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Seminars in Oncology Dec 2010Pituitary carcinoma is defined by metastasis of a pituitary tumor to a distant location. Although rare, pituitary carcinoma is becoming an increasingly recognized entity... (Review)
Review
Pituitary carcinoma is defined by metastasis of a pituitary tumor to a distant location. Although rare, pituitary carcinoma is becoming an increasingly recognized entity among pituitary surgeons. Most pituitary carcinomas have endocrine activity, and as such these lesions may be associated with signs of excess endocrine function. Symptoms also may be a result of direct mass effect. Surgical debulking is the standard treatment for the sellar component of disease. An important recent development has been the discovery that these lesions may significantly respond to systemic chemotherapy. In this review, we will discuss the diagnosis and clinical features of pituitary carcinomas, immunohistochemistry with cytogenetics, and treatment.
Topics: Biomarkers, Tumor; Carcinoma; Combined Modality Therapy; Humans; Mitotic Index; Neoplasm Metastasis; Pituitary Neoplasms
PubMed: 21167378
DOI: 10.1053/j.seminoncol.2010.10.012 -
Expert Review of Endocrinology &... Jan 2023
Topics: Humans; Pituitary Neoplasms; Adenoma
PubMed: 36647572
DOI: 10.1080/17446651.2023.2167710 -
Endocrine Pathology Mar 2022This review summarizes the changes in the 5th Edition of the WHO Classification of Endocrine and Neuroendocrine Tumors that relate to the pituitary gland. The new... (Review)
Review
This review summarizes the changes in the 5th Edition of the WHO Classification of Endocrine and Neuroendocrine Tumors that relate to the pituitary gland. The new classification clearly distinguishes anterior lobe (adenohypophyseal) from posterior lobe (neurohypophyseal) and hypothalamic tumors. Other tumors arising in the sellar region are also discussed. Anterior lobe tumors include (i) well-differentiated adenohypophyseal tumors that are now classified as pituitary neuroendocrine tumors (PitNETs; formerly known as pituitary adenomas), (ii) pituitary blastoma, and (iii) the two types of craniopharyngioma. The new WHO classification provides detailed histological subtyping of a PitNET based on the tumor cell lineage, cell type, and related characteristics. The routine use of immunohistochemistry for pituitary transcription factors (PIT1, TPIT, SF1, GATA3, and ERα) is endorsed in this classification. The major PIT1, TPIT, and SF1 lineage-defined PitNET types and subtypes feature distinct morphologic, molecular, and clinical differences. The "null cell" tumor, which is a diagnosis of exclusion, is reserved for PitNETs with no evidence of adenohypophyseal lineage differentiation. Unlike the 2017 WHO classification, mammosomatotroph and acidophil stem cell tumors represent distinct PIT1-lineage PitNETs. The diagnostic category of PIT1-positive plurihormonal tumor that was introduced in the 2017 WHO classification is replaced by two clinicopathologically distinct PitNETs: the immature PIT1-lineage tumor (formerly known as silent subtype 3 tumor) and the mature plurihormonal PIT1-lineage tumor. Rare unusual plurihormonal tumors feature multi-lineage differentiation. The importance of recognizing multiple synchronous PitNETs is emphasized to avoid misclassification. The term "metastatic PitNET" is advocated to replace the previous terminology "pituitary carcinoma" in order to avoid confusion with neuroendocrine carcinoma (a poorly differentiated epithelial neuroendocrine neoplasm). Subtypes of PitNETs that are associated with a high risk of adverse biology are emphasized within their cell lineage and cell type as well as based on clinical variables. Posterior lobe tumors, the family of pituicyte tumors, include the traditional pituicytoma, the oncocytic form (spindle cell oncocytoma), the granular cell form (granular cell tumor), and the ependymal type (sellar ependymoma). Although these historical terms are entrenched in the literature, they are nonspecific and confusing, such that oncocytic pituicytoma, granular cell pituicytoma, and ependymal pituicytoma are now proposed as more accurate. Tumors with hypothalamic neuronal differentiation are classified as gangliocytomas or neurocytomas based on large and small cell size, respectively. This classification sets the standard for a high degree of sophistication to allow individualized patient management approaches.
Topics: Adenoma; Craniopharyngioma; Humans; Pituitary Gland; Pituitary Neoplasms; World Health Organization
PubMed: 35291028
DOI: 10.1007/s12022-022-09703-7 -
Frontiers in Endocrinology 2022Although pituitary adenomas (PAs) account for 15% of intracranial tumors, pituitary carcinomas (PCs) are a rare entity. Most commonly, PCs evolve from aggressive PAs... (Review)
Review
Although pituitary adenomas (PAs) account for 15% of intracranial tumors, pituitary carcinomas (PCs) are a rare entity. Most commonly, PCs evolve from aggressive PAs invading the surrounding structures and eventually leading to metastatic lesions. Due to the low incidence, the diagnosis and treatment remains challenging. We report a case series of five patients with pituitary carcinoma (PC) treated in our center. At first diagnosis 3 patients had an ACTH-producing adenoma, 1 a prolactinoma and 1 a double secreting adenoma (GH and prolactin). The mean time interval from initial diagnosis to diagnosis of PC was 10.7 years (range 5-20 years). All patients underwent multiple surgical resections and radiotherapy. Four patients were treated with temozolomide for metastatic disease. One patient with concomitant radiochemotherapy for local recurrence. Temozolomide led to a stable disease in 2 patients. One patient had a progressive disease after 9 cycles of temozolomide. In absence of standard treatment, immunotherapy was initiated, resulting in a stable disease. We report five cases of PCs. Three patients obtained a stable disease after tailored multidisciplinary treatment. Additionally, one patient was treated with immunotherapy, opening a new treatment option in PCs. Overall, PCs are rare intracranial neoplasms occurring several years after the initial diagnosis of aggressive PAs. Currently, the absence of predictive factors for an aggressive clinical course, provokes a challenging management.
Topics: Adenoma; Adrenocorticotropic Hormone; Antineoplastic Agents, Alkylating; Humans; Pituitary Neoplasms; Prolactin; Temozolomide
PubMed: 36157469
DOI: 10.3389/fendo.2022.968692 -
World Neurosurgery Nov 2013
Topics: ACTH-Secreting Pituitary Adenoma; Adenoma; Brain Neoplasms; Carcinoma; Female; Humans; Pituitary Neoplasms
PubMed: 22082969
DOI: 10.1016/j.wneu.2011.06.002 -
Nature Reviews. Endocrinology Nov 2021Although usually benign, anterior pituitary tumours occasionally exhibit aggressive behaviour, with invasion of surrounding tissues, rapid growth, resistance to... (Review)
Review
Although usually benign, anterior pituitary tumours occasionally exhibit aggressive behaviour, with invasion of surrounding tissues, rapid growth, resistance to conventional treatments and multiple recurrences. In very rare cases, they metastasize and are termed pituitary carcinomas. The time between a 'classical' pituitary tumour and a pituitary carcinoma can be years, which means that monitoring should be performed regularly in patients with clinical (invasion and/or tumour growth) or pathological (Ki67 index, mitotic count and/or p53 detection) markers suggesting aggressiveness. However, although both invasion and proliferation have prognostic value, such parameters cannot predict outcome or malignancy without metastasis. Future research should focus on the biology of both tumour cells and their microenvironment, hopefully with improved therapeutic outcomes. Currently, the initial therapeutic approach for aggressive pituitary tumours is generally to repeat surgery or radiotherapy in expert centres. Standard medical treatments usually have no effect on tumour progression but they can be maintained on a long-term basis to, at least partly, control hypersecretion. In cases where standard treatments prove ineffective, temozolomide, the sole formally recommended treatment, is effective in only one-third of patients. Personalized use of emerging therapies, including peptide receptor radionuclide therapy, angiogenesis-targeted therapy and immunotherapy, will hopefully improve the outcomes of patients with this severe condition.
Topics: Craniopharyngioma; Humans; Immunotherapy; Pituitary Neoplasms; Temozolomide; Tumor Microenvironment
PubMed: 34493834
DOI: 10.1038/s41574-021-00550-w -
Pituitary Jun 2018Pituitary tumors are the second most common intracranial tumors, however, pituitary carcinoma is a rare clinical entity which represents only 0.1-0.2% of all pituitary... (Review)
Review
PURPOSE
Pituitary tumors are the second most common intracranial tumors, however, pituitary carcinoma is a rare clinical entity which represents only 0.1-0.2% of all pituitary tumors. Diagnosis of pituitary carcinoma requires the presence of metastasis. Early identification of pituitary carcinoma is difficult, and only recently have guidelines been published for the treatment of aggressive pituitary tumors. We present two cases from our institution, with a review of other cases available in literature in order to better characterize this rare disease.
METHODS
A retrospective review of two patients with pituitary carcinoma treated at a tertiary medical center was performed. The MEDLINE database was searched for all cases of pituitary carcinoma. Information for age at diagnosis, sex, pituitary tumor type, latency period from pituitary tumor to presentation of carcinoma, sites of metastasis, number of surgical therapies, radiation and chemotherapy, and survival after diagnosis were collected.
RESULTS
A total of 69 studies were available for review for a total of 72 unique cases. The average age at diagnosis was 46.3 years. The most common tumors were ACTH-secreting (34.7%), Prolactin-secreting (23.6%), and Null Cell (15.3%). The average latency period from pituitary tumor diagnosis to metastasis was 9 years. All patients underwent surgical therapy during their treatment, with an average of 2.76 procedures. The mortality rate was 54.8% with average time to death after diagnosis of approximately 10 months.
CONCLUSIONS
Pituitary carcinoma is a rare disease with high mortality rate and is a diagnostic and treatment challenge. Further study is required but is difficult due to its low incidence.
Topics: Carcinoma; Female; Humans; Pituitary Neoplasms; Retrospective Studies; Uterine Cervical Neoplasms
PubMed: 29404894
DOI: 10.1007/s11102-018-0872-8 -
Pituitary Apr 2018Non-functioning pituitary carcinomas (NFPC) are defined as tumours of adenophyseal origin with craniospinal or systemic dissemination, with the absence of a hormonal... (Review)
Review
Non-functioning pituitary carcinomas (NFPC) are defined as tumours of adenophyseal origin with craniospinal or systemic dissemination, with the absence of a hormonal hypersecretion syndrome. These are a histologically heterogenous group of tumours, comprising gonadotroph, null cell, "silent" tumours of corticotroph, somatotroph or lactotroph cell lineages as well as plurihormonal Pit-1 tumours. NFPC are exceedingly rare, and hence few cases have been described. This review has identified 38 patients with NFPC reported in the literature. Recurrent invasive non-functioning pituitary adenomas (NFPA) were observed in a majority of patients. Various factors have been identified as markers of the potential for aggressive behaviour, including rapid tumour growth, growth after radiotherapy, gain or shift of hormone secretion and raised proliferative markers. Typically, there is a latency of several years from the original presentation with an NFPA to identification of metastases and only 5 cases reported with rapidly progressive malignant disease within 1 month of presentation. Therapeutic options include debulking surgery, radiation therapy and chemotherapy with temozolomide recommended as first line systemic treatment. Although long-term survivors are described, prognosis remains generally very poor (median survival 8 months). Improvements in molecular tumour profiling may assist in predicting tumour behaviour, guide therapeutic choices and identify novel therapies.
Topics: Adenoma; Animals; Cell Transformation, Neoplastic; Dacarbazine; Humans; Pituitary Neoplasms; Temozolomide
PubMed: 29299820
DOI: 10.1007/s11102-017-0857-z -
The American Journal of Case Reports Feb 2022BACKGROUND Pituitary carcinomas are rare tumors that are histologically indistinguishable from pituitary adenoma. This report describes an extremely rare case of...
BACKGROUND Pituitary carcinomas are rare tumors that are histologically indistinguishable from pituitary adenoma. This report describes an extremely rare case of pituitary carcinoma in a patient with clinically diagnosed Cowden syndrome (CS). CS is a rare multisystemic hereditary disease with increase risks of cancer and benign overgrowth of different types of tissues. CASE REPORT A 52-year-old woman with history of CS presented with pituitary adenoma and multiple meningiomata. After surgical resection, there was recurrence of the pituitary tumor. Partial resection of the recurrent pituitary tumor revealed an adenoma. Radiotherapy was administered due to the histopathological aggressive features of the resected pituitary tumor and growth of the residual pituitary tumor on follow-up. Although the pituitary tumor shrank after radiotherapy, there was development of multiple new intracranial extra-axial lesions, including meningiomata. Resection of a rapidly growing extra-axial tumor in the parietal convexity region was performed. Given the similar histopathological features of the convexity tumor with the prior pituitary specimen, the diagnosis of pituitary carcinoma was established. A subsequent MRI scan showed metastatic deposits along the spine. The patient's condition deteriorated and she died shortly after the last surgery. CONCLUSIONS To the best of our knowledge, there is no prior report of pituitary carcinoma in a patient with CS in the literature. Pituitary carcinomas frequently spread within the craniospinal axis, in addition to hematogenous dissemination. Given the high risks of developing malignant tumors in patients with CS, multidisciplinary management including diagnostic imaging and close surveillance are of paramount importance in patient management.
Topics: Adenoma; Carcinoma; Female; Hamartoma Syndrome, Multiple; Humans; Meningeal Neoplasms; Middle Aged; Pituitary Neoplasms
PubMed: 35110519
DOI: 10.12659/AJCR.934846 -
Neurosurgical Focus Apr 2004Pituitary carcinomas, defined as distant metastases of a pituitary neoplasm, are rare; fewer than 140 reports exist in the English literature. The initial presenting... (Review)
Review
Pituitary carcinomas, defined as distant metastases of a pituitary neoplasm, are rare; fewer than 140 reports exist in the English literature. The initial presenting pituitary tumor is usually a secreting, invasive macroadenoma, with adrenocorticotropic hormone (ACTH)--and prolactin (PRL)--secreting tumors being the most common. The latency period between the diagnosis of a pituitary tumor and the diagnosis of a pituitary carcinoma is 9.5 years for ACTH-producing lesions and 4.7 years for PRL-secreting tumors. Survival after documentation of metastatic disease is poor; 66% of patients die within 1 year. Treatment options include additional surgery, radiotherapy, and chemotherapy, all of which are associated with poor results. Future studies will focus on identifying those invasive pituitary tumors most likely to metastasize and treating them aggressively before they progress to pituitary carcinomas.
Topics: Adrenocorticotropic Hormone; Biomarkers, Tumor; Carcinoma; Gene Expression Regulation, Neoplastic; Genes, p53; Genes, ras; Humans; Pituitary Gland; Pituitary Neoplasms; Point Mutation; Prolactin
PubMed: 15191336
DOI: 10.3171/foc.2004.16.4.8