A rare case of congenital hyperinsulinism in a child — when morphology determines treatment tactics

Background. A clinical case of an encapsulated focal form of congenital hyperinsulinism in a young child is presented. A comprehensive examination was performed, including molecular genetic analysis, radionuclide diagnostics (PET-CT with 18F-DOPA) and intraoperative histological examination. During morphological analysis, an encapsulated focus of β-cell adenomatous hyperplasia was verified, located in the head of the pancreas. This finding necessitated an extended organ resection. It is particularly noteworthy that there is a clear fibrous capsule around the pathological focus, which presents a diagnostic challenge in differentiating this pathology from an insulinoma. The postoperative period was uneventful, and stable normoglycemia was achieved, which ultimately confirmed the positive outcome of the treatment and the patient's recovery. The unique feature of this clinical case is that it represents the first recorded example of the focal form of congenital hyperinsulinism, characterized by a well-defined fibrous capsule combined with adenomatosis lacking visible boundaries. As of today, similar observations have not been found in available publications from domestic medical centers, which highlights the rarity of the described case and its significance for expanding knowledge about the spectrum of focal forms of this disease. Analysis of this morphological variant highlights the importance of performing a rapid histological examination of tissues during surgery, which in this clinical case was a key factor in determining the extent of the resection. This decision allowed us not to be limited to just the encapsulated formation and ultimately led to the patient's full recovery.

Conclusion. This case demonstrates the importance of a multidisciplinary approach in the diagnosis of rare forms of congenital hyperinsulinism and highlights the need for a thorough histopathological examination of the surgical material to achieve correct clinical decision.

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