Cases reported "Pituitary Diseases"

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1/431. Interesting radiologic findings in suprasellar mass lesions. Report of three cases.

    The authors report three quite rare lesions of the sellar/parasellar region. They are namely; pituitary abscess, cystic macroadenoma and osteochondroma. In none of the cases, the preoperative diagnostic priority was not same as the final histopathologic diagnosis. The radiologic findings of these pathologies are discussed with emphasis on differential diagnosis. ( info)

2/431. Pituitary deficiency and lack of gonads in an XY pseudohermaphrodite with beta 39/lepore haemoglobinopathy.

    We describe the occurrence of hypothyroidism and hypogonadotropic hypogonadism in an XY pseudohermaphrodite subject affected by beta-thalassemia. The patient, reared as female, diagnosed at 14 months of age as having a beta 39/Lepore hemoglobinopathy, treated with multiple transfusion therapy, was referred at age of 15 years because of delayed puberty. Complete endocrine evaluation showed low levels, both basal and after combined LHRH-TRH and hCG stimuli, of FSH, LH, TSH, estradiol (E2), testosterone (T), progesterone (P), androstenedione (A), and FT4 levels, and normal PRL, cortisol, 17OHP and ACTH levels. Imaging studies (ultrasound, magnetic resonance, radioisotope scanning and gonadal vessels phlebography) did not show internal genitalia and gonads. karyotype resulted 46,XY. PCR amplification of the SRY gene confirmed the presence of the y chromosome. female genitalia without uterus in a subject with y chromosome SRY gene, and no detectable testes indicate a condition of male pseudohermaphroditism associated with testicular regression. Low gonadotropin and sex steroid levels are suggestive of combined acquired hypothalamic-pituitary and gonadal impairment, due to iron deposition in both organs. We cannot exclude congenital failure of testosterone synthesis and action in this case, because lack of gonads is an unusual finding in thalassemic hypogonadic subjects. ( info)

3/431. Probable lymphocytic hypophysitis diagnosed by short-term serial computed tomography and gallium-67 scintigraphy--case report.

    A 61-year-old female presented with headache, malaise, and left oculomotor nerve paralysis. Computed tomography (CT) demonstrated a diffuse pituitary mass and enlarged pituitary stalk with homogeneous contrast enhancement. Her symptoms gradually resolved without treatment. gallium-67 scintigraphy showed abnormal uptake in the pituitary lesion. Serial CT every 2 weeks after admission showed homogeneous contrast enhancement and shrinking of the pituitary mass to a normal size 12 weeks after the onset. The final diagnosis was lymphocytic adenohypophysitis without biopsy. recurrence has not been observed for 8 years after discharge. The patient did not need hormone replacement therapy. Histological examination is not always necessary to diagnose probable lymphocytic adenohypophysitis with the characteristic feature of rapid onset, abnormal gallium-67 uptake in the lesion, and resolution of symptoms in the acute stage with shrinking of the lesion on neuroimaging. ( info)

4/431. Hypertonic saline test for the investigation of posterior pituitary function.

    The hypertonic saline test is a useful technique for distinguishing partial diabetes insipidus from psychogenic polydipsia, and for the diagnosis of complex disorders of osmoreceptor and posterior pituitary function. However, there is little information concerning its use in childhood. The experience of using this test in five children (11 months to 18 years) who presented diagnostic problems is reported. In two patients, in whom water deprivation tests were equivocal or impractical, an inappropriately low antidiuretic hormone (ADH) concentration (< 1 pmol/l) was demonstrated in the presence of an adequate osmotic stimulus (plasma osmolality > 295 mosmol/kg). In two children--one presenting with adipsic hypernatraemia and the other with hyponatraemia complicating desmopressin treatment of partial diabetes insipidus--defects of osmoreceptor function were identified. Confirming a diagnosis of idiopathic syndrome of inappropriate ADH secretion (SIADH) was possible in a patient with no other evidence of pituitary dysfunction. The hypertonic saline test was well tolerated, easy to perform, and diagnostic in all cases. ( info)

5/431. Infratentorial subdural empyema, pituitary abscess, and septic cavernous sinus thrombophlebitis secondary to paranasal sinusitis: case report.

    OBJECTIVE AND IMPORTANCE: Infratentorial empyema, pituitary abscess, and septic cavernous sinus thrombophlebitis are all rare and potentially lethal conditions. The occurrence of all three in a single patient has not previously been described. We present such a case occurring in a young, otherwise healthy man. CLINICAL PRESENTATION: A 26-year-old man with a remote history of sinusitis developed rapidly progressive headache, fever, right eye pain, swelling, proptosis, and visual impairment. magnetic resonance imaging demonstrated diffuse pansinusitis, including sphenoid sinusitis, and extension of inflammation and infection into the adjacent cavernous sinuses, pituitary gland, and posterior fossa. INTERVENTION: Urgent drainage of the ethmoid and maxillary sinuses was performed; pus was not identified. The patient continued to deteriorate clinically with worsening of visual acuity. Computed tomography of the head performed the next day revealed worsening hydrocephalus and an enlarging posterior fossa subdural empyema. Urgent ventricular drainage and evacuation of the empyema was performed, and subsequently, the patient's clinical course improved. The microbiology results revealed alpha hemolytic streptococcus and coagulase-negative staphylococcus species. The patient survived but during the follow-up period had a blind right eye and pituitary insufficiency. CONCLUSION: Paranasal sinusitis can have devastating intracranial sequelae. Involvement of the adjacent pituitary gland and cavernous sinuses can result in serious neurological morbidity or mortality, and retrograde spread of infection through the basal venous system can result in subdural or parenchymal brain involvement. A high index of suspicion and aggressive medical and surgical treatment are crucial for patient survival, but the morbidity rate remains high. Our patient survived but lost anterior pituitary function and vision in his right eye. ( info)

6/431. Lymphocytic hypophysitis with central diabetes insipidus and consequent panhypopituitarism preceding a multifocal, intracranial germinoma in a prepubertal girl.

    We report the clinical course of a prepubertal girl with central diabetes insipidus (DI) and consequent panhypopituitarism evolving over a period of 10 years due to lymphocytic hypophysitis and subsequent germinoma. Two years after the diagnosis of central DI was established, MRI revealed a thickened pituitary stalk. Later pituitary enlargement and increasing thickening of the pituitary stalk impinging on the optic chiasm required a trans-sphenoidal biopsy which disclosed active hypophysitis with lymphocytic infiltrates and necrosis. High dose dexamethasone treatment only temporarily halted the disease process. Therefore, stereotactic radiation therapy was performed as a rescue treatment and MRI findings almost reversed. However, the subsequent MRI showed multiple intracranial lesions identified histologically as a germinoma and a standard chemotherapy and radiation was performed. CONCLUSION: The diagnosis of diabetes insipidus in children requires long-term follow up beyond the pubertal age in order to establish the underlying cause. In contrast to lymphocytic hypophysitis in adults, lymphocytic hypophysitis in prepubertal children may represent the first sign of a host reaction to an occult germinoma. ( info)

7/431. Pituitary involvement by Wegener's granulomatosis: a report of two cases.

    We describe two cases of pituitary involvement by Wegener's granulomatosis. At initial presentation, or during subsequent disease "flares," a pattern of pituitary abnormality was suggested. During periods of remission, we found the pituitary returned to a nearly normal appearance. Loss of the normal posterior pituitary T1 hyper-intensity matched a clinical persistence of diabetes insipidus, suggesting there is permanent damage to this structure by the initial disease process. ( info)

8/431. Rosai-Dorfman disease presenting as a pituitary tumour.

    A 45-year-old woman had pyrexia, headaches, collapse and hyponatraemia. Intracerebral abscess, bacterial meningitis and subarachnoid haemorrhage were excluded. She was given intravenous antibiotics and gradually recovered. One month later she was readmitted with diplopia, headache and vomiting. serum sodium was low (107 mmol/l) and a diagnosis of inappropriate ADH secretion was made. MRI scan showed a suprasellar tumour arising from the posterior pituitary gland. A skin rash gradually faded. serum cortisol, prolactin, gonadotrophins and thyroid hormone levels were low. A pituitary tumour was removed trans-sphenoidally, she had external pituitary radiotherapy, and replacement hydrocortisone and thyroxine. She was well for 12 months when she developed progressive weakness and numbness of both legs. Examination suggested spinal cord compression at the level of T2 where MRI scanning showed an intradural enhancing mass. This spinal tumour was removed and her neurological symptoms disappeared. Nine months after this she developed facial pain and nasal obstruction. CT scan showed tumour growth into the sphenoid sinus and nasal cavities. A right Cauldwell-Luc operation was done and residual tumour in the nasal passages was treated by fractionated external radiotherapy and prednisolone. Histological examination of the specimens from pituitary, spinal mass, and nasal sinuses showed Rosai-Dorfman disease, a rare entity characterized by histiocytic proliferation, emperipolesis (lymphophagocytosis) and lymphadenopathy. aged 48 she developed cranial diabetes insipidus. Although Rosai-Dorfman syndrome is rare, it is being reported with increasing frequency, and should be borne in mind as a possible cause of a pituitary tumour. ( info)

9/431. pituitary gland gumma in congenital syphilis after failed maternal treatment: a case report.

    A preterm, very low birth weight infant was born to a mother with early latent syphilis who was treated 10 days and 3 days before delivery with 2.4 mU of benzathine penicillin. The infant had clinical, laboratory, and radiographic abnormalities consistent with congenital syphilis, ie, a Venereal disease research Laboratory test titer that was fourfold greater than was the maternal titer, hepatosplenomegaly, abnormal liver function tests, pneumonitis, osteochondritis of the long bones, and cerebrospinal fluid (CSF) examination showing a reactive Venereal disease research Laboratory test, pleocytosis, and elevated protein content. The infant died on the third day of life, and an autopsy revealed an evolving gumma of the anterior pituitary. immunoglobulin m immunoblotting of serum and CSF was positive, and polymerase chain reaction detected treponema pallidum dna in endotracheal aspirate and CSF. This case highlights the pathologic abnormalities observed in congenital syphilis and focuses on the rare finding of an evolving anterior pituitary gumma. Furthermore, it documents the failure of maternal syphilis treatment during the last 4 weeks of pregnancy to cure fetal infection and supports the recommendation that all infants born to mothers with syphilis treated during the last 4 weeks of pregnancy should receive penicillin therapy. ( info)

10/431. A rare case of pituitary hyperplasia with suprasellar extension due to primary myxoedema: case report.

    The development of pituitary tumours as a consequence of primary target organ failure is rare. We report here a rare case of pituitary hyperplasia with suprasellar extension due to primary myxoedema. This case presentation suggested the importance of detailed endocrine investigation and repeated magnetic resonance imaging for the differential diagnosis of pituitary enlargement to avoid unnecessary surgery. ( info)
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