Pituitary Carcinoma Occurring as Middle Ear Tumor

1982 ◽  
Vol 90 (5) ◽  
pp. 665-666 ◽  
Author(s):  
David F. Wilson

Pituitary gland tumors are most commonly adenomas of the glandular tissue. They are benign, slow-growing tumors confined to the pituitary fossa region. Pituitary tumors that become aggressive, involving contiguous bony structures and brain, as well as metastasizing out of the confines of the skull, are referred to as pituitary carcinomas. This case of pituitary carcinoma is the first reported in the literature occurring as a middle ear tumor. The literature documents many cases of pituitary carcinoma. The history and medical treatment of this case are presented.

Author(s):  
Nathan J. Wallace ◽  
Anand K. Devaiah

Abstract Background Pituitary carcinomas are challenging tumors to diagnose and treat due to their rarity and limited data surrounding their etiology. Traditionally, these patients have exhibited poor survival. Over the last several decades, our understanding of pituitary carcinomas has dramatically increased, and there have been recent initiatives to improve patient access to health care, including the Affordable Care Act (ACA). This study investigates whether there were any changes in incidence and treatment outcomes of pituitary carcinoma that correlated with these advances. Methods A retrospective case review was conducted utilizing the Surveillance, Epidemiology, and End Results (SEER) database of the National Cancer Institute. Those with primary site pituitary tumors with noncontiguous metastases were identified from 1975 to 2016. Demographic data, overall, and cause-specific outcomes were obtained. The data were analyzed using SPSS to generate 5-year Kaplan–Meier curves. Results The incidence of pituitary carcinoma pre- and post-ACA was 0.31 and 2.14 diagnoses/year, respectively. This represents a significant increase (Chi-square, p < 0.00002). In addition, 1-, 2-, and 5-year overall survival of these patients was determined to be 88.2, 74.0, and 66.6% which was significantly improved compared with prior studies. Cause-specific survival of these patients follow similar trends exhibiting 94.1, 79.0, 71.1% after 1, 2, and 5 years, respectively. Conclusion The survival for pituitary carcinoma has improved significantly which signals a change in how practitioners should counsel their patients. There is a significant surge in the number of cases in the post-ACA timeline, which suggests that improving patient access has played a part in wider recognition and treatment initiation for this disease.


2004 ◽  
Vol 16 (4) ◽  
pp. 1-9 ◽  
Author(s):  
Brian T. Ragel ◽  
William T. Couldwell

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.


2020 ◽  
pp. 014556132094464
Author(s):  
Matthew M. Kwok ◽  
Jagdeep S. Virk ◽  
Michael Michael ◽  
Madeleine McKinley ◽  
Matthew J. R. Magarey

Pituitary carcinomas are rare tumors with only 170 cases reported in the literature.1 They form a very small proportion of pituitary tumors, which are commonly benign adenomas. Metastatic disease diagnosed by fine needle aspiration cytology is extremely rare and has only been reported in 6 patients,2-5 3 of whom had cervical nodal metastases, with other sites of metastases being the liver and cervical vertebra. We report a case of cervical metastatic pituitary carcinoma diagnosed by core needle biopsy.


2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii39-ii40
Author(s):  
Nazanin Majd ◽  
Steven Waguespack ◽  
Janku Filip ◽  
Siqing Fu ◽  
Marta Penas-Prado ◽  
...  

Abstract Pituitary carcinoma is an aggressive tumor characterized by metastatic spread beyond the sellar region that leads to debilitating symptoms and poor survival. Pituitary carcinomas recur despite conventional multimodality treatments. Given the recent advances in the use of immune checkpoint inhibitors (CPIs) to treat various solid cancers, there is interest in exploring the role of immunotherapy for treating aggressive, refractory pituitary tumors. We treated four pituitary carcinoma patients with pembrolizumab as part of a phase II clinical trial (NCT02721732). Here, we present their clinical course and outcomes and correlate responses with available molecular data: hypermutation status, PD-L1 staining, tumor-infiltrating lymphocyte score, microsatellite status and tumor mutational burden. Patients 1 and 2, with heavily pretreated, refractory corticotroph pituitary carcinoma, had partial radiographic (60% and 32% per irRECIST, respectively) and hormonal responses. Patient 1’s response continues 42 months after initiation of pembrolizumab and his baseline tumor tissue obtained after treatment with temozolomide demonstrated a hypermutator phenotype with MSH2 and MSH6 gene mutations. Patient 2’s tumor was not sampled after exposure to temozolomide, but prior somatic mutational testing was negative. Patient 3 (non-functioning corticotroph tumor) had a best response of stable disease for four months. Patient 4 (prolactin-secreting carcinoma) had progressive disease. The latter two patients’ tumors did not demonstrate a hypermutator phenotype after treatment with temozolomide. PD-L1 staining was negative in all tumors. TIL score was 2 in Patients 1 and 4, negative in Patient 3 and not available in Patient 2. All patients tolerated the treatment well with mild adverse events. Our study generates the hypothesis that an alkylating agent-induced hypermutator phenotype may be an indicator of response to CPIs in pituitary carcinomas. The role of CPI in treating patients with pituitary carcinoma and mechanisms of hypermutation in this population require further study.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e13022-e13022 ◽  
Author(s):  
Lynda M. O'Riordan ◽  
Megan Greally ◽  
Niamh Coleman ◽  
Oscar S. Breathnach ◽  
Bryan Hennessy ◽  
...  

e13022 Background: ACTH-producing pituitary carcinomas (APPC) are malignant neuroendocrine tumors affecting the adenohypophysis. They are extremely rare, with a poor prognosis. They are poorly responsive to chemotherapy and radiotherapy. We report the case of a 25 year old female with a diagnosis of APPC, metastatic to bone, treated with temozolamide, followed by bevacizumab and the novel agent, pasireotide. Methods: The patient was diagnosed in 2002 with an ACTH-secreting pituitary macroadenoma causing Cushing’s disease. Despite two transphenoidal surgeries, bilateral adrenalectomies, craniotomy with excision of pituitary fossa tumour and radiotherapy she developed aggressive Nelson’s Syndrome. Following discontinuation of a somatostatin analogue in 2009 her ACTH levels rose dramatically causing progressive skin hyperpigmentation. Her disease continued to progress despite CyberKnife treatment. She was referred for temozolamide chemotherapy for treatment of the pituitary adenoma. At review, she had severe back pain. Investigations suggested metastatic disease and biopsy confirmed a diagnosis of metastatic APPC to bone.We commenced treatment with Temozolamide, however her disease progressed – ACTH levels were >200,000pmol/l in June 2012. Molecular profiling completed on the patient’s tissue was negative. Pasireotide, a novel multi-somatostatin receptor analogue, was commenced concomitantly with the anti-angiogenesis agent bevacizumab. Results: Treatment response is being assessed with imaging, serial monitoring of serum ACTH levels and skin examination. Interval imaging has shown stable disease, ACTH levels have decreased to 113,000pg/ml. Conclusions: Pituitary carcinomas are extremely rare representing 0.1-0.2% of pituitary neoplasms. To date, there is no established standard treatment, although there are reports of response to temozolamide.Our patient exhibited a clinical improvement and a reduction in ACTH levels after 6 months of combination therapy with pasireotide and bevacizumab. This combination is a possible therapeutic option in patients with metastatic ACTH-producing pituitary carcinoma.


Author(s):  
Krishna Kumar ◽  
Jefferson R. Wilson ◽  
Qiuyan Li ◽  
Ryan Phillipson

Pituitary carcinomas have been reported to metastasize systemically and, less commonly, along the craniospinal axis. Metastatic lesions have been reported in the cerebral cortex, cerebellum, spinal cord, leptomeninges, cervical lymph nodes, liver, ovaries, and bone. The authors are unaware of any other examples of subependymal metastases of a pituitary carcinoma. We report such a case.Pituitary carcinoma is rare, accounting for roughly 0.2%1,3-5 of all pituitary tumors, with approximately 140 cases reported in the literature. These tumors are associated with a very high mortality, with 66% of patients dying within the first year after diagnosis. Pituitary carcinomas are differentiated from invasive pituitary adenomas by the presence of non-contiguous craniospinal tumor deposits and/or distant systemic metastases. The majority (88%) of these carcinomas prove to be hormone secreting, with prolactin secreting tumors being the most common. Invasive carcinomas evolve from hormone secreting pituitary adenomas after a latency period. Pituitary carcinomas have a predilection for systemic spread. The rate of systemic metastasis approaches 71% for prolactin producing tumors and 57% for ACTH producing tumors. Thirteen percent of tumors demonstrate both systemic and craniospinal patterns of metastatic spread.


2020 ◽  
Vol 8 (2) ◽  
pp. e001532
Author(s):  
Nazanin Majd ◽  
Steven G Waguespack ◽  
Filip Janku ◽  
Siqing Fu ◽  
Marta Penas-Prado ◽  
...  

Pituitary carcinoma is an aggressive tumor characterized by metastatic spread beyond the sellar region. Symptoms can be debilitating due to hormonal excess and survival is poor. Pituitary carcinomas recur despite conventional multimodality treatments. Given the recent advances in the use of immune checkpoint inhibitors (CPIs) to treat various solid cancers, there has been interest in exploring the role of immunotherapy for treating aggressive, refractory pituitary tumors. We treated 4 patients with pituitary carcinoma with pembrolizumab as part of a phase II clinical trial. Two patients (patients 1 and 2) with functioning corticotroph pituitary carcinomas (refractory to surgery, radiotherapy and chemotherapy) had partial radiographic (60% and 32% per Immune-Related Response Evaluation Criteria In Solid Tumors, respectively) and hormonal responses. Patient 1’s response continues 42 months after initiation of pembrolizumab and his tumor tissue obtained after treatment with temozolomide demonstrated a hypermutator phenotype with MSH2 and MSH6 gene mutations. Patient 2’s tumor after exposure to temozolomide was not sampled, but prior somatic mutational testing was negative. One patient with a non-functioning corticotroph tumor (patient 3) had a best response of stable disease for 4 months. One patient with a prolactin-secreting carcinoma (patient 4) had progressive disease. The latter 2 patients’ tumors did not demonstrate a hypermutator phenotype after treatment with temozolomide. Programmed death-ligand 1 staining was negative in all tumors. We report 2 cases of corticotroph pituitary carcinoma responsive to pembrolizumab after prior exposure to alkylating agents. The role of CPIs in treating patients with pituitary carcinoma, the relationship between tumor subtype and response to immunotherapy and mechanisms of hypermutation in this orphan disease require further study.Trial registration number: NCT02721732.


Neurosurgery ◽  
2004 ◽  
Vol 55 (1) ◽  
pp. 179-190 ◽  
Author(s):  
Alexandre Yasuda ◽  
Alvaro Campero ◽  
Carolina Martins ◽  
Albert L. Rhoton ◽  
Guilherme C. Ribas

Abstract OBJECTIVE: This study was conducted to clarify the boundaries, relationships, and components of the medial wall of the cavernous sinus (CS). METHODS: Forty CSs, examined under ×3 to ×40 magnification, were dissected from lateral to medial in a stepwise fashion to expose the medial wall. Four CSs were dissected starting from the midline to lateral. RESULTS: The medial wall of the CS has two parts: sellar and sphenoidal. The sellar part is a thin sheet that separates the pituitary fossa from the venous spaces in the CS. This part, although thin, provided a barrier without perforations or defects in all cadaveric specimens studied. The sphenoidal part is formed by the dura lining the carotid sulcus on the body of the sphenoid bone. In all of the cadaveric specimens, the medial wall seemed to be formed by a single layer of dura that could not be separated easily into two layers as could the lateral wall. The intracavernous carotid was determined to be in direct contact with the pituitary gland, being separated from it by only the thin sellar part of the medial wall in 52.5% of cases. In 39 of 40 CSs, the venous plexus and spaces in the CS extended into the narrow space between the intracavernous carotid and the dura lining the carotid sulcus, which forms the sphenoidal part of the medial wall. The lateral surface of the pituitary gland was divided axially into superior, middle and inferior thirds. The intracavernous carotid coursed lateral to some part of all the superior, middle, and inferior thirds in 27.5% of the CSs, along the inferior and middle thirds in 32.5%, along only the inferior third in 35%, and below the level of the gland and sellar floor in 5%. In 18 of the 40 CSs, the pituitary gland displaced the sellar part of the medial wall laterally and rested against the intracavernous carotid, and in 6 there was a tongue-like lateral protrusion of the gland that extended around a portion of the wall of the intracavernous carotid. No defects were observed in the sellar part of the medial wall, even in the presence of these protrusions. CONCLUSION: The CS has an identifiable medial wall that separates the CS from the sella and capsule of the pituitary gland. The medial wall has two segments, sellar and sphenoidal, and is formed by just one layer of dura that cannot be separated into two layers as can the lateral wall of the CS. In this study, the relationships between the medial wall and adjacent structures demonstrated a marked variability.


2004 ◽  
Vol 16 (4) ◽  
pp. 1-4 ◽  
Author(s):  
Daniel R. Fassett ◽  
William T. Couldwell

Only 1% of all pituitary surgeries are performed to treat tumors that have metastasized to the pituitary gland; however, in certain cases of malignant neoplasms pituitary metastases do occur. Breast and lung cancers are the most common diseases that metastasize to the pituitary. Breast cancer metastasizes to the pituitary especially frequently, with reported rates ranging between 6 and 8% of cases. Most pituitary metastases are asymptomatic, with only 7% reported to be symptomatic. Diabetes insipidus, anterior pituitary dysfunction, visual field defects, headache/pain, and ophthalmoplegia are the most commonly reported symptoms. Diabetes insipidus is especially common in this population, occurring in between 29 and 71% of patients who experience symptoms. Differentiation of pituitary metastasis from other pituitary tumors based on neuroimaging alone can be difficult, although certain features, such as thickening of the pituitary stalk, invasion of the cavernous sinus, and sclerosis of the surrounding sella turcica, can indicate metastasis to the pituitary gland. Overall, neurohypophysial involvement seems to be most prevalent, but breast metastases appear to have an affinity for the adenohypophysis. Differentiating metastasis to the pituitary gland from bone metastasis to the skull base, which invades the sella turcica, can also be difficult. In metastasis to the pituitary gland, surrounding sclerosis in the sella turcica is usually minimal compared with metastasis to the skull base. Treatment for these tumors is often multimodal and includes surgery, radiation therapy, and chemotherapy. Tumor invasiveness can make resection difficult. Although surgical series have not shown any significant survival benefits given by tumor resection, the patient's quality of life may be improved. Survival among these patients is poor with mean survival rates reported to range between 6 and 22 months.


Endocrinology ◽  
1966 ◽  
Vol 79 (6) ◽  
pp. 1149-1156 ◽  
Author(s):  
ROBERT M. MACLEOD ◽  
M. CAROLYN SMITH ◽  
GERALD W. DEWITT

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