1.Single-stage adrenalectomy and hysterectomy for pheochromocytoma with giant uterine fibroid: A multidisciplinary perioperative challenge
Fei Bing Yong ; Sarojini Devi Simanchalam ; Hidayatil Alimi Keya Nordin ; Nithiya Devi Kandasami ; Sadhana Sadar Mahamad ; Suhaimi Jaafar ; Mohd Wajdi Zanuddin ; Poh Shean Wong ; Chin Voon Tong ; Noor Lita Adam ; Zanariah Hussein
Journal of the ASEAN Federation of Endocrine Societies 2026;41(S1):20-
Introduction:
Pheochromocytoma is a catecholamine-secreting adrenal
tumor associated with major perioperative hemodynamic
instability. When concurrent major pelvic pathology
requires surgery, operative planning becomes particularly
challenging. We describe the successful single-stage
management of pheochromocytoma and a giant uterine
fibroid, highlighting the importance of multidisciplinary
coordination and perioperative optimization.
Case:
A 49-year-old female with symptomatic uterine fibroid
was found to have proliferative endometrium on a pipelle
biopsy. Computed tomography (CT) abdomen incidentally
detected a right adrenal mass alongside a large posterior
uterine fibroid (11.9 × 17.4 × 14.6 cm). CT adrenal protocol
demonstrated a heterogeneously enhancing right adrenal
mass (6.9 × 6.9 × 9.6 cm) with high unenhanced attenuation.
Biochemical evaluation revealed markedly elevated
24-hour urinary metanephrine (4.7× upper limit) and
normetanephrine (2.4× upper limit).
Following multidisciplinary discussions, a single-stage
surgical approach was planned after careful assessment of
feasibility and perioperative risk in view of the uncertain malignant potential of the pelvic mass and to minimize
repeated exposure to anesthesia. Preoperative optimization
included transitioning from terazosin to phenoxybenzamine, with subsequent addition of bisoprolol for
hemodynamic control. The operative strategy prioritized
pheochromocytoma resection first, given its potential
for significant hemodynamic instability. Progression to
hysterectomy was contingent upon achieving adequate
intraoperative hemodynamic stability following adrenalectomy, with continuous reassessment by the anesthetic
and surgical teams.
Right adrenalectomy was performed first, followed by
total abdominal hysterectomy with bilateral salpingooophorectomy. Significant hemodynamic lability occurred
during tumor manipulation, with hypertensive surges
managed using sodium nitroprusside and remifentanil
infusions. Following adrenal vein ligation and tumor
removal, hypotension was managed with noradrenaline
and additional adrenaline support as required. Total
operative time was approximately 6 hours. Postoperatively,
transient noradrenaline support was required but was
rapidly weaned as hemodynamic stability was achieved.
Conclusion
Single-stage adrenalectomy and major pelvic surgery
can be safely performed in selected patients with pheochromocytoma when guided by meticulous preoperative
optimization, clear intraoperative sequencing, and close
multidisciplinary coordination.
Pheochromocytoma
;
Adrenalectomy
;
Leiomyoma
;
Hysterectomy
2.RESPIRATORY FAILURE ASSOCIATED WITH DISTAL RENAL TUBULAR ACIDOSIS IN AN OLDER ADULT WITH SOUTHEAST ASIAN OVALOCYTOSIS AND CONCOMITANT THYROTOXIC ATRIAL FIBRILLATION
Hoe Leong Sii ; Sin Hui Ng ; Kit Hou Chiang ; Voon Fei Wong
Journal of University of Malaya Medical Centre 2023;26(1):1-4
Distal renal tubular acidosis (dRTA) is characterized by hyperchloremic hypokalemic metabolic acidosis, nephrocalcinosis and metabolic bone disease. It has been associated with Southeast Asian Ovalocytosis (SAO), particularly in malaria endemic region. We present a case of an older adult with dRTA who presented with respiratory failure as a result of hyperchloremic normal anion gap metabolic acidosis, in association with nephrocalcinosis and SAO, however, without hypokalaemia due to concurrent acute kidney injury. Oxygen therapy was able to be weaned off after treatment with alkali supplementation. Distal RTA may be associated with SAO, and early recognition and treatment of dRTA is paramount in preventing complications such as chronic kidney disease, metabolic bone disease and life-threatening respiratory arrest.
Acidosis, Renal Tubular


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