68 Ga-DOTANOC PET/CT in 2 Siblings With Von Hippel-Lindau Disease.

ABSTRACT: Von Hippel-Lindau disease is a rare multisystem disorder that shows autosomal dominant inheritance. It is a cancer syndrome that is characterized by the development of a variety of benign and malignant tumors-CNS hemangioblastomas, retinal angiomas, endolymphatic sac tumors, renal cysts and tumors, pancreatic cysts and tumors, adrenal pheochromocytomas, and epididymal cystadenomas. Here we present the 68 Ga-labeled DOTANOC scans of 2 siblings who show an interesting spectrum of findings consistent with Von Hippel-Lindau disease.

Pulmonary Mucoepidermoid Carcinoma Mimicking Carcinoid Lung on 18 F-FDG and 68 Ga-DOTANOC PET/CT.

ABSTRACT: Pulmonary mucoepidermoid carcinoma (PMEC) is a rare pulmonary neoplasm. Although 18 F-FDG PET/CT has been shown to present with increased metabolic activity in PMEC, literature does not report increased somatostatin receptor expression in these tumors. We present the case of a 15-year-old boy where PMEC mimicked a typical carcinoid of the lung on DOTANOC PET/CT by showing significant uptake on 68 Ga-DOTANOC.

Role of FDG PET/CT in definitive and presumed autoimmune encephalitis.

OBJECTIVE: F-18 Fluorodeoxyglucose PET/CT (FDG-PET) is emerging as a useful imaging adjunct to MRI in the initial diagnostic evaluation of autoimmune encephalitis (AIE)-though presently it is not included in the diagnostic criteria. MATERIALS AND METHODS: In this prospective study we enrolled a total of 52 patients with clinically diagnosed and treated AIE. MRI evaluation was done in each case along with CSF and EEG where feasible. FDG-PET was done for all and images were interpreted visually and using SPM. RESULTS: The mean age group of patients included was 38.5 ± 22.6 years with 31 females and 21 males. 23 antibody-positive cases underwent PET, the most common antibody detected was anti-NMDAR type followed by anti-LGI 1. Most common metabolic pattern in NMDARE was hypermetabolism in basal ganglia and hypometabolism in parieto-occipital cortices and ovarian teratoma was detected in two of these patients on whole-body PET. A metabolic pattern consistent with AIE was demonstrated in 22/29 (75.8%) antibody-negative patients with hypermetabolism in basal ganglia and mesial temporal cortices. The overall sensitivity of FDG PET was 86% (45/52). MRI abnormalities were detected in 22/52 (42%) cases, 10/23 antibody positive and 12/29 antibody negative cases. PET was positive in 23/30 (76%) MRI negative cases. CONCLUSION: Sensitivity of FDG PET for supporting a diagnosis of AIE was higher compared to MRI in both antibody-positive (definitive) and antibody-negative (presumed) AIE. Specific metabolic patterns can be demonstrated on FDG PET in AIE, prompting an early diagnosis so that timely treatment can be instituted.

Utility of Simple and Non-Invasive Strategies Alternative to Inferior Petrosal Sinus Sampling and Peripheral CRH Stimulation in Differential Diagnosis of ACTH-Dependent Cushing Syndrome.

We aimed to evaluate the utility of simple, cost-effective, and non-invasive strategies alternative to BIPSS and peripheral CRH stimulation in differential diagnosis of ACTH-dependent CS. First, we performed ROC analysis to evaluate the performance of various tests for differential diagnosis of ACTH-dependent CS in our cohort (CD, n=76 and EAS, n=23) and derived their optimal cut-offs. Subsequently, combining various demographic (gender), clinical (hypokalemia), biochemical (plasma ACTH, HDDST, peripheral CRH stimulation) and imaging (MRI pituitary) parameters, we derived non-invasive models with 100% PPV for CD. Patients with pituitary macroadenoma (n=14) were excluded from the analysis involving non-invasive models. Relative percent ACTH (AUC: 0.933) and cortisol (AUC: 0.975) increase on peripheral CRH stimulation demonstrated excellent accuracy in discriminating CD from EAS. Best cut-offs for CD were plasma ACTH<97.3 pg/ml, HDDST≥57% cortisol suppression, CRH stimulation≥77% ACTH increase and≥11% cortisol increase. We derived six models that provided 100% PPV for CD and precluded the need for BIPPS in 35/85 (41.2%) patients with ACTH-dependent CS and no macroadenoma (in whom BIPSS would have otherwise been recommended). The first three models included basic parameters and avoided both peripheral CRH stimulation and BIPSS in 19 (22.4%) patients, while the next three models included peripheral CRH stimulation and avoided BIPSS in another 16 (18.8%) patients. Using simple and non-invasive alternative strategies, BIPSS can be avoided in 41% and peripheral CRH stimulation in 22% of patients with ACTH-dependent CS and no macroadenoma; such patients can be directly referred for a pituitary surgery.

99mTechnetium-Ubiquicidin Scan with Single-Photon Emission Computed Tomography/Computed Tomography in Skull Base Osteomyelitis.

Malignant otitis externa (MOE) with skull base osteomyelitis (SBO) is an aggressive infection that predominantly affects elderly, diabetic, or immunocompromised patients, and is associated with high disease-specific mortality. Pseudomonas aeruginosa is the most isolated microorganism. External otitis associated with granulation tissue and pain is the most common presenting feature; a biopsy is obtained to rule out malignancy. A proper consensus is lacking regarding the best imaging modality for early initial diagnosis and follow-up. 99mTechnetium (99mTc)-labeled ubiquicidin (UBI) 29-41 is a bacterial attaching peptide that does not bind to activated leukocytes. We report a case of SBO initially misdiagnosed as a chronic otitis media, but later proved to be a case of MOE. 99mTc methylene diphosphonate bone scan and 99mTc-UBI 29-41 scan with single-photon emission computed tomography/computed tomography scans were performed to corroborate the clinical diagnosis. SBO remains a great challenge due to its increasing prevalence and high morbidity are difficult to diagnose and are often confused with cholesteatoma and neoplastic process. The UBI scan could be an auxiliary noninvasive diagnostic alternative in early diagnosis.

Comparison of Neck Ultrasonography, Dual Phase 99mTc-Sestamibi with early SPECT-CT & 18F-Fluorocholine PET-CT as First Line Imaging in Patients with Primary Hyperparathyroidism.

Introduction: Successful surgical treatment for primary hyperparathyroidism requires accurate localization of abnormal parathyroid tissue in terms of location and number. Imaging is important for localizing the parathyroid adenoma, and there has been significant interest in 18F-fluorocholine (FCH) positron emission tomography/computed tomography (PET/CT) for this purpose. Aim: This study attempted to ascertain the utility of 18F-FCH PET/CT as a first-line investigation in preoperative localization of abnormal parathyroid tissue in primary hyperparathyroidism, in comparison with 99mTc-sestamibi dual-phase scintigraphy with early single-photon emission computed tomography (SPECT)/CT and neck ultrasonography. Materials and Methods: Fifty-five patients with biochemical features of primary hyperparathyroidism were enrolled in this study. They underwent neck ultrasonography, 99mTc-sestamibi dual-phase scintigraphy with early SPECT/CT, and 18F-FCH PET/CT for localization of parathyroid lesions. Thirty-three patients underwent surgical resection of the detected lesions. For two patients, clinical and biochemical follow-up was used as a gold standard. Results: A total of 40 lesions were resected in the 33 patients who underwent surgery. A further two lesions were localized in two patients with clinical and biochemical follow-up as the gold standard. Of these 42 lesions, 41 were detected in preoperative imaging and 1 lesion was noted intraoperatively and resected. 41/42 lesions were detected by 18F-FCH PET/CT (detection rate: 97.6%), 33/42 by 99mTc-sestamibi dual-phase scintigraphy with early SPECT/CT (detection rate: 78.5%), and 30/42 by neck ultrasonography (detection rate: 71.4%). Conclusion: Detection rates on 18F-FCH PET/CT were superior to both 99mTc-sestamibi dual-phase scintigraphy with early SPECT/CT and neck ultrasonography in preoperative localization of parathyroid lesions in patients with primary hyperparathyroidism.

Macrofollicular Architecture in Invasive Encapsulated Follicular Variant of Papillary Thyroid Carcinoma: A Pitfall in Thyroid Practice.

BACKGROUND: Predominantly macrofollicular architecture in invasive encapsulated follicular variant of papillary thyroid carcinoma (IEFVPTC-MF) is rare and often a cause of misinterpretation during pre-operative work-up and histopathology evaluation. We comprehensively evaluated the radiological, cytological, gross, microscopic, molecular and follow-up characteristics of four such cases, intending to increase its recognition and add our experience to the limited literature available. METHODS: All such histopathologically-proven cases of IEFVPTC-MF were retrieved from the departmental archives. The clinical details, thyroid ultrasound, cytology and thyroid scan findings were reviewed. Allele-specific PCR for BRAF p.V600E, KRAS, NRAS, and HRAS mutations, and FISH assays for ETV6::NTRK3 fusion and RET fusions were performed. RESULTS: There were four cases of IEFVPTC-MF diagnosed between 2021 and 2022, involving two males and two females. The median age at presentation was 27 years, and the duration of the disease was 1-10 years. Thyroid ultrasound was TR1 (benign; n = 1), TR2 (not suspicious; n = 2), or TR4 (moderately suspicious; n = 1). Cytology was categorized as nondiagnostic (n = 1), benign (n = 1), and atypia of undetermined significance (n = 1). The three nodules with available cytology smears showed abundant colloid. Cells were arranged as sheets/microfollicles/clusters. Nuclei were predominantly round with minimal/focal elongation, membrane irregularity, and cellular crowding. On gross examination, cut surfaces of the tumors showed variable amounts of colloid. The tumors were solid-cystic. Histopathology revealed partially encapsulated multinodular tumors. There were prominent pseudopapillae projecting into the lumina of macrofollicles. Nuclei were predominantly round with variable nuclear atypia, including chromatin clearing and multifocal presence of nuclear grooves. Pseudoinclusions were identified in two. Molecular analysis revealed NRAS codon 61 mutation and ETV6::NTRK3 fusion in one case each. Two patients had cervical lymph node and hematogenous metastases. Post-radio-active iodine, the response was structurally incomplete (n = 2), indeterminate (n = 1) and excellent (n = 1). CONCLUSIONS: Macrofollicular architecture in invasive encapsulated follicular variant of papillary thyroid carcinoma is a major pitfall in thyroid oncology practice. Long-standing disease, and ultrasonographic and cytological features that overlap with benign disease, often lead to underdiagnosis during pre-operative evaluation. As patients may consequently develop distant metastases and have inadequate treatment response, there is a need for more vigilant understanding of the spectrum of macrofollicular thyroid disease for accurate diagnosis. ETV6::NTRK3 or other fusions, when found, present opportunities for targeted therapy.

Long-term survival outcomes of salvage [225Ac]Ac-PSMA-617 targeted alpha therapy in patients with PSMA-expressing end-stage metastatic castration-resistant prostate cancer: a real-world study.

PURPOSE: Despite the existence of various treatment options, the prognosis for patients with metastatic castration-resistant prostate cancer (mCRPC) remains unfavorable. One potential therapeutic approach is the use of [225Ac]Ac-PSMA-617, a targeted alpha therapy (TAT) that administers alpha-particle radiation specifically to prostate cancer cells expressing PSMA. In this study, we report the long-term survival outcomes of this novel therapy in a series of patients with mCRPC who have exhausted all standard treatment options. METHODS: The study enrolled patients with mCRPC who had shown resistance to standard lines of therapies, including next-generation anti-androgen therapies and taxane-based chemotherapies. These eligible patients received treatment with [225Ac]Ac-PSMA-617 at 100-150 kBq/kg doses administered every 8 weeks. The primary objective of the study was to assess overall survival (OS), while secondary objectives included evaluating radiological progression-free survival (rPFS), monitoring serum prostate-specific antigen (PSA) levels as a measure of biochemical response, and assessing adverse events using the CTCAE v5.0 grading system. RESULTS: Among the 63 initially enrolled patients, a total of 56 patients who had completed at least two cycles of [225Ac]Ac-PSMA-617 were included in this study. The mean age was 67 years (range, 39-87) and patients received a total of 204 cycles of [225Ac]Ac-PSMA-617 TAT. 91% of patients exhibited any PSA decline, with 67.8% experiencing a decline of 50% or more. The median follow-up was of 22 months (range: 6-59 months). Imaging-based disease progression was observed in 68% of patients, and 66% of patients succumbed to the disease. The median OS was 15 months (95% CI: 10-19). In univariate analysis, factors such as lack of >50% PSA decline (P=0.031), Eastern Cooperative Oncology Group (ECOG) performance status of 2 or higher (P=0.048), and radiological progression (rPD) (P<0.001) were found to be predictors of poor OS. However, in multivariate analysis, only rPD emerged as an independent prognostic factor with a hazard ratio (HR) of 8.264 (95% CI: 1.429-16.497, P=0.004). The estimated median rPFS was 9 months (95% CI: 7-15). Moreover, patients who demonstrated any PSA decline had a median rPFS of 10 months compared to only 3 months in patients without any PSA decline (multivariate HR: 6.749; 95% CI: 1.949-23.370; P=0.002). Fatigue was one of the most common treatment-emergent adverse events, with grades 1/2 occurring in 70% of patients and grades 3 or higher in 3.5% of patients. This fatigue was transient and resolved before the next treatment cycle. Additionally, approximately one-third of patients experienced xerostomia (grades 1/2: 32.1%). CONCLUSION: [225Ac]Ac-PSMA-617 targeted alpha therapy, was found to be well-tolerated with acceptable adverse events and effective in the treatment of patients with end-stage mCRPC.

Lincoln Sign in a Case of Primary Hyperparathyroidism on 18 F-Fluorocholine PET/CT.

ABSTRACT: Lincoln sign or black beard sign is one of the signs that have been classically described on bone scintigraphy in monostotic Paget disease, when mandible is involved. Extensive involvement of the mandible causes increased radiotracer uptake from one mandibular condyle to the other, resembling a black beard. We present the case of a 14-year-old girl with primary hyperparathyroidism who underwent an 18 F-fluorocholine PE/CT to locate the parathyroid adenoma. MIP image of the PET/CT incidentally showed black beard sign due to increased radiotracer uptake in the mandible.

Malignancy-Associated Sarcoid-Like Reaction in a Case of Triple-Negative Breast Cancer on F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography.

We describe the case of a 54-year-old woman with triple-negative breast cancer whose baseline F-18 fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) showed hypermetabolic left breast primary, ipsilateral axillary lymphadenopathy, lung nodules, and mediastinal lymph nodes. Histopathological examination of tissue from mediastinal lymph nodes confirmed a diagnosis of sarcoid-like reaction. Chemotherapy may induce or cause a flare-up of malignancy-associated sarcoid-like reaction. However, in our patient's post-chemotherapy F-18 FDG PET/CT, there was reduction in size and uptake of the mediastinal lymph nodes along with partial response shown by the other lesions. We aim to describe this rare course of malignancy-associated sarcoid-like reaction and highlight the role of F-18 FDG PET-CT in such cases.

Extensive Metastatic Vascular Calcification in a Patient with Chronic Renal Failure and Tubercular Osteomyelitis as Seen on F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography.

A 48-year-old male with known tubercular osteomyelitis of the left elbow and chronic renal failure presented with PTH independent hypercalcemia and underwent F-18 fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) to look for any underlying malignancy that might be causing his hypercalcemia. The PET/CT did not reveal any malignancy, but extensive metastatic calcification of small- and medium-sized arteries was noted throughout the body with relative sparing of large vessels. Alkaline tissue such as lungs, gastric mucosa, and kidneys that are usually involved in metastatic calcification were also spared. The underlying pathology for this kind of metastatic calcification was most likely chronic granulomatous disease, which was tubercular osteomyelitis in this patient. We present the PET/CT scan images of this unusual case of metastatic vascular calcification.

Tc-99 m Ubiquicidin Imaging in Orbital Aspergilloma: an Illustration.

Aspergillus infection is relatively rare disease, and we present a case of orbital aspergillus infection who presented with right orbital pain and swelling. Right orbital lesion was identified on CT, MRI, and PET-CT imaging followed by confirmation of aspergillus on histopathological examination. We demonstrate that Tc-99 m ubiquicidin scan can yield positive results in aspergillosis too, enabling its differentiation from non-infective pathologies.

Imaging characteristics of phosphaturic mesenchymal tumors.

BACKGROUND: Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome associated with phosphaturic mesenchymal tumors (PMTs). Localization of the causative tumor in these cases is an arduous task since the culprit lesions are usually small, slow-growing, and can be located almost anywhere from head to toe. PURPOSE: To describe the morphological characteristics of histologically proven PMTs on various radiological modalities. MATERIAL AND METHODS: After institutional ethical approval, this retrospective study analyzed 20 cases with a histopathological evidence of PMT. Various imaging characteristics of the tumors on available computed tomography (CT) and magnetic resonance imaging (MRI) scans were evaluated. Descriptive statistical analyses were conducted. RESULTS: The tumors were located in diverse locations: lower extremities (n = 10); head and neck (n = 5); vertebral column (n = 3); pelvis (n = 1); and upper extremities (n = 1). Bone lesions seen on CT had variable morphology: sclerotic (n = 3/8, 37.5%); lytic (n = 3/8, 37.5%), and both lytic and sclerotic (n = 2/8, 25%) with presence of narrow zone of transition in all cases (n = 8/8) and amorphous internal matrix calcifications in 25% of cases (n = 2/8). Of the tumors, 68.4% (n = 13/19) were hypointense on T1 and all of them showed hyperintense signal on T2-weighted and STIR images (n = 19/19) and contrast enhancement (n = 16/16). Of the tumors, 66.7% (n = 6/9) showed restricted diffusion. DOTANOC PET/CT showed tumor uptake in all cases (n = 8/8). CONCLUSION: PMTs may have variable and non-specific tumor appearances on various imaging modalities. However, in an appropriate clinical scenario and a background of suggestive biochemical work-up, the radiologist should keep a high index of suspicion.

Impact of Fluoro-Choline PET/CT in Reduction in Failed Parathyroid Localization in Primary Hyperparathyroidism.

INTRODUCTION: Accurate localization of the pathological parathyroid gland is a prerequisite for minimally invasive surgical management of hyperparathyroidism (HPT). Poor imaging or discordance in odd situations like ectopic adenomas, parathyroid hyperplasia, syndromic HPT results in localization dilemma thus causing failed parathyroidectomy. We studied the impact of Fluoro-Choline (FCH) PET/CT imaging in reduction in localization failure of parathyroid adenoma. MATERIALS AND METHODS: We did a retrospective observational study (2018-2021) of HPT among which 97 patients underwent focused parathyroidectomy (FP). All patients had undergone ultrasound imaging and 99mTc-sestaMIBI scan with early SPECT/CT (MIBI). When this preliminary imaging was doubtful or negative or multiple lesions were expected, FCH PET/CT was performed. We compared the localization accuracy of MIBI scan and FCH PET/CT with surgical outcomes as reference standard. RESULTS: MIBI scan showed overall lesion detection rate (LDR) of 88.65% in localization of pathological parathyroid gland in 97 patients. The addition of FCH PET/CT improved the overall lesion detection to 97.9%. The overall possible localization failure was reduced from 11.34 to 2.06% with the addition of FCH PET/CT (p < 0.05). Out of 97 patients of FP, 87 patients showed features of parathyroid adenoma. Single hyperplastic gland was seen in 7 patients, lipoadenoma was seen in 1 patient and 1 patient had features suggestive of parathyroiditis on histopathology. FCH PET/CT was a useful adjunct and showed significant reduction in localization failure of parathyroid adenoma.

Clinical profile and treatment outcomes of patients with ectopic ACTH syndrome compared to Cushing disease: a single-center experience.

PURPOSE: We aimed to evaluate and compare the clinical, biochemical and radiological profile and outcomes of patients with ectopic ACTH syndrome (EAS) and Cushing disease (CD) treated over a period of 10 years (2013-2022). METHODS: In this ambispective observational study, we collected data for 146 patients with ACTH-dependent CS (EAS, n = 23; CD, n = 94; occult ACTH source, n = 29). Relevant details were filled in a predesigned proforma and outcomes were ascertained at the most recent visit. RESULTS: EAS was more common in males (65.2 vs. 27.6%, p < 0.001). Patients with EAS had a shorter duration of symptoms [12 (6-12) vs. 31.5 (15-48) months, p < 0.001] and were more likely to have hypokalemia (82.6 vs. 21.0%, p = 0.001), pedal edema (65.2 vs. 34.2%, p = 0.015), weight loss (34.8 vs. 4.0%, p < 0.001) and systemic infection (30.4 vs. 6.5%, p = 0.006). They also had significantly higher 8 a.m. serum cortisol, midnight serum and salivary cortisol and 8 a.m. plasma ACTH levels. Bronchial carcinoid (n = 10, 43.5%) was the most common etiology of EAS. Bilateral adrenalectomy was performed in 11 (47.8%) patients with EAS. Eight patients (34.8%) with EAS died at the last follow-up, of whom 7 (87.5%) had metastatic disease. In CD group, overall remission rate was 69.4% (56.1%, early and 13.3%, delayed) and 26.3% of patients with an initial remission had recurrence. CONCLUSIONS: Bronchial carcinoid was the most common cause of EAS in our cohort. Bilateral adrenalectomy was performed in approximately every 1 in 2 patients with EAS and approximately every 1 in 3 patients expired till the last follow-up.

Radioiodine Therapy in Pediatric Differentiated Thyroid Cancer: Dosimetry, Clinical Care, and Future Challenges.

ABSTRACT: Thyroid cancer is very rare in children. 131 I therapy after thyroidectomy is established in pediatric differentiated thyroid cancer (DTC). Pediatric DTC guideline is silent on the optimum amount of 131 I that could be safely and effectively administered to children who are more radiosensitive. Like adult DTC, children are also given 131 I therapy empirically based either on age or body weight. Pediatric DTC guideline recommends that patient-specific dosimetry is important in children. Still, due to the low incidence rate and the practical difficulties of dosimetry, it has neither been established nor adopted in routine practice. This review article aims to discuss current approaches of 131 I therapy in children and young adult patients with DTC and dosimetric data obtained by several investigators. Efforts are required to simplify dosimetric procedures and precise results, especially in determining lesion size. We prefer 3-dimensional dosimetry over planar dosimetry, where lesion size could be measured accurately. 124 I PET/CT-based dosimetry is expected to give accurate dosimetric results. The most challenging aspect is that no randomized controlled trials are available to compare the empiric 131 I therapy results versus dosimetry-based treatment outcomes in children and young adults. Suppose dosimetry-based 131 I therapy could be shown to have better outcomes, namely, successful ablation rate, better disease-free survival, and lesser treatment-emergent adverse events than empirical 131 I treatment. In that case, one can argue in favor of the former. Unfortunately, no convincing study is currently available. Thus, there is a need for a randomized control trial to settle this issue.

Tc-99m Ethambutol Scintigraphy with Single-Photon Emission Computed Tomography in Diagnosis of Tubercular Iliopsoas Abscess.

Very few imaging techniques can demonstrate the presence of Mycobacterium tuberculosis in vivo. Technitium-99m Ethambutol scintigraphy is one of them, although literature on the utility of this technique is scarce. We describe a patient with iliopsoas abscess showing increased uptake on Tc-99 m ethambutol scintigraphy which was later confirmed to be tubercular by the microbiological analysis of pus.

Serendipitous Diagnosis of Tertiary Hyperparathyroidism in a Case Purportedly thought to have Skeletal Metastases.

Tertiary hyperparathyroidism (THPT) is characterized by over secretion of parathyroid hormone caused by long-standing secondary hyperparathyroidism. THPT can affect the bones as well as cause extraskeletal calcifications. The bony lesions often mimic multiple skeletal metastases or multiple myeloma. We report a case of a 48-year-old man with chronic kidney disease on dialysis, who presented with chief complaints of low back ache and swelling over the left clavicle. In view of clinical suspicion of malignancy with bony metastases, he underwent 18F-fluorodeoxyglucose positron emission tomography/computed tomography and was subsequently found to have parathyroid adenomas, which were confirmed on 99mTc-methoxy-isobutyl-isonitrile scintigraphy.

Late Local and Distant Recurrence of Apparently Benign Paraganglioma.

Paraganglioma-pheochromocytoma (PPGLs) are relatively rare catecholamine-secreting tumors of chromaffin origin. Due to the sympathetic effects of catecholamine excess, their presentation may range from non-specific symptoms to dangerous hypertensive crises. We present the case of a 36-year-old lady with recurrent paraganglioma (PGL) who presented in emergency with hypertensive crisis. She had a history of surgery for left-sided PGL 18 years earlier. Imaging showed local recurrence with pulmonary metastases and blood biochemistry showed raised urinary metanephrines. In view of her poor general condition, we undertook a staged surgical approach for management. She first underwent en-bloc excision of recurrent PGL with left nephrectomy. Nine weeks later, she underwent a pulmonary metastasectomy. This staged surgical approach resulted in the stabilization of blood pressure and normalization of urinary catecholamine. Although most of these tumors are indolent by nature, this case highlights the metastatic potential of apparently benign PGL. This case explores the possibility of a staged surgical approach in a high-risk patient and emphasizes the need for long-term follow-up in these cases.