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Resume L’atteinte renale au cours de la sarcoidose est rare et le plus souvent la consequence de troubles du metabolisme calcique ou d’une atteinte granulomateuse interstitielle. L’atteinte glomerulaire reste exceptionnelle. Il peut exister une longue periode de latence entre le developpement d’une sarcoidose active et l’atteinte glomerulaire et inversement. Nous rapportons un cas de glomerulonephrite extra-membraneuse revelant une sarcoidose systemique.

Resume Introduction La survenue successive d’une tamponnade et d’une myocardite au cours d’un syndrome de Churg-Strauss (SCS) est exceptionnellement decrite. Nous rapportons l’observation d’une patiente ayant un SCS revele par une tamponnade et une myocardite. Observation Une patiente, âgee de 58 ans, etait hospitalisee pour une alteration de l’etat general associe a une hypereosinophilie sanguine. Elle etait admise, un mois auparavant, pour une pericardite aigue compliquee d’une tamponnade, traitee par une ponction pericardique. Au cours de sa deuxieme hospitalisation, elle presentait des douleurs thoraciques en rapport avec une myocardite aigue. L’enquete etiologique concluait a un SCS complique d’une double atteinte cardiaque. Une bonne evolution clinicobiologique etait obtenue apres traitement corticoide et immunosuppresseur. Conclusion L’atteinte isolee ou multiple des tuniques cardiaques doit faire rechercher une vascularite systemique. Un bilan initial complet et une surveillance etroite des patients suivis pour un SCS s’impose afin de detecter une atteinte cardiaque et mettre en place un traitement precoce.

Resume Propos La survenue d’une pancreatite aigue au cours du lupus erythemateux systemique (LES) est connue mais rare, et exceptionnellement revelatrice. Sa pathogenie est multifactorielle ; il est extremement difficile de faire la part de ce qui revient a la vascularite, aux phenomenes thrombotiques dans le cadre d’un syndrome des antiphospholipides, ou aux complications iatrogenes ou intercurrentes. Methodes Etude monocentrique retrospective portant sur 110 patients ayant un LES. Le diagnostic de pancreatite lupique a ete retenu en l’absence d’autre cause de pancreatite : lithiase biliaire, toxiques (medicaments ou intoxication ethylique), traumatique et neoplasiques. Resultats Au sein d’une cohorte de 110 patients atteints de LES, six avaient une atteinte pancreatique (5,4 %). Il s’agissait de cinq femmes et un homme âges en moyenne de 36,3 ans. L’atteinte pancreatique etait concomitante du diagnostic de LES chez quatre patients et observee au cours de l’evolution chez les deux autres. Elle etait dans tous les cas associee a d’autres manifestations viscerales. Cliniquement, l’atteinte pancreatique etait toujours symptomatique avec des douleurs epigastriques dans tous les cas, des vomissements dans trois cas et de la fievre dans trois cas. Les enzymes pancreatiques etaient elevees dans tous les cas. Les anticorps antinucleaires et anti-ADN natifs etaient toujours positifs. Les anticorps anti-Sm et anti-RNP etaient positifs respectivement dans cinq et un cas. Les anticorps antiphospholipides etaient positifs dans deux cas. L’evolution etait favorable dans cinq cas. Un patient decedait. Conclusion La pancreatite lupique est rare mais peut reveler le LES. Ses causes sont multifactorielles et son evolution le plus souvent favorable.

Resume L’amylose limitee a l’appareil bronchopulmonaire s’exprime sous forme de depots tracheobronchiques en plaques diffuses pouvant entrainer des stenoses ou des nodules ou masses parenchymateuses. A ce propos, nous rapportons quatre observations d’amylose bronchopulmonaire et nous discuterons les difficultes diagnostiques de cette localisation. Il s’agit de deux femmes et de deux hommes âges respectivement de 60, 68, 44 et 57 ans. Ils ont presente une amylose pulmonaire parenchymateuse dans tous les cas associee a une atteinte bronchique dans un cas. Le diagnostic a ete confirme par l’etude histologique dans tous les cas. Le bilan d’extension etait negatif dans tous les cas. L’evolution a ete marquee par la stabilisation des lesions dans tous les cas. L’amylose localisee, pouvant etre le seul signe revelateur d’une atteinte systemique, son diagnostic impose la recherche d’autres localisations pour mieux adapter la strategie therapeutique.

Propos L’apparition d’une pancreatite aigue au cours du lupus erythemateux systemique (LES) est connue mais rare, et exceptionnellement revelatrice. Sa pathogenie est multifactorielle; il est extremement difficile de faire la part de ce qui revient a la vascularite, aux phenomenes thrombotiques dans le cadre d’un syndrome des antiphospholipides, ou aux complications iatrogenes ou intercurrentes.

Primary cutaneous aggressive CD8+ T-cell lymphoma is an extremely rare entity with distinct clinicopathological features. We report a case of a 28-year-old man that clinically presented with an ulcerated nodule on his trunk and extremities. Histopathologic sections showed an ulcerated epidermis with a diffuse lymphocytic infiltrate in the superficial dermis with focal epidermotropism. Immunohistochemically, the tumor cells were positive for CD3 and CD8 but negative for CD4, CD20, CD30 and CD56. Granzyme B was positif. He received UVB therapy subsequently associated to interferon with an important regression of the nodules.

Resume Introduction La mucormycose est une infection fungique rare, rapidement extensive, touchant avec predilection les diabetiques non controles, notamment en acidocetose. A ce propos, les auteurs rapportent l’observation d’une mucormycose a localisation cervicale avec extension a la glande parotide chez une femme diabetique d’evolution particulierement favorable. Observation Femme âgee de 54 ans, diabetique type 2, ayant presente une masse laterocervicale gauche qui a ete operee. Le diagnostic de mucormycose a ete fait lors de l’examen anatomopathologique. La patiente a ete traitee par amphotericine B avec une evolution favorable. Conclusion La mucormycose est une infection rare. Les formes cliniques sont multiples. Le traitement est medicochirurgical. Le pronostic est severe avec un taux de mortalite globale de 40 %.

Resume Introduction La sarcoidose est une maladie systemique d’etiologie inconnue qui affecte de nombreux organes et notamment le foie. Methodes Il s’agit d’une etude retrospective monocentrique sur 12 ans des patients suivis pour sarcoidose systemique et presentant une atteinte hepatique sarcoidosique. Analyse descriptive des donnees cliniques, paracliniques et de l’evolution. Resultats Parmi 21 patients suivis, sept presentaient une atteinte hepatique. Il s’agissait de cinq femmes et deux hommes, d’âge moyen 42,5 ans. L’atteinte hepatique revelait la sarcoidose dans trois cas. Une hepatomegalie clinique etait notee chez cinq patients et des anomalies biologiques chez quatre. Dans une observation, on notait une hepatomegalie nodulaire sur la tomodensitometrie. L’histologie hepatique montrait chez six patients une granulomatose hepatique typique. Un patient developpait un syndrome de Budd-Chiari d’evolution favorable sous corticotherapie. Six des sept patients ont recu une corticotherapie. Conclusion L’atteinte hepatique au cours de la sarcoidose est frequente et le plus souvent asymptomatique. Son traitement n’est pas systematique.

Islet-cell tumors are the most common neuroendocrine tumors that arise from the endocrine pancreas. They are typically benign and sporadic. Diagnosis is generally established late because clinical signs lack specificity. The insulinoma is difficult to localize since it is very small in size, often not exceeding 2 cm. We report an exceptional case of giant insulinoma initially revealed by a pseudo-polycythemia in an 80-year-old man. He had been treated for hypertension for a few months. Routine biological investigations showed elevated hematocrit and haemoglobin, suggesting Vaquez disease. History taking revealed recent episodes of nocturnal agitation. On admission, he had reddish skin with a suspected enlarged spleen, but total blood volume was normal. Imaging studies showed a voluminous tumor located between the pancreas and the spleen. The presence of an insulinoma was confirmed on the basis of an elevated level of proinsulin at the time of an asymptomatic episode of hypoglycemia. Spleno-pancreatectomy was performed. Histopathological examination revealed a malignant, well-differentiated neuroendocrine malignant tumor.

Resume Introduction. – L'association d'une hypothyroidie primaire a un syndrome tumoral hypophysaire avec hyperprolactinemie est bien connue. Par ailleurs, l'hypothyroidie peut s'associer a des ovaires multikystiques chez les jeunes filles. Exegese. – Nous rapportons l'observation d'une femme âgee de 30 ans, aux antecedents d'anemie ferriprive, hospitalisee pour un syndrome clinique et biologique d'hypothyroidie primaire associe a une hyperprolactinemie. L'IRM hypophysaire a montre une masse tumorale intrasellaire. De plus, une masse ovarienne kystique cloisonnee droite est decouverte par l'echographie et la TDM abdominopelviennes. Sous traitement hormonal substitutif, l'evolution est marquee par une disparition progressive des masses ovarienne et hypophysaire. Il s'agit donc d'un syndrome tumoral hypophysaire et ovarien reactionnels a l'hypothyroidie peripherique. Conclusion. – La connaissance de ce type d'association doit faire penser devant une masse ovarienne et/ou hypophysaire a l'hypothyroidie et permet d'eviter une chirurgie mutilante chez les femmes en âge de procreation.

But Le syndrome des antiphospholipides est defini par l’association d’au moins un evenement clinique (thrombose arterielle et/ou veineuse, pertes fœtales) a la presence d’anticorps diriges contre les phospholipides. Il peut etre associe au lupus systemique ou survenir isolement. Le but de cette observation est de montrer que l’atteinte vasculaire oculaire peut etre revelatrice de ce syndrome et que son diagnostic est d’autant plus important que le pronostic oculaire et meme vital de ces patients souvent jeunes peut etre en jeu. Observation Nous rapportons le cas d’une jeune fille âgee de 20 ans, suivie et traitee pour lupus systemique depuis deux ans, qui, suite a l’arret brutal de son traitement medical, a presente une baisse soudaine et bilaterale de l’acuite visuelle. L’examen du fond d’œil et l’angiographie retinienne a la fluorescence ont revele une occlusion vasculaire retinienne severe et bilaterale. Le syndrome des antiphospholipides a ete pose devant l’apparition d’anticorps anti-cardiolipines. Sous traitement, l’evolution a ete marquee par une legere amelioration fonctionnelle oculaire au debut, suivie d’une degradation de l’etat general au bout de 3 mois avec deces de la patiente dans un tableau d’embolie pulmonaire grave. Commentaire et conclusion Une occlusion d’artere ou de veine centrale de la retine chez le sujet jeune doit faire rechercher le syndrome des antiphospholipides. La bilateralite de l’occlusion vasculaire constitue un facteur de gravite de par ses consequences sur le pronostic fonctionnel visuel mais aussi vital puisqu’elle peut etre le signal d’alarme de l’extension de la maladie a d’autres vaisseaux de l’organisme. Une occlusion vasculaire retinienne severe du sujet jeune doit faire rechercher un syndrome des antiphospholipides, et ce d’autant plus que le pronostic fonctionnel et surtout vital peut etre en jeu.

We report an unusual case of Wegener's granulomatosis revealed by spleen infarction and complicated by reno-vascular hypertension. A 33-year-old man with a history of spleen infraction and cerebral venous thrombosis was admitted for malignant hypertension, renal failure and nephritic syndrome. On Doppler renal ultrasonography, ostial stenosis of the right renal artery was evidenced. Right kidney was non functional on scintigraphy and the patient underwent a right nephrectomy. Granulomatous vasculitis of the right renal artery was found but anti-neutrophilic cytoplasmic antibodies were absent. The diagnosis of Wegener's granulomatosis was suspected. The patient was treated with cyclophosphamide, corticosteroids and plasma exchanges, and renal function markedly improved.

Introduction L’embolie pulmonaire aigue (EPA) constitue une urgence diagnostique et therapeutique frequente et grave. L’objectif de notre etude etait de preciser les caracteristiques epidemiologiques, cliniques, etiologiques et evolutives des patients ayant une thrombose veineuse profonde (TVP) compliquee d’une embolie pulmonaire (EP). Materiels et Methodes Il s’agit d’une etude retrospective qui concerne les patients hospitalises dans le service de medecine interne de l’hopital Habib Thameur pour thrombose veineuse entre janvier 2000 et decembre 2014. Les patients etaient repartis en 2 groupes selon l’existence (groupe 1) ou non (groupe 2) d’une EP. Nous n’avons etudie que les dossiers ou le diagnostic d’EP a ete confirme ou infirme par un angioscanner et/ou une scintigraphie pulmonaire. Les caracteristiques epidemiologiques, cliniques, etiologiques et evolutives ont ete comparees entre les 2 groupes au moyen des tests de X2, avec un seuil de signification p Resultats Durant cette periode, 268 cas ont ete colliges. Une EP a complique l’evolution de 58 patients (21,6 %). Il s’agissait de 21 hommes et de 37 femmes d’âge moyen de 50,24 ans (extremes de 16 et 84 ans). Les facteurs de risque de la thrombose les plus associes dans le groupe 1 (G1) etaient : l’obesite (37,9 %), l’alitement (27,6 %), les antecedents d’accidents thromboemboliques (15,5 %) et l’intervention chirurgicale (8,6 %). Les etiologies les plus frequentes dans G1 etaient dominees par les thrombophilies constitutionnelles (20,7 %), l’origine neoplasique (17,2 %) l’hyperhomocysteinemie (10,3 %), les maladies inflammatoires (5,2 %), le syndrome des antiphospholipides (7 %) et les causes infectieuses (3,4 %). L’EP etait restee sans etiologie malgre une enquete etiologique exhaustive dans 24,6 % des cas. La duree moyenne de traitement dans G1 etait de 14 mois. L’evolution apres un traitement anticoagulant etait marquee par la regression de l’EP (20 %), la recidive (8,6 %) et le deces (2 %). La comparaison des differents parametres des 2 groupes n’avait pas objective de differences statistiquement significatives en ce qui concerne l’âge, le sex-ratio, les etiologies sous-jacentes et le profil evolutif. Seuls ceux qui ont eu des antecedents d’accidents thromboemboliques sont plus exposes a la survenue d’une EP (p : 0,03). Conclusion L’EP est une affection frequente et grave et dont la pathogenie est multifactorielle. Une bonne connaissance des facteurs favorisants ou des maladies associees est indispensable. En effet, notre etude a demontre que le caractere recidivant d’une TV est un facteur de risque important d’EP et qu’elle justifie une duree de traitement et une surveillance beaucoup plus prolongees.

Renal involvement in sarcoidosis is rare and more often related to calcium metabolism disorders or granulomatous interstitial nephritis. Glomerulonephritis is exceptional. There may be a long latency period between the development of active sarcoidosis and glomerular involvement and inversely. We report a case membranous glomerulonephritis revealing systemic sarcoidosis.

The successive occurrence of pericardial tamponade and myocarditis during a Churg-Strauss syndrome is exceptionally described. We report a patient in whom pericardial tamponade and myocarditis were the presenting manifestation of a Churg-Strauss syndrome.A 58-year-old woman was admitted because of alteration of the clinical status with eosinophilia. One month ago, she was hospitalized for a pericardial tamponade treated by pericardial drainage. Acute myocarditis was diagnosed on chest pain during the second hospitalization. The etiologic inquiry ended in the diagnosis of Churg-Strauss complicated with a double cardiac involvement. A good response of clinical and biological anomalies was obtained after corticosteroid and immunosuppressive treatment.Isolated or multiple involvements of cardiac tunics should lead to make diagnosis of systemic vasculitis. A complete initial assessment and a close observation of the patients followed for Churg-Strauss syndrome is imperative to detect a cardiac achievement and set up an early treatment.

Pulmonary artery aneurysm in Behcet disease compromises seriously the diagnosis since its fatal rupture is still the major cause of death in this disease. Pulmonary artery aneurysm requires urgent management and several treatments have been proposed, including steroids, immunosuppressive drugs, embolisation and surgery. Herein, we report a patient with Behçet's disease in whom multiple pulmonary artery aneurysms were completely resolved after a combined treatment with corticosteroids and azathioprine. Pulmonary artery aneurysms in Behçet's disease are reported to indicate poor prognosis and high mortality. Computed tomographic scans of the chest and angiography are the most common diagnostic procedures used in the diagnosis or evaluation of pulmonary artery aneurysm. Immunosuppression is the main therapy for the treatment of a vasculitis.

Insulinoma is a rare tumor. Its annual incidence varies from 1 to 4 cases per million in the United States [1–3] but has not been determined in the different populations. We conducted a retrospective study to evaluate the incidence, the epidemiological characteristics, and the prognosis of insulinoma in Tunisia. Our study is the first to determine the annual incidence of insulinoma in Tunisia. It concerned all cases of insulinomas diagnosed in Tunisia between April 1980 and April 2009. Search was carried out in all university centers including endocrinology, internal medicine, pediatric, and surgery departments in Tunis (6 centers), Sfax, Sousse, Monastir, and Mahdia. We analyzed epidemiological data and patient’s medical records. Over the 29 years period, 21 patients with confirmed insulinoma were identified. So, the annual incidence of insulinoma in Tunisia was 0.1 case per million. This low incidence may be an underestimate because of the difficulties to find out all the cases of insulinoma. The patients were 10 females (47.6%) and 11 males (52.3%). Mean age at diagnosis was 46 years [8–80]. There was only one child in the series. Sex ratio and age at diagnosis were close to that described in other studies [1–5]. Mean diagnosis delay was 5 ± 6.9 years ranging from 4 months to 30 years. The initial presentation was neurogenic symptoms in 13 cases (62%), among them five (24%) with recurrent seizure. Insulinoma was diagnosed in a 49 years old woman with type 2 diabetes known for 14 years. Multiple endocrine neoplasia type I was not reported in our cases. Twenty patients were operated and follow-up data were available in 19 cases. The tumors were located at the head in 4 cases, at the body in 7 cases, and at the tail in 8 cases. The mean diameter was 25 mm, ranging from 3 to 115 mm. Eleven tumors measured 2 cm or more (52%). Insulinoma was multiple in one case (5%). Malignancy was present in three cases (18.7%). The operative mortality was 5.2% (one death secondary to biliary leakage). The recovery rate was 68.4% (13/19). Persistent hypoglycemia was reported in two cases (10.5%) among which one malignant insulinoma with metastasis. Recurrence was noted in three cases. With the results of this study, we can conclude that the incidence of insulinoma seems to be low in Tunisia. Due to the variability and the lack of specificity of clinical presentation, diagnosis of insulinoma was frequently delayed as indicated by the large size of the tumors. Malignancy was frequent. The cure rate was unsatisfactory. M. Chihaoui (&) R. Hmaissi H. Slimane Department of Endocrinology, La Rabta Hospital, Tunis, Tunisia e-mail: mm.chihaoui@planet.tn

Mucormycosis is a rare, devastating, fungal infection, which disproportionately affects non-controlled diabetic patients, notably during ketoacidosis. The authors report the case of cervical mucormycoses with a particularly favorable evolution in diabetic woman.A 54-year-old woman, type 2 diabetic, had presented a left lateral cervical mass. The diagnosis was confirmed by histological examination. She was treated with Amphotericin B with favorable evolution.The mucormycose is a rare infection. The treatment is medical and surgical. The prognosis is severe with an overall mortality rate of 40%.

The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis.This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n=6), vomiting (n=3), and fever (n=3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died.Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.

Amyloidosis limited to bronchopulmonary apparatus is expressed as tracheobronchial deposits in diffuse plaques that can cause stenosis or parenchymal nodules or masses. In this regard, we report four cases of pulmonary amyloidosis and discuss the diagnostic difficulties of this location. These are two women and two men aged 60, 68, 44 and 57 years. They presented a pulmonary parenchymal amyloidosis in all cases associated with bronchial in one case. The diagnosis was confirmed by histology in all cases. The staging was negative in all cases. The evolution was marked by the stabilization of the lesions in all cases. Localized amyloidosis, which may be the only telltale sign of a systemic illness, its diagnosis requires finding other locations to better tailor the treatment strategy.

To investigate the etiologies of the upper limb digital necrosis based on a retrospective analysis of 25 cases.We retrospectively reviewed the medical records of patients treated for digital necrosis of the upper limb in four departments of internal medicine from January 1997 to December 2003.There were 16 women and nine men, mean age 55 years. Eleven patients were smokers. Raynaud's phenomenon was noted in 12 cases. Connective tissue diseases were the most common cause (nine cases), all of them were women. The second cause was atherosclerosis (five cases) and Buerger's disease (five cases). In the other cases, the following diagnoses were found: vasculitis (three cases) and neoplasm (two cases). No cause could be identified in one female smoker.Digital necrosis is a common symptom, revealing a vascular pathology. Its causes are diverse. In women, it first suggests a connective tissue disease whereas in men, a diffuse arteriopathy. The etiological diagnosis strategy should consider drug intake, anamnesis and Raynaud's phenomenon history. However, in all cases the etiology investigations should not delay the treatment in order to preserve functional prognosis.

We report an unusual case of Wegener's granulomatosis revealed by spleen infarction and complicated by reno-vascular hypertension. A 33-year-old man with a history of spleen infraction and cerebral venous thrombosis was admitted for malignant hypertension, renal failure and nephritic syndrome. On Doppler renal ultrasonography, ostial stenosis of the right renal artery was evidenced. Right kidney was non functional on scintigraphy and the patient underwent a right nephrectomy. Granulomatous vasculitis of the right renal artery was found but anti-neutrophilic cytoplasmic antibodies were absent. The diagnosis of Wegener's granulomatosis was suspected. The patient was treated with cyclophosphamide, corticosteroids and plasma exchanges, and renal function markedly improved.

Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the absence of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by neoplasm or interstitial pneumonia that shows a rapid progressive course both of them are associated with a poor prognosis. A 56-year-old woman with no medical history was referred to the department of medicine because of arthralgia with a remarkable weight loss. She also complained of rapidly progressive dyspnea, cough and photosensitivity. Physical examination on admission showed scaly erythema on the dorsum of the hands (Gottron sign) and periorbital edema with a purplish appearance (heliotropic rash), arthritis, but no muscle weakness. Auscultation of the chest identified audible fine crackles on the lower aspects of both lungs. Results of laboratory findings on admission revealed a lymphopenia. The serum creatine kinase and serum lactate dehydrogenase concentration were normal. IRM muscle and electromyography were normal. Antinuclear antibody was positive 1:80 and anti-Jo-1 antibody and other autoantibodies to specific antigens were all negative. High resolution computed tomographic chest scans also revealed diffuse ground-glass opacities in both lungs with basilar predominance. Arterial blood gas analysis revealed hypoxia and hypocapnia. LBA was not performed because of the deterioration of respiratory symptoms. There was no neoplasm associated. The diagnosis of ADM complicated with ADM rapidly progressive interstitial pneumonia was made. Despite of IV methylprednisolone pulse therapy (1g*day-1 for 3 days) and cyclophosphamide, she died by respiratory failure.

Sarcoidosis is a systemic disorder of unknown aetiology that may involve many organs including the liver.This is a monocentre retrospective and descriptive study over 12 years of patients with systemic sarcoidosis who presented with liver involvement.Amongst 21 patients with systemic sarcoidosis, seven presented a liver involvement (five males and two females; mean age 42.5 years). The liver involvement was the presenting manifestation in three. Liver enlargement was noted in five patients and abnormal liver function tests in four. In one patient, abdominal computed tomographic scan showed a nodular hepatomegaly. Liver histology demonstrated typical granulomatous lesions in six. One patient developed a Budd-Chiari syndrome that responded favourably to corticosteroids. Six out of the seven patients were treated with corticosteroids.Liver involvement in common in sarcoidosis and commonly asymptomatic. Treatment is not systematic.

Islet-cell tumors are the most common neuroendocrine tumors that arise from the endocrine pancreas. They are typically benign and sporadic. Diagnosis is generally established late because clinical signs lack specificity. The insulinoma is difficult to localize since it is very small in size, often not exceeding 2cm. We report an exceptional case of giant insulinoma initially revealed by a pseudo-polycythemia in an 80-year-old man. He had been treated for hypertension for a few months. Routine biological investigations showed elevated hematocrit and haemoglobin, suggesting Vaquez disease. History taking revealed recent episodes of nocturnal agitation. On admission, he had reddish skin with a suspected enlarged spleen, but total blood volume was normal. Imaging studies showed a voluminous tumor located between the pancreas and the spleen. The presence of an insulinoma was confirmed on the basis of an elevated level of proinsulin at the time of an asymptomatic episode of hypoglycemia. Spleno-pancreatectomy was performed. Histopathological examination revealed a malignant, well-differentiated neuroendocrine malignant tumor.

Hyperprolactinemia and a pituitary mass syndrome can occur in patient with primary hypoyhroidism. In young women, hypothyroidism can be associated with ovarian cysts.We report a case of a 30 year-old women who was treated for an anemia. She had clinical and biological signs of primary hypothyroidism with hyperprolactinemia at the biology. Pituitary MRI showed a pituitary mass. The pelvic ultrasound examination and CT scan showed a right ovarian cyst. The subsequent thyroid hormone replacement was associated with a clinical, a biological and radiological resolution. It was a pituitary and ovarian mass syndrome secondary to primary hypothyroidism.The recognition of these associations may eliminate unnecessary surgery and lead to the choice of hormone replacement therapy.

The role of exogenous estrogen in the initiation and maintenance of human systemic lupus erythematosus (SLE) remains very controversial. To review the current literature of the safety of using exogenous estrogens in patients with SLE, a Medline search for articles published between 1970 et 2004 regarding this relationship was performed. Although cohort studies suggest an increase in the incidence of SLE with both oral contraceptives and hormone replacement therapy, recent retrospective studies suggest that the risk of flares is not increased with hormone replacement therapy. Large prospective double blind placebo controlled studies inclusive of all ethnic groups such as the Safety of Estrogen in Lupus Erythematosus National Assessment (SELENA) trial had to provide the basis for definitive recommendations but it had been interrupted after WHI study results.

This study shows the contribution of MRI to diagnosis and follow up of the cerebral lesions in neuro-lupus. Ten cases of neuro-lupus have been reported. The affected patients are 7 women and 3 men having an average age of 34 years. For 8 patients, MRI revealed lesions that were not detected using computed tomography. These lesions were high signal zones on T2, scattered with predilection to deep and sub-cortical cerebral white matter. MRI has also shown cortical and sub-cortical cerebral atrophy for 3 cases, cortical and deep infarcts for 2 cases and both atrophy and infarcts in one case. The MRI diagnosis was normal for the two remaining patients. MRI contributes to the diagnosis confirmation and localization of the cerebral lesions and to the follow-up. In spite of MRI sensitivity, the detection of these lesions remains non specific to neuro-lupus. New methods of exploration as the spectroscopy by magnetic resonance and the cerebral scintigraphy with Technetium 99m could be very helpful in diagnosis of cases for which MRI has shown limitations.