Diffuse Intrapulmonary Neuroendocrine Cell Hyperplasia

Abstract

Diffuse intrapulmonary neuroendocrine cell hyperplasia is a rare, potential precursor lesion to typical pulmonary carcinoid tumours. Fewer than 50 cases have been reported in the literature. Their pathogenesis, clinical significance and management is controversial. A patient who presented with diffuse intrapulmonary neuroendocrine cell hyperplasia associated with a primary typical carcinoid tumour of the lung is reported.

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia With Progression to Neuroendocrine Tumor

“…Management decisions depend on patient’s life expectancy, clinical status, and personal preferences. In patients with mild and stable presentation, observation alone may suffice [ 1 , 3 ]. Therapeutic options may include oral and inhaled steroids, chemotherapy, surgical lung resection, and lung transplantation.…”

“…Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) defined by diffuse proliferation of neuroendocrine cells on the bronchial wall is a rare condition, and its true prevalence is unknown. It is a primary entity and must be distinguished from proliferation of secondary neuroendocrine cells, reactive to other pathological processes [1][2][3]. Its first descriptions came from incidental findings on biopsies of lung nodules, and, consequently, it was initially described as a histological entity and recognized by the World Health Organization as a premalignant disorder [1,3].…”

“…It is a primary entity and must be distinguished from proliferation of secondary neuroendocrine cells, reactive to other pathological processes [ 1 - 3 ]. Its first descriptions came from incidental findings on biopsies of lung nodules, and, consequently, it was initially described as a histological entity and recognized by the World Health Organization as a premalignant disorder [ 1 , 3 ]. Based on accumulating evidence from case reports, DIPNECH is now considered a syndrome of unknown cause, with typical clinical, radiological, and histopathological findings [ 3 , 4 ].…”

“…Neuroendocrine cell hyperplasia (NECH) can be superficial and isolated or invade the basement membrane and form tumorlets (cell aggregates <5 mm) or carcinoid tumors (if >5 mm) [ 1 , 2 , 4 ]. Histologically, DIPNECH is characterized by the presence of a variable number of tumorlets and/or carcinoid tumors [ 1 , 4 ]. Neuroendocrine origin is confirmed by cell expression of cluster of differentiation (CD)56, synaptophysin, and chromogranin A [ 3 ].…”

Multifokale neuroendokrine Lungentumoren: Entstehung, Diagnostik und Behandlung

“…Daher ist meist eine thorakoskopisch entnommene Lungenbiopsie zur Diagnosesicherung notwendig. Die chirurgische Lungenbiopsie gilt als der diagnostische Goldstandard [20]. In der pathologischen Untersuchung des entnommenen Lungenparenchymes zeigen sich oft multiple neuroendo- Es wurden zusätzlich Assoziationen der DIPNECH mit Endokrinopathien beschrieben: Akromegalie, ACTH-Produktion und MEN-1-Syndrom [12].…”