NOW Approved

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

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NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®)

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

Patients with desmoid tumors may face uncontrollable growth and a high symptom burden2-5

  • Desmoid tumors are rare, locally aggressive, invasive soft tissue tumors with an unpredictable clinical course that can impact vital organs2,3,6,7
  • Desmoid tumors are associated with a potentially high and multifaceted burden of illness, including pain, disfigurement, and decreased physical function4,*
Find Out More

Data from a Memorial Sloan Kettering/Desmoid Tumor Research Foundation patient-reported outcome (PRO) validation study included patients with desmoid tumors (n=31, age range 20-68, 77% female). Patients participated in 60-minute qualitative phone interviews to provide their perspectives on disease symptoms and impact on their quality of life. The majority of the patients in this study were symptomatic (84%). Tumor site and type varied across patients. The concepts discussed during interviews were used to develop a draft patient-reported outcome scale, which was further refined in cognitive interviews of additional patients with desmoid tumors (n=15).4

Examples of desmoid tumors and potential symptoms

Back

Large desmoid tumor (15 cm x 10 cm) proximal to the spine8

Image adapted from Cohen S, et al. World J Surg Oncol. 2008;6:28. Reused under Creative Commons License 2.0 (creativecommons.org/licenses/by/2.0). Image background changed to gray.

Hand

Desmoid tumor causing severe restriction in the flexion of the hand9

Image reproduced from Scaramussa FS, et al. SM J Orthop. 2016;2(3):1036. Reused under Creative Commons License 4.0 (creativecommons.org/licenses/by/4.0).

Knee

MRI scan showing desmoid tumor behind the right knee associated with electric paresthesias and reduced flexion10

Image adapted from Weschenfelder W, et al. Case Rep Surg. 2015;2015:262654. Reused under Creative Commons License 3.0 (creativecommons.org/licenses/by/3.0). False color added.

Neck

CT scan showing desmoid tumor in the right upper neck involving the brachial plexus associated with pain, numbness, and weakness in the right arm11

Image adapted from Styring E, et al. Am J Med Case Rep. 2019;7(3):36-40. Reused under Creative Commons License 4.0 (creativecommons.org/licenses/by/4.0). False color added.

Learn more about OGSIVEO—the first and only FDA-approved therapy for adult patients with progressing desmoid tumors who require systemic treatment

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Efficacy

Discover the primary and key secondary efficacy end point results in the DeFi study.

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Safety

Learn about the safety profile of OGSIVEO.

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Dosing

See dosing and administration information for OGSIVEO.

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Ordering/Rx

Find out how to prescribe OGSIVEO for your adult patients with progressing desmoid tumors who require systemic treatment.

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Patient Support

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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

CT, computed tomography; DeFi, Desmoid Fibromatosis; FDA, US Food and Drug Administration; HCP, healthcare professional; MRI, magnetic resonance imaging; NCCN, National Comprehensive Cancer Network® (NCCN®).

References
  1. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.3.2023. © National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed December 18, 2023. 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.
  2. Kasper B, Baumgarten C, Garcia J, et al. Desmoid Working Group. An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma Patients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol. 2017;28(10):2399-2408.
  3. Constantinidou A, Scurr M, Judson I, Litchman C. Clinical presentation of desmoid tumors. In: Litchman C, ed. Desmoid Tumors. Springer; 2012:chap 2. Accessed December 18, 2023. https://www.researchgate.net/publication/226455135.
  4. Gounder MM, Maddux L, Paty J, Atkinson TM. Prospective development of a patient-reported outcomes instrument for desmoid tumors or aggressive fibromatosis. Cancer. 2020;126(3):531-539.
  5. Husson O, Younger E, Dunlop A, et al. Desmoid fibromatosis through the patients’ eyes: time to change the focus and organisation of care? Support Care Cancer. 2019;27(3):965-980.
  6. Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017;29(4):268-274.
  7. Sbaraglia M, Bellan E, Dei Tos AP. The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives. Pathologica. 2021;113(2):70-84.
  8. Cohen S, Ad-El D, Benjaminov O, Gutman H. Post-traumatic soft tissue tumors: case report and review of the literature a propos a post-traumatic paraspinal desmoid tumor. World J Surg Oncol. 2008;6:28.
  9. Scaramussa FS, Castro UB. Desmoid tumor in hand: a case report. SM J Orthop. 2016;2(3):1036.
  10. Weschenfelder W, Lindner R, Spiegel C, et al. Desmoid tumor of the popliteal fossa during pregnancy. Case Rep Surg. 2015;2015:262654.
  11. Styring E, Ahlström M, Rissler P, et al. Desmoid fibromatosis in the brachial plexus mimicking an ulnar nerve entrapment. Am J Med Case Rep. 2019;7(3):36-40.