- Departments of Neurosurgery, Karolinska University Hospital, Stockholm, Sweden,
- Department of Oncology, Royal Berkshire NHS Foundation Trust, Reading, United Kingdom,
- Department of Neurosurgery, Bezmialem Vakif University Medical School, İstanbul, Turkey.
- Departments of Medical Radiation Physics and Nuclear Medicine, Karolinska University Hospital, Stockholm, Sweden,
- Departments of Neuropathology, Karolinska University Hospital, Stockholm, Sweden,
Departments of Neuropathology, Karolinska University Hospital, Stockholm, Sweden,
DOI:10.25259/SNI_112_2019Copyright: © 2019 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Georges Sinclair, Martin Olsson, Hamza Benmakhlouf, Yahya Al-saffar, Philippa Johnstone, Mustafa Aziz Hatiboglu, Alia Shamikh. Pituitary carcinomas: Rare and challenging. 09-Aug-2019;10:161
How to cite this URL: Georges Sinclair, Martin Olsson, Hamza Benmakhlouf, Yahya Al-saffar, Philippa Johnstone, Mustafa Aziz Hatiboglu, Alia Shamikh. Pituitary carcinomas: Rare and challenging. 09-Aug-2019;10:161. Available from: http://surgicalneurologyint.com/surgicalint-articles/9569/
Background: Pituitary carcinomas (PCs) are defined as adenohypophyseal tumors with metastatic activity within and outside the boundaries of the central nervous system (CNS). The condition is rare and therefore seldom reported; most lesions are hormone producing and have a tendency for complex evolution. As such, the management of PCs remains difficult. We present an illustrative case of PC with a brief review of the recent medical literature.
Case Description: A 58-year-old patient was diagnosed with prolactinoma in 2005. The ensuing biochemical and radiological evolution proved contentious; local tumor control was never fully achieved despite multimodal management including pharmacological treatment, repeated resections, and radiotherapy. In late 2017, the patient developed metastatic lesions within the confinements of the CNS requiring further surgical interventions, high-dose radiation, and systemic treatment.
Conclusion: As it was the case in our patient, PCs require tailored, multimodal treatments according to the degree of infiltration, site of invasion, and hormone status. Further studies are necessary to understand the mechanisms promoting “extra-sellar” activity, particularly at distant sites; the identification of biomarkers exposing the risk of PC remains a crucial aspect of diagnostics, prevention and future customized therapies.
Keywords: Adenoma, Central nervous system, Metastatic activity, Pituitary carcinoma
Pituitary carcinomas (PCs) are rare malignant neoplasms, accounting for approximately 0.12% of adenohypophyseal tumors and 6% of local invasive adenomas.[
We present a case of a previously healthy, 58-year-old male patient, who developed bitemporal hemianopsia in the first few months of 2005. The ensuing radiological assessment revealed a 22 mm × 20 mm × 15 mm pituitary mass with chiasmatic/bilateral optic nerve upward dislocation [
(×400). Microscopic reassessment of samples from second metastasectomy (November 2017) for the purpose of this article (a) H&E staining: high mitotic activity in a population of large cells with atypical nuclei and prominent nucleoli. (b) KI67: high proliferation (35%). (c) Overexpression of P53 limited to a few tumor cells (anti-p53 antibody). (d) Diffuse Immunostaining for prolactin. Of note, thyroid stimulating hormone, growth hormone, adrenocorticotropic hormone, CK-AE1AE3 proved negative. Samples from the first metastasectomy were not made available for reanalysis.
Hypophyseal tumors account for 15% of all intracranial tumors;[
PC activity prevails in central nervous system (CNS) locations although other sites of dissemination such as the liver, bone, heart, ovaries, and lymph nodes have also been reported.[
As is the case here, a thorough microscopic tumor evaluation from the primary and metastatic sites remains crucial in confirming diagnosis and assessing best treatment options. Histologically, PC lesions may look like typical adenomas but may also display marked pleomorphism and frequent mitoses.[
Treatment modalities of manifest PC
The treatment of PCs remains multimodal and includes surgical resection (transsphenoidal and transcranial), linear accelerator (LINAC)- and proton-beam- based fractionated radiotherapy, single-dose GKRS, chemotherapy, immunotherapy, and the use of other pharmacological agents targeting hormone production itself.[
PCs are rare neoplasms with contentious metastatic evolution and require multimodal, tailored treatments. Unfortunately, despite modern medical technology, the prognosis remains poor. Although the incidence of PCs is low, further studies are necessary to understand the proliferative mechanisms leading to local invasion and metastatic activity. The identification of prognostic biomarkers for risk stratification and treatment response remains necessary in terms of prevention of PCs and future selected therapies.
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
There are no conflicts of interest.
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