scholarly journals An intrasellar pituitary adenoma–gangliocytoma presenting as acromegaly

2017 ◽  
Vol 2017 ◽  
Author(s):  
Melissa H Lee ◽  
Penelope McKelvie ◽  
Balasubramanian Krishnamurthy ◽  
Yi Yuen Wang ◽  
Carmela Caputo
Keyword(s):  
Pituitary Adenoma ◽  
Surgical Resection ◽  
Pituitary Adenomas ◽  
Histological Analysis ◽  
Good Prognosis ◽  
List Type ◽  
Ongoing Research ◽  
Learning Points ◽  
Mixed Tumours ◽  
Gh Excess

Summary Most cases of acromegaly are due to growth hormone (GH)-secreting pituitary adenomas arising from somatotroph cells. Mixed pituitary adenoma and gangliocytoma tumours are rare and typically associated with hormonal hypersecretion, most commonly GH excess. Differentiating these mixed tumours from conventional pituitary adenomas can be difficult pre-operatively, and careful histological analysis after surgical resection is key to differentiating the two entities. There is little literature addressing the possible mechanisms for the development of mixed pituitary adenoma–gangliocytomas; however, several hypotheses have been proposed. It still remains unclear if these mixed tumours differ from a clinical perspective to pituitary adenomas; however, the additional neural component of the gangliocytoma does not appear to modify the aggressiveness or risk of recurrence after surgical resection. We report a unique case of acromegaly secondary to a mixed GH-secreting pituitary adenoma, co-existing with an intrasellar gangliocytoma. Learning points: Acromegaly due to a mixed GH-secreting pituitary adenoma and intrasellar gangliocytoma is rare. These mixed tumours cannot be distinguished easily from ordinary pituitary adenomas on the basis of clinical, endocrine or neuroradiologic findings, and histological analysis is required for a definitive diagnosis. Surgical resection is usually sufficient to provide cure, without the need for adjuvant therapy. These mixed tumours appear to have a good prognosis although the natural history is not well defined. The pathogenesis of these mixed tumours remains debatable, and ongoing research is required.

scholarly journals A case of a co-secreting TSH and growth hormone pituitary adenoma presenting with a thyroid nodule

2018 ◽  
Vol 2018 ◽  
Author(s):  
Laura Hamilton Adams ◽  
Derick Adams
Keyword(s):  
Growth Hormone ◽  
Pituitary Adenoma ◽  
Complete Remission ◽  
Pituitary Adenomas ◽  
Thyroid Nodules ◽  
Pituitary Hormones ◽  
Pituitary Hormone ◽  
List Type ◽  
Learning Points ◽  
Pituitary Macroadenomas

Summary Co-secreting TSH and growth hormone pituitary adenomas are rare. We present a case of a 55-year-old woman who presented with symptoms of neck fullness. Ultrasound revealed multiple thyroid nodules and examination revealed several clinical features of acromegaly. She was found to have a co-secreting TSH and growth hormone pituitary macroadenoma. She underwent surgical resection followed by gamma knife radiation, which resulted in complete remission of her TSH and GH-secreting adenoma. Learning points: TSH-secreting pituitary adenomas are rare and about one-third co-secrete other hormones. Thyroid nodules are common in acromegaly and can be the presenting sign of a growth hormone-secreting pituitary adenoma. In the workup of acromegaly, assessment of other pituitary hormones is essential, even in the absence of symptoms of other pituitary hormone dysfunction. Complete remission of co-secreting GH and TSH pituitary macroadenomas is possible with surgery and radiation alone.

scholarly journals Thyrotoxicosis with absence of clinical features of acromegaly in a TSH- and GH-secreting, invasive pituitary macroadenoma

2015 ◽  
Vol 2015 ◽  
Author(s):  
Philip C Johnston ◽  
Amir H Hamrahian ◽  
Richard A Prayson ◽  
Laurence Kennedy ◽  
Robert J Weil
Keyword(s):  
Pituitary Adenoma ◽  
Clinical Features ◽  
Surgical Removal ◽  
List Type ◽  
Pituitary Macroadenoma ◽  
Pituitary Mass ◽  
Biochemical Evidence ◽  
Learning Points ◽  
Gh Excess

Summary A 54-year-old woman presented with bi-temporal hemianopia, palpitations, and diaphoresis. An invasive pituitary macroadenoma was discovered. The patient had biochemical evidence of secondary hyperthyroidism and GH excess; however, she did not appear to be acromegalic. Surgical removal of the pituitary mass revealed a plurihormonal TSH/GH co-secreting pituitary adenoma. TSH-secreting adenomas can co-secrete other hormones including GH, prolactin, and gonadotropins; conversely, co-secretion of TSH from a pituitary adenoma in acromegaly is infrequent. Learning points This case highlights an unusual patient with a rare TSH/GH co-secreting pituitary adenoma with absence of the clinical features of acromegaly. Plurihormonality does not always translate into the clinical features of hormonal excess. There appears to be a clinical and immunohistochemical spectrum present in plurihormonal tumors.

scholarly journals Macroprolactinoma appearing after resolution of hydrocephalus

2017 ◽  
Vol 2017 ◽  
Author(s):  
Oscar D Bruno ◽  
Ricardo Fernández Pisani ◽  
Gabriel Isaac ◽  
Armando Basso
Keyword(s):  
Pituitary Adenoma ◽  
Pituitary Tumour ◽  
Empty Sella ◽  
List Type ◽  
Liquid Pressure ◽  
Final Size ◽  
Mechanical Factors ◽  
Pressure Resistance ◽  
Learning Points

Summary The role of mechanical forces influencing the growth of a pituitary adenoma is poorly understood. In this paper we report the case of a young man with hyperprolactinaemia and an empty sella secondary to hydrocephalia, who developed a macroprolactinoma following the relief of high intraventricular pressure. Learning points: The volume of a pituitary tumour may be influenced not only by molecular but also by local mechanical factors. Intratumoural pressure, resistance of the sellar diaphragm and intracranial liquid pressure may play a role in the final size of a pituitary adenoma. The presence of hydrocephalus may hide a pituitary macroadenoma.

scholarly journals Fluconazole and acetazolamide in the treatment of ectopic Cushing's syndrome with severe metabolic alkalosis

2015 ◽  
Vol 2015 ◽  
Author(s):  
Verena Schwetz ◽  
Felix Aberer ◽  
Claudia Stiegler ◽  
Thomas R Pieber ◽  
Barbara Obermayer-Pietsch ◽  
...  
Keyword(s):  
Case Report ◽  
Surgical Resection ◽  
Cushing’S Syndrome ◽  
Metabolic Alkalosis ◽  
Cushing's Syndrome ◽  
List Type ◽  
Ectopic Acth ◽  
Ectopic Cushing’S Syndrome ◽  
Ectopic Acth Production ◽  
Learning Points

Summary Cushing's syndrome (CS) due to ectopic ACTH production accounts for about 10% of all types of CS and is frequently associated with metabolic alkalosis. Treatment of CS involves surgical resection and/or medical therapy to control hypercortisolism. We present the case of an 80-year-old woman affected by CS due to an unknown cause. The patient had severe metabolic alkalosis with refractory hypokalemia. To treat the underlying CS, fluconazole was initiated due to unavailability of ketoconazole. In spite of markedly decreasing cortisol levels, metabolic alkalosis persisted. Treatment of metabolic alkalosis with acetazolamide was thus initiated and pH levels successfully lowered. This case report shows that hypercortisolism can be effectively treated with fluconazole in cases where ketoconazole is unavailable or not tolerated and that persistent severe metabolic alkalosis caused by glucocorticoid excess can be safely and successfully treated with acetazolamide. Learning points Hypercortisolism can be effectively treated with fluconazole where ketoconazole is unavailable or not tolerated. Glucocorticoid excess can cause severe metabolic alkalosis. Persistent severe metabolic alkalosis can be safely and successfully treated with acetazolamide.

scholarly journals A patient with a germline SDHB mutation presenting with an isolated pituitary macroprolactinoma

2018 ◽  
Vol 2018 ◽  
Author(s):  
Michelle Maher ◽  
Federico Roncaroli ◽  
Nigel Mendoza ◽  
Karim Meeran ◽  
Natalie Canham ◽  
...  
Keyword(s):  
Genetic Testing ◽  
Pituitary Adenoma ◽  
Pituitary Adenomas ◽  
Biochemical Response ◽  
List Type ◽  
Factor X ◽  
Loss Of Function ◽  
Variable Response ◽  
Sdhb Mutation ◽  
Tumour Shrinkage

Summary Symptomatic pituitary adenomas occur with a prevalence of approximately 0.1% in the general population. It is estimated that 5% of pituitary adenomas occur in a familial setting, either in isolated or syndromic form. Recently, loss-of-function mutations in genes encoding succinate dehydrogenase subunits (SDHx) or MYC-associated factor X (MAX) have been found to predispose to pituitary adenomas in co-existence with paragangliomas or phaeochromocytomas. It is rare, however, for a familial SDHx mutation to manifest as an isolated pituitary adenoma. We present the case of a pituitary lactotroph adenoma in a patient with a heterozygous germline SDHB mutation, in the absence of concomitant neoplasms. Initially, the adenoma showed biochemical response but poor tumour shrinkage in response to cabergoline; therefore, transsphenoidal surgery was performed. Following initial clinical improvement, tumour recurrence was identified 15 months later. Interestingly, re-initiation of cabergoline proved successful and the lesion demonstrated both biochemical response and tumour shrinkage. Our patient’s SDHB mutation was identified when we realised that her father had a metastatic paraganglioma, prompting genetic testing. Re-inspection of the histopathological report of the prolactinoma confirmed cells with vacuolated cytoplasm. This histological feature is suggestive of an SDHx mutation and should prompt further screening for mutations by immunohistochemistry and/or genetic testing. Surprisingly, immunohistochemistry of this pituitary adenoma demonstrated normal SDHB expression, despite loss of SDHB expression in the patient’s father’s paraganglioma. Learning points: Pituitary adenomas may be the presenting and/or sole feature of SDHB mutation-related disease. SDHx mutated pituitary adenomas may display clinically aggressive behaviour and demonstrate variable response to medical treatment. Histological evidence of intracytoplasmic vacuoles in a pituitary adenoma might suggest an SDH-deficient tumour and should prompt further screening for SDHx mutations. Immunohistochemistry may not always predict the presence of SDHx mutations.

scholarly journals Pheochromocytomatosis associated with a novel TMEM127 mutation

2017 ◽  
Vol 2017 ◽  
Author(s):  
Run Yu ◽  
Danielle Sharaga ◽  
Christopher Donner ◽  
M Fernando Palma Diaz ◽  
Masha J Livhits ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Rare Complication ◽  
List Type ◽  
Open Resection ◽  
Rare Form ◽  
Tumor Capsule ◽  
The Right ◽  
Learning Points ◽  
Generation Sequencing ◽  
Capsule Rupture

Summary Pheochromocytomatosis, a very rare form of pheochromocytoma recurrence, refers to new, multiple, and often small pheochromocytomas growing in and around the surgical resection bed of a previous adrenalectomy for a solitary pheochromocytoma. We here report a case of pheochromocytomatosis in a 70-year-old female. At age 64 years, she was diagnosed with a 6-cm right pheochromocytoma. She underwent laparoscopic right adrenalectomy, during which the tumor capsule was ruptured. At age 67 years, CT of abdomen did not detect recurrence. At age 69 years, she began experiencing episodes of headache and diaphoresis. At age 70 years, biochemical markers of pheochromocytoma became elevated with normal calcitonin level. CT revealed multiple nodules of various sizes in the right adrenal fossa, some of which were positive on metaiodobenzylguanidine (MIBG) scan. She underwent open resection of pheochromocytomatosis. Histological examination confirmed numerous pheochromocytomas ranging 0.1–1.2 cm in size. Next-generation sequencing of a panel of genes found a novel heterozygous germline c.570delC mutation in TMEM127, one of the genes that, if mutated, confers susceptibility to syndromic pheochromocytoma. Molecular analysis showed that the c.570delC mutation is likely pathogenic. Our case highlights the typical presentation of pheochromocytomatosis, a rare complication of adrenalectomy for pheochromocytoma. Previous cases and ours collectively demonstrate that tumor capsule rupture during adrenalectomy is a risk factor for pheochromocytomatosis. We also report a novel TMEM127 mutation in this case. Learning points: Pheochromocytomatosis is a very rare form of pheochromocytoma recurrence. Pheochromocytomatosis refers to new, multiple and often small pheochromocytomas growing in and around the surgical resection bed of a previous adrenalectomy for a solitary pheochromocytoma. Tumor capsule rupture during adrenalectomy predisposes a patient to develop pheochromocytomatosis. Surgical resection of the multiple tumors of pheochromocytomatosis is recommended. Pheochromocytoma recurrence should prompt genetic testing for syndromic pheochromocytoma.

scholarly journals Synchronous GH- and prolactin-secreting pituitary adenomas

2014 ◽  
Vol 2014 ◽  
Author(s):  
Maryam Rahman ◽  
Ignacio Jusué-Torres ◽  
Abdulrahman Alkabbani ◽  
Roberto Salvatori ◽  
Fausto J Rodríguez ◽  
...  
Keyword(s):  
Prolactin Level ◽  
Pituitary Adenomas ◽  
Pituitary Tumors ◽  
The Other ◽  
Careful Study ◽  
List Type ◽  
Pathological Analysis ◽  
Level Elevation ◽  
Learning Points ◽  
Endocrinological Investigation

Summary Pituitary adenomas are usually solitary lesions. Rarely, patients may present with two distinct pituitary tumors. We report a case of synchronous secretory pituitary adenomas in a woman who initially presented with elevated prolactin levels. She was initially treated with cabergoline, but, after many years, she began developing symptoms consistent with acromegaly. Imaging revealed two distinct tumors within the pituitary gland. Endocrinological investigation confirmed acromegaly. At the time of surgery, two separate tumors were identified and resected. Pathological analysis demonstrated one tumor as a prolactinoma, and the other tumor as a GH-secreting adenoma. Postoperatively, her GH and IGF1 levels normalized, while the prolactin level remained slightly above normal. This case highlights that GH and prolactin level elevation is not always from co-secretion by the same adenoma. Learning points Synchronous pituitary adenomas represent <0.5% of pituitary tumors requiring surgery. In the setting of elevated GH and prolactin levels, one cannot assume that they are co-secreted by the same adenoma. A careful study of hormonal workup and pre-operative imaging is necessary for synchronous pituitary adenomas to assure resection of both tumors.

scholarly journals Pasireotide therapy in a rare and unusual case of plurihormonal pituitary macroadenoma

2013 ◽  
Vol 2013 ◽  
Author(s):  
Rajesh Rajendran ◽  
Sarita Naik ◽  
Derek D Sandeman ◽  
Azraai B Nasruddin
Keyword(s):  
Glycaemic Control ◽  
Cushing’S Disease ◽  
Pituitary Adenomas ◽  
Unusual Case ◽  
Cushing's Disease ◽  
List Type ◽  
Pituitary Macroadenoma ◽  
Safety And Efficacy ◽  
Gh Excess

Summary We report the use of pasireotide in a rare and unusual case of pituitary macroadenoma co-secreting GH, prolactin and ACTH. A 62-year-old Caucasian man presented with impotence. Clinically, he appeared acromegalic and subsequent investigations confirmed GH excess and hyperprolactinaemia. Magnetic resonance imaging (MRI) of pituitary revealed a large pituitary macroadenoma. He underwent trans-sphenoidal surgery and histology confirmed an adenoma with immunohistochemistry positive for ACTH, GH and prolactin. Acromegaly was not cured following surgery and inadequately controlled despite subsequent octreotide therapy. He underwent further debulking pituitary surgery, following which IGF1 levels improved but still high. This time adenoma cells showed immunohistochemistry positivity for ACTH only, following which subsequent investigations confirmed intermittent hypercortisolaemia compatible with pituitary Cushing's disease. We recommended radiotherapy, but in view of the pluripotential nature of the tumour, we proceeded with a trial of s.c. pasireotide therapy on the basis that it may control both his acromegaly and Cushing's disease. After 3 months of pasireotide therapy, his mean GH and IGF1 levels improved significantly, with improvement in his symptoms but intermittent hypercortisolaemia persists. His glycaemic control deteriorated requiring addition of new anti-diabetic medication. MRI imaging showed loss of contrast uptake within the tumour following pasireotide therapy but no change in size. We conclude that our patient has had a partial response to pasireotide therapy. Long-term follow-up studies are needed to establish its safety and efficacy in patients with acromegaly and/or Cushing's disease. Learning points Plurihormonal pituitary adenomas are rare and unusual. Patients with pituitary adenomas co-secreting ACTH and GH are more likely to present with acromegaly because GH excess can mask hypercortisolaemia. Pasireotide holds potential where conventional somatostatin analogues are not effective in acromegaly due to higher affinity for somatostatin receptor subtypes 1, 2, 3 and 5. Significant deterioration in glycaemic control remains a concern in the use of pasireotide. Currently, long-term safety and efficacy of pasireotide in patients with acromegaly and/or Cushing's disease are not fully clear.

scholarly journals A very rare case of pituitary metastasis infiltrating a non-secretory gonadotroph adenoma

2020 ◽  
Vol 2020 ◽  
Author(s):  
Milad Darrat ◽  
Mohammad Binhussein ◽  
Alan Beausang ◽  
Clare Faul ◽  
Michael W O’Reilly ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Breast Carcinoma ◽  
Pituitary Adenomas ◽  
Symptomatic Relief ◽  
Metastatic Breast ◽  
Breast Adenocarcinoma ◽  
List Type ◽  
Rare Presentation ◽  
The Third ◽  
Pituitary Metastases

Pituitary adenomas are the commonest sellar tumours. Pituitary metastases are very rare, with the most common primaries being breast and lung cancers. We report the case of an 83-year-old man with a history of breast carcinoma who presented with recent-onset headaches and progressive deterioration of visual acuity. MRI brain showed a large sellar and suprasellar mass compressing the optic chiasm and involving the pituitary stalk. Transsphenoidal debulking resulted in symptomatic relief and visual recovery. Specimen examination revealed a combination of a gonadotroph pituitary adenoma that was infiltrated by metastatic breast carcinoma. He had no symptoms of diabetes insipidus. He was subsequently treated with pituitary radiotherapy. This is a very rare presentation of a pituitary mass with mixed pathology. To our knowledge, this is the third description of a breast carcinoma metastasis into a gonadotroph cell pituitary adenoma. Highlight points: Infiltrating metastases into pituitary adenomas are very rare but do occur. To our knowledge this is the third case of breast adenocarcinoma metastasising to a gonadotroph pituitary adenoma. Pituitary metastases should always be considered in rapidly evolving pituitary symptoms in a cancer patient. Not all complex pituitary lesions are associated with panhypopituitarism. Early invasive local management (TSS and post TSS radiotherapy) can provide rapid satisfactory outcomes.

PTTG has a Dual Role of Promotion-Inhibition in the Development of Pituitary Adenomas

2019 ◽  
Vol 26 (11) ◽  
pp. 800-818
Author(s):  
Zujian Xiong ◽  
Xuejun Li ◽  
Qi Yang
Keyword(s):  
Cell Cycle ◽  
Pituitary Adenoma ◽  
Pituitary Adenomas ◽  
Cell Cycle Progression ◽  
Key Factors ◽  
Cycle Progression ◽  
Sister Chromatids ◽  
Regulation Of Cell Cycle ◽  
Late Stages

Pituitary Tumor Transforming Gene (PTTG) of human is known as a checkpoint gene in the middle and late stages of mitosis, and is also a proto-oncogene that promotes cell cycle progression. In the nucleus, PTTG works as securin in controlling the mid-term segregation of sister chromatids. Overexpression of PTTG, entering the nucleus with the help of PBF in pituitary adenomas, participates in the regulation of cell cycle, interferes with DNA repair, induces genetic instability, transactivates FGF-2 and VEGF and promotes angiogenesis and tumor invasion. Simultaneously, overexpression of PTTG induces tumor cell senescence through the DNA damage pathway, making pituitary adenoma possessing the potential self-limiting ability. To elucidate the mechanism of PTTG in the regulation of pituitary adenomas, we focus on both the positive and negative function of PTTG and find out key factors interacted with PTTG in pituitary adenomas. Furthermore, we discuss other possible mechanisms correlate with PTTG in pituitary adenoma initiation and development and the potential value of PTTG in clinical treatment.

Sign in / Sign up

Export Citation Format

Share Document