Insulinomatosis/NIPHS

Insulinomatosis or noninsulinoma pancreatogenous hypoglycaemia (NIPHS)

What is Insulinomatosis or noninsulinoma pancreatogenous hypoglycaemia (NIPHS)?

Insulinomatosis or NIPHS

An insulinoma is a rare type of functional neuroendocrine tumour which produces insulin and causes hypoglycaemia. A small number of people who complete the diagnosis process for an insulinoma are diagnosed with related conditions:

  • Insulinomatosis: multiple insulinomas including micro-insulinomas

  • Proinsulinoma: neuroendocrine tumour which produces proinsulin

  • Noninsulinoma pancreatogenous hypoglycaemia syndrome (NIPHS): beta cells in the pancreas produce excess insulin without forming a tumour

    A very small number of our members are diagnosed with insulinomatosis or NIPHS.  These are very rare conditions that often require lifelong medication. 

    Insulinomatosis involves multiple insulinomas including micro-insulinomas.  NIPHS stands for noninsulinoma pancreatogenous hypoglycaemia.  In this condition beta cells in the pancreas produce excess insulin but do not form a tumour.  NIPHS is also known as nesidioblastosis and islet cell hyperplasia.

    The diagnostic journey starts in the same way as for an insulinoma.  Whereas surgery is curative for a benign insulinoma, treatment for insulinomatosis or NIPHS can be challenging.  Both conditions can affect multiple areas of the pancreas and can reoccur after surgery.  It can take time to find medication that works.

    For people with a confirmed diagnosis of one of these very rare conditions, our Insulinomatosis or noninsulinoma pancreatogenous hypoglycaemia (NIPHS) support group offers emotional and practical support within the trusted framework of the Insulinoma support groups.  For people whose clinicians are investigating either condition, there is a page on the Insulinoma Support Network with more information.

    Peer support can be particularly helpful with a very rare lifelong condition.  Jennifer, who was diagnosed with NIPHS in 2015 and convenes the support group, says: “These can be very lonely diagnoses, particularly before there’s a treatment plan in place or if it’s not working.  Knowing there’s a group of people who understand is empowering.”   

    The British Medical Journal published an article in July 2024 Peer support in chronic health conditions, which observed “Peer support can be particularly helpful at the point of diagnosis or during periods of transition in health or life stages” and that “Self-management alone can be tiring, isolating, overwhelming, and tough on mental health.” 

    Useful information:

  • 2006 Islet Hyperplasia in Adults: Challenge to Preoperatively Diagnose Non-Insulinoma Pancreatogenic Hypoglycemia Syndrome

  • 2017 Proinsulinomas systematic review

  • 2019 MEN1 update

  • 2020 GLP-1R imaging

  • 2021 GLP-1R imaging

  • 2023 ENETS guidance

  • 2023 Insulinomatosis new aspects

  • 2023 NIPHS literature review

    If you have been diagnosed with one of these very rare conditions, please join our Facebook group. Insulinomatosis or noninsulinoma pancreatogenous hypoglycaemia (NIPHS) | Facebook