Cases reported "Pituitary Neoplasms"

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1/17. Secondary infertility as early symptom in a man with multiple endocrine neoplasia-type 1.

    multiple endocrine neoplasia-type 1 (MEN1) is an autosomal dominant familial cancer syndrome characterized by parathyroid hyperplasia, pancreatic endocrine tumours and pituitary adenomas. Here, we report a patient with a history of insulinoma who developed secondary infertility as a further symptom of the disease. When he was first examined at the age of 36 years, he complained of weakness, reduced libido and impotence. Laboratory evaluation revealed non-obstructive azoospermia and hyperprolactinaemia. In contrast to sexual activity and serum prolactin, semen quality did not significantly respond to bromocriptine therapy. During follow-up, a growing pituitary adenoma caused acromegaly with elevated serum concentrations of growth hormone, insulin-like growth factor 1 (IGF-1), and prolactin. After microsurgery of the tumour at the age of 44 years, sperm concentration persistently increased up to 5.6 x 10(6)/ml. In accordance with the clinical diagnosis of MEN1, dna sequencing revealed a mutation in exon 2 of the menin gene which results in a truncated, inactive protein product. In conclusion, MEN1 with pituitary lesions may cause severe hypogonadism and infertility. Both hyperprolactinaemia and overproduction of growth hormone and IGF-1 seem to be involved in testicular dysfunction in the present case. The possible role of menin in the testis, however, remains to be elucidated.
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2/17. meningioma of the pituitary stalk without dural attachment: case report and review of the literature.

    OBJECTIVE AND IMPORTANCE: Tumors in the suprasellar region such as adenomas of the pituitary gland, craniopharyngiomas, nonneoplastic cystic lesions (especially Rathke's cleft cysts), and meningiomas are frequently encountered in neurosurgical practice. Meningiomas originate from the arachnoid layer connected to the dura of the anterior or posterior clinoidal process, or the tuberculum, dorsum, or diaphragma sellae. Tumors originating from the pituitary stalk are rare. Such lesions may include germinomas, astrocytomas, histiocytosis X, hamartomas, and sarcoidosis. We report a patient with a suprasellar meningioma originating from the pituitary stalk with no connection to the adjacent dura. CLINICAL PRESENTATION: A 50-year-old man was assessed for impotence and loss of libido. physical examination revealed no abnormalities. Endocrinological investigations disclosed nearly complete hypopituitarism, and magnetic resonance imaging revealed a suprasellar homogeneously enhancing tumor. INTERVENTION: Complete surgical resection was performed in an endoscope-assisted right-sided supraorbital craniotomy. The tumor originated from the pituitary stalk with no connection to the surrounding dura. The histopathological diagnosis was meningioma. CONCLUSION: Although meningiomas frequently occur in the suprasellar region, this patient with a suprasellar meningioma is unique because the tumor originated from the pituitary stalk with no connection to the surrounding dura. The absence of dural attachment has been described in 43 extracerebral meningiomas, but a suprasellar location has been reported only once previously. Recognition of this phenomenon is important, because meningiomas require a different therapeutic strategy than most other tumors of the pituitary stalk.
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3/17. A case of Rathke's Cleft Cyst inflammation presenting with diabetes insipidus.

    Rathke's Cleft Cyst (RCC), which is located at the intrasellar region, is considered to be the distended remnants of Rathke's pouch, an invagination of the stomodeum. Lined with columnar or cuboidal epithelium of ectodermal origin, RCC usually contains mucoid material and it is found in 13-22% of normal pituitary glands. The cyst rarely leads to the development of symptoms but, when it does, the most common presenting symptoms are headache, visual impairment, hypopituitarism and hypothalamic dysfunction. However, in some cases it presents symptoms of diabetes insipidus, decreased libido and impotence. Recently we experienced a case of RCC inflammation presenting with diabetes insipidus and treated with transsphenoidal surgery. To our knowledge, this is the first report of RCC presenting with symptoms of diabetes insipidus in korea.
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4/17. Intrasellar schwannoma mimicking pituitary adenoma: a case report.

    Intrasellar location of schwannoma is extremely rare, although intracranial schwannomas account for up to 8% of all primary brain tumors. An unusual case of an intrasellar schwannoma radiographically and clinically simulating a pituitary adenoma is reported. A 39-yr-old man presented a 10-month history of visual disturbance and decreased libido. Neurological examination showed poor visual acuity of both eyes with bitemporal hemianopsia. Computed tomography and magnetic resonance imaging showed a sellar tumor with suprasellar extension. Pituitary adenoma was considered as a preoperative diagnosis. The tumor was removed through a trans-sphenoidal approach. Microscopic examination of the tumor revealed schwannoma.
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5/17. Pitfalls in pituitary diagnosis: peculiarities of three cases.

    Due to the increasing availability and sensitivity of diagnostic methods, biochemical and imaging abnormalities of pituitary function and anatomy are becoming more frequent. Hyperprolactinaemia was found in three women without any prolactin (PRL) related clinical features. All three patients had normal libido, regular menses with evidence of ovulation, no galactorrhoea, and normal FSH, LH, TSH and free T4 serum levels. magnetic resonance imaging (MRI) of the sellar region showed images that were compatible with pituitary microadenomas in all three cases. Due to the discordance between laboratory and clinical features, we searched for the presence of PRL aggregates with high molecular weight and low biological activity (macroprolactinaemia). Initially, we screened with a polyethylene glycol precipitation method, and then confirmed the presence of macroprolactinaemia by chromatography. All three cases screened positive for the presence of macroprolactinaemia. MRI alterations, compatible with pituitary microadenomas, may be due to true microincidentalomas, normal anatomical variations or imaging artefacts. In conclusion, we have described the presence of double diagnostic pitfalls that might lead to unnecessary medical or surgical intervention.
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6/17. Extensive spherical amyloid deposition presenting as a pituitary tumor.

    A 71-yr-old man was admitted for further evaluation and trans-sphenoidal surgery of a pituitary tumor. He complained of impotence and decreased libido over a period of about 40 yr. Thirty-eight yr ago he was treated for bilateral gynecomastia with galactorrhea. Endocrinological investigation at presentation revealed only mild hyperprolactinemia and hypogonadotropic hypogonadism. Pituitary magnetic resonance imaging (MRI) showed a tumor up to 2.5 cm in diameter with infiltration of the sphenoid sinus and right cavernous sinus. The tumor exhibited a heterogeneous hyperintense signal on T1-weighted images and hypointense signal on T2-weighted images. Standard trans-sphenoidal surgery was performed and a brownish mass was found inside the sella, which was removed. Histological examination of the mass revealed extensive spherical amyloid deposits with strongly positive immunohistochemical staining for prolactin. Therefore, a prolactinoma with extensive spherical amyloid deposition was diagnosed. Extensive spherical amyloid deposition is a rare finding in prolactin-secreting pituitary adenomas. So far, characteristic radiological findings by MRI have been described only twice. Due to characteristic MRI findings, the diagnosis of extensive intrasellar amyloid deposition can be entertained pre-operatively. Trans-sphenoidal surgical resection is essential to confirm the diagnosis histologically and because of the potential lack of tumor shrinkage under dopaminagonist therapy in this type of prolactinoma.
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7/17. Pituicytoma: report of two cases and clues regarding histogenesis.

    OBJECTIVE AND IMPORTANCE: The pituicytoma is a rare primary tumor of the neurohypophysis. Although histologically benign, the location and vascular nature of these tumors can make surgical resection difficult. We present a report of two patients with pituicytomas and review the literature regarding treatment and prognosis for this unusual lesion. Possible histogenetic relationships of this tumor with other pituitary neoplasms are presented. CLINICAL PRESENTATION: Patient 1 was a 45-year-old man who presented with a 5-year history of decreased libido. He was found to have a 2-cm suprasellar mass on a magnetic resonance imaging scan. Patient 2 was a 48-year-old man who presented with multiple endocrine complaints. He was found to have an intrasellar mass on magnetic resonance imaging. INTERVENTION: Patient 1 underwent a right frontal craniotomy, with a subtotal resection of the suprasellar mass through the lamina terminalis. The residual tumor was treated with fractionated stereotactic radiotherapy. The intrasellar mass in Patient 2 was resected via a transsphenoidal approach. On surveillance magnetic resonance imaging, the tumor was found to have recurred and expanded into the suprasellar space. The patient underwent a right frontal craniotomy for decompression and a subtotal resection of the tumor. The patient experienced a second recurrence 7 years after the initial procedure and was subsequently treated with fractionated stereotactic radiotherapy. CONCLUSION: Pituicytomas are a distinct form of pituitary gland neoplasia that may recur if subtotally resected. These neurohypophysial tumors may contain a small subpopulation of previously unrecognized bcl-2-immunoreactive cells, whose role in the histogenesis of pituicytoma deserves further study.
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8/17. fatigue, weakness, and sexual dysfunction after bariatic surgery - not an unusual case but an unusual cause.

    BACKGROUND: Malabsorptive bariatic surgery for morbid obesity has been very effective in producing weight loss. However, patients may experience some degree of malnutrition, which may lead to various clinical symptoms, such as fatigue and weakness. Morbid obesity is often associated with impaired reproductive function, and weight loss generally improves sexual function in both sexes. However, women with extreme weight loss may experience secondary amenorrhea. In men, zinc deficiency may lead to impaired testosterone synthesis resulting in hypogonadism and impotency. Case Report: A 43-year-old male 5 years after jejunoileal bypass for morbid obesity performed in a foreign institution presented with a recent history of progressive fatigue, general weakness, and declining libido and potency. Unexpectedly, his symptoms were not related to the operation or to his weight loss but rather to a structural cause. Endocrine and radiologic evaluations revealed a cystic tumor in the sella turcica causing partial hypopituitarism and secondary hypogonadism. CONCLUSION: Long-term follow-up is mandatory in patients after bariatic surgery. Non-specific symptoms and findings should be further investigated. A loss of sexual activity and performance may have causes other than the previous bariatic operation.
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9/17. testosterone replacement-induced hyperprolactinaemia: case report and review of the literature.

    Half of all men with prolactin (PRL)-producing macroadenomas present with hypogonadism, decreased libido and impotence, and therefore require testosterone replacement. However, very little is known about the effect of testosterone on prolactinomas. We report a case of an 18-year-old obese man who presented with hypogonadism and hyperprolactinaemia and underwent a transphenoidal hypophysectomy after a computer tomography scan showed the presence of a suprasellar macroadenoma. On separate occasions, we documented a rise in PRL when testosterone replacement was started and a fall in PRL when testosterone replacement was stopped (r = 0.6090, P = 0.0095). Furthermore, imaging studies suggested the possibility of tumour re-growth after testosterone therapy. We hypothesize that the exogenous testosterone was aromatized to oestradiol, which stimulated the release of PRL by the anterior pituitary. This was supported by the increase in oestradiol levels after testosterone replacement, although statistical significance was not achieved due to the availability of only a few data points. This case highlights the need to be aware of testosterone-replacement-induced hyperprolactinaemia, an under-recognized complication of androgen replacement in this setting. The use of aromatase inhibitors together with testosterone-replacement therapy or the use of non-aromatizable androgens might be indicated in such patients. Taken together, this report and previous studies show that dopamine agonists apparently do not suppress the hyperprolactinaemia induced by testosterone replacement.
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10/17. pituitary apoplexy and idiopathic thrombocytopenic purpura: a new case and review of the literature.

    pituitary apoplexy can occur as a complication of idiopathic thrombocytopenic purpura. We report here a new case of such association. A male patient aged 59 years, complaining of decreased libido for one year, was referred to the emergency department for purpura and severe thrombocytopenia (4000 platelets/mm3). 24 hours after the cutaneous rash the patient presented with clinical symptoms of bilateral cavernous sinus compression comprising ptosis, bilateral ophtalmoplegia and right supraorbital hypoesthesia. Cranial CT scan showed an enlarged sella and a pituitary mass with signs of intrapituitary haemorrhage. Hormonal evaluation showed hyperprolactinemia (50 ng/mL) and hypopituitarism, and the patient needed substitution with hydrocortisone and levothyroxine. immunoglobulins and corticosteroids were given to the patient to treat thrombocytopenia, then worsening of neurological and ophtalmological symptoms led to pituitary surgery. Histopathological examination found necrotical pituitary tissue. Immunostaining with an anti-prolactin antibody was positive in several groups of cells. Neurological symptoms subsided and thrombocytopenia was corrected by treatment. In conclusion, we report a case of pituitary apoplexy due to severe thrombocytopenia occurring as a complication of a preexisting macroprolactinoma.
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