ABSTRACT
We describe a very rare case of nonfunctional pituitary adenoma (NFPA) that exhibited corticotrophic activity after resection and radiotherapy. The possible mechanisms of the transformation from NFPA to Cushing disease (CD) are discussed.A 43-year-old man presented with impaired vision, bilateral frontal headaches, and hyposexuality. He had no symptoms suggestive of hypercortisolism, and 8âam plasma cortisol concentration was 67.88âng/mL. Brain imaging revealed a 15â×â15â×â21-mm sellar mass suggestive of a macroadenoma. The tumor was resected by transsphenoidal surgery and identified by immunohistochemical analysis as a chromophobic adenoma that did not stain for pituitary hormones. The patient was treated with prednisone and levothyroxine replacement therapy. After a third recurrence, the patient presented with clinical features and physical signs of Cushing syndrome. Plasma adrenocorticotropic hormone (ACTH) and cortisol concentrations were elevated, and there was a loss of circadian rhythms. Inferior petrosal sinus sampling after desmopressin showed the central-peripheral ACTH ratio was greater than 3:1. A repeat transsphenoidal resection was undertaken. Immunohistochemistry revealed ACTH positivity. Three months following surgery, imaging showed little residual tumor, but plasma ACTH remained elevated. He was referred for postoperative Gamma Knife radiotherapy.The immunological activity and biological features of the hormones secreted from a pituitary adenoma vary with time. Because long-term outcomes are unpredictable, postoperative follow-up is essential to detect postoperative transformation from NFPA to CD.
Subject(s)
Adenoma, Chromophobe/complications , Pituitary ACTH Hypersecretion/etiology , Pituitary ACTH Hypersecretion/physiopathology , Pituitary Neoplasms/complications , Adenoma, Chromophobe/surgery , Adrenocorticotropic Hormone/blood , Adult , Circadian Rhythm , Humans , Hydrocortisone/blood , Male , Pituitary Neoplasms/surgerySubject(s)
Adenoma, Chromophobe/complications , Diagnostic Errors , Pigment Epithelium of Eye/abnormalities , Pituitary Neoplasms/complications , Adenoma, Chromophobe/diagnosis , Adenoma, Chromophobe/radiotherapy , Adenoma, Chromophobe/surgery , Adult , Amenorrhea/etiology , Colonic Polyps/complications , Combined Modality Therapy , Female , Hormone Replacement Therapy , Humans , Hypertrophy/congenital , Hypopituitarism/drug therapy , Hypopituitarism/etiology , Optic Atrophy/etiology , Pituitary Neoplasms/diagnosis , Pituitary Neoplasms/radiotherapy , Pituitary Neoplasms/surgery , Polycystic Ovary Syndrome/diagnosis , Retinitis Pigmentosa/diagnosisABSTRACT
A case of GH and TSH secreting pituitary macroadenoma is reported. A 45-year-old female presented clinical features of acromegaly (the abnormal growth of the hands and feet, with lower jaw protrusion), diabetes mellitus, hypertension, nodular goiter and hyperthyroidism of unclear origin. NMR pituitary imaging revealed intra and extrasellar tumor. The laboratory examinations showed very high plasma levels of GH and IGF-1 and normal level of TSH coexisting with high plasma levels of free thyroid hormones. Pharmacological pretreatment with somatostatin analogues caused the substantial reduction of GH and TSH plasma levels. Histological and immunohistochemical examination of the tissue obtained at transsphenoidal surgery showed GH and TSH secreting adenoma. The laboratory examinations after surgery showed normal GH and IGF-1 plasma levels and reduced insulin requirement, what indicates radical operation. The very low plasma levels of TSH and free thyroid hormones after surgery and immunohistochemical examination suggest central hyperthyroidism due to TSH secreting pituitary tumor (thyrotropinoma).
Subject(s)
Adenoma, Chromophobe/metabolism , Adenoma, Chromophobe/surgery , Growth Hormone/metabolism , Pituitary Neoplasms/metabolism , Pituitary Neoplasms/surgery , Thyrotropin/metabolism , Acromegaly/diagnosis , Acromegaly/etiology , Acromegaly/surgery , Adenoma, Chromophobe/complications , Adenoma, Chromophobe/diagnosis , Female , Growth Hormone/blood , Humans , Hyperthyroidism/blood , Hyperthyroidism/etiology , Middle Aged , Pituitary Gland/pathology , Pituitary Gland/surgery , Pituitary Neoplasms/complications , Pituitary Neoplasms/diagnosis , Thyrotropin/bloodABSTRACT
Acromegaly is a rare disease that, in the majority of cases, is due to the presence of a benign growth hormone (GH)-producing tumor of the pituitary. Growth hormone has profound effects on linear bone growth, bone metabolism, and bone mass. In acromegaly, the skeletal effects of chronic GH excess have been mainly addressed by evaluating bone mineral density (BMD). Most data were obtained in patients with active acromegaly, and apparently high or normal BMD was observed in the absence of hypogonadism. The Autors describe a case of patient affected by acromegaly without hypogonadism with serious osteoporosis and biological signs of osteomalacia.
Subject(s)
Acromegaly/diagnosis , Adenoma, Chromophobe/diagnosis , Osteomalacia/diagnosis , Osteoporosis/diagnosis , Pituitary Neoplasms/diagnosis , Acromegaly/drug therapy , Acromegaly/etiology , Acromegaly/surgery , Adenoma, Chromophobe/complications , Adenoma, Chromophobe/surgery , Adult , Androgens/therapeutic use , Bone Density , Human Growth Hormone/blood , Humans , Hypogonadism/diagnosis , Male , Osteomalacia/drug therapy , Osteomalacia/etiology , Osteomalacia/surgery , Osteoporosis/drug therapy , Osteoporosis/etiology , Osteoporosis/surgery , Pituitary Neoplasms/complications , Pituitary Neoplasms/surgery , Reoperation , Testosterone/therapeutic use , Treatment OutcomeABSTRACT
Chester-Erdheim disease is a rare non-Langerhans histiocytosis, affecting within the CNS mainly the neurohypophyseal unit, the retrobulbar space and the parenchyma of cerebellum, cerebrum and brainstem. Here we present a case of a 55-year-old woman who developed an exophthalmus, edema and dyspnea, finally leading to death 4 months post admission to the hospital. A cMRI showed a retrobulbar fibrosis, a tumor in the sella turcica, and further tumor formation expanding from the pons to the spinal cord, but without involvement of the dural sheet. Autopsy revealed multiple tumors attached to the pituitary gland, the tentorium, and the brainstem as well as a diffuse thickening of the dura. Histologically, the tumor tissue consisted of densely packed lipid-laden foamy macrophages positive for CD68 and intervening fibrillary cords. Interestingly, tumor cells did not infiltrate/affect the parenchyma but showed a strictly extracerebral/ subdural location. In addition, sections of the pituitary tumor revealed a chromophobe giant adenoma of the pituitary gland. As to our knowledge this is the first detailed description of an exceptional case of intracranial CED presenting with strictly extracerebral/subdural tumor masses accompanied by a giant adenoma of the pituitary gland.
Subject(s)
Adenoma, Chromophobe/pathology , Dura Mater/pathology , Erdheim-Chester Disease/pathology , Pituitary Neoplasms/pathology , Subdural Space/pathology , Adenoma, Chromophobe/complications , Brain Stem/pathology , Contrast Media , Erdheim-Chester Disease/complications , Female , Gadolinium DTPA , Humans , Magnetic Resonance Imaging , Middle Aged , Pituitary Gland/pathology , Pituitary Neoplasms/complications , Spinal CordABSTRACT
A pituitary mass was removed by the transsphenoidal approach from a 63-year-old man with the clinical history and laboratory findings characteristic of Cushing's disease with partial hypopituitarism. Histological, immunohistochemical, ultrastructural and immunoelectron microscopic investigation demonstrated a periodic acid-Schiff-positive, adrenocorticotropic hormone (ACTH)-immunoreactive, pituitary corticotroph adenoma with the formation of neural tissue resembling neuropil within the tumor. The neural elements showed immunopositivity for neurofilament protein and ACTH, but were immunonegative for other adenohypophysial hormones and for corticotropin-releasing hormone. Although the molecular mechanism accounting for neural transformation in this corticotroph adenoma remained obscure, based on the clinical, histological and morphological findings it appears that formation of neural tissue most likely indicate a favorable prognosis.
Subject(s)
Adenoma, Chromophobe/ultrastructure , Cell Transformation, Neoplastic/pathology , Neuropil/ultrastructure , Pituitary Neoplasms/ultrastructure , Adenoma, Chromophobe/complications , Adenoma, Chromophobe/metabolism , Adrenocorticotropic Hormone/biosynthesis , Cell Transformation, Neoplastic/metabolism , Cushing Syndrome/etiology , Humans , Immunohistochemistry , Male , Middle Aged , Neurofilament Proteins/biosynthesis , Pituitary Neoplasms/complications , Pituitary Neoplasms/metabolismABSTRACT
A 53-year-old woman was admitted because of hypertension and diabetes mellitus. Elevated diastolic blood pressure, hypertensive retinopathy and renal dysfunction indicated malignant hypertension. Adrenocorticotropic hormone (ACTH) and cortisol levels were high although there were no Cushingoid features. One mg dexamethasone administration decreased neither ACTH nor cortisol levels. Brain magnetic resonance imaging revealed a left pituitary tumor (7 mm x 6 mm). Upon removal, the tumor showed positive ACTH staining by immnohistochemistry, and was diagnosed as pituitary ACTH-secreting adenoma (Cushing's disease). Her blood pressure, renal function, blood glucose and hormone levels subsequently improved. Malignant hypertension and deteriorated diabetes mellitus may have been due to subclinical Cushing's disease.
Subject(s)
Adenoma, Chromophobe/diagnosis , Cushing Syndrome/complications , Cushing Syndrome/diagnosis , Diabetes Complications , Hypertension, Malignant/complications , Pituitary Neoplasms/diagnosis , Adenoma, Chromophobe/complications , Adenoma, Chromophobe/surgery , Adrenocorticotropic Hormone/blood , Antihypertensive Agents/therapeutic use , Cushing Syndrome/surgery , Female , Humans , Hydrocortisone/blood , Hypophysectomy , Magnetic Resonance Imaging , Middle Aged , Pituitary Neoplasms/complications , Pituitary Neoplasms/surgery , Renal Insufficiency/etiology , Retinal Diseases/etiologyABSTRACT
We describe a case in which an incidental left carotid aneurysm was found during the preoperative evaluation of a pituitary adenoma. After magnetic resonance imaging (MRI) and angiography delineated their characteristics, we chose to treat both lesions simultaneously through a left supraorbital keyhole approach. The aneurysm clipping was followed by intracapsular resection of the tumor that was identified later as a non-secretory adenoma. The postoperative course was uneventful. This case shows that in carefully selected patients it is possible to treat successfully concurrent conditions through a minimally invasive approach, with the well known advantages of low morbidity and a short hospital stay.
Subject(s)
Adenoma, Chromophobe/surgery , Carotid Artery Diseases/surgery , Craniotomy/methods , Intracranial Aneurysm/surgery , Pituitary Neoplasms/surgery , Adenoma, Chromophobe/complications , Adenoma, Chromophobe/diagnosis , Angiography , Carotid Artery Diseases/complications , Carotid Artery Diseases/diagnosis , Female , Humans , Intracranial Aneurysm/complications , Intracranial Aneurysm/diagnosis , Magnetic Resonance Imaging , Middle Aged , Pituitary Neoplasms/complications , Pituitary Neoplasms/diagnosis , Treatment OutcomeABSTRACT
A case of the coexistence of nonfunctional pituitary adenoma and pulmonary sarcoidosis is reported. 39 years old female presented symptoms of a pituitary-gonadal axis insufficiency, visual deficit and dizziness. CT pituitary imaging revealed large intra and extrasellar tumour. Histological examination of the tissue obtained at transsphenoidal surgery showed chromophobic adenoma. Hypopituitarism and transient diabetes insipidus occurred after the surgery. The adequate replacement therapy with hydrocortisone and sex steroids was introduced. At the time of the pituitary tumour diagnosis enlargement of pulmonary lymph nodes was also observed. Based on histological examination of tracheal and bronchial epithelium specimens obtained during bronchoscopy the diagnosis of pulmonary sarcoidosis was made. No other systemic sarcoidosis localisation was confirmed. Histological re-evaluation of adenoma showed no noncaseating granuloma tissue. The overlapping symptoms of pituitary adenoma and other intrasellar masses may result in diagnostic difficulties, particularly in the presence of systemic disorders in which this gland may be involved.
Subject(s)
Adenoma, Chromophobe/complications , Pituitary Neoplasms/complications , Sarcoidosis, Pulmonary/complications , Adenoma, Chromophobe/drug therapy , Adenoma, Chromophobe/surgery , Adult , Diabetes Insipidus/etiology , Female , Humans , Hypophysectomy/methods , Hypopituitarism/etiology , Pituitary Neoplasms/drug therapy , Pituitary Neoplasms/surgery , Sarcoidosis, Pulmonary/drug therapy , Sarcoidosis, Pulmonary/physiopathology , Time Factors , Treatment OutcomeABSTRACT
To characterize the morphological and functional aspects of silent somatotroph adenomas with paradoxical responses of GH in TRH or GnRH provocation tests, which are considered to be a useful strategy for endocrinological identification of silent somatotroph adenomas, we examined three silent somatotroph adenomas histopathologically. The adenomas were investigated by immunohistochemistry, including the highly sensitive catalyzed signal amplification system, the non-radioisotopic in situ hybridization method, and confocal laser scanning microscopy. GH production and GH-immunopositive secretory granules in the adenoma cells were demonstrated histopathologically, and the adenomas were interpreted as being densely granulated somatotroph adenomas. Endocrinological identification of silent somatotroph adenomas in combination with paradoxical responses of GH in TRH or GnRH provocation tests may elucidate the increasing number of silent somatotroph adenomas that have been regarded as mammotroph or clinically inactive adenomas. One should be aware of the differences between the previously reported silent somatotroph adenomas, most of which are sparsely granulated somatotroph adenomas, a somatotroph adenomas with paradoxical and the silent somatotroph adenomas, most of which are sparsely granulated somatotroph adenomas, and the silent somatotroph adenomas with paradoxical responses of GH in TRH or GnRH provocation tests, which are densely granulated somatotroph adenomas.
Subject(s)
Adenoma, Chromophobe/pathology , Human Growth Hormone/metabolism , Pituitary Neoplasms/pathology , Adenoma, Chromophobe/blood , Adenoma, Chromophobe/complications , Adenoma, Chromophobe/metabolism , Adult , Amenorrhea/etiology , Female , Galactorrhea/etiology , Human Growth Hormone/blood , Human Growth Hormone/genetics , Humans , Immunoenzyme Techniques , In Situ Hybridization , Microscopy, Confocal , Pituitary Neoplasms/blood , Pituitary Neoplasms/complications , Pituitary Neoplasms/metabolism , RNA, Messenger/metabolism , RNA, Neoplasm/analysisABSTRACT
Three cases of nonphysiologic hyperprolactinemia associated with pituitary disease evidenced by galactorrhea are presented. Two patients had significant pituitary disease associated with low-level prolactin elevations. The third patient had only a history of infertility and expressible galactorrhea on examination. This patient was found to have high prolactin levels and a locally invasive pituitary tumor. Physicians need to be aware of the serious conditions associated with galactorrhea so that appropriate diagnostic studies can be done and treatment instituted.
Subject(s)
Adenoma/complications , Adenoma/diagnosis , Galactorrhea/etiology , Pituitary Neoplasms/complications , Pituitary Neoplasms/diagnosis , Prolactinoma/complications , Adenoma/blood , Adenoma, Chromophobe/complications , Adenoma, Chromophobe/diagnosis , Adult , Female , Galactorrhea/blood , Humans , Hyperprolactinemia/etiology , Pituitary Neoplasms/blood , Prolactinoma/diagnosisSubject(s)
Adenoma, Chromophobe/complications , Pituitary Apoplexy/chemically induced , Pituitary Apoplexy/complications , Pituitary Function Tests/adverse effects , Pituitary Neoplasms/complications , Subarachnoid Hemorrhage/etiology , Vasospasm, Intracranial/etiology , Adenoma, Chromophobe/drug therapy , Adenoma, Chromophobe/pathology , Adenoma, Chromophobe/surgery , Aged , Hormone Replacement Therapy , Humans , Hydrocortisone/therapeutic use , Hypophysectomy , Male , Pituitary Neoplasms/drug therapy , Pituitary Neoplasms/pathology , Pituitary Neoplasms/surgery , Thyroxine/therapeutic use , Treatment OutcomeABSTRACT
We have screened the mitochondrial genome of 15 men with symptomatic vertebral fractures (median age 62 years, range 27-72 years) and 17 male control subjects (median age 61 years, range 40-73 years) for the presence of mitochondrial DNA (mtDNA) deletions in peripheral monocyte DNA. Polymerase chain reaction analysis provided evidence of a common age-related (4.9 kb) mtDNA deletion situated between nucleotides 8470 and 13.460 of the genomic sequence in 5 of the 17 controls (29%) and 9 of the 15 patients (60%) investigated. Southern blotting and polymerase chain reaction revealed a novel 3.7 kb deletion in 2 patients. One of the affected patients, a 27-year-old man with severe osteoporosis (lumbar spine bone mineral density (BMD) 0.381 g/cm(2); Z-score -6.45) was found to harbor deletion in almost 50% of the mitochondria. The patient had a blood lactic acid level (4.6 nM) that was over 3 times the upper reference range (0-1.3 mM), thus confirming the presence of systemic oxidative stress. Further analysis by modified primer shift polymerase chain reaction showed the 5' breakpoint to be between the nucleotides 10.63 kb and 10.80 kb of the mtDNA. The second patient harboring the 3.7 kb deletion was older (62 years) with less severe osteoporosis (lumbar spine BMD 0.727/cm(2); Z-score -2.58) and the proportion of affected mitochondria was lower (25%). The significance of these findings is discussed and the possible relation between oxidative stress and accelerated bone loss is examined.
Subject(s)
DNA, Mitochondrial/genetics , Osteoporosis/genetics , Oxidative Stress , Sequence Deletion/genetics , Spinal Fractures/genetics , Adenoma, Chromophobe/complications , Adult , Aged , Case-Control Studies , DNA Mutational Analysis , DNA, Mitochondrial/isolation & purification , Humans , Male , Middle Aged , Pituitary Neoplasms/complications , Polymerase Chain ReactionABSTRACT
Two rare cases of pituitary adenoma with neuronal choristoma are described. Both patients were female and presented with features of acromegaly with elevated growth hormone and prolactin levels. Radiologically, both lesions were predominantly intrasellar in location with extension into suprasellar region, but hypothalamus was not involved. Histopathological examination revealed a mixture of chromophobe pituitary adenoma cells and neuronal cells. In both cases, the adenoma component was positive for growth hormone and prolactin. Interestingly, immunopositivity for alpha-subunit, cytokeratin and prolactin was seen in the adenoma and neuronal cells in one case. Our findings support the hypothesis that the neuronal cells possibly arise from adenoma cells as a result of metaplasia.
Subject(s)
Acromegaly/etiology , Adenoma, Chromophobe/pathology , Choristoma/pathology , Nerve Tissue , Pituitary Neoplasms/pathology , Prolactinoma/pathology , Acromegaly/pathology , Adenoma, Chromophobe/complications , Adult , Choristoma/complications , Female , Humans , Middle Aged , Pituitary Diseases/complications , Pituitary Diseases/pathology , Pituitary Neoplasms/complications , Prolactinoma/complicationsABSTRACT
Retinal abnormalities, unrelated to visual pathway compression, in acromegalic patients were originally described by Small in 1972 (Smail JM. Primary pigmentary degeneration of the retina and acromegaly in a case of pituitary adenoma. Br J Ophthalmol 1972; 56: 25-31). He illustrated a case of primary pigmentary degeneration of the retina occurring in a patient with a chromophobe adenoma of the pituitary gland. To the best of our knowledge this remains the sole, published case of this association. We report on two male patients, with an acromegalic appearance, one caused by a pituitary adenoma, the other associated with a Rathke's cleft cyst, presenting to our department of ophthalmology with the clinical picture of pigmentary degeneration of the retina.