitation programme for the patient once past the acute phase. It is highly important to formulate a concise, intensive, challenging but achievable rehabilitation plan for such patients, to help regain as much of their premorbid independence.
Neurological rehabilitation includes a multi disciplinary team approach, with the patient being the main focus. From improving mobility to speech and dietetics, it involves a holistic approach to patient management. It is extremely important for patients to be involved in the rehabilitation programme from the acute phase, so that the intensity of the approach can be modified as the patient improves. With regular goal planning and intensive neuro rehabilitation, patients make tremendous progress and the improvement in their condition is remarkable.
I would like to extend my most sincere gratitude to the entire team who worked very hard in the rehabilitation programme for this patient.
- Tam M (2009) A case presentation: medical management of macroprolactinoma in a 16-year-old boy. Endocrine Abstracts 19: 184.
- ‘Prolactinoma’, Venkatesh Babu Segu, Emedicine- Endocrinology, 2008.
- Pinzone JJ, Katznelson L, Danila DC, Pauler DK, Miller CS, et al. (2000) Primary medical therapy of micro- and macroprolactinomas in men. J Clin Endocrinol Metab 85: 3053-3057. [Crossref]
- Johnston PC, Courtney HC, Hunter SJ, Mc Cance DR (2012) Macroprolactinomas presenting as nasal polyps: a series of three cases. Ir J Med Sci 181: 277-279. [Crossref]
- ‘Prolactin Secreting Pituitary Adenomas: Analysis of 429 Surgically Treated Patients, Effect of Adjuvant Treatment Modalities and Review of the Literature ‘, T Ozgen et al, Acta Neurochirurgica 141: 1287-1294.
- Rehabilitation following Acquired brain injury – National Clinical Guidelines, 2003.
- Deepak D, Daousi C, Javadpour M, MacFarlane IA (2007) Macroprolactinomas and epilepsy. Clin Endocrinol (Oxf) 66: 503-507. [Crossref]
- Mankia SK, Weerakkody RA, Wijesuriya S, Kandasamy N, Finucane F, et al. (2009) Spontaneous cerebrospinal fluid rhinorrhoea as the presenting feature of an invasive macroprolactinoma. BMJ Case Rep. [Crossref]