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Table of Contents
CLINICAL ARTICLE
Year : 2021  |  Volume : 22  |  Issue : 2  |  Page : 137-142

Adrenocorticotropic hormone-secreting pituitary macroadenoma: A pictorial case report and review


Associate Professor, Sadhu Vaswani College of Nursing, Pune, Maharashtra, India

Date of Submission26-Dec-2020
Date of Decision30-Nov-2021
Date of Acceptance01-Nov-2021
Date of Web Publication31-Jan-2022

Correspondence Address:
Mrs. Ujjwala Murkute
Sadhu Vaswani College of Nursing, 10-10/1, Koregaon Road, Pune - 411 001, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijcn.ijcn_138_20

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  Abstract 


Pituitary adenomas are the most common type of pituitary disorder. While prolactinoma and non-functioning pituitary adenomas are the most common pituitary tumour, adrenocorticotropic hormone (ACTH)-secreting tumours are relatively rare. ACTH-secreting tumour arises from corticotroph cells from pituitary gland and generally presents as Cushing's disease. Thirty-one years old female with recent history of multiple admission to acute care hospital got admitted to general medicine ward with complaints of gastro intestinal disturbance. Magnetic resonance imaging and lab studies confirmed the diagnosis of ACTH-secreting pituitary macroadenoma with development of Cushing's disease. The onset of illness was very slow and most of the signs and symptoms remained non-specific until patient developed severe metabolic and hormonal disturbances. Over a period of two years patient had multiple diagnosis like poly cystic ovarian disease, diabetes mellitus, depression and hyperacidity by different medical practitioners and received symptomatic treatment. Patient's condition completely improved after transnasal endoscopic excision of pituitary macroadenoma normalising her hormonal levels with disappearances of bodily changes. Most of her complications were successfully treated except diabetes insipidus which was identified during pre-operative period remained unchanged even after the surgery and patient continued treatment for the same. In this paper the condition of pituitary macroadenoma and the nursing care of the patient with pituitary macroadenoma is elaborated.

Keywords: Adrenocorticotropic hormone-secreting pituitary macroadenoma, case report, diabetes insipidus, pictorial, transnasal endoscopic excision


How to cite this article:
Murkute U. Adrenocorticotropic hormone-secreting pituitary macroadenoma: A pictorial case report and review. Indian J Cont Nsg Edn 2021;22:137-42

How to cite this URL:
Murkute U. Adrenocorticotropic hormone-secreting pituitary macroadenoma: A pictorial case report and review. Indian J Cont Nsg Edn [serial online] 2021 [cited 2022 May 28];22:137-42. Available from: https://www.ijcne.org/text.asp?2021/22/2/137/336896




  Introduction Top


Pituitary adenomas are the most common cause of pituitary hormone hypersecretion and hyposecretion syndromes in adults. They account for approximately 10%–15% of all intracranial neoplasms. Morphologically, these tumours may arise from a single polysecreting cell type or include cells with mixed function within the same tumour. Pituitary tumours are classified both according to their hormone content and by their size. Hormonally active tumours (functional) are characterised autonomous hormone secretion with diminished feedback responsiveness to physiological inhibitory pathways.[1],[2],[3],[4] Non-functional pituitary adenomas grow relatively larger until the appearance of symptoms which are mainly due to compression of surrounding structures.[5] Pituitary microadenomas are defined as lesions less than 10 mm in diameter and are located completely within sella, whereas macroadenomas are more than 10 mm in size and are often associated with extrasellar extension.[1],[2],[3],[4] Prolactomas and growth hormone (GH)-producing adenomas are the most frequent pituitary tumour types followed by adrenocorticotropic hormone (ACTH)-producing adenomas and gonadotrophic adenoma. Thyroid-stimulating hormone (TSH)-producing adenomas are rare.[4],[6]

In the past, patients with pituitary tumours often presented with classic endocrine and neuro-ophthalmologic abnormalities caused by advanced tumour. Today most tumours are diagnosed earlier with symptoms of endocrine hyper secretion only. Endocrine hyper secretion depends on secretary properties of the tumour: galactorrhoea and primary/secondary amenorrhea in prolactonemia, Cushing disease in an ACTH-producing tumour and acromegaly or gigantism in a GH-secreting tumour. In later stages, growing tumour may cause symptoms from local mass effects. The most common but least specific mass effect is headache. Compression of neural structures including optic nerve and optic chiasm typically causes bitemporal hemianopia and occasionally visual filed defects.[4],[6],[7] Pituitary apoplexy i.e., bleeding or infarct in pituitary tumour may cause sudden expansion with local compression and acute onset hypopituitarism.[7]

Symptomatic patients with pituitary lesion should undergo complete neurological examination with formal visual field evaluation. Neuro-ophthalmologic examination with visual filed testing and evaluation of acuity, contrast and color vision is necessary to detect subtle field changes and should be performed before and after treatment. A complete endocrinologic blood work up should be considered including prolactin (PRL), GH <TSH, follicle-stimulating hormone, luteinising hormone, fasting early morning cortisol level.[6],[7] Diagnosis of an ACTH-secreting tumour can be difficult and may require dexamethasone suppression test or petrosal sinus sampling especially when tumour is undetectable on magnetic resonance imaging (MRI) or computed tomography.[4],[6] Dynamic high resolution magnetic resonance imaging with intravenous gadolinium contrast medium enhancement is the diagnostic imaging modality of choice for patients with suspected pituitary disease.[8],[9]

The aim of treatment is to lower the mass effect, restore normal endocrine function and prevent reoccurrence. It is essential that the patient's endocrine status is established particularly with regards the ACTH-cortisol axis and PRL. High PRL level may preclude the need for surgery. Instead they can be managed medically with dopamine agonists. Cortisol deficiency must be corrected especially in the perioperative period.[4] All microadenomas and some macroadenomas can be treated with transsphenoidal pituitary surgery, which is associated with minimal morbidity.[2] Transfrontal surgery via craniotomy is reserved for suprasellar tumours. Urgent treatment is required if there is evidence of pressure on visual pathway. All operations on pituitary carry a risk of damaging normal endocrine function. Surgical success is determined by normalisation of endocrine functions and prevention of recurrence. Complications associated with surgeries are cerebrospinal fluid (CSF) leak (3%), visual deterioration (1%), major vessel injury (1%) and pan hypopituitarism (1%). Diabetes insipidus (DI) occurs following manipulation of the pituitary stalk and is usually transient. Pituitary function should be retested 4–6 weeks following surgery to determine the development any new hormonal deficit. Following surgery imaging is repeated usually after few months, if there is residual mass and histology confirms the radiosensitive tumour, external radiation therapy is given to reduce the risk of reoccurrence. But radiation therapy carries risk of lifelong hypopituitarism (50%–70% in first 10 years).[7]


  Case Report Top


A 31-year-old female was admitted to general medicine ward with chief complaints of fever, nausea, vomiting, cramping pain in abdomen, anorexia. swelling over lower limbs and joint pain. She had complaints of intermittent fever, joint pain and giddiness for one week. Later she developed nausea, vomiting and cramping abdominal pain which got aggravated with food intake. She was provisionally diagnosed as having acute gastroenteritis. She was admitted with similar complaints 6 months before and was treated with supportive management. Detailed health history revealed that there was steady increase in weight, (around 15 kg) in spite of regular exercise and diet control over past nine months. In addition to weight gain there was also history of increased frequency of urination, hyperacidity, appearance of acne on scalp, face, chest and back, dryness of lips and skin, increased body hair, irregular menstrual cycles including oligomenorrhea and amenorrhea in the previous 5 months before admission. Patient reported about occasional mood swings with generalised feeling of sleepiness and sluggishness. Patient also was known case of depression and was being treated with Tab. Sertaline 50 mg HS for two years. She was diagnosed with hyperlipidaemia and poly cystic ovarian disease (PCOD) two years back. There was family history of Type 2 Diabetes Mellitus and essential hypertension for both the parents and her mother had a history of hypothyroidism. She was provisionally diagnosed to have acute gastroenteritis with PCOD.

Physical examination findings

The patient weighed 76.5 kg and was 5.6 ft tall. Her body mass index was 28.1 kg/m2. Her blood pressure was 110/70 mmHg, pulse was 96 beats per minute, respiratory rate was 20 breaths per minute, and temperature was 98°C. She appeared to be a well-nourished, mildly dehydrated and tired client. She was polite and cooperative. Her chest examination was normal, revealing normal breath and heart sounds on auscultation. Abdomen was soft and non-tender without hepatosplenomegaly. Neurologically, she was alert and oriented to person, time, and place with a normal attention span and concentration, normal language, and good recent and remote memory. Her muscle strength was 5/5 in all major muscle groups. Important physical finding were moon facies, Striae on abdomen and thighs, acne on face, shoulder and chest, excessive facial hair, Petechial patches on hand, acanthosis nigricans, fat pads in supraclavicular, temporal, and dorsocervical areas, oedema on lower legs [Figure 1]. Vital parameters were in normal range with no evidence of pallor, icterus, cyanosis, clubbing or lymphadenopathy.
Figure 1: Physical examination findings in pituitary adenoma

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Diagnostic evaluation

High blood glucose level (197 mg/dl), decreased level of serum electrolytes (sodium: 121 mEq/L, potassium: 2.6 mEq/L, chloride: 93mEq/L), low blood urea nitrogen (7mg/dl), high creatinine (1.56 mg/dl), slightly lower serum osmolality (275.53 milliosmoles/Kg of H2O), low urine osmolality (63.23 miliosmoles/Kg of H2O) and low haemoglobin (10.4 gm/dl). Findings for endocrine evaluation revealed normal TSH and normal serum cortisol levels at 4pm and 8am (66.32 ng/ml and 127.73 ng/ml respectively) and normal serum ACTH levels (34 pg/dl). There was mild increase in PRL level (37.34 ng/dl) which became normal (24.65ng/ml) when repeated after four days. Insulin like growth factor was low (<25ng/ml). Serum amylase and liver function tests (LFTs) also were done to rule out pancreatitis as there was abdominal pain with GI disturbance. Serum amylase was normal but in LFT the enzymes were increased while albumin was low. Ultrasonography revealed fatty liver and mild splenomegaly. Cardiac colour Doppler and lower venous limb Doppler were done to investigate shortness of breath and lower limb oedema respectively but did not reveal any abnormality.

The patient's MRI brain showed lesion in sella turcica and suprasellarcistern measuring 19.4 mm × 11.4 mm × 12.2 mm (trans × anteroposterior × craniocaudal) present in midline and to the left of the midline. Lesion caused mild displacement of the pituitary stalk to right side. No compression was seen on prechiasmatic optic nerve and optic chiasm. MRI findings along with endocrine evaluation established the final diagnosis of Pituitary Macroadenoma with Cushing's disease.

Course of treatment

Prior to the diagnosis, patient was referred to orthopaedic surgeon for low back ache and lower leg pain, to gynaecologists for irregular menstruation and to the gastroenterologists for deranged LFTs. Orthopaedician prescribed analgesics and interferential therapy for back ache and had also ordered MRI spine. Gynaecologist advised for clindamycin vaginal pessary and a review of ultrasound abdomen. Gastroenterologist advised for laboratory tests such as serum iron, total iron binding capacity, ferritin, cerruloplasmin, lipid profile and serum amylase. Gastroscopy was suggested owing to her frequent hyperacidity and GI upset.

After diagnosis of a pituitary macroadenoma from MRI, the patient was referred to a neurosurgeon, an endocrinologist, and an ophthalmologist. The neurosurgeon reviewed her MRI and laboratory test results and warranted surgical excision owing to size and secretary nature of pituitary adenoma. The endocrinologist ordered the plasma cortisol level, serum ACTH and Dexa suppression test. Patient's physical findings were suggestive of Cushing's disease than hyperprolactinoma even though cortisol and ACTH level were found to be within normal range. Ophthalmological examination revealed mild blurring of vision at nasal margin but no papiloedema. Retina looked normal. Patient received supportive management in pre-operative period with intravenous plasmolyte, injection Nexpro 40 mg, injection emeset 4 mg, injection paracetamol 1 gm and Tab. nitazoxanide 500 mg, Cap Enuff (Racecadotril), Cap vibact, and Tab. Glimesave M1 (Glimipride + metformin).

Patient underwent transnasal endoscopic excision of pituitary macroadenoma and mucosal rotation flap and sphenoid packing for CSF leak 2 days after the diagnosis. Her post-operative recovery was uneventful. Her problem of DI continued post-operatively as her serum and urine osmolality continued to remain on lower side. She was started with Minirin nasal spray twice a day. She was discharged on 4th post-operative day with Tab omnacoortil 2.5mg OD × 5 day, Minirin nasal spray BD (desmopressin), Liq paraffin 2 drops each nostril QID and Tab Rupfo (cefpodoxime) 200 mg BD × 7 days. Patient responded very well after surgery. All her complaints had reduced within few weeks including weight gain. The skin issues resolved completely over a few weeks. Excess weight gain resolved and she lost weight. She returned for regular follow-up and continued treatment for her complaints of anxiety and depression in psychiatry outpatient department.


  Nursing Management Top


Nursing assessment of patient

  • Subjective data:


    • Past health history of any endocrine tumour (pituitary, adrenal, pancreatic or pulmonary), GI bleeding, hyperacidity, frequent infections. Use of corticosteroids or any other medications
    • Health perception, history of weight gain, anorexia, polyurea, prolonged wound healing, easy bruising, amenorrhea insomnia, headache, negative feeling regarding bodily changes, anxiety, mood disturbances etc.


  • Objective data: Presence of truncal obesity, supraclavicluar fat pads, buffalo hump, moon facies, hirsuitism, thinning of body hair, acne, petechiae, purpura, hyperpigmentation, purpulish straie, muscle wasting, electrolyte imbalance
  • Deranged serum electrolyte and hormonal levels
  • MRI findings of pituitary macroadenoma.



  Nursing Care Top


1. Nursing Diagnosis

Imbalanced nutrition more than body requirement related to hormonal imbalance as evidenced by truncal obesity, moon facies and buffalo hump.

Expected outcome

Patient will adhere to prescribed diet.

Interventions

  • Discussed about patient's food preferences to evaluate eating habits and to include preferred food in her diet
  • Planned a dietician meeting to discuss meal planning to help plan her diet
  • Health teaching was given regarding low calorie and nutritious foods to avoid further weight gain
  • Weighed patient daily to monitor changes in weight and effectiveness of diet changes
  • Discussed about exercise progam as per patients preferences and suitability to aid in losing weight.


Evaluation

Patient stated her plan to monitor and maintain specific target weight after discharge.

2. Nursing Diagnosis

Disturbed body image related to alterations in weight and physical appearance as evidenced by patients expression of negative feelings about body.

Expected outcome

Patient will communicate positive feelings about changes in body image.

Interventions

  • Discussed about existing coping pattern to gain insight into patients behaviour
  • Encouraged patient to voice her concern regarding her bodily changes to enhance positive feelings about herself
  • Encouraged patient to participate in daily care to improve the decision making and reduce negative feelings
  • Discussed few coping strategies to help overcome maladaptive behavior.


Evaluation

Patient used coping pattern to deal with changes in body image and participate in planning of care.

3. Nursing Diagnosis

Impaired skin integrity related to excess corticosteroids and altered skin fragility as evidenced by petechaie, straie, oedema of lower limb.

Expected outcome

Patient will exhibit no evidence of skin breakdown.

Interventions

  • Inspected patient's skin regularly to assess any new changes
  • Assisted patient in general hygiene and comfort measures to promote comfort and general sense of well being
  • Patient teaching was given regarding skin care routine to help prevent any damage to skin
  • Explained dietary and fluid restrictions to reduce oedema
  • Reminded patient not to scratch or peel the skin to avoid injury.


Evaluation

Patient skin remained intact and performed skin care routine.

4. Nursing Diagnosis

Acute pain related to surgical intervention as evidenced by patient's verbalisation of pain score 6.

Expected outcome

Patient will express feeling of comfort and relief from pain.

Interventions

  • Assessed the patient's sign and symptoms and administered pain medication as prescribed to relive pain and discomfort
  • Provided comfort devices and changes in position to enhance relaxation and sleep
  • Planned nursing care activities to minimise the interruptions in patient rest
  • Frequent assessment of surgical site is done to identify any problem
  • Application of emollient to smoothen the skin around the nares and to help remove crust.


Evaluation

Patient's need for pain medication decreased after 72 h of post-surgery.

5. Nursing Diagnosis

Deficient fluid volume related to polyurea secondary to DI.

Expected outcome

Patient vital signs remain stable. Urine specific gravity will remain between 1.005 and 1.010.

Interventions

  • Monitored vital signs every 2 h to identify hypotension
  • Maintained intake and output record to identify polyurea and response to treatment regimen
  • Administered Minirin nasal spray as per prescription to prevent polyurea
  • Administered intravenous fluids to replace excessive loss of fluid through urine
  • Test the urine for specific gravity to assess response to treatment regimen
  • Taught the patient regarding DI, about thirst mechanism to enhance self-care.


Evaluation

Patient fluid volume remained adequate and urine specific gravity remained normal.

6. Nursing Diagnosis

Altered mental function, depression related to disease condition as evidenced by low mood and reduced function.

Expected outcome

Patient will be able to cope with the disease process.

Interventions

  • Ensured psychiatric consultation is sought and treatment was initiated for depression
  • Provided safe environment. Explained to family about potential self-harm and the vigilance needed for preventing any harm
  • Administered antidepressants as ordered
  • Psychotherapy was provided as needed
  • Emphasised regular follow-up and explained about the importance of continuing medication.


Evaluation

Patient and family were cognizant of the need for follow-up and medication and expressed understanding about continuation of medication. After surgery the symptoms of depression reduced with medication.


  Discussion Top


Pituitary adenomas are the most common type of pituitary disorder. While prolactinoma and non-functioning pituitary adenomas are the most common pituitary tumour, ACTH-secreting tumours are relatively rare.[10],[11] ACTH-secreting tumour arises from corticotroph cells from pituitary gland and generally presents as Cushing's disease.[12] ACTH-secreting pituitary adenomas occur more frequently in females compared with males (female-to-male ratio is 3:1).[13],[14]

Cushing's disease is characterised by weight gain (central obesity), diabetes, hypertension, moon facies, facial plethora, psychiatric and neurocognitive changes, decreased libido, and osteoporosis. Cushing disease, if not effectively treated, results in morbidity associated with cardiac, cerebrovascular, immunosuppressive, osteoporosis, psychiatric disturbances, and diabetic events.[15] In present case, the patient also suffered with steady weight gain, moon facies, facial plethora and various psychiatric disturbances such as depression, anxiety and lack of concentration. She had also developed secondary diabetes mellitus. Though there were plethora of symptoms and physical signs, the case was not diagnosed until present hospitalisation. The onset was very slow and symptoms developed over period of one year. It was only when all these symptoms became more intense and evident, major endocrine imbalance was suspected and pituitary macroadenoma was diagnosed. All her recent health problems of hyperacidity, frequent infections, irregular menstrual periods, low back ache and mental disturbances was then attributed to the current diagnosis.

Diagnosis of Cushing's disease depends on identification of loss of diurnal variation of ACTH and cortisol, increased serum cortisol and ACTH levels and evaluating damaged negative feedback mechanism involved in the pituitary-adrenal axis. Imaging investigations to confirm the location are also very essential in the diagnosis of Cushing's disease. Secondary to steroid hormone administration, iatrogenic Cushing's syndrome should be taken into consideration and excluded.[16] Elevated ACTH can rule out an adrenocortical tumour. High-dose dexamethasone suppression and corticotropin-releasing-hormone stimulation tests may be used to differentiate between pituitary and ectopic sources of ACTH hypersecretion, but both tests have false-negative response rates of 10%.[17]

In spite of clinical picture being consistent with Cushing's disease in present case, the serum cortisol level (both at 8am and 4 pm) and serum ACTH level were normal. Sometimes the repetition of the tests for several times is also recommended to identify abnormality.[18] Though dexamethasone suppression test and urinary free cortisol was ordered by endocrinologist in the present case, it was not carried out as decision for surgical excision was already made. In pre-operative lab work up, the urine osmolality was found to be very low (63.23 miliosmoles/Kg of H2O) which is a classic finding in DI. The large pituitary tumours may compress the posterior part of pituitary which may result in decreased secretion of antidiuretic hormone. This may result in temporary or permanent DI. Surgical resection is the treatment of choice in ACTH-secreting pituitary adenoma. Pituitary surgery generally results in biochemical remission in the majority of cases, with rates of remission at experienced centres >80%.[19] In present case, patient's biochemical values and physical changes returned to normal over 8 to 12 weeks post-surgery. MRI was repeated after 9 months of surgery which appeared normal with empty sella with no enhancing mass and unremarkable infundibular stalk.

DI is one of the most common post-operative complications among patients undergoing pituitary surgery other than infection and post-surgical haemorrhage. Post-operative DI is generally transient and benign in the majority of cases. But permanent DI, which is due to damage to the hypothalamus or proximal infundibulum may also occur in some patients.[20] Risk of developing DI is associated with presence of Cushing's disease, intraoperative CSF leak, microadenoma, tumour volume, preoperative to post-operative serum sodium change and tumour location.[21],[22],[23],[24] Patient in the present study had also developed DI preoperatively and remained unchanged post-operatively even after one year. The patient was advised to continue Minirin nasal spray twice a day for the same.


  Conclusion Top


ACTH-secreting pituitary adenoma can lead to spectrum of physical manifestations along with mental disturbances and cognitive difficulties. It is important to diagnose pituitary adenoma as early as possible so that it can be treated promptly to ensure good prognosis and less complications. Prolonged hypercortisolism is not only associated with an increased risk of various cardiovascular and metabolic disturbances, but also can cause life threatening complications if not treated on time. The goal of treatment for macroadenoma is complete excision. If complete excision is not possible, reducing tumour mass, restoring hormone function, restoring normal vision are attempted using medication, surgery or radiation. Surgical interventions are often required for cure. Prompt surgical excision drastically reduces the symptoms though in some cases it might take time.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Raappana A, Koivukangas J, Ebeling T, Pirilä T. Incidence of pituitary adenomas in Northern Finland in 1992-2007. J Clin Endocrinol Metab 2010;95:4268-75.  Back to cited text no. 13
    
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Gruppetta M, Mercieca C, Vassallo J. Prevalence and incidence of pituitary adenomas: A population based study in Malta. Pituitary 2013;16:545-53.  Back to cited text no. 14
    
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Abstract
Introduction
Case Report
Nursing Management
Nursing Care
Discussion
Conclusion
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