Raras
Buscar doenças, sintomas, genes...
Carcinoma hipofisário
ORPHA:300385CID-10 · C75.1DOENÇA RARA

É um tipo raro de câncer (adenocarcinoma) com prognóstico sério, que se desenvolve a partir das células da parte da frente da glândula hipófise ou de tumores benignos (adenomas) que já existiam nessa região. A maioria desses tumores produz hormônios em excesso, geralmente prolactina ou ACTH. O diagnóstico só é confirmado quando o câncer já se espalhou para outras partes do corpo (formando metástases). Problemas de saúde associados a esses cânceres da hipófise incluem a hiperprolactinemia (excesso de prolactina), a doença de Cushing e a acromegalia.

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Introdução

O que você precisa saber de cara

📋

É um tipo raro de câncer (adenocarcinoma) com prognóstico sério, que se desenvolve a partir das células da parte da frente da glândula hipófise ou de tumores benignos (adenomas) que já existiam nessa região. A maioria desses tumores produz hormônios em excesso, geralmente prolactina ou ACTH. O diagnóstico só é confirmado quando o câncer já se espalhou para outras partes do corpo (formando metástases). Problemas de saúde associados a esses cânceres da hipófise incluem a hiperprolactinemia (excesso de prolactina), a doença de Cushing e a acromegalia.

Pesquisas ativas
1 ensaio
5 total registrados no ClinicalTrials.gov
Publicações científicas
399 artigos
Último publicado: 2026 Apr
Medicamentos
2 registrados
IPILIMUMAB, NIVOLUMAB

Tem tratamento?

2 medicamentos registrados
Ver detalhes, fases e interações →
IPILIMUMABNIVOLUMAB

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C75.1
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

📏
Crescimento
11 sintomas
👁️
Olhos
2 sintomas
👂
Ouvidos
1 sintomas
🧠
Neurológico
1 sintomas

+ 8 sintomas em outras categorias

Características mais comuns

100%prev.
Carcinoma hipofisário
90%prev.
Aumento da concentração circulante de prolactina
Muito frequente (99-80%)
90%prev.
Cefaleia
Muito frequente (99-80%)
90%prev.
Neoplasia maligna do sistema nervoso central
Muito frequente (99-80%)
90%prev.
Adenoma de células corticotróficas hipofisárias
Muito frequente (99-80%)
90%prev.
Adenoma de células produtoras de prolactina hipofisárias
Muito frequente (99-80%)
23sintomas
Muito frequente (7)
Frequente (5)
Ocasional (7)
Muito raro (3)
Sem dados (1)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 23 características clínicas mais associadas, ordenadas por frequência.

Carcinoma hipofisárioPituitary carcinoma
Muito frequente100%
Aumento da concentração circulante de prolactinaIncreased circulating prolactin concentration
Muito frequente (99-80%)90%
CefaleiaHeadache
Muito frequente (99-80%)90%
Neoplasia maligna do sistema nervoso centralMalignant neoplasm of the central nervous system
Muito frequente (99-80%)90%
Adenoma de células corticotróficas hipofisáriasPituitary corticotropic cell adenoma
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico399PubMed
Últimos 10 anos200publicações
Pico202232 papers
Linha do tempo
2026Hoje · 2026🧪 1996Primeiro ensaio clínico📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
2Fase 24
Medicamentos catalogadosEnsaios clínicos· 2 medicamentos · 2 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Carcinoma hipofisário

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

5 ensaios clínicos encontrados, 1 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
205 papers (10 anos)
#1

Aggressive and malignant pituitary tumours: does the sex matter?

Pituitary2026 Mar 07

Aggressive pituitary tumours (APT) and pituitary carcinomas (PC) are rare pituitary neoplasms and occur more frequently in men. Corticotroph and lactotroph tumours represent the most frequent secreting subtypes, showing different sex-related distribution, clinical features, and behaviour compared with benign disease. This review evaluates whether these sex-related differences persist in APT/PC and their clinical and therapeutic implications. A literature review of case reports, series, and clinical studies, published between 2000 and October 2025, reporting sex-related data on corticotroph and lactotroph APT/PC, was conducted. Clinical presentation, pathological features, and outcomes were extracted and analysed by sex. 93 corticotroph APT/PC (38 W, 55 M) and 80 lactotroph APT/PC (29 W, 51 M) were included. Among corticotroph APT/PC, men more commonly had non-functioning lesions (36.5%, vs. 17.6%), showed loss of hormonal secretion (7.7%, n = 4/52), and had invasive disease (100%, vs. 73.3%, p: 0.03). Women more frequently harboured Crooke’s cell tumours (26.3% vs. 5.5%, p: 0.006) and showed complete radiological responses (22.2%, vs. 9.1%). In lactotroph APT/PC, men had giant tumours (27.3%, vs. 5.0%), invasive disease (83.3%, vs. 63.6%), higher prolactin levels, and dopamine agonist resistance. Complete tumour responses to temozolomide occurred exclusively in women (22.7%, n = 5/22, p: 0.02), whereas partial durable responses predominated in men (46.2%, n = 18/39). Male sex is associated with higher prevalence and more aggressive disease, whereas women achieve more profound tumour and hormonal responses. Sex-specific stratification may support personalized management of these rare pituitary tumours. The online version contains supplementary material available at 10.1007/s11102-026-01656-y.

#2

Life-Threatening Radiation Necrosis After Complete Response to Pembrolizumab in a Patient With a Metastatic Silent PIT1 Pituitary Neuroendocrine Tumor (PitNET).

Journal of immunotherapy (Hagerstown, Md. : 1997)2026 Feb 23

Metastatic pituitary neuroendocrine tumors (metPitNETs) are rare neoplasms with limited therapeutic options. Temozolomide is the first-line therapy, but primary or secondary resistance frequently occurs. Immune checkpoint inhibitors (ICIs) are emerging as a promising second-line option; however, clinical experience remains limited. We report the long-term follow-up of a 62-year-old male patient who received pembrolizumab (PBZ) treatment for a silent metPitNET derived from the PIT1 lineage after multiple surgical and radiation therapies and temozolomide failure. PBZ was proposed based on extensive PD-L1 expression by tumor cells. A remarkable clinical, radiologic, and metabolic response was soon observed, progressively leading to complete disease remission after 21 months of treatment, with moderate immune-related adverse events. However, an unexpected rapid neurological deterioration occurred, due to the progression of a pseudotumoral temporal radionecrosis surrounded by an impressive vasogenic oedema, requiring emergency neurosurgery 7 weeks after PBZ withdrawal. The temporal mass had progressively developed on a previous small temporal metastasis treated through stereotactic radiosurgery, the corresponding area was hypometabolic at 18FDG PET-CT imaging, and histopathologic examination confirmed extensive radionecrosis and the absence of residual tumor cells. This is the first documented complete response to ICI in a PIT1-derived metPitNET. However, this remarkable response was complicated by the severe evolution of a brain radionecrosis, probably favoured by long-term PBZ. This case underscores the need for multidisciplinary expertise to differentiate treatment effects from neoplastic progression and to carefully follow-up the patients for potentially severe late treatment-related complications. It also questions the optimal duration of treatment in responsive cases. The pituitary gland occupies the sella turcica, a bony depression within the sphenoid bone, and consists of anterior and posterior segments. The anterior portion, known as the adenohypophysis, contains glandular tissue, while the posterior segment, or neurohypophysis, consists of neural tissue. Connection to the hypothalamus occurs through the pituitary stalk, which provides a portal circulation to the anterior pituitary and a neuronal pathway to the posterior pituitary. Hormones secreted by the hypothalamus regulate the anterior pituitary, whereas the hypothalamus produces the posterior pituitary hormones that remain stored in the posterior lobe until release. Tumors arising from the anterior pituitary gland represent approximately 15% of all intracranial neoplasms and are most often benign adenomas. However, in rare cases, these tumors acquire the ability to metastasize, becoming classified as pituitary carcinoma. Pituitary carcinoma differs from secondary metastases to the pituitary gland originating from systemic malignancies, eg, lung or breast cancer, and, less frequently, from prostate, kidney, or colon cancer, among others.

#3

Imaging and clinical course of metastatic pituitary neuroendocrine tumors (PitNET): A single center case series.

The neuroradiology journal2026 Apr

PurposeMetastatic pituitary neuroendocrine tumors (PitNET) are exceedingly rare tumors that are typically diagnosed at the time of metastasis. Given the rarity of metastatic PitNET tumors, the pattern of metastasis and imaging appearance of CNS and extra neural metastasis is under reported and poorly understood.MethodsIn this retrospective case series, we present 14 consecutive patients with pathology-confirmed metastatic PitNET tumors. We assess imaging features of primary pituitary disease at the time of diagnosis, temporal evolution of local disease, and distant metastasis on multimodality imaging. We also explore potential association of initial pathology diagnosis and time to metastasis and survival.ResultsAt the time of metastasis, nine patients had evidence of disease in the sella and three additional had evidence of disease in the cavernous sinus. The most common site of metastatic disease was the CNS (78.6%). Dural deposits were the most common brain CNS metastatic disease manifestation (81.8%). Eight patients (57.1%) demonstrated multiple metastatic disease sites, with five of them (62.5%) showing both CNS and extra CNS metastases. Osseous metastatic disease was the most common extra CNS disease site (75.0%). Median time to metastasis did not significantly differ between patients with adenoma and those with more aggressive pathologies at initial diagnosis (p value = .39). Similarly, median overall survival from metastasis detection was not significantly affected by pathology (p value = .84).ConclusionMetastatic PitNET is a rare neuroendocrine neoplasm. In the present case series, we detail temporal imaging findings of the disease at the primary site and patterns of metastasis.

#4

The 2022 WHO classification of tumors of the pituitary gland: An update on aggressive and metastatic pituitary neuroendocrine tumors.

Brain pathology (Zurich, Switzerland)2025 Jan

The vast majority of pituitary neuroendocrine tumors (PitNETs) are benign and slow growing with a low relapse rate over many years after surgical resection. However, about 40% are locally invasive and may not be surgically cured, and about one percentage demonstrate an aggressive clinical behavior. Exceptionally, these aggressive tumors may metastasize outside the sellar region to the central nervous system and/or systemically. The 2017 (4th Edition) WHO Classification of Pituitary Tumors abandoned the terminology "atypical adenoma" for tumors previously considered to have potential for a more aggressive behavior since its prognostic value was not established. The 2022 (5th Edition) WHO Classification of the Pituitary Tumors emphasizes the concept that morphological features distinguish indolent tumors from locally aggressive ones, however, the proposed histological subtypes are not consistent with the real life clinical characteristics of patients with aggressive tumors/carcinomas. So far, no single clinical, radiological or histological parameter can determine the risk of growth or malignant progression. Novel promising molecular prognostic markers, such as mutations in ATRX, TP53, SF3B1, and epigenetic DNA modifications, will need to be verified in larger tumor cohorts. In this review, we provide a critical analysis of the WHO guidelines for prognostic stratification and diagnosis of aggressive and metastatic PitNETs. In addition, we discuss the new WHO recommendations for changing ICD-O and ICD-11 codes for PitNET tumor behavior from a neoplasm either "benign" or "unspecified, borderline, or uncertain behavior" to "malignant" neoplasm regardless of the clinical presentation, histopathological subtype, and tumor location. We encourage multidisciplinary initiatives for integrated clinical, histological and molecular classification, which would enable early recognition of these challenging tumors and initiation of more appropriate and aggressive treatments, ultimately improving the outcome.

#5

Long-term survival in a patient with metastatic prolactinoma: a 12-year follow-up.

Proceedings (Baylor University. Medical Center)2025

Metastatic prolactinomas are an extremely rare type of neuroendocrine tumors, comprising <0.2% of all pituitary tumors. These tumors are typically associated with poor prognosis, with an average survival of 10 months. Temozolomide, an alkylating chemotherapy agent, has previously been shown to be effective in treating these rare cases once first-line medical and surgical therapies have failed. We present a patient with metastatic prolactinoma to the cerebellopontine angle, thoracic spine, and lumbar spine who has achieved 12 years of survival with clinical improvement and stabilization after 4 years of temozolomide therapy. Metastatic prolactinomas are extremely rare but aggressive entities within the spectrum of pituitary tumors, comprising 0.1% to 0.2% of all pituitary tumors.Metastatic prolactinomas are often indistinguishable from aggressive benign adenomas until metastases are detected. The prognosis for patients with metastatic prolactinomas is generally poor, with a mean survival time of around 10 months following the diagnosis of metastatic spread.Temozolomide is an alkylating chemotherapy agent used as an off-label treatment for metastatic prolactinoma, although patients’ responses to therapy can vary significantly.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC205 artigos no totalmostrando 198

2026

Aggressive and malignant pituitary tumours: does the sex matter?

Pituitary
2026

Life-Threatening Radiation Necrosis After Complete Response to Pembrolizumab in a Patient With a Metastatic Silent PIT1 Pituitary Neuroendocrine Tumor (PitNET).

Journal of immunotherapy (Hagerstown, Md. : 1997)
2025

Long-term survival in a patient with metastatic prolactinoma: a 12-year follow-up.

Proceedings (Baylor University. Medical Center)
2025

Transformation of Lactotroph Pituitary Adenoma to Metastatic Pituitary Carcinoma: A Case Report.

Cureus
2025

Rare Occurrence of Distant Metastases in Prolactinoma: Insights From 2 Cases and Review of the Literature.

AACE endocrinology and diabetes
2026

Imaging and clinical course of metastatic pituitary neuroendocrine tumors (PitNET): A single center case series.

The neuroradiology journal
2025

Revised European Society of Endocrinology Clinical Practice Guideline for the management of aggressive pituitary tumours and pituitary carcinomas.

European journal of endocrinology
2025

Prolactin-secreting pituitary carcinomas with intra- and extracranial metastasis: case report and review.

Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko
2024

Genome-wide methylation profiling differentiates benign from aggressive and metastatic pituitary neuroendocrine tumors.

Acta neuropathologica
2024

Clinical features of pituitary carcinoma: analysis based on a case report and literature review.

Frontiers in endocrinology
2024

[Efficacy of standard methods in the treatment of prolactin-secreting pituitary carcinoma].

Problemy endokrinologii
2024

High-Grade Progression, Sarcomatous Transformation, and/or Metastasis of Pituitary Neuroendocrine Neoplasms (PitNENs): The UCSF Experience.

Endocrine pathology
2024

Endoscopic endonasal approach for resection of sellar leiomyosarcoma metastasis: illustrative case.

Journal of neurosurgery. Case lessons
2025

The 2022 WHO classification of tumors of the pituitary gland: An update on aggressive and metastatic pituitary neuroendocrine tumors.

Brain pathology (Zurich, Switzerland)
2024

Prolactin Secreting Pituitary Carcinoma and the Role of Peptide Receptor Radionuclide Therapy: A Brief Report.

Neurology India
2024

Adrenocorticotropic Hormone Producing Pituitary Carcinoma in the Falx Cerebri, Retroclival Region, Ethmoidal Cells, and Other Locations.

Cureus
2024

Aggressive Prolactinoma with Progression to Pituitary Carcinoma: A Case Report.

Endocrine, metabolic &amp; immune disorders drug targets
2024

[Aggressive pituitary tumors and carcinomas: modern classification, advances and prospects in treatment].

Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko
2024

Thyroid and neurobehavioral effects of DiNP on GH3 cells and larval zebrafish (Danio rerio).

Chemosphere
2024

Genome-wide loss of heterozygosity predicts aggressive, treatment-refractory behavior in pituitary neuroendocrine tumors.

Acta neuropathologica
2024

A Rare Prolactin-secreting Pituitary Carcinoma With Epidural and Thecal Metastases.

JCEM case reports
2023

Historical Control Background Incidence of Spontaneous Neoplastic Lesions of Sprague-Dawley Rats in 104-Week Toxicity Studies.

Toxicologic pathology
2024

Aggressive pituitary tumors and carcinomas: medical treatment beyond temozolomide.

Minerva endocrinology
2024

Immunotherapy for endocrine tumours: a clinician's perspective.

Endocrine-related cancer
2023

Spontaneous Pituitary Neoplasm in Two Female Geriatric Southern Giant Pouched Rats (Cricetomys ansorgei).

Comparative medicine
2023

Case report: Identification of potential prognosis-related LAG3 overexpression and DICER1 mutation in pituitary carcinoma: two cases.

Frontiers in neuroscience
2023

Efficacy and safety of temozolomide in the treatment of aggressive pituitary neuroendocrine tumours in Spain.

Frontiers in endocrinology
2023

Therapeutic targeting of the pituitary tumor microenvironment.

Pharmacology &amp; therapeutics
2023

Metastatic pituitary tumors: an institutional case series.

Pituitary
2023

[Aggressive pituitary adenoma and pituitary carcinoma].

Orvosi hetilap
2023

[WHO Histological Classification of Pituitary Tumors].

No shinkei geka. Neurological surgery
2023

Somatostatin Receptor-PET/CT/MRI of Head and Neck Neuroendocrine Tumors.

AJNR. American journal of neuroradiology
2023

The treatment of aggressive prolactinomas with everolimus.

Pituitary
2023

The Role of Immunotherapy in the Treatment of Rare Central Nervous System Tumors.

Current oncology (Toronto, Ont.)
2023

Non-functioning pituitary carcinoma: Case report and literature review.

International journal of surgery case reports
2023

Recent Therapeutic Advances in Pituitary Carcinoma.

Journal of immunotherapy and precision oncology
2023

Medical therapy for refractory pituitary adenomas.

Pituitary
2023

The World Health Organization classifications of pituitary neuroendocrine tumours: a clinico-pathological appraisal.

Endocrine-related cancer
2023

Knowing when to discontinue Temozolomide therapy in responding aggressive pituitary tumors and carcinomas: a systematic review and Padua (Italy) case series.

Expert review of endocrinology &amp; metabolism
2023

Outcome and prognostic factors for pituitary carcinomas: lessons from a systematic review.

Endocrine-related cancer
2023

Chronic Use of Deslorelin in Dogs: Six Cases (2005-2022).

Animals : an open access journal from MDPI
2023

Why is pituitary carcinoma so rare?

Expert review of endocrinology &amp; metabolism
2023

Natural history of pituitary carcinoma with metastasis to the cervical spine: illustrative case.

Journal of neurosurgery. Case lessons
2022

[New pathological classification and clinical implications of pituitary neuroendocrine tumors of the 2022 WHO version].

Zhonghua yi xue za zhi
2025

A case of aggressive pituitary neuroendocrine tumour with extremely rapid progression: possible diagnostic value of TERT promoter methylation.

British journal of neurosurgery
2023

Pituitary carcinoma: A cytopathologic dilemma when metastatic to cervical lymph nodes.

Diagnostic cytopathology
2022

Pituitary adenoma & nuclear medicine: Recent outcomes and ongoing developments.

Presse medicale (Paris, France : 1983)
2022

Temozolomide in aggressive pituitary tumours and pituitary carcinomas.

Best practice &amp; research. Clinical endocrinology &amp; metabolism
2022

Epigenomic and somatic mutations of pituitary tumors with clinical and pathological correlations in 111 patients.

Clinical endocrinology
2022

Pituitary carcinoma - case series and review of the literature.

Frontiers in endocrinology
2022

Cytomorphology of pituitary carcinoma metastatic to liver diagnosed by fine-needle aspiration: A rare case report and review of literature.

CytoJournal
2022

Immunotherapy for Aggressive and Metastatic Pituitary Neuroendocrine Tumors (PitNETs): State-of-the Art.

Cancers
2022

Aggressive corticotroph tumors and carcinomas.

Journal of neuroendocrinology
2022

Succinate dehydrogenase and MYC-associated factor X mutations in pituitary neuroendocrine tumours.

Endocrine-related cancer
2022

A Pituitary Carcinoma Patient With Cerebrospinal Fluid Dissemination Showing a Good Response to Temozolomide Combined With Whole-Brain and Spinal Cord Radiotherapy: A Case Report and Literature Review.

Frontiers in oncology
2022

Update in Pathogenesis, Diagnosis, and Therapy of Prolactinoma.

Cancers
2022

An aggressive lactotroph pituitary tumor in a young male: A pituitary carcinoma without metastasis.

Clinical neuropathology
2022

Paraneoplastic cast nephropathy associated with malignant prolactinoma: A case report and literature review.

Clinical nephrology
2022

Immunotherapy in aggressive pituitary tumors and carcinomas: a systematic review.

Endocrine-related cancer
2022

Pituitary blastoma, expanding the spectrum of newly recognized entities. A case report.

Indian journal of pathology &amp; microbiology
2022

Pituitary carcinoma: reclassification and implications in the NET schema.

Endocrine oncology (Bristol, England)
2022

Corticotroph pituitary carcinoma with skeletal metastases masquerading as ectopic ACTH syndrome: a long and winding road to diagnosis.

Endocrinology, diabetes &amp; metabolism case reports
2022

Overview of the 2022 WHO Classification of Pituitary Tumors.

Endocrine pathology
2022

COVID-19 and Cushing's disease in a patient with ACTH-secreting pituitary carcinoma.

Endocrinology, diabetes &amp; metabolism case reports
2022

ACTH-secreting pituitary carcinoma with TP53, NF1, ATRX and PTEN mutations Case report and review of the literature.

Endocrine
2021

Pituitary adenoma or pituitary neuroendocrine tumor: a narrative review of controversy and perspective.

Translational cancer research
2022

Pituitary Carcinoma in a Patient with Cowden Syndrome.

The American journal of case reports
2022

Distant Pituitary Adenoma Spread: A Systematic Review and Report of 2 Cases.

Operative neurosurgery (Hagerstown, Md.)
2022

A Rare Case of Pituitary Carcinoma With Bone Metastases: 68Ga-DOTATATE PET/CT Findings.

Clinical nuclear medicine
2021

A Silent Corticotroph Pituitary Carcinoma: Lessons From an Exceptional Case Report.

Frontiers in endocrinology
2022

Pituitary carcinoma as a rare cause of liver metastases successfully treated with temozolomide.

Polish archives of internal medicine
2022

Aggressive prolactinoma (Review).

Experimental and therapeutic medicine
2023

Cranial and Spinal Metastasis of a Nonfunctioning Pituitary Adenoma: Report of a Case.

Journal of neurological surgery. Part A, Central European neurosurgery
2022

Adrenocorticotropic Hormone Secreting Pituitary Carcinoma: Rare, Durable Response to Concurrent Chemotherapy and Reirradiation With a Review of the Literature.

Advances in radiation oncology
2023

Should pituitary carcinoma be treated using a NET-like approach? A case of complete remission of a metastatic malignant prolactinoma with multimodal therapy including immunotherapy.

Clinical endocrinology
2021

Tumor invasion to the spinal region after primary surgery: A case report.

Caspian journal of internal medicine
2021

Evolution of a refractory prolactin-secreting pituitary adenoma into a pituitary carcinoma: report of a challenging case and literature review.

BMC endocrine disorders
2021

Growth hormone secreting pituitary carcinomas: Case report and review of literature.

Growth hormone &amp; IGF research : official journal of the Growth Hormone Research Society and the International IGF Research Society
2021

Primary Tumors of the Pituitary Gland: Radiologic-Pathologic Correlation.

Radiographics : a review publication of the Radiological Society of North America, Inc
2022

Epidemiology of common and uncommon adult pituitary tumors in the U.S. according to the 2017 World Health Organization classification.

Pituitary
2021

Metastases to the Pituitary Gland: Histological Patterns of Spread and Review of the Literature.

Journal of neuropathology and experimental neurology
2021

Aggressive pituitary tumours and pituitary carcinomas.

Nature reviews. Endocrinology
2021

Synergism of Checkpoint Inhibitors and Peptide Receptor Radionuclide Therapy in the Treatment of Pituitary Carcinoma.

Journal of the Endocrine Society
2024

Aggressive pituitary adenoma in the context of Lynch syndrome: a case report and literature review on this rare coincidence.

British journal of neurosurgery
2021

Aggressive pituitary neuroendocrine tumors: current practices, controversies, and perspectives, on behalf of the EANS skull base section.

Acta neurochirurgica
2022

Immune-checkpoint inhibitors in pituitary malignancies.

Anti-cancer drugs
2021

68Ga-DOTATATE PET/CT in Pituitary Carcinoma.

Clinical nuclear medicine
2021

Aggressive Cushing's Disease: Molecular Pathology and Its Therapeutic Approach.

Frontiers in endocrinology
2021

Stereotactic Radiosurgery for Pituitary Carcinoma.

Acta neurochirurgica. Supplement
2021

[Corrigendum] Identification of microRNAs associated with the aggressiveness of prolactin pituitary tumors using bioinformatic analysis.

Oncology reports
2021

Recent Understanding and Future Directions of Recurrent Corticotroph Tumors.

Frontiers in endocrinology
2021

Challenges in the Diagnosis of Pituitary Neuroendocrine Tumors.

Endocrine pathology
2021

Clinical Implications of the New WHO Classification 2017 for Pituitary Tumors.

Experimental and clinical endocrinology &amp; diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
2022

Non-functional recurrent pituitary adenoma with intracranial metastasis-Pituitary carcinoma: A case report and review of the literature.

Neuro-Chirurgie
2020

Is Seed and Soil Theory Suitable for Metastatic Spread of Pituitary Carcinomas?

Frontiers in endocrinology
2022

Delayed Craniospinal Metastasis of Aggressive Nonfunctioning Pituitary Adenomas as Pituitary Carcinomas.

Journal of neurological surgery. Part B, Skull base
2022

Pituitary Carcinoma Diagnosis and Survival Improvement, with Affordable Care Act Correlation: A SEER Database Study.

Journal of neurological surgery. Part B, Skull base
2020

Efficacy of pembrolizumab in patients with pituitary carcinoma: report of four cases from a phase II study.

Journal for immunotherapy of cancer
2020

Case Report: A Case of Pituitary Carcinoma Treated With Sequential Dual Immunotherapy and Vascular Endothelial Growth Factor Inhibition Therapy.

Frontiers in endocrinology
2021

Immune checkpoint inhibitor therapy for ACTH-secreting pituitary carcinoma: a new emerging treatment?

European journal of endocrinology
2021

Corticotroph Aggressive Pituitary Tumors and Carcinomas Frequently Harbor ATRX Mutations.

The Journal of clinical endocrinology and metabolism
2020

Clinical features and their course of pituitary carcinoma with distant metastasis in a dog.

The Journal of veterinary medical science
2020

CAUTION ADVISED USING COMBINATION KETOCONAZOLE AND PD-1 INHIBITORS.

AACE clinical case reports
2020

Approach to the Treatment of a Patient with an Aggressive Pituitary Tumor.

The Journal of clinical endocrinology and metabolism
2020

Immunotherapy in Corticotroph and Lactotroph Aggressive Tumors and Carcinomas: Two Case Reports and a Review of the Literature.

Journal of personalized medicine
2020

Structure, Function, and Morphology in the Classification of Pituitary Neuroendocrine Tumors: the Importance of Routine Analysis of Pituitary Transcription Factors.

Endocrine pathology
2020

Malignant Prolactinoma With Liver Metastases Masquerading as Metastatic Gastrointestinal Stromal Tumor: A Case Report and Literature Review.

Frontiers in endocrinology
2022

Cervical Nodal Metastatic Pituitary Carcinoma: A Case Report.

Ear, nose, &amp; throat journal
2020

Aggressive Pituitary Adenomas and Carcinomas.

Endocrinology and metabolism clinics of North America
2020

Malignancy course of pituitary adenoma in MEN1 syndrome: Clinical-Neuroradiological signs.

European journal of radiology open
2020

Contemporary Management of Clinically Non-functioning Pituitary Adenomas: A Clinical Review.

Clinical medicine insights. Endocrinology and diabetes
2020

Liver metastases from pituitary carcinomas mimicking visceral well-differentiated neuroendocrine tumors: a series of four cases.

Diagnostic pathology
2020

Newer Concepts in the Classification of Pituitary Adenomas.

Neurology India
2020

Are aggressive pituitary tumors and carcinomas two sides of the same coin? Pathologists reply to clinician's questions.

Reviews in endocrine &amp; metabolic disorders
2020

Pituitary Adenomas with Changing Phenotype: A Systematic Review.

Experimental and clinical endocrinology &amp; diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
2020

Metastatic Pituitary Carcinoma Causing Cord Compression.

World neurosurgery
2020

Long-course temozolomide in aggressive pituitary adenoma: real-life experience in two tertiary care centers and review of the literature.

Pituitary
2020

Results and predictors of outcome of endoscopic endonasal surgery in Cushing's disease: 20-year experience of an Italian referral Pituitary Center.

Journal of endocrinological investigation
2020

A Population-Level Analysis of Pituitary Carcinoma from the National Cancer Database.

Journal of neurological surgery. Part B, Skull base
2020

Pituitary tumours - a large retrospective single-centre study of over 2300 cases. Experience of a tertiary reference centre.

Endokrynologia Polska
2020

Temozolomide therapy for aggressive pituitary tumours - current understanding and future perspectives.

Reviews in endocrine &amp; metabolic disorders
2020

Pituitary carcinoma: Two case reports and review of literature.

World journal of clinical oncology
2020

Exploring the Role of Novel Medical Therapies for Aggressive Pituitary Tumors: A Review of the Literature-"Are We There Yet?".

Cancers
2019

Evaluation of the potential of the Ki67 index to predict tumor evolution in patients with pituitary adenoma.

International journal of clinical and experimental pathology
2019

Evaluation of prognostic utility of Ki-67, P53, and O-6-methylguanine-DNA methyltransferase expression in pituitary tumors.

Journal of laboratory physicians
2020

Definition and diagnosis of aggressive pituitary tumors.

Reviews in endocrine &amp; metabolic disorders
2020

Efficacy of Temozolomide Therapy in Patients With Aggressive Pituitary Adenomas and Carcinomas-A German Survey.

The Journal of clinical endocrinology and metabolism
2020

Multimodal therapy in aggressive pituitary tumors.

Endocrinologia, diabetes y nutricion
2019

Nelson-Salassa Syndrome Progressing to Pituitary Carcinoma: A Case Report and Review of the Literature.

Cureus
2019

The Microenvironment of Pituitary Tumors-Biological and Therapeutic Implications.

Cancers
2020

Aggressive prolactinomas: how to manage?

Pituitary
2020

Extraordinary case presentations in pediatric pituitary adenoma: report of 6 cases.

Journal of neurosurgery. Pediatrics
2019

Pituitary carcinomas: Rare and challenging.

Surgical neurology international
2019

[Pituitary carcinoma: is it too late?].

Revista de neurologia
2019

Treatment and long-term outcomes in pituitary carcinoma: a cohort study.

European journal of endocrinology
2019

Unusual Course of an Aggressive Pituitary Prolactinoma: Case Report and Review of the Literature.

Case reports in neurology
2019

Lower all-cause mortality rates in patients harboring pituitary carcinoma following the introduction of temozolomide.

Endocrine
2020

Pituitary carcinomas: review of the current literature and report of atypical case.

British journal of neurosurgery
2019

An Institutional Experience of Tumor Progression to Pituitary Carcinoma in a 15-Year Cohort of 1055 Consecutive Pituitary Neuroendocrine Tumors.

Endocrine pathology
2018

Identification of Novel Genes Involved in the Pathogenesis of an ACTH-Secreting Pituitary Carcinoma: A Case Report and Literature Review.

Frontiers in oncology
2019

Long-Term Survival After Transformation of an Adrenocorticotropic Hormone-Secreting Pituitary Macroadenoma to a Silent Corticotroph Pituitary Carcinoma.

World neurosurgery
2018

Current Standards and Recent Advances in Biomarkers of Major Endocrine Tumors.

Frontiers in pharmacology
2018

Marked Response of a Hypermutated ACTH-Secreting Pituitary Carcinoma to Ipilimumab and Nivolumab.

The Journal of clinical endocrinology and metabolism
2018

Pathological Findings in the Pituitary Glands of Dogs and Cats.

Veterinary pathology
2018

Prolactin-Producing Pituitary Carcinoma, Hypopituitarism, and Graves' Disease-Report of a Challenging Case and Literature Review.

Frontiers in endocrinology
2018

Combined modalities of surgery, radiotherapy, radiosurgery and chemotherapy for invasive pituitary carcinoma.

Annales d'endocrinologie
2018

Temozolomide therapy for aggressive functioning pituitary adenomas refractory to surgery and radiation: a case series.

Neuro-oncology practice
2018

Prolactin Secreting Pituitary Carcinoma with Extracranial Spread Presenting with Pathological Fracture of Femur.

Journal of neurosciences in rural practice
2018

Corticotrophic pituitary carcinoma with cervical metastases: case series and literature review.

Pituitary
2018

MicroRNAs and Target Genes in Pituitary Adenomas.

Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme
2018

Mid-term prognosis of non-functioning pituitary adenomas with high proliferative potential: really an aggressive variant?

Journal of neuro-oncology
2018

Neuro-Ophthalmic Manifestations of Pituitary Carcinoma.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2018

Malignant transformation in non-functioning pituitary adenomas (pituitary carcinoma).

Pituitary
2017

Successful treatment of leptomeningeally metastasised pituitary carcinoma with temozolomide.

The Netherlands journal of medicine
2017

Metastatic prostate adenocarcinoma to intradural foramen magnum.

Journal of surgical case reports
2017

Failure of a second temozolomide cycle in a patient with a prolactin-secreting pituitary carcinoma.

Endocrinologia, diabetes y nutricion
2018

European Society of Endocrinology Clinical Practice Guidelines for the management of aggressive pituitary tumours and carcinomas.

European journal of endocrinology
2017

Corticotroph Pituitary Carcinoma in a Patient With Lynch Syndrome (LS) and Pituitary Tumors in a Nationwide LS Cohort.

The Journal of clinical endocrinology and metabolism
2017

Pituitary carcinoma: A case report and discussion of potential value of combined use of Ga-68 DOTATATE and F-18 FDG PET/CT scan to better choose therapy.

Surgical neurology international
2017

Meningeal dissemination of a pituitary carcinoma to the cauda equina in a dog.

The Canadian veterinary journal = La revue veterinaire canadienne
2017

Significant response of pituitary carcinoma to carboplatin, leucovorin and fluorouracil chemotherapy: a pediatric case report and review of the literature.

Journal of neuro-oncology
2017

Seeding of a Pituitary Adenoma or Atypical Pituitary Carcinoma?

Cureus
2018

A Case of Pituitary Carcinoma Initially Diagnosed as an Ectopic Growth Hormone Producing Pituitary Adenoma with a High Ki-67 Labeling Index.

Journal of neurological surgery. Part A, Central European neurosurgery
2017

Advancements in the study of miRNA regulation during the cell cycle in human pituitary adenomas.

Journal of neuro-oncology
2017

Novel aspects in histopathology of the pituitary gland.

Endocrinologia, diabetes y nutricion
2017

Case report: ACTH-secreting pituitary carcinoma metastatic to the liver in a patient with a history of atypical pituitary adenoma and Cushing's disease.

Diagnostic pathology
2017

Molecular targeted therapies in adrenal, pituitary and parathyroid malignancies.

Endocrine-related cancer
2017

Metastatic Pituitary Carcinoma to Cervical Lymph Node: Diagnosis by Fine Needle Aspiration and Review of the Literature.

Acta cytologica
2017

Successful treatment of pituitary carcinoma with concurrent radiation, temozolomide, and bevacizumab after resection.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2017

Pituitary Carcinoma in a Patient with an SDHB Mutation.

Endocrine pathology
2017

Cytomorphology of metastatic pituitary carcinoma to the bone.

Diagnostic cytopathology
2017

Risk factors for cerebrospinal fluid leak in pediatric patients undergoing endoscopic endonasal skull base surgery.

International journal of pediatric otorhinolaryngology
2016

Anterior and posterior suprasellar extensions of a symmetrical trilobed nonfunctional giant pituitary adenoma in the sagittal plane: a case report and review of literature.

Radiology case reports
2016

Genetic aspects of pituitary carcinoma: A systematic review.

Medicine
2016

Paradoxical and atypical responses to pasireotide in aggressive ACTH-secreting pituitary tumors.

Pituitary
2016

Widely metastatic atypical pituitary adenoma with mTOR pathway STK11(F298L) mutation treated with everolimus therapy.

CNS oncology
2017

How effective is temozolomide for treating pituitary tumours and when should it be used?

Pituitary
2016

Temozolomide treatment of pituitary carcinomas and atypical adenomas: systematic review of case reports.

Neuro-oncology practice
2016

Secreting Follicle-Stimulating Hormone Pituitary Carcinoma with Cervical Metastasis.

World neurosurgery
2016

Temozolomide therapy: Focus on patients with pituitary carcinoma.

Journal of neurosciences in rural practice
2016

Prolactin-Secreting Pituitary Carcinoma with Dural Metastasis: Diagnosis, Treatment, and Future Directions.

World neurosurgery
2016

Biomarkers of pituitary carcinomas.

Expert review of endocrinology &amp; metabolism
2016

Is there a role for early chemotherapy in the management of pituitary adenomas?

Neuro-oncology
2016

Radiotherapy with concurrent temozolomide for the management of extraneural metastases in pituitary carcinoma.

Pituitary
2015

Outcomes in pituitary surgery in Nelson's syndrome--therapeutic pitfalls.

Endokrynologia Polska
2016

Temozolomide therapy in patients with aggressive pituitary adenomas or carcinomas.

Journal of neuro-oncology
2015

[Diagnosis and Treatment of Pituitary Carcinoma].

Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae
2015

Pituitary adenoma-neuronal choristoma is a pituitary adenoma with ganglionic differentiation.

Experimental and molecular pathology
2015

Pituitary carcinoma with intraspinal metastasis: report of two cases and review of the literature.

International journal of clinical and experimental pathology
2015

A retrospective study of skull base neoplasia in 42 dogs.

Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, Inc
2015

Progression of an Invasive ACTH Pituitary Macroadenoma with Cushing's Disease to Pituitary Carcinoma.

Case reports in oncological medicine
2015

Histological criteria for atypical pituitary adenomas - data from the German pituitary adenoma registry suggests modifications.

Acta neuropathologica communications
2015

Prolactin-producing pituitary adenoma with atypical spindle cell morphology: a case report.

World journal of surgical oncology
2015

Management of macroprolactinomas.

Clinical diabetes and endocrinology
2015

[New aspects of tumor pathology of the pituitary].

Der Pathologe
2015

MicroRNA involvement in a metastatic non-functioning pituitary carcinoma.

Pituitary
2015

The role of temozolomide in the treatment of aggressive pituitary tumors.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2015

Invasive, atypical and aggressive pituitary adenomas and carcinomas.

Endocrinology and metabolism clinics of North America
Ver todos os 205 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Aggressive and malignant pituitary tumours: does the sex matter?
    Pituitary· 2026· PMID 41793533mais citado
  2. Life-Threatening Radiation Necrosis After Complete Response to Pembrolizumab in a Patient With a Metastatic Silent PIT1 Pituitary Neuroendocrine Tumor (PitNET).
    Journal of immunotherapy (Hagerstown, Md. : 1997)· 2026· PMID 41725077mais citado
  3. Imaging and clinical course of metastatic pituitary neuroendocrine tumors (PitNET): A single center case series.
    The neuroradiology journal· 2026· PMID 40576016mais citado
  4. The 2022 WHO classification of tumors of the pituitary gland: An update on aggressive and metastatic pituitary neuroendocrine tumors.
    Brain pathology (Zurich, Switzerland)· 2025· PMID 39218431mais citado
  5. Long-term survival in a patient with metastatic prolactinoma: a 12-year follow-up.
    Proceedings (Baylor University. Medical Center)· 2025· PMID 41341093mais citado
  6. A Case of Thyrotropin-Secreting Pituitary Carcinoma With Bone Metastases.
    JCEM Case Rep· 2026· PMID 41940233recente
  7. Temozolomide in aggressive and metastatic pituitary tumors: a Brazilian multicenter real-world cohort study.
    Pituitary· 2026· PMID 41894067recente
  8. Pituitary Cancer.
    · 2026· PMID 32644525recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:300385(Orphanet)
  2. MONDO:0017582(MONDO)
  3. GARD:9371(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q18556544(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Carcinoma hipofisário
Compêndio · Raras BR

Carcinoma hipofisário

ORPHA:300385 · MONDO:0017582
Prevalência
Unknown
Herança
Unknown
CID-10
C75.1 · Neoplasia maligna da glândula hipófise (pituitária)
Ensaios
1 ativos
Medicamentos
2 registrados
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0346300
EuropePMC
Wikidata
Papers 10a
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