Zeis gland carcinoma

Primary hyperaldosteronism (PHA) represents less than 1 to 2% of all causes of hypertension (HT). We report 2 cases of primary hyperaldosteronism which emphasize the difficulty of distinguishing neoplastic PHA from idiopathic PHA, observed in a 60-year-old woman and a 42-year old woman, respectively. In both cases, the diagnosis of PHA was suggested by marked hypokalaemia with inappropriate potassium excretion and was confirmed by hyperaldosteronaemia and low and poorly stimulated renin activity. In the first case, computed tomography showed nodular hyperplasia of the 2 adrenal glands. The patient was treated with spironolactone and calcium channel blockers which controlled blood pressure and serum potassium. In the second case, computed tomography and magnetic resonance imaging revealed an adrenocortical adenoma confirmed by pathological examination after the operation. The diagnosis of primary hyperaldosteronism is based on three steps: detection, positive diagnosis and aetiological diagnosis. Detection is essentially based on demonstration of hypokalaemia. Positive diagnosis is based on demonstration of elevated aldosterone secretion with inhibited renin secretion. The aetiological diagnosis is dominated by the differentiation between Conn's adenoma and bilateral adrenal hyperplasia, which has therapeutic implications.     

Preventive effects of superoxide dismutase derivatives modified with monosaccharides on reperfusion injury in rat liver transplantation

Focal nodular hyperplasia (FNH) and adenoma are rare benign hepatic tumors, and the standards for diagnosis and treatment still remain controversial. Usually adenoma is an indication for resection, due to its tendency to bleed and to degenerate; FNH, on the contrary, may be treated conservatively. Preoperation differential diagnosis is, however, difficult, often impossible. MATERIALS AND METHODS: Thirty-eight patients with presumed hepatic adenoma and/or FNH were studied at our department from 1984 to 1996. Preoperative assessment included clinical evaluation and symptoms, laboratory tests, liver biopsy, ultrasound scan, computed tomography scan, magnetic resonance imaging, scintigraphy, and angiography. Thirteen patients had a presumed diagnosis of FNH, 16 of adenoma, and 9 of undetermined benign lesions; 27 had hepatic resections (3 with laparoscopic technique), and 11 were not operated on and are actually under a strict follow-up observation. RESULTS: The final diagnosis was 19 FNH and 19 adenomas (2 of which contained areas of hepatocarcinoma). Presumed diagnosis was confirmed in 71% of cases. Use of oral contraceptives, abdominal symptoms, and pathologic liver test results were frequent in patients with adenomas. There were no deaths after surgery. All resected patients were tumor free during the follow-up, and in 10 of the 11 nonoperated cases, the size of the nodules remained unchanged. We conclude that precise diagnosis of these benign liver tumors remains difficult and sometimes impossible, despite new imaging techniques. Hepatic resections can be performed under very safe conditions; laparoscopic surgery may play a role in selected cases. Adenomas and uncertain cases are clear indications for surgery. Only when a diagnosis of FNH can be firmly confirmed in asymptomatic patients is strict observation without surgery recommended.     

Hyperplasia of pulmonary neuroendocrine cells in a case of childhood pulmonary emphysema

Pulmonary emphysema is very uncommon in children in the first decade of life. The few cases documented in the literature were all due to alpha1-antitrypsin deficiency. We present the case of a 6-year-old white boy with chronic cough and dyspnea on exertion. Lung biopsy showed panacinar type emphysema with patent airways and diffuse hyperplasia of pulmonary neuroendocrine cells revealed after immunostaining for bombesin, a peptide produced by these cells. We speculate that idiopathic diffuse hyperplasia of bombesin-producing pulmonary neuroendocrine cells may contribute to the pathogenesis of unusual COPD in childhood.     

Idiopathic primary hyperaldosteronism: significance of functional diagnosis

Primary aldosteronism is a rare cause of hypertension caused by increased aldosterone secretion. The two essential stimuli for aldosterone production, potassium and angiotensin II, tend to be low. At a time when imaging procedures are becoming more common, more patients are being found with adrenal masses, and patients with incidentalomas and low renin hypertension could be considered as candidates for surgery. Apart from an appropriate work-up for the diagnosis of primary aldosteronism, the differential diagnosis between unilateral adenoma and idiopathic ("hyperplasia") hyperaldosteronism is relevant for the choice of therapy. Based on data from the literature and four patients we observed as outpatients in 1997, we suggest that a properly conducted and carefully interpreted postural stimulation test can be useful in identifying patients who are successfully treated by drugs.     

The role of PET-FDG in questionable diagnosis of relapse in the presence of radionecrosis of brain tumors

INTRODUCTION: Although CT and MR are sensitive techniques for the detection of cerebral tumours, both have limitations in distinguishing between tumour relapse (TR) and post-treatment radionecrosis (RN). PATIENTS AND METHODS: In this study we have determined the usefulness of metabolic imaging with PET-FDG in such situations. We assessed 70 patients with CNS tumours (22 low grade astrocytomas, 25 high grade astrocytomas, 3 oligodendrogliomas, 13 metastatic tumours and 7 other tumours. All had been treated with radiotherapy and other treatments such as radiosurgery, chemotherapy or different types of surgery, and presented clinical pictures which made it necessary to decide the differential diagnosis of relapse or radionecrosis. RESULTS: In the PET-FDG study visual and semiquantitative analysis was done by SUV (Standardized Update Value). Confirmation of the findings was obtained in 44 cases (24 TR and 20 RN). MR was doubtful or inconclusive in most cases, whilst with PET correct diagnosis was made in all cases. CONCLUSIONS: Metabolic imaging with PET-FGD is better than anatomostructural imaging techniques for differential diagnosis between tumour relapse and radionecrosis in CNS tumours which have been treated. Prospective studies are necessary for evaluation of SUV as a factor for prognosis of survival.     

Lipoma of the superficial lobe of the parotid gland. A case report

We report the case of a patient with a lipoma of the superficial lobe of the right parotid gland. Lipomas of the parotid gland are very rare. They are said to constitute one to two percent of all parotid tumours. Only six cases were found in the recent literature. Preoperative diagnosis is difficult when the physical examination reveals a renitent, well-localized and painless mass. However, magnetic resonance imaging is now the imaging modality of choice to facilitate the diagnosis, but the diagnosis can only be confirmed by histological examination of the tumour. We discuss the value of superficial parotidectomy, while some authors suggest an enucleation of lipomas of the superficial lobe of parotid.     

Trends of morbidity in general medicine at United Bulawayo Hospitals, Bulawayo, Zimbabwe

OBJECTIVE: To find out whether recent techniques of investigating hypophyseal adenoma (computed tomography, magnetic resonance imaging and hormonal measurements) have brought about earlier diagnosis. PATIENTS AND METHODS: The time of onset of first symptoms and the duration from first symptoms to diagnosis (anamnestic time) were determined retrospectively from clinical, endocrinological, ophthalmological and localizing data on 517 patients (311 women, 206 men) with hypophyseal adenoma. RESULTS: The most common initial symptom was oligo- or amenorrhoea, in 57.9% of women, and disturbed libido or potency in 38.5% of men. Next most common were visual field deficits (women: 11.6%, men: 26.7%), signs of acromegaly (women: 11.3%, men 18.2%) and headaches (women: 11.3%, men 15.5%). The median anamnestic time in women, 3.0 years, was significantly longer than the 2.3 years in men (P < 0.001). In hormonally inactive adenoma the corresponding times were 2.0 years in women and 1.0 year in men; in prolactinoma 4.2 years and 2.0 years; in growth-hormone secreting adenoma 5.0 years and 5.5; and in ACTH-secreting adenoma 5.0 years and 1.0 year. CONCLUSIONS: Although diagnostic techniques have greatly improved, the anamnestic time for hypophyseal adenoma is not shorter than in earlier studies. This findings underlines the need for clearly directed history-taking and for good training in endocrinology.     

A case of Erdheim-Chester disease with orbital involvement

The Erdheim-Chester disease is a rare idiopathic, systemic, histiocytic disorder. To our knowledge, ocular involvement has been reported in only 16 cases. We describe a 55-year-old man who had symmetrical exophthalmos and several skin nodules on the arms and trunk. A magnetic resonance imaging scan confirmed the presence of bilateral, intraconal, retrobulbar tumors. An examination of the histopathologic features of orbital and skin biopsy specimens revealed xanthogranulomatous infiltrate with Touton giant cells. Further systemic investigations showed bone and retroperitoneal involvement. Three years later, multiple eyelid xanthelasmas developed in the patient. These findings are consistent with the diagnosis of the Erdheim-Chester disease. The patient's condition is stable under therapy with low-dose corticosteroids. His survival is longer than usually described in the literature.     

Subarachnoid hemorrhage as a form of onset of systemic lupus erythematosus

INTRODUCTION: The most frequent cause of spontaneous subarachnoid hemorrhage (SAH) is rupture of intracranial arterial aneurysms (> 70%). The remainder are due to many different aetiologies. Although SAH is a relatively common neuropathological finding in systemic lupus erythematosus (SLE), it is normally due to the extent of the intercerebral hemorrhage and not to its isolated presentation. CLINICAL CASE: We report the case of a 34 year old woman who presented with non-traumatic SAH at the onset of her lupus disorder. The patient was attended for SAH and at the same time a multisystemic disorder and severe thrombocytopenia were found, leading to a diagnosis of SLE. The neuroimaging techniques, selective cerebral arteriography, cerebral and spinal magnetic resonance, and magnetic resonance angiography did not show any vascular malformations. The patient was treated with immunosuppressive therapy, nimodipine, and following angiographic tests, with antiaggregants and anticoagulants. CONCLUSIONS: The greater frequency of SAH in patients with lupus, as compared to the general population, has been attributed to the presence of intracranial vasculitis. However, neuropathological studies have shown that true vasculitis is very infrequent in the central nervous system of SLE patients. In the case we describe, the first in which SAH appeared at the onset of the disease, we consider that the origin of the hemorrhage was her high arterial blood pressure and thrombopenia.     

Acute presentation of leukodystrophy due to mitochondrial cytopathology and multiple deletions of mitochondrial DNA

INTRODUCTION: Deletions of mitochondrial DNA (mtDNA) are a known cause of various mitochondrial cytopathies, which are sporadic and usually not due to maternal transmission. The multiple deletions are usually transmitted on a Mendelian pattern, and are frequently of autosomal dominant character. Leukodystrophy may be part of the picture, or even the form of presentation, of some mitochondrial cytopathies. Thus, in a case of leukoencephaly of unknown origin, mitochondrial cytopathy should be considered in the differential diagnosis. CLINICAL CASE: We present the case of a boy with no previous clinical abnormalities who, at the age of 13, suddenly fell to the floor with an encephalopathy which required aggressive treatment, needing mechanical ventilation and prolonged sedation. Following partial recovery spastic-dystonic quadriplegia remained. Neuroimaging showed advanced leukodystrophy with small hemorrhages in the white matter, which later disappeared. After rejecting other aetiologies, mitochondrial cytopathies in muscle were studied. A partial defect of the I and IV complexes of the respiratory chain and two deletions of mtDNA were shown. CONCLUSIONS: This case is another example of the variable clinical presentation of mitochondrial cytopathies and yet another argument for their inclusion in the diagnosis of leukodystrophy of unknown origin.     

The localization of aldosterone-producing adenoma on computed tomography--a comparative study with adrenal scintigraphy and plasma aldosterone concentration in the adrenal or renal vein

The evaluation of computed tomography (CT) for detecting aldosterone-producing adenoma in primary aldosteronism was performed by comparison with adrenal scintiscan; determination of aldosterone in adrenal or renal veins, retroperitoneal pneumography and adrenal venography was reliable for diagnosis of adrenal tumors in pheochromocytoma or Cushing's syndrome, but not so effective for small adenoma of primary aldosteronism. An abdominal CT scan was performed on six patients with primary aldosteronism, one with idiopathic hyperaldosteronism and one with glucocorticoid responsive hyperaldosteronism; in an attempt to evaluate the utility of this noninvasive procedure. Diagnosis of hyperaldosteronism was made by demonstrating the elevated plasma aldosterone concentration and aldosterone secretion rate, normal excretion rate of urinary 17-OHCS and 17-KS, and low plasma renin activity. The CT scan correctly predicted unilateral adrenal adenoma in all the patients with primary aldosteronism of which the findings were identical to those demonstrated by surgery. The diameter of these tumors ranged from 10 X 7 X 6 to 19 X 17 X 14 mm. Also the CT scan in idiopathic hyperaldosteronism and glucocorticoid responsive hyperaldosteronism showed bilateral adrenal hyperplasia and bilateral normal adrenal glands, respectively. The pathological findings in these two cases disclosed the adrenal hyperplasia of zona glomerulosa and adrenal hyperplasia of zona subglomerulosa accompanied by a normal thickness of the adrenal gland, respectively. The precision of the CT scan, adrenal scintigraphy and determination of plasma aldosterone in the adrenal or renal veins were almost equal to the diagnosis of the localization of adrenal adenoma. It is concluded that the CT scan is a noninvasive and most useful method for the localization of aldosterone-producing adenoma and helpful in distinguishing adrenal adenoma from adrenal hyperplasia.     

Middle-ear lipoma as a cause of otomastoiditis

We present a case report which describes a rare cause of a common clinical problem; eustachian tube dysfunction. A seven-year-old child presented with a history of chronic draining ears, despite rigorous medical therapy and multiple ventilation tubes. At myringotomy a mass was noted in the middle ear, and she was taken to the operating room for exploration. The patient was found to have a pedunculated lipoma arising from the anterior medial aspect of the middle-ear cleft producing intermittent obstruction of the eustachian tube orifice. This case represents the fourth case of a middle-ear lipoma in the world literature. We present a review of the literature and an exploration of possible aetiologies of this unusual entity in the differential diagnosis of eustachian tube dysfunction.     

Carpal tunnel syndrome in childhood. A case of early onset

INTRODUCTION: The carpal tunnel syndrome (CTS) is the commonest neuropathy due to compression to be seen in adults. There are very few cases in the literature referring to patients of paediatric age, particularly those under ten years old. Most of these young patients had a metabolic disorder (mucopolysaccharidosis (MPS) or mucolipidosis (ML). In fact, as many as 90% of the MPS had CTS, sometimes subclinically. This syndrome is caused by compression of the median nerve at the level of the carpal tunnel, to which multiple factors may contribute, both local and systemic, as reviewed in this paper. The clinical findings differ from those in adults, but the appearance of suggestive symptoms and signs should make one suspect the condition and request an electromyographic study (EMG) which would be diagnostic. CLINICAL CASE: We describe the case of a five year old girl, with a clinical history suggesting the presence of a carpal tunnel syndrome for 12 months and characterized by paraesthesia and limitation of flexon-extension movements of the fingers of the affected hand, with pain on movement. The symptoms appeared on waking in the morning, gradually improved as the day advanced and became bilateral over a period of six months. The diagnosis was confirmed by EMG and MR helped to clarify the aetiology. CONCLUSION: The interesting aspect of this article is the youth of the patient, the absence of known etiological factors and the presence of tenosynovitis detected on MR as has been described in some idiopathic/familial forms.     

Radioisotopic study of the adrenal glands using 131I-19-iodocholesterol

Scintigraphy of the adrenal gland with 131I-19-iodocholesterol has recently been added to radiological techniques in adrenal imaging and has been used successfully to demonstrate anatomical and functional disorders of the adrenals in a variety of clinical situations. A review of the authors' experience stresses the diagnostic value of this method. Radiological findings and results of scintillation imaging are complementary: their comparison improves and clarifes indications for scintigraphy. Hyperadrenal cortical diseases always gave satisfactory scintigrams, the most interesting results being obtained in adrenal cortical hyperplasia and unilateral hyperfunctioning adenomas. In these cases the evaluation of the response to stimulation or suppression tests was very useful. On the other hand scintigraphy was less valuable in demonstrating malignant and non malignant tumours.     

Magnetic resonance imaging of posterior pituitary for evaluation of the neurohypophyseal function in idiopathic and autosomal dominant neurohypophyseal diabetes insipidus

We investigated the role of MR imaging for evaluation of the functional status of the neurohypophyseal system in both idiopathic central diabetes insipidus (DI) and familial autosomal dominant neurohypophyseal DI. The patients and family with DI were analyzed retrospectively for the presence or absence of posterior pituitary gland hyperintense signal on MR images. A total of 19 adult patients with idiopathic central DI, 7 members of a family with autosomal dominant DI and 20 control subjects were included in the study. Diagnosis of idiopathic DI was based on the presence of central DI in the absence of any alteration that is known to be responsible for DI. The patients were studied retrospectively and the morphology and intensity of the posterior lobe by MR imaging was assessed by blinded reading. In all patients with idiopathic central DI and the affected members of the family, the posterior bright signal was absent while the stalk was normal on MR images. In contrast, normal posterior pituitary bright signal and stalk were found in unaffected members of the family and all control subjects. We conclude that MR imaging of the posterior pituitary lobe can be used to evaluate the functional status of the neurohypophyseal system in idiopathic central DI and familial autosomal dominant DI.     

The hypereosinophilic variant of Ph''-positive chronic myeloleukemia

AIM: To confirm clonal nature of idiopathic hypereosinophilic syndrome (IHES), its relevance to Ph'-positive chronic myeloid leukemia. MATERIALS AND METHODS: 3 cases of idiopathic hypereosinophilic syndrome are reported with morphologic analysis of bone marrow cells and cytogenetic examinations. In one patient the presence of Ph'-chromosome was confirmed at fluorescent in situ hybridization (FISH) and molecular-genetic analysis (bcr/abl). Samples of bone marrow, spleen and liver were examined pathohistologically. RESULTS: The presence of chromosome anomaly t(9;22), i.e. Ph'-chromosome, associated with chronic myeloid leukemia (CML) was identified in all the 3 cases. There was also myeloid hyperplasia in the bone marrow (with primarily mature, eosinophilic granulocytes), spleen and liver, depression of megakaryocyto- and erythropoiesis. 2 patients had similar clinical symptoms which was not typical for CML in chronic phase: fever, elevated ESR, clear-cut anemia and thrombocytopenia. In the absence of hyperleukocytosis, blood and bone marrow eosinophils remained high (42.5, 21.5, 42.5% and 21.4, 7.1, 6.5%, respectively) due to "mature" forms. The number of blasts in the bone marrow was maximum 2.4%. CONCLUSION: The literature and the obtained data suggest closeness of idiopathic hypereosinophilic syndrome and Ph'-positive CML within myeloproliferative diseases.     

Splenomegalic pancytopenia of probable immune origin: cure after treatment with glucocorticoids

Two female patients of 54 and 48 years, respectively, with pancytopenia and splenomegaly of probable immune origin are reported. The first presented anemic syndrome and fever, and the second bleeding. In both cases large sized splenomegaly was found on physical examination. Serologic and immunologic studies excluded chronic infection or systemic autoimmune disease. Bone marrow biopsy showed hematopoietic hyperplasia with lymphoid nodules and reticulin fibrosis. Treatment with glucocorticoids and splenectomy was performed in one case and only glucocorticoids were administered in the other case with the cytopenias disappearing and the bone marrow becoming normocellular. Reactive follicular hyperplasia was observed in the white pulp of the spleen in one of the patients, as were abundant macrophagic activity in the red pulp, and myeloid metaplasia. The patients remain asymptomatic at 4 and 12 years of follow up. The origin of the process was attributed probably to an immune etiology and differential diagnosis with idiopathic myelofibrosis and non tropical idiopathic splenomegaly must be considered.     

Oncocytic lesions of the caruncle and other ocular adnexa

This is a clinicopathologic study of 18 oncocytic lesions involving the ocular adnexa. These lesions most commonly occur in elderly patients (median age 73), and there is a striking predilection for women. The broad spectrum of lesions included oncocytic adenomatous hyperplasia, oncocytomas, and oncocytic carcinoma. Ten lesions, all of which were oncocytomas, involved the caruncle, four the conjunctiva, and one the mucocutaneous junction of the eyelid. Two tumors involved the lacrimal sac and one the lacrimal gland. A review of the literature disclosed only 17 previously reported oncocytic lesions of the ocular adnexa. A small yellowish-tan or reddish caruncular mass, especially in an elderly woman, should alert the ophthalmologist to consider the possibility of oncocytoma in his differential diagnosis.     

Spontaneous perinephric hematoma in a case of AIDS

OBJECTIVE: To review the wide spectrum of urological manifestations in the HIV-infected population. METHODS: We report a case of spontaneous perinephric hematoma (non-traumatic) in a young male drug user with HIV infection. The patient was submitted to emergency nephrectomy after acute bleeding secondary to a subcapsular hematoma. He had no previous history of trauma. RESULTS: No underlying disorder was found and it is not known if the association with HIV infection is merely casual or not. This is an uncommon condition that is usually secondary to benign or malignant renal tumors, vascular anomalies and inflammatory disorder. In some cases, however, the underlying cause is never found. CONCLUSION: It must be emphasized that HIV-infected patients may consult for diverse urological disorders whose differential diagnosis may be difficult to establish.     

The relationship between serum PSA value and the weight of the inner gland of the prostate in the cases of benign prostatic hyperplasia

(BACKGROUND): To study reasons why serum PSA value (Markit-F) elevates in benign prostatic hyperplasia (BPH). (METHODS): The relationship between total prostatic weight and the outer gland weight in 48 cases of BPH undergoing subcapsular prostatectomy was studied by ultrasound measurement. PSA was compared with prostatic weight, inner gland weight, outer gland weight, growth speed of the inner gland and age in the 48 cases. (RESULTS): 1) The weight of the outer gland increased slightly in proportion to the development of BPH. However, the increase was not significant statistically. 2) PSA correlated with the inner gland weight and growing speed of the inner gland well. 3) PSA did not correlate with the outer gland weight and age. (CONCLUSIONS): It is well known that PSA elevates not only in the cases of prostatic cancer but also in the cases of BPH and that PSA correlates with the whole prostatic weight. This study revealed that the elevation of PSA in the cases of BPH was caused by enlargement of the inner gland.