How best to dose octreotide for carcinoid crisis associated with neuroendocrine tumor?

Comment by InpharmD Researcher

Octreotide dosing for carcinoid crisis associated with neuroendocrine tumors varies based on clinical presentation and procedural context. For acute episodes, intravenous boluses of 100-500 mcg can be administered and repeated as needed, followed by continuous infusion at 50-300 mcg/h or higher in severe cases. The optimal dosing of octreotide to prevent carcinoid crisis remains unclear; however, prophylactic administration before surgery is common, with regimens such as 50-100 mcg/h IV infusions initiated 12 hours preoperatively and continued for 48 hours postoperatively. While evidence supporting specific dosing strategies remains limited, maintaining long-acting somatostatin analogs during the perioperative period and adjusting doses based on clinical response are typically standard practices. Higher doses may be required in patients with prior prolonged octreotide use.

Background

A 2017 consensus guideline from the European Neuroendocrine Tumor Society (ENETS) explored the optimal pre- and perioperative management of patients with neuroendocrine tumors (NETs), emphasizing the prevention of complications like carcinoid syndrome and carcinoid crisis. The optimal dosing of octreotide to prevent carcinoid crisis remains unclear. Intravenous (IV) octreotide reverses rapid crisis and is the cornerstone of prophylaxis, replacing older therapies. For patients on long-acting somatostatin analogs, these should be continued. No standard regimen exists, but for minor procedures, subcutaneous octreotide (100–200 mcg, 2–3 times/day) may suffice, with IV infusions available if needed. For major operations, subcutaneous octreotide (100 mcg, 3 times/day for 2 weeks) or IV octreotide starting at 50–100 mcg/h (mean 100–200 mcg/h) is commonly used, initiated 12 hours before surgery and continued for 48 hours postoperatively. Doses up to 500 mcg/h may be required for severe symptoms. Studies indicate lower complication rates with intraoperative octreotide, though data are limited by small sample sizes and inconsistent terminology. In atypical carcinoid syndrome, octreotide is recommended before and during surgery, with higher doses (100–200 mcg/h) and saline infusion for severe cases. [1]

Per the 2024 National Comprehensive Cancer Network (NCCN) guidelines on neuroendocrine and adrenal tumors, octreotide should be available as needed during surgical procedures for patients with carcinoid syndrome who develop hemodynamic instability that could indicate carcinoid crisis. Doses of octreotide (100–500 mcg IV) can be used, potentially followed by IV infusion of octreotide (50–300 mcg/h). Prophylactic administration of octreotide (dose unspecified) prior to surgery can be utilized although intraoperative complications can still arise, and the routine use of prophylactic octreotide has been called into question. [2]

A 2004 consensus report provided a detailed framework for the clinical use of somatostatin analogs in managing NETs of the gastroenteropancreatic system. During surgery, carcinoid crisis with hypotension is managed with IV boluses of 500–1000 mcg octreotide repeated every 5 minutes until symptoms are controlled. Alternatively, continuous IV infusion at 50–200 mcg/h can follow a bolus dose. Postoperatively, patients requiring supplemental dosing during the procedure should receive 50–200 mcg/h for 24 hours, then resume their preoperative regimen. [3], [4]

A 2013 systematic review analyzed the evidence for employing high doses of IV octreotide to manage carcinoid crises. The systematic review included 18 articles, comprising 17 case reports and one retrospective chart review, and aimed to evaluate the efficacy and safety of octreotide doses exceeding 1,500 mcg. Articles included described patients treated with IV octreotide for suspected or confirmed carcinoid crisis. Findings from the review highlighted that carcinoid crises were effectively managed in case reports with IV bolus doses ranging from 25 to 500 mcg and infusion rates between 50 and 150 mcg/h. No deaths were reported during any crisis event, and most crises were resolved promptly following octreotide administration. However, patients with prior prolonged exposure to octreotide for carcinoid syndrome or those with carcinoid heart disease appeared to require higher doses during crises. However, adverse events related to high-dose octreotide were not well-documented, and the review underscored the inconsistent use of the term "carcinoid crisis" and the paucity of outcomes data as major limitations. Although doses exceeding 1,500 mcg were used in a subset, the clinical justification and outcomes for these higher doses require further elucidation through robust investigations. [5]

A 2013 abstract reports use of higher dosing patterns beyond label recommendations for intramuscular octreotide long-acting release (LAR) in patients treated for carcinoid or pancreatic NETs (pNETs). Of 1,886 patients across 7 NCCN institutions, a total of 271 patients received octreotide LAR, of which 40% and 23% of carcinoid and pNET patients, respectively, received dosing greater than 30 mg q4w. Rationale for above-label dosing included uncontrolled symptoms, tumor progression, high urine 5-HIAA, and unknown. Dosing regimens among carcinoid patients were 40 mg q4w, 40 mg q3w, and 30 mg q2w, while dosing regimens among pNET patients include 40 mg q4w, 30 mg q2w, and 60 mg q4w. Prospective studies are still required to validate these dosing strategies, as well as to determine the most optimal dosing schedule. [6]

References:

[1] Kaltsas G, Caplin M, Davies P, et al. ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Pre- and Perioperative Therapy in Patients with Neuroendocrine Tumors. Neuroendocrinology. 2017;105(3):245-254. doi:10.1159/000461583
[2] National Comprehensive Cancer Network (NCCN). NCCN Clinical Practice Guidelines in Oncology. Neuroendocrine and Adrenal Tumors. Version 2.2024. Updated August 1, 2024. Accessed December 3, 2024. https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1448
[3] Oberg K, Kvols L, Caplin M, et al. Consensus report on the use of somatostatin analogs for the management of neuroendocrine tumors of the gastroenteropancreatic system. Ann Oncol. 2004;15(6):966-973. doi:10.1093/annonc/mdh216
[4] Borna RM, Jahr JS, Kmiecik S, Mancuso KF, Kaye AD. Pharmacology of Octreotide: Clinical Implications for Anesthesiologists and Associated Risks. Anesthesiol Clin. 2017;35(2):327-339. doi:10.1016/j.anclin.2017.01.021
[5] Seymour N, Sawh SC. Mega-dose intravenous octreotide for the treatment of carcinoid crisis: a systematic review. Can J Anaesth. 2013;60(5):492-499. doi:10.1007/s12630-012-9879-1
[6] Strosberg JR, Bobiak S, Zornosa CC, et al. Dosing patterns for octreotide LAR in neuroendocrine tumor (Net) patients: NCCN NET outcomes database. JCO. 2013;31(15_suppl):4142-4142.

Literature Review

A search of the published medical literature revealed 4 studies investigating the researchable question:

How best to dose octreotide for carcinoid crisis associated with neuroendocrine tumor?

Level of evidence

C - Multiple studies with limitations or conflicting results  Read more→



Please see Tables 1-4 for your response.


 

Octreotide LAR Dosage and Survival Among Elderly Patients With Distant-Stage Neuroendocrine Tumors

Design

Retrospective, observational, cross-sectional, multi-center study

N= 222

Objective

To evaluate the association between initial octreotide long-acting repeatable (LAR) dosage and overall survival of elderly patients with neuroendocrine tumors (NETs)

Study Groups

Study cohort (N= 222)

Inclusion Criteria

Older adult (aged ≥65 years) patients with distant-stage NET; received octreotide LAR treatment within 12 months of diagnosis

Exclusion Criteria

Aged <65 years; enrolled in health maintenance organizations; without continuous enrollment in Medicare Parts A and B

Methods

Patients were identified from the Surveillance, Epidemiology, and End Results (SEER)-Medicare database. Dosage level received in the first 3 months of therapy was calculated based on HCPCS codes and NDC codes, then averaged per 28 days. Cohorts were then categorized by low-dosage group (≤20 mg), medium-dosage group (21-30 mg), and high-dosage group (>30 mg). Clinical and demographic characteristics (e.g., tumor size, other treatments received) and comorbidities were controlled for in clinical and demographic characteristics and comorbidities.

Duration

January 1999 to December 2009

Outcome Measures

Five-year survival

Baseline Characteristics

 

Study cohort (N= 222)

 

Octreotide LAR dosages

Low

Medium

High

 

81 (36%)

82 (37%)

59 (27%)

 

Descriptive statistics of the study cohort by dosage level were not fully presented in the study.

Older patients (>75 years of age) were more likely to get low dosages. No other significant differences in baseline characteristics were observed.

Results

Endpoint

Hazard ratio (95% confidence interval)

p-value

Dosage, mg

≤20

21-30

>30

 

2.000 (1.318 to 3.035)

1

1.094 (0.671 to 1.7884)

 

0.0011

-

0.7193

Carcinoid syndrome

1.398 (0.922 to 2.119)

 

0.1144 

Age, years

65-69

70-74

≥75

 

1

1.645 (0.986 to 2.745)

1.185 (0.731 to 1.922)

 

-

0.0569

0.4901

Female

1.450 (0.995 to 2.113)

0.0531

Race/ethnicity other than non-Hispanic white

0.936 (0.554 to 1.582)

0.8056

Comorbidity score ≥1

1.322 (0.893 to 1.959)

0.1636

Tumor size, cm

≤2

>2

Unknown

 

1

0.892 (0.515 to 1.544)

1.495 (0.838 to 2.668)

 

-

0.6836

0.1738

Histology grade

Well-differentiated

Moderately/poorly differentiated

Not determined/unknown/mixed

 

1

2.078 (1.068 to 4.044)

1.450 (0.886 to 2.375)

 

-

0.0313

0.1392

Primary site

Small intestine

Cecum and appendix

Colon

Larynx, bronchus, lung, trachea, other respiratory organs

Pancreas

Others

 

1

2.559 (1.291 to 5.071)

2.807 (1378 to 5.714)

3.914 (1.855 to 8.259)

2.313 (1.293 to 4.139)

1.923 (0.961 to 3.848)

 

-

0.0071

0.0044

0.0003

0.0047

0.0647

Surgery of primary site

0.589 (0.341 to 1.091) 0.0584

Liver surgery

0.830 (0.423 to 1.627) 0.5874

Chemotherapy

1.376 (0.942 to 2.009) 0.0987

Radiation therapy

1.467 (0.855 to 2.516) 0.1641

Data listed are for the Cox proportional hazard model for survival.

Low octreotide LAR dosage was significantly negatively associated with survival vs medium dosage, but there is no survival benefit with high vs medium dosage.

Patients with moderately or poorly differentiated histology had significantly worse survival outcomes vs well-differentiated histology.

Patients having the primary cancer site in the cecum and appendix, colon, and larynx, bronchus, lung, trachea, and other respiratory organs or pancreas had significantly worse survival outcomes vs small intestine as the primary cancer site.

Adverse Events

N/A

Study Author Conclusions

To our knowledge, this is the first population-based study about the association between octreotide LAR dosage and the survival of elderly patients with NET. We found that an initial dosage level of 21-30 mg per 28 days starting within 12 months of diagnosis was significantly associated with better survival compared with lower dosage level (≤20 mg per 28 days). We did not identify statistically significant survival differences between the higher dosage (>30 mg per 28 days) and the medium dosage (21-30 mg per 28 days) groups. These results suggest potential survival benefits for octreotide LAR provided within 12 months of diagnosis at a level of 21-30 mg per 28 days as an initial dosage among elderly patients with distant-stage NET. Further studies examining the dose-dependent effects of octreotide LAR on tumor control are warranted.

InpharmD Researcher Critique

This study has limitations inherent to that of retrospective and observational studies. Total dosage received over the 3 months since diagnosis was calculated based on HCPCS codes and NDC codes, and presence of carcinoid syndrome was defined based on ICD-9 codes, all of which may have been missing or inaccurate.



References:

Shen C, Xu Y, Dasari A, Shih YC, Yao JC. Octreotide LAR Dosage and Survival Among Elderly Patients With Distant-Stage Neuroendocrine Tumors. Oncologist. 2016;21(3):308-313. doi:10.1634/theoncologist.2015-0381

 

Periprocedural Management of Patients Undergoing Liver Resection or Embolotherapy for Neuroendocrine Tumor Metastases
Design

Retrospective review

N= 75 

Objective

To describe the periprocedural management of patients with well-differentiated neuroendocrine tumors with hepatic metastases who underwent liver-directed procedures

Study Groups

All patients (N= 75)

Inclusion Criteria

Adult patients with metastatic neuroendocrine tumors (NETs) with liver metastases who underwent hepatic resection, ablation, or embolotherapy at the institution between June 1, 2012, and December 31, 2016  

Exclusion Criteria

Poorly differentiated tumors based on histology, mitotic rate greater than 20 per 2 mm², and/or Ki-67 index greater than 20%

Methods

Retrospective review of electronic health records, including anesthesia records, operative notes, and discharge summaries. Data on clinicopathologic characteristics, periprocedural management (frequency of periprocedural octreotide administration), and outcomes were collected. 

Duration

June 1, 2012, to December 31, 2016  

Outcome Measures

Primary: Occurrence of carcinoid crisis (CC; defined as ≥10 minutes of systolic blood pressure [SBP] <80 or >180 mmHg, or pulse greater >120 beats/minute.) or hemodynamic instability (HDI)

Secondary: Postprocedural complications graded by Clavien-Dindo criteria 

Baseline Characteristics  

All patients (N= 75)

Age, years (range)

61 (25–79)  

Female

39 (52%)  

Primary tumor location

Small bowel primary or unknown primary NET

48 (64%)

Prior history of carcinoid syndrome

35 (47%)

Urine 5-HIAA level >2-fold the upper limit of normal

29 (39%)

Procedure

Liver resection
or ablation

Liver embolotherapy

 

38 (51%)

37 (50%)

Preprocedural octreotide administration route*

Infusion

IV bolus or IM 

 

27 (36%)

21 (28%)

Preprocedural octreotide administration median dose (range)*

Infusion dose, mcg/hour

Bolus dose, mcg

 

 

150 (50–300)

150 (100–300)

Intraprocedural octreotide administration route*

Infusion

IV bolus or IM 

 

48 (64%)

20 (27%)

Intraprocedural octreotide  administration median dose (range)*

Infusion dose, mcg/hour

Bolus dose, mcg

 

150 (50–300)

150 (20–510)

Abbreviations:  5-HIAA= 5-hydroxyindoleacetic acid; IV= intravenous; IM= intramuscular; NET= neuroendocrine tumors 

*Route and dose of both preprocedural and intraprocedural octreotide administration varied widely

Results

Endpoint 

All patients 

CC or HDI

HDI alone

Both CC and HDI

CC alone

24 (32%)

21 (28%)

2 (3%)

1 (1%)

Clavien-Dindo: Patients with CC or HDI (n= 24)*

0–1

2–4

 

14 (58%)

10 (42%)

Clavien-Dindo: Patients with no CC or HDI event (n= 51)*

0–1

2–4

 

43 (84%)

8 (16%)

Hypotension requiring vasopressors: Patients with CC or HDI (n= 24)

 

2 (8%)

Hypotension requiring vasopressors: Patients with no CC or HDI event (n= 51)

 

1 (2%)

*p-value= 0.01

No clinicopathologic or procedural factors, including procedure type, octreotide or long-acting somatostatin analog use, and history of carcinoid syndrome, were associated with CC/HD.

Adverse Events

Higher-grade postprocedural complications in patients with CC or HDI, including pulmonary embolism (8% vs. 0%) and tachyarrhythmias requiring IV nodal blockers (8% vs. 0%).

Study Author Conclusions

A significant portion of patients developed CC/HDI, associated with severe postprocedural complications. Periprocedural octreotide use was not associated with lower CC/HDI occurrence, but its continued use is advised given its safety profile

Critique

The study provides insights into the periprocedural management of NETs with liver metastases. However, it has several limitations, including its single-institution retrospective design, small sample size, and reliance on the HDI as a surrogate for CC. Additionally, the varying octreotide dosing and routes of administration utilized make it challenging to draw definitive conclusions about optimal management strategies.

References:

Kwon DH, Paciorek A, Mulvey CK, et al. Periprocedural Management of Patients Undergoing Liver Resection or Embolotherapy for Neuroendocrine Tumor Metastases. Pancreas. 2019;48(4):496-503. doi:10.1097/MPA.0000000000001271

 

A Challenging Case of Carcinoid Crisis in a Patient With Neuroendocrine Tumor

Design

Case report

Case presentation

A 2021 case report detailed the management of a 37-year-old hemodialysis-dependent male with end-stage renal disease (ESRD) and metastatic neuroendocrine tumor (NET) who presented with refractory hypotension and abdominal pain, later identified as carcinoid crisis. This patient’s prior medical course included a diagnosis of high-grade neuroendocrine carcinoma with extensive metastasis, intestinal obstruction necessitating hemicolectomy and ileostomy, and subsequent complications, including surgical wound infections.

Upon admission, despite broad-spectrum antibiotics and vasopressor therapy, the hypotension persisted. Laboratory results revealed elevated chromogranin-A levels but low serum serotonin, while key differentials such as septic shock and adrenal insufficiency were deemed less likely based on clinical and biochemical findings. Management efforts pivoted toward a suspected carcinoid crisis, highlighted by the initiation of intravenous (IV) octreotide infusion at 50 mcg/hour. Within 30 hours, blood pressure stabilized, allowing for the discontinuation of norepinephrine. This dramatic response to octreotide supported the diagnosis and demonstrated its clinical efficacy in reversing refractory hypotension in carcinoid crisis. Notably, factors such as prior invasive procedures, infection, and chemotherapy might have served as inciting events, though the precise trigger remained unclear. 

Study Author Conclusions

Carcinoid crisis is an important complication of neuro-endocrine tumors, which can masquerade as septic shock in cases where infection is a potential etiology. Refractoriness to vasopressors should increase the index of suspicion. Somatostatin analog therapy can be considered both diagnostic and therapeutic.

References:

Mahdi M, Ozer M, Tahseen M. A Challenging Case of Carcinoid Crisis in a Patient With Neuroendocrine Tumor. Cureus. 2021;13(6):e15626. Published 2021 Jun 13. doi:10.7759/cureus.15626

 

Adrenaline bolus rescue for refractory carcinoid crises during surgical manipulation of metastatic neuroendocrine tumor of pancreas: A case report

Design

Case report

Case presentation

A 2024 case report highlighted the intraoperative management of a 25-year-old female with metastatic pancreatic neuroendocrine tumor presenting with recurrent carcinoid crises. Despite preoperative optimization with octreotide infusion at 50 mcg/hour for 24 hours, severe hemodynamic instability ensued during surgical manipulation of metastatic liver and pancreatic lesions. Initial interventions with phenylephrine boluses and infusion, vasopressin infusion, and norepinephrine proved ineffective in maintaining an adequate mean arterial pressure (MAP). Hemodynamic deterioration persisted with a MAP decline to 45 mmHg and significant tachycardia despite these therapeutic measures. Adrenaline boluses of 10 mcg, titrated alongside infusion doses ranging from 0.05 to 0.3 mcg/kg/min, were subsequently introduced and successfully stabilized MAP to 60–65 mmHg. Post-resection of the pancreatic lesion, vasopressor requirements decreased, allowing for the gradual tapering of adrenaline and noradrenaline infusions. The intraoperative course included significant blood loss (1,800 mL), requiring transfusion of packed red blood cells while maintaining a urine output of 40–50 mL/hour. Postoperatively, metabolic acidosis with elevated lactate (6 mmol/L) resolved following surgical completion. Notably, pink urine syndrome was observed, attributed to uric acid crystal precipitation secondary to the neuroendocrine tumor and associated physiological stress. Urinalysis confirmed high uric acid concentrations with sterile urine culture, and the pink discoloration resolved by postoperative day two without intervention. By the two-month follow-up, no further episodes of carcinoid syndrome were noted.

Study Author Conclusions

Discussion with the endocrinologist and the operating surgeon regarding the nature of the tumor, the patients’ response in the past, associated adverse effects, and response to past treatment and the surgical steps can be helpful to an anesthesiologist in anticipating and preparing for intraoperative management. This communication should be continued throughout the perioperative period. An effective evidence‑based anesthetic management of carcinoid syndrome and crisis management is imperative.

References:

Murugesan AM, Nandagopan NM, Senthilnathan M, Kumar MR. Adrenaline bolus rescue for refractory carcinoid crises during surgical manipulation of metastatic neuroendocrine tumor of pancreas: A case report. Saudi J Anaesth. 2024;18(4):599-601. doi:10.4103/sja.sja_319_24