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About OGSIVEO

OGSIVEO is the first and only FDA-approved targeted therapy for adult patients with progressing desmoid tumors who require systemic treatment.

What is OGSIVEO?

OGSIVEO is indicated for adult patients with progressing desmoid tumors who require systemic treatment. It is the first and only FDA-approved targeted gamma secretase inhibitor. OGSIVEO was evaluated in the DeFi study, a Phase 3 pivotal trial that included 142 adult patients with progressing desmoid tumors.1,2

NCCN Category 1 Preferred Logo

Nirogacestat (OGSIVEO) is recommended as a treatment option by the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®)

NCCN Guidelines® for Soft Tissue Sarcoma recommend nirogacestat (OGSIVEO) as a Category 1 Preferred systemic therapy option for patients with desmoid tumors (aggressive fibromatosis).3

DeFi, Desmoid Fibromatosis; FDA, US Food and Drug Administration; NCCN, National Comprehensive Cancer Network® (NCCN®).

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OGSIVEO offers convenient oral dosing1

The recommended dosage of OGSIVEO is 150 mg administered orally twice daily until 
disease progression or unacceptable toxicity

Fork and spoon icon

OGSIVEO may be taken with or without food

Tablet in hand icon

Instruct patients to swallow OGSIVEO tablets whole and not to break, crush, or chew prior to swallowing

Alarm clock and pill icon

If a patient vomits or misses a dose, instruct the patient to take the next dose at its scheduled time

Starfruit and grapefruit icon

Avoid concomitant use of OGSIVEO with grapefruit products, Seville oranges, and starfruit

Dosage modifications for OGSIVEO

The dose of OGSIVEO may be reduced to 100 mg twice daily for patients who experience certain adverse reactions.1

Dosage Modifications Table

Recommended dose modifications for adverse reactions1

Adverse Reaction
Severity
OGSIVEO Dosage Modifications
Diarrhea persisting for ≥3 days despite maximal medical therapy
Grades 3 or 4
Withhold OGSIVEO until resolved to Grade ≤1 or baseline, then restart at a dose of 100 mg twice daily.
ALT or AST increased
Grade 2
(≥3 to 5 x ULN)
Withhold OGSIVEO until ALT, AST, or both are resolved to <3 × ULN or baseline, then restart at a dose of 100 mg twice daily.
Grades 3 or 4 (>5 x ULN)
Permanently discontinue.
Hypophosphatemia persisting for ≥3 days despite maximal replacement therapy
Grades 3 or 4
Withhold OGSIVEO until resolved to Grade ≤1 or baseline, then restart at a dose of 100 mg twice daily.
Hypokalemia despite maximal replacement therapy

For other severe adverse reactions, life-threatening adverse reactions, or persistent intolerable Grade 2 adverse events, withhold drug until resolved to Grade ≤1 or baseline. Only restart at a dose of 100 mg twice daily after considering the potential benefit and likelihood of recurrence of the adverse reaction. Permanently discontinue OGSIVEO for recurrence of severe or life-threatening adverse reaction upon rechallenge at the reduced dose.1

ALT, alanine aminotransferase; AST, aspartate aminotransferase; ULN, upper limit of normal.

Drug Interactions With OGSIVEO

Exposure to OGSIVEO may be affected by other drugs.1

Effects of Other Drugs on OGSIVEO1
  • Strong or moderate CYP3A inhibitors: OGSIVEO is a CYP3A substrate. Strong or moderate CYP3A inhibitors increase OGSIVEO exposure, which may increase the risk of OGSIVEO adverse reactions. Avoid concomitant use of OGSIVEO with strong or moderate CYP3A inhibitors, including grapefruit products, Seville oranges, and starfruit
  • Strong or moderate CYP3A inducers: OGSIVEO is a CYP3A substrate. Strong or moderate CYP3A inducers decrease serum OGSIVEO exposure, which may reduce the effectiveness of OGSIVEO. Avoid concomitant use of OGSIVEO with strong or moderate CYP3A inducers
  • Gastric acid reducing agents: OGSIVEO is poorly soluble at pH ≥6. Gastric acid reducing agents may decrease serum OGSIVEO exposure, which may reduce the effectiveness of OGSIVEO. Avoid concomitant use with proton pump inhibitors and H2 blockers. If concomitant use cannot be avoided, OGSIVEO can be staggered with antacids (e.g., administer OGSIVEO 2 hours before or 2 hours after antacid use)*,†
  • For additional information about potential drug interactions with OGSIVEO, see Table 4 (Section 7.1) and Table 5 (Section 7.2) of the full Prescribing Information

Gastric acid reducing agents include PPIs (e.g., omeprazole, lansoprazole, esomeprazole), H2 blockers (e.g., cimetidine, famotidine, nizatidine), and antacids (e.g., Mylanta®, Rolaids®, Tums®). In accordance with the guidance in the OGSIVEO Prescribing Information, antacids should be administered either 2 hours before or 2 hours after OGSIVEO.

Prior to use of any concomitant medication, please refer to Section 7 (Drug Interactions) of the OGSIVEO Prescribing Information.

PPIs, proton pump inhibitors.

OGSIVEO is supplied in 50 mg, 100 mg, and 150 mg tablets

OGSIVEO is available in 150 mg and 100 mg tablets in blister packs

  • Each blister pack contains a 7-day supply
  • Four blister packs provide a 28-day supply

Reduces pill burden and may help simplify tracking of AM/PM dosing

OGSIVEO 150 mg blister pack

150 mg tablet

Tablets shown are not actual size.

OGSIVEO 100 mg blister pack

100 mg tablet

Tablets shown are not actual size.

NDC numbers1

  • 14-count blister pack (150 mg): 82448-150-14
  • 14-count blister pack (100 mg): 82448-100-14
  • 180-count bottle (50 mg): 82448-050-18

Storage and handling1

  • Store at 20°C-25°C (68°F-77°F)
  • Temperature excursions permitted between 
15°C-30°C (59°F-86°F)
  • See USP Controlled Room Temperature

OGSIVEO is the first and only FDA-approved targeted gamma secretase inhibitor

Proposed mechanism of action based on in vitro models

Nuclear accumulation of beta-catenin
Notch activation by gamma secretase
Pathway crosstalk
OGSIVEO mechanism of action

APC, adenomatous polyposis coli; CTNNB1, catenin beta 1.

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Patient Support

SpringWorks CareConnections™ provides personalized support to help your patients start and stay on track with OGSIVEO.

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Efficacy & Safety
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Important Safety Information

Indication 

OGSIVEO is indicated for adult patients with progressing desmoid tumors who require systemic treatment.

Important Safety Information

Warnings And Precautions

Diarrhea: Diarrhea, sometimes severe, can occur in patients treated with OGSIVEO. Diarrhea occurred in 84% of patients treated with OGSIVEO, and included Grade 3 events in 16% of patients. Median time to first diarrhea event was 9 days (range: 2 to 434 days). Monitor patients and manage using antidiarrheal medications. Modify dose as recommended.

Ovarian Toxicity: Female reproductive function and fertility may be impaired in patients treated with OGSIVEO. Impact on fertility may depend on factors like duration of therapy and state of gonadal function at time of treatment. Long-term effects of OGSIVEO on fertility have not been established. Advise patients on the potential risks for ovarian toxicity before initiating treatment. Monitor patients for changes in menstrual cycle regularity or the development of symptoms of estrogen deficiency, including hot flashes, night sweats, and vaginal dryness.

Hepatotoxicity: ALT or AST elevations occurred in 30% and 33% of patients, respectively. Grade 3 ALT or AST elevations (>5 x ULN) occurred in 6% and 2.9% of patients. Monitor liver function tests regularly and modify dose as recommended.

Non-Melanoma Skin Cancers: New cutaneous squamous cell carcinoma and basal cell carcinoma occurred in 2.9% and 1.4% of patients, respectively. Perform dermatologic evaluations prior to initiation of OGSIVEO and routinely during treatment.

Electrolyte Abnormalities: Decreased phosphate (65%) and potassium (22%) occurred in OGSIVEO-treated patients. Phosphate <2 mg/dL occurred in 20% of patients. Grade 3 decreased potassium occurred in 1.4% of patients. Monitor phosphate and potassium levels regularly and supplement as necessary. Modify dose as recommended.

Embryo-Fetal Toxicity: OGSIVEO can cause fetal harm when administered to pregnant women. Oral administration of nirogacestat to pregnant rats during the period of organogenesis resulted in embryo-fetal toxicity and death at maternal exposures below human exposure at the recommended dose of 150 mg twice daily. Advise pregnant women of the potential risk to a fetus. Advise females and males of reproductive potential to use effective contraception during treatment with OGSIVEO and for 1 week after the last dose.

Adverse Reactions

The most common (≥15%) adverse reactions were diarrhea (84%), ovarian toxicity (75% in the 36 females of reproductive potential), rash (68%), nausea (54%), fatigue (54%), stomatitis (39%), headache (30%), abdominal pain (22%), cough (20%), alopecia (19%), upper respiratory tract infection (17%), and dyspnea (16%).

Serious adverse reactions occurred in 20% of patients who received OGSIVEO. Serious adverse reactions occurring in ≥2% of patients were ovarian toxicity (4%).

The most common laboratory abnormalities (≥15%) were decreased phosphate, increased urine glucose, increased urine protein, increased AST, increased ALT, and decreased potassium.

Drug Interactions

CYP3A Inhibitors and Inducers: Avoid concomitant use with strong or moderate CYP3A inhibitors (including grapefruit products, Seville oranges, and starfruit) and strong or moderate CYP3A inducers.

Gastric Acid Reducing Agents: Avoid concomitant use with proton pump inhibitors and H2 blockers. If concomitant use cannot be avoided, OGSIVEO can be staggered with antacids (e.g., administer OGSIVEO 2 hours before or 2 hours after antacid use).

Consult the full Prescribing Information prior to and during treatment for important drug interactions.

Use in Specific Populations

Because of the potential for serious adverse reactions in breastfed children, advise women not to breastfeed during treatment with OGSIVEO and for 1 week after the last dose.

Please click here for full Prescribing Information.

Important Safety Information

ALT, alanine aminotransferase; APC, adenomatous polyposis coli; AST, aspartate aminotransferase, BID, twice daily; CTNNB1, catenin beta 1; Defi, desmoid Fibromatosis; FDA, US Food and Drug Administration; HCP, healthcare professional; NCCN, National Comprehensive Cancer Network® (NCCN®); NDC, National Drug Code; PPIs, proton pump inhibitors; ULN, upper limit of normal; USP, US Pharmacopeia.

References
  1. OGSIVEO. Prescribing Information. SpringWorks Therapeutics, Inc.
  2. Gounder M, Ratan R, Alcindor T, et al. Nirogacestat, a gamma-secretase inhibitor for desmoid tumors. N Engl J Med. 2023;388(10):898-912.
  3. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.1.2024. © 2024 National Comprehensive Cancer Network, Inc. All rights reserved. Accessed 
May 2, 2024. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.
  4. Crago AM, Chmielecki J, Rosenberg M, et al. Near universal detection of alterations in CTNNB1 and Wnt pathway regulators in desmoid-type fibromatosis by whole-exome sequencing and genomic analysis. Genes Chromosomes Cancer. 2015;54(10):606-615.
  5. Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017;29(4):268-274.
  6. Gronchi A, et al. Desmoid Tumor Working Group. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients [supplementary appendix]. Eur J Cancer. 2020;127:96-107.
  7. Salas S, Chibon F, Noguchi T, et al. Molecular characterization by array comparative genomic hybridization and DNA sequencing of 194 desmoid tumors. Genes Chromosomes Cancer. 2010;49(6):560-568.
  8. Carothers AM, Rizvi H, Hasson RM, et al. Mesenchymal stromal cell mutations and wound healing contribute to the etiology of desmoid tumors. Cancer Res. 2012;72(1):346-355
  9. Shang H, Braggio D, Lee YJ, et al. Targeting the Notch pathway: a potential therapeutic approach for desmoid tumors. Cancer. 2015;121(22):4088-4096
  10. Siebel C, Lendahl U. Notch signaling in development, tissue homeostasis, and disease. Physiol Rev. 2017;97(4):1235-1294.
  11. Andersson ER, Sandberg R, Lendahl U. Notch signaling: simplicity in design, versatility in function. Development. 2011;138(17):3593-3612.