Giant plurihormonal pituitary neuroendocrine tumor [Neurosurgery Wiki]

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====== Giant plurihormonal pituitary neuroendocrine tumor ======

Of all the tumors reported in studies as [[Plurihormonal PIT-1–Positive pituitary neuroendocrine tumor]] (PP1) or silent subtype 3 (SS3), 99% were [[macroadenoma]]s and 18% were [[giant pituitary neuroendocrine tumor]]s (>4 cm)
((Andrews JP, Joshi RS, Pereira MP, Oh T, Haddad AF, Pereira KM, Osorio RC, Donohue KC, Peeran Z, Sudhir S, Jain S, Beniwal A, Chopra AS, Sandhu NS, Tihan T, Blevins L, Aghi MK. Plurihormonal PIT-1-Positive pituitary neuroendocrine tumors: A Systematic Review and Single-Center Series. World Neurosurg. 2021 Jul;151:e185-e191. doi: 10.1016/j.wneu.2021.04.003. Epub 2021 Apr 20. PMID: 33862299.)).

In the case series of Aydin et al. twenty-four patients (88.8%) had macroadenomas, including 6 giant adenomas (≥4 cm) (22.2%)
((Aydin S, Comunoglu N, Ahmedov ML, Korkmaz OP, Oz B, Kadioglu P, Gazioglu N, Tanriover N. Clinicopathologic Characteristics and Surgical Treatment of Plurihormonal pituitary neuroendocrine tumors. World Neurosurg. 2019 Oct;130:e765-e774. doi: 10.1016/j.wneu.2019.06.217. Epub 2019 Jul 8. PMID: 31295602.)).

===== Case reports =====


A 12-year-old girl with a rare plurihormonal pituitary [[macroadenoma]] secreting [[prolactin]] (PRL), [[growth hormone]] (GH), [[Thyroid stimulating hormone]] ([[TSH]]), and [[alpha subunit]] (α-SU).

The patient experienced recurrent [[headache]]s and progressing [[loss of vision]] in one [[eye]]. During the [[examination]], abnormalities such as tall stature, [[coarse facial features]], enlarged [[feet]] and [[hand]]s, [[tachycardia]], [[hand tremor]], [[hyperhidrosis]], [[galactorrhea]], and [[goiter]] were observed. [[Cranial magnetic resonance imaging]] (MRI) revealed a [[solid tumor]] in the anterior and [[middle cranial fossa]], measuring 80 × 50 × 55 mm. A [[stereotactic biopsy]] revealed plurihormonal [[Pit-1]] positive pituitary neuroendocrine tumor secreting PRL, GH, and TSH. A pituitary hyperfunction with PRL, GH, TSH, and α-SU excess was diagnosed. The patient was successfully treated pharmacologically with [[dopamine agonist]]s and [[somatostatin analogue]], and a decrease of [[tumor volume]] (30%) was achieved.

When neurosurgery is not possible, long-term pharmacological treatment of Giant [[plurihormonal pituitary neuroendocrine tumor]] can be a safe and relatively effective alternative
((Moszczyńska E, Grajkowska W, Maksymowicz M, Malicka J, Szalecki M, Prokop-Piotrkowska M. [[Giant plurihormonal pituitary neuroendocrine tumor]] in a [[child]] - [[case study]]. J Pediatr Endocrinol Metab. 2021 Jul 21. doi: 10.1515/jpem-2021-0094. Epub ahead of print. PMID: 34284528.)).
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a case of a monomorphous plurihormonal pituitary neuroendocrine tumor that co-secreted TSH and GH in a pediatric patient. A 13-year-old male presented with increasing height velocity (17.75 cm/year, 9.55SD), weight loss, and visual impairment. Initial biochemical evaluations revealed [[secondary hyperthyroidism]]. A giant pituitary tumor compressing the surrounding structures was detected by magnetic resonance, and a transsphenoidal surgery was initially performed. Pathological examinations revealed an atypical, monomorphous plurihormonal Pit-1 lineage tumor with mixed features of silent subtype 3 adenoma and acidophil stem cell adenoma. In the postoperative period, secondary hyperthyroidism recurred with high levels of both GH and IGF1. In addition, due to tumor re-growth, a multimodality treatment plan was undertaken including surgery, somatostatin analogs, and radiotherapy. We report the first pediatric case of a plurihormonal TSH- and GH-secreting pituitary neuroendocrine tumor, further expanding the clinical manifestations of pediatric pituitary tumors. Comprehensive pathological evaluation and close follow-up surveillance are crucial to the prompt delivery of the best therapeutic options in the context of this particularly aggressive pituitary tumor
((Pereira BD, Raimundo L, Mete O, Oliveira A, Portugal J, Asa SL. Monomorphous Plurihormonal pituitary neuroendocrine tumor of Pit-1 Lineage in a Giant Adolescent with Central Hyperthyroidism. Endocr Pathol. 2016 Mar;27(1):25-33. doi: 10.1007/s12022-015-9395-2. PMID: 26330191.)).