CANCER - Tadtad, Izah - 2A

TADTAD, IZAH KYLENE O.
MLS 2A
CYTOGENETICS 12-12-2022
__________________________________________________________________________
A Case Report of Adrenal Pheochromocytoma
Mrs. Afroza, a 21 years old housewife, was admitted into FSW-II of FMCH with the
complaints of paroxysmal attacks of palpitation, dizziness, blurring of vision, and headache for
the last six (6) months. Each attack persists for a few minutes to half an hour and occurs
irregularly within two or three days to 2 to 3 times a day. On examination, the patient had no
abnormal physical findings except Blood Pressure (BP) is high during paroxysmal attack
(Sylostic BP varies from 140 to 210 mmHg and diastolic BP varies from 100 to 140 mmHg).
Blood Count, Random Blood Sugar, Blood Urea, Chest X-rays, and ECG reports were within
normal amount.
A provisional diagnosis of secondary hypertension due to adrenal

pheochromocytoma was made and further biochemical investigations (abdominal USG, 24-
hour urinary catecholamine and metanephrine level) confirmed the diagnosis. CT (Computed
Tomography) scan of abdomen showed right sided adrenal mass of a size about 6cm x 10 cm.
Surgical removal of right adrenal gland (Rt. Adrenalectomy) was planned. Patient was
prepared for surgery with collaboration with Cardiologist and Anesthesiologist. Patient was
given Phenoxybenzamine (α blocker) for 10 days preoperatively, starting with 10 mg 12
hourly and gradually increasing up to 30 mg 12 hourly and BP decreases to normal level.
Patient was also given Propanolol (β blocker) 10 mg three times daily starting from 11th day
or preoperative preparation for five (5) days.
Operation was done on 16th day of preoperative preparation and last dose of drugs
were given on the morning of the day of surgery. Open right adrenalectomy was done by right
subcostal incision. Adrenal vein was ligated first and tumour was removed. Operative
procedure was uneventful except marked fluctuation in BP. BP increased up to a level of
260/150 mmHg during handling of tumour and fell to a non recordable BP following ligation of
adrenal vein. The anesthesiologist team managed the situation using preoperative IV fluid
overload, intravenous phentolamine and intravenous esmolol. A sudden fall of blood pressure
following ligation of adrenal vein was managed by rapid infusion of large volumes of fluid and
intravenous ephedrine. Postoperative recovery was uneventful and patient was discharged on
8th Post Operative Day. Blood pressure becomes normal (Systolic 110 to 90 mmHg and
diastolic 80 to 60 mmHg) from 1st POD without any drug. Histopathology report further
confirmed the adrenal tumour was pheochromocytoma.
DISCUSSION
Pheochromocytoma is a rare, usually benign tumor that develops in the adrenal

glands, usually as a cause of secondary hypertension originating from the chromaffin cells of
the adrenal glands. It reportedly occurs in 0.05% to 0.2% of hypertensive individuals. Globally,
the prevalence of the disease is estimated to be at 1 to 8 per million cases, but may occur
frequently in certain populations (Walther et.al, 1999). According to a Dutch study by Berends
et al (2018), there was an increase in the age-standardized incidence rate (ASR) of
pheochromocytomas and sympathetic paragangliomas in the Netherlands between 1995 and
2015. It was reported that between 1995 and 1999, the ASR was 0.29 pero 100,000 persons.
In comparison, 2011 and 2015 saw an increase with 0.46 per 100,000 persons. In the
Philippines, a hospital census from the 1980’s showed that of 101 cases of secondary
hypertension, only four (4) were found to have pheochromocytoma. Moreover, the disease’s
peak incidence is from the third to fifth decades of life. Approximately 10% occur in children
with 50% of cases being solitary intra-adrenal lesions, 25% present bilaterally, and 25% are
extra-adrenal.
Most cases of pheochromocytoma have unknown causes for they usually occur
randomly. Approximately, 35% of phechomocytoma cases are a result of genetic disruptions
or mutations of certain genes. It may be caused by mutations in one of at least ten different
genes, which include the RET gene, the VHL gene, the neurofibromatosis (NF1) gene, the
succinate dehydrogenase subunit genes (SDA, SDHB, SDHC, SDHD, SDHAF2), the
TMEM127 gene (specifically on chromosome 2q11), and the MAX gene on chromosome
14q23. These mutations are inherited in an autosomal dominant pattern with almost 30% of
cases reported to be hereditary. This inherited form of pheochromocytoma is more likely to be
malignant (cancerous) than those that appear randomly. Furthermore, some cases of
pheochromocytoma occur alongside a genetic condition. It has been a secondary finding to
several diseases such as von Hippel-Lindau syndrome, endocrine neoplasia types IIA and IIB,
paraganglioma type 1 to type 5, neurofibromatosis I, and isolated familial pheochromocytoma.
The exact cause of the tumor is still unknown and no avoidable risk factors have been
found. As of recent studies, it is reported that the following factors may raise a person’s risk
for this type of tumor: a) inherited syndromes and gene changes, which lead to nearly 40%
of cases of pheochromocytoma; b) the age usually doesn’t play a major factor as the tumor
can develop at any age, however it is most prevalent among people aged 30 to 50; c) and
development of Carney triad in which the patient has paragangliomas, GISTs, and tumors in
the cartilage. This condition almost exclusively affects women. Morever, the case study
presented the patient exhibiting various symptoms such as dizziness, blurriness of vision,
headache, palpitations, and a dramatic increase in BP during paroxysmal attacks. These
clinical manifestations are a result of excessive catecholamine secretion by the tumour.
These secretions may occur either intermittently or continuously. Additionally, regulation for
catecholamine secretion in pheochromocytomas is different compared to secretions in a
healthy adrenal tissue. In particular, normal adrenal medulla secretions are composed roughly
of 85% epinephrine whereas most pheochromocytomas predominantly secret norepinephrine.
Furthermore, a study in 2016 suggest that catecholamine toxicity in pheochromocytoma may
be a factor for long-lasting myocardial changes in patients.
Diagnosis for pheochromocytoma typically begins with a confirmation of significant

catecholamine excess. A 24-hour urine test and a blood test will be requested first to
determine the levels of epinephrine, norepinephrine, and other substances in the body. The
blood test, specifically called Plasma metanephrine test, has a 90% sensitivity rate, but a
lower specificity. On the other hand, a 24-hour urine test is considered an ideal diagnostic
method because catecholamine release varies throughout the day. In this test, it is
considered positive if the catecholamine levels exceed twice the upper limit of normal.
Consequently, one or more imaging tests will be requested to locate a possible tumour.
These tests include a CT Scan, MRI Scan, M-iodobenzylguanidine (MIBG) Imaging, and
Positron Emission Tomography (PET). Healthcare providers may recommend some genetic
testing afterward in order to investigate if the disease is hereditary or not.
Figure 1. Histopathologic results Figure 2. USG showing right adrenal Figure 3. CT scan of
adrenal mass
Histopathologic findings in the case study showed alveolar or trabecular patterns of

polygonal or spindle-shaped cells in a rich vascular network. The USG and CT scan provided
a clear presentation of the right sided adrenal mass with a size of about 6 cm x 10 cm. After
diagnosis, various tests will be performed to determine the spread of the tumour. For this
condition, medical professionals use the TNM system to classify whether the tumour is
localized, regional, or metastatic. The primary treatment for pheochromocytoma is to
surgically remove the tumour. Blood pressure medications will be provided during the
preoperative period to block high-adrenaline hormones to lower the risk of dangerously high
BP during the operation. Once the disease is classified as malignant (cancerous), treatments
such as targeted therapies, chemotherapy, and radiation therapy are recommended. There
are no specific ways to prevent pheochromocytoma given its nature as a possibly hereditary
condition. Continuous research is still attempting to understand the true cause of this cancer
and as a medical technologist, it is our duty to support that movement by properly performing
the necessary tests--whether it be blood or urine--and aiding other medical-allied
professionals in reaching correct diagnoses and creating solutions and treatments that will
improve the patient’s health.
REFERENCES:
[01] Biswas, S., Alam, M., Rahman, M., & Islam, S.-U. (2011, April 8). A Case Report of
Adrenal Pheochromocytoma. ResearchGate; Bangladesh Journals Online.
[02] Blake, M. A. (2022, January 24). Pheochromocytoma: Practice Essentials,

Pathophysiology, Etiology. Medscape.com
[03] View of Terazosin as First Line Preoperative Blockade in Filipino Patients Diagnosed with
Pheochromocytoma | Journal of the ASEAN Federation of Endocrine Societies. (2022).
[04] Walther, M., Keiser, H. & Linehan, W. Pheochromocytoma: evaluation, diagnosis, and
treatment. World J Urol 17, 35–39 (1999). https://doi.org/10.1007/s003450050102
[05] Pheochromocytoma/Paraganglioma - NORD (National Organization for Rare Disorders).

(2021, October 19). NORD (National Organization for Rare Disorders); NORD.
[06] MacMillan, A. (2016, May 22). Pheochromocytoma. WebMD; WebMD.
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[08] Pheochromocytoma - Diagnosis and treatment - Mayo Clinic. (2022). Mayoclinic.org
[09] Garg, M., Kharb, S., Brar, K., Gundgurthi, A., & Mittal, R. (2011). Medical management of
pheochromocytoma: Role of the endocrinologist. Indian Journal of Endocrinology and
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[10] All About Pheochromocytoma | OncoLink. (2021, December 16). Oncolink.org.
[11] Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A

nationwide study and systematic review. (2018). Qxmd.com.
[12] Cañizares Hernandez, F., Sánchez, M., Alvarez, A., Díaz, J., Pascual, R., Pérez, M.,
Tovar, I., & Martínez, P. (2000). A Five-Year Report on Experience in the Detection of
Pheochromocytoma. Clinical Biochemistry, 33(8), 649
655.https://doi.org/10.1016/s0009-9120(00)00172-7
[13] Pheochromocytoma Is Characterized by Catecholamine-Mediated Myocarditis, Focal and

Diffuse Myocardial Fibrosis, and Myocardial Dysfunction. (2016). Qxmd.com.

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TADTAD, IZAH KYLENE O.

A Case Report of Adrenal Pheochromocytoma

A provisional diagnosis of secondary hypertension due to adrenal

Pheochromocytoma is a rare, usually benign tumor that develops in the adrenal

Diagnosis for pheochromocytoma typically begins with a confirmation of significant

Histopathologic findings in the case study showed alveolar or trabecular patterns of

[02] Blake, M. A. (2022, January 24). Pheochromocytoma: Practice Essentials,

[05] Pheochromocytoma/Paraganglioma - NORD (National Organization for Rare Disorders).

[06] MacMillan, A. (2016, May 22). Pheochromocytoma. WebMD; WebMD.

[08] Pheochromocytoma - Diagnosis and treatment - Mayo Clinic. (2022). Mayoclinic.org

[10] All About Pheochromocytoma | OncoLink. (2021, December 16). Oncolink.org.

[11] Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A

[13] Pheochromocytoma Is Characterized by Catecholamine-Mediated Myocarditis, Focal and

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