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Double PitNETs: A Case Report and Literature Review
Mitsuru Nishiyama1,2, Noriaki Fukuhara3, Hiroshi Nishioka3
1Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School, Kochi University, 1-185, Kohasu, Oko-cho, Nankoku City 783-8505, Kochi, Japan.
Cancers
|February 26, 2025
Summary
Double pituitary neuroendocrine tumors (PitNETs) are rare, often presenting with hormonal excess like acromegaly or Cushing's disease. These tumors can appear as one or two distinct masses, requiring careful diagnosis.
Area of Science:
- Endocrinology
- Neuro-oncology
- Pathology
Background:
- Double pituitary neuroendocrine tumors (PitNETs) involve two distinct tumors within the pituitary gland, a rare clinical occurrence.
- These tumors may present as separate masses on imaging or be identified as two independent entities only upon pathological examination.
- Understanding the characteristics of double PitNETs is crucial due to their complex clinical, radiological, and pathological profiles.
Purpose of the Study:
- To analyze a large cohort of double PitNET cases to elucidate their clinical, pathological, and radiological features.
- To identify the most common clinical presentations and hormonal excesses associated with double PitNETs.
- To characterize the predominant tumor cell types and transcription factor lineages involved in double PitNETs.
Main Methods:
- A comprehensive literature review was conducted, analyzing data from 142 cases of double PitNETs.
- Clinical features, pathological findings (including tumor cell types and transcription factors), and radiological data were extracted and analyzed.
- Statistical analysis was performed to determine the prevalence of different clinical syndromes and tumor characteristics.
Main Results:
- Acromegaly (45.5%) was the most frequent clinical manifestation, followed by Cushing's disease (35.1%) and prolactinoma (17.9%).
- Pathological analysis revealed somatotroph (28.9%) and corticotroph (26.8%) tumors as predominant, with a rising proportion of gonadotroph tumors.
- Radiological findings showed a near-equal incidence of double (45.1%) versus single (47.6%) tumor presentation, highlighting the potential for missed micro-tumors.
Conclusions:
- Double PitNETs are typically diagnosed due to hormonal excess from at least one functional tumor.
- The presentation can be misleading, with a significant proportion appearing as a single lesion, necessitating vigilance for co-existing micro-lesions.
- Advancements in diagnostic and therapeutic strategies are improving the management of these complex pituitary tumors.
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